Left ventricular noncompaction (LVNC) is a form of cardiomyopathy resulting from a disorder of endomyocardial morphogenesis. It has been associated with significant morbidity and mortality. The aim of this study was to characterize associated cardiac findings in children with LVNC and to identify risk factors associated with increased mortality. From our echocardiography database, we identified 46 patients diagnosed with LVNC between December 1999 and February 2005. The mean age at presentation was 3.6 +/- 5.6 years, and the mean duration of follow-up was 1.9 +/- 2.1 years. Left ventricular ejection fraction was decreased in 24 patients (52%; mean 39.5% +/- 13.1%). Thirty-six patients (78%) had associated cardiac lesions, including atrial septal defect (n = 16 [35%]), ventricular septal defect (n = 17 [37%]), patent ductus arteriosus (n = 14 [30%]), and Ebstein's anomaly (n = 5 [11%]). Electrocardiogram abnormalities were found in 80% of patients; most commonly they included left (n = 15 [43%]) and right ventricular hypertrophy (n = 19 [54%]). Documented arrhythmias included ectopic atrial rhythm (n = 2), junctional rhythm (n = 2), supraventricular tachycardia (n = 2), and ventricular tachycardia (n = 1). Overall mortality was 20%, and there was no association with ejection fraction, morphologic defect, or arrhythmia. Mean age at diagnosis in survivors (4.5 +/- 6.1 years) was higher than nonsurvivors (0.4 +/- 0.7 years) (p < 0.0001). LVNC is a rarely isolated form of cardiomyopathy, and it is associated with significant additional cardiac abnormalities. Although it does not have an invariably fatal course, early presentation in infancy does carry an increased risk of mortality.

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Use of central lines in the neonatal intensive care unit (NICU) has led to the formation of intracardiac thrombi. A paucity of data exists on the management of neonatal cardiac thrombi, with the few reported cases focusing on outcomes following thrombolytic therapy. This study was undertaken to evaluate the outcome of cardiac thrombi in neonates who do not receive thrombolytic therapy. Nineteen patients younger than 3 months of age diagnosed with cardiac thrombi were included. All 19 patients had a central line. Management consisted of a combination of antibiotics and low-molecular-weight heparin (n = 16) or surgical removal (n = 2). In one case, no treatment was instituted. One patient was lost to follow-up after partial resolution of the thrombus. Complete thrombus resolution occurred in 18 patients, 9 with negative blood cultures and 9 with positive blood cultures. It took longer for resolution of thrombi associated with positive blood cultures than for sterile thrombi. No patient had evidence of thrombus embolization. From these data we concluded that the natural history of cardiac thrombi is resolution. Infected thrombi require more prolonged therapy. Surgery is seldom required and thrombolytics are not usually necessary for clot resolution.

Digoxin has been an effective treatment for fetal supraventricular tachycardia (SVT), but second-line therapy remains more controversial. Thirty-seven cases of fetal SVT were identified that received digoxin as first-line therapy. Seventeen fetuses (46%) converted to and maintained normal sinus rhythm. Flecainide was used in 13/15 patients requiring second-line therapy; 12/13 (92%) converted to sinus rhythm. Of seven hydropic fetuses, five required second-line therapy and were then successfully converted with flecainide. The improved efficacy of flecainide was statistically significant with a p value <0.01. Complete follow-up was available in 13 digoxin-treated and in 12 second-line therapy infants. Prolonged or multiple drug therapy for postnatal arrhythmia management was required in 3/13 (23%) patients in the digoxin group and in 8/12 (67%) patients requiring second-line therapy. This demonstrated a correlation between the need for second-line fetal therapy and more complex postnatal management with a p value of 0.003. Digoxin remains an effective first-line therapy in the treatment of fetal SVT. Flecainide is an effective second-line therapy, especially in the face of fetal hydrops. Use of second-line therapy in fetal SVT is a predictor of complex postnatal course, and these patients should be followed more closely.

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Children with single ventricles and those with palliated d-loop transposition of the great arteries suffer somatic growth delay prior to definitive surgery, despite being of normal size at birth. Catch-up growth in weight occurs after the Fontan and Mustard operations. In the Mustard population, height also normalizes, whereas in patients with univentricular circulation, height remains abnormally low.