Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery

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1 CURRENT REVIEW Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery David B. Lerberg, M.D., John A. Ogden, M.D., J. R. Zuberbuhler, M.D., and Henry T. Bahnson, M.D. ABSTRACT Three patients with anomalous origin of the right coronary artery from the pulmonary artery are presented together with a review of the 14 patients with this anomaly previously reported. One of the current patients is the youngest to be diagnosed before operation and the youngest to undergo corrective operation. Although the lesion is usually asymptomatic, it may contribute to sudden death. The only suggestive physical finding is a continuous murmur with diastolic accentuation, which is present only in some patients. There are no diagnostic electrocardiographic or chest roentgenographic changes. Diagnosis is made best by selective left coronary arteriography showing retrograde filling of the right coronary artery from collateral vessels. Operative repair should consist either of direct reimplantation of the proximal right coronary artery into the aortic root or of saphenous vein bypass graft to the coronary artery with ligation of its origin. Operation is recommended when the lesion is recognized. Anomalous origin of the right coronary artery from the pulmonary artery is a rare coronary artery anomaly. While more than 140 patients with anomalous origin of the left coronary artery from the pulmonary artery have been described [9,20], only 14 patients with anomalous origin of the right coronary artery have been reported [l, 3,4,6-8,11-15,18,19). This report describes 3 additional patients with the latter anomaly, 1 of whom is the youngest patient to be diagnosed prior to operation and to undergo a corrective procedure. In addition, the previous 14 patients are reviewed together with these 3 patients in an attempt to provide addi- From the Departments of Surgery and Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, and the Department of Surgery, Yale University School of Medicine, New Haven, CT. Address reprint requests to Dr. Lerberg, Department of Surgery, University of Pittsburgh School of Medicine, Pittsburgh, PA tional insight into the diagnosis and management of this unusual condition. Case Reports Patient 1 The patient was 6 years old when a systolic heart murmur was first noted. When he was 10 years old, a diastolic component to the murmur appeared, and he was referred to Children s Hospital of Pittsburgh for evaluation. The only symptoms were fatigue compared with his peers and frequent colds. Physical examination showed a well-developed youngster with normal vital signs for his age. Peripheral pulses were full and equal. The first and second heart sounds were normal with accentuation of P2. There was a grade 316 continuous murmur with diastolic accentuation at the apex and left lower sternal border. The remainder of the physical examination was normal. Electrocardiogram was suggestive of left ventricular hypertrophy; the chest roentgenogram was normal. Cardiac catheterization was performed because a coronary cardiac fistula was suspected. Oxygen saturation demonstrated a slight stepup at the pulmonary artery level. All intracardiac pressures were normal. Aortic root cineangiograms demonstrated a single left coronary artery arising from the aorta with retrograde filling of a large right coronary artery which drained into the main pulmonary artery (Fig 1). Operative correction of the anomaly was performed under cardiopulmonary bypass using a median sternotomy incision. Except for its origin, the right coronary artery had a normal distribution but was dilated, thin-walled, and veinlike in character. A small button of tissue incorporating the orifice of the right coronary artery was excised from the pulmonary artery and directly transposed to the aorta. The patient s postoperative course was uncomplicated, and he was discharged on the eighth postoperative day with no murmur. He is well by David B. Lerberg

2 88 The Annals of Thoracic Surgery Vol 27 1 January 1979 Fig I. Right anterior oblique view of the aortic root angiogram with line interpretation. There is retrograde filling of the anomalous right coronary artery (RCA) from the left anterior descending coronary artery (LAD) with subsequent flow into the pulmonary artery (PA). (A0 = aorta.) RCA Fig 2. Postoperative aortic root angiogram and line interpretation showing the right coronary artery filling from the aorta (lateral projections).

