Transposition with absent pulmonary valve syndrome: early repair of a rare case.

The congenital absence of the pulmonary valve cusps can occur either isolated or in association with other heart lesions. We report a very rare case of a 40-day-old infant with transposition of the great arteries, ventricular septal defect, pulmonary annular stenosis, absent pulmonary valve and aneurysmal dilation of the central pulmonary arteries, who received surgical treatment at our institution.

Twenty-five-year experience with rastelli repair for transposition of the great arteries.

OBJECTIVE: Our purpose was to describe the outcome of the Rastelli repair in D -transposition of the great arteries and to determine the risk factors associated with unfavorable events. METHODS: From March 1973 to April 1998, 101 patients with D -transposition of the great arteries and ventricular septal defect underwent a Rastelli type of repair. Median age and weight were 3.1 years (10th to 90th percentiles 0.3-9.9 years) and 12.8 kg (5.9-28.2). Pulmonary stenosis was present in 73 patients and pulmonary atresia in 18; 10 patients had no left ventricular outflow tract obstruction. RESULTS: There were 7 early deaths (7%) and no operative deaths in the last 7 years of the study. Risk factors for early death, by univariable analysis, included straddling tricuspid valve (P =.04) and longer aortic crossclamping times (P =.04). At a median follow-up of 8.5 years, there were 17 late deaths and 1 patient had undergone heart transplantation. Forty-four patients had reoperations for conduit stenosis, 11 for left ventricular outflow tract obstruction, and 28 had interventional catheterization to relieve conduit stenosis. Nine patients had late arrhythmias, and there were 5 sudden deaths. Overall freedom from death or transplantation (Kaplan-Meier) was 82%, 80%, 68%, and 52% at 5, 10, 15, and 20 years, respectively. Freedom from death or reintervention (catheterization or surgical treatment) was 53%, 24%, and 21% at 5, 10, and 15 years of follow-up, respectively. CONCLUSIONS: The Rastelli repair can be performed with low early mortality. However, substantial late morbidity and mortality are associated with conduit obstruction, left ventricular outflow tract obstruction, and arrhythmia.

Routine left atrial catheterization for the post-operative management of cardiac surgical patients: is the risk justified?

OBJECTIVE: To assess the risk/benefit ratio, including cost, associated with routine left atrial catheterization for the post-operative management of patients after cardiac surgery. METHODS: From November 1991 to June 1998, out of 6187 open heart procedures performed at our institution, 5815 patients (94%) receive a left atrial monitoring line inserted invasively by a unique and reproducible technique. Catheters were removed on the first or second postoperative day before chest tube removal. A subgroup of 385 patients (7%) were switched to a Swan-Ganz catheter postoperatively. RESULTS: A total of 14 patients suffered a complication related to the left monitoring line (0.24%). Bleeding necessitating transfusion occurred in 10 patients (0.17%), seven of whom required surgical reexploration (0.12%). Catheter retention occurred in four cases (0.07%), requiring a mediastinal reexploration in one and a minimally invasive procedures in three, for removal. No other complications related to the presence of a left atrial line emerged in this series. Apart from providing crucial hemodynamic information on a routine basis, in nine selected patients (0.15%) in a low cardiac output state with increased pulmonary vascular resistance and right ventricular failure, the left atrial line was used as a preferential route for catecholamine infusion, with significant hemodynamic improvement. CONCLUSIONS: Complications of left atrial monitoring catheters in cardiac surgery do occur but at a very low and acceptable rate. No mortality was correlated to their use in our series. Complication rate can be further lowered by a meticulous management of the device. The wealth of information and therapeutic options offered by this line appears to outweigh the associated risk.