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Case Report Adult Type Anomalous Left Coronary Artery from Pulmonary Artery GP Parale*, SS Pawar** Abstract Anomalous origin of Left Coronary Artery from Pulmonary Artery (ALCAPA) is a rare congenital anomaly. Its survival into adulthood is further rare. Clinical manifestations result from evolving morphological - functional alterations in pulmonary circulation that occur after the birth. We report a case of 43 year old adult patient with effort angina and without any ECG or Echo abnormalities. On coronary angiography, typical anatomy of ALCAPA was revealed. © At present our patient is awaiting surgery. INTRODUCTION A nomalous origin of left coronary artery from pulmonary artery (ALCAPA) is a rare congenital defect with reported incidence of 1 in 3 lac live births accounting for 0.25% of congenital heart diseases1 and survival to adulthood is furthermore rare accounting for only 10-15% of these cases.2 Here, we report a case of adult survivor of ALCAPA diagnosed at our institution. CASE REPORT A 43 year old male hypertensive since two years and known chronic smoker was evaluated for history of class II effort angina over previous two months. Physical examination was within normal limits except for blood pressure, which was 156/108 mm of Hg in right upper arm in supine position. X-ray chest was not remarkable. The ECG showed ST/T changes in leads I, avL and V6 suggesting lateral ischaemia. 2-dimensional echocardiography revealed trivial mitral regurgitation (MR) and concentric left ventricular hypertrophy (LVH) with preserved left ventricular systolic and diastolic function. There was no regional wall motion abnormality. [Echocardiography was done before as well as after the angiography with prior knowledge of coronary artery anatomy. But it did not given any clue of ALCAPA, mainly because echo window did not permit visualisation of left coronary artery]. With this background, patient was taken for coronary angiography which was done in multiple views. This revealed typical anatomy of ALCAPA. *Chief Cardiologist; **Research Assistant, Ashwini Co-operative Hospital and Research Centre. Received : 17.12.2004; Revised : 3.1.2006; Re-revised : 4.2.2006; Accepted : 27.3.2006 © JAPI • VOL. 54 • MAY 2006 DISCUSSION ALCAPA or Blannd-Garland-White syndrome is a rare congenital anomaly with incidence of 1 in 3 lac live births, accounting for 0.25% of congenital heart disease. The clinical expression of syndrome results from evolving morphological-functional alterations in pulmonary circulation that occur after birth.1 Soon after birth, resistance of the pulmonary circulation is so high permitting antegrade flow from the pulmonary artery (PA) to left coronary artery (LCA), which perfuses the left ventricle. Therefore, occurrence of sudden death is extremely rare in this age group. As pulmonary vascular Fig. 1a : Right coronary angiography in RAO position showing filling of left coronary system through collaterals. www.japi.org 397 Fig. 1b : Right coronary angiography in delayed phase showing filling of pulmonary artery from left coronary artery confirming diagnosis ALCAPA. resistance falls in following weeks flow from PA to LCA stops and left ventricular perfusion totally depends upon collaterals to LCA developed from right coronary artery (RCA). Death ensues if collaterals are poorly developed while on the other hand if collaterals enlarge after an initial period of decompensation, improvement and survival into adulthood occurs - so called adult type of ALCAPA. In patients who survive into adulthood, pulmonary circulation acts as low resistance siphon and collaterals flow uses LCA as only conduit into pulmonary circulation thus bypassing the left ventricular myocardium. This coronary steal may cause overt ischaemia as well as left ventricular diastolic overload from left to right shunting. Our patient was nearly asymtomatic till date and living normal life, including normal professional life. The presenting symptoms of retrosternal pain and fatigue on strenuous exercise probably results from myocardial ischemia due to failure in collateral circulation associated with concentric left ventricular hypertrophy. On electrocardiography signs of infarction are absent in adults,3 which is consistent with our finding as electrocardiograph showed only ST/T wave changes in leads I, avL and V6. Two dimensional (2-D) echocardiography is considered major support examination for diagnosing ALCAPA. This examination however has its limitations.4 Diagnosis can sometimes be achieved on the basis of echocardiography alone if it reveals a grossly enlarged RCA and a dilated left ventricle with global hypokinesia. Pulsed and color-flow Doppler can demonstrate reversal 398 of flow from the ALCAPA into the PA, which constitutes a left-to-right shunt. In older patients, abundant highvelocity flow may be seen in the interventricular septum, as a result of intercoronary collaterals. Two-dimensional echocardigraphy can equally visualize the actual anatomical origin of the ALCAPA and assess the degree of