Bleeding Heart and Coronary Cameral Fistulae

Dear Editor:

We read the article regarding coronary-cameral fistula after septal myomectomy by Walters D, et al,¹ published in the December 2017 issue of the Journal of Invasive Cardiology, with great interest. We hereby differ on certain points from the authors, which can be further clarified by them.  

As multiple coronary cameral fistulae (CCFs) can be congenitally associated with hypertrophic cardiomyopathy (HCM),^2-4 the attribution of these fistulae to myomectomy is doubtful, especially in the absence of presurgery angiographic findings. The reasons for reduction in ejection fraction from 60% to 25% following myomectomy also need further clarification. Certain factors, such as decreased septal motion, septal myocardial infarction, and post-surgery myocardial depression, could have contributed in reducing ejection fraction. The contribution of these small CCFs to low ejection fraction and congestive heart failure is unlikely in the presence of insignificant left to left shunt. Referring to the term “bleeding heart” used by the authors,¹ we would like to share an interesting related case. 

A 58-year-old male who was hypertensive and a chronic smoker presented with history of angina on exertion class II for the past month. Systemic and cardiovascular examinations were unremarkable. Electrocardiogram showed normal sinus rhythm and left ventricular hypertrophy, without any ST-T changes. Echocardiography showed left ventricular ejection fraction of 60% and absence of any regional wall-motion abnormality. Coronary angiography revealed insignificant mid left anterior descending artery stenosis, and the distal diagonal (D1) branch gave rise to multiple, extensive CCFs, which were draining into the left ventricle (Figure 1; Video 1). These fistulae were so extensive that the entire left ventricular cavity became opacified in the late phase of imaging, a condition described as “coronary injection ventriculography.”⁵

Sincerely,

Prof (Dr) Rajesh Vijayvergiya, MD, DM, FSCAI, FISES, FACC, and Bhupendra Kumar Sihag, MD

Department of Cardiology, Advanced Cardiac Centre, Post Graduate Institute of Medical Education & Research, Chandigarh, India. Email: rajeshvijay999@hotmail.com 

References

1.     Walters D, Reeves R, Ang L, Pourdjabbar A, Mahmud E. A bleeding heart: coronary-cameral fistula after septal myomectomy. J Invasive Cardiol. 2017;29:E199-E200.

2.    Hong GR, Choi SH, Kang SM, et al. Multiple coronary artery-left ventricular microfistulae in a patient with apical hypertrophic cardiomyopathy: a demonstration by transthoracic color Doppler echocardiography. Yonsei Med J. 2003;44:710-714.

3.    Dresios C, Apostolakis S, Tzortzis S, Lazaridis K, Gardikiotis A. Apical hypertrophic cardiomyopathy associated with multiple coronary artery-left ventricular fistulae: a report of a case and review of the literature. Eur J Echocardiogr. 2010;11:E9.

4.    Alyan O, Ozeke O, Golbasi Z. Coronary artery-left ventricular fistulae associated with apical hypertrophic cardiomyopathy. Eur J Echocardiogr. 2006;7:326-329. 

5.    Kocabas U, Kaya E, Akin M. Coronary injection ventriculography: multiple coronary-cameral fistulas as a rare cause of stable angina pectoris. Internat J Cardiovasc Acad. 2016;2:68-70. 

Authors’ Response

We appreciate the interest garnered by our recent contribution and the authors’ commentary in response. 

The authors present another striking example of a coronary cameral fistula (CCF), here in a patient without hypertrophic cardiomyopathy (HCM) but rather presenting with typical exertional angina. The phrase “coronary injection ventriculography” may be more clinically relevant than the tongue-in-cheek “bleeding heart” expression; however, CCF alone is adequately descriptive in most scenarios.

In the context of our patient with HCM, the decline in ejection fraction after myomectomy was likely multifactorial. Post-myomectomy myocardial infarction, septal dysmotility, and other primary myocardial issues were undoubtedly contributory toward the development of congestive heart failure; the further overload and high output demand created by the multiple CCFs were another exacerbating factor within the broader clinical context. 

As stated in the initial publication, the CCF phenomena has many different etiologies, including congenital, as is probable in the patient referenced by the authors, and acquired/iatrogenic including in the context of surgical trauma. With regard to the HCM patient, although no prior coronary angiography was available, echocardiography and CT coronary imaging before myomectomy demonstrated no evidence of CCF, supporting myomectomy as the iatrogenic cause as opposed to congenital development. The etiology is broad. More important is an emphasis on management moving forward, as little is known regarding treatment and long-term outcomes of those patients without spontaneous closure. Further research is warranted within this field.

Daniel Walters, MD, Ryan Reeves, MD, and Ehtisham Mahmud, MD

UC San Diego Health System, Sulpizio Cardiovascular Center, 9434 Medical Center Dr, La Jolla, CA 92037. Email: dcwalters@ucsd.edu