Catheterization and Cardiovascular Interventions 86:E99–E102 (2015)

Case Reports Percutaneous Closure of an Iatrogenic Fistula From the Left Ventricle to the Coronary Sinus Zachary M. Gertz,1* MD, Jose-Luis E. Velazquez-Cecena,2 MD, and John. V. (Ian) Nixon,1 MD A patient with a history of rheumatic mitral valve disease and valve replacement in childhood presented with severe, persistent dyspnea. During an electrophyisiologic procedure, she was discovered to have a fistula from the left ventricle to the coronary sinus. She had severe pulmonary hypertension and elevated filling pressures, with a significant left-to-right shunt. Percutaneous closure of the fistula was performed using two vascular plugs. Subsequently the patient’s hemodynamics improved and her symptoms subsided. Here, we describe the case and review the literature. VC 2014 Wiley Periodicals, Inc. Key words: vascular plug; fistula; closure; coronary sinus; left ventricular outflow tract

INTRODUCTION

Fistulas from the left ventricle to the coronary sinus are rare events, and may be congenital or iatrogenic [1–3]. The first documented iatrogenic case occurred after mitral valve surgery, and most of the reported cases have resulted from mitral valve replacement [1,2]. Fistulas have also been noted after other procedures involving the inferolateral wall of the left ventricle and have even occurred spontaneously after myocardial infarction [4,5]. Treatment has consisted of surgical correction or medical management. Here, we present a case of a fistula that occurred after mitral valve replacement and was treated with percutaneous closure, with excellent results. CASE REPORT

A 51-year-old woman presented with chronic dyspnea and palpitations. The patient had an extensive cardiac history, beginning with rheumatic heart disease diagnosed at age 9. Her mitral valve was then replaced in 1987, at age 25, with a bioprosthesis, then the following year with a mechanical valve after an episode of endocarditis (StarrEdwards valve, Edwards Lifesciences, Irvine, CA). She continued to experience signs and symptoms of heart failure. She underwent tricuspid valve repair and then replacement for persistent severe regurgitation and right ventricular dysfunction in 2012, at age 50 (29 mm Trifecta valve, St. Jude Medical, St. Paul, MN). Her C 2014 Wiley Periodicals, Inc. V

symptoms did not resolve, despite optimal medical therapy. Although she continued to have normal left ventricular function and appropriate function of her prosthetic valves, she had persistent right ventricular dysfunction and elevated pulmonary pressure. In 2013, she presented to her primary cardiologist with persistent heart failure and palpitations and was referred for pacemaker placement. The decision was made to place the ventricular pacing lead in the coronary sinus, rather than through her prosthetic tricuspid valve. During the procedure, the catheter took an unexpected course and entered the ascending 1

Pauley Heart Center, Virginia Commonwealth University Medical Center Richmond, Virginia 2 Heart and Vascular Institute, Pikeville Medical Center Pikeville, Kentucky Additional Supporting Information may be found in the online version of this article. Conflict of interest: Nothing to report. *Correspondence to: Zachary M. Gertz, MD, 1200 E Broad St. West Hospital, 5th Floor, West Wing, Room 529-B, Richmond, VA 23298. E-mail: [email protected] Received 11 March 2014; Revision accepted 14 June 2014 DOI: 10.1002/ccd.25579 Published online 3 July 2014 in Wiley Online Library (wileyonlinelibrary.com)

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Fig. 3. Two-dimensional cardiac CT scans. (A) The fistula courses around the mechanical mitral valve (MV) and enters the coronary sinus (CS). The origin and insertion are denoted by white arrows. (B) After percutaneous closure, two vascular plugs can be seen in the origin and insertion of the fistula. RV 5 Right Ventricle.

Fig. 1. Cineangiogram image showing a catheter originating in the left subclavian vein and entering the coronary sinus (black arrow), with a multipurpose catheter extending from it into the ascending aorta (white arrow).

