Summary
Development of membranous ventricular septal defects (VSD) is a rare complication of transcatheter aortic valve replacements (TAVR), and is recognized using intraoperative and postoperative imaging. We present two cases of this rare but serious complication; one was successfully managed conservatively and the other with valve-in-valve therapy. Management strategies for post-TAVR VSDs varies, but should be individualized to the clinical scenario. We performed a literature search and sought to identify various risk factors which may predispose patients to the development of VSD after TAVR.
Highlights
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Balloon expandable transcatheter valves, relative prosthesis oversizing and LVOT calcification appear to be common predictors of increased risk for development of ventricular septal defect after transcatheter aortic valve replacement.
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Management strategies vary, based on the patient’s clinical status, with seemingly better long term outcomes using early percutaneous closure compared to conservative management.
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Valve-in-valve therapy can be used as a bail-out strategy to seal the defect in a patient with acute hemodynamic instability in select cases.
1
Introduction
Transcatheter aortic valve replacement (TAVR) has emerged as an alternative to surgery in patients with severe aortic stenosis, who are at intermediate, high or prohibitive risk for a traditional surgical aortic valve replacement . Development of a membranous ventricular septal defect (VSD) after TAVR carries a high mortality and the optimal management strategy remains unclear. Reported treatments have included percutaneous closure, surgical repair and conservative management. We present two cases of membranous ventricular septal defects at our institution and a literature review of currently reported cases of post-TAVR VSD from January 2002 until June 2016. Furthermore, we seek to identify potential risk factors which can predispose patients to this rare but potentially fatal complication.
2
Case 1
The patient was an 87-year-old male with severe aortic stenosis and New York Heart Association (NYHA) Class III symptoms that was referred for TAVR. His other medical history included coronary artery disease (CAD), chronic kidney disease (CKD), cerebrovascular accident (CVA) and paroxysmal atrial fibrillation on anticoagulation. Echocardiography revealed normal left ventricular systolic function and severe aortic stenosis with peak and mean aortic valve gradients of 90 mmHg and 58 mmHg, respectively. The calculated aortic valve area was 0.93 cm 2 . Based on multi-slice computer tomography (MSCT), the average annulus diameter was 26 mm with an average area of 530 mm 2 and mild left ventricular outflow tract (LVOT) calcification. Both iliofemoral arteries were suitable for percutaneous TAVR access ( Fig. 1 A and B ). His calculated Society of Thoracic Surgeons (STS) risk score was 6.1%. Despite his intermediate risk score, he was felt to be high risk for a surgical aortic valve replacement due to advanced age and frailty.
The procedure was performed via transfemoral access. The aortic valve was predilated with a Z MED II 22 mm × 5 cm × 100 cm valvuloplasty balloon. A 29 mm Edwards Sapien XT valve (Edwards Lifesciences, Irving CA) was deployed during rapid ventricular pacing ( Fig. 1 C). The procedure was complicated by the development of a left bundle branch block, without requiring a permanent pacemaker. Post procedural echocardiography revealed a well seated valve with a mean gradient of 5 mmHg, a peak gradient of 8 mmHg and a mild paravalvular leak. His post-operative course was unremarkable and he was discharged home after 3 days.
One week after his discharge, he had a brief episode of chest pain and pre-syncope and was found to be in monomorphic ventricular tachycardia (VT) at a rate of 250 beats per minute without hemodynamic compromise. He received an amiodarone bolus in the field and converted to atrial fibrillation prior to transfer to our institution. Transthoracic echocardiography revealed a new ventricular septal defect of 4 mm, with normal left and right ventricular function ( Fig. 1 D, Supplemental Video 1 ). The estimated pulmonary artery pressure was within normal range. Amiodarone was continued and no further episodes of VT were observed. An implantable cardioverter defibrillator was recommended by the electrophysiology team, but the patient preferred conservative management with amiodarone. He was discharged home with a defibrillator vest while he continued his amiodarone load.
At 30 days and 6-month follow-up visits, he was doing well and reported NYHA Class II symptoms. He had no further hospitalizations for heart failure and no documented arrhythmias. Thirty-day follow-up transthoracic echocardiogram noted the VSD to be stable in size with a left to right shunt. The right ventricle remained normal in size and function with normal pulmonary artery pressures. As a result, it was decided not to proceed with percutaneous closure. Patient is now 2 years out of his initial procedure and doing well, with minimal residual VSD.
