Ann Thorac Surg 2009;88:1000-1001. doi:10.1016/j.athoracsur.2009.02.029
© 2009 The Society of Thoracic Surgeons
Case Reports
Aortic Aneurysm Formation Five Decades After Tetralogy of Fallot Repair
George Dimitrakakis, MDa,
Ulrich Von Oppell, FCSa,
David Bosanquet, MRCSa,
Dirk Wilson, FRCPb,
Heyman Luckraz, FRCSa,*
a Cardiothoracic Unit, University Hospital of Wales, Cardiff, United Kingdom
b Adult Congenital Heart Disease Unit, University Hospital of Wales, Cardiff, United Kingdom
Accepted for publication February 9, 2009.
* Address correspondence to Dr Luckraz, Cardiothoracic Unit, Block C5, University Hospital of Wales, Cardiff, CF14 4XW, United Kingdom (Email: heymanluckraz{at}aol.com).
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Abstract
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Surgical repair of tetralogy of Fallot is recognized as one of the most successful palliative cardiac surgical procedures. We report a patient in whom cystic medial necrosis developed 50 years after tetralogy of Fallot repair that caused a pathologic ascending aortic aneurysm requiring operative repair. Thus, long-term clinical follow-up along with echocardiography, when indicated, is mandatory in patients undergoing tetralogy of Fallot repair.
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Introduction
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Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, and surgical repair of TOF is one of the most successful operations in the treatment of congenital heart diseases. We report a patient who underwent a successful two-stage repair of TOF in 1950 and 1961 by Lord Brock and who recently presented with aortic root and ascending aorta dilatation. The surgical technique and the postoperative management are described.
A 69-year-old woman with a history of TOF repair was referred for investigation of New York Heart Association (NYHA) class III dyspnea. Comorbidities included a history of hypercholesteremia and arthritis. TOF was diagnosed when she was 12 years old, and she underwent a Blalock-Taussig shunt through a left thoracotomy by Lord Brock in 1950. She remained well until 1961, when breathlessness, effort intolerance, syncope, cyanotic attacks, and polycythemia developed. She then underwent an uncomplicated definitive repair of TOF through a median sternotomy. The patient had a normal life after this reoperation, which included one normal pregnancy.
During the last 2 years, increasing breathlessness developed. An examination noted the complete disappearance of the clubbing that she had had before the definitive TOF repair. Echocardiography showed severe aortic valve regurgitation, mild mitral valve regurgitation, mild tricuspid valve regurgitation, sclerosis of the pulmonary valve, and good left ventricular ejection fraction. A computed tomography coronary angiogram showed prosthetic material in the upper ventricular septum consistent with the previous ventricular septal defect (VSD) repair, a mildly dilated left ventricle, a dilated aortic root (51 mm) and ascending thoracic aorta (52 mm), and no significant stenosis in her coronary arteries (Fig 1). A magnetic resonance image scan confirmed severe aortic valve regurgitation (50% of stroke volume), dilated aortic root and ascending thoracic aorta (Fig 1), a left-sided aortic arch, and an occluded left subclavian artery just beyond its origin, with filling of the distal left subclavian from the collateral circulation. The left vertebral arose directly from the aortic arch. The pulmonary artery was normally sized (21 mm). There was no pulmonary valve regurgitation and mild pulmonary valve stenosis. We were unable to obtain copies of the previous operation notes from Guy's Hospital, London.
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Fig 1. Magnetic resonance image of the thorax shows aneurysmal dilatation of the ascending aorta (A) and occlusion of the left subclavian artery just after its origin (B).
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She underwent repeat sternotomy, with aortic root and ascending aorta replacement using a composite valve-graft consisting of a 30-mm Hemashield graft (Boston Scientific, Natick, MA) with a 27-mm Mosaic porcine biologic prosthetic valve (Medtronic Inc, Minneapolis, MN), with reimplantation of coronary ostia, and closure of a small residual VSD.
Operative details included mild systemic hypothermic cardiopulmonary bypass (CPB time of 152 minutes) and aortic cross-clamping (117 minutes) using combined antegrade and retrograde cold blood cardioplegia. A preoperative transesophageal echocardiogram showed residual pulmonary artery stenosis with a 21-mm gradient, and we determined this did not warrant surgical correction. The histopathology of the aorta was consistent with focal, cystic medial degeneration.
The postoperative period was uneventful apart from an episode of paroxysmal atrial fibrillation that required readmission and cardioversion 4 weeks after discharge. At follow-up, she remains well 2 years after this latest procedure.
