Permanent transvenous dual-chamber pacing using the coronary sinus in a patient with a mechanical prosthetic tricuspid valve.

Ventricular pacing in patients with mechanical prosthetic tricuspid valves usually requires an epicardial approach. Epicardial pacing requires at least a limited thoracotomy and long-term pacing thresholds are generally inferior to thresholds achieved with transvenous pacing. This case report describes the use of a coronary sinus lead designed for left ventricular pacing as the sole ventricular lead in a dual-chamber pacing system in a young male with persistent complete atrioventricular block following remote ventricular septostomy and mechanical tricuspid valve replacement. With the development of dedicated, transvenous, left ventricular leads with stable positions and reliably low thresholds, ventricular pacing via the coronary sinus is a viable option when right ventricular pacing is impossible.

Concentric placement of stents to relieve an obstructed anomalous pulmonary venous connection.

A 9-month-old male with asplenia and complex congenital heart disease experienced progressive stenosis of an anomalous pulmonary venous connection. He developed pulmonary edema and growth failure. Two stents were placed concentrically to relieve the stenosis, and the pulmonary edema and growth failure resolved. Definitive surgery was accomplished 8 months later.

Multiple congenital anomalies associated with weekly low-dose methotrexate treatment of the mother.

This report describes an infant with multiple congenital anomalies born to a 20-year-old mother with juvenile rheumatoid arthritis who had been taking weekly low-dose methotrexate (MTX) during the first trimester of pregnancy. The abnormalities found were consistent with those associated with maternal ingestion of MTX at dosage levels used to induce abortions, i.e., the group of abnormalities referred to as the "aminopterin syndrome." Although weekly low-dose MTX has been associated with spontaneous abortions, this is, to our knowledge, the first case report describing multiple congenital abnormalities consistent with MTX embryopathy secondary to weekly low-dose MTX treatment.

Neonatal mitral valve endocarditis: diagnosis and successful management.

Although still considered to be a rare and mostly fatal illness, the reported incidence of neonatal bacterial endocarditis has increased during the past decade. This parallels the establishment of intensive supportive management of severely ill newborns with multiple medical problems. An extremely difficult diagnostic problem with protean manifestations, the presence of neonatal endocarditis should be vigorously sought in the at risk neonate. The authors report the first known case in America of successfully diagnosed and treated neonatal endocarditis involving the mitral valve.

Transatrial repair of double-chambered right ventricle.

Double-chambered right ventricles (DCRVCs) traditionally have been repaired via a right ventriculotomy. We describe the cases of 8 children (aged 7 months to 6 years; weight, 6.3 to 21.5 kg), who underwent repair of DCRV via a right atriotomy. Six patients had ventricular septal defects. Peak systolic gradients in the right ventricular outflow tract were 26 to 135 mm Hg. The QP/QS were 1.1 to 2.5. Cardiopulmonary bypass times were 52 to 89 min (mean, 65.5 min) with aortic cross-clamp times of 20 to 56 min (mean, 39 min). Mean intraoperative gradients following repair were 5.8 mm Hg. Three patients had transient postoperative atrioventricular block requiring temporary pacing. Two patients required minimal inotropic support (dopamine, 2.5 to 5 micrograms/kg/min). The children have all been asymptomatic after hospitalization with follow-up times of 5 months to 4.5 years. No patient requires cardiac medications or has significant, persistent dysrhythmias; however, 1 patient required reoperation. Transatrial repair of DCRV allows excellent operative anatomical visualization and should be considered as an alternative to the transventricular approach in patients with this congenital heart defect.

False aneurysm of the aorta secondary to an esophageal foreign body.

False aneurysm of the aorta developed in a 14-month-old child following endoscopic removal of an open safety pin from the esophagus. The diagnostic techniques and operative repair of this rare complication of foreign body ingestion are described.

Tetralogy of Fallot and coarctation of the aorta: successful repair in an infant.

A 4 1/2-month-old infant was referred to our center with the rare combination of tetralogy of Fallot and coarctation of the aorta. Despite the presence of severe right ventricular outflow obstruction, the infant was virtually acyanotic because of the systemic afterload associated with the coarctation. She had marked systemic hypertension. The embryological and therapeutic implications of this child's defects are discussed.

Aortic atresia with double inlet left ventricle, rudimentary left sided right ventricle, and ventriculoarterial discordance.

Aortic atresia associated with ventriculoarterial discordance (transposition) was found at necropsy in a 3 month old neonate. This is a rare association.