Ann Thorac Surg 2006;82:e31-e32
© 2006 The Society of Thoracic Surgeons
Case Reports
Giant Aortic Aneurysm 18 Years After Repair of Double-Outlet Right Ventricle With Pulmonary Stenosis
Julie Cleuziou, MDa,*,
Norbert Mayr, MDa,
Christian Schreiber, MDa,
Jürgen Hörer, MDa,
Stefanie Sassen, MDb,
Harald Kaemmerer, MD, PhDc,
Rüdiger Lange, MD, PhDa
a Clinic of Cardiovascular Surgery, German Heart Centre Munich, Germany
c Clinic of Pediatric Cardiology and Congenital Heart Disease, German Heart Centre Munich, Germany
b Department of Pathology, Technical University Munich, Munich, Germany
Accepted for publication June 23, 2006.
* Address correspondence to Dr Cleuziou, Clinic of Cardiovascular Surgery, German Heart Centre Munich, Lazarettstrasse 36, D-80636 Munich, Germany. (Email: cleuziou{at}dhm.mhn.de).
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Abstract
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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.
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Introduction
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Aortic aneurysm and aortic dissection are well recognized late sequelae after correction of tetralogy of Fallot (TOF) [1, 2]. 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.
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Fig 1. Volume rendering technique of a computed tomography scan of the chest depicts the aneurysm of the ascending aorta.
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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).
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Fig 2. Inner half of the aortic wall shows loss and disruption of the red-stained elastic fibers and adjacent smooth muscle fibers (arrow) and deposits of pale-stained intercellular substance (double arrow), diagnostic for medionecrosis (Verhoeff's van Gieson stain, original magnification x150).
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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.
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Comment
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Aortic root dilatation is a well-known complication late after repair of TOF and occurs in about 15% of patients [3]. 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 [1, 2]. 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 [4]. 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 [1]. In rare cases, aortic root dilatation may proceed to dissection and rupture [5, 6].
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 [7, 8]. 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.
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References
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- Dodds GA, Warnes CA, Danielson GK. Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot J Thorac Cardiovasc Surg 1997;113:736-741.[Abstract/Free Full Text]
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- Niwa K, Siu SC, Webb GD, Gatzoulis MA. Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot Circulation 2002;106:1374-1378.[Abstract/Free Full Text]
- Tan JL, Davlouros PA, McCarthy KP, Gatzoulis MA, Ho SY. Intrinsic histological abnormalities of the aortic root and ascending aorta in tetralogy of Fallot Circulation 2005;112:961-968.[Abstract/Free Full Text]
- Kim WH, Seo JW, Kim SJ, Song J, Lee J, Na CY. Aortic dissection late after repair of tetralogy of Fallot Int J Cardiol 2005;101:515-516.[Medline]
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- Wacker 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;95:776-779.[Medline]
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Abstract
Introduction
