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Background: We manage patients with pulmonary atresia, ventricular septal defect, major systemic-to-pulmonary collateral arteries, and diminutive central pulmonary arteries with a staged approach. The first procedure is a central end-to-side aortopulmonary shunt (Melbourne shunt) intended to cause growth and development of the central pulmonary arteries. We subsequently measured central pulmonary artery growth after Melbourne shunt., Methods: Forty consecutive patients were followed after Melbourne shunt. The maximum pulmonary artery diameter was measured at the time of surgery and at subsequent catheterizations or surgery., Results: Median pulmonary artery size at surgery was 2 mm. The median pulmonary artery diameter was 5.5 mm at first assessment (median, 6.35 months) and 7 mm at most recent assessment (19.7 months). Mean modified Nakata index increased from 27 mm(2)/m(2) at surgery to 138 mm(2)/m(2) at first assessment, and 176 mm(2)/m(2) at final assessment. There was one acute shunt failure from anastomotic stenosis. Thirteen patients (32.5%) required 21 percutaneous interventions. There were 4 deaths during a median follow-up of 68 months. At the time of complete repair (n = 25) all patients required pulmonary artery augmentation, and 8 are in various stages of palliation. The remaining patients are considered not reparable owing to irreversible pulmonary hypertension (n = 4) or have required fenestration of ventricular septal defect after complete repair (n = 2)., Conclusions: Melbourne shunt promotes modest growth of central pulmonary arteries leading to complete repair in the majority of patients. There is considerable need for further interventions in these patients to augment the size of the pulmonary arteries.

Objective: To assess the results of a staged surgical approach for tetralogy of Fallot with pulmonary atresia, hypoplastic or absent pulmonary arteries, and major aortopulmonary collateral arteries., Methods: We retrospectively reviewed a consecutive series of these patients from a single institution., Results: From July 1993 to April 2001, 46 consecutive patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collateral arteries were treated with staged surgical repair. The operative sequence usually began with a central aortopulmonary shunt followed by unifocalization of aortopulmonary collateral arteries depending on the source and distribution of pulmonary blood flow. Twenty-eight patients (61%) subsequently underwent complete repair with ventricular septal defect closure and right ventricle to pulmonary artery connection. Those patients who underwent complete repair had a median of 3 total operations (range 1-6). The ratio of the mean pulmonary artery pressure to the mean systemic blood pressure at the time of complete repair was 0.36 (range 0.19-0.58). Two of the 28 repaired patients (7.1%) required subsequent fenestration of the ventricular septal defect closure due to later development of supersystemic right ventricular pressure and right ventricular failure. Eighteen patients (39%) have undergone 1 or more staging operations and are considered good candidates for eventual complete repair. There were no hospital deaths. There was 1 late death (2.2%; 95% CI 0.4-11.3%) in a patient born prematurely who developed severe bronchopulmonary dysplasia precluding complete repair., Conclusions: For tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries, a staged surgical approach yields low overall mortality and acceptable hemodynamics after complete repair.

We developed a rat model of pulmonary arteriovenous malformations after cavopulmonary anastomosis. We sought to determine whether this model reproduces the angiographic and histologic features seen in the human condition. Eight Sprague-Dawley rats underwent a right superior cavopulmonary anastomosis with the use of microsurgical techniques. Between 2 and 13 mo, pulmonary angiography was performed, the animals were euthanized, and the lungs were removed. Microscopic sections of the lung were stained with an endothelial-specific antibody (von Willebrand factor). Microvessel density was determined by counting vessels staining positively for von Willebrand factor, and the shunted and nonshunted (control) lungs were compared for each animal. Pulmonary angiography revealed time-dependent development of arteriovenous malformations. Microvessel density demonstrated a time-dependent increase in the shunted lung compared with the control lung (simple linear regression of the ratio of the microvessel density of the shunted lung divided by the microvessel density of the control lung on time; R(2) = 0.79, P = 0.003). This animal model reproduces the same angiographic and microscopic features of pulmonary arteriovenous malformations that develop in humans after cavopulmonary anastomosis. This appears to be a valid model that may be used to further study etiologic mechanisms for this phenomenon.

Background: Apical ventricular septal defects (VSDs) are difficult to visualize through a transatrial approach, while the use of a left ventriculotomy may result in progressive ventricular dysfunction. Transcatheter closure has not been reliable, especially in small infants. Apical right ventriculotomy provides good exposure, preserves left ventricular function, and allows secure closure of apical VSDs.Methods: From November 1994 through April 2002, nine children, median age 8 months (range, 74 days to 2.5 years) underwent 10 operations for VSD closure via apical right ventriculotomy (one patient with a residual defect underwent successful VSD closure via a second apical right ventriculotomy). Two patients were status post pulmonary artery banding and two patients had previous unsuccessful attempts at closure via a transatrial approach.Results: There was no hospital mortality; there were 2 late deaths (78% survival; 95% confidence interval [CI], 45% to 94%), 3 months and 4 years postoperatively. Postoperative echocardiography demonstrated no residual VSDs in 3 and insignificant residual VSDs in 4 of the survivors. All survivors currently exhibit normal biventricular function during a median follow-up of 25 months (range, 11 to 104 months).Conclusions: Apical right ventriculotomy provides excellent exposure allowing safe and effective closure of apical VSDs. The observed late morbidity and mortality reflects the complexity that often exists in these cases due to additional irreparable lesions. [Copyright &y& Elsevier]

Objective: To determine the outcomes for a program that utilizes the double switch operation as the primary approach for congenitally corrected transposition. Methods: The records of 46 consecutive patients from a single institution who had undergone a double switch operation from October 1993 to March 2002 were reviewed. The records of 24 patients who were evaluated during the same period and felt not to be candidates for the double switch operation or who are awaiting double switch after pulmonary artery banding were also reviewed. Results: The median age at operation was 28 months (range 2 months to 16.3 years). Associated defects included ventricular septal defect 40, pulmonic stenosis 13 and pulmonary atresia 16. Twenty-six patients underwent an arterial switch operation combined with a Senning procedure while 20 patients underwent combined Rastelli and Senning procedures. Before the double switch, 12 patients had required pulmonary artery banding and 21 patients had systemic to pulmonary artery shunts. The median duration of stay in the intensive care unit was 3.5 days (range 2–60 days) and the median duration of total hospital stay was 8 days (range 5–60 days). There were no hospital deaths; one patient died 5 months after discharge due to an arrhythmogenic cardiac arrest during a median follow-up of 24 months [long-term survival 98% (95% CI 89–100%)]. Conclusions: The double switch operation may be performed with excellent hospital and long-term survival. The theoretical advantages of this procedure which enables the morphologic left ventricle and mitral valve to support a systemic pressure load must be established by careful follow-up of these patients. [Copyright &y& Elsevier]