Kerala Heart Journal -thrudeep sagar

Case report

Kerala Heart J  2016; 6(2):xx-xx. 

Multiple Gaint Coronary Aneurysm and Atriovenous Fistulas  in TOF

Sagar Thrudeep (MD)*, George Geofi (MD,DM), Gopinath Rajesh (MD,DM), Saidumuhamad Abdulkhadar (MD,DM)

Corresponding author

Dr S Thrudeep, MD
Senior Resident (DM),
Department of Cardiology,
Amala Institute of Medical Sciences,
Amala Nagar, Thrissur-680 555, Kerala, India Email:


               An adult case of congenital cyanotic heart disease(tetrology of fallot) with incidentally detected  gaint coronary artery aneurysm. The case reported due to its rare association of multiple  gaint coronary artery aneurysm(CAA)and atrio-venous  fistula (AVF)  with tetrology of fallot(TOF) in adults.

Keywords: multiple gaint coronary artery aneurysm,tetrology of fallot



                                       CAA is defined as a localized dilatation exceeding the diameter of adjacent normal segment by 50% or by 1.5 times .3 CAAs diameter exceeds the reference vessel diameter by 4 times or  a  diameter more than 2 cm is termed as "giant CAA ". Congenital heart diseases are rare causes of CAA .2 Coronary artery aneurysm are rarely associated with TOF; is reported only in 3 cases.CAA are symptomatic with most common presentation being chest pain.

Case report:

                         She is a 40 yr old diagnosed case of CCHD TOF came to Department of Cardiology , Amala Institute of Medical science  with complaints of non specific symtoms. On examination she had clubbing,central cyanosis, single second sound, short systolic murmur of grade 3/6 close to lower left sternal border. Electrocardiography showed nonspecific ST-T changes with tall R wave in V1. During routine 2D echocardiography she was suspected to have coronary artery aneurysm(CAA) along with features suggestive of TOF,so after baseline investigation advised  CT coronary angiography at our hospital.

       CT coronary angiography was showing gross dilation and tourtousity of all the coronary vessels, aneurysmal dilation of LMCA(70.7mm) and proximal RCA(25.3mm), aneurysmal dilation with fistulous communication of terminal branches of LAD,LCX OM, RCA, acute marginal and PDA (fig1).  Our patient is on regular follow up since she was asymptomatic and with no features suggestive of obstructive coronary disease.

Discussion :

                   Coronary artery aneurysm(CAA) is defined as any coronary artery dilation more than the diameter of adjacent segment or the diameter of the largest coronary artery by 1.5 times.3 Giant CAA  refers to an aneurysm with a diameter larger than 20 mm.Incidence of CAA on angiography is  0.15% to 4.9%.2  Incidence of Giant CAA  only 0.02%.3 Single Gaint CAA have been reported.2 Only one case of multiple gaint CAA has been reported due to atherosclerosis.4 There are only 3 reported cases of Tetrology of Fallot(TOF)  with CAA and coronary AVF .1  Only one case of CAA with AVF  in a case of TOF reported previously . We are reporting first case of gaint coronary aneurysm with AVF  in a case of TOF. Atherosclerosis accounts for the vast majority of CAAs in adults, whereas Kawasaki disease is responsible for most cases in children.1 Aneurysmal  coronary arteries may need medical and surgical treatments whereas some of them  remain asymptomatic .5 The decision to treat CAA with AVF is recommended in the presence of symptoms,fistula location, its size, and the resultant shunt volume.5  Therefore, Mutiple Giant CAA is an uncommon lesion with varied clinical presentation. Surgical intervention is generally reserved for single, large, symptomatic lesions (with angina, cardiac decompression, or complications) with circulatory overload , so warrants surgical treatment even if  patients are asymptomatic.6



1. Ali Fedakar, MD e t al  Coronary Artery Aneurysm and Coronary Fistula in Tetrology of Fallot: A Case Presentation of A Young Adult Undergoing Total Correction 


2. Li D, Wu Q, Sun L, Song Y, Wang W, Pan S, et al. Surgical treatment of giant coronary artery aneurysm. J Thorac Cardiovasc Surg 2005;130(3):81721


3.Syed M, Lesch M. Coronary artery aneurysm: a review. Prog Cardiovasc Dis 1997;40(1):7784


4. Rammohan Marla, Rachel Ebel, Marcus Crosby, BS, and G. Hossein Almassi, Multiple Giant Coronary Artery Aneurysms Tex Heart Inst J. 2009; 36(3): 244246

5. A case of asymptomatic giant coronary aneurysm with atrioventricular fistula Hyun Ju Yoon (MD) et al Journal of Cardiology Cases (2010) 2, e71e73

6. Saxena P, Konstantinov IE, Burstow D, Tam R. Surgical repair of a large coronary artery aneurysm with arteriovenous fistula. J Thorac Cardiovasc Surg 2006;131:11678.




Authors are grateful to the valuable help of Dr. Rupeesh G (MD, DM), Additional Professor,

Dr. T.G.Jayakumar (MD, DM), Additional Professor, Dr  Gagan S, Department of Cardiology Amala Institute of Medical Sciences, Amala Nagar, Thrissur, Kerala, India


  • There are currently no refbacks.