Quality Improvement in Congenital Heart Surgery.

Congenital heart disease is a major public health concern in the United States. Outcomes of surgery for children with congenital heart disease have dramatically improved over the last several decades with current aggregate operative mortality rates approximating 3%, inclusive of all ages and defects. However, there remains significant variability among institutions, especially for higher-risk and more complex patients. As health care moves toward the quadruple aim of improving patient experience, improving the health of populations, lowering costs, and increasing satisfaction among providers, congenital heart surgery programs must evolve to meet the growing scrutiny, demands, and expectations of numerous stakeholders. Improved outcomes and reduced interinstitutional variability are achieved through prioritization of quality assurance and improvement.

Double aortic arch and aortopulmonary window: an uncommon presentation in a newborn.

We describe a rare association of aortopulmonary window and double aortic arch in a 1.7-kg newborn who presented with severe respiratory distress. A staged surgical approach was used because of the size of the patient and significant comorbidity. This approach resulted in excellent outcome.

Two-stage repair of truncus arteriosus associated with interrupted aortic arch in critically ill patients.

This report describes 2 newborns with truncus arteriosus associated with an interrupted aortic arch who underwent a 2-stage repair due to poor preoperative condition. A repair of the interrupted aortic arch and ventricular-to-pulmonary artery conduit with a 6-mm Gore-Tex tube was performed as a first stage. Closure of a ventricular septal defect and placement of a right ventricular-to-pulmonary artery homograft conduit were performed electively at 8 months of age.

Development of the pulmonary arteries after the Norwood procedure: comparison between Blalock-Taussig shunt and right ventricular-pulmonary artery conduit.

BACKGROUND: The Norwood-Sano procedure for hypoplastic left heart syndrome (HLHS) has been associated with improved postoperative hemodynamics and outcome. This study compared the effect of a Blalock-Taussig shunt with right ventricular-pulmonary artery (RV-PA) conduit before bidirectional Glenn on the development of the PAs. METHODS: Between January 2000 and June 2007, 42 patients with HLHS underwent bidirectional Glenn: 19 (mean age, 7 +/- 1.5 months) had a Blalock-Taussig shunt (3.5 mm in 15; and 4 mm in 4); 23 patients (mean age, 5 +/- 1 month) had RV-PA conduit (5-mm tube). Patients underwent postoperative cardiac catheterization and echocardiograms. RESULTS: Total PA (Nakata) index was 192 +/- 10 mm2/m2 in the Blalock-Taussig group and 238 +/- 18 mm2/m2 in the RV-PA conduit group (p = 0.03). In the RV-PA conduit patients, the left and right PAs had comparable diameters. In the Blalock-Taussig group, the left PA was smaller than the right (p = 0.02). The mean PA pressure at the end of the operation was 14 +/- 2 mm Hg in the Blalock-Taussig group and 11 +/-1 mm Hg in the RV-PA conduit group (p = 0.06). Persistent pleural effusion occurred in 4 Blalock-Taussig patients (21%) and in 1 (4%) with RV-PA conduit (p < 0.05). Postoperative arterial oxygen saturation was 80% +/- 2% in the RV-PA conduit group and 74% +/- 2% in the Blalock-Taussig group (p < 0.05). CONCLUSIONS: The Norwood procedure with RV-PA conduit may have favorable effects on the development of the PA due to even distribution of pulmonary blood flow.

Reimplantation of anomalous left coronary artery from the pulmonary artery without mitral valve repair.

