piperidines and Heart-Defects--Congenital

Topics: Anesthesia, General; Anesthesia, Inhalation; Anesthetics; Cardiac Catheterization; Cardiac Surgical Procedures; Child; Child, Preschool; Fentanyl; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Infant; Infant, Newborn; Methyl Ethers; Perioperative Care; Piperidines; Postoperative Complications; Remifentanil; Sevoflurane; Vascular Resistance

In this study, we have investigated feasibility of remifentanil and sufentanil anesthesia in children with congenital heart disease surgery and its effects on cardiac function and serological parameters. For this purpose, a retrospective study was conducted on 120 children with congenital heart disease who underwent repair of ventricular septum or atrial septum in our hospital, specifically from January 2016 to January 2018, and 60 patients in each group were randomly divided into the control and treatment groups, respectively. The control group was anesthetized with sufentanil, and the treatment group was anesthetized with remifentanil. The heart function, serological indexes, and adverse reactions were observed and compared. We have observed that there was no significant difference in HR levels between these groups (

Topics: Anesthesia; Child; Heart Defects, Congenital; Humans; Piperidines; Remifentanil; Retrospective Studies; Sufentanil

The authors compared the effects of remifentanil with fentanyl on the hemodynamic and respiratory variables in children with left-to-right shunting and pulmonary hypertension.. A prospective, randomized, and controlled design.. University hospital.. Children aged between 3 months and 6 years undergoing pediatric cardiac surgery for correction of left-to-right intracardiac shunting.. Children were assigned to 1 of the 2 opioids for intraoperative use. Fentanyl was given as a 20 microg/kg intravenous bolus followed by infusion at a rate of 20 microg/kg/h in group 1 (control, n=15), and remifentanil was given as a 2 microg/kg intravenous bolus followed by infusion at a rate of 2 microg/kg/min in group 2 (n=18).. Mean arterial pressures at 30 to 40 minutes postbypass and the first 2 hours postsurgery were higher in the remifentanil group (p<0.05). Heart rates, pulmonary artery pressures, and airway pressures did not differ at any time between groups. Peripheral oxygen saturation values were lower at 30 and 45 minutes in the prebypass period and higher at 1 to 4 hours in the intensive care unit in the remifentanil group (p<0.05). After protamine administration, transient peripheral oxygen desaturation was observed with 10 children in the remifentanil group and with 3 children in the fentanyl group without any hemodynamic deterioration (p=0.029).. There were no clinically important differences in hemodynamic and respiratory measurements intraoperatively and during the initial 24 hours postoperatively between fentanyl and remifentanil in pediatric patients undergoing surgical repair of defects with left-to-right shunts.

Topics: Cardiovascular Surgical Procedures; Child; Child, Preschool; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Infant; Intraoperative Care; Piperidines; Prospective Studies; Remifentanil

Remifentanil is recommended for use in procedures with painful intraoperative stimuli but minimal postoperative pain. However, bradycardia and hypotension are known side-effects. We evaluated haemodynamic effects of i.v. glycopyrrolate during remifentanil-sevoflurane anaesthesia for cardiac catheterization of children with congenital heart disease.. Forty-five children undergoing general anaesthesia with remifentanil and sevoflurane were randomly allocated to receive either saline, glycopyrrolate 6 microg kg(-1) or glycopyrrolate 12 microg kg(-1). After induction of anaesthesia with sevoflurane, i.v. placebo or glycopyrrolate was administered. An infusion of remifentanil at the rate of 0.15 microg kg(-1) min(-1) was started, sevoflurane continued at 0.6 MAC and cisatracurium 0.2 mg kg(-1) was given. Heart rate (HR) and non-invasive arterial pressures were monitored and noted every minute for the first 10 min and then every 2.5 min for subsequent maximum of 45 min.. Baseline HR [mean (SD)] of 117 (20) beats min(-1) decreased significantly from 12.5 min onwards after starting the remifentanil infusion in the control group [106 (18) at 12.5 min and 99 (16) beats min(-1) at 45 min]. In the groups receiving glycopyrrolate, no significant decrease in HR was noticed. Glycopyrrolate at 12 microg kg(-1) induced tachycardia between 5 and 9 min after administration. Systolic and diastolic arterial pressures decreased gradually, but there were no significant differences in the pressures between groups.. I.V. glycopyrrolate 6 microg kg(-1) prevents bradycardia during general anaesthesia with remifentanil and sevoflurane for cardiac catheterization in children with congenital heart disease. Administering 12 microg kg(-1) of glycopyrrolate temporarily induces tachycardia and offers no additional advantage.

