cardiovascular-agents and Heart-Defects--Congenital

Topics: Age Factors; Cardiovascular Agents; Heart Defects, Congenital; Heart Failure; Humans; Risk Factors; Survivors; Treatment Outcome

Patients with systemic morphological right ventricles (RVs), including congenitally corrected transposition of the great arteries and dextro-transposition of the great arteries with a Mustard or Senning atrial baffle repair, have a high likelihood of developing systemic ventricular dysfunction. Unfortunately, there are a limited number of clinical studies on the efficacy of medical therapy for systemic RV dysfunction. We performed a systematic review and meta-analysis to assess the effect of angiotensin-converting enzyme (ACE) inhibitors, angiotensin-receptor blockers (ARBs), beta blockers, and aldosterone antagonists in adults with systemic RVs. The inclusion criteria included age ≥18 years, systemic RVs, and at least 3 months of treatment with ACE inhibitor, ARB, beta blocker, or aldosterone antagonist. The outcomes included RV end-diastolic and end-systolic dimensions, RV ejection fraction, functional class, and exercise capacity. EMBASE, PubMed, and Cochrane databases were searched. The selected data were pooled and analyzed with the DerSimonian-Laird random-effects meta-analysis model. Between-study heterogeneity was assessed with Cochran's Q test. A Bayesian meta-analysis model was also used in the event that heterogeneity was low. Bias assessment was performed with the Newcastle-Ottawa Scale and Cochrane Risk of Bias Tool, and statistical risk of bias was assessed with Begg and Mazumdar's test and Egger's test. Six studies met the inclusion criteria, contributing a total of 187 patients; treatment with beta blocker was the intervention that could not be analyzed because of the small number of patients and diversity of outcomes reported. After at least 3 months of treatment with ACE inhibitors, ARBs, or aldosterone antagonists, there was no statistically significant change in mean ejection fraction, ventricular dimensions, or peak ventilatory equivalent of oxygen. The methodological quality of the majority of included studies was low, mainly because of a lack of a randomized and controlled design, small sample size, and incomplete follow-up. In conclusion, pooled results across the limited available studies did not provide conclusive evidence with regard to a beneficial effect of medical therapy in adults with systemic RV dysfunction. Randomized controlled trials or comparative-effectiveness studies that are sufficiently powered to demonstrate effect are needed to elucidate the efficacy of ACE inhibitors, ARBs, beta blockers, and aldosterone antagon

Topics: Adolescent; Adult; Age Factors; Cardiology; Cardiovascular Agents; Consensus; Evidence-Based Medicine; Heart Defects, Congenital; Heart Ventricles; Humans; Middle Aged; Practice Guidelines as Topic; Treatment Outcome; Ventricular Function, Right; Young Adult

The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol. The reasons for the guarded prognosis of the SRV in comparison with the systemic left ventricle are multifactorial, including distinct fibromuscular architecture, shape and function, coronary artery supply mismatch, intrinsic abnormalities of the tricuspid valve, intrinsic or acquired conduction abnormalities, and varied SRV adaptation to pressure or volume overload. Management of the SRV remains an ongoing challenge because SRV dysfunction has implications on short- and long-term outcomes for all patients irrespective of underlying cardiac morphology. SRV dysfunction can be subclinical, underscoring the need for tertiary follow-up and timely management of target hemodynamic lesions. Catheter interventions and surgery have an established role in selected patients. Cardiac resynchronization therapy is increasingly used, whereas pharmacological therapy is largely empirical. Mechanical assist device and heart transplantation remain options in end-stage heart failure when other management strategies have been exhausted. The present report focuses on the SRV with its pathological subtypes, pathophysiology, clinical features, current management strategies, and long-term sequelae. Although our article touches on issues applicable to neonates and children, its main focus is on adults with SRV.

Topics: Age Factors; Cardiac Catheterization; Cardiac Resynchronization Therapy; Cardiac Resynchronization Therapy Devices; Cardiac Surgical Procedures; Cardiovascular Agents; Defibrillators, Implantable; Electric Countershock; Heart Defects, Congenital; Heart Transplantation; Heart Ventricles; Heart-Assist Devices; Humans; Phenotype; Reoperation; Risk Factors; Treatment Outcome; Ventricular Dysfunction, Right; Ventricular Function, Right

Topics: Cardiovascular Agents; Female; Heart Defects, Congenital; Humans; Medication Therapy Management; Patient Care Management; Pregnancy; Pregnancy Complications, Cardiovascular; Risk Adjustment

Heart failure is a serious complication that can occur in patients with a variety of congenital and acquired disorders including congenital heart disease, cardiomyopathy, and myocarditis. Furthermore, heart failure patients comprise an increasing number of ICU admissions. Thus, it is important for those caring for patients with critical cardiovascular disease to have a thorough understanding of the medications used for the treatment of heart failure. The aim of this review is to provide an overview, rationale, indications, and adverse effects of medications used in the treatment of chronic heart failure.. PubMed, Medline, Cochrane Database of Systemic Reviews.. Studies were selected on their relevance for pediatric heart failure. When limited data on pediatric heart failure were available, studies in adult patients were selected.. Relevant findings from studies were selected by the authors.. The rationale for the efficacy of most heart failure medications used in pediatric patients is extrapolated from studies in adult heart failure. Commonly used medications for chronic heart failure include β-receptor antagonists (e.g., carvedilol and metoprolol), and medications aimed at blocking the renin-angiotensin-aldosterone system (e.g., angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, aldosterone receptor antagonists). In addition, diuretics are useful for symptoms of fluid overload. For patients with impaired perfusion, inotropic agents are useful acutely, but may be associated with worse outcomes when used chronically. Newer medications that have been recently approved in adults (e.g., serelaxin, ivabradine, and neprilysin inhibitor [angiotensin receptor blocker]) may prove to be important in pediatric heart failure.. Heart failure patients are in an important population of critically ill children. The pharmacologic approach to these patients is aimed at treating symptoms of congestion and/or poor perfusion and improving long-term outcomes.

Topics: Adrenergic beta-Antagonists; Adult; Angiotensin-Converting Enzyme Inhibitors; Cardiovascular Agents; Child; Chronic Disease; Coronary Care Units; Critical Care; Diuretics; Heart Defects, Congenital; Heart Failure; Humans; Intensive Care Units, Pediatric

To review the pharmacologic treatment options for pulmonary arterial hypertension in the cardiac intensive care setting and summarize the most-recent literature supporting these therapies.. Literature search for prospective studies, retrospective analyses, and case reports evaluating the safety and efficacy of pulmonary arterial hypertension therapies.. Mechanisms of action and pharmacokinetics, treatment recommendations, safety considerations, and outcomes for specific medical therapies.. Specific targeted therapies developed for the treatment of adult patients with pulmonary arterial hypertension have been applied for the benefit of children with pulmonary arterial hypertension. With the exception of inhaled nitric oxide, there are no pulmonary arterial hypertension medications approved for children in the United States by the Food and Drug Administration. Unfortunately, data on treatment strategies in children with pulmonary arterial hypertension are limited by the small number of randomized controlled clinical trials evaluating the safety and efficacy of specific treatments. The treatment options for pulmonary arterial hypertension in children focus on endothelial-based pathways. Calcium channel blockers are recommended for use in a very small, select group of children who are responsive to vasoreactivity testing at cardiac catheterization. Phosphodiesterase type 5 inhibitor therapy is the most-commonly recommended oral treatment option in children with pulmonary arterial hypertension. Prostacyclins provide adjunctive therapy for the treatment of pulmonary arterial hypertension as infusions (IV and subcutaneous) and inhalation agents. Inhaled nitric oxide is the first-line vasodilator therapy in persistent pulmonary hypertension of the newborn and is commonly used in the treatment of pulmonary arterial hypertension in the ICU. Endothelin receptor antagonists have been shown to improve exercise tolerance and survival in adult patients with pulmonary arterial hypertension. Soluble guanylate cyclase stimulators are the first drug class to be Food and Drug Administration approved for the treatment of chronic thromboembolic pulmonary hypertension.. Literature and data supporting the safe and effective use of pulmonary arterial hypertension therapies in children in the cardiac intensive care are limited. Extrapolation of adult data has afforded safe medical treatment of pulmonary hypertension in children. Large multicenter trials are needed in the search for safe and effective therapy of pulmonary hypertension in children.

Topics: Adult; Calcium Channel Blockers; Cardiovascular Agents; Child; Coronary Care Units; Critical Care; Endothelin Receptor Antagonists; Heart Defects, Congenital; Heart Failure; Humans; Hypertension, Pulmonary; Intensive Care Units, Pediatric; Nitrous Oxide; Vasodilator Agents

Congestive heart failure (CHF) is the inability of the heart to meet the metabolic demands of the body. As a disease of the advanced age with a frequency of 1-2% of the population it is rare in infancy and childhood. The incidence ranges from 2.95 (in all US children's hospitals) to 23.2 (University's Childrens Hospital Essen) on 1 000 discharges. Among the diagnostic procedures echocardiography is the primary modality of imaging. Tei-index and tissue-Doppler are more sensitive parameters for LV-dysfunction. BNP/NT-proBNP are age-dependent and can guide the long-term therapy. Treatment in childhood does not differ basically from that in adulthood, recommended by several guidelines, but data regarding the various substances - outlined in detail - are very limited. Here a big work has to be done in future!