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Comment
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Lord Alfred Blalock performed the first palliative systemic to pulmonary artery shunt for cyanotic congenital heart disease in 1945. Subsequently, the first definitive surgical repair of TOF was done in 1954 by C. Walter Lillehei, thus making TOF a correctable condition. That patient went on to live an active and normal life as a professional musician until 1986, three decades after the operation.
The patient presented in this current report underwent both operations within 5 and 7 years, respectively, of the first description of each procedure. Lord Brock, a pioneer of cardiothoracic surgery, did both operations. This patient has now survived 58 years after the initial palliative Blalock-Taussig shunt and 47 years after the definitive TOF correction. In 1986 Lillehei and colleagues [1] reported 106 patients who underwent a corrective TOF operation between 1954 and 1960 and described a healthy 75-year-old grandmother with 26 to 32 years of survival after TOF correction. The overall survival rate at 30 years was 77%. A follow-up of 658 patients by Nollert and colleagues [2] in 1997 documented a 30-year survival rate of 89%.
Aortic root dilatation has become increasingly recognized as a long-term complication after repair of TOF. In 1988 Zahka and colleagues [3] analyzed long-term valvular function after total repair of TOF in 59 patients. Aortic regurgitation was demonstrated in 21% of patients and correlated with aortic root size. In 1997 Dodds and coworkers [4] looked specifically at aortic valve replacement after corrective intervention for pulmonary atresia, VSD, and TOF. At a median of 13.5 years after the corrective procedure, 16 patients required aortic valve replacements because of dilated aortic sinuses.
Subsequent studies have found aortic regurgitation to be associated with aortic root dilatation in 12% to 20% of patients after TOF repair [5, 6]. Trojnarska and colleagues [7] reported that aortic regurgitation appeared more prevalent in the elderly and was unrelated to patient's age at time of operation or to the time elapsed since the repair. The reason why aortic root dilatation occurs is not well understood, although aortic root dilatation has been shown to develop in patients with increased aortic stiffness, with reduced strain and distensibility [5]. Histologic abnormalities in the media layer of the aortic root of these patients may also contribute to aortic root dilatation [8]. Senzakai and coworkers [9] recently reported that all patients demonstrated abnormal arterial hemodynamics after TOF repair. These patients have higher impedance and pulse wave velocity and lower total peripheral arterial compliance, which suggests an increase in central and peripheral arterial wall stiffness after TOF repair. The increase in aortic wall stiffness is closely associated with the increase in aortic root diameter [9].
This case report highlights the long-term success of the early pioneering procedures developed for treating congenital heart disease as well as the importance of long-term follow-up of patients after successful TOF repairs. Significant aortic root dilatation and aortic regurgitation can occur as late as 45 years after a corrective operation for TOF.
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References
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- Lillehei CW, Varco RL, Cohen M, et al. The first open heart corrections of tetralogy of Fallot. A 26–31 year follow-up of 106 patients. Ann Surgery 1986;204:490-502.[Medline]
- Nollert G, Fischlein T, Bouterwek S, Bohmer C, Klinner W, Reichart B. Long-term survival in patients with repair of tetralogy of Fallot: 36-year follow-up of 490 survivors of the first year after surgical repair J Am Coll Cardiol 1997;30:1374-1383.[Abstract]
- Zahka KG, Horneffer PJ, Rowe SA, et al. Long-term valvular function after total repair of tetralogy of Fallot. Relation to ventricular arrhythmias. Circulation 1988;78:III14-III19.[Medline]
- Dodds 3rd GA, Warnes CA, Danielson G. 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]
- Chong WY, Wong WH, Chiu CS, Cheung YF. Aortic root dilation and aortic elastic properties in children after repair of tetralogy of Fallot Am J Cardiol 2006;97:905-909.[Medline]
- Niwa K. Aortic root dilatation in tetralogy of Fallot long-term after repair – histology of the aorta in tetralogy of Fallot: evidence of intrinsic aortopathy Int J Cardioly 2005;103:117-119.
- Trojnarska O, Siwi
ska A, Mularek-Kubzdela T, Szyszka A, Cie
liski A. Aortic regurgitation in adults after surgical repair of tetralogy of Fallot Kardiol Pol 2003;59:484-491.[Medline] - Tan JL, Davlouros PA, McCarthy KP, Gatzoulis MA, Ho SY. Intrinsic histological abnormalities of aortic root and ascending aorta in tetralogy of Fallot: evidence of causative mechanism for aortic dilatation and aortopathy Circulation 2005;112:961-968.[Abstract/Free Full Text]
- Senzaki H, Iwamoto Y, Ishido H, et al. Arterial hemodynamics in patients after repair of tetralogy of Fallot: influence on left ventricular afterload and aortic dilation Heart 2008;94:70-74.[Abstract/Free Full Text]
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Abstract
Introduction
