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- W2015499342 abstract "Substantial long-term morbidity after a successful operation in complex congenital heart defects is a matter of concern. We present a patient with a giant ascending aortic aneurysm 18 years after repair of a double-outlet right ventricle with pulmonary stenosis. Our report emphasizes the need for ongoing follow-up into adulthood. Substantial long-term morbidity after a successful operation in complex congenital heart defects is a matter of concern. We present a patient with a giant ascending aortic aneurysm 18 years after repair of a double-outlet right ventricle with pulmonary stenosis. Our report emphasizes the need for ongoing follow-up into adulthood. Aortic aneurysm and aortic dissection are well recognized late sequelae after correction of tetralogy of Fallot (TOF) [1Dodds G.A. Warnes C.A. Danielson G.K. Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.J Thorac Cardiovasc Surg. 1997; 113: 736-741Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar, 2Warnes C.A. Child J.S. Aortic root dilatation after repair of tetralogy of Fallot Pathology from the past?.Circulation. 2002; 106: 1310-1311Crossref PubMed Scopus (22) Google Scholar]. We report a rare finding in a patient with a giant ascending aortic aneurysm 18 years after repair of a double-outlet right ventricle (DORV) with pulmonary stenosis. Substantial long-term morbidity after a successful operation in complex congenital heart defects, such as in our described case of double outlet right ventricle with pulmonary stenosis, is a matter of concern. Our case report emphasizes the need for ongoing follow-up into adulthood. A 27-year-old man presented with cardiac failure and new onset of atrial fibrillation. Eighteen years before admission, he had undergone repair of DORV and pulmonary stenosis with transventricular closure of the ventricular septal defect and placement of a right ventricular outflow tract Gore-Tex patch (W. L. Gore & Associates, Inc, Flagstaff, Ariz). Four years before corrective surgery, he had undergone placement of a left Blalock-Taussig shunt and transannular patch enlargement of the right ventricular outflow tract. He had not been under cardiac review for many years. On admission, echocardiography revealed a massive aneurysm of the ascending aorta and moderate aortic regurgitation. The patient underwent successful cardioversion in laryngeal mask anesthesia, but respiratory failure developed and endotracheal intubation was required because of a beginning pneumonia. A chest computed tomograpic scan confirmed the diagnosis of a giant aneurysm of the ascending aorta measuring 85 mm at its widest part (Fig 1). No evidence of dissection was detected. The patient was in a poor general condition, and a microbiologic work-up confirmed a fulminant Haemophilus influenzae pneumonia. The inflammation markers normalized slowly under antibiotic treatment; however, the patient could not be weaned from the ventilator. Fourteen days after admission, the patient required urgent cardiac surgery owing to progressive aortic insufficiency. After repeat median sternotomy and cannulation of the ascending aorta just below the innominate artery and cannulation of the right atrium, cardiopulmonary bypass was initiated, and the patient was cooled to a body temperature of 28°C. The aorta was clamped and opened longitudinally. Cold crystalloid cardioplegia was given directly into the coronary ostiae. The aneurysm was resected as well as the aortic valve. A 31-mm composite valved conduit (St. Jude Medical, Inc, St. Paul, Minn) was sewn into the aortic base using interrupted plegeted sutures. The coronary ostiae were reimplanted into the conduit, which reached up to the cannulation site. Histologic examination of the resected aortic wall revealed medionecrosis with elastic fragmentation (Fig 2). The patient’s postoperative course was turbulent, with low cardiac output requiring insertion of an intraaortic balloon pump and renal failure requiring dialysis for many weeks. Respiratory failure required prolonged ventilatory support and tracheostomy. The patient eventually recovered and was discharged in a stable condition. Aortic root dilatation is a well-known complication late after repair of TOF and occurs in about 15% of patients [3Niwa K. Siu S.C. Webb G.D. Gatzoulis M.A. Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot.Circulation. 