Topics: Biomarkers; Cardiovascular Agents; Child; Child, Preschool; Chronic Disease; Cross-Sectional Studies; Echocardiography; Heart Defects, Congenital; Heart Failure; Humans; Infant; Survival Rate; Ventricular Dysfunction, Left

Valvular heart diseases (VHDs) place a hemodynamic load on the left and/or right ventricle that, if severe, prolonged, and untreated, damages the myocardium, leading to heart failure and death. Because all VHDs are mechanical problems, definitive therapy usually requires valve repair or replacement. In most valve disease the onset of symptoms marks a change in disease prognosis and is usually an indication for prompt surgical correction. Echocardiography is an indispensable modality for assessing lesion severity, its effect on cardiac function, and the proper timing for lesion correction. Intervention enhanced with percutaneous options now allows patients to benefit from mechanical correction.

Topics: Aortic Valve; Bicuspid Aortic Valve Disease; Cardiovascular Agents; Echocardiography; Heart Defects, Congenital; Heart Valve Diseases; Hemodynamics; Humans; Physical Examination; Prognosis

Topics: Antihypertensive Agents; Aortic Valve Insufficiency; Aortic Valve Stenosis; Cardiac Catheterization; Cardiology; Cardiovascular Agents; Disease Management; Echocardiography; Evidence-Based Medicine; Exercise Test; Heart Defects, Congenital; Heart Valve Diseases; Heart Valve Prosthesis Implantation; Hemodynamics; Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Mitral Valve Insufficiency; Mitral Valve Stenosis; Patient Care Team; Pharyngitis; Referral and Consultation; Rheumatic Fever; Secondary Prevention; Severity of Illness Index; Streptococcal Infections; United States; Vasodilator Agents; Ventricular Dysfunction, Left

Topics: Cardiovascular Agents; Child; Coronary Artery Bypass; Heart Defects, Congenital; Heart Failure; Humans; Intraoperative Care; Oxygen; Postoperative Complications; Pulmonary Circulation; Risk Factors

This review focuses on events subsequent to planning a pregnancy and addresses three components of concern for women with systemic lupus erythematosus: maternal, placental, and fetal. Flare rates are generally low for patients who are clinically stable at conception. For patients who have never had renal disease, there is no frm evidence that they will develop active renal disease simply due to being pregnant. For patients who begin pregnancy with an abnormal creatinine (> 2 mg/dl is ill advised), risks include hypertension, preeclampsia, high rate of fetal loss, and possible further deterioration of renal function. Discontinuation of angiotensin converting enzyme inhibitors, angiotensin receptor blockers, and mycophenalate is mandatory. Elevated levels of sVEGF-1 may be a harbinger of preeclampsia. For patients with anti-phospholipid antibodies detected in the frst trimester of pregnancy, the lupus anticoagulant per se may be the strongest predictor of pregnancy complications. For women with anti-SSA/Ro antibodies the risk of having a child with congenital heart block is 2% which rises to a recurrence rate of 18%. Information on current approaches to prevention and treatment of heart complications of neonatal lupus is provided.

Topics: Antibodies, Antinuclear; Cardiovascular Agents; Female; Fetus; Health Status Indicators; Heart Defects, Congenital; Humans; Kidney Diseases; Lupus Erythematosus, Systemic; Maternal-Fetal Exchange; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Risk Assessment; Risk Factors; Severity of Illness Index; Steroids

Folic acid (FA) is a member of the B-vitamin family with cardiovascular roles in homocysteine regulation and endothelial nitric oxide synthase (eNOS) activity. Its interaction with eNOS is thought to be due to the enhancement of tetrahydrobiopterin bioavailability, helping maintain eNOS in its coupled state to favor the generation of nitric oxide rather than oxygen free radicals. FA also plays a role in the prevention of several cardiac and noncardiac malformations, has potent direct antioxidant and antithrombotic effects, and can interfere with the production of the endothelial-derived hyperpolarizing factor. These multiple mechanisms of action have led to studies regarding the therapeutic potential of FA in cardiovascular disease. To date, studies have demonstrated that FA ameliorates endothelial dysfunction and nitrate tolerance and can improve pathological features of atherosclerosis. These effects appear to be homocysteine independent but rather related to their role in eNOS function. Given the growing evidence that nitric oxide synthase uncoupling plays a major role in many cardiovascular disorders, the potential of exogenous FA as an inexpensive and safe oral therapy is intriguing and is stimulating ongoing investigations.

Topics: Animals; Atherosclerosis; Cardiovascular Agents; Cardiovascular Diseases; Endothelium, Vascular; Folic Acid; Heart Defects, Congenital; Hemodynamics; Homocysteine; Humans; Nitric Oxide; Nitric Oxide Synthase Type III

Topics: Abortion, Induced; Anticoagulants; Cardiovascular Agents; Contraceptives, Oral; Contraindications; Counseling; Female; Fetal Diseases; Heart Defects, Congenital; Humans; Life Expectancy; Postnatal Care; Preconception Care; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome; Pregnancy, High-Risk; Prenatal Care; Recurrence; Risk Factors

Topics: Angioplasty, Balloon, Coronary; Atrial Fibrillation; Cardiomyopathy, Hypertrophic; Cardiovascular Agents; Coronary Artery Bypass; Coronary Artery Disease; Heart Defects, Congenital; Heart Valve Diseases; Heart Valve Prosthesis Implantation; Humans; Randomized Controlled Trials as Topic

Botallo's duct connects the systemic and pulmonary circulation. It can play a crucial hemodynamic role in some cardiac and respiratory diseases of the newborn, and strongly influences the outcome if it remains patent after birth or if it closes rapidly. The recently acquired in-depth knowledge on the genesis of these events have advanced the so called ''pharmacological manipulation of Botallo's duct'', i.e. pharmacological treatment to regulate the opening or closure of the ductus depending on clinical requirements and that, as will be described, is a key issue in managing newborns with severe cardiac and/or respiratory distress. This study illustrates the main underlying mechanisms of duct patency during intrauterine life and its closure after birth, and then describes the clinical conditions of the newborn where Botallo's duct must be kept patent after birth (duct-dependent cardiac malformations) and where its closure must be accelerated (patent duct associated idiopathic respiratory syndrome). It also reports the recent findings on the use of prostaglandins (PGE1) and prostaglandin synthesis inhibitors (indomethacin, ibuprofen) and the potential use of drugs capable of favouring or inhibiting nitric oxide in the duct endothelium.

Topics: Alprostadil; Anti-Inflammatory Agents, Non-Steroidal; Cardiovascular Agents; Cerebral Angiography; Cyanosis; Cyclooxygenase Inhibitors; Ductus Arteriosus; Ductus Arteriosus, Patent; Heart Defects, Congenital; Heart Failure; Hemodynamics; Humans; Ibuprofen; Indomethacin; Infant, Newborn; Infant, Premature, Diseases; Respiratory Distress Syndrome, Newborn; Vasodilator Agents

The provision of dental treatment under both local anaesthesia and sedation has an excellent safety record, although medical problems may occur. The high prevalence of cardiac disease in the population, particularly ischaemic heart disease, makes it the most common medical problem encountered in dental practice. Additionally, the increasing survival of children with congenital heart disease makes them a significant proportion of those attending for dental treatment. While most dental practitioners feel confident in performing cardio-pulmonary resuscitation, treating patients with co-existent cardio-vascular disease often causes concern over potential problems during treatment. This article aims to allay many of these fears by describing the commoner cardiac conditions and how they may affect dental treatment. It outlines prophylactic and remediable measures that may be taken to enable safe delivery of dental care.

Topics: Anesthesia, Dental; Angina Pectoris; Anticoagulants; Arrhythmias, Cardiac; Cardiovascular Agents; Dental Anxiety; Dental Care for Chronically Ill; Drug Interactions; Emergency Treatment; Endocarditis, Bacterial; Heart Defects, Congenital; Heart Diseases; Heart Valve Diseases; Humans; Hypertension; Monitoring, Intraoperative

Pregnancy is associated with major hemodynamic changes in the cardiovascular system that can contribute to greater morbidity and mortality in women with underlying heart disease. Therefore, the diagnosis and management of these disorders in the pregnant patient require understanding of cardiovascular physiology during pregnancy, labor, delivery, and the puerperium. It is also essential to have knowledge about safety and utility of various diagnostic modalities and drugs during pregnancy to treat maternal heart disease without compromising fetal well-being.

