Superior cavopulmonary anastomosis timing and outcomes in infants with single ventricle.

Objectives: We sought to identify factors associated with the timing and surgical outcomes of the superior cavopulmonary anastomosis.

Methods: The Pediatric Heart Network's Infant Single Ventricle trial database identified participants who underwent superior cavopulmonary anastomosis. Factors potentially associated with age at superior cavopulmonary anastomosis, length of stay and death by 14 months of age were evaluated.

Challenges in echocardiographic assessment of mitral regurgitation in children after repair of atrioventricular septal defect.

The validity and reproducibility of echocardiographic methods used to quantify mitral regurgitation (MR) in children with congenital heart disease are unknown. We evaluated the usefulness of methods used to quantify MR in children enrolled in a multicenter trial of enalapril 6 months after surgical repair of an atrioventricular septal defect (AVSD). MR severity in this trial was assessed using body surface area (BSA)-adjusted vena contracta lateral (i-VCW(lat)) and anterior-posterior (i-VCW(ap)) dimensions and cross-sectional area (i-VCA), regurgitant volume/BSA, regurgitant fraction, and qualitative MR grade.

Late status of Fontan patients with persistent surgical fenestration.

Objectives: This study was undertaken to determine the effects of creating a systemic-to-pulmonary venous atrial-level communication (fenestration) at the time of the Fontan procedure on late outcomes.

Background: Fenestrations are frequently performed during Fontan procedures, but late consequences are not well described.

Methods: Patient characteristics were compared between those with and without surgical fenestration among 536 subjects (mean age 11.

Retropharyngeal edema: an unusual manifestation of Kawasaki disease.

Background: Kawasaki disease (KD) is an acute multisystem vasculitis of unknown etiology that typically affects young children. KD presenting as a retropharyngeal inflammatory process is very rare.

Objectives: To report a case of KD initially presenting as a retropharyngeal edema mimicking a deep neck infection, and to review previously published reports in the literature.

Video-assisted ductal ligation in premature infants.

Background: Video-assisted thoracic surgery has been shown to be a safe and effective method of closing the patent ductus arteriosus in infants and children. We have applied this technique in low birth weight premature infants and now report our experience.

Methods: Since 1996, we have used video-assisted thoracic surgery ligation as the treatment of choice for all patent ductus arteriosus, including 100 performed on premature infants (23 to 31 weeks' gestation, mean 25.

A combination of physical examination and ECG detects the majority of hemodynamically significant heart defects in neonates with Down syndrome.

Echocardiography has become the method of choice in the diagnosis of a congenital heart defect (CHD) in neonates with Down syndrome. The most compelling argument for diagnosis of CHD in the neonatal period is the need for early surgical intervention (ideally prior to 6 months of age) in those with a complete atrioventricular (AV) canal. We evaluated the efficacy of a combined approach of physical examination (PE) and electrocardiography (ECG) in the detection of CHD in 49 neonates with Down syndrome.

Primary care physicians' use of screening echocardiography.

Objective: To survey primary care physicians to understand their reasons for using echocardiography to screen for congenital heart disease in children and to assess their understanding of the costs associated with cardiology services.

Design: A questionnaire.

Participants: Eight hundred sixty-seven pediatricians and family physicians in our region were surveyed, 494 (57%) responded and 466 were used for the analysis.

Growth of the right heart after balloon valvuloplasty for critical pulmonary stenosis in the newborn.

Eight newborns with critical pulmonary stenosis and intact ventricular septum underwent successful percutaneous balloon dilation. Follow-up using echocardiography showed sustained relief of the obstruction across the pulmonary valve and excellent growth of the right heart structures.

Cardiac effects of dexamethasone in very low birth weight infants.

Objective: To characterize the cardiac effects of dexamethasone in very low birth weight infants.

Design: Prospective, randomized, placebo-controlled, double-blind trial. Enrolled subjects were randomized to receive either a 42-day tapering course of dexamethasone or a saline placebo.

Cardiovascular function during rest and exercise in patients with sickle-cell anemia and coexisting alpha thalassemia-2.

