Ectopia cordis.

Ectopia cordis is a rare congenital defect in which the heart is placed externally on the surface of the chest. This article describes the embrylogic events that lead to the various classifications of the defect and how they possibly explain the process of its unusual occurrence in children. While survival in some cases is possible, the ultimate repair is difficult and survival is rare. The most extreme forms of ectopia cordis, especially those with intracardiac defects, have a poor prognosis. Several cases of this rare anomaly with primary repair and staged repair are discussed.

Removal of an infected ventricular septal defect patch after tetralogy repair.

An infected Dacron ventricular septal defect (VSD) patch was removed 15 years after repair of tetralogy of Fallot. Cardiopulmonary bypass was not utilized, and no significant hemorrhage was encountered. The patient recovered uneventfully and continues to do well 7 years after surgery without infection or residual VSD.

Hemi-Fontan procedure for hypoplastic left heart syndrome: outcome and suitability for Fontan.

BACKGROUND: Following the Norwood procedure for hypoplastic left heart syndrome (HLHS), pulmonary artery distortion and hypoplasia are common and may negatively impact late outcome. The hemi-Fontan procedure (HFP) augments the central pulmonary arteries and establishes a connection between the right atrial/superior vena cava junction and the pulmonary arteries, while excluding the inferior vena cava. METHODS: The hospital records of all 114 patients undergoing a HFP for HLHS between August 1993 and April 1998 were reviewed to assess patient, procedural, and morphologic determinations of outcome. The results of cardiac catheterization, Doppler/echocardiography, 12 lead electrocardiograms, hospital and subsequent course, as well as suitability and outcome for the Fontan procedure were analyzed. RESULTS: Mean age was 5.4 months (range 1.5 to 15 months). Right ventricular function was normal in 95 patients, moderately depressed in 14, and severely depressed in five. Tricuspid regurgitation was absent or mild in 91 patients, moderate in 13, and severe in 10. Concomitant procedures included left superior vena cava to pulmonary artery anastomosis (12), tricuspid valve repair (10), pulmonary artery stent placement (3), coarctation repair (2), and aortic pseudoaneurysm repair (1). Hospital survival was 112/114, 98% (95% confidence interval [CI]: 95% to 100%). There were two late deaths, one noncardiac. Sinus rhythm is present in 105 patients (92%, 95% CI: 87% to 97%). To date, 79 of these patients have undergone the Fontan procedure with 74 survivors (94%, 95% CI: 89% to 99%). CONCLUSIONS: The HFP may be performed with excellent results for HLHS. It effectively augments the central pulmonary arteries while preserving sinus rhythm in the majority. In addition, the HFP facilitates the subsequent Fontan procedure and has significantly improved the overall outcome.

Arteriovenous malformation of the esophagus disguised as a leiomyoma.

Hemangiomas, benign tumors of vascular origin, compromise less than 3% of all nonmalignant esophageal neoplasms. Because of their infrequent occurrence, such vascular tumors may be mistaken for more common benign tumors of the esophagus. We report the successful surgical management of an esophageal arteriovenous malformation presenting with clinical, radiographic, and endoscopic features resembling those of a leiomyoma.

Emergency vascular access in a patient with limb agenesis.

A 19-year-old white man with congenital agenesis of all four limbs arrived in the emergency room in full cardiopulmonary arrest secondary to Reye's syndrome. The patient was intubated but had no vascular access. After a percutaneous femoral venous line proved unsuccessful, an internal jugular line was placed for venous access. Repeated attempts at percutaneous femoral and axillary arterial line placements were unsuccessful. A femoral cutdown showed 2-mm femoral vessels. The autopsy showed increasingly hypoplastic aortic, iliac, and femoral vessels below the takeoff of the renal vessels. The subclavian vessels were reasonably normal in caliber. This case shows that femoral or axillary vessels in a congenitally amputated limb are not useful for vascular access, and a low threshold should exist for using internal jugular/subclavian veins and the subclavian/carotid arteries if emergency access is necessary.