Anomalous Right Coronary Artery Arising from Left Coronary Sinus in Two Brothers - PDF Document

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  1. Anomalous Coronary Artery in Brothers Case Report Acta Cardiol Sin 2011;27:124?7 Anomalous Right Coronary Artery Arising from Left Coronary Sinus in Two Brothers Hung-Yu Chang and Wei-Hsian Yin Wereporttwobrothersaged61and69respectivelywithabnormalrightcoronaryartery(RCA)originatingfromthe left sinus of Valsalva. Both of them presented with exertional dyspnea and chest tightness in their seventh decade. Another younger brother of theirs suffered from sudden cardiac death at the age of 60. Other symptomatic family members were screened by multi-slice computed tomography (MSCT), but no coronary artery anomalies were found. Considering different risk factors of these two brothers, one patient underwent coronary artery bypass surgery, while another took medical therapy. Previously reported familial cases of anomalous coronary arteries are very rare. Key Words: Anomalous coronaries ? Computed tomography ? Familial clustering INTRODUCTION CASE REPORT Coronary artery anomalies are reported in 1.3% of patients undergoing coronary angiography,1and may be associated with sudden death, myocardial ischemia, ar- rhythmia and syncope. Some anatomic presentations of coronary anomalies are considered to be high-risk group.2,3However, many patients were asymptomatic before their presentation of sudden cardiac death,4indi- cating that early detection of potential lethal cases is difficult. Familial cases of anomalous coronary arteries have been very rare. In this case report, we report two brothers with abnormal right coronary artery (RCA) origin from the left coronary cuspid with identical courses of RCA, and we suggest clinicians pay atten- tion to the possibility of familial clustering of coronary anomalies. A 61-year-old man (patient 1) presented to our insti- tution because of exertional dyspnea and chest distress. He had hypercholesterolemia and was a casual smoker. He received treadmill exercise test, which revealed ST segment depression of 1 mm at leads V4-V6during the stage III exercise. Exercise thallium-201 myocardial per- fusion scan with dipyridamole also demonstrated re- versible perfusion defect in the proximal inferior walls of the left ventricle. Cardiac catheterization showed ab- errant origin of the right coronary artery (RCA) from the left coronary cuspid. There were also mild atherosc- lerotic changes in the coronary arteries (Figure 1A). Multi-slice computed tomography (MSCT) proved the diagnosis of anomalous RCA arising from the left sinus of Valsalva and taking an interarterial course between the aorta and pulmonary artery (Figures 1B&C). The pa- tient received anti-atherosclerotic medication and his condition remained stable during 3 years of follow-up. A 69-year-old brother (patient 2) of patient 1 was admitted to our hospital 2 years after his brother’s coro- nary examination. Another brother of the two had died suddenly 3 months previous to this hospitalization at his age of 60, but the diagnosis was uncertain. Patient 2 suf- Received: September 22, 2010 Division of Cardiology, Cheng-Hsin General Hospital, Taipei, Taiwan. Address correspondence and reprint requests to: Dr. Wei-Hsian Yin, Cheng Hsin General Hospital, No. 45, Cheng-Hsin Street, Pei-Tou, Taipei 112, Taiwan. Tel: 886-2-2826-4400; Fax: 886-2-2826-1242; E-mail: yin.wh@msa.hinet.net Accepted: December 30, 2010 124 Acta Cardiol Sin 2011;27:124?7