Cardiac function was measured at rest and during exercise in 9 patients with sickle-cell anemia (SS) and coexisting homozygous alpha thalassemia-2 (alpha thal-2). Results were compared with 18 sickle cell patients with normal alpha globin genes, who were matched to the study group by age, gender, and size, and to published normal values. SS alpha thal-2 patients were less anemic: 9.

The heart in sickle cell anemia. The Cooperative Study of Sickle Cell Disease (CSSCD).

The objective of this study was to obtain representative echocardiographic measurements of cardiac size and function in stable patients with sickle cell disease. This prospective, multicenter study utilized central reading of echocardiograms by an investigator blinded to other patient data. Stable outpatients from a balance of inner city and rural settings with SS phenotype and a broad age range were selected, because conflicting results from earlier studies were believed to be due to these patient selection criteria.

A mutation in FBN1 disrupts profibrillin processing and results in isolated skeletal features of the Marfan syndrome.

Dermal fibroblasts from a 13-yr-old boy with isolated skeletal features of the Marfan syndrome were used to study fibrillin synthesis and processing. Only one half of the secreted profibrillin was proteolytically processed to fibrillin outside the cell and deposited into the extracellular matrix. Electron microscopic examination of rotary shadowed microfibrils made by the proband's fibroblasts were indistinguishable from control cells.

Fetal cardiovascular hemodynamics in the presence of complete atrioventricular block.

Objective: Our purpose was to follow serially the hemodynamic adaptation to a congenital complete heart block in a human fetus.

Study Design: Longitudinal and serial M-mode and Doppler echocardiography over a 10-week span were performed on a fetus affected by complete heart block. Ventricular fractional shortening, size, and flow across the atrioventricualr valves and outflow tracts were determined starting at 20 weeks up to the time of delivery.

Echocardiographic demonstration of a ventriculocoronary artery communication in a neonate with hypoplastic left heart syndrome.

A neonate diagnosed with aortic atresia and mitral hypoplasia had a ventriculocoronary artery communication demonstrated by use of echocardiography. These communications have been noted by angiography and on pathologic specimens in the past, but their clinical importance in the surgical management of these patients remains unclear. The ability to noninvasively identify these communications and study their flow characteristics would help to define their impact on surgical outcome.

Najjar syndrome revisited.

We describe 2 brothers with cardiomyopathy and hypergonadotropic hypogonadism and conclude that this is the first description of the Najjar syndrome in the United States. The inheritance may be autosomal recessive.

The significance of ductal shunting during extracorporeal membrane oxygenation.

The purpose of this study was to evaluate the significance and direction of shunts at the level of the foramen ovale or ductus arteriosus in full-term newborns with neonatal respiratory failure who were placed on extracorporeal membrane oxygenation (ECMO). A decrease in left ventricular dimension was expected when infants were placed on ECMO but did not occur. A left-to-right shunt was demonstrated at the ductal level in nine of 12 infants early in the course of ECMO before pulmonary resistance decreased.

Left ventricular filling in sickle cell anemia.

M-mode echocardiography was performed on 11 normal black subjects and 38 patients with sickle cell anemia while they were at rest to evaluate their left ventricular (LV) systolic and diastolic function. The patients with sickle cell anemia were also evaluated by radionuclide exercise tests and, based on their ejection fraction (EF) response, were separated into 2 groups: a group with a normal EF response to exercise (73 +/- 9%, mean +/- standard deviation) and a group with an abnormal EF response to exercise (53 +/- 9%). Computer-assisted analysis of the M-mode echocardiograms identified abnormalities of diastolic function (impaired left ventricular filling) in patients with sickle cell anemia compared with the normal subjects.

The conduction system in patients with a prolonged QT interval.

The conduction system and the myocardium of five patients with Romano-Ward syndrome and one patient with the Jervell and Lange-Nielsen syndrome were studied to determine the cause of the prolonged QT interval. The patients were 9 and 15 months and 2, 5 and 19 years of age, respectively. All had a prolonged QTc interval.