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Congenital coronary artery anomalies are rare, often an incidental finding in asymptomatic patients. They occur in 1% of all congenital heart disease. Coronary artery anomalies are classified in several patterns. -- with regard to the origin/course/termination/number/intrinsic anatomy Many cases are clinically in significant,only some cases are diagnosed when another surgical cardiac procedure is carried out. But in some cases it can lead to life-threatening complications like myocardial infarction/ arrhythmia/ sudden cardiac death early in life.
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Hypoplastic coronary artery disease (HCAD) was first reported in 1970. It is underdevelopment of one or more major branches of the coronary arteries characterized by a narrowed lumen or shorter course. Its incidence is 0.02% of the general population and 2.2% of all the congenital coronary artery anomalies. Etiology still unknown. However, it was postulated to result from various conditions, -- stenosis of the coronary artery orifice -- an aberrant course between the pulmonary artery and aorta. -- a coronary artery ostium in ectopic position --stenosis of the coronary ostium.
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The condition is mostly asymptomatic. However, some presents with chest pain and palpitations It bears a high risk of sudden cardiac death (SCD) as a result of ventricular arrhythmia during effort, consistent with a sudden and total occlusion of the artery. Mechanisms involved - coronary artery spasm reflecting abnormal vasodilator mechanisms and endothelial dysfunction leading to myocardial ischemia.
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VESSELSNORMAL DIAMETER (MM) RIGHT CORONARY ATRERY 2.71 - 5.6 LEFT CORONARY ARTERY 3.82 - 6.09 LEFT ANTERIOR DESCENDING ARTERY 1.46 - 5.28 LEFT CIRCUMFLEX ARTERY 1.5 - 5.27
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A 39 year old female patient came complaints of - Crushing type of chest pain lasting for 10 minutes, 3 episodes since 2007. Associated with palpitations and giddiness. K/C/O Hypertension for 7 years, under medication --Patient underwent TREAD MILL TEST in 2007 and was diagnosed with Inducible Ischemia and is under medication. No further investigations have been done. --Diabetes mellitus for two months. She has no significant family history
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GENERAL EXAMINATION : Afebrile. No pallor/icterus/clubbing/cyanosis/lymphadenopathy/edema. Blood pressure - 130/90 mm Hg. Pulse Rate - 82/min. SYSTEM EXAMINATION: CVS - S1, S2 +, no murmurs/thrill. RS/CNS - No abnormality detected. P/A - soft, no organomegaly.
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In our case,the patient underwent a Treadmill test in 2007 and was diagnosed with Induced ischemia. Electrocardiogram: T wave inversions in Leads III, V1-V5. Echocardiogram : Trivial tricuspid regurgitation Left ventricular function-normal No Regional wall motion abnormalities. Cardiac chambers - Normal.
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No significant abnormality seen.
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A) Normal ostium (yellow arrow) of left main coronary artery (LMCA) --Reduced caliber of Left main coronary artery -1.1mm (green arrow). --No plaque in the ostium or left main coronary artery. B) Proximal left anterior descending artery (LAD- red arrow). --Left circumflex artery (black arrow). --Diagonal branch of LAD (white arrow). --Ramus intermedia artery (blue arrow). --Tapering of mid and distal segments of LAD (violet arrow).
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Vieussen’s Ring (white arrow). Right main coronary artery (black arrow). Conus branch (blue arrow).
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Right main coronary artery and its branches were normal in course and caliber. Left main coronary artery: - Arising from the left main coronary sinus sharing a common ostium (1.6mm) with interventricular septal branch. -Left main coronary artery is reduced in its caliber (1.1mm) in its entire length(1.5cms) - It trifurcates in to Left anterior descending artery, Ramus Intermedius artery and Left circumflex artery.
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Left anterior descending artery : - Type III - Gives two diagonal branches and are normal in opacification. - Conus branch is seen arising from the right coronary sinus, separate from the right coronary artery ostium. - It measures 3mm in caliber and coursing right to left anterior to pulmonary conus and anastamosing with Left anterior descending artery distal to D1 branch - VIEUSSEN’S RING.
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Left anterior descending artery : -proximal to anastamosis - 3mm -Between D1 and D2 branches -1.5 mm -After D2 branch(small in caliber)-1mm Left circumflex artery - 1.6mm - Its bifurcates to two obtuse marginal branches and they measure 1.4 and 1.2 mm each. Ramus Intermedius artery: 2.2mm(normal in caliber)
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Normal appearing right coronary artery (yellow arrow) with conus branch not cannulated.
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Non selective injection into left coronary sinus : --Hypoplastic left main coronary artery (blue arrow). --Hypoplastic mid and distal left anterior descending artery (green arrow). --Normal caliber proximal left anterior descending artery (orange artery). -- Diagonal branch of LAD (yellow arrow).
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In the view of above symptoms, imaging findings and risk of sudden cardiac death the patient underwent below mentioned procedure. Coronary Artery Bypass Grafting: -- 2 grafts were placed : a) Left internal mammary artery for D1 branch (1.5mm). b) Saphenous vein graft for Obtuse marginal artery (1.5mm). -Left anterior descending artery was too small to graft. POST-OP period : Uneventful.
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Hypoplastic coronary artery disease (HCAD) is a very rare entity among the congenital coronary artery anomalies. This unusual clinical entity has rarely been diagnosed in living individuals. Most of them are asymptomatic and a high proportion experience sudden cardiac death. Diagnosis is often made at autopsy.
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1. Funabashi, N., Kobayashi, Y., Perlroth, M. and Rubin, G. Coronary Artery: Quantitative Evaluation of Normal Diameter Determined with Electron-Beam CT Compared with Cine Coronary Angiography— Initial Experience1. Radiology, (2003). 226(1), pp.263-271. 2. Ogden JA. Congenital anomalies of the coronary arteries. Am J Cardiol. 1970;25:474–9. 3. Roberts WC, Glick BN. Congenital hypoplasia of both right and left circumflex coronary arteries. Am J Cardiol 1992;70:121–3. 4. Zugibe FT, Zugibe FT Jr, Costello JT, et al. Hypoplastic coronary artery disease in the spectrum of sudden unexpected death in young and middle age adults. Am J Forensic Med Pathol 1993;14:276–83. 5. Go¨l MK, O¨ zatik MA, Kunt A, et al. Coronary anomalies in adult patients. Med Sci Monit 2002;8:CR636–41. 6. Casta A. Hypoplasia of the left coronary artery complicated by reversible ischemia in a newborn. Am Heart J 1987;114: 1238–41. 7. Angelini P, Velasco JA, Flamm S. Coronary anomalies: incidence, pathophysiology and clinical relevance. Circulation 2002;105:2449–54.
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