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Sixth Aortic Root Replacement, Aortopulmonary Fistula And Pulmonary Hemorrhage In A Patient With Truncus Arteriosus
Raghav A. Murthy, Howaida El-Said, John Lamberti.
Rady Children's Hospital, San Diego, CA, USA.
OBJECTIVE: This case discusses a complex adult congenital problem. Its describes the comprehensive, multidisciplinary management a patient with 5 previous aortic and pulmonary root replacements, with an underlying diagnosis of truncus arteriosus, presenting with hemoptysis secondary to an acquired aorto-pulmonary 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 work-up that was negative, imaging studies including CT scan and cardiac catheterization revealed an aortic root pseudoaneurysm, contained rupture of the aorta, severe right pulmonary artery stenosis, fistula between the aorta and the main pulmonary artery and prosthetic valve dysfunction. Brain imaging revealed an incidental berry aneurysm. Pre-operatively, a balloon was placed in the catheterization lab 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 7th time redo-sternotomy was performed. The previously placed gortex membrane allowed for contained rupture. Under TEE guidance bypass was initiated and the pulmonary artery occluded to allow for cooling. The aortic and pulmonary root was resected, the right pulmonary artery reconstructed from hilum to the central pulmonary artery, the aortic and pulmonary roots were replaced with homografts and the coronaries re-implanted utilizing the Cabrol technique. The circulatory arrest, aortic cross clamp and cardiopulmonary bypass time were 30, 260 and 421 minutes.
RESULTS: The patient made a fabulous recovery and was discharged home after a total hospitalization of 51 days
LEGEND: The image shows pre-operative CT scan and cardiac catheterization showing aortopulmonary fistula, severe RPA stenosis, RCA pseudoaneurysm and balloon occlusion of the main pulmonary artery. The last image shows the post-operative reconstruction of the aorta.
CONCLUSIONS: Complex adult congenital cases require a multi-disciplinary and innovative approach to manage.
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