3 89 Current Review: Lerberg et al: Anomalous Origin of RCA from PA and without symptoms 4 years after operation. Repeat angiograms showed some mild stenosis of the anastomotic site and "kinking" of the right coronary artery but the flow pattern was normal (Fig 2). Patient 2 A 2-year-old boy was admitted as a crib death. There was no mention of symptoms or presence of a murmur. ECG and chest roentgenogram were not obtained and cardiac catheterization was not performed. examination showed the right coronary artery ostium 5 mm above the pulmonary valve with a small membranous flap over part of the ostium. There were small anastomoses from the left anterior descending coronary artery to the right coronary artery across the right ventricular outflow tract. Histological studies demonstrated ischemic changes over the distribution of the right coronary artery, especially posteriorly. Patient 3 A 72-year-old man had no symptoms except dyspnea as a terminal event. A murmur had been noted since early adult life. On admission there was a continuous murmur in the third and fourth right intercostal spaces. ECG was normal and chest roentgenogram revealed slight cardiomegaly. The patient died of pneumonia and congestive heart failure before cardiac catheterization could be performed. examination showed mild left ventricular hypertrophy, cardiomegaly, dilatation of the left anterior descending coronary artery, and a veinlike, dilated right coronary artery, which arose from the pulmonary artery. Mate r i a 1 In an attempt to define characteristics that might help identify this unusual lesion, the 3 patients just described and the 14 patients previously reported were reviewed (Table). The 17 patients ranged in age from 2 to 90 years. Half of them were 30 years old or younger. There were 10 male and 7 female patients. A variety of cardiovascular symptoms occurred in the 17 patients: congestive heart failure in 4; fatigue, 3; cardiac arrest, 3; angina, 1; and dyspnea on exertion, 1. Some patients had more than one symptom. Associated congenital heart disease accounted for the symptoms in 3 patients, and acquired cardiovascular disease, such as hypertension or arteriosclerosis, explained most of the symptoms in the other patients. The small number of patients makes the identification of a common symptom complex difficult, and no symptom was clearly due to the anomalous right coronary artery. However, sudden death may occur without antecedent symptoms. In the 3 individuals who were seen in cardiac arrest, no lesion other than an anomalous right coronary artery was present to explain this dramatic event. A murmur was noted in 11 of the patients who were alive at the time of initial examination. It was systolic in 3 and systolic and diastolic or continuous in the remaining 8. Four of the patients had the classic continuous murmur that might be expected of an arteriovenous shunt. Diastolic accentuation of the murmur was noted in several and was the key to suspecting the diagnosis before catheterization and operation in the more recent patients. Age at onset of the murmur varied. murmur could be heard in 2 of the patients who were alive when initially examined, and in 4 patients the presence of a murmur was either not mentioned or the diagnosis was made postmortem. other characteristic physical signs were present. ECGs were not recorded or mentioned in 4 of the 17 patients. In 6, left ventricular hypertrophy was either suggested or definitely present; in one patient there was biventricular hypertrophy and in 1 there was right ventricular hypertrophy. Two patients demonstrated atrial fibrillation, and 3 had evidence of intraventricular conduction defects. ECG showed evidence,of ischemia or infarction. In the 11 patients for whom a chest roentgenogram was described, 9 showed some evidence of increased cardiac size, generally slight, or prominence of the left ventricle. In only 2 patients was the heart size considered normal on chest roentgenogram. Pulmonary vasculature was almost always described as normal in the isolated defect. Catheterization data were recorded in only 7 of the 17 patients. In 2 of them, the anomalous origin of the right coronary artery was asso-

4 90 The Annals of Thoracic Surgery Vol 27 1 January 1979 Summary of Clinical Data on Patients with Anomalous Right Coronary Artery Author Monckeberg [81 30, M ne Schley [15] Jordan et a1 [71 Cronk et a1 [41 Rowe and Young 1141 Pribble [ll] Burroughs et a1 [31 Ranniger et a1 [121 Rhantigan and de la Torre [131 Wald et a Tingelstad et a1 [181 Eugster and Oliva [6] Bregman et a1 [l] Lerberg et a1 [this report Clinically Shunt- Vein- Age, Symp- sus- ing Dilated like Associated Diag- Sex toms Murmur pected ted LCA RCA Conditions nosed 61, M CHF 74, M CHF 90, M ne 7, M Fatigue 60, F Angina 16, M Fatigue 6, F ne 59, F ne 42, F ne 11, F ne 12, M ne 64, F CHF 25, F ne ll,m ne 2, M ne 72, M ne Systolicdiastolic Systolic systolic Systolicdiastolic Systolic Systolicdiastolic Continuous Continuous Systolicdiastolic ne ne Continuous ne Continuous -k Epilepsy, subdural hematoma Lues, AI, CHF HBP, CHF HBP, encephalomalcia ASD RHD, MS AP window, VSD TOF, PDA Diverticular disease Postop. breast cancer Cardiac arrest AF Cardiac arrest Crib death Operation Operation AngiograPhY Angiog- aphy Angiog- aphy Angiog- aphy Angiog- aphy AngiograPhY LCA = left coronary artery; RCA = right coronary artery; = not stated; CHF = congestive heart failure; AI = aortic insufficiency; HBP = hypertension; ASD = atrial septal defect; RHD = rheumatic heart disease; MS = mitral stenosis; AP window = aortopulmonary window; VSD = ventricular septal