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 Table of Contents  
Year : 2020  |  Volume : 6  |  Issue : 2  |  Page : 187-191

Ventricular tachycardia, rheumatic heart disease, and bilateral coronary pulmonary artery fistula: A rare association

1 Department of Cardiology, Cardio-Thoracic Surgery, Yashoda Hospitals, Hyderabad, Telangana, India
2 Cardio-Thoracic Surgery, Yashoda Hospitals, Hyderabad, Telangana, India

Date of Submission08-Jun-2020
Date of Decision20-Jun-2020
Date of Acceptance12-Jul-2020
Date of Web Publication27-Aug-2020

Correspondence Address:
Dr. Pankaj Jariwala
Department of Cardiology, Yashoda Hospitals, Somajiguda, Raj Bhavan Road, Hyderabad - 500 082, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcs.jpcs_62_20

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Coronary-pulmonary artery fistulas are rare congenital or acquired anomalies of the coronary artery that may originate from any of the three major coronary arteries and drain into the right-sided cardiac chambers and or large vessels draining into the pulmonary circulation. A triple combination of ventricular tachycardia, rheumatic heart disease, and bilateral coronary pulmonary fistula is a rare association. A rare combination of observations was detected in a single patient with three distinct etiopathogenesis that is not described in the literature.

Keywords: Chronic rheumatic heart disease, coronary arteriovenous fistula, mitral valvulopathy, ventricular tachycardia

How to cite this article:
Jariwala P, Jadhav K, Kale SS. Ventricular tachycardia, rheumatic heart disease, and bilateral coronary pulmonary artery fistula: A rare association. J Pract Cardiovasc Sci 2020;6:187-91

How to cite this URL:
Jariwala P, Jadhav K, Kale SS. Ventricular tachycardia, rheumatic heart disease, and bilateral coronary pulmonary artery fistula: A rare association. J Pract Cardiovasc Sci [serial online] 2020 [cited 2023 Feb 1];6:187-91. Available from: https://www.j-pcs.org/text.asp?2020/6/2/187/293592

  Introduction Top

Coronary-pulmonary artery fistula (CPAF) is very rare anomalies with fistulous communication between coronary and pulmonary circulation, mainly to the pulmonary arteries constituting 17% of them.[1] The majority of fistulas originate from the left anterior descending artery is followed by the right coronary artery (RCA), rarely from the left circumflex artery (LCx). They commonly drain in a low-pressure venous side including right-sided chambers, pulmonary artery, superior vena cava, and coronary sinus.

We report a case of bilateral CPAF diagnosed incidentally in a patient who presented with ventricular tachycardia (VT) and associated underlying chronic rheumatic heart disease (CRHD) with multi-valvular involvement.

  Case Report Top

A 45-year-old woman complained about the abrupt onset of palpitations and the transient loss of consciousness in the emergency room. The vital parameters recorded were pulse rate 188 per minute, respiratory rate 18 per minute, and blood pressure 80/50 mm Hg. Electrocardiography showed monomorphic VT; therefore, direct current cardioversion was administered due to hypotension. Echocardiography revealed the presence of mild rheumatic mitral stenosis, mild mitral regurgitation, and normal aortic valve with normal left ventricular function. Furthermore, moderate organic tricuspid valve stenosis and regurgitation with the right ventricular pressure of 58 mm Hg [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. Laboratory parameters including acute phase reactants (C-reactive protein) were normal. Amiodarone and metoprolol stabilized her rhythm to normal. Coronary angiography demonstrated normal epicardial coronary arteries with bilateral coronary pulmonary fistula from the proximal segments of the left main coronary artery (LMCA), LCx, and RCA draining into the main pulmonary arterial trunk [Figure 2]a, [Figure 2],b [Figure 2]c, [Figure 2]d VT could not be triggered during the electrophysiology study. Stress myocardial scintigraphy has not shown any evidence of myocardial ischemia. Computed tomography (CT) angiography confirmed the presence of these fistulas and further clarified that CPAF from the coronary artery originated from LMCA, LCx, and RCA [Figure 3]. The patient discharged with optimal medical management advice including prophylaxis of penicillin, oral spironolactone, beta-blocker, and close follow-up. Timeline of the clinical events, investigations, medical management, and follow-up is summarized in flow chart [Figure 4].
Figure 1: (a-d) Echocardiography demonstrated thickened and doming mitral leaflets suggestive of CRHD. The short-axis view at the level of the mitral valve measures the mitral valve area of 1.5 cm2 using planimetry (a). The continuous-wave Doppler across the mitral valve recorded the peak gradient of 7 mm Hg and a mean gradient of 3 mm Hg (b). Four chamber view revealed thickening of the tricuspid valve with mild dilatation of right atrium and ventricle secondary to tricuspid stenosis and regurgitation (c). The continuous-wave Doppler across the tricuspid valve measured peak velocity of 3.7 cm/s and right ventricular systolic pressure of 58 (48 + 10) mm Hg (d).

