Catheterization and Cardiovascular Diagnosis 9:373-380 (1983)

Transpulmonary Artery Correction of Anomalous Origin of the Left Coronary Artery by Saphenous Vein Graft

Jeffrey Fisher, MD, Garth McDonald, MD, Jeffrey Brinker, MD, Catherine A. Neill, MD, James S. Donahoo, MD, and Kenneth L. Baughman, MD

A transpulmonary saphenous vein graft was used to repair an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Postoperative cardiac catheterization revealed correction of the previous systemic arterial to venous shunt and a patent intrapulmonary artery conduit. The patient has experienced substantial symptomatic and functional improvement from the operation despite development of mild pulmonary outflow obstruction. Saphenous vein transpulmonary artery correc- tion for ALCAPA offers an alternative surgical approach and may be the procedure of choice when anatomy or prior surgery preclude the more standard modes of revas- cularization.

Key words: anomalous coronary artery, Bland-White-Garland syndrome, transpulmonary ar- tery correction

INTRODUCTION

Anomalous origin of the left coronary artery from the pulmonary artery (AL- CAPA), or Bland-White-Garland syndrome [ 1,2], is a rare congenital cardiovascular disorder. Though the clinical spectrum is broad and some patients have presented as teenagers or adults without symptoms [ 3 ] , survival beyond infancy is uncommon. More than 85 % of infants born with this defect die in the first year of life if corrective surgery is not performed [2,4]. Patients surviving infancy without corrective surgery have an estimated life expectancy of 35 years with an increased risk of sudden and often unexpected death [5,6] . Therefore operation has been the mainstay of therapy for all age groups diagnosed as having this anomaly.

From the Divisions of Adult and Pediatric Cardiology and Cardiovascular Surgery, Johns Hopkins Hospital, Baltimore.

Address reprint requests to: Jeffrey Fisher, MD, Division of Cardiology, New York Hospital, 525 East 68th Street, New York, NY 10021.

Received January 22, 1983; revision accepted April 20. 1983.

0 1983 Alan R. Liss, Inc.

374 Fisher et al

The surgical approaches in ALCAPA have attempted to improve oxygenation to the left ventricle both by revascularization of the ventricle and by decreasing the left- to-right shunt. The results of early methods such as aorticopulmonary anastomosis [7], pericardial poudrage with de-epicardialization [8], and banding of the pulmonary artery [9] were disappointing and were abandoned for the technique of ligation of the anomalous artery which proved more successful [ 101. However, in infants without substantial right-to-left intercoronary collateral channels, mortality with ligation has been high [ 111. Subsequently, aortocoronary saphenous vein bypass graft [ 121, reim- plantation of the anomalous coronary artery [ 13 , 141, and left subclavian artery to anomalous coronary artery anastomosis [ 151 have been employed to revascularize the ventricle. Recently creation of an aortopulmonary window and tunnel within the pulmonary artery to direct aortic blood through the pulmonary artery to the origin of the anomalous left coronary artery has been utilized. Tunnels have been fashioned with a flap of pulmonary arterial wall [ 161, pericardium [ 171, and segments of the left subclavian artery [ 181. Transpulmonary artery corrections for ALCAPA have been reported [16-181 in children and infants.

We report a patient with ALCAPA who underwent phenol de-epicardialization and talc poudrage at 3 months of age, and transpulmonary artery correction at age 22, utililzing a saphenous vein “tunnel.”

CASE REPORT The patient was admitted to Johns Hopkins Hospital in 1958 at the age of 3 months

after several weeks of progressive dyspnea. She was tachypneic and tachycardic with normal heart sounds and hepatomegaly. Chest x-ray showed an enlarged heart without pulmonary vascular congestion. The electrocardiogram revealed an anterior myocar- dial infarction and left ventricular hypertrophy. The patient was diagnosed as having anomalous origin of the left coronary artery from the pulmonary artery. The diagnosis was confirmed by cardiac catheterization 1 month later. The left ventricle was noted to be dilated with decreased ventricular function. A phenol de-epicardialization and talc poudrage was performed and the thymus and left lung were sewn to the left ventricular surface. The postoperative course was uneventful.