Topics: Adjuvants, Anesthesia; Anesthetics, Combined; Blood Pressure; Bradycardia; Cardiac Catheterization; Child, Preschool; Dose-Response Relationship, Drug; Double-Blind Method; Female; Glycopyrrolate; Heart Defects, Congenital; Heart Rate; Humans; Infant; Intraoperative Care; Intraoperative Complications; Male; Methyl Ethers; Piperidines; Prospective Studies; Remifentanil; Sevoflurane

Opioids are used routinely to eliminate the stress response in the pre-bypass phase of paediatric cardiac surgery. Remifentanil is a unique opioid allowing a rapidly titratable effect. No data are available regarding a suitable remifentanil dose regimen for obtunding stress and cardiovascular responses to such surgery.. We recruited 49 infants and children under 5 yr old who were randomized to receive one of four remifentanil infusion rates (0.25, 1.0, 2.5, or 5.0 micro g kg(-1) min(-1)). Blood samples were obtained at induction, pre-surgery, 5 min after opening the chest, and immediately pre-bypass. Whole blood glucose was measured at all time points while cortisol and neuropeptide Y (NPY) were measured in the first and last samples. Heart rate and arterial pressure were also recorded.. There was a significant increase in whole blood glucose 5 min after opening the chest and pre-bypass (P=0.009, P=0.002) in patients receiving remifentanil 0.25 micro g kg(-1) min(-1), but not in those receiving higher doses. Increased remifentanil dosage was associated with reduced plasma cortisol during surgery (P<0.001). Baseline NPY showed considerable variation and there was no association between pre-bypass NPY and remifentanil dose. There was a significantly higher heart rate at the pre-bypass stage of surgery in the remifentanil 0.25 micro g kg(-1) min(-1) group compared with higher doses (P=0.0006). Four out of five neonates with complex cardiac conditions showed severe bradycardia associated with remifentanil.. In infants and children under 5 yr, remifentanil infusions of 1.0 micro g kg(-1) min(-1) and greater can suppress the glucose increase and tachycardia associated with the pre-bypass phase of cardiac surgery, while 0.25 micro g kg(-1) min(-1) does not. Remifentanil should be used with caution in neonates with complex congenital heart disease.

Topics: Analgesics, Opioid; Blood Glucose; Blood Pressure; Child, Preschool; Dose-Response Relationship, Drug; Double-Blind Method; Drug Administration Schedule; Female; Heart Defects, Congenital; Heart Rate; Humans; Hydrocortisone; Infant; Male; Neuropeptide Y; Piperidines; Prospective Studies; Remifentanil; Stress, Physiological

