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Myocardial ischemia secondary to dual coronary artery fistulas draining into main pulmonary artery
International Journal of Cardiology 140 (2010) e30 – e33 www.elsevier.com/locate/ijcard
Letter to the Editor
Myocardial ischemia secondary to dual coronary artery fistulas draining into main pulmonary artery Rajesh Vijayvergiya a,⁎, Prateek S. Bhadauria a , Harsha Jeevan a , Bhagwant Rai Mittal b , Anil Grover a a
Department of Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh- 160012, India b Nuclear Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh- 160012, India Received 9 November 2008; accepted 15 November 2008 Available online 9 January 2009
Abstract Bilateral coronary artery to pulmonary artery fistulas is an uncommon congenital anomaly. These fistulas have a clinical and embryological significance. We report a rare case of combination of right coronary and left circumflex coronary fistula draining into main pulmonary artery, who presented in the emergency room with acute pain chest. The fistula had significant 1.7:1 left to right shunt and also myocardial ischemia as demonstrated by exercise Thallium201 SPECT myocardial imaging. He was managed conservatively during the last 5 years without any percutaneous or surgical intervention. © 2008 Elsevier Ireland Ltd. All rights reserved. Keywords: Coronary fistula; Dual coronary fistula; Myocardial ischemia
1. Introduction
2. Case report
Coronary artery fistula is an unusual anomaly that consists of abnormal communication between an epicardial coronary artery and a cardiac chamber, major vessel or other vascular structures. Most of the times, these fistulae are small and incidentally detected during coronary angiography, while others are identified as cause of continuous murmur, angina, acute myocardial infarction, sudden death, coronary steal, congestive heart failure, infective endocarditis, arrhythmias, coronary aneurysm formation, or superior vena cava syndrome [1]. Incidence of coronary artery fistula is 0.1% among patients subjected for coronary angiography [2]. We report a case of myocardial ischemia secondary to dual coronary artery to pulmonary artery fistulae.
A 45 year old non-diabetic, normotensive male presented to emergency with anginal chest pain in January 2003. His general and systemic examination was unremarkable. ECG showed ST depression and T inversion in inferior limb leads and V4–V6 chest leads. Routine biochemical investigations and cardiac enzymes were in normal range. Chest X-ray did not show any cardiomegaly. Echocardiogram showed left ventricular ejection fraction of 60%, and no regional wall motion abnormality or structural heart disease. The left atrial and left ventricles were not enlarged on 2-D Echocardiography. Coronary angiography revealed one fistula from proximal part of left circumflex (Fig. 1) and two fistulae from posterior descending branch of right coronary artery (Fig. 2), draining into main pulmonary artery (Fig. 3). There was no significant atherosclerotic stenosis of any of the coronaries. Oximetry data showed significant 10% step up at main pulmonary artery level. Pulmonary artery pressure was 24/13 mm Hg. The pulmonary blood flow to systemic
⁎ Corresponding author. Tel.: +91 172 2756512; fax: +91 172 2744401. E-mail address: [email protected] (R. Vijayvergiya). 0167-5273/$ - see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2008.11.074
R. Vijayvergiya et al. / International Journal of Cardiology 140 (2010) e30–e33
Fig. 1. Left circumflex (LCx) coronary angiogram in lateral view showing fistula from proximal LCx to main pulmonary artery.
e31
Fig. 3. Pulmonary artery angiogram in antero-posterior view shows retrograde filling of distal end of coronary fistula at the level of main pulmonary artery.
follow-up, he is doing well in the last 5 years without any worsening of symptoms. blood flow ratio (Qp/Qs) was 1.7:1. An exercise Thallium201 SPECT myocardial imaging revealed reversible perfusion defect in inferio-lateral region of left ventricle (Fig. 4) involving 18% of myocardium. An option of percutaneous closure of fistula was discussed with the patient but he declined for the same. He was treated with beta-blockers. On regular
Fig. 2. Right coronary artery (RCA) angiogram in LAO 60° view shows two fistulae from posterior descending branch to main pulmonary artery.