The patient developed normally and was without symptoms during childhood. At age 8 she was noted to have a continuous murmur with mild diastolic accentuation and an S3 gallop. At age 16 she developed angina pectoris and ventricular ectopy that increased with exercise. The patient was readmitted to the hospital at age 22 with angina pectoris and ventricular tachycardia. Digitalis, quinidine, disopyramide, and propranolol were utilized without adequate suppression of ventricular ectopy.

Cardiac catheterization was performed in December 1979. There was a 1 :5: 1 left- to-right shunt from the right coronary artery through the anomalous left coronary artery to the pulmonary artery. Pulmonary artery pressure was normal. Ventriculog- raphy revealed a moderately enlarged left ventricle with mild mitral regurgitation. There was marked hypokinesis of the anterior wall and septum with fair motion of the lateral and inferior walls. The right coronary artery was in its normal position, but was enlarged and supplied collaterals to the left anterior descending coronary artery and circumflex coronary artery. The left main coronary artery had its origin from the posterior aspect of the main pulmonary artery (Fig. 1).

Correction of Anomalous Left Coronary Artery 375

Fig. 1. Left anterior oblique projection of a right coronary artery injection revealing a markedly enlarged right coronary artery, extensive collateral development, retrograde filling of the left anterior descending the circumflex coronary arteries, and visualization of the origin of the left main coronary segment from the pulmonary artery.

At operation there was adhesive pericarditis involving the right atrium, right ventricle, aorta, and pulmonary artery. Encasing the left ventricular surface was a thick layer of fibrous connective tissue which prevented direct aortocoronary bypass. The operative procedure is illustrated in Figure 2. The enlarged main pulmonary artery was opened transversely and a 6 mm aortopulmonary window was created. A short segment of saphenous vein was interposed between the origin of the anomalous coronary artery and the aortopulmonary window. The pulmonary artery was closed with an anterior pericardial patch.

Postoperative convalescence was uneventful. Postoperative auscultation revealed a high-pitched systolic murmur over the pulmonic area with radiation to the back and precordium which increased with inspiration and a new diastolic blowing murmur at the right sternal border.

The patient underwent postoperative cardiac catheterization in April 1980. There was no measurable systemic arterial to venous shunt. Aortography revealed prompt filling of the intrapulmonary artery conduit and the left anterior descending and circumflex coronary arteries. The right coronary artery had decreased in size and there was minimal filling of septa1 collateral vessels (Fig. 3). There was a suprapul- monic valve outflow gradient of 25 mm Hg. A right ventricular outflow tract injection demonstrated the intrapulmonary artery conduit just above the pulmonic valve.

The patient was last evaluated 30 months after surgery at which time she was asymptomatic on no medications. Ambulatory electrocardiographic monitoring re-

376 Fisher et al

Fig. 2. A) Pictoral representation of the operative field after cannulation for heart lung bypass. Note the posterior position of the anomalous coronary artery and the proposed aortopulmon- ary window sites. B) The transpulmonary artery conduit.

Correction of Anomalous Left Coronary Artery 377

Fig. 3. Postoperative left anterior oblique right coronary artery injection. The right coronary has diminished in size, and neither the left anterior descending nor the left circumflex coronary arteries are visualized.

vealed only infrequent multiform ventricular premature contractions. The results of a radionuclide excercise stress test showed good exercise capacity (13.5 min; heart rate 175 bpm) without symptoms or signs (Electrocardiographic changes, arrhythmias, or regions of thallium redistribution) of ischemia.