Topics: Cardiovascular Agents; Female; Heart Defects, Congenital; Heart Diseases; Heart Function Tests; Heart Transplantation; Heart Valve Prosthesis; Humans; Pregnancy; Pregnancy Complications, Cardiovascular

Disorders of the heart frequently cause pulmonary dysfunction because of the close structural and functional association of the heart and lungs. The pulmonary vasculature is very commonly affected by cardiac pathology. The pulmonary vasculature is normally a low-pressure, low-resistance circuit with high compliance and tremendous vascular reserve. Although resting vascular tone is low, there are many identified mediators of pulmonary arterial tone that may help mediate pulmonary blood flow. Alveolar hypoxia is clearly a stimulus for increasing pulmonary vascular resistance although factors that mediate the response to hypoxia are not fully understood. Patients with left-to-right shunting due to congenital heart disease because of elevations in pulmonary artery flow and pressure tend to develop progressive anatomic changes in the pulmonary vasculature. This leads to an increase in pulmonary vascular resistance, irreversible pulmonary hypertension, right heart failure, reversal of shunt flow, and Eisenmenger's syndrome. The degree of anatomic vascular damage due to left-to-right shunting can be graded histologically. Lesser grades of damage are reversible with corrective surgery, whereas more severe grades show no improvement or progression with operation. Chronic left-sided congestive heart failure seen in rheumatic mitral stenosis can cause secondary changes in the pulmonary vasculature. Pulmonary hypertension and increased pulmonary vascular resistance can increase reflexly and form a "second stenosis" that further limits cardiac output. Unlike congenital heart disease, severe grades of pulmonary arterial damage are not seen in left heart failure from mitral stenosis or other causes, and consequently with surgical correction pulmonary hypertension reverses. Pulmonary function testing is adversely affected by congestive heart failure. Both restrictive (stiff lungs) and obstructive (cardiac asthma) defects are observed in congestive heart failure. DLCO is abnormally decreased. With treatment of heart failure these defects reverse. Both elevated systemic and pulmonary venous pressures affect fluid filtration in the pleural space and cause pleural fluid accumulation. The fluid is transudative with low protein, low lactate dehydrogenase, and low cell counts. Transudative effusions from heart failure resolve with treatment. With large effusions and cardiomegaly, pulmonary dysfunction results because of atelectasis from compression and space-occupying effects o

Topics: Cardiovascular Agents; Heart Defects, Congenital; Heart Diseases; Humans; Hypertension, Pulmonary; Lung Diseases; Pleural Diseases; Pulmonary Circulation; Pulmonary Embolism; Respiratory Function Tests

The objectives of this study were: (i) to evaluate the effects of perfusion modes (pulsatile vs. nonpulsatile) on vital organs recovery and (ii) to investigate the influences of two different perfusion modes on the homeostasis of thyroid hormones in pediatric patients undergoing cardiopulmonary bypass (CPB) procedures. Two hundred and eighty-nine consecutive pediatric patients undergoing open heart surgery for repair of congenital heart disease were prospectively entered into the study and were randomly assigned to two groups: the pulsatile perfusion group (Group P, n = 208) and the nonpulsatile perfusion group (Group NP, n = 81). All patients received identical surgical, perfusional, and postoperative care. Study parameters included total drainage, mean urine output in the intensive care unit (ICU), intubation time, duration of ICU and hospital stay, the need for inotropic support, pre- and postoperative enzyme levels (ALT [alanine aminotransaminase] and AST [aspartate aminotransaminase]), c-reactive protein, lactate, albumin, blood count (leukocytes, hematocrit, platelets), creatinine levels, and thyroid hormones (thyroid stimulating hormone [TSH], FT(3) [free triiodothyronine], FT(4) [free thyroxine]). All patients survived the perioperative and postoperative periods. There were no statistically significant differences in either preoperative or operative parameters between the two groups. Group P, compared to Group NP, required significantly less inotropic support, had a shorter intubation period, higher urine output in ICU, and shorter duration of ICU and hospital stay. Lower lactate levels and higher albumin levels were observed in Group P and there were no significant differences in creatinine, enzyme levels, blood counts, or drainage amounts between two groups. TSH, Total T(3) , Total T(4) , and FT(3) , FT(4) levels were markedly reduced versus their preoperative values in both groups. FT(3) and FT(4) levels were reduced significantly further in the nonpulsatile group both during CPB and at 72 h postoperation. The results of this study confirm our opinion that pulsatile perfusion leads to better vital organ recovery and clinical outcomes in the early postoperative period as compared to nonpulsatile perfusion in pediatric patients undergoing CPB cardiac surgery. The plasma concentrations of thyroid hormones are dramatically reduced during and after CPB, but pulsatile perfusion seems to have a protective effect of thyroid hormone homeostasis compared

Topics: Adolescent; Biomarkers; Cardiopulmonary Bypass; Cardiovascular Agents; Chi-Square Distribution; Child; Child, Preschool; Critical Care; Female; Heart Defects, Congenital; Homeostasis; Humans; Infant; Infant, Newborn; Length of Stay; Male; Pennsylvania; Perfusion; Prospective Studies; Pulsatile Flow; Recovery of Function; Respiration, Artificial; Thyroxine; Time Factors; Treatment Outcome; Triiodothyronine; Urination

In the present study, 0.2-0.6 mg/kg (0.4+/-0.2, mean +/- standard deviation) indomethacin was administered intravenously to close a patent ductus arteriosus in 13 infants with co-existing congenital heart defects whose ages ranged from 3 to 48 (14+/-14) days. All of them were hemodynamically ductus-independent and symptomatic. Echocardiography demonstrated that the ductus had closed in 8 infants, aged 3-33 (12+/-10) days (responders), but had not closed in 5 infants aged 6-48 (19 +/-19) days (non-responders). There was no significant difference between the responders and non-responders in their age, body weight, minimal diameter of the duct, dose of indomethacin, gestational age, birthweight, or Apgar score. One possible major complication might be associated with indomethacin. However, intravenous indomethacin should be considered prior to surgical ligation as one option for infants with a symptomatic patent ductus arteriosus complicated by other congenital heart defects.

Topics: Cardiovascular Agents; Ductus Arteriosus, Patent; Echocardiography; Female; Heart Defects, Congenital; Humans; Indomethacin; Infant; Infant, Newborn; Infant, Premature; Injections, Intravenous; Male; Retreatment

Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure.. A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included.. Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation.. A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

Topics: Adult; Arrhythmias, Cardiac; Cardiovascular Agents; Congenitally Corrected Transposition of the Great Arteries; Heart Defects, Congenital; Heart Failure; Heart Ventricles; Humans; Retrospective Studies; Transposition of Great Vessels; Ventricular Function; Ventricular Function, Right

Most medications are not labeled for use in the pediatric population because they have not been formally studied in children. Data on off-label use of cardiovascular (CV) drugs in the home therapy of children with CV disease are scanty. The study included 325 pediatric patients with CV disease and on ≥1 CV medication who underwent ≥1 visit during 2019 at the Pediatric Cardiology outpatient clinic of Giovanni XXIII Pediatric Hospital in Bari, Italy. A total of 287 patients (88.3%) received ≥1 off-label medication, whereas 113 patients (34.7%) received ≥2 off-label medications, and 22 patients (6.7%) ≥3 off-label medications. In CV medications (n = 27) 85% were used off-label in all cases, and 92.5% were used off-label in ≥50% of patients. Adverse events occurred in 8 patients, leading to drug discontinuation in 2 of them. In all 8 cases, medications were used off-label. In multivariate analysis, congenital heart disease patients with single-ventricle physiology (odds ratio 8.4, 95% confidence interval 2.25 to 54.4) and those with heart failure (odds ratio 2.0, 95% confidence interval 1.1 to 3.6) were at higher risk for receiving ≥2 off-label drugs. The off-label use of CV drugs in the home therapy of children with congenital or acquired heart disease is common and adverse events may occur. Patients with congenital heart disease with single-ventricle physiology and those with heart failure have a higher probability to receive ≥2 off-label medications. This study highlights the need for larger safety and efficacy trials in this specific cohort of pediatric patients.

Topics: Cardiovascular Agents; Child; Heart Defects, Congenital; Heart Failure; Hospitals, Pediatric; Humans; Off-Label Use

Advances in surgical management strategies have substantially reduced fatality from congenital heart defects (CHD). Decreased infant mortality might be expected, consequentially to result in greater morbidity in older children due to complications later in childhood and adolescence. This study aims to evaluate the use of cardiovascular medication (CVM) as an indicator of disease burden in children born with CHD in the first 10 years of life.. Population-based cohort study.. Six population-based registries from the European Surveillance of Congenital Anomalies (EUROCAT) network participated. Data from live born children with major congenital anomalies (CA) born from 2000 to 2014 were linked to prescription databases. Four groups of children were analysed: CA, CHD, severe CHD (sCHD) and ventricular septal defect (VSD) without sCHD. Live born children without CA were included as reference group.. We obtained data on 61 038 children born with a CA, including 19 678 with CHD, 3392 with sCHD, 12 728 children with VSD without sCHD, and 1 725 496 reference children.. Children born with sCHD were the most likely to receive a CVM prescription (42.9%, 95% CI, 26.3 to 58.5) in the first year of life compared with 13.3% (6.7 to 22.0) of children with any CHD, 5.9% (3.7 to 8.7) of children with any CA and 0.1% (0.0 to 0.1) of reference children. Medication was less likely to be prescribed after the first year of life for sCHD; 18.8% (14.8 to 23.1) for children 1-4 years and 15.8% (12.0 to 20.1) 5-9 years. Children with sCHD were most likely to receive a diuretic (36.4%, 18.6 to 54.5), an antihypertensive (6.9%, 3.7 to 11.3) or a beta-blocker (5.5%, 2.9 to9.2).. Almost half of all children with sCHD were prescribed CVM in their first year of life. For all four groups of children with anomalies, the proportion of children with a CVM prescription decreased with age.

