Acquired von Willebrand Syndrome in an Infant With Coarctation of the Aorta and Williams Syndrome.

An infant with coarctation of the aorta and Williams syndrome was noted to have petechiae in cardiology clinic prior to planned surgical intervention. Workup revealed acquired von Willebrand syndrome secondary to the high shear force generated by the aortic coarctation. He was treated with intra- and postoperative Humate P; there were no postoperative bleeding complications. His acquired von Willebrand syndrome resolved postoperatively.

The Impact of the Left Ventricle on Right Ventricular Function and Clinical Outcomes in Infants with Single-Right Ventricle Anomalies up to 14 Months of Age.

BACKGROUND: Children with single-right ventricle anomalies such as hypoplastic left heart syndrome (HLHS) have left ventricles of variable size and function. The impact of the left ventricle on the performance of the right ventricle and on survival remains unclear. The aim of this study was to identify whether left ventricular (LV) size and function influence right ventricular (RV) function and clinical outcome after staged palliation for single-right ventricle anomalies. METHODS: In the Single Ventricle Reconstruction trial, echocardiography-derived measures of LV size and function were compared with measures of RV systolic and diastolic function, tricuspid regurgitation, and outcomes (death and/or heart transplantation) at baseline (preoperatively), early after Norwood palliation, before stage 2 palliation, and at 14 months of age. RESULTS: Of the 522 subjects who met the study inclusion criteria, 381 (73%) had measurable left ventricles. The HLHS subtype of aortic atresia/mitral atresia was significantly less likely to have a measurable left ventricle (41%) compared with the other HLHS subtypes: aortic stenosis/mitral stenosis (100%), aortic atresia/mitral stenosis (96%), and those without HLHS (83%). RV end-diastolic and end-systolic volumes were significantly larger, while diastolic indices suggested better diastolic properties in those subjects with no left ventricles compared with those with measurable left ventricles. However, RV ejection fraction was not different on the basis of LV size and function after staged palliation. Moreover, there was no difference in transplantation-free survival to Norwood discharge, through the interstage period, or at 14 months of age between those subjects who had measurable left ventricles compared with those who did not. CONCLUSIONS: LV size varies by anatomic subtype in infants with single-right ventricle anomalies. Although indices of RV size and diastolic function were influenced by the presence of a left ventricle, there was no difference in RV systolic function or transplantation-free survival on the basis of LV measures.

Hyperoxia Is Associated With Poor Outcomes in Pediatric Cardiac Patients Supported on Venoarterial Extracorporeal Membrane Oxygenation.

OBJECTIVES: Patients who require venoarterial extracorporeal membrane oxygenation because of cardiac failure frequently have supranormal blood oxygen tensions (hyperoxia). Recent studies have suggested worse outcomes in patients with hyperoxia after resuscitation from cardiac or respiratory arrests, presumably because of oxidative stress. There are limited data regarding the effect of hyperoxia on outcomes in pediatric patients on venoarterial extracorporeal membrane oxygenation. DESIGN: Retrospective chart review. SETTING: Pediatric cardiothoracic ICU. PATIENTS: Cardiac surgery patients less than 1 year old requiring venoarterial extracorporeal membrane oxygenation in the postoperative period from 2007 to 2013. MEASUREMENTS AND MAIN RESULTS: In 93 infants (median time on extracorporeal membrane oxygenation, 5 d), mortality at 30 days post surgery (primary outcome) was 38%. Using a receiver operating characteristic curve, a mean PaO2 of 193 mm Hg in the first 48 hours of extracorporeal membrane oxygenation was determined to have good discriminatory ability with regard to 30-day mortality. Univariate analysis identified a mean PaO2 greater than 193 mm Hg (p = 0.001), longer cardiopulmonary bypass times (p = 0.09), longer duration of extracorporeal membrane oxygenation (p < 0.0001), and higher extracorporeal membrane oxygenation pump flows (p = 0.052) as possible risk factors for 30-day mortality. In multivariable analysis controlling for the variables listed above, a mean PaO2 greater than 193 mm Hg remained an independent risk factor for mortality (p = 0.03). In addition, a mean PaO2 greater than 193 mm Hg was associated with the need for renal dialysis (p = 0.02) but not with neurologic injury (p = 0.41) during the hospitalization. CONCLUSIONS: In infants with congenital heart disease who are placed on venoarterial extracorporeal membrane oxygenation postoperatively, hyperoxia (defined as a mean PaO2 > 193 mm Hg in the first 48 hr of extracorporeal membrane oxygenation) was an independent risk factor for 30-day mortality after surgery. Future studies are needed to delineate the causative or associative role of hyperoxia with outcomes, especially in children with baseline cyanosis who may be more susceptible to the effects of oxidative stress.

Biventricular Badness: Rare Images of Ebstein Anomaly of the Tricuspid Valve in a Patient with Hypoplastic Left Heart Syndrome.

We report a rare case of hypoplastic left heart syndrome coexisting in a patient with Ebstein anomaly of the tricuspid valve, which has previously been described only in pathological studies. A fetal echocardiogram at 27-weeks gestation showed severe aortic stenosis with evolving hypoplastic left heart syndrome, significant endocardial fibroelastosis, a dysplastic tricuspid valve with moderate regurgitation, right atrial and ventricular dilation, and signs of fetal congestive heart failure. Due to inadequate left heart size, the patient was not a candidate for fetal intervention for critical aortic stenosis, and repeat studies showed progression of the lesion through the pregnancy. The infant was delivered at 36-weeks gestation with signs of hydrops, and a postnatal echocardiogram confirmed hypoplastic left heart syndrome as well as severe Ebstein anomaly of the tricuspid valve. The infant did not survive to intervention.

Accuracy of the fetal echocardiogram in double-outlet right ventricle.

OBJECTIVE: Double-outlet right ventricle (DORV) is a complex congenital heart defect with heterogeneous anatomy. Patients require a variety of surgical interventions, and the long-term prognosis is variable. Therefore, accurate prenatal diagnosis is important in providing parents with appropriate counseling. DESIGN: Medical records were reviewed in patients with a diagnosis of DORV who had fetal echocardiography at our institution from 1998 to 2004. Pre- and postnatal diagnoses were compared, anticipated surgical procedure was compared with surgery performed, and neonatal outcome was assessed. RESULTS: The study group consisted of 49 fetal patients with 6 in utero deaths (including 4 terminations), 2 patients lost to follow-up, and 41 live births. Postnatal echocardiograms or autopsy results were available on 42 patients. The overall accuracy of fetal echocardiography in making a correct diagnosis of DORV was 76%. Accurate prenatal prediction of the type of cardiac surgery that would be performed was made in 91% of patients who had surgery. Among live births, survival to hospital discharge was 76%. Survival was 50% in patients with extracardiac or chromosomal anomalies, compared with 92% in infants without additional anomalies. CONCLUSION: In most cases, careful evaluation by fetal echocardiography can determine essential anatomic details in fetuses with DORV with enough accuracy to allow for accurate counseling regarding the type of surgery needed. Survival is better than previously reported but is poor in patients with extracardiac or chromosomal abnormalities.