2
Case 1
The patient was an 87-year-old male with severe aortic stenosis and New York Heart Association (NYHA) Class III symptoms that was referred for TAVR. His other medical history included coronary artery disease (CAD), chronic kidney disease (CKD), cerebrovascular accident (CVA) and paroxysmal atrial fibrillation on anticoagulation. Echocardiography revealed normal left ventricular systolic function and severe aortic stenosis with peak and mean aortic valve gradients of 90 mmHg and 58 mmHg, respectively. The calculated aortic valve area was 0.93 cm 2 . Based on multi-slice computer tomography (MSCT), the average annulus diameter was 26 mm with an average area of 530 mm 2 and mild left ventricular outflow tract (LVOT) calcification. Both iliofemoral arteries were suitable for percutaneous TAVR access ( Fig. 1 A and B ). His calculated Society of Thoracic Surgeons (STS) risk score was 6.1%. Despite his intermediate risk score, he was felt to be high risk for a surgical aortic valve replacement due to advanced age and frailty.
The procedure was performed via transfemoral access. The aortic valve was predilated with a Z MED II 22 mm × 5 cm × 100 cm valvuloplasty balloon. A 29 mm Edwards Sapien XT valve (Edwards Lifesciences, Irving CA) was deployed during rapid ventricular pacing ( Fig. 1 C). The procedure was complicated by the development of a left bundle branch block, without requiring a permanent pacemaker. Post procedural echocardiography revealed a well seated valve with a mean gradient of 5 mmHg, a peak gradient of 8 mmHg and a mild paravalvular leak. His post-operative course was unremarkable and he was discharged home after 3 days.
One week after his discharge, he had a brief episode of chest pain and pre-syncope and was found to be in monomorphic ventricular tachycardia (VT) at a rate of 250 beats per minute without hemodynamic compromise. He received an amiodarone bolus in the field and converted to atrial fibrillation prior to transfer to our institution. Transthoracic echocardiography revealed a new ventricular septal defect of 4 mm, with normal left and right ventricular function ( Fig. 1 D, Supplemental Video 1 ). The estimated pulmonary artery pressure was within normal range. Amiodarone was continued and no further episodes of VT were observed. An implantable cardioverter defibrillator was recommended by the electrophysiology team, but the patient preferred conservative management with amiodarone. He was discharged home with a defibrillator vest while he continued his amiodarone load.
At 30 days and 6-month follow-up visits, he was doing well and reported NYHA Class II symptoms. He had no further hospitalizations for heart failure and no documented arrhythmias. Thirty-day follow-up transthoracic echocardiogram noted the VSD to be stable in size with a left to right shunt. The right ventricle remained normal in size and function with normal pulmonary artery pressures. As a result, it was decided not to proceed with percutaneous closure. Patient is now 2 years out of his initial procedure and doing well, with minimal residual VSD.
3
Case 2
The patient was a 90-year-old female with severe aortic stenosis who was admitted to our institution after an episode of syncope and a small left frontal subarachnoid hemorrhage. She had a history of atrial fibrillation on anti-coagulation, stroke, and rheumatoid arthritis on daily prednisone therapy. Her baseline transthoracic echocardiogram revealed normal left ventricular systolic function with severely restricted aortic valve leaflets. The right ventricle was mildly dilated with mildly reduced systolic function. The peak transaortic velocity was 4.4 m/s with peak and mean gradients of 77 mmHg and 45 mmHg, respectively. Her CT scan revealed an average annular diameter of 25 mm and area of 498 mm 2 . There was significant aortic annular calcification with extension into the anterior mitral leaflet, anteromedial LVOT and interventricular septum ( Fig. 2 A and B ). Both iliofemoral arteries were suitable for transfemoral TAVR access. Her calculated STS risk score was 8.1% and not a surgical candidate.
