Ann Thorac Surg 2001;71:355-356
© 2001 The Society of Thoracic Surgeons
Case report
Pulmonary atresia, ventricular septal defect, and coronary-pulmonary artery fistula
Yukihiro Kaneko, MDa,b,
Hideo Okabe, MDa,b,
Nobuhiro Nagata, MDa,b,
Jotaro Kobayashi, MDa,b,
Arata Murakami, MDa,b,
Shinichi Takamoto, MDa,b
a Department of Thoracic and Cardiovascular Surgery, Kanagawa Children’s Medical Center, Yokohama, Japan
b Department of Cardiothoracic Surgery, University of Tokyo, Tokyo, Japan
Accepted for publication March 13, 2000.
Address reprint requests to Dr Kaneko, Department of Cardiothoracic Surgery, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-0033, Japan
e-mail: kaneko-tho{at}h.u-tokyo.ac.jp
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Abstract
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We report 2 patients with pulmonary atresia, ventricular septal defect, and coronary-pulmonary fistula. The fistula originated from the left anterior descending artery in 1 patient, and from the right coronary artery in the other. Both patients survived staged correction in which right ventricular outflow was reconstructed with autologous fistula tissue. One patient with pulmonary hypertension suddenly died 9 months after surgery. Twenty-four patients with pulmonary atresia, ventricular septal defect, and coronary-pulmonary fistula have been reported previously.
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Introduction
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Pulmonary blood supply in patients with pulmonary atresia (PA) and ventricular septal defect (VSD) is usually through a patent arterial duct or major aortopulmonary collateral arteries (MAPCAs). Rarely, pulmonary blood supply is through a fistula from the coronary artery.
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Case reports
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Case 1
A 1-month-old girl with PA, VSD, MAPCAs to the left lung, and anophthalmia was admitted to a hospital for heart failure and cyanosis. Surgical ligation and catheter embolization of the MAPCAs did not improve heart failure. She was transferred to Kanagawa Children’s Medical Center at 1 year and 6 months of age. Angiography confirmed VSD, PA, left anterior descending coronary artery-to-pulmonary artery fistula, vestigial left pulmonary artery, and bilateral superior caval veins (Fig 1). Right pulmonary artery configuration suggested pulmonary vascular obstructive change.
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Fig 1. Configuration of coronary and pulmonary artery in relation to the aorta (Ao) in case 1. A fistula arising from the left anterior descending artery (LAD) gave rise to the main pulmonary artery. The left pulmonary artery (LPA) was thread-like. The pulmonary artery is colored in gray. (RPA = right pulmonary artery; RCA = right coronary artery.)
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At operation, we placed a right subclavian-to-right pulmonary artery prosthetic shunt, divided the coronary-pulmonary fistula, and fixed its pulmonary arterial stump onto the right ventricular surface so that the right ventricular outflow tract could be reconstructed by using autologous fistula tissue at a later operation. Four months later, we reconstructed the right ventricular outflow tract with a gusset, and occluded the prosthetic shunt. At 2 years and 5 months of age, the VSD was closed. Postoperative catheter study revealed a right-to-left ventricular pressure ratio of 0.68, and a pressure gradient of 4 mm Hg across the subpulmonary outflow. At 3 years and 2 months of age, she suddenly died during a holiday trip. Necropsy was declined by the parents.
Case 2
A 6-month-old girl was referred to the University of Tokyo for cyanosis and failure to thrive. Angiography disclosed PA, VSD, and right coronary artery-to-pulmonary artery fistula. There were two communicating MAPCAs to the right lung, and two noncommunicating MAPCAs to the left lung (Fig 2). Right pulmonary arterial pressure was systemic. At the first palliation, the MAPCAs to the right lung were ligated through a right thoracotomy. Seven months later, a second palliation was performed through a median sternotomy with the use of cardiopulmonary bypass: the coronary-pulmonary fistula was divided, the pulmonary arterial stump of the fistula was fixed onto the right ventricle, the left pulmonary artery was unifocalized by anastomosing MAPCAs to the left pulmonary artery, a diminutive right pulmonary artery was enlarged with a patch, and a systemic-pulmonary shunt was placed. At correction 3 months later, the shunt was divided, VSD was closed, and right ventricular outflow tract was reconstructed with a gusset. Right-to-left ventricular pressure ratio and pressure gradient across the subpulmonary outflow were 0.40 and 16 mm Hg, respectively. One year after correction, she enjoys normal development.
