Premature closure or restriction of foramen ovale (FO) is a rare but known entity. FO diameter <2 mm and Doppler velocity >120 cm/s, diameter <3 mm with Doppler velocity measured gradient >5 mmHg have all being used by various authors to describe this entity. Some neonates with restrictive FO have been noted to have severe pulmonary hypertension with no clinical signs or symptoms and with spontaneous resolution without any intervention. Seven consecutive neonates were indentified in the database between 01/01/2003 and 06/30/2010 with diagnosis of restrictive PFO (diameter <2 mm) with structurally normal heart and their initial and follow-up echocardiogram as well as hospital medical records were reviewed. As a control, seven neonates with diagnosis of pulmonary hypertension and respiratory distress syndrome or meconium aspiration syndrome were randomly selected. Eighty-six percent of the patients in the control group were symptomatic and required treatment as compared to 14% in the restrictive FO group (p = 0.03). Further, the fall in the peak instantaneous pulmonary artery pressure on follow-up echocardiogram was greater in the restrictive FO group compared with the non-restrictive group (p = 0.03). Patients with pulmonary hypertension and a restrictive FO with no other associated congenital heart disease and/or lung pathology behave differently when compared to neonates with non-restrictive FO and pulmonary hypertension with associated lung disease. They seldom manifest symptoms requiring intervention and tend to show a faster drop in their pulmonary artery pressure toward the normal.

Left ventricular noncompaction is a rare form of cardiomyopathy, which results from multiple trabeculations in the left ventricular myocardium. The clinical presentation is highly variable, and spectrum includes asymptomatic patients diagnosed during family screening on one end to patients with depressed systolic function, heart failure, thromboembolic complications, and cardiac arrhythmias on the other (Kim et al in J Am Coll Cardiol 53: 2009, 2009). Further, the progression of the condition is highly variable. Hence, these patients require close follow-up, and management for each patient needs to be individualized and periodically reevaluated. Here, we present a series of five cases that have been followed in our practice and present our experience. A literature review of this rare form of congenital cardiomyopathy is also presented.

Repair for tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) has been reported with good early and intermediate outcomes. Morbidity, however, remains significantly high. To date, repair of CAVSD/TOF using a pulmonary valve-sparing technique (PVS) and freedom from valve reoperation are not well defined. A study was undertaken to investigate outcomes. This study was conducted in as a retrospective investigation. Between January 1988 and December 2008, 13 consecutive patients with CAVSD/TOF were identified, and their records were reviewed retrospectively. Of these 13 patients, 9 had Rastelli type C CAVSD. Trisomy 21 was present in 9 cases (69 %; 7 with type C). Five patients had received a systemic-to-pulmonary shunt (SPS) before complete repair at a mean age 1.7 ± 0.6 months. All the patients survived until complete repair. At complete CAVSD/TOF repair, AVSD was corrected with a two-patch technique in all patients. For eight patients (61.5 %), PVS was used. The remaining five patients had transannular patch (TAP) repair. The mean age at complete repair was 6.3 ± 2.4 months. At complete repair, the mean cardiopulmonary bypass time was 173.5 ± 30.6 min, and the cross-clamp time was 134.7 ± 28.8 min. There was one hospitalization and no late deaths. The median follow-up period was 9.2 years [interquartile range (IQR), 4.7-13.3 years]. The actuarial survival was 90.0 ± 9.5 % at 1 year, 90 ± 9.5 % at 5 years, and 90 ± 9.5 % at 8 years. Of the 12 survivors, 6 had some reintervention during the follow-up period. Within the first 11 years after complete repair, two patients underwent left atrioventricular (AV) valve repair, and one patient had right AV valve repair. Two patients had residual VSD closure. Four patients underwent the first right ventricular outflow tract (RVOT) reintervention for critical insufficiency or stenosis at a mean interval of 6 ± 21) months. One patient had a second RVOT reoperation. Findings showed that CAVSD/TOF with PVS was related to significantly higher freedom from RVOT reintervention (100 % at 1, 5, and 8 years compared with 80 ± 17.9 % at 1 year, 60 ± 21.9 % at 5 years, and 40 ± 21.9 % at 8 years for CAVSD/TOF using TAP; P < 0.05). No patient who underwent PVS had left ventricular outflow tract obstruction requiring reoperation. Overall freedom from any reintervention was 90.9 ± 8.6 % at 1 year, 71.6 ± 14.0 % at 5 years, and 53.7 ± 8.7 % at 8 years in this group of patients. Correction of TOF with CAVSD can be performed at low risk with favorable intermediate-term survival and satisfactory freedom from reoperation. Use of TAP can be avoided in almost two thirds of patients and may influence freedom from early RVOT reintervention.

A rare case of hemolytic anemia in a 3-year-old child due to mitral valve ring dehiscence is described. The dehiscence of mitral valve ring was diagnosed utilizing real-time three-dimensional echocardiography. The patient subsequently underwent reoperation with successful resolution of hemolysis after replacement of the ring.

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