Case Reports
Retrograde Percutaneous Closure of a Ventricular Septal Defect after Myectomy for Hypertrophic Obstructive Cardiomyopathy
Vikas Singh, MD Apurva O. Badheka, MD Syed S. Bokhari, MD Eduard Ghersin, MD Pedro Martinez Clark, MD William W. O’Neill, MD
Key words: Cardiac catheterization/methods; cardiomyopathy, hypertrophic/therapy; heart septal defects, ventricular/therapy; iatrogenic disease; myectomy; postoperative complications; prosthesis implantation/methods; septal occluder device; ventricular septal defect Drs. Singh and Badheka contributed equally to this manuscript. From: Cardiovascular Division, University of Miami Hospital, Miller School of Medicine, Miami, Florida 33136 Dr. O’Neill is now at the Center for Structural Heart Disease, Henry Ford Hospital, Detroit, Michigan. Address for reprints: Vikas Singh, MD, University of Miami Hospital, 1400 NW 12th Ave., Suite 1179, Miami, FL 33136 E-mail:
[email protected]
© 2013 by the Texas Heart ® Institute, Houston
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In patients with hypertrophic obstructive cardiomyopathy, hemodynamically significant ventricular septal defect after septal myectomy is a rare sequela that warrants closure. Percutaneous closure provides a safer alternative to repeated sternotomy, which is associated with significant morbidity and mortality rates. We report a possibly unique case of successful retrograde percutaneous closure, with an AMPLATZER Muscular VSD Occluder, of an iatrogenic ventricular septal defect consequent to surgical therapy for hypertrophic obstructive cardiomyopathy. (Tex Heart Inst J 2013;40(4):468-71)
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atients with hypertrophic obstructive cardiomyopathy (HOCM) are candidates for surgical myectomy or ethanol ablation if, despite medical therapy, they remain symptomatic with New York Heart Association functional class III disease and severe left ventricular outflow tract (LVOT) gradients.1 A rare sequela of myectomy via the Morrow procedure is iatrogenic ventricular septal defect (VSD). Although surgical closure remains the mainstay of treatment for most VSDs, treatment of clinically significant postoperative residual VSDs remains a challenge. Percutaneous closure provides a safer alternative to reoperation.2,3 We report a possibly unique case of successful retrograde percutaneous closure of an iatrogenic VSD, consequent to surgical therapy for HOCM, by means of an AMPLATZER® Muscular VSD Occluder (St. Jude Medical, Inc.; St. Paul, Minn).
Case Report A 68-year-old woman presented at another medical center after experiencing a syncopal episode while walking. She had a history of hypertension, dyslipidemia, coronary artery disease (including drug-eluting stent placement to the left circumflex coronary artery), and medically managed HOCM. Evaluation revealed asymmetric left ventricular (LV) hypertrophy with LVOT obstruction and moderate-to-severe mitral valve regurgitation. The patient underwent a septal myectomy, bileaflet mechanical mitral valve replacement, and tricuspid annuloplasty; however, soon after these procedures, she developed progressively worsening dyspnea and bilateral leg edema. During the next few weeks, she underwent multiple thoracenteses to relieve recurrent transudative pleural effusions. Two-dimensional (2D) echocardiograms, transesophageal echocardiograms, and cardiac computed tomograms revealed a muscular VSD 0.7 cm in size, associated with a significant left-to-right shunt. The patient was referred to our facility for percutaneous closure of the VSD (Figs. 1 and 2A). First Attempt
Using a Cook needle, we cannulated the left femoral vein and advanced a 10F intracardiac echocardiographic catheter (SOUNDSTAR¥, Biosense-Webster Inc.; Diamond Bar, Calif ) into the right atrium. We then cannulated the left femoral artery and inserted a 7F sheath. Subsequently, we advanced a Judkins right 4 (JR4) diagnostic catheter into the LV. Using the guidance from the JR4 catheter, we advanced a Wholey wire (Mallinckrodt Inc.; Hazelwood, Mo) from the LV to the right ventricle (RV), through the VSD. Attempts to advance the wire through the tricuspid valve into the right atrium were unsuccessful because of the presence of the annuloplasty
Retrograde Percutaneous VSD Closure
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ring. The procedure was then aborted, and all guidewires and catheters were removed.
A
Second Attempt
After 48 hours, the patient was taken to the cardiac catheterization laboratory again with the intent of closing the VSD in a retrograde fashion, after the failed antegrade attempt. We obtained right radial artery access by using an argon needle and advanced a 6F pigtail catheter into the LV to perform a left ventriculogram. We cannulated the right femoral artery and inserted a 7F sheath. The JR4 catheter and the Wholey wire were subsequently advanced into the LV and then through the VSD to the RV. Simultaneous measurement of RV
A B
B
Fig. 2 Two-dimensional color-flow Doppler echocardiograms (4-chamber views) show A) a ventricular septal defect with large left-to-right flow (arrow), and B) no flow between the left and right ventricles after closure of the defect.
Fig. 1 Electrocardiographic-gated multidetector computed tomograms (4-chamber views) of the heart show a 0.7-cm ventricular septal defect (VSD) (arrows) at the membranous septum during A) end-diastole and B) peak systole. A jet of dense intravenous contrast medium (arrowheads), from the relatively hyperdense left ventricular lumen through the VSD toward the relatively hypodense right ventricular lumen, indicates a left-toright shunt.
