deamino-arginine-vasopressin and Heart-Defects--Congenital

The term von Willebrand disease includes many bleeding disorders caused by abnormalities of vWF. Frequent or severe bleeding may be indicative of vWD or other bleeding conditions. Primary care practitioners need to be familiar with vWD and evaluate possibly affected individuals with appropriate laboratory studies. Patients with vWD should be educated about their disorder and preventive measures to limit its effect. Medications are available that can treat or prevent bleeding complications for most patients with vWD. Intervention with blood products is occasionally necessary.

Topics: Adolescent; Child; Child, Preschool; Chromosomes, Human, Pair 12; Deamino Arginine Vasopressin; Gene Deletion; Heart Defects, Congenital; Humans; Hypothyroidism; Incidence; Platelet Count; von Willebrand Diseases; von Willebrand Factor

Desmopressin (DDAVP) has been evaluated in many randomized clinical trials as a means to reduce blood loss and transfusion of allogeneic blood in cardiac operation requiring cardiopulmonary bypass. Desmopressin reduces blood loss in adult patients with excessive bleeding after cardiac operation. Its usefulness in patients undergoing complex congenital heart repair with cardiopulmonary bypass is unproved.. Sixty patients younger than 40 years of age scheduled for complex congenital heart operation (44 redo, 16 primary) were enrolled in this prospective, randomized, double-blind trial. Desmopressin 0.3 microg/kg or placebo was administered 10 minutes after protamine administration. Transfusion requirements and postoperative blood loss were recorded. Differences were analyzed using analysis of variance with a p value of 0.05 or less used to denote statistical significance.. There were no differences in demographic or surgical characteristics between the DDAVP or placebo groups. There was no difference in blood loss and transfusion requirements between the DDAVP and placebo groups. During the intraoperative postinfusion time period, the median blood loss for redo patients was 343 versus 357 mL/m2 for placebo versus DDAVP, respectively, and for primary patients, the median blood loss was 277 versus 228 mL/m2.. The prophylactic use of DDAVP to reduce excessive bleeding or transfusion requirements in patients undergoing complex congenital heart operations is not warranted.

Topics: Adolescent; Adult; Blood Coagulation Tests; Blood Loss, Surgical; Blood Transfusion; Cardiopulmonary Bypass; Child; Child, Preschool; Deamino Arginine Vasopressin; Double-Blind Method; Female; Heart Defects, Congenital; Hemostasis, Surgical; Humans; Infant; Male; Prospective Studies; Reoperation

In children with congenital heart disease (CHD), excessive perioperative bleeding is associated with increased morbidity and mortality, thus making adequate perioperative hemostasis crucial. We investigate the prevalence of acquired von Willebrand syndrome type 2A (aVWS) in CHD and develop a treatment algorithm for patients with aVWS and CHD (TAPAC) to reduce perioperative blood loss.. Retrospective cohort study.. Single-center study.. A total of 627 patients with CHD, undergoing corrective cardiac surgery between January 2008 and May 2017.. The evaluation of perioperative bleeding risk was based on the laboratory parameters von Willebrand factor (VWF) antigen, ristocetin cofactor activity, platelet function analyzer (PFA) closure time adenosine diphosphate, and PFA epinephrine. According to the bleeding risk, treatment was performed with desmopressin or VWF.. aVWS was confirmed in 63.3 %, with a prevalence of 45.5% in the moderate and 66.3 % in the high-risk group. In addition, prevalence increased with ascending peak velocity above the stenosis (v max ) from 40.0% at less than or equal to 3 m/s to 83.3% at greater than 5 m/s. TAPAC reduced mean blood loss by 36.3% in comparison with a historical control cohort ( p < 0.001), without increasing the number of thrombotic or thromboembolic events during the hospital stay. With ascending v max , there was an increase in perioperative blood loss in the historical cohort ( p < 0.001), which was not evident in the TAPAC cohort ( p = 0.230).. The prevalence of aVWS in CHD seems to be higher than assumed and leads to significantly higher perioperative blood loss, especially at high v max . Identifying these patients through appropriate laboratory analytics and adequate treatment could reduce blood loss effectively.

Topics: Adenosine Diphosphate; Algorithms; Blood Loss, Surgical; Child; Deamino Arginine Vasopressin; Epinephrine; Heart Defects, Congenital; Humans; Retrospective Studies; Syndrome; von Willebrand Diseases; von Willebrand Factor

A 7-year-8-month-old boy with cardiofaciocutaneous syndrome caused by the D638E mutation of the B-Raf proto-oncogene (BRAF) presented with new-onset seizures. He was incidentally found to have advanced Tanner staging on physical examination. Hormonal testing revealed pubertal levels of gonadotropins and sex steroid hormones. On brain imaging, a lack of visualisation of the posterior pituitary bright spot was observed, in addition to mild thinning of the corpus callosum and the lateral gyri of the cerebellar hemispheres. A diagnosis of idiopathic central precocious puberty was made and the patient was started on leuprolide depot treatment. Pituitary hormone testing revealed hyperprolactinemia for which the patient did not receive treatment as he was asymptomatic. During a subsequent hospital admission for seizures, the patient was diagnosed with transient central diabetes insipidus for which he required treatment with a desmopressin infusion.

Topics: Antineoplastic Agents, Hormonal; Child; Deamino Arginine Vasopressin; Diabetes Insipidus, Neurogenic; Ectodermal Dysplasia; Facies; Failure to Thrive; Heart Defects, Congenital; Hemostatics; Humans; Leuprolide; Male; Proto-Oncogene Mas; Proto-Oncogene Proteins B-raf; Puberty, Precocious; Seizures; Treatment Outcome

Between August 1989 and July 2003 14 Jehovah's Witness children with congenital heart defects (CHD) aged under 14 years (median 2.9 years) and with a median weight of 14 kg underwent 16 operations with cardiopulmonary bypass (CPB). Five children had been operated on previously between one to three times. Preoperatively, 7 children were prepared with oral iron supplementation and 10 received erythropoietin. Mean hemoglobin (Hb) at admission was 14.4 g/dl (range 10.9 - 19.2). The cardiopulmonary bypass (CPB) circuit was modified to reduce total priming volume. High doses of aprotinin were administered. The modified ultrafiltration (MUF) circuit, used in 7 patients, was parallel to the ECC circuit with continuous circulation of the blood through a small shunt between the arterial and venous lines. Operations performed consisted of VSD closure (3 pts.), ASD closure (3 pts.), Fontan operation (2 pts.), and complete AV canal correction, aortic commissurotomy, Ross operation, Glenn shunt, cor triatriatum correction, MV reconstruction combined with left outflow tract stenosis resection, correction of absent pulmonary valve syndrome, and correction of tetralogy of Fallot in one patient each. There were no deaths. Mean duration of CPB was 192 min and mean aortic cross-clamp time 40 min. The Hb value at the end of the operation was 4.9 - 14.5 g/dl (mean 9.6) and at discharge it was 7.1 - 14.5 g/dl (mean 15.5). No blood or blood products were used in any patient.. Bloodless cardiac surgery with and without CPB can be safely performed in Jehovah's Witness infants and children.

Topics: Aprotinin; Cardiopulmonary Bypass; Child; Child, Preschool; Deamino Arginine Vasopressin; Erythropoietin; Heart Defects, Congenital; Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Hemoglobins; Hemostatics; Humans; Infant; Jehovah's Witnesses

Topics: Deamino Arginine Vasopressin; Heart Defects, Congenital; Humans; Infant; Male; Vasomotor System