Ann Thorac Surg 2000;70:2145-2147
© 2000 The Society of Thoracic Surgeons
Case report
Complete surgical repair of aortic atresia with a normal left ventricle
Sanjiv K. Gandhi, MDa,
Ralph D. Siewers, MDa,
Frank A. Pigula, MDa
a Division of Pediatric Cardiothoracic Surgery, Department of Surgery, Children’s Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, USA
Accepted for publication January 26, 2000.
Address reprint requests to Dr Pigula, Division of Pediatric Cardiothoracic Surgery, Children’s Hospital of Pittsburgh, Room 2820, 3705 Fifth Ave, Pittsburgh, PA 15213-2583
e-mail: pigulaf{at}heart.chp.edu
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Abstract
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Aortic atresia with a normal left ventricle and normal mitral valve is an uncommon congenital lesion. We present two such cases and describe two different approaches of achieving biventricular surgical repair.
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Introduction
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Aortic atresia most commonly presents as a component of the hypoplastic left heart syndrome. Occasionally, the presence of an interventricular communication will permit normal development of the mitral valve and left ventricle in association with this lesion [1]. Though comprising fewer than 5% of cases of aortic atresia, infants with this anomaly present an opportunity for complete biventricular repair. We describe two children with this anatomic complex and outline two separate approaches for operative correction.
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Case reports
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Patient 1
A 3.7-kg female infant born at term was transferred to our institution with cyanosis and progressive acidosis, which was stabilized with the infusion of prostaglandin E1. Echocardiography confirmed the presence of aortic atresia. A large, nonrestrictive, perimembranous outlet ventricular septal defect (VSD) was noted, with equal pressures in the left and right ventricles and excellent biventricular contractility. The left ventricle was normal sized and apex forming. Atrioventricular valves of a satisfactory size were visualized. Mild tricuspid insufficiency and trace pulmonary insufficiency was noted.
At 3 days of age, the patient underwent a standard Norwood arch repair with a 4.0-mm modified right Blalock–Taussig shunt. The postoperative course was uneventful and the patient was discharged on postoperative day 9. Following this initial procedure, the child did well at home. Interim cardiac catheterization revealed satisfactory hemodynamic values but also moderate tricuspid regurgitation. At 6 months of age, she underwent an operation that involved intracardiac baffling of the VSD to the neoaortic valve and placement of a 13-mm valved pulmonary cryopreserved homograft from the right ventricle to the main pulmonary artery with closure of her Blalock–Taussig shunt. Because of her tricuspid insufficiency, a tricuspid annuloplasty was also performed at this time. She was discharged home on postoperative day 5 without complication. At the 2-year follow-up she was thriving. Serial echocardiograms have demonstrated normal biventricular function, an unobstructed right ventricle to pulmonary artery conduit, and no obstruction across the intracardiac baffle or aortic arch.
Patient 2
A 2.4-kg male infant of 36 weeks estimated gestational age was transferred to our institution for evaluation of a cardiac murmur, though he remained acyanotic. Soon after arrival, he became hypotensive and required volume resuscitation and intubation. Prostaglandin E1 infusion was started to maintain ductal patency. Echocardiogram demonstrated a hypoplastic ascending aorta, measuring 2 mm. A nonrestrictive 7-mm subarterial VSD was noted, with equal pressures in the left and right ventricles and excellent biventricular function. There was mild tricuspid, mitral, and pulmonary insufficiency. An interatrial communication with left to right shunting was also visualized. Cardiac catheterization confirmed the echocardiographic findings and also established the presence of a common orifice for both carotid arteries, distal origin of the right subclavian artery, and coarctation of the aorta (Fig 1).
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Fig 1. (A) Angiogram demonstrating the hypoplastic ascending aorta. (B) Lateral view illustrates the nonrestrictive subarterial ventricular septal defect (VSD).