Topics: Adolescent; Cardiovascular Agents; Child; Cohort Studies; Drug Prescriptions; Female; Heart Defects, Congenital; Heart Septal Defects, Ventricular; Humans; Infant; Parturition; Pregnancy; Registries

A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However, these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition.. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA.. To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases.. Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Topics: Cardiac Surgical Procedures; Cardiovascular Agents; Child; Child, Preschool; Consensus; Heart Defects, Congenital; Humans; Infant; Time-to-Treatment

Background In women with congenital heart defects (CHD), changes in blood volume, heart rate, respiration, and edema during pregnancy may lead to increased risk of adverse outcomes and conditions. The American Heart Association recommends providers of pregnant women with CHD assess cardiac health and discuss risks and benefits of cardiac-related medications. We described receipt of American Heart Association-recommended cardiac evaluations, filled potentially teratogenic or fetotoxic (Food and Drug Administration pregnancy category D/X) cardiac-related prescriptions, and adverse conditions among pregnant women with CHD compared with those without CHD. Methods and Results Using 2007 to 2014 US healthcare claims data, we ascertained a retrospective cohort of women with and without CHD aged 15 to 44 years with private insurance covering prescriptions during pregnancy. CHD was defined as ≥1 inpatient code or ≥2 outpatient CHD diagnosis codes >30 days apart documented outside of pregnancy and categorized as severe or nonsevere. Log-linear regression, accounting for multiple pregnancies per woman, generated adjusted prevalence ratios (aPRs) for associations between the presence/severity of CHD and stillbirth, preterm birth, and adverse conditions from the last menstrual period to 90 days postpartum. We identified 2056 women with CHD (2334 pregnancies) and 1 374 982 women without (1 524 077 pregnancies). During the last menstrual period to 90 days postpartum, 56% of women with CHD had comprehensive echocardiograms and, during pregnancy, 4% filled potentially teratogenic or fetotoxic cardiac-related prescriptions. Women with CHD, compared with those without, experienced more adverse conditions overall (aPR, 1.9 [95% CI, 1.7-2.1]) and, specifically, obstetric (aPR, 1.3 [95% CI, 1.2-1.4]) and cardiac conditions (aPR, 10.2 [95% CI, 9.1-11.4]), stillbirth (aPR, 1.6 [95% CI, 1.1-2.4]), and preterm delivery (aPR, 1.6 [95% CI, 1.4-1.8]). More women with severe CHD, compared with nonsevere, experienced adverse conditions overall (aPR, 1.5 [95% CI, 1.2-1.9]). Conclusions Women with CHD have elevated prevalence of adverse cardiac and obstetric conditions during pregnancy; 4 in 100 used potentially teratogenic or fetotoxic medications, and only half received an American Heart Association-recommended comprehensive echocardiogram.

Topics: Adolescent; Adult; Cardiovascular Agents; Databases, Factual; Drug Prescriptions; Echocardiography; Female; Heart Defects, Congenital; Humans; Insurance, Health; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Prevalence; Private Sector; Retrospective Studies; Risk Assessment; Risk Factors; Ultrasonography, Prenatal; United States; Young Adult

Background Our objective was to estimate receipt of preconception health care among women with congenital heart defects (CHD), according to 2017 American Heart Association recommendations, as a baseline for evaluating recommendation implementation. Methods and Results Using 2007 to 2013 IBM MarketScan Commercial Databases, we identified women with CHD diagnosis codes ages 15 to 44 years who became pregnant and were enrolled in health insurance for ≥11 months in the year before estimated conception. We assessed documentation of complete blood count, electrolytes, thyroid-stimulating hormone, liver function, ECG, comprehensive echocardiogram, and exercise stress test, using procedural codes, and outpatient prescription claims for US Food and Drug Administration category D and X cardiac-related medications. Differences were examined according to CHD severity, age, region of residence, year of conception, and documented encounters at obstetric and cardiology practices. We found 2524 pregnancies among 2003 women with CHD (14.4% severe CHD). In the 98.3% of women with a healthcare encounter in the year before conception, <1% received all and 22.6% received no American Heart Association-recommended tests or assessments (range: 54.4% for complete blood count to 3.1% for exercise stress test). Women with the highest prevalence of receipt of recommended care were 35 to 44 years old, pregnant in 2012 to 2013, or had a documented obstetric or cardiology encounter in the year before conception (

Topics: Adolescent; Adult; American Heart Association; Blood Cell Count; Blood Chemical Analysis; Cardiology; Cardiovascular Agents; Disease Management; Echocardiography; Electrocardiography; Exercise Test; Female; Guideline Adherence; Heart Defects, Congenital; Humans; Implementation Science; Insurance, Health; Liver Function Tests; Obstetrics; Practice Guidelines as Topic; Preconception Care; Pregnancy; Pregnancy Complications, Cardiovascular; Thyrotropin; United States; Young Adult

Junctional ectopic tachycardia (JET) is a relatively common narrow complex rhythm typically characterized by atrioventricular dissociation or retrograde atrial conduction in a 1:1 pattern. Junctional ectopic tachycardia can be a life-threatening disorder, causing severe hemodynamic compromise and increased morbidity and mortality. The treatment of refractory JET can be very difficult, even with multimodal therapeutic interventions. The purpose of this study was to assess the role of ivabradine in cases of JET refractory to amiodarone and esmolol.. A total of 480 congenital heart surgeries were carried out at our center in 2017. Twenty (4.16%) patients had postoperative JET. Among these, five infants, aged 7 to 12 months (median: 8 months), had refractory JET. These patients (three tetralogy of Fallot, one ventricular septal defect, one complete atrioventricular septal defect) were treated with oral ivabradine in the dose range of 0.1 to 0.2 mg/kg/12 h as an adjunct to amiodarone.. All five patients achieved rate reduction and eventual conversion to sinus rhythm. Mean duration to achieve heart rate of <140 bpm after initiation of ivabradine therapy was 16.8 hours (±7.2 hours), while mean duration to achieve sinus rhythm was 31.6 hours (±13.6 hours). No patient had any recurrence of JET. No patient exhibited any hemodynamic derangement nor side effects attributable to oral ivabradine.. Oral ivabradine has the potential to be used as an adjunct to amiodarone in the treatment of JET in infants after surgery for congenital heart disease.

Topics: Cardiac Surgical Procedures; Cardiovascular Agents; Electrocardiography; Female; Heart Defects, Congenital; Heart Rate; Humans; Infant; Ivabradine; Male; Postoperative Complications; Retrospective Studies; Tachycardia, Ectopic Junctional; Treatment Outcome

As adult congenital heart disease (ACHD) patients are aging, a high prevalence of cardiovascular risk factors is encountered similar to the general population. Currently, data regarding the primary and secondary prevention of acquired cardiovascular disease in ACHD is lacking.. The German National Register of Congenital Heart Defects was systematically screened for ACHD patients with established cardiovascular risk factors or documented acquired cardiovascular conditions. Data were analyzed with regard to the according medical treatment.. Overall, 539 patients were included (mean age 38.4 ± 17.7 years, 49.2% female). Diabetes was present in 57 pts. (10.6%), arterial hypertension in 113 pts. (21.0%), hyperlipidaemia in 81 pts. (15.0%) and obesity in 271 pts. (50.2%). 31 pts. (5.8%) were smokers. Coronary artery disease was established in 16 pts. (3.0%), peripheral vascular disease in 9 pts. (1.7%), and cerebrovascular accidents in 141 pts. (26.2%). Out of the patients with coronary artery disease only 81.3% received antithrombotic treatment. Only 18.8% were prescribed a statin. Of the pts. with peripheral arterial disease, 44.4% received an antiplatelet drug, and only 22.2% were on a statin. Patients with arterial hypertension received antihypertensive drugs in 66.4%.. Primary and secondary prevention of acquired cardiovascular disease in ACHD is underutilized. This highlights the importance of educating primary physicians as well as ACHD physicians about the need of primary and secondary prevention for acquired cardiovascular disease.

Topics: Adult; Cardiovascular Agents; Cardiovascular Diseases; Female; Germany; Heart Defects, Congenital; Humans; Hydroxymethylglutaryl-CoA Reductase Inhibitors; Male; Middle Aged; Platelet Aggregation Inhibitors; Registries; Risk Factors; Treatment Outcome; Young Adult

Despite the progress in the management of patients with adult congenital heart disease (ACHD), a significant proportion of patients still develop pulmonary hypertension (PH). We aimed to highlight the rate of the complications in PH-ACHD and the predicting factors of cumulative mortality risk in this population.. Data were obtained from the cohort of the national registry of ACHD in Greece from February 2012 until January 2018.. Overall, 65 patients receiving PH-specific therapy were included (mean age 46.1±14.4 years, 64.6% females). Heavily symptomatic (New York Heart Association (NYHA) class III/IV) were 53.8% of patients. The majority received monotherapy, while combination therapy was administered in 41.5% of patients. Cardiac arrhythmia was reported in 30.8%, endocarditis in 1.5%, stroke in 4.6%, pulmonary arterial thrombosis in 6.2%, haemoptysis in 3.1% and hospitalisation due to heart failure (HF) in 23.1%. Over a median follow-up of 3 years (range 1-6), 12 (18.5%) patients died. On univariate Cox regression analysis history of HF hospitalisation emerged as a strong predictor of mortality (HR 8.91, 95% CI 2.64 to 30.02, p<0.001), which remained significant after adjustment for age and for NYHA functional class.. Long-term complications are common among patients with PH-ACHD. Hospitalisations for HF predict mortality and should be considered in the risk stratification of this population.

Topics: Adult; Arrhythmias, Cardiac; Cardiovascular Agents; Female; Follow-Up Studies; Greece; Heart Defects, Congenital; Heart Failure; Hospitalization; Humans; Hypertension, Pulmonary; Male; Middle Aged; Mortality; Prognosis; Registries; Risk Assessment; Stroke

Short QT syndrome (SQTS) predisposes afflicted patients to sudden cardiac death. Until now, only one drug-quinidine-has been shown to be effective in patients with SQTS type 1(SQTS1). The objective of this study was to use human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) from a patient with SQTS1 to search for potentially effective drugs for the treatment of SQTS1 patients. Patch clamp and single-cell contraction measurements were employed to assess drug effects. Ivabradine, mexiletine, and ajmaline but not flecainide, ranolazine, or amiodarone prolonged the action potential duration (APD) in hiPSC-CMs from an SQTS1 patient. Ivabradine, ajmaline, and mexiletine inhibited KCNH2 channel currents significantly, which may underlie their APD-prolonging effects. Under proarrhythmic epinephrine stimulation in spontaneously beating SQTS1 hiPSC-CMs, ivabradine, mexiletine, and ajmaline but not flecainide reduced the epinephrine-induced arrhythmic events. The results demonstrate that ivabradine, ajmaline, and mexiletine may be candidate drugs for preventing tachyarrhythmias in SQTS1 patients.

