Case report of combined pediatric heart-lung-liver transplantation.

We present the youngest successful patient with combined heart-lung-liver transplantation. The patient was a 2.5-year-old child with Alagille syndrome suffering from tetralogy of Fallot with pulmonary atresia and multiple aortopulmonary collaterals and familial cholestasis.

Interim mortality in infants with systemic-to-pulmonary artery shunts.

BACKGROUND: This retrospective review examines the risks and causes of death in infants with aortopulmonary shunts between the time of hospital discharge and planned reintervention. METHODS: From January 1991 through December 2000, a total of 146 infants aged 60 days or less underwent placement of systemic-to-pulmonary artery shunts and were discharged from the hospital alive. Inpatient, outpatient, and autopsy records were reviewed. RESULTS: Indications for surgery were single-ventricle anatomy in 90 cases and complex double-ventricle anatomy in 56. Of the patients, 21 (14%) died after discharge and before further planned surgery. Of these 21 infants, 17 (81%) were clinically doing well before sudden death. Autopsies were obtained in 15 cases and attributed the cause of death to shunt thrombosis in 5 infants (33%), myocardial infarction in 2 (13%), and pneumonia or lung disease in 3. Five autopsies were nondiagnostic. The mortality of patients discharged on aspirin (11.1%) was almost identical to that of patients discharged on no anticoagulation (12.3%). Four infants with sudden death had been notably irritable for 24 to 48 hours before death. CONCLUSIONS: There is a significant incidence of sudden death among infants who have undergone shunting. Death may be preceded by unexplained irritability, and such symptoms should therefore be carefully evaluated. Autopsy-proven shunt thrombosis is one of the leading causes of interim sudden death, and aspirin therapy may not be helpful. Options to reduce interim mortality include alternative regimens of anticoagulation (such as low-molecular weight heparin), alternative conduit material, and earlier reoperation.

Successful late reintervention after the arterial switch procedure.

BACKGROUND: The arterial switch operation is the therapy of choice for transposition of the great arteries. METHODS: A retrospective analysis of all children undergoing the arterial switch operation between November 1985 and October 2000 was conducted, highlighting the frequency and nature of late invasive reintervention. RESULTS: One hundred forty-four children were operated on. Operative survival was 89% (128 of 144). Late reintervention was required in 23% (29 of 128) of survivors. Neopulmonary stenosis (PS) was the most common complication requiring treatment, occurring in 16% (21 of 128) of patients. Eleven of 21 patients with PS required reoperation, whereas 10 were managed with percutaneous techniques. Other indications for reintervention included aortic arch obstruction (3 patients), ventricular septal defect (with PS in 2 patients), bronchial stenosis (2 patients), coronary stenosis (2 patients), aortic stenosis (with PS in 1 patient), and residual atrial septal defect (1 patient). There has been no mortality or major morbidity in those children who have undergone reintervention. CONCLUSIONS: Invasive reintervention after the arterial switch operation is occasionally required. The most common indication is PS. Reintervention is well tolerated by those children who require it. Continued follow-up for late complications is required in this patient population.

Autologous patch angioplasty of the left main coronary artery in a pediatric patient: 7-year follow-up.

We present a patient who developed ischemia after an arterial switch procedure for transposition of the great vessels secondary to left coronary artery stenosis. The excellent intermediate-term result of patch angioplasty of the left main coronary artery with the use of an internal thoracic artery patch is outlined.