Saurabh Kumar1, Alexander F A Androulakis1, Jean-Marc Sellal1, Philippe Maury1, Estelle Gandjbakhch1, Xavier Waintraub1, Anne Rollin1, Pascale Richard1, Philippe Charron1, Samuel H Baldinger1, Ciorsti J Macintyre1, Bruce A Koplan1, Roy M John1, Gregory F Michaud1, Katja Zeppenfeld1, Frederic Sacher1, Neal K Lakdawala1, William G Stevenson1, Usha B Tedrow2. 1. From the Cardiovascular Division, Brigham and Women's Hospital, Boston, MA (S.K., S.H.B., B.A.K., R.M.J., G.F.M., N.K.L., W.G.S., U.B.T.); Department of Cardiology, Leiden University Medical Centre, The Netherlands (A.F.A.A., K.Z.); Hôpital Cardiologique du Haut-Lévêque (CHU), Bordeaux-Pessac & L'Institut de RYthmologie et Modélisation Cardiaque (LIRYC), Institut Hospitalo-Universitaire (IHU), Bordeaux, France (J.-M.S., F.S.); Centre Hospitalier Universitaire de Nancy (J.-M.S.); Toulouse University Hospital, Rangueil, Toulouse (P.M., A.R.); Département de Cardiologie, Hôpital Pitié-Salpêtrière, AP-HP (J.-M.S., F.S., E.G., X.W.); Cardio-myogenetic, Department of Biochimie and INSERM U582, University Hospital Pitié-Salpêtrière, AP-HP (P.R.); Centre de Référence Maladies Cardiaques Héréditaires, ICAN, Hôpital Pitié-Salpêtrière, Paris (P.C.); and Université de Versailles-Saint Quentin, Hôpital Ambroise Paré, AP-HP, Boulogne-Billancourt, France (P.C.). 2. From the Cardiovascular Division, Brigham and Women's Hospital, Boston, MA (S.K., S.H.B., B.A.K., R.M.J., G.F.M., N.K.L., W.G.S., U.B.T.); Department of Cardiology, Leiden University Medical Centre, The Netherlands (A.F.A.A., K.Z.); Hôpital Cardiologique du Haut-Lévêque (CHU), Bordeaux-Pessac & L'Institut de RYthmologie et Modélisation Cardiaque (LIRYC), Institut Hospitalo-Universitaire (IHU), Bordeaux, France (J.-M.S., F.S.); Centre Hospitalier Universitaire de Nancy (J.-M.S.); Toulouse University Hospital, Rangueil, Toulouse (P.M., A.R.); Département de Cardiologie, Hôpital Pitié-Salpêtrière, AP-HP (J.-M.S., F.S., E.G., X.W.); Cardio-myogenetic, Department of Biochimie and INSERM U582, University Hospital Pitié-Salpêtrière, AP-HP (P.R.); Centre de Référence Maladies Cardiaques Héréditaires, ICAN, Hôpital Pitié-Salpêtrière, Paris (P.C.); and Université de Versailles-Saint Quentin, Hôpital Ambroise Paré, AP-HP, Boulogne-Billancourt, France (P.C.). utedrow@partners.org westpennant@gmail.com.
Abstract
BACKGROUND: Lamin A/C (LMNA) cardiomyopathy is a genetic disease with a proclivity for ventricular arrhythmias. We describe the multicenter experience with percutaneous catheter ablation of sustained monomorphic ventricular tachycardia (VT) in LMNA cardiomyopathy. METHODS AND RESULTS: Twenty-five consecutive LMNA mutation patients from 4 centers were included (mean age, 55±9 years; ejection fraction, 34±12%; VT storm in 36%). Complete atrioventricular block was present in 11 patients; 3 patients were on mechanical circulatory support for severe heart failure. A median of 3 VTs were inducible per patient; in 82%, mapping was consistent with origin from scar in the basal left ventricle, particularly the septum, but also basal inferior wall and subaortic mitral continuity. After multiple procedures (median 2/patient; transcoronary alcohol in 6 and surgical cryoablation in 2 patients), acute success (noninducibility of any VT) was achieved in only 25% of patients. Partial success (inducibility of a nonclinical VT only: 50%) and failure (persistent inducibility of clinical VT: 12.5%) was attributed to intramural septal substrate in 13 of 18 patients (72%). Complications occurred in 25% of patients. After a median follow-up of 7 months after the last procedure, 91% experienced ≥1 VT recurrence, 44% received or were awaiting mechanical circulatory support or transplant for end-stage heart failure, and 26% died. CONCLUSIONS: Catheter ablation of VT associated with LMNA cardiomyopathy is associated with poor outcomes including high rate of arrhythmia recurrence, progression to end-stage heart failure, and high mortality. Basal septal scar and intramural VT origin makes VT ablation challenging in this population.