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Surgical intervention was undertaken at 8 days of age. In this child, we elected to perform the complete cardiac repair in one stage (Fig 2). A single arterial cannula was placed through the ductus arteriosus through the pulmonary artery and a single venous cannula in the right atrium. Core cooling was completed to 18°C. While the patient was still on cardiopulmonary bypass, the main pulmonary artery was transected proximal to the bifurcation. The ventricular septum was examined through the pulmonary valve leaflets. The VSD was slightly enlarged anteriorly and then baffled to the pulmonary valve using glutaraldehyde-fixed autologous pericardium. Following a single dose of cold blood cardioplegia, bypass was halted and all cannulas were removed. Using a technique we described recently [2], regional low-flow perfusion through a 3.5-mm Gore-Tex (W.L. Gore and Assoc, Flagstaff, AZ) shunt anastomosed to the right common carotid artery was then initiated. The aorta was incised from the ascending aorta to several millimeters distal to the coarctation and enlarged with a gusset of pulmonary homograft, with a side-to-side aorta to pulmonary artery anastomosis. The neoascending aorta and right atrium were recannulated and bypass reinitiated. A 10-mm valved, cryopreserved pulmonary homograft was used to reestablish right ventricle to pulmonary artery continuity. An intraoperative epicardial echocardiogram revealed good biventricular function and no residual VSD. The sternum was closed primarily. The infant’s postoperative course was uncomplicated. He was extubated on postoperative day 5 and discharged home on postoperative day 12. At 1-year of follow-up, the child is doing well.
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Fig 2. Completed repair. The hypoplastic aorta was enlarged with a gusset of pulmonary homograft and anastomosed to the pulmonary artery. A 10-mm valved pulmonary homograft was used to reconstruct the right ventricular outflow tract. Inset shows the ventricular septal defect baffled to the pulmonary (neoaortic) valve (arrow).
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Comment
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Most newborns with aortic atresia and hypoplastic aortic arch present with a diminutive left ventricle and are palliated with a Norwood operation. Recognizing the presence of a satisfactory left ventricle and mitral valve allows the surgeon to contemplate an operative approach that involves biventricular repair. Cardiac catheterization is helpful in defining the spatial relationships between the VSD, the atrioventricular valves, and the great vessels. Our two patients illustrate disparate surgical strategies for achieving biventricular repair.
As with our first patient, a variety of two-stage repairs have been described [3]. Although this approach has been successful, the first stage necessitates using a shunted pulmonary circulation. This subjects the patient to potential shunt complications, including pulmonary artery distortion and shunt thrombosis, in addition to the hemodynamic consequences of chronic hypoxia and myocardial volume overload. Indeed, in patient 1, a volume-loaded right ventricle may have contributed to annular dilatation and the need for a tricuspid annuloplasty.
Single-stage correction, as in our second patient, can be completed successfully, as manifested here and by others [4, 5]. This method permits early conversion to two ventricle physiology and avoidance of a shunt-dependent circulation. The postoperative fluctuations in pulmonary vascular resistance characteristic of early infancy are hemodynamically much better tolerated by two ventricles. Reconstruction of the aorta with a Norwood operation maintains its growth potential, confining future operative interventions to the pulmonary conduit. Thus, we recommend prompt, single-stage anatomic correction for neonates presenting with aortic atresia and a normal left ventricle.
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References
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Roberts W.C., Perry L.W., Chandra R.S., Myers G.E., Shapiro S.R., Scott L.P. Aortic valve atresia. A new classification based on necropsy study of 73 cases. Am J Cardiol 1976;37:753-756.[Medline]
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Pigula F.A., Siewers R.D., Nemoto E.M. Regional perfusion of the brain during neonatal aortic arch reconstruction. J Thorac Cardiovasc Surg 1999;117:1023-1024.[Free Full Text]
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Rychik J., Murdison K.A., Chin A.J., Norwood W.I. Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis. J Am Coll Cardiol 1991;18:809-816.[Abstract]
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Austin E.H., Jonas R.A., Mayer J.E., Casteñada A.R. Aortic atresia with normal left ventricle. Single-stage repair in the neonate. J Thorac Cardiovasc Surg 1989;97:392-395.[Abstract]
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Francois K., Dollery C., Elliott M.J. Aortic atresia with ventricular septal defect and normal left ventricle: one-stage correction in the neonate. Ann Thorac Surg 1996;58:878-880.[Abstract]
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Abstract
Introduction
