Ann Thorac Surg 2004;78:e69-e71
© 2004 The Society of Thoracic Surgeons
Case report
Complete Atrioventricular Canal and Tetralogy of Fallot With Pulmonary Atresia
Toru Okamura, MDa,*,
Yuzo Nagase, MDa,
Yasutoshi Matsumoto, MDb,
In-sam Park, MDc,
Fujio Mitsui, MDa,
Masao Shibairi, MDa
a Department of Cardiovascular Surgery, Chiba, Japan
b Department of Pediatrics, Matsudo City Hospital, Chiba, Japan
c Department of Pediatrics, The Sakakibara Heart Institute, Tokyo, Japan
Accepted for publication December 22, 2003.
* Address reprint requests to Dr Okamura, Department of Cardiovascular Surgery, Matsudo City Hospital, 4005 Kamihongo, Matsudo, Chiba 271-8511, Japan
tokamura{at}rr.iij4u.or.jp
 |
Abstract
|
|---|
Our patient was diagnosed with complete atrioventricular canal and Tetralogy of Fallot with pulmonary atresia at the age of 1 month. Then he underwent right and left Blalock-Taussig shunts at the ages of 2 months and 5 years, respectively. His cyanosis had increased at 20 years of age. Cardiac catheterization showed occlusion of the left Blalock-Taussig shunt and absence of the left pulmonary artery. Lung perfusion scintigram showed late phase perfusion in the left lung. Chest computed tomographic scan demonstrated the left pulmonary artery. We describe the operative technique of total correction.
|
Introduction
|
|---|
Complete atrioventricular septal defect with tetralogy of Fallot (AVSD-TOF) is relatively uncommon. Furthermore, AVSD-TOF with pulmonary atresia is rare. We present a case of the repair of AVSD-TOF with pulmonary atresia. The technical aspects and operative considerations are discussed.
The patient was a 20-year-old man with slight cyanosis who did not have Down's syndrome or heterotaxy syndrome. He was diagnosed as having AVSD-TOF with pulmonary atresia at the age of 1 month. He did not have any major aortopulmonary collateral arteries. Original right Blalock-Taussig shunt was performed at 2 months of age. Stenosis of the central pulmonary artery (PA) developed, and a modified left Blalock-Taussig shunt was performed at 5 years of age. Cyanosis had increased at 10 years of age. Cardiac catheterization showed stenosis of both shunts. Catheter intervention was performed for stenosis, which maintained continuity between the right and left PAs. We offered surgical treatment to the family, but it was refused, and he was treated medically. Recently, he began to experience shortness of breath and requested further investigation. Preoperative blood tests showed that his hemoglobin was 18.9 g/dL and his hematocrit was 56.4%. Hepatic and renal functions were within normal limits. Chest roentgenogram showed mild cardiac enlargement and a decrease of left pulmonary blood flow. Cardiac catheterization showed a right PA pressure of 26/16 mm Hg, pulmonary resistance of 2.2 U/m2, a mean left pulmonary venous wedge pressure of 11 mm Hg, and a SaO2 of 85.9%. The left and right ventriculogram demonstrated a left ventricular end-diastolic volume of 178% of the normal value with an ejection fraction of 65%, and a right ventricular end-diastolic volume of 217% of the normal value with an ejection fraction of 51%. Pulmonary artery angiogram (Fig 1A) showed a defect of the left PA and the Nakata index for right PA was 432. There was common atrioventricular valve (CAVV) regurgitation, grade 1 of Sellars' classification. The modified Blalock-Taussig shunt was not contrasted at the left subclavian artery angiogram (Fig 1B). A chest computed tomographic scan demonstrated the left PA (Fig 2A). A lung perfusion scintigram showed blood perfusion in the late phase (Fig 2B). We decided to performed total correction of the AVSD-TOF associated with pulmonary atresia and the LPA interruption.
View larger version (93K):
[in this window]
[in a new window]
|
Fig 1. (A) Pulmonary angiogram (PAG) shows absence of the left pulmonary artery (lt-PA), and the Nakata index of 432 for only the right PA. (B) The left subclavian artery (SCA) angiogram shows occlusion of the left modified Blalock-Taussig shunt.
|
|
View larger version (86K):
[in this window]
[in a new window]
|
Fig 2. (A) Chest computed tomographic scan demonstrated the left pulmonary artery that is indicated with a white arrow. (B) Lung perfusion scintigram showed blood perfusion in the left lung at the late phase.
