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Case Report

Anomalous Origin of Right Coronary Artery from Pulmonary Artery and Severe Mitral Regurgitation (see full title below)

Harshit Modi, MD, Aekarach Ariyachaipanich, MD, Muhyaldeen Dia, MD, FACC
April 2010

Anomalous Origin of Right Coronary Artery from Pulmonary Artery and Severe Mitral Regurgitation Due to Myxomatous Mitral Valve Disease: A Case Report and Literature Review

ABSTRACT: Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is an uncommon congenital coronary artery anomaly. In contrast to anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), most of the patients with ARCAPA remain asymptomatic. However, few cases of angina, heart failure and sudden cardiac death depicting the malignant nature of the disease are described in the literature. We report an unusual case of ARCAPA with severe mitral valve regurgitation and pulmonary hypertension. Echocardiography and angiography were utilized for the diagnosis. Surgical correction was provided to our patient and should be considered for all patients with this potentially fatal disease. J INVASIVE CARDIOL 2010;22:E49–E55 Key words: anomalous coronary artery, pulmonary artery, mitral regurgitation, Transesophageal echocardiography, coronary angiography
The incidence of anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is 0.002%. The natural history of the disease and its clinical features are not well described in the literature except for few a case reports. The diagnosis of anomalous ARCAPA is usually done incidentally with other cardiac anomalies such as aortopulmonary window or tetralogy of Fallot. We report the case of a patient with severe mitral regurgitation who was incidentally found to have ARCAPA. Case 1. A 62-year-old Caucasian male with a past medical history of rheumatic fever in early childhood and dyslipidemia presented with worsening shortness of breath with minimal exertion for the past 3 months. In addition, the patient did not complain of chest pain, orthopnea or paroxysmal nocturnal dyspnea. There was no family history of coronary artery disease or cardiovascular disease. He had a 10-pack per year history of smoking which he quit 30 years ago and drank alcohol occasionally. He was taking aspirin, atorvastatin and omprazole. Upon physical examination, his vital signs were normal. A cardiac examination revealed a grade 3/6 pan-systolic murmur at the apex radiating towards the left axilla. The laboratory workup was unremarkable except for a high B-type natriuretic peptide of 489 pg/dl. Electrocardiography showed normal sinus rhythm with new antero-inferior ST-T depression. An increased cardiothoracic ratio and clear lung fields were seen on chest radiograph. With the suspicion of mitral valve regurgitation, transthoracic (TTE) and transesophageal echocardiography (TEE) were performed, showing mild left ventricular hypertrophy with no significant segmental wall motion abnormality and both left and right ventricular dilatation. Evaluation of the mitral valve revealed myxomatous changes with prolapse of the P2 segment of the mitral valve and evidence of a ruptured chordae. A severe jet of insufficiency was identified over the anterior mitral valve leaflet directed toward the anterior aspect of the enlarged left atrium. The ejection fraction was around 50%. A transesophageal long-axis view showed a probable anomalous coronary artery. To confirm the diagnosis and assess the hemodynamic significance, combined right and left cardiac catheterizations were performed. Selective coronary angiography showed a large dilated and tortuous coronary artery from the left coronary sinus with a typical course of the left anterior descending artery (LAD) and left circumflex artery (LCX) (Figure 1B). The origin of the right coronary artery (RCA) was not demonstrated on aortic root angiography (Figure 1A). The RCA was later contrasted through several large epicardial collateral vessels originating from the LAD and LCX (Figure 1B). The RCA itself was dilated and drained into the pulmonary artery (Figures 1B, 1C, 1D). Left ventriculography revealed severe mitral regurgitation with an ejection fraction of about 50%. The right-heart catheterization findings are described in Table 1. The patient was found to have elevated pulmonary artery and pulmonary capillary wedge pressures. There was no significant “oxygen step-up” between the right ventricle (69.8%) and the pulmonary artery (70.6%). A diagnosis of ARCAPA with severe mitral regurgitation and severe pulmonary hypertension was made. The patient underwent surgery for reimplantation ofthe anomalous RCA to an anterior aortic sinus and mitral valve repair by ring annuloplasty with no complications. He was discharged afterwards to cardiac rehabilitation. TTE at 3 months post surgery showed an intact mitral valve annuloplasty ring with no residual mitral regurgitation. Review of the literature. Since the first case of ARCAPA was reported in 18851, 99 cases have been described. Table 3 shows the characteristics of 93 of these patients. We conducted a literature review of ARCAPA by performing a MEDLINE database search for reported cases published between 1950 and June 2009 using the following keywords: “ARCAPA,” “anomalous origin of coronary artery from pulmonary artery” and “coronary artery anomaly.” All searches were limited to studies published in English. We extracted information from abstracts and published articles when possible. Discussion. ARCAPA is a rare yet serious congenital coronary disorder. In 1945, Soloff described four possible types of anomalies of the coronary artery originating from the pulmonary artery.2 These can be an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), ARCAPA, both coronary arteries from the pulmonary artery and an accessory coronary artery from the pulmonary artery. Among these, ALCAPA is the most common anomaly, with an incidence of 0.008% in the general population compared to a rate of 0.002% for ARCAPA.44,76 From the literature review, we found that only 25–30% cases of ARCAPA are associated with structural heart defects. To the best of our knowledge, this is the second case report where ARCAPA was diagnosed with severe mitral regurgitation and severe pulmonary hypertension. Many patients with isolated ARCAPA remain asymptomatic in early on in life and it is diagnosed incidentally with other types of cardiac anomalies. 3 Table 3 shows the characteristics of 98 reported cases including the current case. Out of 98 cases, 50 were males and 39 were females, while for 9 cases, the gender was unknown. There was a vast difference in age of the patients when the disease was identified, ranging from 1,13 Twelve cases were diagnosed in infants ≤ 1 year, 44 were diagnosed in children ≤ 18 years of age, 17 were adults > 60 years of age, and 8 subjects were of unknown age. The pathophysiology and manifestations of ARCAPA depend on the direction of blood flow in the coronary artery and the impact on myocardial oxygen delivery. Massive collateralization between the normal and anomalous vessel is needed to maintain adequate myocardial perfusion in the presence of coronary steal. Coronary steal occurs when the anomalous vessel acts as a vein, which collects blood from the normal coronary artery through collaterals and delivers it to the pulmonary artery. Long-term coronary steal phenomena and blood supply from a single coronary system causes myocardial ischemia and heart failure. An increase in myocardial oxygen demand due to physiological factors like exercise, stress or anatomical lesions such as valvular abnormalities, increases the risk of ischemia. Many patients with isolated ARCAPA remain asymptomatic in childhood, however, patients with ALCAPA show signs of mitral insufficiency or anterolateral myocardial ischemia during early childhood.20 In ARCAPA, the most common presenting symptom was angina in 18% of cases. Other common symptoms were dyspnea on exertion for 13% of patients, congestive heart failure in 11%, sudden cardiac arrest in 6%,13,15,42,53,56,59 cyanosis in 4 patients, as well as acute myocardial infarction, 6,30,77 palpitation,70,83 atrial fibrillation,24,85 bradycardia24,60 and myocarditis.23 Five patients were diagnosed at the time of autopsy.13,15,42,53,59 The most frequent presenting sign is murmur found in 41% of patients. Patients with associated cardiac anomalies are diagnosed early in life compared to patients with isolated ARCAPA. Associated cardiac defects were reported in 22 (22%) of the patients reported with ARCAPA. The most common cardiac defects found in these patients were aortopulmonary window5,10,25,27,36,41,57 and tetralogy of Fallot. 