BACKGROUND:Lamin A/C (LMNA) cardiomyopathy is a genetic disease with a proclivity for ventricular arrhythmias. We describe the multicenter experience with percutaneous catheter ablation of sustained monomorphic ventricular tachycardia (VT) in LMNAcardiomyopathy. METHODS AND RESULTS: Twenty-five consecutive LMNA mutation patients from 4 centers were included (mean age, 55±9 years; ejection fraction, 34±12%; VT storm in 36%). Complete atrioventricular block was present in 11 patients; 3 patients were on mechanical circulatory support for severe heart failure. A median of 3 VTs were inducible per patient; in 82%, mapping was consistent with origin from scar in the basal left ventricle, particularly the septum, but also basal inferior wall and subaortic mitral continuity. After multiple procedures (median 2/patient; transcoronary alcohol in 6 and surgical cryoablation in 2 patients), acute success (noninducibility of any VT) was achieved in only 25% of patients. Partial success (inducibility of a nonclinical VT only: 50%) and failure (persistent inducibility of clinical VT: 12.5%) was attributed to intramural septal substrate in 13 of 18 patients (72%). Complications occurred in 25% of patients. After a median follow-up of 7 months after the last procedure, 91% experienced ≥1 VT recurrence, 44% received or were awaiting mechanical circulatory support or transplant for end-stage heart failure, and 26% died. CONCLUSIONS: Catheter ablation of VT associated with LMNAcardiomyopathy is associated with poor outcomes including high rate of arrhythmia recurrence, progression to end-stage heart failure, and high mortality. Basal septal scar and intramural VT origin makes VT ablation challenging in this population.
Authors: Edmond M Cronin; Frank M Bogun; Philippe Maury; Petr Peichl; Minglong Chen; Narayanan Namboodiri; Luis Aguinaga; Luiz Roberto Leite; Sana M Al-Khatib; Elad Anter; Antonio Berruezo; David J Callans; Mina K Chung; Phillip Cuculich; Andre d'Avila; Barbara J Deal; Paolo Della Bella; Thomas Deneke; Timm-Michael Dickfeld; Claudio Hadid; Haris M Haqqani; G Neal Kay; Rakesh Latchamsetty; Francis Marchlinski; John M Miller; Akihiko Nogami; Akash R Patel; Rajeev Kumar Pathak; Luis C Saenz Morales; Pasquale Santangeli; John L Sapp; Andrea Sarkozy; Kyoko Soejima; William G Stevenson; Usha B Tedrow; Wendy S Tzou; Niraj Varma; Katja Zeppenfeld Journal: J Interv Card Electrophysiol Date: 2020-10 Impact factor: 1.900
Authors: Edmond M Cronin; Frank M Bogun; Philippe Maury; Petr Peichl; Minglong Chen; Narayanan Namboodiri; Luis Aguinaga; Luiz Roberto Leite; Sana M Al-Khatib; Elad Anter; Antonio Berruezo; David J Callans; Mina K Chung; Phillip Cuculich; Andre d'Avila; Barbara J Deal; Paolo Della Bella; Thomas Deneke; Timm-Michael Dickfeld; Claudio Hadid; Haris M Haqqani; G Neal Kay; Rakesh Latchamsetty; Francis Marchlinski; John M Miller; Akihiko Nogami; Akash R Patel; Rajeev Kumar Pathak; Luis C Sáenz Morales; Pasquale Santangeli; John L Sapp; Andrea Sarkozy; Kyoko Soejima; William G Stevenson; Usha B Tedrow; Wendy S Tzou; Niraj Varma; Katja Zeppenfeld Journal: Europace Date: 2019-08-01 Impact factor: 5.214
Authors: Jens Cosedis Nielsen; Yenn-Jiang Lin; Marcio Jansen de Oliveira Figueiredo; Alireza Sepehri Shamloo; Alberto Alfie; Serge Boveda; Nikolaos Dagres; Dario Di Toro; Lee L Eckhardt; Kenneth Ellenbogen; Carina Hardy; Takanori Ikeda; Aparna Jaswal; Elizabeth Kaufman; Andrew Krahn; Kengo Kusano; Valentina Kutyifa; Han S Lim; Gregory Y H Lip; Santiago Nava-Townsend; Hui-Nam Pak; Gerardo Rodríguez Diez; William Sauer; Anil Saxena; Jesper Hastrup Svendsen; Diego Vanegas; Marmar Vaseghi; Arthur Wilde; T Jared Bunch; Alfred E Buxton; Gonzalo Calvimontes; Tze-Fan Chao; Lars Eckardt; Heidi Estner; Anne M Gillis; Rodrigo Isa; Josef Kautzner; Philippe Maury; Joshua D Moss; Gi-Byung Nam; Brian Olshansky; Luis Fernando Pava Molano; Mauricio Pimentel; Mukund Prabhu; Wendy S Tzou; Philipp Sommer; Janice Swampillai; Alejandro Vidal; Thomas Deneke; Gerhard Hindricks; Christophe Leclercq Journal: Europace Date: 2020-08-01 Impact factor: 5.214
Authors: Gabriella Captur; Eloisa Arbustini; Petros Syrris; Dina Radenkovic; Ben O'Brien; William J Mckenna; James C Moon Journal: Open Heart Date: 2018-10-25
Authors: Mashael Alfarih; Petros Syrris; Eloisa Arbustini; João B Augusto; Alun Hughes; Guy Lloyd; Luis R Lopes; James C Moon; Saidi Mohiddin; Gabriella Captur Journal: Acta Myol Date: 2019-09-01