|
|
Complete repair was performed under cardiopulmonary bypass, moderate hypothermia, and intermittent cold blood cardioplegia. The original right Blalock-Taussig shunt was closed and was divided at the start of bypass. Cardioplegia was administered though the aortic root after application of the cross clamp. The CAVV component was inspected through a right atriotomy. We diagnosed it as a Rastelli type C because the superior leaflet was free floating. Cold saline solution was infused through the CAVV to distend the ventricles and the CAVV apparatus. The anatomy of the CAVV was clearly demonstrated in the functional position, allowing identification of the ideal site for coaptation of the superior and inferior bridging leaflets. The coaptation site was marked with a suture. A right ventriculotomy was performed. A comma-shaped patch (a collagen-coated Dacron tube [InterGard, InterVascular, LA Ciotat, France]) was tailored to the dimensions of the defect with redundancy anteriorly. Eight interrupted sutures were buttressed with e-polytetrafluoroethylene pledgets on the right ventricular side of the septum. The patch was sewn using a continuous suture around the aortic root. The cleft in the left side of the CAVV was approximated with five interrupted sutures. The atrial septal defect was closed using a separate patch (a collagen-coated Dacron tube [InterGard]) so that the atrioventricular valves were sandwiched between the atrial and ventricular patches. The suture line was carried through the previous marked coaptation point on each bridging leaflet. On reaching the annulus of the atrioventricular valve posteriorly, the suture line was carried toward the left ventricle for a few bites to avoid injuring the atrioventricular node. The coronary sinus was left on the right side. The LPA was dissected and opened longitudinally. The length of the LPA interruption was almost 5 mm. The LPA was 10 mm in diameter. The anterior patch angioplasty of the LPA was performed using an autopericardial patch. A composite graft consisted of a 23-mm Carpentier-Edwards stented bovine pericardial aortic bioprosthesis (Edwards Lifesciences LLC, Irvine, CA) incorporated in a 28-mm collagen-coated Dacron tube (Intergard) was required from the right ventricle (RV) to the PA. The patient was easily weaned from cardiopulmonary bypass. There was no heart failure. Inotoropic support was required for a few days. The patient stayed in the intensive care unit for 2 days. His postoperative course was uneventful. He was discharged from the hospital 2 weeks postoperatively. Postoperative cardiac catheterization showed a systolic RV pressure of 50 mm Hg. The right ventriculogram revealed good flow to the left PA (Fig 3).
View larger version (181K):
[in this window]
[in a new window]
|
Fig 3. Right ventriculogram (RVG) shows good flow to the left (lt) pulmonary artery (PA). (rt = right.)
|
|
|
Comment
|
|---|
Atrioventricular septal defect with tetralogy of Fallot is a relatively uncommon lesion. We could only find a few cases of AVSD-TOF with pulmonary atresia in a series reported by Vargas and colleagues [1] and Delius and colleagues [2]. Atrioventricular septal defect with tetralogy of Fallot with pulmonary atresia associated with interruption of the LPA is very uncommon.
Postoperative competence of the atrioventricular valve is among the important factors contributing to successful management of patients with a complete atrioventricular septal defect. The presence of concomitant tetralogy of Fallot, with its attendant right ventricular outflow tract obstruction, can potentially worsen right atrioventricular valve regurgitation. The resulting volume load or right ventricular hypertension can potentially worsen right valve regurgitation. Furthermore, significant regurgitation of the left atrioventricular valve can elevate PA pressure and increase pulmonary insufficiency if a transannular patch is present. These factors usually result in a greater risk of mortality and morbidity with repair of combined atrioventricular septal defect and tetralogy of Fallot than the risk of repair for either lesion alone. For a similar reason, pulmonary valve incompetence influences postoperative cardiac function [3–6].
Our case had interruption of the LPA. The LPA was demonstrated on the chest computed tomographic scan. Nevertheless, if the LPA cannot be found at operation, RV-PA reconstruction must be carried out on only the right PA. With RV-PA reconstruction of only the right PA, the PA and RV pressure increase. Regurgitation of the right side of the atrioventricular valve increases with a rise of RV pressure. Selection of a surgical procedure that does not produce pulmonary regurgitation is necessary. On the other hand, a large RV incision is needed for all procedures, and the ventricular septal defect can be closed through the right atrium and the right ventricle. This RV incision can improve exposure of the subaortic region for accurate closure of the ventricular septal defect and preserves an unrestricted left ventricular outflow tract. Therefore, we selected the two-patch technique that used a Dacron graft (Intergard) as a patch and RV-PA reconstruction that used a composite graft with a bioprosthetic valve.
In our patient, we obtained an improvement in pulmonary blood flow, and subaortic stenosis has not occurred postoperatively. Use of a valved conduit with a bioprosthetic valve for RV-PA reconstruction and the two-patch technique for intracardiac repair provided a good functional outcome.
|
References
|
|---|
- Vargas FJ, Coto EO, Mayer JE, Jonas RA, Castaneda AR. Complete atrioventricular canal and tetralogy of Fallot: surgical consideration. Ann Thorac Surg. 1986;42:258–263[Abstract]
- Delius RE, Kumar RV, Elliott MJ, Stark J, de Leval MR. Atrioventricular septal defect with tetralogy of Fallot: a 15-year experience. Eur J Cardiothorac Surg. 1997;12:171–176[Abstract]
- Guo-wei H, Mee RBB. Complete atrioventricular canal associated with tetralogy of Fallot or double-outlet right ventricle and right ventricular outflow tract obstruction: a report of successful surgical treatment. Ann Thorac Surg. 1986;41:612–615[Abstract]
- Ilbawi M, Cua C, Deleon S, et al. Repair of complete atrioventricular septal defect with tetralogy of Fallot. Ann Thorac Surg. 1990;50:407–412[Abstract]
- O'Blenes SB, Ross DB, Nanton MA, Murphy DA. Atrioventricular septal defect with tetralogy of Fallot: result of surgical correction. Ann Thorac Surg. 1998;66:2078–2084[Abstract/Free Full Text]
- Najm HK, Van Arsdell GS, Watzka S, Hornberger L, Coles JG, Williams WG. Primary repair is superior to initial palliation in children with atrioventricular septal defect and tetralogy of Fallot. J Thorac Cardiovasc Surg. 1998;116:905–913[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
J. H. Shuhaiber, S. Y. Ho, M. Rigby, and B. Sethia
Current options and outcomes for the management of atrioventricular septal defect
Eur. J. Cardiothorac. Surg.,
May 1, 2009;
35(5):
891 - 900.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