Fig. 2. Three-dimensional cardiac CT scan showing the origin and insertion of the fistula (white arrows), arising from the left ventricular outflow tract and inserting into the coronary sinus (CS). LV 5 Left Ventricle; RV 5 Right Ventricle.

aorta (Fig. 1). The procedure was aborted, and the patient was referred for a computed tomography (CT) scan. The CT scan revealed a fistula from the left ventricle at the level of the outflow tract extending into the coro-

nary sinus (Figs. 2 and 3A). She was transferred to a tertiary care center for further treatment. Her physical exam revealed elevated jugular venous pressure and lower extremity edema. She had a regular rhythm with appropriate prosthetic valve clicks with a palpable pulmonic component of the second heart sound. She had systolic murmurs at the left sternal border as well as at the apex radiating to the axilla. The electrocardiogram revealed a prolonged PR interval, incomplete right bundle branch block, and rightward axis deviation. A chest X-ray revealed an enlarged cardiac silhouette and dilated pulmonary artery, with minimal pulmonary edema. Echocardiography revealed both prosthetic valves to be functioning well, with minimal regurgitation. Left ventricular function was preserved, while right ventricular function was significantly reduced. Even with the knowledge that the fistula was present, it was not clearly seen by echocardiography. Most likely the fistula was obscured by the color signal arising from the ball-cage mitral valve (Supporting Information Movie 1). Right heart catheterization was performed, and was notable for a right atrial pressure of 35 mm Hg and a pulmonary artery pressure of 93/42 (mean 64) mm Hg (aortic pressure 133/87 mm Hg). The pulmonary capillary wedge pressure was 25 mm Hg (pulmonary vascular resistance 8 Wood units). There was a left-to-right shunt, with a pulmonary-to-systemic flow ratio of 1.7:1. An invasive treatment approach was selected, because of the patient’s symptoms and hemodynamics. Given her history of four prior sternotomies, percutaneous closure was chosen rather than open surgery. Arterial access was obtained with an 8F sheath in the left femoral artery, and heparin was given to maintain an activated clotting time (ACT) > 250 sec. One gram of cefazolin was given. After initial ventriculography, the fistula was engaged via a retrograde approach using a 6F JR4 diagnostic catheter. A

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

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Wood units. Her mixed venous and pulmonary artery oxygen saturations were equal, consistent with the cessation of left-to-right shunting. Her symptoms had improved from NYHA Class 3 to Class 1.

DISCUSSION

Fig. 4. Angiogram showing two vascular plugs in the fistula (black arrows). MV 5 Mechanical mitral valve.

hydrophilic wire was passed from the left ventricle, through the fistula, into the coronary sinus and stabilized in the right atrium. After advancing the catheter, the wire was exchanged for a 0.03500 stiff wire, and the catheter was exchanged for an 8F multipurpose guide catheter. The 0.03500 wire was then replaced with an Iron Man guide wire (Abbott Vascular, Abbott Park, IL), which was left in the right atrium to help maintain stable catheter position. Sizing of the fistula had already been performed by CT, with the diameter ranging from 14 mm at the inflow to 10 mm at the insertion into the coronary sinus. The guide catheter was then withdrawn into the coronary sinus, and a 12 mm Amplatzer Vascular Plug II (St. Jude Medical, St. Paul, Minnesota) was deployed at the insertion of the fistula into the coronary sinus (Supporting Information Movie 2). A 16 mm plug was then deployed at the origin of the fistula from the left ventricle (Supporting Information Movie 3). Final imaging revealed an excellent result (Figs. 3B and 4, Supporting Information Movie 4). Over the subsequent days, the patient noted a marked improvement in symptoms and was discharged home, with continued warfarin and aspirin. The patient returned for repeat right heart catheterization 6 months later. The right atrial pressure had decreased to 14 mm Hg, the pulmonary artery pressure was 67/28 (mean 45) mm Hg, and the wedge pressure was 17 mm Hg (aortic pressure 114/63 mm Hg). The pulmonary vascular resistance had decreased to 5