3. Discussion Coronary fistula is an unusual anomaly of coronary circulation; the right coronary artery is the most common site (60%), followed by left coronary artery (35%) and dual coronary artery fistula (5%) [3]. It usually drains in right heart chambers, most commonly in right ventricle (40%), followed by in right atrium (25%), pulmonary artery (15–20%) and in coronary sinus (7%) [3]. Most of the published case reports have demonstrated dual fistula from right coronary and left anterior descending coronary artery draining into pulmonary artery [4–6], however the combination of right coronary and left circumflex coronary fistula to pulmonary artery as present in the index case is not being reported earlier in published English literature. An embryological basis in the form of persistence of coronary artery anlagen at pulmonary sinus and immature supernumerary coronary artery communication between pulmonary sinus and coronary artery had been explained for this uncommon coronary anomaly [6,7]. Most often, the fistulas are of small magnitude without significant left to right shunt or myocardial ischemia (secondary to “coronary steal” phenomenon); but large fistulas likely to have complications like cardiac failure, myocardial ischemia or infarction, infective endocarditis and rupture [1,2,4]. The index case had myocardial ischemia and a significant left to right shunt, for which a therapeutic intervention either in the form of percutaneous intervention or surgical repair would have been required [1,8,9]. However, as the index case was not willing for intervention, we treated him conservatively for the last 5 years, during which he
e32
R. Vijayvergiya et al. / International Journal of Cardiology 140 (2010) e30–e33
Fig. 4. Exercise Thallium201 SPECT myocardial imaging shows stress induced reversible perfusion defect in inferio-lateral region of left ventricle.
remained stable with no worsening of symptoms and did not have any complications related to coronary fistulae. Acknowledgement The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology [10].
References [1] Waller BF. Nonatherosclerotic coronary artery disease: coronary artery fistula. In: Fuster V, Alexander RW, O'Rourke RA, editors. Hurst's the heart. 11th edition. McGraw-Hill publications; 2004. p. 1180–1.
[2] Vavuranakis M, Bush CA, Boudoulas H. Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Catheter Cardiovasc Diagn 1995;35:116–20. [3] Congenital anomalies of the coronary arteries: coronary arteriovenous fistula. In: Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB, editors. Kirklin, Barratt-Boyes cardiac surgery. 3rd edition. Churchill Livingstone; 2003. p. 1241–2. [4] Baim DS, Kline H, Silverman FJ. Bilateral coronary artery–pulmonary artery fistulas, report of five cases and review of the literature. Circulation 1982;65:810–5. [5] Okmen AS. Bilateral coronary artery fistulas terminating at the same location case report. Int J Cardiol 2007;116:e53–4. [6] Vaidyanathan KR, Theodore SA, Sankar MN, Cherian KM. Coronary artery to pulmonary artery fistula with dual origin—embryological, clinical and surgical significance. Eur J Cardiothorac Surg 2007;31: 318–9.
R. Vijayvergiya et al. / International Journal of Cardiology 140 (2010) e30–e33 [7] Klein LW. A new hypothesis of the developmental origin of congenital left anterior descending coronary artery to pulmonary artery fistulas. Catheter Cardiovasc Interv 2008;71:568–71. [8] Okubo M, Nykanen D, Benson LN. Outcomes of transcatheter embolization in the treatment of coronary artery fistulas. Catheter Cardiovasc Interv 2001;52:510–7.
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[9] Kamiya H, Yasuda T, Nagamine H, et al. Surgical treatment of congenital coronary artery fistulas: 27 years' experience and a review of the literature. J Card Surg 2002;17:173–7. [10] Coats AJ. Ethical authorship and publishing. Int J Cardiol 2009;131: 149–50.