Topics: Action Potentials; Arrhythmias, Cardiac; Cardiovascular Agents; Dose-Response Relationship, Drug; Epinephrine; ERG1 Potassium Channel; Heart Conduction System; Heart Defects, Congenital; Humans; Induced Pluripotent Stem Cells; Myocytes, Cardiac

Topics: Aortic Valve; Atherectomy, Coronary; Bicuspid Aortic Valve Disease; Cardiovascular Agents; Coronary Artery Disease; Coronary Vessel Anomalies; Heart Defects, Congenital; Heart Failure; Heart Valve Diseases; Humans; Stroke Volume; Treatment Outcome; Vascular Calcification; Ventricular Function, Left

IntroductionOwing to massive improvements in the diagnostics and surgery of children with CHD, fatality has decreased substantially. As more children with CHD survive from infancy into later childhood, more will need medication for chronic heart failure. However, surprisingly little is actually known about which drugs are being used to treat children with CHD, and whether prescription rates and CHD prevalence have changed over time.. The objective of this study was to assess the total prescription of cardiovascular drugs to children during an 18-year period and to assess concomitant CHD prevalence.. All prescription data of cardiovascular drugs to children aged 0-19 years were extracted from publicly available databases in Norway and Denmark from 1999 to 2016. This was coupled with data on CHD prevalence and birth rates.. The number of defined daily doses of cardiovascular drugs prescribed to children doubled in the study period. This was because of an increased use of beta blockers, angiotensin-converting-enzyme inhibitors/angiotensin receptor blockers, and anti-arrhythmic agents. The use of some classes of drugs was significantly reduced over time. The prevalence of CHD remained constant in both countries - 80 per 10,000 births.. We show that there is an increase in the overall prescription of cardiovascular drugs to children. Beta blockers, angiotensin-converting enzyme/angiotensin receptor blockers, and anti-arrhythmics account for the largest increase. Birth rates decreased or remained constant together with CHD prevalence, suggesting that the increased use of cardiovascular drugs reflected increased prescription per patient, rather than more patients receiving a constant amount of drugs.

Topics: Adolescent; Cardiovascular Agents; Child; Child, Preschool; Denmark; Drug Prescriptions; Female; Follow-Up Studies; Forecasting; Heart Defects, Congenital; Humans; Infant; Infant, Newborn; Male; Norway; Prevalence; Registries; Young Adult

The aim of catheter intervention for vascular stenosis is the restoration of lumen area and optimization of distal blood flow. In pediatric practice, this has traditionally been a compromise between less effective balloon angioplasty and bare metal stent insertion with its attendant limitations of size. Bioabsorbable stents offer short-term relief of stenosis, radial support of the healing lesion, return of endothelial function and crucially, in children, the potential for long-term growth. Initial experience, in pediatric practice, with metal-based bioabsorbable stents was relatively disappointing with frequent restenosis secondary to early reabsorption. Design modifications resulting in polymer-based, drug eluting, bioabsorbable vascular scaffolds (BVS) have reportedly overcome some of these faults. We describe the first reported use of a drug eluting BVS in three patients with: (1) A newborn with severe right pulmonary artery (RPA) stensosis post repair of type two common arterial trunk. (2) A child with pulmonary atresia/ventricular septal defect (VSD) and major aorto-pulmonary collateral arteries (MAPCAs), and (3) An infant with severe left pulmonary artery (LPA) stenosis in the setting of an LPA sling.

Topics: Absorbable Implants; Angioplasty, Balloon; Cardiovascular Agents; Child; Coated Materials, Biocompatible; Female; Heart Defects, Congenital; Humans; Infant; Infant, Newborn; Lactic Acid; Male; Polyesters; Polymers; Prosthesis Design; Pulmonary Artery; Recurrence; Treatment Outcome; Vascular Patency

Interstage mortality (IM) remains significant after stage 1 palliation (S1P) for single-ventricle heart disease (SVD), with many deaths sudden and unexpected. We sought to determine whether digoxin use post-S1P is associated with reduced IM, utilizing the multicenter database of the National Pediatric Cardiology Quality Improvement Collaborative (NPCQIC).. From June 2008 to July 2013, 816 infants discharged after S1P from 50 surgical sites completed the interstage to stage II palliation, transplant, or IM. Arrhythmia during S1P hospitalization or discharge on antiarrhythmic medications were exclusions (n=270); 2 patients were lost to follow-up. Two analyses were performed: (1) propensity-score adjusted logistic regression with IM as outcome and (2) retrospective cohort analysis for patients discharged on digoxin versus not, matched for surgical site and other established IM risk factors. Of 544 study patients, 119 (21.9%) were discharged on digoxin. Logistic regression analysis with propensity score, site-size group, and digoxin use as predictor variables showed an increased risk of IM in those not discharged on digoxin (odds ratio, 8.6; lower confidence limit, 1.9; upper confidence limit, 38.3; P<0.01). The retrospective cohort analysis for 60 patients on digoxin (matched for site of care, type of S1P, post-S1P ECMO use, genetic syndrome, discharge feeding route, ventricular function, tricuspid regurgitation, and aortic arch gradient) showed 0% IM in the digoxin at discharge group and an estimated IM difference between the 2 groups of 9% (P=0.04).. Among SVD infants in the NPCQIC database discharged post-S1P with no history of arrhythmia, use of digoxin at discharge was associated with reduced IM.

Topics: Cardiac Surgical Procedures; Cardiovascular Agents; Digoxin; Female; Heart Defects, Congenital; Heart Ventricles; Humans; Infant; Infant Mortality; Infant, Newborn; Kaplan-Meier Estimate; Logistic Models; Male; Odds Ratio; Palliative Care; Patient Discharge; Propensity Score; Registries; Retrospective Studies; Risk Assessment; Risk Factors; Time Factors; Treatment Outcome; United States

Mortality for infants with single ventricle congenital heart disease remains as high as 8% to 12% during the interstage period, the time between discharge after the Norwood procedure and before the stage II palliation. The objective of our study was to determine the association between digoxin use and interstage mortality in these infants.. We conducted a retrospective cohort study using the Pediatric Heart Network Single Ventricle Reconstruction Trial public use dataset, which includes data on infants with single right ventricle congenital heart disease randomized to receive either a Blalock-Taussig shunt or right ventricle-to-pulmonary artery shunt during the Norwood procedure at 15 institutions in North America from 2005 to 2008. Parametric survival models were used to compare the risk of interstage mortality between those discharged to home on digoxin versus those discharged to home not on digoxin, adjusting for center volume, ascending aorta diameter, shunt type, and socioeconomic status. Of the 330 infants eligible for this study, 102 (31%) were discharged home on digoxin. Interstage mortality for those not on digoxin was 12.3%, compared to 2.9% among those on digoxin, with an adjusted hazard ratio of 3.5 (95% CI, 1.1-11.7; P=0.04). The number needed to treat to prevent 1 death was 11 patients. There were no differences in complications between the 2 groups during the interstage period.. Digoxin use in infants with single ventricle congenital heart disease is associated with significantly reduced interstage mortality.

Topics: Blalock-Taussig Procedure; Cardiovascular Agents; Databases, Factual; Digoxin; Female; Heart Defects, Congenital; Heart Ventricles; Humans; Infant; Infant Mortality; Infant, Newborn; Male; Norwood Procedures; Patient Discharge; Randomized Controlled Trials as Topic; Retrospective Studies; Risk Factors; Survival Analysis; Time Factors; Treatment Outcome

No studies have been conducted on rational drug use among children in Uzbekistan. This study aimed to analyze drug uses based on pharmaco-epidemiologic (PE) data from Regional Children's Multi-Profile Medical Centre (RCMPMC) in Andijan, Uzbekistan. Our study assessed drug usage in children with cardiovascular (CV) diseases, without intervening in the treatment processes or in the course of the diseases.. Subjects were 853 children aged 0 to 180 months (median age, 60 months; inter-quartile range, 24-108 months) who were hospitalized in the department of Cardiology and Rheumatology in RCMPMC from January to December, 2013 and were prescribed one or more drugs during hospitalization. Drugs used for a different disease or medical condition, given in a different way and/or given in a different dose were analyzed and considered to be irrational drugs.. The most commonly used medications among 10 drug groups prescribed by the doctors of RCMPMC were as follows: anti-arrhythmic (aspartic acid - 54.0 %), glycosides (digoxin - 44.0 %), diuretics (furosemide - 34.0 %), vitamins (ascorbic acid - 25.0 %), steroid anti-inflammatory drugs (prednisolone - 19.0 %), non-steroid anti-inflammatory drugs (diclofenac - 17.0 %), antibiotics (amoxicillin - 16.0 %), non-steroid anabolic drugs (potassium orotas - 14.0 %) and angiotensin-converting enzyme inhibitors (captopril - 11.0 %).. The study found that irrational drug schemes were quite frequent among pediatric CV patients and they are most frequent in children aged 2-3 years and younger.