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Figure 2: (a-d) Coronary angiography of the left coronary artery in the right (a) and left anterior oblique (b) caudal views demonstrating a fistula formed of a thin tortuous vessel arising from the proximal segments of the left main coronary artery and the LCx (black arrows) and shows faint evidence of filling the main pulmonary artery (white arrows). Right coronary angiogram in left anterior oblique cranial projection (c, zoomed view, d) shows a well-developed fistula formation from the conus branch to the main pulmonary artery (black arrows) manifested as blushing and pooling of contrast medium into the main pulmonary artery (white arrows).

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Figure 3: Computed tomography angiography constructed maximum-intensity-projection image revealed fistula (arrows) from the proximal segments of the left main coronary artery and the left circumflex artery to the proximal pulmonary artery

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Figure 4: Flow chart summarizing the timeline of the clinical events and treatment offered to patient during hospitalization, postdischarge, and follow-up plan.

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We searched for various medical databases such as PubMed, Google Scholar, Crossref using the keywords “coronary-pulmonary artery fistula” and or “rheumatic heart disease;” “coronary-pulmonary artery fistula;” and or “ventricular tachycardia.” There were no specific language or time limits set for specific review papers, case series, and studies.

We found eight titles of CPAF associated with rheumatic heart disease (RHD)[2],[3],[4],[5],[6],[7],[8],[9] and one title of CPAF and VT.[10] No publication could be found describing the association between CPAF, RHD, and VT as observed in our case. The details of the cases relating to the association between CPAF and RHD are summarized in [Table 1]. Of the eight publications, three titles were without a full-text article which did not provide details of the cases.[2],[3],[5] Branco et al. documented three cases of CPAF linked to CRHD.[3] In total, 11 cases of CPAF were identified in association with CRHD, including our case in the literature. This confirms that our case is a rare combination of etiologies.
Table 1: List of publications showing association between coronary-pulmonary artery fistula and rheumatic heart disease

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  Discussion Top

CPAF is characterized by abnormal communication between one or more branches of the coronary arteries, cardiac chambers, and adjacent cardiac structures such as vena cava, coronary sinus, and pulmonary arteries. The coronary artery fistula (CAF), first described in 1865 by Krause,[2] is a major congenital coronary anomaly occurring in <1%. CAPF is very uncommon and comprises only 20% of all CAFs.[11]

Verdini et al. reviewed 103 cases of CPAF which compromised their cases and those reported in the literature in the past 30 years. The most common coronary artery of origin for a CPAF was the left main/left anterior descending (84%), followed by the RCA (38%) with multiple CPAFs were present in 45%.

The CPAF most commonly terminated in the main pulmonary artery (89%). In patients with bilateral CPAFs, origin from the LMCA/LCx and the RCA was less common.[12] CPAFs are incidental findings during the conventional coronary angiography.[13] The prevalence of CPAFs is increasing with the advent of advanced CT angiography.