DISCUSSION

Operation has been the only definitive therapy for patients with anomalous left coronary artery from the pulmonary artery. Ligating the anomalous artery may prevent ischemia by eliminating the systemic arterial to venous shunt and coronary steal [ 191. However, in the absence of adequate intercollateral channels, ligation alone may result in ischemia, infarction, or death [ 3 , I I , 181. Moreover, patients with angiographically adequate collateral vessels may develop signs of ischemia following ligation [20, 2 13. Though techniques of indirect revascularization [7-91 were unsuc- cessful, direct myocardial revascularization has provided an alternative approach. Although there are the added risks of cardiopulmonary bypass and subsequent graft closure [22] or stenosis [23], direct revascularization may provide better long-term results [24].

To our knowledge this patient is the first adult reported to undergo transpulmonary artery correction for ALCAPA, and the first patient of any age in whom a saphenous vein graft was employed as the intrapulmonary artery conduit. The patient’s survival

378 Fisher et al

to adulthood was probably unrelated to the indirect revascularization attempted in infancy. However, the dense fibrous connective tissue overlying the left ventricle from the prior procedure prevented creation of a direct aortocoronary bypass graft. In addition, the origin of the anomalous left coronary artery was located on the posterior wall of the pulmonary trunk making reimplantation of the anomalous artery to the aorta technically hazardous.

There have been three previous reports involving six patients (3 infants, 3 children) of transpulmonary artery correction for ALCAPA [ 16-18]. One infant died postop- eratively. The remaining five patients have done well with evidence of improved left ventricular function, though the maximum postoperative follow-up reported has been 17 months. Our patient has been followed for 30 months following surgery and has been asymptomatic. There has been a marked decrease in the frequency and severity of ventricular premature beats which were, prior to surgery, refractory to convention- ial antiarrhythmic therapy. In addition, she has excellent exercise capacity without signs of ischemia.

Arciniegas et a1 [18] report the use of a free segment of the left subclavian artery for the intrapulmonary artery conduit instead of the flap of pulmonary arterial wall employed by Takeuchi et a1 [ 161 or pericardium used by Hamilton et a1 [ 171. They claim that the use of the subclavian artery allows better exposure and easier construc- tion of the anastomoses. Moreover, they claim that the subclavian artery may be used regardless of left coronary artery diameter, and that arterial tissue should not undergo late fibrous thickening and aneurysmal dilatation as occurs with saphenous veins and pericardial baffles when subjected to systemic arterial pressure. Use of pulmonary artery flaps and pericardium as intrapulmonary artery conduits requires more exten- sive intra- and extrapulmonary suture lines than does use of the free segment of the subclavian artery. However, in standard coronary artery bypass grafts, free arterial tissue has not offered a significant advantage to saphenous veins [25], although intimal hyperplasia associated with use of radial artery segments for myocardial revasculari- zation may be less common when larger-caliber arterial segments are employed such as hypogastric and subclavian arteries. In addition, use of saphenous veins is techni- cally easier and avoids the potentially more serious complications inherent in subcla- vian artery resection and repair. However, though saphenous vein reconstruction is feasible in adults with ALCAPA, it may be a technically more difficult procedure in infants and children. Moreover, the risk of late fibrosis with subsequent occlusion of the vein graft is a possibility, particularly in the young patient.

It is our hope that direct revascularization of the left ventricle performed by saphenous vein transpulmonary artery correction will improve our patient’s long-term prognosis. It is unlikely that the mild pulmonary outflow obstruction will progress. Whether similar changes will occur with arterial conduits is at present not known. Saphenous vein transpulmonary artery correction offers an alternative approach for ALCAPA and may be the procedure of choice when anatomy or prior surgery precludes the more standard modes of revascularization.

ADDENDUM

The patient gave birth to a healthy 7 lb 14 oz daughter on July 2 , 1983. The pregnancy was uncomplicated; digitalis was the only medication. There were no clinical or echocardiographic signs of cardiac decompensation during pregnancy and delivery.

Correction of Anomalous Left Coronary Artery 379

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