Topics: Antirheumatic Agents; Cardiovascular Agents; Cardiovascular Diseases; Child; Child, Preschool; Cross-Sectional Studies; Developing Countries; Female; Heart Defects, Congenital; Hospitals, District; Hospitals, Pediatric; Humans; Inappropriate Prescribing; Infant; Infant, Newborn; Male; Practice Patterns, Physicians'; Rheumatic Diseases; Rheumatic Heart Disease; Uzbekistan

This study aimed to evaluate Fontan circulation which was observed over 30 years.. Forty patients who underwent a Fontan operation between 1974 and 1986 and survived (group S, n = 20) or died in the late period (group LD, n = 20) were evaluated. The median age at operation was 10 years (range 2-32 years). The diagnoses were tricuspid atresia in 21, single ventricle in 9, and others in 10. The Fontan procedure was a right atrium-pulmonary artery graft in 2, atriopulmonary connection in 28, and right atrium-right ventricle anastomosis in 10 (Björk in 4, graft in 6).. Causes of late death were congestive heart failure in 6, sudden death in 4, arrhythmia in 4, and others in 6. Sixteen patients underwent reoperation 23.3 ± 6.5 years after Fontan. Cardiac catheterization was performed at 1 month and 12 years. There were no significant differences in central venous pressure, ventricular ejection fraction, or pulmonary vascular resistance between the two groups. However, changes in ventricular end-diastolic volume suggested volume and pressure overload in group LD. In group S, the latest chest radiographs showed cardiothoracic ratio 51.3% ± 7.6%, oxygen saturation 94% ± 2%, brain natriuretic peptide 153 ± 111 pg ċ mL. For long-term Fontan survival, timely total cavopulmonary connection conversion and medication to decrease ventricular volume and pressure load might be important.

Topics: Adolescent; Adult; Cardiovascular Agents; Cause of Death; Child; Child, Preschool; Female; Fontan Procedure; Heart Defects, Congenital; Hemodynamics; Humans; Kaplan-Meier Estimate; Male; Postoperative Complications; Reoperation; Retrospective Studies; Risk Factors; Time Factors; Tomography, X-Ray Computed; Treatment Outcome; Young Adult

Topics: Adult; Calcium Channel Blockers; Cardiac Surgical Procedures; Cardiovascular Agents; Contraindications; Diagnostic Techniques, Cardiovascular; Disease Management; Eisenmenger Complex; Heart Defects, Congenital; Hemodynamics; Humans; Hypertension, Pulmonary; Nitric Oxide; Patient Selection; Practice Guidelines as Topic; Prognosis; Vasodilator Agents

Prospective data on the safety and efficacy of the wearable cardioverter defibrillator (WCD) in a real-world setting are lacking. The Prospective Registry of Patients Using the Wearable Defibrillator (WEARIT-II) Registry was designed to provide real-world data on the WCD as a strategy during a period of risk stratification.. The WEARIT-II Registry enrolled 2000 patients with ischemic (n=805, 40%), or nonischemic cardiomyopathy (n=927, 46%), or congenital/inherited heart disease (n=268) prescribed WCD between August 2011 and February 2014. Clinical data, arrhythmia events, implantable cardioverter defibrillator implantation, and improvement in ejection fraction were captured. The median age was 62 years; the median ejection fraction was 25%. The median WCD wear time was 90 days, with median daily use of 22.5 hours. There was a total of 120 sustained ventricular tachyarrhythmias in 41 patients, of whom 54% received appropriate WCD shock. Only 10 patients (0.5%) received inappropriate WCD therapy. The rate of sustained ventricular tachyarrhythmias by 3 months was 3% among patients with ischemic cardiomyopathy and congenital/inherited heart disease, and 1% among nonischemic patients (P=0.02). At the end of WCD use, 840 patients (42%) were implanted with an implantable cardioverter defibrillator. The most frequent reason not to implant an implantable cardioverter defibrillator following WCD use was improvement in ejection fraction.. The WEARIT-II Registry demonstrates a high rate of sustained ventricular tachyarrhythmias at 3 months in at-risk patients who are not eligible for an implantable cardioverter defibrillator, and suggests that the WCD can be safely used to protect patients during this period of risk assessment.

Topics: Aged; Arrhythmias, Cardiac; Cardiomyopathies; Cardiovascular Agents; Combined Modality Therapy; Death, Sudden, Cardiac; Defibrillators; Defibrillators, Implantable; Electric Countershock; Female; Follow-Up Studies; Heart Defects, Congenital; Humans; Male; Middle Aged; Myocardial Ischemia; Patient Compliance; Prescriptions; Prospective Studies; Registries; Stroke Volume

Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension.

Topics: Cardiovascular Agents; Child; Child, Preschool; Combined Modality Therapy; Diagnostic Imaging; Disease Management; Extracorporeal Membrane Oxygenation; Genetic Counseling; Heart Defects, Congenital; Hernias, Diaphragmatic, Congenital; Humans; Hypertension, Pulmonary; Infant; Infant, Newborn; Lung; Lung Transplantation; Nitric Oxide; Oxygen Inhalation Therapy; Persistent Fetal Circulation Syndrome; Postoperative Complications; Respiration, Artificial; Ventilator-Induced Lung Injury

Submitral aneurysm is a rare cardiac disease, predominantly being diagnosed among the black Africans. A Chinese adult was admitted as submitral aneurysm of the left ventricle in our department recently. We present this case for its rarity among Xanthoderm.

Topics: Adult; Anticoagulants; Asian People; Cardiovascular Agents; China; Heart Aneurysm; Heart Defects, Congenital; Humans; Male; Time Factors; Tomography, X-Ray Computed; Treatment Outcome

In 2012, the European Society of Cardiology (ESC) published an updated version of its guidelines on the management of valvular heart disease. Novelties include the use of advanced techniques for risk stratification and prognostic evaluation, such as stress and 3D echocardiography as well as measurement of left ventricular strain by speckle tracking. Equally important is the inclusion of percutaneous valve replacement or repair procedures, reflecting their ever-increasing use in clinical practice. Finally, the importance of a multidisciplinary approach to valvular heart disease, with collaboration of multiple specialities in a heart team has been put forward. We discuss practical aspects of the diagnostic and therapeutic approach to aortic valvular disease, including an outline of the surgical indications according to the ESC guidelines.

Topics: Aortic Valve; Bicuspid Aortic Valve Disease; Cardiac Surgical Procedures; Cardiovascular Agents; Choice Behavior; Diagnostic Techniques, Cardiovascular; Heart Defects, Congenital; Heart Valve Diseases; Humans

Topics: Acute Disease; Age Factors; Aneurysm, False; Aorta, Abdominal; Aorta, Thoracic; Aortic Diseases; Aortic Dissection; Aortic Valve; Atherosclerosis; Bicuspid Aortic Valve Disease; Cardiovascular Agents; Clinical Laboratory Techniques; Diagnostic Imaging; Early Diagnosis; Endovascular Procedures; Female; Genetic Diseases, Inborn; Heart Defects, Congenital; Heart Valve Diseases; Hematoma; Humans; Long-Term Care; Male; Neoplasms, Vascular Tissue; Physical Examination; Risk Factors; Vascular Calcification; Vascular Stiffness; Vascular Surgical Procedures

Following national guidance on management of sudden unexplained death (SUD) in the young, inherited cardiac conditions (ICC) clinics were established to identify and treat relatives thought to be at increased risk. Studies have examined diagnostic yield of these clinics but outcome of clinical management has not been reported.. Observational outcome study of consecutively referred relatives of SUD victims.. Regional ICC clinic.. 193 individuals (108 families) referred to a regional ICC clinic following SUD/aborted cardiac arrest of a young relative (mean follow-up 16.5 months, range 0.1-61).. All individuals underwent assessment by history, examination, ECG and echocardiography. Exercise electrocardiography, ajmaline provocation, further imaging techniques and genetic testing were performed in selected individuals. Implantable cardioverter-defibrillator (ICD) insertion based on national guidelines.. Forty-five patients (23%) from 38 families (35%) were diagnosed with an inheritable cause of sudden death. Eighteen had potentially prognostically important medication commenced and 4 had an ICD inserted on clinic recommendation (2 hypertrophic cardiomyopathy, 1 dilated cardiomyopathy, 1 arrhythmogenic right ventricular cardiomyopathy). Two other individuals had ICDs removed after negative testing for familial RYR2 mutations. No deaths have occurred during follow-up to date.. A diagnosis of an inheritable cause of sudden death was obtained in a significant minority of those with a family history of SUD/aborted cardiac arrest. The number of ICDs inserted as a result of specialist assessment was very small (2%). A major function of the clinic is reassurance of the clinically normal and cessation of treatment after exclusion of familial disease by genetic testing.