Fistulas between the left ventricle and coronary sinus are rare, but have been reported many times in the literature. The first mention was in a case report from 1972, describing a fistula that occurred after mitral valve replacement with a Starr-Edwards valve [1], and most reported cases have been subsequent to mitral valve replacement [2]. The proposed mechanisms include a direct stab wound during surgery, or excessive debridement of the posterior wall in the setting of a heavily calcified mitral annulus, which predisposes to spontaneous dissection and eventual fistula formation [1,2]. Other etiologies have been reported, such as after myocardial infarction [4] and after ventricular tachycardia ablation [5]. Congenital cases have been reported as well [3]. Symptoms typically develop due to increased pulmonary pressure and heart failure as a result of left-to-right shunting, as in the case presented here. Typically, left ventricle to coronary sinus fistulas have been treated surgically [1–3]. However, patients have also been treated conservatively, with medical therapy. In the case of a post-infarction fistula reported by Perugini et al., the patient died 2 weeks after discharge [4]. Better outcomes with noninvasive therapy were reported by Caldwell et al., in a case that occurred after ventricular tachycardia ablation, where the patient remained asymptomatic 6 months after the procedure [5]. Mackie et al. reported a case of a fistula that occurred after mitral valve surgery and was treated medically and survived for 16 years [2]. In these cases, the decision whether to proceed with invasive therapy appears to have hinged on the patient’s surgical candidacy. Percutaneous closure represents a less invasive alternative to surgery for left ventricle to coronary sinus fistulas. To the best of our knowledge, this is the first report of percutaneous closure of a left ventricle to coronary sinus fistula. In our case, the patient developed significant pulmonary hypertension during 25 years after the mitral surgery that likely caused the fistula. While closure of her fistula resulted in a marked reduction in pulmonary pressure, the pressure remained elevated, suggesting some irreversible injury. We believe that this argues in favor of early closure for fistulas with a significant left-to-right shunt, particularly given the likely reduced risk of the procedure when compared to surgery. Without the need for sternotomy and general anesthesia, more patients might be considered candidates for treatment, which may improve outcomes. There are no firm guidelines when closure of this type of lesion

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

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should be considered, but we can reasonably extrapolate from the current guidelines regarding other lesions that cause left-to-right shunting. Closure of atrial septal defects, ventricular septal defects, and patent ductus areteriosus, is recommended when there is evidence of chamber enlargement, or for significant net shunting (pulmonary-to-systemic flow ratio > 1.5) [6]. We recommend that the same considerations be applied to fistulas from the left ventricle to the coronary sinus. CONCLUSIONS

We presented the case of a patient who developed a fistula from the left ventricle to the coronary sinus as the result of mitral valve surgery. Due to significant left-to-right shunting, she developed severe pulmonary hypertension and heart failure. Percutaneous closure of the fistula resulted in cessation of shunting and symptom improvement. We suggest that percutaneous closure be considered in similar fistulas when a significant shunt is present, even in cases without symptoms of heart failure or ventricular dysfunction. ACKNOWLEDGMENTS

We acknowledge Michael G. Antimisiaris MD, John D. Grizzard MD, and Brian S. Sestak RT(R), for

assistance with the care of this patient, development of the treatment plan, and preparation of this manuscript. REFERENCES 1. Chambers RJ, Rogers MA. Left ventricle-to-coronary vein fistula following mitral valve replacement. Ann Thorac Surg 1972; 14:305–308. 2. Mackie BD, Clements SJ. Left ventricular to coronary sinus fistula following multiple mitral valve replacement surgeries. J Card Surg 2008;23:65–67. 3. McGarry KM, Stark J, Macartney FJ. Congenital fistula between left ventricle and coronary sinus. Br Heart J 1981;45: 101–104. 4. Perugini E, Sbarzaglia P, Pallotti MG, Pavesi PC, Fattori R, Di Pasquale G. Myocardial rupture with left ventricle to coronary sinus communication: An unusual post-infarction mechanical complication. J Cardiovasc Med 2008;9:97–100. 5. Caldwell J, Johri AM, Baranchuk A, Redfearn D. Left ventricle to coronary sinus fistula following ventricular tachycardia ablation. Heart Rhythm 2013;10:1556–1557. 6. Warnes CA, Williams RG, Bashore TM, Child JS, Connolly HM, Dearani JA, del Nido P, Fasules JW, Graham TP Jr, Hijazi ZM, Hunt SA, King ME, Landzberg MJ, Miner PD, Radford MJ, Walsh EP, Webb GD. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: A report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines for the Management of Adults With Congenital Heart Disease). Circulation 2008;118:e714– e833.

Catheterization and Cardiovascular Interventions DOI 10.1002/ccd. Published on behalf of The Society for Cardiovascular Angiography and Interventions (SCAI).

Percutaneous closure of an iatrogenic fistula from the left ventricle to the coronary sinus.

A patient with a history of rheumatic mitral valve disease and valve replacement in childhood presented with severe, persistent dyspnea. During an ele...
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