Biküspit Aort Kapak ve Sağ Koroner Sinüsten Köken Alan İnterarteriyel Seyirli Sol Ön İnen Arter Anomalisi: Nadir Bir Olgu
Özet
Biküspit aort kapak (BAK), erişkinlerde sık görülen konjenital kardiyak anomalilerden biri olup aortopati ile yakından ilişkilidir. Koroner arterin anormal aortik kökeni ise nadir görülmekle birlikte, özellikle interarteriyel seyir gösteren sol koroner sistem anomalileri ani kardiyak ölüm riski açısından önem taşımaktadır. Bu vakamızda 34 yaşında erkek hasta kardiyoloji polikliniğine nefes darlığı ve göğüs ağrısı şikayetleriyle başvurdu. Transtorasik ekokardiyografide ejeksiyon fraksiyonu %60 olarak ölçüldü, simetrik füzyon paternli biküspit aort kapak izlendi ve asendan aort çapı 42 mm bulundu. Koroner arter hastalığını dışlamak amacıyla yapılan koroner bilgisayarlı tomografi anjiyografide aterosklerotik koroner arter hastalığı saptanmadı; ancak sol ön inen arterin sağ koroner sinüsten köken aldığı, aorta ile pulmoner arter arasında interarteriyel seyir gösterdiği ve orijin düzeyinde kalibre azalması bulunduğu görüldü. Fonksiyonel değerlendirme amacıyla yapılan egzersiz testinde hasta 11.90 METS düzeyine ulaştı ve objektif iskemi ya da ciddi aritmi izlenmedi. Olgu kardiyoloji ve kalp damar cerrahisi ekipleri tarafından multidisipliner olarak değerlendirilerek yakın klinik takip ve konservatif izlem kararı verildi.
Bicuspid aortic valve is one of the most common congenital cardiac anomalies and is frequently associated with aortopathy. Anomalous aortic origin of a coronary artery is rare; however, interarterial anomalies involving the left coronary system may be clinically important because of the potential risk of myocardial ischemia and sudden cardiac death. We present a 34-year-old man who was admitted with dyspnea and chest pain. Transthoracic echocardiography showed a bicuspid aortic valve with symmetric fusion, preserved left ventricular systolic function, and an ascending aortic diameter of 42 mm. Coronary computed tomography angiography, performed to exclude coronary artery disease, demonstrated that the left anterior descending artery originated from the right coronary sinus and followed an interarterial course between the aorta and pulmonary artery, with caliber narrowing at its origin. Exercise testing showed good functional capacity (11.90 METS) without objective ischemia or significant arrhythmia. Following multidisciplinary evaluation, conservative follow-up was selected. This case highlights the importance of individualized management based on anatomical risk features, clinical presentation, functional evaluation, and multidisciplinary decision-making.
Referanslar
Verma S, Siu SC. Aortic dilatation in patients with bicuspid aortic valve. N Engl J Med. 2014;370(20):1920-1929.
Michelena HI, Della Corte A, Evangelista A, Maleszewski JJ, Edwards WD, Roman MJ, et al. International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional, and research purposes. J Thorac Cardiovasc Surg. 2021;162(3):781-797.
Cheezum MK, Liberthson RR, Shah NR, Villines TC, O'Gara PT, Landzberg MJ, et al. Anomalous aortic origin of a coronary artery from the inappropriate sinus of Valsalva. J Am Coll Cardiol. 2017;69(12):1592-1608.
Gaudino M, Mery CM, Bakaeen FG, Choi JW, Kurlansky P, Krieger EV, et al. Management of adults with anomalous aortic origin of the coronary artery. J Am Coll Cardiol. 2023;82(21):2034-2054.
Baumgartner H, De Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, et al. 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2021;42(6):563-645.
Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, et al. 2018 AHA/ACC Guideline for the management of adults with congenital heart disease. Circulation. 2019;139(14).
Brothers JA, Frommelt MA, Jaquiss RDB, Myerburg RJ, Fraser CD Jr, Tweddell JS. Expert consensus guidelines: anomalous aortic origin of a coronary artery. J Thorac Cardiovasc Surg. 2017;153(6):1440-1457.
Vallabhajosyula S, Fuchs M, Yang LT, Medina Inojosa J, Tajouri TH, Enriquez-Sarano M, et al. Anomalous coronary artery origin from the opposite sinus in patients with bicuspid aortic valve: comparison with tricuspid aortic valve. Open Heart. 2021;8(1):e001567.
Molossi S, Doan T, Sachdeva S. Anomalous coronary arteries: a state-of-the-art approach. Cardiol Clin. 2023;41(1):51-69.
Doan TT, Wilkes JK, Reaves O'Neal DL, et al. Clinical presentation and medium-term outcomes of children with anomalous aortic origin of the left coronary artery: high-risk features beyond interarterial course. Circ Cardiovasc Interv. 2023;16(5):e012635.
Jegatheeswaran A, Devlin PJ, McCrindle BW, et al. Features associated with myocardial ischemia in anomalous aortic origin of a coronary artery: a Congenital Heart Surgeons' Society study. J Thorac Cardiovasc Surg. 2019;158(3):822-834.e3.
Qasim A, Doan TT, Dan Pham T, et al. Is exercise stress testing useful for risk stratification in anomalous aortic origin of a coronary artery? Semin Thorac Cardiovasc Surg. 2023;35(4):759-768.
Mery CM, De León LE, Molossi S, Sexson-Tejtel SK, Agrawal H, Krishnamurthy R, et al. Outcomes of surgical intervention for anomalous aortic origin of a coronary artery: a large contemporary prospective cohort study. J Thorac Cardiovasc Surg. 2018;155(1):305-319.
Padalino MA, Franchetti N, Sarris GE, Hazekamp M, Carrel T, Frigiola A, et al. Anomalous aortic origin of coronary arteries: early results on clinical management from an international multicenter study. Int J Cardiol. 2019;291:189-193.
Chua F, Vongbunyong K, Urgun DA, et al. Anomalous origin of left main coronary artery from the right sinus of Valsalva. BMC Cardiovasc Disord. 2023;23:618.
