Ann Thorac Surg 2001;72:2124-2125
© 2001 The Society of Thoracic Surgeons
Case report
Combined primary repair of tetralogy of Fallot and aortic root replacement
H. Tarik Kiziltan, MD*a,
Semra Topcu, MDb,
Nazan Ozbarlas, MDc,
Fazilet Kayaselcuk, MDd
a Department of Cardiovascular Surgery, Dr. Turgut Noyan Clinic and Research Center, Baskent University, Adana, Turkey
b Department of Cardiology, Dr. Turgut Noyan Clinic and Research Center, Baskent University, Adana, Turkey
c Department of Pediatric Cardiology, Dr. Turgut Noyan Clinic and Research Center, Baskent University, Adana, Turkey
d Department of Pathology, Dr. Turgut Noyan Clinic and Research Center, Baskent University, Adana, Turkey
Accepted for publication March 14, 2001.
* Address reprint requests to Dr Kiziltan, Department of Cardiovascular Surgery, Dr. Turgut Noyan Clinic and Research Center, Baskent University, Adana 01250, Turkey
e-mail: tkiziltan{at}turk.net
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Abstract
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Tetralogy of Fallot associated with ascending aortic aneurysm and aortic valve regurgitation is unusual. This combination necessitates a change in operative strategy during complete tetralogy repair. We present a 43-year-old woman who successfully underwent composite graft replacement of the ascending aorta and aortic valve during primary complete tetralogy repair. Histologic examination of the aorta revealed medial degeneration.
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Introduction
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A subgroup of patients with tetralogy of Fallot (TOF) has dilation of the aorta and aortic valve regurgitation [1–3]. We report our experience treating a patient with TOF, aortic aneurysm, and aortic regurgitation. The patient underwent complete repair of TOF combined with composite graft replacement of the aortic valve and ascending aorta.
A 43-year-old woman with TOF who had marked limitation of physical activity (New York Heart Association [NYHA] class III), frequent cyanotic spells, clubbing of the fingers, and poor stamina was admitted to our service. She weighed 46 kg and her hemoglobin was 17.4 g/dL. Preoperative echocardiography and cardiac catheterization confirmed the diagnosis of TOF. Diagnostic workup also defined an aortic aneurysm (5.7 cm in diameter; Fig 1), a right-sided aortic arch, moderate aortic valve, and severe tricuspid valve (TV) insufficiency. The aortic root measured 52 mm. Coronary anatomy was normal. The bilateral pulmonary arteries were satisfactory in size. Repair of aortic aneurysm along with repair of tetralogy was planned to prevent complications related to aortic aneurysm, such as aortic dissection, intramural hematoma, and spontaneous rupture [4].
Surgical technique included median sternotomy, cardiopulmonary bypass (CPB) through the femoral artery and bicaval cannulation, left ventricular venting, and antegrade and retrograde coronary sinus cardioplegia. A composite graft (Hall 25, Medtronic, Inc, Minneapolis, MN) was implanted in the aortic root. Coronary buttons were anastomosed on the graft. Circulatory arrest (CA) and retrograde cerebral perfusion (300 to 600 mL/min) were utilized for the distal anastomosis. The TV was found to be dysplastic and apically displaced. There was a nonrestrictive, malaligned ventricular septal defect (VSD). A Dacron patch (C. R. Bard, Haverhill, MA) was utilized to close the VSD transventricularly. Transannular right ventricular outflow tract reconstruction was performed using an autologous pericardial patch. No benefit was anticipated by doing a tricuspid plasty due to the dysplastic nature of the TV. The patient was weaned from CPB in sinus rhythm. Total CPB time, cross-clamp time, and CA time (at 15°C) were 354, 284, and 23 minutes, respectively. The right-to-left ventricular pressure ratio was found to be 0.61 (pRV = 71 mm Hg, pLV = 117 mm Hg). Postoperative cardiac catheterization was performed on postoperative day 5 and showed good functions of the prosthetic aortic valve and right ventricle and satisfactory orientation of the composite graft (Fig 2). There was mild to moderate TV insufficiency. Right ventricular pressure was 45/5 mm Hg. The patient was discharged from the hospital on postoperative day 13. Histologic examination of the aortic specimen showed fragmentation in the elastic lamina (Fig 3). Seven months postoperatively she is doing well with a physical capacity of NYHA class I. She is on digitalis and Coumadin (Du Pont Pharmaceuticals, Wilmington, DE).
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Fig 3. Aortic cross section showing intimal thickening, subintimal edema, and fragmentation in the elastic fibers. (Elastic Van Gieson; x100.)
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Comment
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Dilation of the aorta and aortic valve regurgitation have been described in patients with TOF [1–3, 5]. As the aorta receives most of the blood ejected from both ventricles, the aortic root and ascending aorta can become dilated due to hemodynamic stress [1]. Also, previous aortopulmonary shunts [1], congenital abnormalities of the aortic valve [2], and acquired infective endocarditis [3] are reported causes of aortic regurgitation and aortic dilation in TOF.
Histologic findings in our patient showed medial degeneration of the aortic wall [6]. Cystic medial necrosis, a severe form of medial degeneration, was reported in 3 of 5 patients who underwent resection of an aneurysmal ascending aorta during aortic valve operation after uncomplicated complete repair of TOF or pulmonary atresia and VSD [5]. Medial degeneration may be secondary to the stress on the aorta. The effects of chronic hypoxia and other factors in the process of medial degeneration associated with aortic dilation/regurgitation in TOF are yet to be defined.
Our case, as well as the experiences of others [5], demonstrates that medial degeneration with aortic dilation and aortic valve regurgitation can occur in patients with TOF. Aortic root replacement may be necessary during a primary complete tetralogy repair in patients with associated dilation of the aortic root and aortic valve regurgitation.
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References
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Ergin M.A., Spielvogel D., Apaydin A., et al. Surgical treatment of the dilated ascending aorta. Ann Thorac Surg 1999;67:1834-1839.[Abstract/Free Full Text]
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Dodds G.A., Warnes C.A., Danielson G.K. Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or Fallot. J Thorac Cardiovasc Surg 1997;113:736-741.[Abstract/Free Full Text]
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Downing W.D., Kouchoukos N.K. Ascending aortic aneurysm. In: Edmunds L.H., ed. Cardiac surgery in the adult, 1st ed. New York: McGraw-Hill, 1997:1165-1195.
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Abstract
Introduction
