Ellis-van Creveld syndrome and congenital heart defects: presentation of an additional 32 cases.

Ellis-van Creveld (EVC) syndrome is a rare genetic abnormality that has been linked to a mutation in the EVC or EVC2 genes. Common atrium (CA) is an uncommon cardiac malformation, and yet it is commonly found in patients with EVC. We performed a retrospective review of the cases submitted to the Pediatric Cardiac Care Consortium (PCCC) between 1982 and 2007.

Pediatric Cardiac Care Consortium: an instrument for evidence-based clinical decision support.

Pediatric Cardiac Care Consortium is a registry of cardiac catheterizations, surgical operations, and autopsies performed for infants, children, and adults with congenital heart disease. Four examples of use of PCCC data to evaluate variability in morphology, management, and outcomes for the procedures are described. Consideration is given to the following clinical problems: (1) the experience with surgical heart block in operative closure of perimembranous VSD, (2) the transition away from atrial baffle operations to the arterial switch operation for simple transposition of the great arteries, (3) the experience of planned 3 stage palliation of hypoplastic left heart syndrome, and (4) the identification of a high risk combination of cardiovascular anomalies in Williams syndrome.

Cardiac catheterization and operative outcomes from a multicenter consortium for children with williams syndrome.

Williams syndrome is a multifaceted disorder that includes a spectrum of cardiovascular anomalies. Due to its rare occurrence, outcome data for operations and cardiac catheterization are limited. We reviewed data from 242 individuals from the Pediatric Cardiac Care Consortium (PCCC) with Williams syndrome and associated cardiovascular lesions, and their frequency, and assessed their effects on mortality.

Early outcomes of tricuspid valve replacement in young children.

Background: Early outcomes after tricuspid valve replacement in young children are ill defined. The experience of the Pediatric Cardiac Care Consortium (45 centers, 1984 to 2002) was reviewed to evaluate the results of tricuspid valve replacement in children <6 years of age.

Methods And Results: Ninety-seven patients who underwent initial tricuspid valve replacement are included in the present analysis.

Cri du chat syndrome and congenital heart disease: a review of previously reported cases and presentation of an additional 21 cases from the Pediatric Cardiac Care Consortium.

Objectives: To analyze the cases submitted to the Pediatric Cardiac Care Consortium (PCCC) database from 1982 to 2002 to determine the frequency and distribution of congenital heart disease (CHD) found in this population, to review the literature for previously published accounts of CHD in this population, and to review current genotype-phenotype associations for cri du chat (CDC) syndrome with CHD.

Methods: We performed a retrospective review of the 98422 CHD cases submitted to the PCCC between 1982 and 2002, to find patients who had a noncardiac diagnosis of CDC syndrome.

Results: A total of 21 patients (15 female and 6 male patients) were identified.

Early outcome after Glenn shunt and Fontan palliation and the impact of operation during viral respiratory season: analysis of a 19-year multi-institutional experience.

Background: This study was done to investigate the impact of season of operation, age at operation, and surgical era on short-term outcome after single ventricle palliation. One small study suggests that operation during viral respiratory season is associated with prolonged pleural effusion and hospitalization. Single-institution studies also find improved outcomes after the Glenn shunt and Fontan over time, despite operation at a younger age.

Effectiveness of cardiac surgery in trisomies 13 and 18 (from the Pediatric Cardiac Care Consortium).

Because of severely reduced lifespan in children with trisomies 13 and 18, surgical repair of congenital heart lesions has rarely been offered. With data from a multicenter registry, we report 35 cases of cardiac surgery in infants and children with trisomy 13 or 18 with a 91% hospital survival rate. Those patients without an extended preoperative ventilatory requirement did not require prolonged mechanical ventilation after surgery.

Predictors of prosthesis survival, growth, and functional status following mechanical mitral valve replacement in children aged <5 years, a multi-institutional study.

Background: Prosthesis survival, growth, and functional status after initial mechanical mitral valve replacement (MVR) in children <5 years of age are poorly defined.

Methods And Results: The experience of the Pediatric Cardiac Care Consortium (45 centers, 1982 to 1999), which included 102 survivors after initial MVR, was analyzed. Median follow-up: 6.