Biomedical subjects
Publications and source records attributed to Athar Qureshi.
Aortic insufficiency is not a part of the hypoplastic left heart syndrome. This report describes a rare case of congenital aortic insufficiency from a detached leaflet in a patient with hypoplastic left heart syndrome and 45,X/46XY mosaicism. The patient was subsequently treated with the modified Norwood procedure along with suture closure of aortic valve.
BACKGROUND: To determine outcomes for the arterial switch operation individualized according to the underlying anatomy and clinical status. METHODS: A retrospective review of a consecutive series of infants less than 90 days of age who underwent the arterial switch operation at a single institution. RESULTS: From July 1993-April 2001, 117 infants underwent an arterial switch operation before 90 days of age. Seventy-five patients (64%) had transposition of the great arteries with intact ventricular septum with the aim of operation before 14 days of age; however, 8 of these patients had delayed presentation (range 15-46 days). Thirty-five patients (30%) had transposition with a ventricular septal defect (30 patients) or double outlet right ventricle (5 patients) and normal arch anatomy and were repaired within the first 90 days of life depending on the severity of heart failure at a median of 12 days of age (range 3-83 days). Seven patients (6%) had associated aortic coarctation (5 patients) or interrupted aortic arch (2 patients). One patient died during hospitalization (0.85% hospital mortality) and one patient died from noncardiac causes during a median follow-up of 35 months (1.7% total mortality). Four patients required intervention during follow-up (3.4%) for new aortic coarctation (2 patients), supravalvar pulmonic stenosis (1 patient), or right hemi-diaphragm paralysis (1 patient). CONCLUSIONS: Individualized timing for the arterial switch operation within the first ninety days of life produces excellent survival rates for all types of transposition physiology with the expectation of a satisfactory course during follow-up.
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.
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.