Ann Thorac Surg 2008;86:295-297. doi:10.1016/j.athoracsur.2007.12.080
© 2008 The Society of Thoracic Surgeons
Case Reports
Severe Pulmonary Valve Incompetence Late After Debanding: Repair by Bicuspidization
Engin Usta, MDa,
Renate Kaulitz, MD, PhDb,
Shahriar Salehi-Gilani, MDa,
Michael Hofbeck, MD, PhDa,
Gerhard Ziemer, MD, PhDa,*
a Department of Thoracic, Cardiac and Vascular Surgery, Children's Hospital, University of Tübingen, Tübingen, Germany
b Department of Pediatric Cardiology, Children's Hospital, University of Tübingen, Tübingen, Germany
Accepted for publication December 3, 2007.
* Address correspondence to Dr Ziemer, Department of Thoracic-, Cardiac- and Vascular Surgery, Eberhard-Karls-University Tübingen, Hoppe-Seyler-Str. 3, Tübingen, 72076, Germany (Email: gerhard.ziemer{at}med.uni-tuebingen.de).
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Abstract
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We report a simple valve repair for severe pulmonary incompetence in a 25-year-old patient. The patient had been operated on twice before for ventricular septal defect and coarctation of the aorta. The first operation consisted of pulmonary artery banding and coarctectomy and end-to-end anastomosis at 4 months, followed by debanding and transinfundibular ventricular septal defect closure at 6 years of age. Massive pulmonary incompetence was due to destruction of one valve cusp with the right ventricular outflow tract and pulmonary artery dilated secondarily. Repair consisted of pulmonary valve bicuspidization and right ventricular remodelling.
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Introduction
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Pulmonary incompetence (PI) can be without clinical impact for a long time. Later, symptoms of right ventricular (RV) dilatation and heart failure may occur. Although the most frequent cause of PI is status posttransannular RV outflow tract (RVOT)-patch enlargement for congenital lesions, symptoms developed in the patient presented in this report after previous surgical palliation in the presence of a normal pulmonary valve and RVOT to start with.
A 25-year old man presented with progressive exercise intolerance and signs of congestive cardiac failure. He had undergone an operation for patent ductus arteriosus, aortic coarctation, and pulmonary artery banding at age 4 months. After normal growth and development, he had undergone a transinfundibar ventricular septal defect (VSD) closure and PA debanding at age 6 years.
The patient had an uneventful course for 18 years, when progressive dyspnea developed on exertion. Diagnostic studies consisted of echocardiography, angiography, cardiac magnetic resonance imaging, and computed tomography scan. The result was a massive pulmonary regurgitation (PI III to IV°) with dilatation of the right ventricle and enormous dilatation of the pulmonary trunk (diameter = 55 mm) and the RVOT (Fig 1).
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Fig 1. (A) Preoperative angiography shows the pulmonary annulus diameter is 35 mm and the pulmonary trunk is 55 mm. The right ventricular outflow tract (RVOT) is also massively enlarged. (Right ventricle, 1; RVOT, 2; pulmonary annulus, 3; pulmonary trunk, 4.) (B) The 1-month follow-up angiography shows the obvious reduction of the monstrous pulmonary trunk, which now has a 33-mm diameter. The diameter of the pulmonary annulus now is about 22 mm. The bar (arrow) indicates 10 mm. The contour of the RVOT and pulmonary trunk is marked with a gray line.
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After median resternotomy and exposure of the anatomy, the RV dilatation and the dilatated pulmonary trunk were evident. On normothermic cardiopulmonary bypass with the heart beating, we excised the transannular GoreTex patch (W. L. Gore & Associates, Flagstaff, AZ) out of the RVOT. The incision was then continued into the pulmonary trunk, up to the bifurcation. To our surprise, we found 2 intact large pulmonary semilunar valve cusps (dorsolateral and left), with the ventral cusp missing. This was different from isolated congenital absence of a single pulmonary valve cusp [1].
During the last operation, the pulmonary annulus had been incised to remove the stenosis caused by the prior PA banding. The already damaged anterior cusp had also been divided. The incised RVOT and the pulmonary trunk had been reconstructed with a GoreTex patch. The divided ventral cusp had been sutured into the patch, remaining nonfunctional thereafter.
During the current procedure, we had planned to implant a pulmonary valved homograft, but we now favored repair. Using the bicuspidization technique, we sutured the left-to-anterior commissure to the right-to-anterior commissure, leaving only a minimal distance for eventual further diameter plication. In the testing that followed, the bicuspidized pulmonary valve revealed just a trivial regurgitation. After that, we resected the ventral wall of the pulmonary trunk distally to a remaining diameter of 22 mm, which equalled the reconstructed valve diameter.