Topics: Adult; Algorithms; Cardiovascular Agents; Death, Sudden, Cardiac; Defibrillators, Implantable; Electrocardiography; Exercise Test; Female; Genetic Testing; Heart Arrest; Heart Defects, Congenital; Humans; Male; Middle Aged; Pedigree; Prognosis; Young Adult

Topics: Aortic Aneurysm; Aortic Dissection; Cardiomyopathies; Cardiovascular Agents; Cooperative Behavior; Female; Heart Defects, Congenital; Heart Valve Diseases; Heart Valve Prosthesis; Humans; Hypertension, Pulmonary; Infant, Newborn; Interdisciplinary Communication; Pregnancy; Pregnancy Complications, Cardiovascular; Prenatal Diagnosis; Risk Assessment; Venous Thromboembolism

Noncompaction myocardium (NCM) is a genetic heterogeneous primary cardiomyopathy which affects both children and adults and can be either isolated or combined with other congenital heart disorders. It has common pathogenesis of symptoms but is distinguished by pronounced clinical polymorphism. We have observed 25 adult patients (15 men, 10 women aged from 20 to 62 years, mean age 42.9+/-13.3 years) with NCM syndrome. Heart failure have been found in 96% of patients (functional class [FC] I in 7, II - in 6, III in 7, and IV - in 4 patients). Ninety two percent of patients have ventricular extrasystoles, 32% - atrial fibrillation, 28% - FC I-III angina. Mean end diastolic left ventricular dimension is 6.5+/-0.8cm, ejection fraction 29.7+/-13.0%, mean pulmonary artery pressure - 42.6+/-13.5 mm Hg. Intracardiac thrombosis have been found in 24% of patients. In 7 patients morphological study of myocardium has been performed. NCM syndrome was diagnosed at initial investigation just in 1 case. We distinguished the following clinical masks (variants of diagnosis) of NCM: 1) clinically not manifest, is revealed at accidental examination (4%); 2) exists under mask of "idiopathic" rhythm disturbances (8%); 3) has a mask of ischemic heart disease; 4) is revealed in patients with acute or subacute myocarditis (12%); 5) has a mask of dilated cardiomyopathy (52%); 6) NCM in patients with other primary cardiomyopathies (hypertrophic, restrictive, genetic myopathy, arrhythmogenic right ventricular dysplasia). Combination of NCM with congenital heart defects has been found in 20% of patients. In 56% of cases myocarditis was diagnosed (it was viral in no less than 44%). Only in 32% of patients it is possible to consider presence of isolated NCM syndrome. This paper contains discussion of problems of diagnostics (including morphological) and treatment in the presented group of patients, significance of myocarditis for development of decompensation, role of NCM in patients with other primary cardiomyopathies, possibility of compensatory (secondary) character of NCM in severe systolic dysfunction.

Topics: Adult; Arrhythmias, Cardiac; Biopsy; Cardiomyopathies; Cardiovascular Agents; Diagnosis, Differential; Disease Management; Electrocardiography; Female; Heart Defects, Congenital; Heart Failure; Heart Function Tests; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Myocarditis; Myocardium; Prognosis; Syndrome; Tomography, Spiral Computed; Treatment Outcome

Congenital heart defects that have a component of right ventricular outflow tract obstruction, such as tetralogy of Fallot, are frequently palliated in childhood by disruption of the pulmonary valve. Although this can provide an initial improvement in quality of life, these patients are often left with severe pulmonary valve insufficiency. Over time, this insufficiency can lead to enlargement of the right ventricle and to the deterioration of right ventricular systolic and diastolic function. Pulmonary valve replacement in these patients decreases right ventricular volume overload and improves right ventricular performance. To date, few studies have examined the effects of pulmonary valve replacement on left ventricular function in patients with biventricular dysfunction. We sought to perform such an evaluation.Records of adult patients who had undergone pulmonary valve replacement from January 2003 through November 2006 were analyzed retrospectively. We reviewed preoperative and postoperative echocardiograms and calculated left ventricular function in 38 patients.In the entire cohort, the mean left ventricular ejection fraction increased by a mean of 0.07 after pulmonary valve replacement, which was a statistically significant change (P < 0.01). In patients with preoperative ejection fractions of less than 0.50, mean ejection fractions increased by 0.10.We conclude that pulmonary valve replacement in patients with biventricular dysfunction arising from severe pulmonary insufficiency and right ventricular enlargement can improve left ventricular function. Prospective studies are needed to verify this finding.

Topics: Adult; Cardiac Surgical Procedures; Cardiovascular Agents; Child; Child, Preschool; Female; Heart Defects, Congenital; Heart Valve Prosthesis Implantation; Humans; Hypertrophy, Right Ventricular; Male; Middle Aged; Pulmonary Valve; Pulmonary Valve Insufficiency; Recovery of Function; Reoperation; Retrospective Studies; Stroke Volume; Texas; Treatment Outcome; Ultrasonography; Ventricular Dysfunction, Left; Ventricular Dysfunction, Right; Ventricular Function, Left; Ventricular Function, Right; Young Adult

Balloon atrial septostomy is ordinarily a safe palliative procedure for cyanotic congenital heart disease; however, if echocardiographic guidance is unavailable and fluoroscopy is used, distortions in the cardiac anatomy can invalidate the usual landmarks. Herein, we report iatrogenic mitral papillary muscle rupture during balloon atrial septostomy in a 4-day-old male neonate with total anomalous connection of the pulmonary veins. The anomalous connection and severe mitral regurgitation were emergently corrected, and the patient grew and developed normally. At age 24 years, he had only mild residual mitral regurgitation and was in New York Heart Association functional class I.In addition to describing the surgical treatment and positive late outcome of a rare complication, we highlight the importance of accurately evaluating balloon catheter location during atrial septostomy, especially in patients with a small left atrium.

Topics: Cardiac Surgical Procedures; Cardiovascular Agents; Catheterization; Cyanosis; Echocardiography, Transesophageal; Heart Defects, Congenital; Heart Failure; Heart Injuries; Humans; Iatrogenic Disease; Infant, Newborn; Male; Mitral Valve; Mitral Valve Insufficiency; Palliative Care; Severity of Illness Index; Time Factors; Treatment Outcome; Young Adult

To examine the overall morbidity of patients who underwent surgery for congenital cardiac defect during childhood.. A congenital cardiac defect treated with surgery is seldom totally cured. The incidence of residua, sequelae, and comorbidity is quite high. The morbidity has not been thoroughly examined.. Medication was used as an indicator of morbidity. Data from the Finnish Research Registry of Paediatric Cardiac Surgery were linked to data from the medication registry of Finland's Social Insurance Institution. This study includes 5116 patients with a mean age of 33.5 (ranged from 14.7 to 64.8) years, who had undergone surgery for congenital cardiac defect between 1953 and 1989. The use of medicines among patients in 2004 was compared with 10232 age- and sex-matched control subjects.. The overall use of medicines was frequent; 62% of patients and 53% of controls had purchased at least one prescribed medicine (risk ratio: 1.2, 95% confidence interval: 1.1-1.2). The number of patients using cardiovascular medicines (17%) and anti-thrombotic agents (5%) was higher than that of control subjects (risk ratio: 2.2 and 8.4). In addition, the patients needed medicinal care for epilepsy (3%), asthma (7%), and psychiatric diseases (10%) more often than did controls (risk ratio: 2.2, 1.5, and 1.3, respectively).. Patients operated on for congenital cardiac defect had more chronic diseases and used more medicines than did controls.

Topics: Adolescent; Adult; Anti-Bacterial Agents; Asthma; Cardiac Surgical Procedures; Cardiovascular Agents; Cardiovascular Diseases; Child; Chronic Disease; Epilepsy; Follow-Up Studies; Heart Defects, Congenital; Humans; Mental Disorders; Middle Aged; Young Adult

This study was performed to clarify the present global clinical status, including medication(s) and social abilities, of adult patients with single ventricular physiology (SVP).. Clinical charts were retrospectively reviewed to evaluate the global clinical status, including functional capacity, medication(s), complications, and social activities of 68 adult patients with SVP aged 20-53 years (41 males); 50 had undergone the Fontan operation and they were divided into the young adult (25 patients) and adult Fontan groups. The others were cyanotic patients without the Fontan operation (non-Fontan group). Although the Fontan groups showed better functional capacity, higher arterial oxygen saturation and brain natriuretic peptide levels, and a better renal function, there was no difference in the cardiovascular events during follow-up between the 3 groups. The most frequent complication was arrhythmia without a significant group difference, although the non-Fontan group showed a high percentage of heart failure. Only 41 patients (60%) had a job and 8 (12%) were married.. Although the Fontan groups had favorable pathophysiological conditions, the high rate of cardiovascular events, as well as unsatisfactory social situations, indicate the importance of meticulous life-long management of patients with SVP, regardless of the type of surgical intervention.

Topics: Adult; Age Factors; Arrhythmias, Cardiac; Biomarkers; Cardiovascular Agents; Disease-Free Survival; Employment; Female; Fontan Procedure; Heart Defects, Congenital; Heart Failure; Heart Ventricles; Hemodynamics; Humans; Japan; Kaplan-Meier Estimate; Male; Marital Status; Middle Aged; Natriuretic Peptide, Brain; Palliative Care; Quality of Life; Retrospective Studies; Risk Assessment; Survivors; Time Factors; Treatment Outcome; Young Adult

Topics: Aortic Dissection; Aortic Valve; Cardiovascular Agents; Coronary Aneurysm; Coronary Angiography; Drug Therapy, Combination; Echocardiography; Electrocardiography; Heart Defects, Congenital; Humans; Male; Middle Aged; Treatment Outcome

In this paper, we investigate various confidence intervals for the risk ratio under inverse sampling (also known as negative binomial sampling). Three existing confidence intervals (namely, the confidence intervals that are based on Fieller's theorem, the delta method and the F-statistic) are reviewed and three new confidence intervals (namely, the score, likelihood ratio and saddlepoint approximation (SA)-based confidence intervals) are developed. Comparative studies among these confidence intervals through Monte Carlo simulations are evaluated in terms of their coverage probabilities and expected interval widths under different settings. Our simulation results suggest that the SA-based confidence interval is generally more appealing. We illustrate these confidence interval construction methods with real data sets from a drug comparison study and a congenital heart disease study.