Letter to the Editor
Myocardial ischemia secondary to dual coronary artery fistulas draining into main pulmonary artery Rajesh Vijayvergiya a,⁎, Prateek S. Bhadauria a , Harsha Jeevan a , Bhagwant Rai Mittal b , Anil Grover a a
Department of Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh- 160012, India b Nuclear Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh- 160012, India Received 9 November 2008; accepted 15 November 2008 Available online 9 January 2009
Abstract Bilateral coronary artery to pulmonary artery fistulas is an uncommon congenital anomaly. These fistulas have a clinical and embryological significance. We report a rare case of combination of right coronary and left circumflex coronary fistula draining into main pulmonary artery, who presented in the emergency room with acute pain chest. The fistula had significant 1.7:1 left to right shunt and also myocardial ischemia as demonstrated by exercise Thallium201 SPECT myocardial imaging. He was managed conservatively during the last 5 years without any percutaneous or surgical intervention. © 2008 Elsevier Ireland Ltd. All rights reserved. Keywords: Coronary fistula; Dual coronary fistula; Myocardial ischemia
1. Introduction
2. Case report
Coronary artery fistula is an unusual anomaly that consists of abnormal communication between an epicardial coronary artery and a cardiac chamber, major vessel or other vascular structures. Most of the times, these fistulae are small and incidentally detected during coronary angiography, while others are identified as cause of continuous murmur, angina, acute myocardial infarction, sudden death, coronary steal, congestive heart failure, infective endocarditis, arrhythmias, coronary aneurysm formation, or superior vena cava syndrome [1]. Incidence of coronary artery fistula is 0.1% among patients subjected for coronary angiography [2]. We report a case of myocardial ischemia secondary to dual coronary artery to pulmonary artery fistulae.
A 45 year old non-diabetic, normotensive male presented to emergency with anginal chest pain in January 2003. His general and systemic examination was unremarkable. ECG showed ST depression and T inversion in inferior limb leads and V4–V6 chest leads. Routine biochemical investigations and cardiac enzymes were in normal range. Chest X-ray did not show any cardiomegaly. Echocardiogram showed left ventricular ejection fraction of 60%, and no regional wall motion abnormality or structural heart disease. The left atrial and left ventricles were not enlarged on 2-D Echocardiography. Coronary angiography revealed one fistula from proximal part of left circumflex (Fig. 1) and two fistulae from posterior descending branch of right coronary artery (Fig. 2), draining into main pulmonary artery (Fig. 3). There was no significant atherosclerotic stenosis of any of the coronaries. Oximetry data showed significant 10% step up at main pulmonary artery level. Pulmonary artery pressure was 24/13 mm Hg. The pulmonary blood flow to systemic
⁎ Corresponding author. Tel.: +91 172 2756512; fax: +91 172 2744401. E-mail address: [email protected] (R. Vijayvergiya). 0167-5273/$ - see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2008.11.074
R. Vijayvergiya et al. / International Journal of Cardiology 140 (2010) e30–e33
Fig. 1. Left circumflex (LCx) coronary angiogram in lateral view showing fistula from proximal LCx to main pulmonary artery.
e31
Fig. 3. Pulmonary artery angiogram in antero-posterior view shows retrograde filling of distal end of coronary fistula at the level of main pulmonary artery.
follow-up, he is doing well in the last 5 years without any worsening of symptoms. blood flow ratio (Qp/Qs) was 1.7:1. An exercise Thallium201 SPECT myocardial imaging revealed reversible perfusion defect in inferio-lateral region of left ventricle (Fig. 4) involving 18% of myocardium. An option of percutaneous closure of fistula was discussed with the patient but he declined for the same. He was treated with beta-blockers. On regular
Fig. 2. Right coronary artery (RCA) angiogram in LAO 60° view shows two fistulae from posterior descending branch to main pulmonary artery.
