One-Stage Operation for Cantrell's Pentalogy

Cantrell's pentalogy may be defined as a failure of fusion of the midline from the sternum to the umbilicus. Thus, this malady consists of multiple anomalies of the sternum, heart, pericardium, diaphragm and umbilicus or anterior abdominal wall. According to the degrees of each anomaly, various operations can be planned as a one-stage operation or as a multi-stage operation and then palliative or corrective operations. The authors experienced a case of Cantrell's pentalogy that consisted of a bifid sternum, ventricular septal defect, atrial septal defect, ventricular diverticulum, dextrocardia, pericardial defect, anterior diaphragmatic defect and diastasis recti; all of these problems were corrected by a one-stage operation.

Results of Extracardiac Pericardial-flap Lateral Tunnel Fontan Operation / 대한흉부외과학회지

BACKGROUND: Extracardiac pericardial-flap lateral tunnel Fontan operation has theoretical advantage of growth potentiality of the extracardiac tunnels. The mid-term results of this technique and morphologic change of the lateral tunnel were studied. MATERIAL AND METHOD: Clinical data was reviewed in 42 patients who underwent extracardiac pericardial-flap lateral tunnel Fontan operation between November 1993 and December 2004. The age was 2.8+/-1.5 years and the body weight was 12.3+/-3.2 kg. Extracardiac tunnel was constructed using the pedicled pericardium with the base undetached. By reviewing the follow-up cardiac angiograms, the diameter and the cross-sectional area of the lateral tunnel was compared to those of inferior vena cava. RESULT: There were four operative mortality cases (9.8%) and the causes of death were low cardiac output for all four cases. Postoperatively, five patients had prolonged pleural effusion longer than two weeks and one patient required a permanent pacemaker due to complete heart block. Follow-up was possible in 37 patients and the follow up duration was 3.8+/-2.2 years. During that period, one patient died, of upper gastrointestional bleeding combined with heart failure and one patient died a sudden death of unknown cause. Two patients required reoperation due to subaortic stenosis and anastomosis site stenosis between inferior vena cava and lateral tunnel. In one patient, bradyarrhythmia was anew but there was no thromboembolic complication. The lateral tunnel showed growth in proportion to the size of the inferior vena cava. CONCLUSION: Extracardiac pericardial-flap lateral tunnel Fontan operation is relatively simple and safe. The mid-term result was favorable and the extracardiac tunnel showed potential for growth.