Cardiac catheterization of children with congenital heart disease is increasingly being performed under general anesthesia. Haemodynamic stability during anesthesia and fast and adequate recovery after the procedure is crucial in these patients. We performed a pilot study to evaluate hemodynamic stability when using remifentanil for anesthesia during cardiac catheterization. We also evaluated extubation times and recovery characteristics.. In a randomized, prospective, double-blind study 30 children (aged 1.5-20 months) received a continuous infusion of either 0.2 (group 0.2) or 0.3 microg/kg/min remifentanil (group 0.3) as part of a balanced anesthesia with 0.6 MAC sevoflurane. Heart rate, noninvasive arterial blood pressure, end tidal CO2 and pulse oxymetry were monitored throughout the procedure. Extubation times were noted, and recovery from anesthesia was evaluated using Aldrete scores.. : Haemodynamic response to intubation was well blunted in both groups. No significant changes in hemodynamic variables were noted from induction of anesthesia until 10 min after intubation. From then on there was a decrease in HR and systolic arterial pressure, which remained significant throughout the procedure in both groups. Extubation times were similar in both groups: 7.3 min (2,1) in group 0.2 vs. 6.6 min (2,1) in group 0.3 (NS). The number of patients with an Aldrete score of nine or more was 14 (group 0.2) vs. 15 (group 0.3), 10 min after extubation (NS).. Both dose regimens of remifentanil provided stable hemodynamic conditions during anesthesia for cardiac catheterization of children with congenital heart disease and allowed for rapid and adequate recovery.

Topics: Adjuvants, Anesthesia; Analgesics, Opioid; Anesthesia Recovery Period; Anesthesia, General; Anesthetics, Inhalation; Blood Pressure; Cardiac Catheterization; Child; Double-Blind Method; Female; Heart Defects, Congenital; Heart Rate; Humans; Intubation, Intratracheal; Male; Methyl Ethers; Monitoring, Intraoperative; Piperidines; Prospective Studies; Remifentanil; Sevoflurane

Short QT syndrome (SQTS) is an inherited cardiac channelopathy, but at present little information is available on its pharmacological treatment. SQT3 variant (linked to the inward rectifier potassium current I. The ten Tusscher et al model of human ventricular myocyte action potential (AP) was modified to recapitulate the changes in I. At the single cell level, the drugs quinidine, disopyramide, and E-4031 prolonged APD at 90% repolarization (APD. This study substantiates a causal link between quinidine and QT interval prolongation in SQT3 Kir2.1 mutations and highlights possible pharmacological agent quinidine for treating SQT3 patients.

Topics: Action Potentials; Arrhythmias, Cardiac; Disopyramide; Heart Conduction System; Heart Defects, Congenital; Heart Ventricles; Humans; Models, Biological; Piperidines; Pyridines; Quinidine

Several drugs blocking the rapidly activating potassium (K(r)) channel cause malformations (including cardiac defects) and embryonic death in animal teratology studies. In humans, these drugs have an established risk for acquired long-QT syndrome and arrhythmia. Recently, associations between cardiac defects and spontaneous abortions have been reported for drugs widely used in pregnancy (e.g. antidepressants), with long-QT syndrome risk. To investigate whether a common embryonic adverse-effect mechanism exists in the human, rat, and rabbit embryos, we made a comparative study of embryonic cardiomyocytes from all three species.. Patch-clamp and quantitative-mRNA measurements of K(r) and slowly activating K (K(s)) channels were performed on human, rat, and rabbit primary cardiomyocytes and cardiac samples from different embryo-foetal stages. The K(r) channel was present when the heart started to beat in all species, but was, in contrast to human and rabbit, lost in rats in late organogenesis. The specific K(r)-channel blocker E-4031 prolonged the action potential in a species- and development-dependent fashion, consistent with the observed K(r)-channel expression pattern and reported sensitive periods of developmental toxicity. E-4031 also increased the QT interval and induced 2:1 atrio-ventricular block in multi-electrode array electrographic recordings of rat embryos. The K(s) channel was expressed in human and rat throughout the embryo-foetal period but not in rabbit.. This first comparison of mRNA expression, potassium currents, and action-potential characteristics, with and without a specific K(r)-channel blocker in human, rat, and rabbit embryos provides evidence of K(r)-channel inhibition as a common mechanism for embryonic malformations and death.

