Etiology and management of chylothorax following pediatric heart surgery.

BACKGROUND: Chylothorax after congenital heart surgery (CHD) is a potentially challenging complication. The purpose of this study was to review our experience with the management of chylothorax following congenital heart surgery. METHODS: Between September 1997 and August 2006, of 1341 pediatric patients undergoing correction of congenital heart disease in our institution, 18 (1.3%) developed chylothorax postoperatively. Surgical procedures included tetralogy of Fallot repair in 10 patients, ventricular septal defect closure (one), atrial septal defect with pulmonary stenosis repair (one), Fontan procedure (three), coarctation of the aorta repair (one), aortopulmonary shunt (one), and ligation of patent ductus arteriosus in one patient. All patients followed a therapeutic protocol including complete drainage of chyle collection and controlled nutrition. Somatostatin was used adjunctively in six (33.3%) patients. Surgical intervention was reserved for persistent lymph leak despite maximal therapy. Following resolution of chylothorax, a medium-chain triglyceride diet was implemented for six weeks. RESULTS: There were no deaths. Fifteen patients (83.3%) responded to conservative therapy. Lymph leak ranged from 2.5 to 14.7 mL/kg per day for 8 to 42 days. Three patients with persistent drainage required thoracotomy with pleurodesis to achieve resolution, in two of which previously attempted chemical pleurodesis with doxycycline proved ineffective. Duration of lymph leak in this subgroup ranged from 15 to 47 days with 5.1 to 7.4 mL/kg per day output. CONCLUSIONS: Postoperative chylothorax is an infrequent complication of surgery for congenital heart disease and can occur even after median sternotomy in the absence of pathologically elevated venous pressure or Fontan circulation. Although hospitalization can be prolonged, conservative therapy is effective in most cases, while surgical pleurodesis proved successful in the refractory cases.

Hybrid procedures for complex congenital cardiac lesions.

BACKGROUND: We present an alternative treatment employing a hybrid approach used in 3 patients with congenital heart disease. The goal was to provide optimal therapy by minimizing the potentially harmful effects of methods that accompany conventional surgical procedures. METHODS: Two patients aged 4 and 6 months underwent beating-heart closure of a muscular ventricular septal defect (VSD) with an occluding device. In addition, an 8-year-old patient with supraaortic, main, and branch pulmonary artery (PA) stenosis underwent conventional surgical patch augmentation of the ascending aorta and the main PA and intraoperative stenting of the branch PA stenoses. RESULTS: No patient deaths occurred. One patient developed a postoperative pneumothorax. Median intensive care unit and hospital stays for the VSD patients were 1 and 5 days and for the other patients 2 and 20 days, respectively. At median follow-up of 25 months, all patients were well and had required no further interventions. CONCLUSIONS: Patients with muscular VSD can currently be treated with the hybrid approach. Intraoperative PA stenting in addition to conventional surgical repair can be performed safely and may be complementary in patients with complex lesions.

Mid-term results following surgical treatment of congenital cardiac malformations in adults.

The long term consequences of untreated of residual or recurrent lesions pose unique challenges in the growing population of adults with congenitally malformed hearts. In our unit, 335 patients aged from 18 to 72 years, with a mean age of 35 plus or minus 14 years, presented for correction of congenital cardiac disease from September, 1997, through December, 2006. Of the group, 42 (12.5%) had undergone one or more prior surgical procedures, 3 were admitted as emergencies, and a further 10 (3%) had suffered prior cardiac related complications. Symptoms had been noted by 181 patients (54%), and 42 (12.5%) had an established arrhythmia. Chromosomal anomalies were identified in 13 (3.8%), and diagnostic catheterisation was required in 201 (60%) patients. Of the overall group, 2 patients died early (0.6%). Complications occurred in 61 patients (18%), including atrial fibrillation, pneumothorax, postoperative haemorrhage, pericardial or pleural effusions requiring drainage, stroke, complete heart block, endocarditis, wound dehiscence, and peripheral neuropathy. The median length of stay in the intensive care unit and hospital were 2 and 7 days, respectively. Death occurred later in 2 further patients (0.6%), due to atrial fibrillation and pulmonary hypertension. At mean follow-up of 63 plus or minus 30 months, the majority of the remaining patients are well with resolution or significant improvement in their symptoms. Despite the long term deleterious effects of untreated, residual or recurrent congenital cardiac lesions in adults, therefore, we conclude that surgical correction can be achieved with low mortality and acceptable morbidity. Most significant complications are related to arrhythmias.

Partial right-sided pericardial defect associated with congenital aortic valve disease.

Partial right-sided pericardial defect is an extremely rare congenital anomaly and is often associated with other congenital abnormalities. We describe a unique case of congenital aortic valve disease associated with right-sided pericardial defect. The clinical implications are discussed and a review of the literature is presented.