Cardiomyopathy in Marfan syndrome

European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery
Roland HetzerEva Maria Delmo Walter

Abstract

This report aims to evaluate the existence of primary and secondary cardiomyopathy in patients with Marfan syndrome (MFS) who underwent surgical management for primary cardiovascular sequelae of this genetic disorder. Likewise, we aim to determine whether the myocardium in MFS is susceptible to ischaemia independent of myocardial protection used during surgery. Between April 1986 and May 2012, 421 patients with MFS were surgically treated for cardiovascular manifestations. Among them, 47 (mean age: 39.45 ± 12.64, median: 36, range: 19-66, years) eventually were surgically treated for cardiomyopathy. They were grouped into A: patients who subsequently developed ischaemic cardiomyopathy and eventually underwent coronary revascularization for coronary artery disease (n = 11); B: patients who subsequently developed end-stage cardiomyopathy for which a mechanical circulatory support device was implanted to support the failing heart (n = 13) and C: patients who subsequently developed end-stage cardiomyopathy (n = 23), among whom 21 underwent primary heart transplantation, while 2 patients are still waiting for donor hearts. Retrospective analysis of the medical records of 47 patients revealed the following: In Group A, 3 (27.2%) pati...Continue Reading

References

Feb 1, 1989·The American Journal of Cardiology·M J RomanM C Spitzer
Mar 1, 1985·American Journal of Diseases of Children·S ChenJ L Donahoe
Jan 15, 1995·The American Journal of Cardiology·D I SilvermanP Tsipouras
May 1, 1994·European Heart Journal·A SavolainenM Kupari
Dec 1, 1996·The Annals of Thoracic Surgery·J C MullenM J Bentley
Apr 25, 2000·Annual Review of Medicine·R E Pyeritz
Sep 25, 2002·Clinical Cardiology·Maria Cristinna PorcianiGian Franco Gensini
Jan 22, 2003·Journal of the American College of Cardiology·Anji T YetmanBrian W McCrindle
Apr 2, 2003·The American Journal of Cardiology·Ritu ChatrathDavid J Driscoll
Mar 11, 2005·The American Journal of Cardiology·Lilian J MeijboomBarbara J M Mulder
Nov 8, 2005·American Journal of Medical Genetics. Part C, Seminars in Medical Genetics·Harry C DietzFrancesco Ramirez
Dec 1, 2005·International Journal of Cardiology·Julie F De BackerJohan De Sutter
Jan 31, 2007·Clinical Cardiology·Meike RybczynskiYskert von Kodolitsch
Apr 28, 2007·The Annals of Thoracic Surgery·Christoph KnosallaRoland Hetzer
May 1, 2007·Current Opinion in Genetics & Development·Francesco Ramirez, Harry C Dietz

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Citations

Jun 22, 2018·Histochemistry and Cell Biology·Felke SteijnsMarjolijn Renard
May 18, 2019·European Journal of Cardio-thoracic Surgery : Official Journal of the European Association for Cardio-thoracic Surgery·Eva Maria Javier DelmoRoland Hetzer
Jan 26, 2020·International Journal of Molecular Sciences·Quynh NguyenToshifumi Yokota
Sep 30, 2020·International Journal of Molecular Sciences·Felke SteijnsPatrick Sips
Dec 24, 2018·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Reed E Pyeritz
Oct 9, 2020·Scientific Reports·Jeffrey AaldersJolanda van Hengel
Oct 25, 2020·Orphanet Journal of Rare Diseases·Laura Muiño-MosqueraJulie De Backer
Jul 1, 2020·Current Problems in Cardiology·Michael P SeitzYenal Harper
Apr 23, 2021·Clinical Research in Cardiology : Official Journal of the German Cardiac Society·Niels H AndersenKirstine Stochholm
Dec 12, 2020·The Annals of Thoracic Surgery·Hao XuHongjia Zhang
Aug 3, 2021·Frontiers in Pediatrics·Laura Muiño-Mosquera, Julie De Backer
Aug 20, 2021·Journal of Experimental Pharmacology·Violette DeleeuwPatrick Sips

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