Topics: Action Potentials; Animals; Atrioventricular Block; Cells, Cultured; Electrocardiography; ERG1 Potassium Channel; Ether-A-Go-Go Potassium Channels; Gene Expression Regulation, Developmental; Gestational Age; Heart Defects, Congenital; Humans; KCNQ1 Potassium Channel; Kinetics; Long QT Syndrome; Myocytes, Cardiac; Organogenesis; Patch-Clamp Techniques; Piperidines; Polymerase Chain Reaction; Potassium Channel Blockers; Potassium Channels, Voltage-Gated; Pyridines; Rabbits; Rats; Rats, Sprague-Dawley; Rats, Wistar; RNA, Messenger; Species Specificity; Teratogens

Topics: Animals; Heart Defects, Congenital; Humans; Myocytes, Cardiac; Piperidines; Potassium Channel Blockers; Potassium Channels, Voltage-Gated; Pyridines; Teratogens

We present an emergency anesthetic management of craniotomy for a 22-year-old man with congenital cyanotic heart disease due to brain abscess. Pulmonary blood flow was completely supplied via major arteriopulmonay collatelal artery (MAPCA). This patient complicated with Eisenmenger syndrome, has no history of cardiac surgery but several times of craniotomy due to repeated brain abscess. Total intravenous anesthesia with propofol and remifentanil was induced and maintained. SpO2 and PaO2 were elevated after oxygen administration via mask. Although systemic blood pressure level was decreased by anesthesia and continuous infusion of vasopressors was required. Oxygenation parameters were unchanged. This indicates that blood flow through MAPCA did not respond to changes in of respiratory and circulatory circumstances during anesthesia.

Topics: Anesthesia, Intravenous; Brain Abscess; Craniotomy; Cyanosis; Eisenmenger Complex; Emergencies; Heart Defects, Congenital; Humans; Intraoperative Care; Male; Piperidines; Propofol; Recurrence; Remifentanil; Vasoconstrictor Agents; Young Adult

Topics: Abnormalities, Multiple; Administration, Intranasal; Administration, Topical; Anesthetics, Local; Conjunctiva; Deep Sedation; Eye Injuries, Penetrating; Heart Defects, Congenital; Humans; Infant; Lidocaine; Limb Deformities, Congenital; Male; Midazolam; Piperidines; Remifentanil; Spinal Dysraphism; Syndrome

The population of children undergoing successful surgery for congenital heart disease (CHD) continues to increase. Increasing number of patients with a Fontan circulation is reaching adulthood and requiring anesthesia for noncardiac surgeries. We anesthetized a patient with a Fontan circulation (9 years old) for giant internal carotid-ophthalmic artery aneurysm trapping. There was a difficulty in cannulating internal jugular vein for measuring central venous pressure because of abnormal collateral arteries around the internal jugular vein. Central venous pressure of around 15 mmHg seemed appropriate to keep good hemodynamic condition during this surgery. In this condition, cerebral cortex was not edematous. Careful management of circulatory volume stabilized hemodynamic state. However, when heart rate decreased below 75, ectopic atrial rhythm occured. Anesthetic time was 13 hr and 40 min. The patient was extubated in the operating room. We have to take into consideration that there are anatomical abnormalities and arrhythmia in the patients with a Fontan circulation.

Topics: Anesthesia, Intravenous; Carotid Artery, Internal; Child; Craniotomy; Fontan Procedure; Heart Defects, Congenital; Humans; Intracranial Aneurysm; Male; Ophthalmic Artery; Piperidines; Propofol; Remifentanil; Vascular Surgical Procedures

We describe the use of remifentanil in an infant with a partially repaired Shone's syndrome who required tendon lengthening because of congenital clubfoot. Remifentanil has unique properties, making it a potentially useful and predictable agent for infants with significant comorbidity.

