Technical Challenges: Pitfalls and Disasters

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Raghav A. Murthy, Howaida El-Said, John J. Lamberti. Rady Children's Hospital, San Diego, CA USA.
Objective: This case illustrates a complex adult congenital problem. It describes the comprehensive, multidisciplinary management of a patient with 5 previous aortic and pulmonary root replacements, with an underlying diagnosis of truncus arteriosus, presenting with hemoptysis secondary to an acquired aortopulmonary fistula, right coronary button pseudoaneurysm, severe right pulmonary artery stenosis, prosthetic valve dysfunction, and contained rupture of his aorta.
Methods: After an extensive infectious disease workup that was negative, imaging studies including a computed tomography scan and cardiac catheterization revealed an aortic root pseudoaneurysm, contained rupture of the aorta, severe right pulmonary artery stenosis, a fistula between the aorta and the main pulmonary artery, and prosthetic valve dysfunction. Brain imaging revealed an incidental berry aneurysm. Preoperatively, in the catheterization laboratory, a balloon was placed via the right femoral vein into the pulmonary artery across the mechanical valve for intraoperative occlusion of the fistula. After axillary artery and right femoral vein cannulation, a seventh redo sternotomy was performed. The previously placed Gore-tex membrane had contained the rupture. Under the guidance of transesophageal echocardiography, the bypass was initiated, and the pulmonary artery was occluded to allow for cooling. The aortic and pulmonary roots were resected; the right pulmonary artery was reconstructed from the hilum to the central pulmonary artery; the aortic and pulmonary roots were replaced with homografts; and the coronary arteries were re-implanted using the Cabrol technique (Fig. C1-1). The circulatory arrest, aortic cross-clamp, and cardiopulmonary bypass times were 30, 260, and 421 minutes, respectively.
Results: The patient made a fabulous recovery and was discharged home after a total hospitalization of 51 days.
Conclusions: Complex adult congenital cases require a multidisciplinary, innovative approach.

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