mitral insufficiency.5 In the present case, 2-D echo examination revealed trivial mitral regurgitation with left ventricular hypertrophy with preserved systolic and diastolic function and there was no regional wall abnormality. Coronary arteries could not be visualised properly. Coronary angiography which is considered as the most accurate diagnostic technique was performed since echocardiography could not reveal the abnormality. Three angiographic criteria are established for diagnosing ALCAPA.4 They are as follows: 1. Retrograde filling of LCA. 2. Connection of LCA with PA. 3. Absence of LCA originating from aorta. Since all the three criteria are fulfilled, diagnosis of ALCAPA is confirmed. The adult form of ALCAPA may occur in children and necessarily occurs in adults in approximately 10-15% of cases. It is characterised by exuberance in collateral coronary circulation, which allows survival until adulthood, with case being reported at the age of 72 years.6 Wesselhoeft et al.2 classified the clinical spectrum of ALCAPA as follows: 1. Infantile Syndrome : This is the most common form. Patient develops acute episode of respiratory insufficiency, cyanosis, irritability and profuse sweating. Most of them die within two years. 2. Mitral Regurgitation : It is characterised by mitral regurgitation murmur, congestive heart failure, cardiomegaly and atrial arrythmias in children, adolescent and adults. 3. Syndrome of Continuous Murmur : This occurs in asymptomatic patients with angina pectoris. A continuous murmur results from great volume of blood flowing through collateral branches between right and left coronary arteries. 4. Sudden Death in Adolescents or Adults : Most of the patients are asymptomatic, but some may experience angina on exertion, cardiac arrhythmias and sudden death. Once the diagnosis of ALCAPA is established, early surgical repair for correction of defect and prevention of complications and sequelae inherent in natural history of disease is mandatory. www.japi.org © JAPI • VOL. 54 • MAY 2006 TA, eds. Heart diseases in Infants, Children and Adolescents, 4th ed. Baltimore : Williams and Wilkins, 1989;627-35. REFERENCES 1. Celso K Takimura, Allyson Nakamoto, Viviane Hotta. Anomalous origin of left coronary artery from pulmonary artery. Report of an adult case. Arq Bras Cardiol 2002;78: 312-14. 2. Wesselhoft H, Fawcet JS, Johnson AL. Anomalous origin of left coronary artery from pulmonary trunk: its clinical spectrum, its pathology and pathophysiology based on a review of 140 cases with seven further cases. Circulation 1968;38:403-25. 3. Takashi M, Lurie PR. Abnormalities and diseases of coronary vessels. In: Adams FH, Emmanouilides GC, Reimenschneider 4. Culham JAG. Abnormalities of coronary arteries. In: Freedom RM, Mawson JB, Yoo SJ, Benson LN, eds. Congenital Heart Disease. Textbook of Angiocardiography. Armonk, NY : Futura Publishing Company Inc., 1997;862-66. 5. Jureidini SB, Nouri S, Pennington DG. Anomalous origin of the left coronary artery from the pulmonary trunk: repair after diagnostic cross-sectional echocardiography. Br Heart J 1987;58:173-75. 6. Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Management of Book Review Hematology Today 2006 Edited By Prof MB Agarwal This is a state of the art masterpiece by a clinician par excellence. It is a world-class compendium of some latest articles in hemato-oncology, which will useful not only to hematologist, pathologist but also to general physcians. It is a multiauthor (74 to be precise) textbook with blend of academia and Practioners as well as international authorities across the globe. Each chapter has a succinct abstract, selected reading, illustration in colour and “Carry Home Message”. The sections are Anemia, Haemostatic and Thrombotic Disorders, Acute Leukemia and MDS, Chronic Leukemia, Lymphoma, Plasma cell dyscrasia, Transfusion medicine and Transplantation, Support Therapy, Laboratory Science, etc. The whole book is hardbound and printed on quality art paper with colour illustrations. This is a must for every physician’s library. Published By Ashirwad Hematology Center Vijay Sadan, Ground Floor, 18-B, Dr. B. Ambedkar Road, Dadar T.T., Mumbai, India (On behalf of the XIIth National CME in Haematology and Haemato-Oncology, Bombay Hospital Institute of Medical Sciences, Marine Lines, Mumbai – 400 020, India) Tel: 91-22-24142272/24144453 Fax: 91-22-24140048 Email: [email protected] Website: www.hemato-onco.com Price Rs 800/Hon Editor Announcement API, Rewa Chapter is organizing 16th MP APICON on 15th October 2006. All members of API and PostGraduate Students of S.S. Medical College, Rewa are cordially invited to attend the conference. For registration details and other details : Dr. MK Jain, Chairman, MP APICON, Rewa Tel: 07662-254498, 403342, Cell: 9425424555 Dr. Anand Singh, Organizing Secretary, MP, APICON, Rewa Tel: 07662-406880, 407579, Cell: 9300644166 Conference Secretariat: Dept. of Medicine, S.S.M.C. and S.G.M.H, Rewa (M.P.) Tel: 07662-254606, Email: [email protected]; Fax: 07662-251167 © JAPI • VOL. 54 • MAY 2006 www.japi.org 399