BACKGROUND: Early establishment of a two-coronary artery system has become the standard surgical approach in patients with anomalous origin of the left coronary artery from the pulmonary artery. Improved surgical outcome is related to better perioperative management and surgical techniques. The need for mitral valve repair is still controversial, however. We report our long-term results with aortic implantation of the left coronary artery. METHODS: Between January 1992 and July 2005, 23 patients who had severe left ventricular dysfunction and mitral insufficiency underwent aortic implantation of the left coronary artery. Mean age and weight were 5 +/- 3 months (range, 2 to 14 months) and 5 +/- 1.5 kg (range, 3.2 to 7 kg). Repair was accomplished by creating a wide anastomosis between the left coronary artery and aorta that was augmented with autologous pericardium to avoid tension and distortion of the anastomosis. None of the patients had concomitant mitral valve repair. RESULTS: There were no operative or late deaths. One patient required extracorporeal membrane oxygenation for 86 hours and another for 100 hours because of sustained ventricular tachycardia and respiratory insufficiency. A younger age (<6 months) was associated with a longer stay in the intensive care unit (p = 0.04). During a mean follow-up of 78 +/- 30 months (range, 6 to 156 months) all patients were in the New York Heart Association functional class I. Serial echocardiograms showed complete recovery of left ventricular function in all patients within 6 months. Mitral valve function was normal in 17 patients and mildly impaired in 6. The mean shortening fraction increased from 0.2 +/- 0.05 preoperatively to 0.43 +/- 0.07 postoperatively (p = 0.03), and left ventricular end-diastolic dimension decreased from 44 +/- 7 mm preoperatively to 29 +/- 8 mm postoperatively (p = 0.02). CONCLUSIONS: Aortic implantation of the left coronary artery results in complete recovery of left ventricular function and no late mitral valve dysfunction.

Effects of controlled antegrade pulmonary blood flow on cardiac function after bidirectional cavopulmonary anastomosis.

BACKGROUND: Bidirectional cavopulmonary anastomosis (BCPA) has been used as an intermediate stage in the treatment of patients with single-ventricle physiology. Leaving additional antegrade pulmonary blood flow has been shown to improve postoperative arterial blood oxygen saturations; however, controversy continues over whether the potential increase in systemic venous pressure is detrimental. We studied the effects of controlled antegrade pulmonary blood flow on cardiac function in patients after BCPA. METHODS: From January 1993 to July 2000, 128 patients underwent BCPA. Mean age at operation was 6.2 +/- 4 months (range 2 to 36 months). In group 1 (n = 72), restricted antegrade pulmonary blood flow was maintained through a native narrowed pulmonary valve or by adjustment of previously placed pulmonary artery band with the goal of maintaining the mean pulmonary artery pressure less than 16 mm Hg. In group 2 (n = 56), BCPA was the only source of pulmonary blood flow. RESULTS: One hospital death (0.8%) occurred. The mean pulmonary artery pressure at the end of the operation was 13 +/- 2 mm Hg in group 1 compared with 12 +/- 2 mm Hg in group 2, a difference that was not significant. Patients in group 1 had higher arterial oxygen saturations (84% +/- 3% compared with 74% +/- 3% in group 2, p < 0.05), and shorter mean hospital stay (9 +/- 3 days compared with 15 +/- 2 days, p < 0.05). Persistent pleural effusion (> 10 days) or late chylothorax occurred in 4 patients from group 1 and 3 from group 2, a difference that was not significant. During a mean follow-up of 36 +/- 10 months no late deaths occurred. The mean oxygen saturation remained higher in group 1, 80% +/- 3% compared with 74% +/- 4% in group 2, and the hematocrit was lower, at 38% +/- 3% compared with 46% +/- 4% (p < 0.05 for both comparisons). Cardiac catheterizations were performed in 68 patients before completion Fontan. Total pulmonary artery (Nakata) index was 263 +/- 34 mm(2)/m(2) in group 1 (n = 40) and 188 +/- 13 mm(2)/m(2) in group 2 (n = 28) (p < 0.05). The mean pulmonary artery pressure and mean ventricular end-diastolic pressure were similar. CONCLUSIONS: Controlled antegrade pulmonary blood flow may have favorable effects on cardiac function for a selected group of patients and does not appear to have adverse effects on subsequent suitability for completion Fontan.

Interrupted Aortic Arch.

Interruption of the aortic arch is defined as absence of luminal continuity between the ascending and the descending aorta. This is not an isolated defect; it occurs in conjunction with other cardiac defects such as ventricular septal defect and patent ductus arteriosus. It is a rare malformation. It occurs in about 1% of the patients with congenital heart defects.

Treatment of persistent chylothorax after Norwood procedure with somatostatin.

A newborn who had undergone Norwood procedure for hypoplastic left heart syndrome developed a voluminous chylothorax that persisted despite weeks of prolonged complete bowel rest, total parenteral nutrition, and effective chest tube drainage. Chest tube output diminished immediately following initiation of intravenous somatostatin, allowing restoration of full enteral feeds and removal of chest tubes within 6 days.