In 1979, Liberthson et al. identified 13 CAF cases, one of which had associated rheumatic mitral regurgitation which was the first description of RHD and CAF association.[6] Lanzillo et al. performed first surgical replacement of the mitral valve for a case of CPAF and rheumatic mitral valve disease.[9]

Tamura et al. established the triple combination of an atrial septal aneurysm, CPAF, and RHD that underwent complete septal aneurysm and CPAF correction, mitral valve replacement, and tricuspid repair surgery.[2] Barrios et al. performed double valve replacement for rheumatic aortic and mitral valve disease and surgical correction of bilateral CPAF.[4] Branco et al. in their case series, of 14 patients, 3 patients had RHD associated with CAF.[3] Tanriverdi et al. and Lee et al. described the association of bilateral CPAF and RHD and their cases had severe mitral stenosis and aortic regurgitation, respectively.[7],[8] The left-to-right shunt secondary to CPAF associated with rheumatic valvular involvement may increase the cardiopulmonary symptoms depending on the severity of the valvular disease, the number of valves affected, and amount of the shunt lesion.[9]

In our case, the mechanism of VT was unclear. Stojišić-Milosavljević et al. reported a case of bilateral CPAF with who presented with recurrent VTs. The electrophysiological analysis did not yield any positive information and required an automated implantable cardioverter-defibrillator implantation.[10] In our situation, VT was responding to medical therapy and it was the first episode, so we implemented a “wait and watch” approach.

The main indications for closure for CPAFs are patient symptoms, particularly congestive heart failure, coronary ischemia, or significant shunting. Treatment options include observation, transcatheter embolization, or surgical intervention.

As per the literature search, bilateral CPAF, RHD, and VT have no recognized associations or syndromes.

  Conclusion Top

The majority of CPAF is benign with a subtle effect on coronary physiology. Such irregular vascular pathways may be triggered (either in the uterus or in life) by local cardiopulmonary developmental abnormalities or injuries that tend to stimulate vascular growth and migration from systemic cardio-pericardial arteries to link up with pulmonary arterial circulation. The presence of VT in combination with RHD and multiple bilateral CPAFs is a mere correlation, and this set of findings is not previously established, and such associations should be recognized.

Patient Perspectives: From emergency room to discharge…

I woke up early in the morning, with a sense of dread and apprehension…. With extreme fatigue I had to struggle with palpitation in my throat. I was trying to get out of my bed, but I felt giddy and had a blackout…. I could not remember afterwards. Once I recovered my consciousness, I found myself in the bed with the beeping sound and chest burning feeling a lot of hurry around….

I realized I'm hospitalized. A person came near me, looking like a doctor, and asked about my condition, how I felt now. He gave no further detail, and looked at the monitor, and left that location… A lot of infusions were applied to my right hand, so I felt I was in critical shape. Nurses were consoling me as I seemed apprehensive… They gave me some drinking water, as I felt thirsty.

I could see my worried-looking son… He said I had dropped due to low blood pressure and after doing a few tests and observation at the intensive cardiac care unit they will release after a few days…

Then I was moved to the cardiac intensive care unit where numerous doctors and nurses asked about my condition… After a few hours I was feeling better. They gave me a diet that was liquid like soup. I slept later while the monitor's beeping sounds were difficult…

I woke up the next day and the nurse advised me I had to undergo a CT scan, heart echocardiography and I would be fasting… Doctors came for rounds and after conversation and reading my blood tests, they discussed something that I did not understand among themselves in their medical language.

After another day of observation, they informed me that I was going to undergo a special investigation called electrophysiological study because I had the very fast heart beating that required electrical shock to restore normalcy. Yet I do not remember those… I was relocated to a special room where a doctor who described her as an anesthesiologist gave me sedation and I was taken out of that room after the procedure.

The doctor who looked after me the next day told me that everything was fine with me and I'm going to be transferred from here to room…

They directed one investigation after observation which was a last significant examination as per them. They made me walk on the treadmill in those tests and it was a really laborious exercise and afterwards took entire day under the camera to look for the flow of heart blood.