3. Discussion Coronary fistula is an unusual anomaly of coronary circulation; the right coronary artery is the most common site (60%), followed by left coronary artery (35%) and dual coronary artery fistula (5%) [3]. It usually drains in right heart chambers, most commonly in right ventricle (40%), followed by in right atrium (25%), pulmonary artery (15–20%) and in coronary sinus (7%) [3]. Most of the published case reports have demonstrated dual fistula from right coronary and left anterior descending coronary artery draining into pulmonary artery [4–6], however the combination of right coronary and left circumflex coronary fistula to pulmonary artery as present in the index case is not being reported earlier in published English literature. An embryological basis in the form of persistence of coronary artery anlagen at pulmonary sinus and immature supernumerary coronary artery communication between pulmonary sinus and coronary artery had been explained for this uncommon coronary anomaly [6,7]. Most often, the fistulas are of small magnitude without significant left to right shunt or myocardial ischemia (secondary to “coronary steal” phenomenon); but large fistulas likely to have complications like cardiac failure, myocardial ischemia or infarction, infective endocarditis and rupture [1,2,4]. The index case had myocardial ischemia and a significant left to right shunt, for which a therapeutic intervention either in the form of percutaneous intervention or surgical repair would have been required [1,8,9]. However, as the index case was not willing for intervention, we treated him conservatively for the last 5 years, during which he
e32
R. Vijayvergiya et al. / International Journal of Cardiology 140 (2010) e30–e33
Fig. 4. Exercise Thallium201 SPECT myocardial imaging shows stress induced reversible perfusion defect in inferio-lateral region of left ventricle.
remained stable with no worsening of symptoms and did not have any complications related to coronary fistulae. Acknowledgement The authors of this manuscript have certified that they comply with the Principles of Ethical Publishing in the International Journal of Cardiology [10].
References [1] Waller BF. Nonatherosclerotic coronary artery disease: coronary artery fistula. In: Fuster V, Alexander RW, O'Rourke RA, editors. Hurst's the heart. 11th edition. McGraw-Hill publications; 2004. p. 1180–1.
[2] Vavuranakis M, Bush CA, Boudoulas H. Coronary artery fistulas in adults: incidence, angiographic characteristics, natural history. Catheter Cardiovasc Diagn 1995;35:116–20. [3] Congenital anomalies of the coronary arteries: coronary arteriovenous fistula. In: Kouchoukos NT, Blackstone EH, Doty DB, Hanley FL, Karp RB, editors. Kirklin, Barratt-Boyes cardiac surgery. 3rd edition. Churchill Livingstone; 2003. p. 1241–2. [4] Baim DS, Kline H, Silverman FJ. Bilateral coronary artery–pulmonary artery fistulas, report of five cases and review of the literature. Circulation 1982;65:810–5. [5] Okmen AS. Bilateral coronary artery fistulas terminating at the same location case report. Int J Cardiol 2007;116:e53–4. [6] Vaidyanathan KR, Theodore SA, Sankar MN, Cherian KM. Coronary artery to pulmonary artery fistula with dual origin—embryological, clinical and surgical significance. Eur J Cardiothorac Surg 2007;31: 318–9.
R. Vijayvergiya et al. / International Journal of Cardiology 140 (2010) e30–e33 [7] Klein LW. A new hypothesis of the developmental origin of congenital left anterior descending coronary artery to pulmonary artery fistulas. Catheter Cardiovasc Interv 2008;71:568–71. [8] Okubo M, Nykanen D, Benson LN. Outcomes of transcatheter embolization in the treatment of coronary artery fistulas. Catheter Cardiovasc Interv 2001;52:510–7.
e33
[9] Kamiya H, Yasuda T, Nagamine H, et al. Surgical treatment of congenital coronary artery fistulas: 27 years' experience and a review of the literature. J Card Surg 2002;17:173–7. [10] Coats AJ. Ethical authorship and publishing. Int J Cardiol 2009;131: 149–50.