3,28,37,38,58,67,76 Other uncommon associated cardiac anomalies included atrial septal defect,16,26,34 ventricular septal defect,48 double outlet right ventricle, 29 patent ductus arteriosus, 76 coarctation of the aorta,76 pulmonary stenosis,28 aortic stenosis, 22,75 mitral stenosis,76 mitral regurgitation 21 and tricuspid regurgitation.21 The modality of diagnosis of ARCPA has changed over time. Before 1965, most of the diagnoses were made during autopsy and surgery. Since 1965, angiography was the most commonly used modality for diagnosis of ARCAPA. In 1985, the first case of ARCAPA was diagnosed with echocardiography.52 Since 1985, echocardiography has gained popularity in the diagnosis of ARCAPA. The diagnosis by echocardiography was described in 21 cases (21%) from this database. The common findings on echocardiography are an anomalous RCA, left ventricular dilatation, collaterals between the left and right coronary arteries and dilated coronary arteries.90 Angiography was used as a confirmatory method when the initial diagnosis made by echocardiography in many case reports. In two cases, diagnosis was made by multislice computed tomographic (CT) angiography. 88,89 Cardiovascular magnetic resonance (CMR) was used for the first time in 2007 in for the diagnoses of ARCAPA. 19 Surgical treatment is a definitive therapy that is recommended even for asymptomatic patients. 69,84 Standard therapy is to establish a double-ostium coronary system by translocation of the aberrant vessel to the aortic root, which decreases the risk of sudden cardiac death and also provides relief from coronary steal, which may be responsible for myocardial ischemia. 8,40,84 In addition, it can prevent further dilatation and enlargement of the coronary artery, which in turn reduces the size of the shunt. 86 Ligation of the RCA at its origin and coronary artery bypass grafting (CABG) with a saphenous vein graft is also described. In 2006, Yamamoto et al performed ligation of the RCA and CABG with the radial a artery. 19 Simple ligation of the coronary system is less traumatic and is the simplest alternative, but the risk of a single ostium coronary system still persists.46 From the literature review, 57 patients underwent surgical correction. Forty-seven (47%) of these patients with surgical correction underwent reimplantation and 10 (10%) of those with surgical correction had resorted to simple ligation of the aberrant coronary vessel. In some cases, reimplantation was tried initially but could not be performed due to the fragility of the aberrant vessel. As a result, the patients underwent ligation of the anomalous vessel.24 Only a few patients were treated medically, with no follow up available.9,52 If necessary, mitral valve repair should be provided simultaneously at the time of surgery.17,19 Follow up with ARCAPA after surgical treatment is not well described in the literature. According to the case reports, reimplantation of the anomalous vessel to aorta was the successful. However, persistent symptoms and myocardial ischemia on imaging studies were described in some patients after reimplantation.14,57,74 In 1 patient, there was a reduction in the size of the LAD after reimplantation of the anomalous RCA into the aorta.51 In another case, the patient developed a clot in the RCA 4 years after reimplantation. 76 CMR was used in 1 case for serial follow ups. 19 In our case, echocardiography performed on a regular basis showed improved cardiac function without mitral regurgitation.85 We recommend long-term follow up with the patients on a regular basis to evaluate their ventricular function and the patency of the reimplanted vessel. Conclusion. ARCAPA is a rare congenital coronary anomaly that can be diagnosed by careful echocardiographic evaluation or angiography. Myocardial ischemia, heart failure or sudden cardiac deaths have been observed. Surgical reimplantation is the treatment of choice and should be considered as soon as possible for this potentially fatal condition. Long-term follow up on a regular basis is required to assess ventricular function and patency of the reimplanted vessel.

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________________________________________________
From the University of Illinois at Chicago/Advocate Christ Medical Center, Oak Lawn, Illinois. The authors report no conflicts of interest regarding the content herein. Manuscript submitted August 14, 2009, provisional acceptance given September 25, 2009, final version accepted October 6, 2009. Address for correspondence: Harshit Modi, MD, 15613 Plumtree Drive, Orland Park, IL 60462. E-mail: modiharshit@hotmail.com


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