© 2013, Wiley Periodicals, Inc. DOI: 10.1111/echo.12496
Echocardiography
A Coronary–Pulmonary Artery Fistula with Giant Aneurysm in a Child with Ventricular Septal Defect with Pulmonary Atresia Jayaranganath Mahimaiha, D.M., Soumya Patra, M.D., Usha Mandikal Kodanda Rama Sastry, M.D., Anand Subramaniam, D.M., Navin Agrawal, D.M., and Manjunath Cholenahally Nanjappa, D.M. Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences & Research, Bangalore, India
(Echocardiography 2014;31:E130–E131) Key words: coronary–pulmonary artery fistula, giant aneurysm, pulmonary atresia, ventricular septal defect, echocardiography A 6-year-old girl born of a consanguinous marriage, with an uneventful birth history presented with cyanosis, clubbing, and effort intolerance. Her physical examination revealed cyanosis (oxygen saturation 78%), clubbing, single second heart sound, and a grade II/VI ejection systolic murmur over the left parasternal region. Echocardiography (Philips HD7 XE System, Eindhoven, The Netherlands) revealed the presence of a large subaortic ventricular septal defect, overriding of aorta, aneurysm of the left sinus of Valsalva at the level of the left coronary artery and pulmonary atresia (Fig. 1). Parasternal shortaxis view at the aortic valve level demonstrated confluent pulmonary arteries and left coronary to pulmonary artery fistula with a giant aneurysm at
Figure 2. Parasternal short-axis view shows the aneurysm at the origin of left coronary artery with left coronary to pulmonary artery fistula connecting to main pulmonary artery (PA) (marked with red arrow).
Figure 1. Parasternal long- and short-axis views show a large subaortic ventricular septal defect (VSD) with pulmonary atresia (P At) and an aneurysm (An) at the left sinus at the origin of the left coronary artery (marked with red arrow). Address for correspondence and reprint requests: Soumya Patra, M.D., Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences & Research, Bangalore, Pin-560069, India. Fax: 91-80-2297-7422; E-mail: [email protected]
E130
Coronary–Pulmonary Artery Fistula with Giant Aneurysm
Figure 3. Left ventricular and aortic root angiogram reveals a giant coronary artery aneurysm, coronary to pulmonary artery fistula, and a confluence of branch pulmonary arteries (marked with red arrow).
the origin of the left coronary artery (Fig. 2; movie clip S1). This finding was confirmed by an invasive left ventricular and aortic root angiogram (Fig. 3; movie clip S2). Coronary to pulmonary artery fistula was the only source of pulmonary blood supply in our case. She was referred for intra-cardiac repair for this cardiac malformation. Pulmonary atresia with ventricular septal defect has a highly variable source of pulmonary blood flow and coronary artery anatomy.1 Rarely, coronary–pulmonary artery fistula is seen as the sole source of pulmonary blood flow.2 Association of giant aneurysm of the coronary artery with the fistula is extremely rare. Though echocardiography can demonstrate this malformation, aortic root aortogram with or without a selective coronary angiogram should be considered in all these cases to delineate the coronary artery anatomy.3 The prognostic implication of this coronary–pulmonary artery fistula remains unclear. During intra-cardiac repair, any injury to the supplying coronary artery should be prevented. Our case was unique as there was both aneurysm of coronary artery and a coronary–pulmonary artery fistula as the sole source of pulmonary blood flow.
References 1. Collison SP, Dagar KS, Kaushal SK, et al: Coronary artery fistulas in pulmonary atresia and ventricular septal defect. Asian Cardiovasc Thorac Ann 2008;16:29–32. 2. Kapoor A, Walia R, Jain S, et al: A large coronary-pulmonary artery fistula in a cyanotic patient leading to severe biventricular dysfunction and heart failure. Echocardiography 2011;28:E207–E211. 3. Kaneko Y, Okabe H, Nagata N, et al: Pulmonary atresia, ventricular septal defect, and coronary-pulmonary artery fistula. Ann Thorac Surg 2001;71:355–356.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Parasternal short-axis view with color compare shows the aneurysm at origin of the left coronary artery with left coronary to pulmonary artery fistula connecting to main pulmonary artery. Movie clip S2. Left ventricular angiogram reveals a giant coronary artery aneurysm, coronary to pulmonary artery fistula and a confluence of branch pulmonary arteries.