defect; TOF = tetralogy of Fdot; PDA = patent ductus arteriosus; AF = atrial fibrillation. ciated with other congenital heart disease. In the remaining 5 patients with isolated anomalous origin of the right coronary artery, pressures were found to be normal and there was little or no oxygen step-up in the main pulmonary artery. All five cineangiograms showed similar findings with a tortuous left coronary artery filling a dilated and tortuous anomalous right coronary artery through numerous collateral vessels. The lesion was clinically suspected in only 3 of the 17 patients. The anomaly was diagnosed in 9 at postmortem examination, in 6 with an- giography, and in 2 during an operative procedure for another cardiac anomaly. In all patients the right coronary artery arose from the right side of the pulmonary artery and had a normal distribution along the atrioventricular groove and to the right ventricular myocardium. Minor anomalies of terminal distribution were noted [7]. In several instances the ostium to the right coronary artery was located above the sinus ridge. The artery was described as thin and dilated or veinlike on gross inspection in 15 patients; all were presumed to have normal pulmonary artery pressures. His-

5 91 Current Review: Lerberg et al: Anomalous Origin of RCA from PA tological study, however, showed the basic structure to be arterial [ll].' Two of the patients [3, 121 had congenital heart disease with pulmonary hypertension. In both, the right coronary artery was described as normal in appearance. In all patients the left coronary artery had a normal origin from the left aortic sinus and a normal distribution, and in 14 the left coronary artery was described as dilated. In adult patients arteriosclerotic changes were seen in the left coronary artery but not in the anomalous right coronary artery. In most patients the main anastomotic site was over the right ventricular outflow tract between the left anterior descending coronary artery and the right coronary artery. Operation Three separate surgical procedures have been used in the operative repair of this anomaly. Simple ligation of the right coronary artery at its origin from the pulmonary artery was performed in 1 patient, and no adverse effects resulted from this procedure [14]. Ligation of the anomalous coronary artery at its origin from the pulmonary artery with subsequent aortocoronary saphenous vein bypass graft to the distal portion of the right coronary artery was done in an elderly patient [6]. The subsequent postoperative course was uneventful. Four of the patients who underwent surgical correction had direct implantation of the anomalous right coronary artery along with a cuff of adjacent pulmonary artery into the aorta [l, 3, 181. Comment Soloff [161 described four possible types of anomalous origin of the coronary arteries from the pulmonary artery: (1) origin cf the left coronary artery from the pulmonary artery; (2) origin of the right coronary artery from the pulmonary artery; (3) origin of both coronary arteries from the pulmonary artery; and (4) origin of an accessory coronary artery from the pulmonary artery. Anomalous origin of the left coronary artery from the pulmonary artery is the most frequently encountered of the four types and usually causes severe ischemia of the left ventricle early in infancy. If abundant anastomoses are present between the two coronary arteries, the onset of symptoms may be long delayed [ZOI. An accessory coronary artery arising from the pulmonary artery has been recognized in only a few instances and probably represents an incidental finding with no hemodynamic importance [2]. Anomalous origin of both coronary arteries from the pulmonary artery is incompatible with life, and infants die within days of birth [17] as pulmonary vascular resistance and, therefore, pulmonary artery perfusion pressure fall. Anomalous origin of the right coronary artery from the pulmonary artery is the second most common of the four types and an increasing number of instances of this malformation have been described and recognized in recent years. Unlike anomalous left coronary artery, anomalous origin of the right coronary artery may often be an incidental finding without serious cardiovascular sequelae. However, in 4 of the 17 reported patients, the lesion may have contributed to cardiac arrest or death. Pribble [ll] believed that the lesion contributed to the death of the patient he described. Angina pectoris had been a prominent symptom throughout the patient's illness and no occlusive disease was found to account for it. Ischemic changes seen microscopically were attributed to an inadequate supply of oxygen by an anomalous right coronary artery to a myocardium heavily involved with rheumatic heart disease. anastomoses between the left and right coronary artery were described. One patient had a cardiac arrest at home, and postmortem examination revealed fresh thrombus in the single left coronary artery system [191. Microscopic studies showed changes of shortand long-term ischemic injury. It is likely that shunting of blood from the left ventricular myocardium to the anomalous right coronary artery contributed to the patient's early death. A second patient also was seen in cardiac arrest [ll. This patient was resuscitated, and no lesion other than an anomalous right coronary artery was demonstrable. Surgical repair of the anomaly was successful. Our Patient 2 was seen as a crib death and presumed cardiac arrest. Although there may be another cause for death, it is difficult to overlook the only pathological finding of an anomalous right coronary artery.

