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J Thorac Cardiovasc Surg 1994;108:385-387
© 1994 Mosby, Inc.
LETTERS TO THE EDITOR |
Marie Lannelongue Hospital
Department of Pediatric Cardiac Surgery
Le Plessis Robinson, France
To the Editor:
We report a case of the rare association of truncus arteriosus and complete atrioventricular (AV) canal defect in a neonate. Only 11 cases have been reported in the literature and all were postmortem cases. 
1-7
After an uneventful pregnancy, a 3600 gm boy was born at term with global cardiac and respiratory failure, and anasarca necessitating intubation and mechanical ventilation. Disseminated intravascular coagulopathy related to fetal-maternal incompatibility led to total exsanguinous transfusion and repeated platelet transfusions. Diagnosis was made at birth by echocardiography, which showed type II truncus arteriosus with no truncal valve stenosis or regurgitation, good-sized pulmonary artery branches, normal aortic arch, and a complete AV canal defect with grade I to II AV valve regurgitation and a large inlet plus infundibular ventricular septal defect (Fig. 1).
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An operation was performed when the patient was 18 days of age and weighed 3100 gm. With bicaval cannulation and continuous cardiopulmonary bypass at 22° C, intracardiac analysis revealed (1) Rastelli type C complete AV canal with nearly fused left ventricular papillary muscles and an extremely large ventricular septal defect from the inlet to the truncal valve, (2) type II truncus arteriosus with a large branch from the right coronary artery crossing the anterior wall of the right ventricle and a small left anterior descending coronary artery. The left ventricular papillary muscle was split and the mitral cleft was left open because of a small mitral valve orifice. Ventricular septal defect closure was performed with a Dacron polyester fiber patch through a low infundibulotomy, avoiding the anomalous coronary artery, and through the right atrium. The ostium primum was closed with an autologous pericardial patch. After transection of the aorta and detachment of the pulmonary bifurcation, a Lecompte maneuver was performed. Right ventricle–pulmonary artery connection was established with an 8 mm cryopreserved aortic homograft. Total aortic crossclamp time was 95 minutes. Suprasystemic right ventricular pressures at discontinuation from bypass required reestablishment of cardiopulmonary bypass and placement of a pericardial patch to relieve stenosis of the origin of the right pulmonary artery, resulting in a right ventricular pressure 80% systemic. With sinus rhythm and a regimen of 0.1µg/kg/min isoproterenol (Isuprel) and 0.05µg/kg/min prostaglandin E1, the sternum was left open and the patient was transferred to the intensive care unit. The patient's postoperative course was marked by gradual loss of preoperative edema and hemodynamic stability, allowing closure of the chest on postoperative day 7. Thrombocytopenia of unknown origin necessitated transfusion of several units of platelets. Doppler echocardiography showed a 20 mm Hg gradient at the origin of the right pulmonary artery, mild mitral regurgitation, and no residual shunt. The patient could be extubated on postoperative day 25 and was discharged from the hospital 35 days after the operation. Three months after the operation, the patient's cardiac condition was good but with persistent leukopenia and thrombocytopenia that are under investigation.
The association of truncus arteriosus and complete AV canal septal defect is rare. The literature reports only 11 such cases. 1-7 Surgical correction was performed only once,without success. 5 No details on the operative procedure or anatomy in this case are available. Gumbiner, McManus, and Ratson 6 reported an autopsy subject with asplenia syndrome, truncus arteriosus, and complete AV canal with a small, left-sided ventricle. These authors' review assembled three other published cases but failed to mention the five cases of common AV orifice reported by Bharati and coworkers. 2 As pointed out by Van Praagh andVan Praagh, 1 however, common AV valve seems infrequent in truncus arteriosus. 1 A necropsy study of 66 cases of truncus arteriosus 8 and a clinical report of 167 patients operated on 9 did not mention any cases of AV canal defect.
The posterior limit of the ventricular septal defect in truncus arteriosus depends of the development of the ventriculoinfundibular fold and the posterior limb of the trabecula septomarginalis. 8 In our case, the ventricular septal defect extended instead to the inlet septum below a common AV valve.
The association of a complete AV canal defect can only increase the magnitude of left-to-right shunt, resulting in severe heart failure. Characteristic of our case was the persistence of high pulmonary vascular resistance before operation, necessitating assisted ventilation and prostaglandin infusion to ensure adequate oxygenation.
Another feature of our case was the presence of a large branch of the right coronary artery coursing over the anterior right ventricular wall toward the anterior interventricular groove. This anomaly, already noted by Bharati and associates, 2 can be a source of difficulty in the location of ventriculotomy. The low right ventriculotomy led us to perform a Lecompte maneuver to facilitate homograft connection to the pulmonary artery bifurcation.
Nearly fused left ventricular papillary muscles with potential for inflow obstruction (parachute-type mitral valve) were split and a mitral cleft was left open, resulting in satisfactory postoperative mitral continence. Reconstruction of the right ventricle–pulmonary artery continuity with a valved conduit offers improved results 10 compared with nonvalved conduits, and a cryopreserved homograft is used whenever available. Precarious hemodynamic and respiratory conditions dictated early, semiurgent repair in our patient. These conditions were also responsible for slowly progressive postoperative recovery. Associated cardiac defects have been shown to be an incremental risk factor for death after repair of truncus arteriosus. 5-7 Palliative operations consisting of separate banding of the pulmonary arteries offer no advantage, however, and are responsible for their own complications. To the best of our knowledge, this is the first report of successful repair in a neonate of truncus arteriosus and complete AV canal defect.
References
This article has been cited by other articles:
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  I. Adachi, S. Y. Ho, M. M. Bartelings, K. P. McCarthy, A. Seale, and H. Uemura Common arterial trunk with atrioventricular septal defect: new observations pertinent to repair. Ann. Thorac. Surg., May 1, 2009; 87(5): 1495 - 1499. [Abstract] [Full Text] [PDF]
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S. Conte, T. Jensen, J. R. Jacobsen, F. S. Joyce, P. Lauridsen, and G. Pettersson One-Stage Repair of Truncus Arteriosus, CAVC, and TAPVC Ann. Thorac. Surg., June 1, 1997; 63(6): 1781 - 1783. [Abstract] [Full Text]
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 F. Lacour-Gayet, A. Serraf, T. Komiya, M. Sousa-Uva, J. Bruniaux, A. Touchot, D. Roux, P. Neuville, and C. Planche TRUNCUS ARTERIOSUS REPAIR: INFLUENCE OF TECHNIQUES OF RIGHT VENTRICULAR OUTFLOW TRACT RECONSTRUCTION J. Thorac. Cardiovasc. Surg., April 1, 1996; 111(4): 849 - 856. [Abstract] [Full Text]
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