Before discontinuation of cardiopulmonary bypass, the distal RVOT was reconstructed with a small GoreTex patch, and in the apical part of the RVOT, the RV was remodelled by a volume-reducing direct suture.
The patient had an uneventful recovery, and echocardiographic and angiographic evaluation featured only trivial PI postoperatively. At the 3- and 9-month follow-up visits, the bicuspidized pulmonary valve maintained a satisfactory function aside from the trivial PI (Figs 2 and 3).
The preoperative and postoperative pulmonary vessel diameters, hemodynamic variables, and the pulmonary valve function are reported in Table 1.
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Fig 2. (A) A preoperative computed tomography (CT) scan shows the diameter of the pulmonary trunk is 55 mm. (B) The 3-month postoperative CT scan shows the diameter of the reduced pulmonary trunk maintained constant at 33 mm. Each picture contains a 50-mm scale. (Aorta ascendens, 1; pulmonary trunk, 2; left main pulmonary artery, 3.)
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Fig 3. Diameters of the pulmonary annulus and trunk at the 9-month follow-up are visualized by cardiac magnetic resonance imaging. (A) Sagittal slice presents the right ventricular outflow tract (RVOT), pulmonary annulus, and pulmonary trunk. Line 1 indicates the diameter of the pulmonary annulus is 21.98 mm. (B) An axial slice features the pulmonary annulus and pulmonary trunk. Line 1 indicates the diameter of the pulmonary annulus is 22.52 mm. Line 2 marks the diameter of the pulmonary trunk, just before the bifurcation. A 50-mm scale is given in each panel. (Ao = aorta ascendens; LPA = left pulmonary artery; PA = pulmonary artery.)
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In the current 9-month follow-up, the diameters of the pulmonary annulus and trunk, and also the PI, maintained largely constant. Just like in the 3-month follow-up, an unobstructed flow still exists over the pulmonary valve, indicating not more than a tolerable mild pulmonary stenosis (Table 1).
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Comment
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Even significant PI may be clinically absent for a long period of time before symptoms of RV dilatation and heart failure occur. Most patients with PI have an underlying congenital heart lesion, such as tetralogy of Fallot [2, 3], that was corrected in the past. It is quite common that patients are left with PI after surgical repair of obstructive lesions of the RVOT [2]. Although many patients with significant PI after relief of RVOT obstruction will tolerate this long term, a certain percentage will not. In those cases, the surgical repair consists of replacement of the incompetent pulmonary valve either with a homograft or with a bioprothesis. In a few patients, such as in isolated congenital absence of a single pulmonary valve cusp, the reconstruction of the incompetent pulmonary valve can be performed [4].
In our patient, we favored the reconstruction of the pulmonary valve by bicuspidization instead of replacement with a homograft. Perhaps the reason why this operation was possible was growth of the 2 functional cusps during the 19-year interval since the outflow tract patch procedure. This contrasts with the prior reports of this technique that were mainly applied in congenital forms with a single absent pulmonary valve cusp [1, 5].
The uneventful recovery, satisfactory pulmonary function, and unobstructed flow assessed by echocardiography and angiography postoperatively and in the 3- and 9-month follow-up visits justifies our regimen. The bicuspidization technique could be a satisfying alternative in selective patients presenting with 2 sufficient cusps and a dilated pulmonary valve annulus.
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References
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- Kadri MA, Lazzara RR, McLellan BA, Starr A. Repair of congenital pulmonary incompetence by bicuspidization of the pulmonary valve Ann Thorac Surg 1997;63:1482-1483.[Abstract/Free Full Text]
- Kanter KR, Budde JM, Parks WJ, et al. One hundred pulmonary valve replacements in children after relief of right ventricular outflow tract obstruction Ann Thorac Surg 2002;73:1801-1806.[Abstract/Free Full Text]
- Tanabe Y, Takahashi M, Kuwano H, et al. Long-term fate of isolated congenital absent pulmonary valve Am Heart J 1992;124:526-529.[Medline]
- Sayger P, Lewis M, Arcilla R, Ilbawi M. Isolated congenital absence of a single pulmonary valve cusp Pediatr Cardiol 2000;21:487-489.[Medline]
- Westaby S, Katsumata T. Congenital absence of a single pulmonary valve cusp Ann Thorac Surg 1997;64:849-851.[Abstract/Free Full Text]
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Abstract
Introduction
