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.

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.

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.