  2. Anomalous Coronary Artery in Brothers fered increasing shortness of breath and dyspnea on exertion for months. His past medical history were sig- nificant for hypertension and dyslipidemia. Treadmill exercise test showed marked 2 mm ST segment depres- sion at inferior leads and leads V4-V6during the stage III exercise and recovery phases. Exercise thallium-201 myocardial perfusion scan with dipyridamole demon- strated significant myocardial ischemia involving the left ventricular anteroseptal wall and apex. Cardiac cathe- terization showed total occlusion at the proximal portion of the left anterior descending artery (LAD) and tortuous atherosclerotic left circumflex artery. The RCA arose from the left coronary cuspid, and a narrow orifice was suspected (Figure 1D). MSCT revealed abnormal orifice of the RCA from the left coronary sinus, with inter- arterial course and narrowed ostium (Figures 1E&F). Coronary artery bypass surgery was performed and di- rect compression of the RCA proximal portion by the pulmonary artery was found during the operation. There- fore, the LAD and RCA were revascularized. The patient was in stable status during 6 months of follow-up. Other symptomatic family members were screened by MSCT, but no coronary artery anomalies were found. DISCUSSION Coronary artery anomalies were reported in 1.3% of patients undergoing coronary angiography by Yamanaka.1 Important clinical problems of coronary anomaly are sudden cardiac death, myocardial ischemia, arrhythmia and syncope. Sudden death mostly happens before 35 A B C D F E Figure 1. (A) Coronary angiography in left anterior oblique projection reveals aberrant origin of RCA (arrow) from left coronary cuspid in patient 1. (B) Reconstructed contrast-enhanced computed tomography shows anomalous RCA (arrow) arising from the left sinus of Valsalva and taking an interarterial course between aorta and pulmonary artery in patient 1. (C) Reconstructed computed tomography of patient 1. Anomalous RCA is marked by arrow. (D) Coronary angiography in left anterior oblique projection reveals aberrant origin of RCA (arrow) from left coronary cuspid in patient 2. A narrow orifice of RCA was suspected. (E) Reconstructed contrast-enhanced computed tomography shows anomalous RCA (arrow) arising by the side of left main coronary artery and taking an interarterial course in patient 2. Total occlusion of LAD is also noted (arrowhead). (F) Reconstructed computed tomography of patient 2. Note the narrowed RCA ostium (arrow) and total occlusion of LAD (arrowhead). Ao, aorta; PA, pulmonary artery; LMCA, left main coronary artery. 125 Acta Cardiol Sin 2011;27:124?7

  3. Hung-Yu Chang et al. years of age. Increased risk of sudden cardiac death with this anomaly has been associated with four risk factors: interarterial course between the aorta and pulmonary ar- tery; slit-like coronary orifice; acute angle of take-off of the anomalous coronary artery from the aorta; and the presence of aortic intramural coronary arteries.2Symp- toms premonitory to a fatal event, such as exertional syncope, chest pain or palpitations, are common in pa- tients at risk, so surgical correction is indicated in symp- tomatic patients at any age. However, only about 20% of fatal anomalous coronary artery cases had prodromic symptoms. RCA arising from the left coronary sinus is more common than left coronary artery (LCA) from the right sinus. In Yamanaka’s report, RCA arising from the left coronary cuspid was noted in 0.17% of coronary angiographies, while LCA from the right coronary cuspid was seen in 0.047%. Data from Garg reported that 15 out of 4100 patients (0.37%) had abnormal ori- fice of RCA from the left sinus of Valsalva, but only 1 patient (0.02%) had LCA from the right sinus.5How- ever, the clinical course of LCA from the right coronary sinus is more malignant than that of RCA from left coro- nary sinus. Basso reported 27 sudden deaths in young athletes. Of these, 23 had LCA arising from the right coronary sinus and only 4 had RCA from the left coro- nary sinus.4Thus, surgical repair is recommended in pa- tients with anomalous LCA.6Nevertheless, treatment in patients with anomalous RCA is still controversial be- cause most conditions of this disease are benign. MSCT can be used to evaluate the orifice and the course of anomalous coronary artery, which can guide the treat- ment policy. Familial clustering of anomalous coronary arteries was only described in five previous reports.7-11De- vanagondi reported one 10-year-old boy with aberrant origin of LCA from the right sinus of Valsalva present- ing with sudden cardiac death, while his 8-year-old brother, who had aberrant