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Images in Cardiovascular Medicine Serpentine Coronary Arteries in a Patient with Apical Hypertrophic Cardiomyopathy Prashanth Panduranga, MD, MRCP Abdulla Amour Riyami, FRCP, PhD A 54-year-old woman with a history of hypertension, hypothyroidism, and apical hypertrophic nonobstructive cardiomyopathy was referred to our hospital for coronary angiography. She had recurrent exercise-induced chest pain and shortness of breath, which resolved with rest and the taking of nitrates. Results of a myocardial perfusion scan were normal. Coronary and left ventricular (LV) angiography showed extreme tortuosity of the left anterior descending coronary artery, the left circumflex coronary artery, and 1st obtuse marginal branch, with coiling of the distal segments (Fig. 1). There was no fixed coronary stenosis. Left ventriculography revealed severe hypertrophic cardiomyopathy with a spade deformity of the LV at A Section Editor: Raymond F. Stainback, MD, Department of Adult Cardiology, Texas Heart Institute at St. Luke’s Episcopal Hospital, 6624 Fannin St., Suite 2480, Houston, TX 77030 B From: Department of Cardiology, Royal Hospital, Muscat 111, Oman Address for reprints: Prashanth Panduranga, MD, MRCP, Department of Cardiology, Royal Hospital, Post Box 1331, Muscat 111, Oman E-mail: [email protected] © 2011 by the Texas Heart ® Institute, Houston 594 Serpentine Coronary Arteries Fig. 1 A) Coronary angiogram shows extreme tortuosity of the proximal and mid segments of the left anterior descending and left circumflex coronary arteries, without fixed coronary stenosis, in a patient with apical hypertrophic cardiomyopathy. B) End-diastolic frame from a left ventriculogram shows markedly tortuous distal segments of the left anterior descending and left circumflex coronary arteries. Click here forimage real-time Real-time motion of Figure 1A is available www.texasheart.org/journal. motionatimage: Fig. 1A. Volume 38, Number 5, 2011 A end-diastole, apical systolic obliteration of the LV cavity, and serpentine coronary arteries (Fig. 2). Comment B Severe coronary tortuosity, defined as 2 consecutive 180° turns by visual estimation in a major epicardial artery, was found in 152 of 1,221 patients in 1 study.1 The cause of coronary tortuosity is not clear. Traction and pressure in the lumen are known to lengthen a vessel, and these forces are opposed by a retractive force that results in a stable length of the vessel.2 The retractive force is generated by elastin. Elastin degeneration in the arterial wall leads to arterial tortuosity 2 that is seen with age and in hypertension, aneurysms, ectasias, and congenital arterial kinking. In a study of patients with hypertension and aortic regurgitation, tortuosity was more common in women and was more pronounced in patients with chronic pressure than in those with volume overload. The determinants of coronary tortuosity were sex, age, LV volume, and muscle mass.3 Our patient had 2 significant conditions that caused increased muscle mass. Coronary tortuosity might lead to flow alteration that reduces coronary pressure distal to the tortuous arterial segment, causing ischemia.4 It is postulated that the sharper and more numerous the coronary bends, the greater the energy loss and subsequent pressure loss.4 In 1 study, severe coronary tortuosity was associated with a lower incidence of coronary artery disease, and coronary artery disease risk factors were not predictors of severe coronary tortuosity.1 To our knowledge, this is the 1st report of extreme tortuosity of the coronary arteries in association with hypertrophic cardiomyopathy. References Fig. 2 Left ventriculography shows A) severe hypertrophic cardiomyopathy with a spade deformity of the left ventricle at enddiastole and B) apical systolic obliteration of the left ventricular cavity along with tortuous, serpentine coronary arteries. Real-time motion image is available at www.texasheart.org/ Click here for real-time motion image: Fig. 2B. journal. Texas Heart Institute Journal 1. Groves SS, Jain AC, Warden BE, Gharib W, Beto RJ 2nd. Severe coronary tortuosity and the relationship to significant coronary artery disease. W V Med J 2009;105(4):14-7. 2. Dobrin PB, Schwarcz TH, Baker WH. Mechanisms of arterial and aneurysmal tortuosity. Surgery 1988;104(3):568-71. 3. Jakob M, Spasojevic D, Krogmann ON, Wiher H, Hug R, Hess OM. Tortuosity of coronary arteries in chronic pressure and volume overload. Cathet Cardiovasc Diagn 1996;38(1): 25-31. 4. Zegers ES, Meursing BT, Zegers EB, Oude Ophuis AJ. Coronary tortuosity: a long and winding road. Neth Heart J 2007; 15(5):191-5. Serpentine Coronary Arteries 595