Ann Thorac Surg 1998;65:1464-1465
© 1998 The Society of Thoracic Surgeons
Case Reports
Division of the Main Pulmonary Artery: Suboptimal Palliation for the Patient With a Univentricular Heart
Jeffrey A. Wong, MDa,
Robert H. Beekman, III, MDa
a Division of Cardiology, Children’s Hospital Medical Center, Cincinnati, Ohio, USA
Accepted for publication December 10, 1997.
Address reprint requests to Dr Beekman, Division of Cardiology, Children’s Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229
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Abstract
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We describe a patient in whom a thrombus formed in the distal main pulmonary artery stump after a bidirectional Glenn procedure with division of the main pulmonary artery and in whom a restrictive inlet ventricular septal defect also developed. These findings emphasize the importance of maintaining antegrade flow through the pulmonary trunk and providing dual sources of ventricular outflow with a Damus-Kaye-Stansel anastomosis in patients with a univentricular heart without pulmonary stenosis.
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Introduction
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The management of the main pulmonary artery (MPA) in the initial palliation of an infant with a univentricular heart without pulmonary stenosis is controversial. Options include banding or dividing the MPA or performing a Damus-Kaye-Stansel (DKS) MPA–aortic anastomosis. Pulmonary artery banding is generally avoided because it may promote the development of subaortic stenosis. We present the case of a child in whom the MPA was divided, but who had subsequent development of a large thrombus in the distal MPA stump and subaortic stenosis because of a restrictive inlet ventricular septal defect (VSD). This case provides a further rationale for avoiding MPA division and favoring instead the DKS procedure in infants with a univentricular heart.
The patient presented at birth with situs inversus, levocardia, an unbalanced complete atrioventricular canal defect, a hypoplastic left ventricle, and an interrupted inferior vena cava with azygous continuation to the right superior vena cava. She initially underwent pulmonary artery banding at 3 months of age, and at 25 months of age she underwent a bidirectional Glenn (Kawashima) procedure with division of the MPA. A preoperative cardiac catheterization demonstrated a nonrestrictive inlet VSD. Postoperatively the patient did well and echocardiography demonstrated an unobstructed Glenn anastomosis, no evidence of pulmonary artery thrombus, and a moderately restrictive inlet VSD (peak Doppler gradient = 40 mm Hg).
Three months later the patient underwent repeat cardiac catheterization, which demonstrated a large clot in the distal MPA stump extending into the left pulmonary artery (Fig 1). In addition, the inlet VSD was restrictive, with a 40 mm Hg peak systolic gradient between the right and left ventricles. An echocardiogram confirmed the presence of the thrombus in the stump of the MPA distal to the surgical division and extending into the left pulmonary artery, occluding its lumen by 50% (Fig 2). The patient was subsequently started on a regimen of warfarin. Three months later the clot had dissolved and the patient underwent placement of a right ventricle-to-aorta homograft.
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Fig 1. Pulmonary arteriogram in the anteroposterior view demonstrating a large clot at the junction of the main and left pulmonary arteries.
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Fig 2. Two-dimensional echocardiogram from a high parasternal short-axis view demonstrating a thrombus in the main pulmonary artery at the junction with the left pulmonary artery (arrow).
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Comment
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This case demonstrates two disadvantages of MPA division in a patient with a univentricular heart: distal MPA stump thrombosis and development of ventricular outflow obstruction. In patients with cavopulmonary shunts (Glenn or Fontan procedures), the development of thrombi in the right atrium, in the venae cavae, and at the shunt anastomotic site has been well described [1–4]. Additionally, the occurrence of thrombi in the proximal MPA stump (ie, on the ventricular side) after distal ligation of the main pulmonary artery has recently been described [5]. We now describe a case of a large thrombus in the distal MPA stump (which extended into the left pulmonary artery) in a patient who had undergone the bidirectional Glenn procedure with MPA division. Despite the MPA having been divided as close to the bifurcation site as possible, the distal MPA stump appears to have been a site for stasis and subsequent thrombus formation. Although there was no evidence of subsequent pulmonary artery embolism, the risk for such and associated morbidity or mortality is clear. In the patient we report, ventricular outflow obstruction also developed because of a restrictive inlet VSD. Prior division of the MPA required her right ventricle to eject through the VSD to the aorta. Had a DKS procedure been performed, the development of outflow obstruction would have been avoided.
Thus, we believe that most patients with a univentricular heart (without pulmonary stenosis) are best served by a DKS anastomosis between the native MPA and aorta [6]. The DKS procedure maintains antegrade flow through the native MPA, thus eliminating the risk of stasis and subsequent thrombus formation in the distal MPA stump, as reported herein after MPA division. The DKS procedure also provides dual pathways for ventricular outflow, thus reducing the risk of late outflow obstruction from a restrictive VSD or bulboventricular foramen.
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References
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- Dajee H., Dutsch L.S., Benson L.N., Perloff J.K., Laks H. Thrombolytic therapy for superior vena caval thrombosis following superior vena cava–pulmonary artery anastomosis. Ann Thorac Surg 1984;38:637-639.[Abstract/Free Full Text]
- Dobell A.R., Trusler G.A., Smallhorn J.F., Williams W.G. Atrial thrombi after the Fontan operation. Ann Thorac Surg 1986;42:664-667.[Abstract/Free Full Text]
- Fyfe D.A., Kline C.H., Sade R.M., Gillette P.C. Transesophageal echocardiography detects thrombus formation not identified by transthoracic echocardiography after the Fontan operation. J Am Coll Cardiol 1991;18:1733-1737.[Abstract]
- Jahangiri M., Ross D.B., Redington A.N., Lincoln C., Shinebourne E.A. Thromboembolism after the Fontan procedure and its modifications. Ann Thorac Surg 1994;58:1409-1414.[Abstract/Free Full Text]
- Rosenthal D.N., Bulbul Z.R., Friedman A.H., Hellenbrand W.E., Kleinman C.S. Thrombosis of the pulmonary artery stump after distal ligation. J Thorac Cardiovasc Surg 1995;110:1563-1565.[Free Full Text]
- Jonas R.A. Intracardiac thrombus after the Fontan procedure [Editorial]. J Thorac Cardiovasc Surg 1995;110:1502-1503.[Free Full Text]
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Abstract
Introduction