RCA from the left coronary si- nus with interarterial course and slit-like orifice, was asymptomatic. Un-roofing operation was performed for this 8-year-old boy, with good short-term outcome.7 Laureti demonstrated two brothers with anomalous LCA from the right coronary sinus. The junior was found to have interarterial course and was treated by reimplan- tation of LCA, while the senior with retroaortic course was treated medically.8Horan described a father with ab- normal origin of RCA from the circumflex artery and his daughter with abnormal LCA arising from the RCA.9In a study of combined coronary and perfusion cardiovas- cular magnetic resonance imaging, Bunce demonstrated two sisters having anomalous coronary arteries. One had abnormal RCA arising from the left coronary cuspid, and the other sister had single coronary artery arising from the right sinus of Valsalva.10Rowe reported anomalous origin of the left circumflex artery in a father and his son and daughter.11In these previous reports, only one mor- tality was noted.7A brother of our index cases suffered sudden cardiac death, but no autopsy was done, so the definitive diagnosis was undetermined. Comparing to other reports, our patients also had atherosclerotic coro- nary disease in addition to coronary anomalies, making the cause of clinical symptoms uncertain. Patient 2 had several risk factors: severe atherosclerotic coronary ar- tery disease, narrow RCA orifice, interarterial course of RCA, and suspected family history of sudden death. Sur- gical intervention thus is reasonable for him. The treat- ment of patient 1 is relatively questionable. Since most anomalous RCA with interarterial courses are benign, medical treatment was decided. According to our cases and other previous reports, we believe that familial clustering of coronary anomalies does exist. Recent study demonstrated that gene Tbx1 defect is associated with anomalous coronary artery in mice, though definitive gene of humans is unknown yet. In this case report, we suggest that if family members of a patient with coronary anomalies present with exer- tional syncope or chest pain, the possibility of familial clustering of anomalous coronaries must still be kept in mind. Coronary angiogram or MSCT can be arranged in symptomatic family members with positive stress test. REFERENCES 1. Yamanaka O, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990;21:28-40. 2. LorenzEC,MookadamF,MookadamM,etal.Asystematicover- view of anomalous coronary anatomy and an examination of the association with sudden cardiac death. Rev Cardiovasc Med 2006;l7:4:205-13. 3. Chen IC, Chao TH, Tsai LM. Simultaneous anterior and inferior wall myocardial infarction in a patient with unusual coronary 126 Acta Cardiol Sin 2011;27:124?7

  4. Anomalous Coronary Artery in Brothers anatomy. Acta Cardiol Sin 2010;26:119-22. 4. Basso C, Maron BJ, Corrado D, Thiene G. clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive ath- letes. J Am Coll Cardiol 2000;35:1493-501. 5. Garg N, Tewari S, Kapoor A, et al. Primary congenital anomalies of the coronary arteries: a coronary arteriographic study. Int J Cardiol 2000;74:39-46. 6. Gersony WM. Management of anomalous coronary artery from the contralateral coronary sinus. J Am Coll Cardiol 2007;50: 2083-4. 7. Devanagondi R, Brenner J, Vricella L, Ravekes W. A tale of two brothers: anomalous coronary arteries in two siblings. Pediatr Cardiol 2008;29:816-9. 8. Laureti JM, Singh K, Blankenship J. Anomalous coronary ar- teries: a familial clustering. Clin Cardiol 2005;28:488-90. 9. Horan PG, Murtagh G, McKeown PP. Single coronary artery: a familial clustering. Heart 2003;89:e27. 10. Bunce NH, Rahman SL, Keegan J, et al. Anomalous coronary arteries: anatomic and functional assessment by coronary and perfusion cardiovascular magnetic resonance in three sisters. J Cardiovasc Magn Reson 2001;3:361-9. 11. Rowe L, Carmody TJ, Ashkenazi J. Anomalous origin of the left circumflex coronary artery from the right aortic sinus: a familial clustering. Cathet Cardiovasc Diagn 1993;29:277-8. 127 Acta Cardiol Sin 2011;27:124?7