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Fig 2. Configuration of coronary and pulmonary artery in case 2. A fistula was formed between the right coronary artery (RCA) and the main pulmonary artery. A left-sided MAPCA originated from the aberrant left subclavian artery (SCA) branching from the right aortic arch. The pulmonary artery and MAPCAs are colored in dark and light gray, respectively. (Ao = aorta.)
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Comment
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In previous literature, 24 patients with PA and VSD were reported as having main pulmonary blood supply through coronary-pulmonary fistulae [1–6, and references therein]. The fistula originated from the left coronary artery in 17 patients, from the right in 4, and from the single coronary artery in 3 patients. Description indicative of MAPCAs was found in 14 of 24 (58%) of the reported cases. In 22 cases, the fistula was connected to the central pulmonary artery, whereas in the other 2 patients, the fistula gave rise to MAPCAs [7]. The majority of the patients had increased pulmonary flow. Although coronary-pulmonary fistula theoretically steals coronary blood, symptomatic coronary insufficiency has not been reported even after palliative surgery to decrease pulmonary blood flow with the fistula open.
Diagnosis of coronary-pulmonary fistula was made by echocardiography in 1 patient, by angiography in 16, during operation in 5, and by necropsy in 2 patients. Surgery was conducted in 21 patients: exploration in 3, palliation in 4, staged correction in 3, and primary correction in the remaining 11 patients. Two patients died early postoperatively.
In case 1, late death may be attributable to pulmonary hypertension. If surgery normalizing right pulmonary blood flow had been performed in infancy, postrepair pulmonary pressure would have been lower. We reconstructed pulmonary outflow with autologous fistula tissue hoping that it may grow. In retrospect, use of a valved pulmonary homograft might have decreased right ventricular volume load.
In case 2, we considered ligation of the right-sided MAPCAs as the safest first-stage palliation. Although this procedure might have worsened coronary steal, we anticipated that coronary insufficiency would not occur because of a stenotic right pulmonary artery.
Two surgical implications are suggested. First, early surgical intervention is recommended because pulmonary vascular obstructive change may occur as early as in infancy. Second, the literature indicates feasibility of surgical palliation to reduce pulmonary blood flow with the fistula open. Coronary insufficiency from the steal phenomenon has not been reported, even after palliation that may worsen coronary steal.
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References
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Metras D.R., Kreitmann B., Tatou E., Riberi A., Wernert F. Tetralogy of Fallot with pulmonary atresia, coronary artery-pulmonary artery fistula, and origin of left pulmonary artery from descending aorta: total correction in infancy. J Thorac Cardiovasc Surg 1993;105:186-188.[Medline]
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Krongrad E., Ritter D.G., Hawe A., Kincaid O.W., McGoon D.C. Pulmonary atresia or severe stenosis and coronary artery-to-pulmonary artery fistula. Circulation 1972;46:1005-1012.[Abstract/Free Full Text]
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Rastelli G.C., Ongley P.A., Davis G.D., Kirklin J.W. Surgical repair for pulmonary valve atresia with coronary-pulmonary artery fistula: report of a case. Mayo Clin Proc 1965;40:521-527.[Medline]
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Dark J.H., Pollock J.C.S. Coronary artery-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia. Eur Heart J 1985;6:714-716.[Abstract/Free Full Text]
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Pahl E., Fong L., Anderson R.H., Park S.C., Zuberbuhler J.R. Fistulous communication between a solitary coronary artery and the pulmonary arteries as the primary source of pulmonary blood supply in tetralogy of Fallot with pulmonary valve atresia. Am J Cardiol 1989;63:140-143.[Medline]
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Suzuki K., Matsui M., Nakamura Y., Kurosawa H., Ogawa K., Hoshino K. A case of coronary artery-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries. J Jpn Assn Thorac Surg 1992;40:2252-2257.
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Anderson R.H. Pulmonary atresia with ventricular septal defect and coronary arttery fistula: a late presentation. Br Heart J 1988;60:264.
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Abstract
Introduction