Two months after her initial hospitalization, she underwent placement of a 26 mm Edwards Sapien THV (Edwards Lifesciences, Irving CA) via the right common femoral artery after predilatation with a Z MED II-X 22 mm × 5 cm × 100 cm balloon. The valve was post-dilated due to mild–moderate paravalvular regurgitation, which resulted in hypotension requiring epinephrine and norepinephrine infusions. Post deployment ventriculogram revealed a VSD with a passage of contrast from the left to right ventricle ( Fig. 2 C, Supplementary Video 2 ). TEE confirmed the presence of a large membranous VSD with left to right shunting associated with severe right ventricular dilatation and dysfunction ( Fig. 3 A and B , Supplementary Video 3 ). Pulmonary artery saturation increased to 91%, suggesting a significant left to right shunt. Given her rapidly deteriorating hemodynamics, a decision was made to deploy a second valve further into the LVOT in an attempt to seal the septal defect. A second 26 mm Edwards Sapien THV valve was deployed and post-dilated. Pulmonary artery saturation improved to 78% along with a marked decrease in pulmonary artery pressures. After deployment of the second valve, TEE documented initial improvement in the shunt flow across the ventricular septum, but shortly thereafter the VSD was seen extending into the muscular septum ( Fig. 4 A and B , Supplementary Video 4 ). The TEE continued to demonstrate a moderate sized defect in the muscular septum, however the right ventricle was only moderately enlarged and with moderate systolic dysfunction. Ventriculography also confirmed the persistent VSD ( Fig. 2 D, Supplementary Video 5 ). An intra-aortic balloon pump was inserted via the left common femoral artery for added hemodynamic support. Over the next three days, despite supportive care, she developed multiorgan system failure and sepsis. After a long discussion with her family, the patient was transitioned to comfort measures and she expired on post-operative day 3.
4
Discussion
With the growing popularity of TAVR, it is important to recognize the potential complications involved with this procedure. The incidence of VSD after TAVR is rare (1%), but is associated with a high mortality rate . We present two cases of membranous VSD’s that demonstrate the varying time course of diagnosis and hemodynamic consequences. The underlying etiology is speculated to be direct trauma to the annulus and adjacent structures from the deployed valve, resulting in focal annular rupture. The injury can have a variety of manifestations, including extension into the RV outflow tract , right atrium and/or the left atrium .
We were able to identify 25 reports of iatrogenic VSD after TAVR . Tables 1 and 2 give an overview of the patient characteristics, procedural details, and clinical outcomes, along with possible risk factors which may have contributed to the development of a VSD. Our 2 cases are included in the tables. The majority of cases were female (60%) with a median age of 86 (range 57–93) years. All patients with available details had associated cardiac and/or noncardiac comorbidities with an overall high STS (9.4 ± 4.2, n = 11) mortality risk or Logistic EuroSCOREs (19.9 ± 7.4, n = 11). Two cases were part of the PARTNER intermediate risk registry . Two cases, including one of our cases underwent TAVR due to advanced age and high index of frailty . Point of access was predominantly transfemoral (83%) and there was a marked predominance of balloon-expandable valves compared to self-expandable valves (88% vs. 12%, respectively). Pre-dilation and post-dilation data was available for 13 cases only. Pre-dilation was performed in 12 cases while post-dilation was performed in 5 out of 11 reported cases.