Topics: Analgesics, Opioid; Anesthesia; Clubfoot; Heart Defects, Congenital; Humans; Infant; Male; Piperidines; Remifentanil; Syndrome

Endothelin-1 (ET-1) has been implicated in the pathophysiology of pulmonary hypertension. In 1-month-old lambs with increased pulmonary blood flow, we have demonstrated early alterations in the ET-1 cascade. The objective of this study was to investigate the role of potential later alterations of the ET cascade in the pathophysiology of pulmonary hypertension secondary to increased pulmonary blood flow.. Eighteen fetal lambs underwent in utero placement of an aortopulmonary vascular graft (shunt) and were studied 8 weeks after spontaneous delivery. Compared with age-matched control lambs, lung tissue ET-1 levels were increased in shunt lambs (317.2+/-113.8 versus 209.8+/-61.8 pg/g, P<0.05). In shunt lambs (n=9), exogenous ET-1 induced potent pulmonary vasoconstriction, which was blocked by the ETA receptor antagonist PD 156707 (n=3). This pulmonary vasoconstriction was mimicked by exogenous Ala1,3,11,15 ET-1 (4 Ala ET-1), the ETB receptor agonist, and was blocked by the ETB receptor antagonist BQ 788 (n=3). However, in control lambs (n=7), ET-1 and 4 Ala ET-1 did not change pulmonary vascular tone. In contrast to 4-week-old shunt lambs, immunohistochemistry revealed the emergence of ETB receptors on smooth muscle cells in the vasculature of 8-week-old shunt lambs.. Over time, increased pulmonary blood flow and/or pressure results in the emergence of ETB-mediated vasoconstriction, which coincides with the emergence of ETB receptors on smooth muscle cells. These data suggest an important role for ETB receptors in the pathophysiology of pulmonary hypertension in this animal model of increased pulmonary blood flow.

Topics: Animals; Dioxoles; Endothelin Receptor Antagonists; Endothelin-1; Endothelins; Heart Defects, Congenital; Hemodynamics; Hypertension, Pulmonary; Lung; Muscle, Smooth, Vascular; Oligopeptides; Piperidines; Pulmonary Circulation; Receptor, Endothelin A; Receptor, Endothelin B; Receptors, Endothelin; Sheep; Vasoconstriction

We report a case of vagal hypertonia syndrome in a newborn infant, developed after surgical repair of an aortic coarctation combined with banding of the pulmonary artery trunk. The parasympathetic activity had adverse repercussions on haemodynamics. The diagnosis was confirmed by prolonged asystole on the oculocardiac reflex and by concomitant arrhythmia and disorders of conduction demonstrated by Holter recordings. To our knowledge, no other case of vagal hypertonia associated with a congenital cardiopathy has yet been reported. Infants with this syndrome are at a high risk of sudden death. Treatment with vagolytic drugs is of questionable value, and prolonged supervision of the patient is mandatory.

Topics: Bradycardia; Cranial Nerve Diseases; Follow-Up Studies; Heart Defects, Congenital; Humans; Hypertension, Pulmonary; Infant, Newborn; Male; Parasympatholytics; Piperidines; Sudden Infant Death; Syndrome; Vagus Nerve

Topics: Abnormalities, Drug-Induced; Animals; Cats; Cattle; Chromosome Aberrations; Chromosome Disorders; Disease Models, Animal; Dogs; Ehlers-Danlos Syndrome; Freemartinism; Genetic Diseases, Inborn; Genetic Linkage; Heart Defects, Congenital; Humans; Immunologic Deficiency Syndromes; Karyotyping; Leukocytes; Mercury Poisoning; Mink; Mosaicism; Nicotiana; Piperidines; Plant Extracts; Plant Poisoning; Plants, Medicinal; Plants, Toxic; Sheep; Swine; Veratrum

Topics: Adolescent; Adult; Blood Pressure; Child; Female; Heart Defects, Congenital; Heart Valve Diseases; Humans; Hypertension, Pulmonary; Male; Middle Aged; Oxygen Consumption; Piperidines; Pyrazoles; Vascular Resistance