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Ann Thorac Surg 2001;72:2129-2130
© 2001 The Society of Thoracic Surgeons
k, MDac, MDa
cz, MDaa Department of Pediatric Cardiac Surgery, Polish-American Children’s Hospital, Collegium Medicum Jagiellonian University, Cracow, Poland
Accepted for publication December 13, 2000.
* Address reprint requests to Dr Malec, Department of Pediatric Cardiac Surgery, Polish-American Children’s Hospital, Collegium Medicum, Jagiellonian University, 265 Wielicka St, 30-663 Cracow, Poland
e-mail: mimalec{at}cyf-kr.edu.pl
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A 3,100-g male, term infant was delivered vaginally with Apgar score 10. The pregnancy was complicated by viral infection at 2 months. When the child was 2 days old, a physical examination showed cyanosis, a systolic murmur at the left sternal border, tachycardia, and palpable peripheral pulses. Metabolic acidosis was present. On the sixth day, the patient was admitted to our hospital with a preliminary diagnosis of HLHS. A continuous infusion of alprostadil (prostaglandin E1; 0.03 µg · kg-1 · min-1) was administered. Echocardiography revealed right atrial enlargement, a small atrial septal defect, atretic mitral and aortic valve, rudimentary left ventricle, diminutive ascending aorta, and decreased cardiac function. Systemic arterial blood gas values were pH 7.31; partial pressure of oxygen, 41 mm Hg; partial pressure of carbon dioxide, 42 mm Hg. When the boy was 11 days old, a classic Norwood procedure was performed. Through a midline stemotomy, the thymus gland was excised. The head vessels were exposed and looped with tourniquets in preparation for circulatory arrest. Cardiopulmonary bypass was established by cannulation of the MPA and the right atrial appendage. The branches of the right and left pulmonary arteries were immediately occluded and systemic cooling was begun. Cold crystalloid cardioplegia solution (15 mL/kg) was administered through the pulmonary artery cannulation site after clamping the descending thoracic aorta. When the patient was cooled to 18°C the cannulas were removed and the septum primum was excised working through the atrial appendage purse string. The MPA was distally transected, and the bifurcation was patched with homograft material. A cryopreserved pulmonary allograft was trimmed to fashion a patch that would serve to augment the aorta and allow an anastomosis to the distal end of the MPA. The circulatory arrest time was 42 minutes. During rewarming we commenced the procedure of grafting a 4-mm polytetrafluoroethylene-modified Blalock-Taussig shunt. Ventricular fibrillation occurred, which is extremely rare at this stage of the operation. After defibrillation, the slow extrasinus rhythm was restored and the anterior wall of the right ventricle showed low contractility. Differences in myocardium perfusion were distinctly visible, with a vertical border between the two differently perfused parts transversing the anterior wall of the right ventricle. The left part was akinetic and poorly perfused. Suspecting an air embolus in the LCA, we decided to increase the pump-generated flow. When the shunt was grafted and the vascular clip was released, the difference in perfusion subsided and normal sinus rhythm was restored. When another attempt at shunt clipping was made, the same anomalies of perfusion and deteriorated myocardial function were observed. We suspected abnormalities of the coronary arteries, the perfusion of which depends on the patency of the systemic-to-pulmonary shunt. The shunt diameter was decreased because of a partial closure. In the course of inspecting the pulmonary artery the LCA was found to originate from the posterior aspect of the midright pulmonary artery (Fig 1). The vessel extended forward behind the aorta and the pulmonary artery, toward the anterior interventricular groove. Continuing the operation, the left and right pulmonary arteries were looped with tourniquets, distally with respect to the origin of LCA and the site of shunt grafting. Cardioplegic solution was administered simultaneously to the "neoaorta" and pulmonary artery. The LCA orifice was excised and transferred to the neoaorta to the site where the native ascending aorta formed its wall. The aortic cross-clamp time was 14 minutes. In the course of rewarming, the RPA was repaired with autologous pericardium. Postoperatively, homogenous perfusion was observed, along with normal contractility and sinus rhythm. Five hours after the completion of the procedure peripheral perfusion deteriorated, oliguria developed, and the mean arterial blood pressure decreased. Despite infusions of dopamine and sodium nitroprusside, low cardiac output symptoms increased and extracorporeal membrane oxygenation (ECMO) support was initiated. The aorta and right atrium were cannulated in typical places. The setting was about 100 mL · kg-1 · min-1 and the diameter of the systemic-to-pulmonary shunt was temporarily decreased. Inotropic support was markedly diminished. We targeted a mean arterial pressure of 40 to 50 mm Hg. In the course of ECMO, diuresis normalized and the electrocardiographic recording did not show any ischemia. After 48 hours of ECMO, the support was gradually decreased. Echocardiography revealed satisfactory contractility of the right ventricle. The child was weaned, but low cardiac output was still present and 1 day later the boy died due to sudden circulatory collapse. The autopsy confirmed the final diagnosis and the patency of the transplanted LCA and did not reveal any additional anomalies.
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