Anomalous origin of left main coronary artery from right sinus of valsalva in addition to bicuspid aortic valve

Abstract

Bicuspid aortic valve is one of the most frequent heart abnormalities with an incidence of 1 to 2% [[1]Chadha S. Husain S.I. Shetty V. Hollander G. Frankel R. Shani J. Anomalous origin of left main coronary artery from right sinus of valsalva.Image Cardiovasc. Med. 2014; 41: 558-559Google Scholar]. Coronary artery anomalies are rare, with an estimated prevalence of around 5% [[2]Bonachea E.M. Zender G. White P. Corsmeier D. Newsom D. Fitzgerald-Butt S. Garg V. McBride K.L. Use of a targeted, combinatorial next-generation sequencing approach for the study of bicuspid aortic valve.BMC Med. Genom. 2014; 7: 56Crossref PubMed Scopus (42) Google Scholar]. Anomalous origin of a coronary artery has the potential clinical repercussions, including chest pain and the risk of sudden cardiac death. The origin of the left main coronary artery form the right sinus of valsalva is extremely rare with an approximate prevalence of 0.15% [[2]Bonachea E.M. Zender G. White P. Corsmeier D. Newsom D. Fitzgerald-Butt S. Garg V. McBride K.L. Use of a targeted, combinatorial next-generation sequencing approach for the study of bicuspid aortic valve.BMC Med. Genom. 2014; 7: 56Crossref PubMed Scopus (42) Google Scholar]. An 48-year old female patient was admitted to the hospital because of chest pain. Her blood pressure was elevated with 165/105 mm Hg. Her ECG was normal, an echocardiographic examination revealed normal left ventricular function with slight apical hypokinesia. Coronary angiography showed at first typical signs of bicuspid aortic valve (Fig. 1) and anomalous origin of left main coronary artery at an acute angle form right sinus of valsalva. No external compression of the left main coronary artery between the aortic root and the pulmonary trunk could be seen. Dominant right coronary artery and small left coronary artery were without any obstructions. With a 5F JR-4 diagnostic catheter the injection of the left stem (Fig. 2) and simultaneous injection of the right and left coronary arteries was performed (Fig. 3).Fig. 2Simultaneous injection in the right and left coronary arteries.View Large Image Figure ViewerDownload Hi-res image Download (PPT)Fig. 3Typical presentation of bicuspid aortic valve.View Large Image Figure ViewerDownload Hi-res image Download (PPT) The rest of hospitalization was uneventful, and the patient was discharged in stable condition with a normal blood pressure at blood pressure measurement over 24 h. Additionally, 100 mg aspirin was given to antihypertensive medication. For all we know, this is the first description of bicuspid aortic valve and anomalous origin of the left main coronary artery from the right sinus of valsalva with chest pain in elevated blood pressure [[3]Topaz O. DeMarchena E.J. Perin E. Sommer L.S. Mallon S.M. Chahine R.A. Anomalous coronary arteries: angiographic findings in 80 patients.Int. J. Cardiol. 1992; 34: 129-138Abstract Full Text PDF PubMed Scopus (212) Google Scholar]. The combination of these two anomalies was only reported in Syrian hamsters [[4]Durán A.C. Arqué J.M. Fernández B. Fernández M.C. Fernández-Gallego T. Sans-Coma V. Separate origin of the main components of the left coronary artery in Syrian hamsters (Mesocricetus auratus).J. Vet. Med. A Physiol. Pathol. Clin. Med. 2007; 54: 297-301Crossref PubMed Scopus (9) Google Scholar]. As no coronary obstructions could be seen an intervention was not necessary, but the risk of sudden cardiac death exists.