2002; 106: 1374-1378Crossref PubMed Scopus (221) Google Scholar]. Furthermore, patients who have undergone initial palliative surgery, such as a systemic-to-pulmonary artery shunt, are also at increased risk of aortic root dilatation with consequent aortic regurgitation [1Dodds G.A. Warnes C.A. Danielson G.K. Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.J Thorac Cardiovasc Surg. 1997; 113: 736-741Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar, 2Warnes C.A. Child J.S. Aortic root dilatation after repair of tetralogy of Fallot Pathology from the past?.Circulation. 2002; 106: 1310-1311Crossref PubMed Scopus (22) Google Scholar]. The underlying mechanism is attributed to the increased flow through the aorta before corrective surgery, especially if a shunt was placed previously. Preexisting histologic abnormalities in the media layer of the aortic root and ascending aorta of patients with TOF may also contribute to aortic root dilatation [4Tan J.L. Davlouros P.A. McCarthy K.P. Gatzoulis M.A. Ho S.Y. Intrinsic histological abnormalities of the aortic root and ascending aorta in tetralogy of Fallot.Circulation. 2005; 112: 961-968Crossref PubMed Scopus (142) Google Scholar]. The histologic changes with loss of smooth muscle cells, mucoid degeneration, and fragmentation of the elastic fibers in the media resemble those observed in patients with bicuspid aortic valves or Marfan syndrome. Important underlying mechanisms may be apoptosis, particularly in patients with bicuspid aortic valves or cellular abnormalities in patients with conotruncal anomalies [1Dodds G.A. Warnes C.A. Danielson G.K. Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot.J Thorac Cardiovasc Surg. 1997; 113: 736-741Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar]. In rare cases, aortic root dilatation may proceed to dissection and rupture [5Kim W.H. Seo J.W. Kim S.J. Song J. Lee J. Na C.Y. Aortic dissection late after repair of tetralogy of Fallot.Int J Cardiol. 2005; 101: 515-516Abstract Full Text Full Text PDF PubMed Scopus (79) Google Scholar, 6Rathi V.K. Doyle M. Williams R.B. Yamrozik J. Shannon R.P. Biederman R.W. Massive aortic aneurysm and dissection in repaired tetralogy of Fallot; diagnosis by cardiovascular magnetic resonance imaging.Int J Cardiol. 2005; 101: 169-170Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar]. We have reported a rare case of aortic root dilatation late after repair of DORV and pulmonary stenosis. This morphologic substrate shows a similar pathophysiology as TOF with pulmonary stenosis. Hence, it may be speculated that the mechanism of development of an aortic aneurysm might be the same for both cardiac defects. Our patient had systemic-to-pulmonary artery shunt placement in early childhood and did not have corrective surgery until he was 9 years old. Thus, increased aortic flow persisted for many years before corrective surgery, presumably leading to progressive dilatation of the aortic root. Because the patient failed to undergo cardiologic follow-up examinations for many years, he eventually presented in a deplorable condition. Poor compliance with follow-up is, unfortunately, a frequent problem in adults with congenital cardiac disease. This dilemma puts patients at a considerable risk and ultimately leads to a high rate of emergent admissions and consequent urgent surgical procedures [7Kaemmerer H. Fratz S. Bauer U. et al.Emergency hospital admissions and three-year survival of adults with and without cardiovascular surgery for congenital cardiac disease.J Thorac Cardiovasc Surg. 2003; 126: 1048-1052Abstract Full Text Full Text PDF Scopus (63) Google Scholar, 8Wacker A. Kaemmerer H. Hollweck R. et al.Outcome of operated and unoperated adults with congenital cardiac disease lost to follow-up for more than five years.Am J Cardiol. 2005; 95: 776-779Abstract Full Text Full Text PDF PubMed Scopus (73) Google Scholar]. In this patient, successful repair of the aortic root could be achieved at the price of a prolonged postoperative course due to the poor preoperative condition. In addition to patients with TOF, those with DORV and pulmonary stenosis should remain under close medical supervision, even late after corective surgery. Echocardiographic assessment of the aortic root should be performed routinely." @default.
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- W2015499342 title "Giant Aortic Aneurysm 18 Years After Repair of Double-Outlet Right Ventricle With Pulmonary Stenosis" @default.
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