6 92 The Annals of Thoracic Surgery Vol 27 1 January 1979 Since this lesion is usually asymptomatic, the incidence is probably higher than that reported in the literature. Several of the reported patients were more than 50 years old and worked up to the time of death. Although it is doubtful that infantile and adult types exist, differences in the development of collateral circulation may cause the anomaly to be diagnosed or become evident at an earlier or later age. The only characteristic physical finding is a continuous murmur with diastolic accentuation. Tingelstad and associates El81 demonstrated that the murmur could be recorded by intracardiac phonocardiography with the microphone placed near the bifurcation of the main pulmonary artery. In the patient reported by Burroughs and colleagues [31 a diastolic component to the murmur would not be expected since flow through the anomalous right coronary artery was antegrade because of pulmonary hypertension. The presence of a diastolic component to the murmur in 1 reported patient may be attributed to a patent ductus arteriosus The absence of a diastolic component to the murmur in 2 other patients is unexplained especially since shunting indicating collateral circulation was noted at the time of operation [4, 141. The lack of a typical murmur in the second patient reported by Wald and colleagues [19] might be explained by thrombosis of the left coronary artery or marginal shunting through collateral vessels or both these situations. In our Patient 1, the diastolic component did not appear until 4 years after the systolic component had first been noted. This may be indicative of developing collaterals. Those patients without diastolic murmurs may not have had large collaterals; however, the patient described by Rowe and Young [141, who had only a systolic murmur, demonstrated definite right-to-left shunting at operation. In contrast to anomalous origin of the left coronary artery that often shows evidence of infarction or ischemia [20], anomalous right coronary artery was not associated with these changes. In several of the reported instances, the abnormal ECG findings could be attributed to associated acquired or congenital heart disease. A lower wall tension in the right ventricle may permit adequate right ventricular myocardial blood flow and adequate nourishment de- spite antegrade flow of poorly oxygenated blood from the pulmonary artery or retrograde flow through the anomalous right coronary artery [lo]. Epicardial ECGs were obtained during the operative procedure in 1 reported patient, and there was no evidence of ischemia in the region of the distribution of the anomalous right coronary artery when it was ligated at the pulmonary artery [14]. Ischemic changes would have been expected to occur if antegrade flow was present in the ligated vessel. The gross cardiomegaly seen in patients with anomalous origin of the left coronary artery would not be expected in an anomalous right coronary artery because there are differences in the reactions of the right and left ventricle to periods of prolonged low perfusion pressure and low oxygen saturation [lo]. The cardiomegaly that was found could be attributed to other forms of cardiovascular disease, such as longstanding hypertension, aortic insufficiency, or arteriosclerotic vascular disease. Wald and colleagues commented that in 1 of their patients the shunt was moderate in size and may have contributed, by virtue of volume overloading, to the left ventricular enlargement suggested on ECG and chest roentgenogram. In addition, they suggested the prominent upper left cardiac border was produced by the dilated proximal left coronary artery. Because the changes in the right ventricle are minimal and because the left-to-right shunt is usually small, there is normally no evidence of the coronary anomaly on chest roentgenogram. Diagnosis must be made at arteriography, operation, or postmortem examination. Cardiac catheterization showed normal pressures and little or no oxygen step-up in the pulmonary artery. In 2 patients, our Patient 1 and a patient reported by Tingelstad and coworkers [181, a slight step-up in oxygen saturation was noted at the pulmonary artery level. Ranniger and co-workers [121 stressed the angiographic appearance and claimed their patient was the first to be diagnosed clinically. Cardiac catheterization alone is not helpful, and the diagnosis must be made by cineangiography. The characteristic picture of retrograde filling of the right coronary artery from collateral vessels and subsequent flow into the pulmonary artery is unmistakable. In 1885 Brooks