Texas Heart Institute Journal
and LV pressures showed the LV pressure to be 118 mmHg, with an end-diastolic pressure of 16 mmHg, and the RV pressure to be 44 mmHg, with an end-diastolic pressure of 16 mmHg. We then exchanged the 7F arterial sheath for an 8F delivery sheath, which we advanced across the aortic valve into the LV, then through the VSD into the RV. A 12-mm AMPLATZER VSD closure device was advanced and deployed under real-time fluoroscopy and transthoracic echocardiography. Twodimensional echocardiography and repeat left ventriculography confirmed that there was no residual VSD (Figs. 2B and 3). We removed the sheath and achieved Retrograde Percutaneous VSD Closure
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Fig. 3 Repeat left ventriculogram shows resolution of the ventricular septal defect.
hemostasis at the arteriotomy site with an 8F AngioSeal¥ closure device (St. Jude Medical). A transradial band was applied to the right radial site.
Discussion Ventricular septal defect, a rare sequela of septal myectomy, is reported in 1% of HOCM patients who undergo this procedure.4 The presence of a hemodynamically significant VSD with left-to-right shunting can result in LV volume overload with signs and symptoms of heart failure.5 Closure of the VSD is needed to prevent sequelae such as LV dysfunction, arrhythmias, aortic regurgitation, recurrent endocarditis, and pulmonary hypertension. Surgical closure of VSDs, first performed in 1954, has long been considered the gold-standard treatment. However, surgical closure that involves cardiopulmonary bypass and sternotomy is associated with significant morbidity and mortality rates, especially in adults who have undergone prior sternotomy.6,7 Percutaneous device closure of congenital or iatrogenic VSDs has been performed since the late 1980s with reasonable success in both pediatric and adult populations.3,8,9 The procedure is technically challenging and can cause rhythm disturbances or damage the tricuspid valve. Moreover, conduction tissue can run near the edges of the VSD and be damaged by manipulation of the device. This risk is of greater magnitude if the VSD is located near the aortic or tricuspid valve, in which case device placement can result in temporary or permanent atrioventricular block.2,3 Transcatheter closure of postoperative VSDs has been described chiefly in patients who have had residual VSDs after primary VSD closure. These cases are substantially distinct from those involving VSD after myectomy for HOCM, in 470
Retrograde Percutaneous VSD Closure
which instances the defect is surrounded by relatively thick myocardium—depending on the amount of myocardial tissue excised during surgery. An extensive literature review revealed only 4 reported cases of iatrogenic VSD after septal myectomy, which were treated with a transcatheter technique.3,9-11 However, in all these cases the occluder device was delivered antegrade via the femoral vein or internal jugular vein. To our knowledge, ours is the first reported case of a successful percutaneous VSD closure after surgical therapy for HOCM wherein the AMPLATZER occluder device was delivered retrograde through the femoral artery. The presence of the annuloplasty ring in the tricuspid valve made it difficult to achieve an arteriovenous loop or to advance the catheter in an antegrade fashion. This report adds to the increasing evidence of the AMPLATZER Muscular VSD Occluder’s suitability for treatment of a postsurgical VSD after septal myectomy in patients with HOCM. Salient features of this case include a failed antegrade attempt at closure of a septal myectomy VSD, use of radial access for left ventriculography, and retrograde closure of the defect.
References 1. Maron BJ, McKenna WJ, Danielson GK, Kappenberger LJ, Kuhn HJ, Seidman CE, et al. American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. J Am Coll Cardiol 2003;42(9):1687-713. 2. Okubo M, Benson LN, Nykanen D, Azakie A, Van Arsdell G, Coles J, Williams WG. Outcomes of intraoperative device closure of muscular ventricular septal defects. Ann Thorac Surg 2001;72(2):416-23. 3. Chessa M, Carminati M, Cao QL, Butera G, Giusti S, Bini RM, Hijazi ZM. Transcatheter closure of congenital and acquired muscular ventricular septal defects using the Amplatzer device. J Invasive Cardiol 2002;14(6):322-7. 4. Schonbeck MH, Brunner-La Rocca HP, Vogt PR, Lachat ML, Jenni R, Hess OM, Turina MI. Long-term follow-up in hypertrophic obstructive cardiomyopathy after septal myectomy. Ann Thorac Surg 1998;65(5):1207-14. 5. Carminati M, Butera G, Chessa M, De Giovanni J, Fisher G, Gewillig M, et al. Transcatheter closure of congenital ventricular septal defects: results of the European Registry. Eur Heart J 2007;28(19):2361-8. 6. Holzer R, Balzer D, Cao QL, Lock K, Hijazi ZM; Amplatzer Muscular Ventricular Septal Defect Investigators. Device closure of muscular ventricular septal defects using the Amplatzer muscular ventricular septal defect occluder: immediate and mid-term results of a U.S. registry. J Am Coll Cardiol 2004;43(7):1257-63. 7. Rose EA. Off-pump coronary-artery bypass surgery. N Engl J Med 2003;348(5):379-80. 8. Knauth AL, Lock JE, Perry SB, McElhinney DB, Gauvreau K, Landzberg MJ, et al. Transcatheter device closure of congenital and postoperative residual ventricular septal defects. Circulation 2004;110(5):501-7.
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9. Al-Kashkari W, Balan P, Kavinsky CJ, Cao QL, Hijazi ZM. Percutaneous device closure of congenital and iatrogenic ventricular septal defects in adult patients. Catheter Cardiovasc Interv 2011;77(2):260-7. 10. De Wolf D, Taeymans Y, Suys B, Matthys D, Van Nooten G. Percutaneous closure of a ventricular septal defect after surgical treatment of hypertrophic cardiomyopathy. J Thorac Cardiovasc Surg 2006;132(1):173-4. 11. Spies C, Ujivari F, Schraeder R. Transcatheter closure of a ventricular septal defect following myectomy for hypertrophic obstructive cardiomyopathy. Cardiology 2009;112(1):31-4.
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