Eventually, a day came when my doctor and family told me that only on medication I can be released, and I have to come for frequent check-up. Dietitian came and told me to take more fruit and vegetables, as my potassium seems to have been low. It's also recommended every 3 weeks for regular penicillin injections…

Later, I came to know that I'm suffering from some heart condition called RHD which is one of our country's common problems and I've had the quick heart beating which requires only medical therapy. My heart condition is also not severe, and no heart surgery is required at present.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Athanasias DA, van Ommen V, Ba¨r F. Coronary artery-pulmonary artery fistula originating from the left anterior descending artery: A case report and literature review. Hellenic J Cardiol 2002;43:78-81.  Back to cited text no. 1
Tamura S, Ebine K, Sato K, Lee M, Matii K, Aihara M. A surgical case of atrial septal aneurysm associated with rheumatic mitral and tricuspid disease and coronary-pulmonary artery fistula. Nihon Kyobu Geka Gakkai Zasshi 1990;38:336-40.  Back to cited text no. 2
Branco L, Agapito A, Ramos JM, Patrício L, Monteiro I, Galrinho A, et al. Coronary fistula-”clinical and angiographic review. Rev Port Cardiol 1994;13:243-52, 193-4.  Back to cited text no. 3
Barrios V, Jiménez-Nácher JJ, Ojeda JL, Jiménez-Mena M, Epeldegui A, Oliva E, et al. Surgical correction of double congenital coronary – Pulmonary artery fistulas and rheumatic mitral and aortic valve disease – A case report. Vasc Endovascular Surg 1993;27:543-8.  Back to cited text no. 4
Wang C. Dual-source CT in diagnosis of coronary artery fistula and its value. BMC Med Imaging 2020;DOI:10.21203/rs.3.rs-28294/v1.  Back to cited text no. 5
Liberthson RR, Sagar K, Berkoben JP, Weintraub RM, Levine FH. Congenital coronary arteriovenous fistula. Report of 13 patients, review of the literature and delineation of management. Circulation 1979;59:849-54.  Back to cited text no. 6
Lee SJ, Her SH, Jin SW, Lee JM, Yoon HJ, Lee HY, et al. A case of bilateral coronary to pulmonary artery fistulas associated with severe aortic regurgitation. Korean Circ J 2008;38:331-4.  Back to cited text no. 7
Tanriverdi H, Seleci D, Semiz E. Angiographic detection of the left anterior descending and the right coronary artery after fistulas into the pulmonary artery in a patient with rheumatic mitral stenosis. Int J Cardiol 2006;109:139-41.  Back to cited text no. 8
Lanzillo G, Alessandrini F, Bartoccioni S, Bombardieri G, Intonti MA, Pragliola C, et al. Surgical correction of congenital left coronary-pulmonary artery fistula and rheumatic mitral valve disease. Case report. Scand J Thorac Cardiovasc Surg 1989;23:189-91.  Back to cited text no. 9
Stojišić-Milosavljević A, Bikicki M, Ivanović V, Šobot N, Popin TM, Kovačević D. Bilateral Coronary Artery - Pulmonary Artery Fistula with Recurrent Ventricular Tachycardia: Case Report. Srp Arh Celok Lek 2015;143:609-14.  Back to cited text no. 10
Gholoobi A, Poorzand H. Multiple coronary fistula formation associated with a huge left atrial thrombus in rheumatic mitral valve stenosis. J Cardiol Cases 2014;9:124-6.  Back to cited text no. 11
Verdini D, Vargas D, Kuo A, Ghoshhajra B, Kim P, Murillo H, et al. Coronary-Pulmonary Artery Fistulas: A Systematic Review. J Thorac Imaging 2016;31:380-90.  Back to cited text no. 12
Dourado LO, Góis AF, Hueb W, César LA. Large bilateral coronary artery fistula: The choice of clinical treatment. Arq Bras Cardiol 2009;93:e48-9.  Back to cited text no. 13


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]


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