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Objective The objective of this study was to review the long-term results of symptomatic patients with Ebstein anomaly in the neonatal period. Methods The medical records of 40 neonates with a diagnosis of Ebstein anomaly who were admitted to our institution between January 1988 and June 2008 were retrospectively reviewed. Primary outcomes studied included patient survival and need for reintervention. Results No early intervention was required in 16 of the 40 patients with a hospital survival of 94% (15/16) and no late mortality. The remaining 24 patients underwent surgical intervention in the neonatal period. A shunt alone was performed in 9 patients with an actuarial survival of 88.9% at 1 year and 76.2% at 5 and 10 years. For the patients undergoing intervention on the tricuspid valve, survival estimates for the 11 patients with a right ventricular exclusion procedure were 63.6% at 1, 5, and 10 years and 47.7% at 15 years compared with 25.0% at 1, 5, and 10 years for the 4 patients with tricuspid valve repair. All long-term survivors were in New York Heart Association class I or II, and only 1 patient required antiarrhythmic medication. Conclusion Symptomatic neonates with Ebstein anomaly requiring no intervention or shunting alone have good long-term survival. For patients needing intervention on the tricuspid valve, overall survival is lower. For these patients, right ventricular exclusion may be superior to tricuspid valve repair.

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.

Background— Pulmonary venous Doppler (PVD) flow patterns in the fetus with hypoplastic left heart syndrome (HLHS) have been correlated with restrictive interatrial communication or intact atrial septum (RAS) postnatally; however, the ability of PVD to identify the neonate requiring emergent atrial septoplasty (EAS) for severe left atrial hypertension and hypoxemia has not been critically evaluated. It was the purpose of this study to determine the predictive power of fetal PVD in identifying the need for EAS in newborns with HLHS and RAS. Methods and Results— Forty-one patients with fetal PVD flow analysis and postnatally confirmed HLHS were studied. Pulsed-wave assessment of PVD flow included S-, D-, and A-wave velocity, time-velocity integral (VTI) of forward and reverse flow, and S/D velocity and forward/reverse VTI ratio. Neonatal EAS was used as the primary clinical outcome variable. Receiver operating characteristic curves were used to determine cutpoints at which PVD indices best predicted EAS. Cutpoints were evaluated for clinical accuracy and usefulness by use of Bayesian analysis. Eight of 41 subjects underwent EAS. Need for EAS was most accurately predicted by forward/reverse VTI ratio Conclusions— In the fetus with HLHS, a PVD forward/reverse VTI ratio of

Thrombus formation in the native aortic root is a rare but potentially life-threatening complication in patients with hypoplastic left heart syndrome. The native aortic root in these patients serves as a conduit for the retrograde filling of the coronary arteries. Thrombus in this location could result in myocardial ischemia. We present three cases of native aortic root thrombosis at our institution with clinical presentations ranging from asymptomatic to sudden cardiac arrest. Our experience with these patients argues for careful and consistent evaluation of the native aortic root regardless of clinical symptoms.

Infants with an unbalanced atrioventricular septal defect (AVSD) frequently present with comorbidities that may have an impact on their medical course and outcome. This study aimed to assess outcomes and explore possible prognostic indicators for patients undergoing surgical palliation for an unbalanced AVSD. The medical records of all infants presenting to the authors’ institution with an unbalanced AVSD over a 5-year period were retrospectively reviewed for assessment of outcomes and comorbidities. The study group consisted of 44 patients with an overall survival rate of 51% for the entire follow-up period. The majority of these patients (88%) underwent single-ventricle palliation, with an 83% rate of survival to initial hospital discharge and an overall long-term survival rate of 50%. The midterm outcome was significantly worse than that for a cohort of hypoplastic left heart syndrome patients undergoing single-ventricle palliation during the same period (P = 0.03). In addition, 30% of the patients required either repair or replacement of their systemic atrioventricular valve at initial palliation or during subsequent follow-up evaluation. Of the patients with an unbalanced AVSD, 75% had associated congenital anomalies. In conclusion, infants with an unbalanced AVSD are a high-risk population with diminished midterm survival compared with palliated patients who have more classic forms of hypoplastic heart syndromes. This may be due to the higher incidence of both severe atrioventricular valve regurgitation and important associated congenital anomalies.

HLA class II molecules are associated with a number of invariant chain glycoproteins including γl or Ii, the ~ 31 Kd predominant form of invariant chain; the ~ 33 Kd species γ2 and γ3; the 41 Kd species p41, the 25 Kd species, p25; and a number of acidic forms. In addition, we demonstrated that the class II molecules are also associated with a 40–70 Kd chondroitin sulfate proteoglycan molecule (CSPG) (1), and further demonstrated that the core protein of the CSPG is Ii (2).