Topics: Binomial Distribution; Cardiovascular Agents; Chemical and Drug Induced Liver Injury; Computer Simulation; Confidence Intervals; Data Interpretation, Statistical; Dose-Response Relationship, Drug; Female; Heart Defects, Congenital; Humans; Infant, Low Birth Weight; Infant, Newborn; Likelihood Functions; Monte Carlo Method; Myocardial Infarction; Odds Ratio; Pregnancy; Pregnancy Complications, Cardiovascular; Sample Size

A few cases of isolated pulmonary artery have been successfully palliated by stenting the arterial duct using coronary stents. However, progressive luminal narrowing within the stent due to neointimal proliferation and peal formation is a considerable problem. We report the successful interventional palliation in a 7-week-old infant with isolated left pulmonary artery using sirolimus-eluting stents. In this unusual case, the isolated pulmonary artery was supplied by a duct-like remnant of a persistent fifth aortic arch, whereby the distal part of this vessel showed severe constriction. Implantation of two sirolimus-eluting coronary stents re-established good perfusion of the left pulmonary artery. Seven months after the procedure, echocardiography revealed that perfusion of the stented vessel and the left pulmonary artery was still very good. Stents eluting antimitotic agents also help to preserve the patency of small vessels in infants, and may be useful for ductal stenting.

Topics: Angioplasty, Balloon, Coronary; Aorta, Thoracic; Cardiac Catheterization; Cardiovascular Agents; Coronary Angiography; Ductus Arteriosus; Echocardiography; Female; Heart Defects, Congenital; Humans; Infant; Palliative Care; Prosthesis Design; Pulmonary Artery; Pulmonary Circulation; Sirolimus; Stents; Treatment Outcome; Vascular Patency

To describe clinical and demographic characteristics at baseline of a European cohort of adults with congenital heart disease (CHD) and to assess mortality and morbidity in a 5 year follow-up period.. Data collected as part of the Euro Heart Survey on adult CHD was analysed. This entailed information transcribed from the files of 4110 patients diagnosed with one of eight congenital heart conditions ('defects'), who consecutively visited the outpatient clinics of one of the participating centres in 1998. The patients were included retrospectively and followed until the end of 2003 for a median follow-up of 5.1 years. Notwithstanding their overall relatively good functional class and low mortality over the follow-up period, a considerable proportion of the patients had a history of endocarditis, arrhythmias, or vascular events. There were major differences between the eight defects, both in morbidity and regarding specific characteristics. Outcomes were worst in cyanotic defects and in the Fontan circulation, but a considerable proportion of the other patients also suffer from cardiac symptoms. In particular, arrhythmias are common.. The spectrum of adult CHD in Europe emerging from this survey is one of a predominantly young population with substantial morbidity but relatively low mortality in a 5 year period.

Topics: Adolescent; Adult; Aged; Ambulatory Care; Cardiovascular Agents; Data Collection; Europe; Female; Follow-Up Studies; Heart Defects, Congenital; Humans; Male; Middle Aged; Patient Acceptance of Health Care; Pregnancy; Pregnancy Complications, Cardiovascular; Survival Analysis

To evaluate the maternal and fetal outcome of pregnancies complicated by cardiac disease in a developing country.. A retrospective analysis was carried out of 207 pregnancies in women with cardiac disease who delivered at >or=28 weeks of gestation from June 1994 through December 2000 at a tertiary care center.. Rheumatic heart disease (n=183, 88%) with isolated mitral stenosis (n=71) was the predominant cardiac problem. Septal defects were the most common form of congenital heart disease (n=24). In 28 (13.52%) women, the diagnosis of cardiac disease was made during pregnancy. Cardiac complications were noted in 62 (29.95%) and fetal complications in 42 (20.28%) pregnancies. Patients in NYHA class I/II (n=175, 84.54%) had fewer maternal complications and their babies had a higher birth weight than those in NYHA class III/IV (n=32, 15.45%). Cardiac intervention was performed prior to pregnancy in 111 (60.65%) patients with rheumatic heart disease: PTMC/CMV in 73 and valve replacement (VR) in 38. Maternal and fetal outcome was better in patients with prosthetic valves (n=38) and the majority (97.4%) of them remained in NYHA class I/II. Cardiac intervention was safely carried out during pregnancy in 10 women (PTMC in 7, CMV in l, and VR in 2). One of them developed congestive cardiac failure during labor. None of the newborns of the 41 women who had received anticoagulants had any congenital malformation.. Rheumatic heart disease was the predominant type. Patients in NYHA class I/II had a better maternal and fetal outcome than those in NYHA class III/IV. Surgical correction of the cardiac lesion prior to pregnancy was associated with better pregnancy outcome. Pregnant women with prosthetic valves tolerated pregnancy well.

Topics: Adolescent; Adult; Cardiovascular Agents; Female; Heart Defects, Congenital; Heart Diseases; Heart Valve Prosthesis; Humans; India; Infant, Low Birth Weight; Infant, Newborn; Labor, Obstetric; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome; Pregnancy, High-Risk; Retrospective Studies; Rheumatic Heart Disease; Risk Assessment; Severity of Illness Index

Medically refractory heart failure may be present in children with cardiomyopathy (CMP) or complex congenital heart disease (CHD). In adults, the surgical management of this condition is either heart transplantation or the Batista operation. From March 1995 to January 2000, a total of 6 children, aged from 1 to 16 years, with medically refractory heart failure associated with CMP or complex CHD underwent cardiac transplantation and one of them also had the Batista operation as a bridge to transplantation. One of the 6 patients died of intractable sepsis 17 days after the operation, but the other 5 were discharged with satisfactory hemodynamics. Immunosuppressive agents, including azathioprine, cyclosporin or FK-506, were given. One patient experienced moderate acute rejection, but it was controlled by FK-506, OKT-3 and solumedrol. However, another suffered from lymphoproliferative disease 8 months after transplant, but it was controlled by intravenous immunoglubulin, alpha-interferon and acyclovir. Cardiac function during serial follow-up (range, 1 month to 5 years) revealed normal systolic and diastolic function and none received any anticongestive medications. Almost all patients received an oversized donor heart. The left ventricle (LV) mass was remodeled, initially as an decrease and later as an increase. The patient who underwent the Batista operation was discharged 1 month after the operation with an increased LV ejection fraction (from 10% to 22%). She was successfully bridged to heart transplantation 7 months after the Batista operation. The results of cardiac transplantation in growing children are satisfactory and remain the mainstay of surgical treatment for medically refractory heart failure in these patients. However, with a shortage of donor hearts, the Batista operation may be adopted as a bridge to heart transplant with a fair response.

Topics: Adolescent; Cardiomyopathy, Dilated; Cardiomyopathy, Restrictive; Cardiovascular Agents; Child; Child, Preschool; Drug Resistance; Female; Follow-Up Studies; Graft Rejection; Heart Defects, Congenital; Heart Failure; Heart Function Tests; Heart Transplantation; Heart Ventricles; Humans; Immunosuppressive Agents; Male; Organ Size; Postoperative Complications; Severity of Illness Index; Survival Rate; Treatment Outcome; Ventricular Remodeling

Emergencies in pediatric cardiology are heart failure, cyanosis and rhythm disturbances. The signs of heart failure are tachycardia, tachypnea and hepatomegaly. The therapy consists of oxygen, diuretics and digoxin. Occasionally, intubation with mechanical ventilation and intravenous catecholamines are needed. Cyanosis is often the only sign of a severe heart malformation, and prompt hospitalization is mandatory. Oxygen and warm environment is important during transport, correction of a possible metabolic acidosis and prostaglandin infusion are done in the hospital. Beyond the newborn period, so-called cyanotic spells are seen, particularly in tetralogy of Fallot. In supraventricular tachycardia, vagal manoeuvres can be tried first, if not successful, intravenous adenosine or electroconversion will restore sinus rhythm. In the older child, intravenous isoptin can be given. Slow heart rates from total AV block or sinus node affection are treated with atrophic, isuprel or electrical pacing.

Topics: Arrhythmias, Cardiac; Cardiovascular Agents; Child; Child, Preschool; Combined Modality Therapy; Critical Care; Cyanosis; Drug Therapy, Combination; Heart Defects, Congenital; Heart Failure; Humans; Infant; Infant, Newborn

The practice of pediatric cardiology has undergone remarkable changes over the past decade. Cardiac structural abnormalities may frequently be diagnosed with echocardiography alone, and treatment for many of these structural problems may be corrected during cardiac catheterization. Arrhythmia diagnosis and management have made similar progress, such that diagnosis of arrhythmia mechanisms and catheter ablative therapy may be performed during a single procedure, sparing the young patient a lifetime of antiarrhythmic drug therapy. Cardiac transplantation is now included among the treatment options for some patients with severe congenital or acquired cardiac abnormalities unresponsive to standard therapy. In this paper, we provide a brief description of current applications of recent advances in the practice of pediatric cardiology.

Topics: Arrhythmias, Cardiac; Cardiac Catheterization; Cardiovascular Agents; Echocardiography; Female; Fetal Monitoring; Heart Defects, Congenital; Heart Transplantation; Humans; Infant; Infant, Newborn; Male

Topics: Blood; Cardiovascular Agents; Chlorides; Dexamethasone; Glucocorticoids; Heart Defects, Congenital; Heart Failure; Humans; Natriuresis; Potassium; Prednisone; Rheumatic Heart Disease; Sodium; Triamcinolone; Urine; Water-Electrolyte Balance