Ann Thorac Surg 1995;59:738-740
© 1995 The Society of Thoracic Surgeons
Case Reports
Surgical Repair of Severe Bilateral Branch Pulmonary Artery Stenosis
Charles D. Fraser, Jr, MD,
Larry A. Latson, MD,
Roger B. B. Mee, FRACS
Department of Thoracic and Cardiovascular Surgery, The Cleveland Clinic Foundation, Cleveland, Ohio
Accepted for publication June 29, 1994.
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Abstract
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Management options for severe, bilateral branch pulmonary artery stenosis include percutaneous balloon dilation and direct surgical correction. Results with both balloon angioplasty and operation have been somewhat unpredictable. We report a case of staged surgical correction involving bilateral branch pulmonary artery reconstruction.
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Introduction
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Congenital branch pulmonary artery stenosis (PAS) is a challenging clinical problem with symptoms ranging from mild dyspnea to profound cyanosis and right heart failure [1, 2]. Management options include direct surgical repair and balloon angioplasty (BA) with or without stenting. We report here a case of staged surgical management of isolated branch PAS.
An 8-year-old girl was referred for treatment of severe bilateral branch PAS. Her past medical history was unremarkable. The child was minimally symptomatic but less active than her peers. She had no physical findings consistent with Williams' syndrome.
Cardiac catheterization revealed severe bilateral PAS and systemic right ventricular and main pulmonary artery (PA) pressures (Figs 1, 2
; Table 1). Distal PA pressures were low. Radioisotope lung perfusion studies demonstrated maldistribution of pulmonary blood flow (see Table 1). Balloon angioplasty was considered high risk because of systemic proximal pressures and the length of tubular vascular hypoplasia.
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Fig 1. . Pulmonary arteriogram demonstrating severe stenosis of the left pulmonary artery and branches. Note stenosis of the left upper lobe branch.
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Fig 2. . Pulmonary arteriogram demonstrating multiple severe stenoses of the right pulmonary artery and repair of the left pulmonary artery.
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Through a left thoracotomy, the left PA was reconstructed by excision of stenotic segments, augmentation of stenotic branching points using a cut-back angioplasty (Fig 3), and restoration of arterial continuity using an autologous pericardial roll. Subsequent study demonstrated a 50% decline in main PA and right ventricular pressures and redistribution of flow to the left lung (Fig 4; see also Table 1
).
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Fig 3. . ``Cut-back'' technique demonstrating incision in the septum dividing stenotic branches, and transverse closure resulting in an augmented orifice.
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A second operation through a median sternotomy employed cardiopulmonary bypass support. Stenotic right PA segments were excised and orifices were augmented. Direct anastomosis reestablished continuity. Postoperative study revealed normal PA and right ventricular pressures, favorable PA architecture, and normal blood flow distribution (see Table 1).
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Comment
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There is no consensus on the optimal management strategy for severe branch PAS. Results with both BA and operation have been somewhat unpredictable. Lock and colleagues [3–5] reported a low success rate and significant complications for BA using low-pressure balloons. High-pressure balloons improved results, but the mean increase in vessel diameter was only 37%. Investigators in the Valvuloplasty and Angioplasty of Congenital Anomalies Registry performed BA in 156 patients and reported significant complications in 21 patients, including five procedure-related deaths [6]. Although vessel diameter increased in successful cases, this afforded only minor reductions in proximal PA pressure. More recently, O'Laughlin and associates [7] combined BA with expandable intravascular stents. The short-term results are encouraging, but long-term results are unclear.
Considerable progress has been made in surgical angioplasty of small and stenotic pulmonary arteries, primarily in conditions including pulmonary atresia/ventricular septal defect with multiple aortopulmonary collateral arteries. These techniques include PA reconstruction using autologous pericardium, native arterial angioplasty, and unifocalization [8]. In this case, these methods were favored due to concern that BA had significant potential for postreperfusion pulmonary edema and hemorrhage. The surgical approach allowed simultaneous correction of all stenoses in one lung, minimizing the risk of reperfusion injury. Operation successfully reduced right ventricular and PA pressures to normal and achieved appropriate distribution of pulmonary blood flow.
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Footnotes
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Address reprint requests to Dr Fraser, Congenital Heart Surgery Service, The Cleveland Clinic Foundation, 9500 Euclid Ave, Desk F25, Cleveland, OH 44195.
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References
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- Papadopoulous GS, Folger GM. Progressive pulmonary arterial stenosis. Am J Cardiol 1983;51:1462–8.[Medline]
- Cheatham JP. Pulmonary stenosis. In: Garson A, Bricker T, and McNamara D, eds. The science and practice of pediatric cardiology. Philadelphia: Lea and Febiger, 1990:1382–1420.
- Perry SB, Keane JF, Lock JE. Interventional catheterization in pediatric congenital and acquired heart disease. Am J Cardiol 1988;61:109G–17G.[Medline]
- Ring JC, Bass JL, Marvin W, et al. Management of congenital stenosis of a branch pulmonary artery with balloon dilation angioplasty. J Thorac Cardiovasc Surg 1985;90:35–44.[Abstract]
- Gentles TL, Lock JE, Perry SB: High pressure balloon angioplasty for branch pulmonary artery stenosis: early experience. J Am Coll Cardiol 1993;22:867–72.[Abstract]
- Kan JS, Marvin WJ, Bass JL, Muster AJ, Murphy J. Balloon angioplasty-branch pulmonary artery stenosis: results from the valvuloplasty and angioplasty of congenital anomalies registry. Am J Cardiol 1990;65:798–801.[Medline]
- O'Laughlin MP, Slack MC, Grifka RG, Perry SB, Lock JE, Mullins CE. Implantation and intermediate-term follow-up of stents in congenital heart disease. Circulation 1993;88:605–14.[Abstract/Free Full Text]
- Stanley CJ, Lupinetti FM, Shah NL, et al. Primary unifocalization for the absence of intrapericardial pulmonary arteries in the neonate. J Thorac Cardiovasc Surg 1993;106:237–47.[Abstract]
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J. Thorac. Cardiovasc. Surg.,
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[Abstract]
[Full Text]
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Abstract
Introduction