7 93 Current Review: Lerberg et al: Anomalous Origin of RCA from PA RCA RCA P in t ercorona ry col la t era I s I vc - Fig 3. lllustrations of findings and operation in Patient I. The anomalous right coronary artery (RCA) was transferred with a cuff of the zoall of the pulmonary artery (PA). 121 suggested and Edwards [5] in 1958 affirmed that flow in an aberrant right coronary artery was toward the pulmonary artery by way of collaterals from the normally arising left coronary artery. Although blood flow in an anomalous left coronary artery may rarely be from the pulmonary artery to the anomalous left coronary, in the patients reviewed direction of blood flow in the anomalous right coronary artery was always from the anomalous vessel to the pulmonary artery when the direction of flow was demonstrated by angiography or at operation. The metabolic requirements of the myocardial regions supplied by the right coronary artery are usually met but evidence of ischemia has been described along the course of the anomalous right coronary artery. Three patients underwent operative repair of the anomaly and subsequently were recatheterized. rmal antegrade flow into the right coronary artery was demonstrated on cineangiograms of all 3. Persistent dilatation and kinking of the coronary artery were present. Flow through the left coronary artery and intercoronary collateral anastomoses had diminished. The prognosis in this anomaly appears to be good. The overall incidence is probably greater than that recorded in the literature since it is a relatively asymptomatic anomaly. In those patients surviving to adulthood, however, considerable arteriosclerosis was present in the left coronary artery. This may have been the cause of death in 1 of the patients recorded. In several of the other patients, pathological abnormalities including ischemia (our Patient 2 and [ll, 19]), left-to-right shunting [18, 191, heart failure [61, fibrosis [lll, and infarcts [191 were documented. Indications for operation may include the establishment of a two coronary system, the elimination of pathological abnormalities noted in several of the patients, and a prophylactic procedure to avoid the onset of left ventricular dysfunction when a single coronary artery system is associated with common acquired cardiovascular disease, such as arteriosclerosis, hypertension, or rheumatic heart

8 94 The Annals of Thoracic Surgery Vol 27 1 January 1979 disease. In addition, there is a definite similarity between this malformation and a coronary arteriovenous fistula, which may cause cardiac failure and death at any age. Of the several operative approaches available, the most promising is direct implantation of the anomalous right coronary artery along with a cuff of the pulmonary artery into the aorta (Fig 3). This is technically feasible since the right coronary artery is long enough and the inclusion of a cuff of pulmonary artery permits a large anastomosis. This type of operation does not necessarily require cardiopulmonary bypass. The long-term patency of the direct arterial anastomosis is probably higher than that of a venous graft. References 1. Bregman D, Brennan F, Singer A, et al: Anomalous origin of the right coronary artery from the pulmonary artery. J Thorac Cardiovasc Surg 72:626, Brooks H St J: Two cases of an abnormal coronary artery of the heart arising from the pulmonary artery. J Anat Physiol 20:26, Burroughs JT, Schmutzer KJ, Linder F, et al: Anomalous origin of the right coronary artery with aortico-pulmonary window and ventricular septa1 defect. J Cardiovasc Surg 3:142, Cronk ES, Sinclair JG, Rigdon RH: An anomalous coronary artery arising from the pulmonary artery. Am Heart J 42:906, Edwards JE: Anomalous coronary arteries with special reference to arteriovenous-like communications (editorial). Circulation 17: 1001, Eugster GS, Oliva PB: Anomalous origin of the right coronary artery from the pulmonary arteiy. Chest 63:294, Jordan RA, Dry TJ, Edwards JE: Anomalous origin of the right coronary artery from the pulmonary trunk. Mayo Clin Proc 25:673, Monckeberg JG: Uber eine seltene anomalie des koronararterienabgangs. Zentralbl Herz Gefasskr 6:441, Neches WH, Mathews RA, Park SC, et al: Anomalous origin of the left coronary artery from the pulmonary artery. Circulation 50:582, Ogden JA: Secondary coronary artery fistulas. J Pediatr 78:78, Pribble RH: Anatomic variations of the coronary arteries and their clinical significance. J Indiana State Med Assoc 54:329, Ranniger K, Thilenius OG, Cassels DE: Angiographic diagnosis of an anomalous right coronary artery arising from the pulmonary artery. Radiology 88:29, Rhantigan RM, de la Torre A: Anomalous origin of the right coronary artery. Vasc Surg 5:196, Rowe GG, Young WP: Anomalous origin of the coronary arteries with special reference to surgical treatment. J Thorac Cardiovasc Surg 39:777, Schley J: Abnormer ursprung der rechten kranzarterie aus de pulmonalis bei einem 61 jahrigen mann. Frankfurt Z Pathol 32:1, Soloff LA: Anomalous coronary arteries arising from the pulmonary artery. Am Heart J 24:118, Tedschi CG, Helbern MM: Heterotopic origin of both coronary arteries from the pulmonary artery. Pediatrics 14:53, Tingelstad JB, Lower RR, Eldredge WJ: Anomalous origin of the right coronary artery from the main pulmonary artery. Am J Cardiol 30:670, Wald S, Stonecipher K, Baldwin BJ, et al: Anomalous origin of the right coronary artery from the pulmonary artery. Am J Cardiol27:677, Wesselhoeft H, Fawcett JS, Johnson AL: Anomalous origin of the left coronary artery from the pulmonary trunk. Circulation 38:403, 1968