| Prosthesis | PUBLICATION AUTHOR | AGE/Sex | CO-MORBID CONDITIONS | STS risk Score (%); LOGISTIC EUROSCORE | LVEF – Echo | Annulus Size – CT (mm) | Annular Area – CT (mm 2 ) | LVOT Calcification Severity |
|---|---|---|---|---|---|---|---|---|
| Balloon-Expandable | Massabuau, Dumonteil et al. 2011 8 | 86/M | CAD s/p CABG 26 years ago, HLD, prostate cancer | 7; 28 | 56 | NR | NR | NR |
| Barbanti et al. 2013 | NR | NR | NR | NR | 25.1 × 21.1 | 397 | mild | |
| Aminian, Lalm and et al. 2013 | 85/F | CAD s/p PCI’s, CKD, bilateral carotid artery stenosis | NR; 18.5 | 65 | NR | NR | NR | |
| Rodgers, Gobeil et al. 2014 | 82/F | NR | NR; NR | 60 | 17 × 20 | 329 | severe | |
| Baruteau and Petit 2014 | 89/M | AF, CKD | NR; NR | NR | NR | NR | NR | |
| Salizzoni, Marra et al. 2014 | 88/F | CHF | 10.9; 26.64 | NR | NR | 450 | severe | |
| Yanes-Bowden, Bosa-Ojeda et al. 2014 | 87/M | HTN, COPD, bilateral knee replacements, CHF (NYHA III) | NR;13.92 | mild dysfunction | 21 × 23 | NR | NR | |
| Patel, Vassileva et al. 2014 | 85/F | HTN, HLD, CAD s/p CABG in 2000 | >10; NR | NR | NR | NR | NR | |
| Patel, Vassileva et al. 2014 | 89/F | HTN,DM, HLD, hypothyroidism, CAD s/p CABG | 16; NR | NR | NR | NR | NR | |
| Patel, Vassileva et al. 2014 | 88/F | DM, HTN, PAH, AF, OSA, TIA, CKD(Stage 4) | 16; NR | NR | NR | NR | NR | |
| Garrido, Ferreiro et al. 2014 | 79/F | HTN, AF, respiratory dysfunction (on home oxygen), CHF (NYHA III-IV) | NR; 11.66 | NR | NR | NR | moderate | |
| Mauri, Aldebert et al. 2014 | 78/F | CHF (NYHA III) | NR; NR | 56 | NR | NR | NR | |
| Dursun, Erdal et al. 2015 | 73/F | NR | NR; 33.8 | NR | NR | NR | mild | |
| Levi, Salem et al. 2015 | 57/F | Hodgkins Lymphoma s/p radiation at 16 years of age, Mitral valve replacement | NR; NR | NR | NR | NR | NR | |
| Shakoor, Islam et al. 2015 | 89/M | severe COPD, Pulmonary fibrosis, TIA, Laryngeal carcinoma s/p resection and radiation therapy, CAD s/p CABG, s/p AV balloon valvuloplasty in 2013 | NR; NR | 60 | 26 × 28 | NR | NR | |
| Mark, Prasanna et al. 2015 | 89/M | NR | 5.6; NR | NR | NR | NR | NR | |
| Lee, Hegde et al. 2015 22 | 80/F | severe COPD | NR; NR | 60 | NR | NR | NR | |
| Rene, Jagasia et al. 2016 | 89/M | AF, moderate MR, myelodysplastic syndrome, gastric lymphoma s/p chemo and radiation 5 years prior to presentation | 5.7; NR | 55 | 24 × 30 | 546 | Severe | |
| Rene, Jagasia et al. 2016 | 86/F | HTN, CKD Stage III, COPD, ankylosing spondylitis | 4.8; NR | 60 | 23 × 28 | 552 | Moderate | |
| Rene, Jagasia et al. 2016 | 82/F | CAD, AF, severe COPD, previous AAA repair | 14.2; NR | 60 | 26.2 × 29 | 574 | Severe | |
| Rene, Jagasia et al. 2016 | 93/M | AF, CHF, HTN, CKD, DVT, myeloproliferative disorder | 9.37; NR | 70 | 18.6 × 27 | 460 | Severe | |
| OUR CASE 1 | 87/M | CAD, CKD, CVA, paroxysmal Atrial fibrillation on anticoagulation, CHF (NYHA III) | 6.1;17.5 | 60 | 23.6 × 29 | 530 | Mild | |
| OUR CASE 2 | 90/F | AF, CVA, rheumatoid arthritis on daily prednisone | 8.1;15.8 | 68 | 23 × 25 | 498 | Severe | |
| Self-Expanding | Tzikas, Schultz et al. 2009 | 89/M | None | NR; 15 | 55 | NR | NR | NR |
| Revilla Martinez, Gutierrez Garcia et al. 2012 | 76/F | COPD, HTN | NR; 17 | NR | NR | NR | moderate | |
| Gerckens, Latsios et al. 2013 | 86/M | Chronic Atrial Fibrillation, CKD, CHF (NYHA IV) | NR; 21 | 40 | 27.2 | NR | severe | |
| NR | Al-Attar, Ghodbane et al. 2009 | NR | NR | NR; NR | NR | NR | NR | NR |
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