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Asian Cardiovasc Thorac Ann 2001;9:234-236
© 2001 Asia Publishing EXchange Pte Ltd

CASE STUDY

Ventriculoarterial Connection for Coronary-Pulmonary Artery Fistula

Lee Hyun Sung, MD, Park Young Hwan, MD, Cho Bum Koo, MD

Department of Thoracic and Cardiovascular Surgery Yonsei Cardiovascular Center & Research Institute Yonsei University College of Medicine Seoul, Korea
For reprint information contact: Park Young Hwan, MD Tel: 82 2 361 7283 Fax: 82 2 313 2992 email: yhpark{at}yumc.yonsei.ac.kr Yonsei Cardiovascular Center & Research Institute, Yonsei University College of Medicine, 134 Shinchon-dong, Seodaemun-ku, Seoul 120-752, Korea.

   Abstract
TOP
 Abstract
INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 
One-stage ventriculoarterial connection for correction of a coronary artery-to-main pulmonary artery fistula, and transfer of 2 major aortopulmonary collateral arteries from the descending aorta to the right pulmonary artery were successfully accomplished in a 4-month-old boy with pulmonary atresia and a ventricular septal defect.


   INTRODUCTION
TOP
 Abstract
 INTRODUCTION
CASE REPORT
 DISCUSSION
 REFERENCES
 
The pulmonary blood supply in patients with pulmonary atresia and ventricular septal defect (VSD) is most often through a patent ductus arteriosus or major aortopulmonary collateral arteries (MAPCA).1–5 Other less frequent sources are aortopulmonary window and persistent fifth aortic arch. It is rare to find a coronary artery-to-pulmonary artery fistula as a significant source of pulmonary blood supply.


   CASE REPORT
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
DISCUSSION
 REFERENCES
 
A 3-month-old boy was referred to our institution because of congenital heart disease and mild cyanosis. His birth weight was 3.3 kg after a normal pregnancy and full-term normal delivery. He had a sucking difficulty since birth, a heart murmur was detected at 1 month of age, and echocardiography indicated pulmonary atresia and VSD. On admission, he was malnourished, dehydrated, and in moderate cardiac failure. Physical examination revealed a slender boy 4.6 kg in weight (3–10 percentiles) and 61 cm in height (50–75 percentiles). Cyanosis was moderate and there was no clubbing of the fingers or toes. A systolic murmur grade 4/6 persisted over the left upper thorax, peripheral pulses were normal, and the liver was not enlarged. Cardiomegaly and increased pulmonary vascular markings were noted on a chest radiograph. An electro-cardiogram showed sinus tachycardia with biventricular hypertrophy. Blood hemoglobin was 118 g•L–1 and the hematocrit was 36.3%. The infant was stable on room air with a systemic arterial oxygen saturation of 89%. Echocardiography showed pulmonary atresia with a large VSD (1.2 cm), a coronary artery fistula from the left coronary artery to the pulmonary artery, as well as mitral, aortic, and tricuspid regurgitation (all grade 1). The ejection fraction was 65%. Cardiac catheterization and angiography demonstrated that the blood supply to the pulmonary circulation was via a large fistulous communication arising from the left main coronary artery and connecting with the main pulmonary artery. The fistula was 5 mm in diameter (Figure 1Go). The origin of the right coronary artery was normal. The aortic arch was left-sided. The right and left pulmonary arteries were fairly well developed. Two MAPCA from the descending aorta communicated with the posterior and apical segments of the right upper lobe, the superior and lingular segments of the left lower lobe, and the medial basal segment of the right lower lobe (Figure 2Go).



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  		Figure 1. Preoperative angiography showing the fistulous connection between the left main coronary artery and the main pulmonary artery.

 



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  		Figure 2. Balloon-block angiography of the descending thoracic aorta. (A) Major aortopulmonary collateral arteries communicate with the posterior and apical segments of the right upper lobe, (B) the superior and lingular segments of the left lower lobe, and the medial basal segment of the right lower lobe.

 
The patient underwent corrective surgery at 4 months of age. The repair was performed using standard arterial and bicaval cannulation with hypothermic cardioplegic arrest at 29.3°C and an aortic crossclamp interval of 62 minutes. Operative findings consisted of pulmonary atresia with a large perimembranous VSD. A large fistulous communi-cation was seen extending from the left main coronary artery to the main pulmonary trunk. The fistula was transected and the left coronary artery side was oversewn without compromising the origin of the left coronary artery from the fistula. The VSD was patched with double velour via a vertical right ventriculotomy. The main pulmonary artery was separated from the left main coronary artery and sutured directly to the right ventriculotomy site. Anteriorly, the repair was completed by placement of a patch of autologous pericardium with 5 absorbable sutures. The 2 MAPCA from the descending aorta were transferred to a unifocal site on the right pulmonary artery. Postoperatively, the patient did well and was discharged on day 14. A week later, right heart failure developed, which was managed with diuretics and inotropics. He was discharged again 1 month later. One year after the operation, he remained asymptomatic and was gaining weight appropriately.


   DISCUSSION
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
 DISCUSSION
REFERENCES
 
A coronary artery fistula supplying pulmonary blood flow in a case of pulmonary atresia with VSD was first reported by Allanby and colleagues1 in 1950. The fistula that arose from the left coronary artery, was found at autopsy. A large coronary artery-to-pulmonary artery fistula was described by Krongrad and colleagues2 as an "aortico-pulmonary tunnel". Prior to the present report, 20 cases have been described in the literature.3

Early recognition of a fistulous communication is important because irreversible pulmonary vascular disease may develop in the lung parenchyma if flow is un-obstructed. Thus, coronary-pulmonary fistulas should be considered a possible source of pulmonary blood supply in patients with pulmonary atresia and VSD, even when other sources of pulmonary flow are evident. Precise definition depends on selective coronary angiography. This is particularly crucial in planning the optimal surgical approach. The flow of blood through the fistula into a pulmonary artery with low resistance causes myocardial ischemia, both by producing coronary steal and by imposing volume overload on the left ventricle. Delay in correction of a coronary artery fistula causes congestive heart failure in infancy. In a single-stage correction, the number of operations, length of hospitalization, and cost are reduced. Early normalization of cardiovascular physi-ology with good future growth of new pulmonary arteries is expected. Early surgical correction is recommended.


   REFERENCES
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 

  1. Allanby KD, Brinton WD, Campbell M, Gardner F. Pulmonary atresia and the collateral circulation to the lungs. Guys Hosp Rep 1950;99:110–52.

  2. Krongrad E, Ritter DG, Kincaid OW. Aorticopulmonary tunnel: angiographic recognition of pulmonary atresia and coronary artery-to-pulmonary artery fistula. Am J Roentg Rad 1973;119:498–502.

  3. Solowiejczyk DE, Cooper MM, Barst RJ, Quaegebeur JM, Gersony WM. Pulmonary atresia and ventricular septal defect with coronary artery to pulmonary artery fistula: case report and review of the literature. Pediatr Cardiol 1995;16:90–4.[Medline]

  4. Rastelli GC, Patrick OA, Davis GD, Kirklin JW. Surgical repair for pulmonary valve atresia with coronary pulmonary artery fistula: report of case. Mayo Clin Proc 1965;40: 521–7.[Medline]

  5. Bogers AJJC, Rohmer J, Wolsky SAE, Quaegebeur JM, Huysmans HA. Coronary artery fistula as source of pulmonary circulation in pulmonary atresia with ventricular septal defect. Thorac Cardiovasc Surg 1990;38:30–2.[Medline]





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