E131
Echocardiography
A Coronary–Pulmonary Artery Fistula with Giant Aneurysm in a Child with Ventricular Septal Defect with Pulmonary Atresia Jayaranganath Mahimaiha, D.M., Soumya Patra, M.D., Usha Mandikal Kodanda Rama Sastry, M.D., Anand Subramaniam, D.M., Navin Agrawal, D.M., and Manjunath Cholenahally Nanjappa, D.M. Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences & Research, Bangalore, India
(Echocardiography 2014;31:E130–E131) Key words: coronary–pulmonary artery fistula, giant aneurysm, pulmonary atresia, ventricular septal defect, echocardiography A 6-year-old girl born of a consanguinous marriage, with an uneventful birth history presented with cyanosis, clubbing, and effort intolerance. Her physical examination revealed cyanosis (oxygen saturation 78%), clubbing, single second heart sound, and a grade II/VI ejection systolic murmur over the left parasternal region. Echocardiography (Philips HD7 XE System, Eindhoven, The Netherlands) revealed the presence of a large subaortic ventricular septal defect, overriding of aorta, aneurysm of the left sinus of Valsalva at the level of the left coronary artery and pulmonary atresia (Fig. 1). Parasternal shortaxis view at the aortic valve level demonstrated confluent pulmonary arteries and left coronary to pulmonary artery fistula with a giant aneurysm at
Figure 2. Parasternal short-axis view shows the aneurysm at the origin of left coronary artery with left coronary to pulmonary artery fistula connecting to main pulmonary artery (PA) (marked with red arrow).
Figure 1. Parasternal long- and short-axis views show a large subaortic ventricular septal defect (VSD) with pulmonary atresia (P At) and an aneurysm (An) at the left sinus at the origin of the left coronary artery (marked with red arrow). Address for correspondence and reprint requests: Soumya Patra, M.D., Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Sciences & Research, Bangalore, Pin-560069, India. Fax: 91-80-2297-7422; E-mail: [email protected]
E130
Coronary–Pulmonary Artery Fistula with Giant Aneurysm
Figure 3. Left ventricular and aortic root angiogram reveals a giant coronary artery aneurysm, coronary to pulmonary artery fistula, and a confluence of branch pulmonary arteries (marked with red arrow).
the origin of the left coronary artery (Fig. 2; movie clip S1). This finding was confirmed by an invasive left ventricular and aortic root angiogram (Fig. 3; movie clip S2). Coronary to pulmonary artery fistula was the only source of pulmonary blood supply in our case. She was referred for intra-cardiac repair for this cardiac malformation. Pulmonary atresia with ventricular septal defect has a highly variable source of pulmonary blood flow and coronary artery anatomy.1 Rarely, coronary–pulmonary artery fistula is seen as the sole source of pulmonary blood flow.2 Association of giant aneurysm of the coronary artery with the fistula is extremely rare. Though echocardiography can demonstrate this malformation, aortic root aortogram with or without a selective coronary angiogram should be considered in all these cases to delineate the coronary artery anatomy.3 The prognostic implication of this coronary–pulmonary artery fistula remains unclear. During intra-cardiac repair, any injury to the supplying coronary artery should be prevented. Our case was unique as there was both aneurysm of coronary artery and a coronary–pulmonary artery fistula as the sole source of pulmonary blood flow.
References 1. Collison SP, Dagar KS, Kaushal SK, et al: Coronary artery fistulas in pulmonary atresia and ventricular septal defect. Asian Cardiovasc Thorac Ann 2008;16:29–32. 2. Kapoor A, Walia R, Jain S, et al: A large coronary-pulmonary artery fistula in a cyanotic patient leading to severe biventricular dysfunction and heart failure. Echocardiography 2011;28:E207–E211. 3. Kaneko Y, Okabe H, Nagata N, et al: Pulmonary atresia, ventricular septal defect, and coronary-pulmonary artery fistula. Ann Thorac Surg 2001;71:355–356.
Supporting Information Additional Supporting Information may be found in the online version of this article: Movie clip S1. Parasternal short-axis view with color compare shows the aneurysm at origin of the left coronary artery with left coronary to pulmonary artery fistula connecting to main pulmonary artery. Movie clip S2. Left ventricular angiogram reveals a giant coronary artery aneurysm, coronary to pulmonary artery fistula and a confluence of branch pulmonary arteries.
E131
