Letter by Baruteau et al Regarding Article, “Peripartum Cardiomyopathy as a Part of Familial Dilated Cardiomyopathy”

Abstract

HomeCirculationVol. 123, No. 2Letter by Baruteau et al Regarding Article, “Peripartum Cardiomyopathy as a Part of Familial Dilated Cardiomyopathy” Free AccessLetterPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessLetterPDF/EPUBLetter by Baruteau et al Regarding Article, “Peripartum Cardiomyopathy as a Part of Familial Dilated Cardiomyopathy” Alban-Elouen Baruteau, MD, Erwan Donal, MD, PhD and Jean-Claude Daubert, MD, PhD Alban-Elouen BaruteauAlban-Elouen Baruteau CHU RennesService de Cardiologie et Maladies VasculairesUniversité de Rennes 1Rennes, France (Baruteau, Donal, Daubert) Search for more papers by this author , Erwan DonalErwan Donal CHU RennesService de Cardiologie et Maladies VasculairesUniversité de Rennes 1Rennes, France (Baruteau, Donal, Daubert) Search for more papers by this author and Jean-Claude DaubertJean-Claude Daubert CHU RennesService de Cardiologie et Maladies VasculairesUniversité de Rennes 1Rennes, France (Baruteau, Donal, Daubert) Search for more papers by this author Originally published18 Jan 2011https://doi.org/10.1161/CIRCULATIONAHA.110.971978Circulation. 2011;123:e8We have read with great interest the article by van Spaendonck-Zwarts et al, which strongly suggests that a subset of peripartum cardiomyopathy (PPCM) is part of the spectrum of familial dilated cardiomyopathy.1 Entering PPCM in the field of familial dilated cardiomyopathy strongly modifies thinking on PPCM pathogenesis and management. We respectfully disagree in accepting the authors' interpretations of study findings. To our mind, they underestimate the clinical implication of their results and recommend overly restrictive indications for familial echocardiographic screening, which led us to write this letter.In the first systematic approach examining the familiality and genetics of PPCM, they found that cardiological screening of first-degree relatives of 3 PPCM patients who did not show full recovery revealed unknown dilated cardiomyopathy in all 3 families. Unfortunately, they decided not to perform such a familial screening in the 7 PPCM patients who experienced a complete normalization of left ventricular size and function. They also recommend “presymptomatic cardiological screening for covert dilated cardiomyopathy in first-degree family members of PPCM patients without recovery of left ventricular function and dimensions.”1Familial occurrence of PPCM was suggested in the literature by 6 previous anecdotal cases reporting a total of 8 kinships with more than 1 member affected by PPCM.1–3 Catastrophic outcomes were reported in 5 of these 6 publications with no recovery, death or heart transplantation for intractable heart failure in PPCM patients.However, this is not consistent with our experience, and our case report was the only one assessing a familial occurrence in 2 sisters who experienced severe PPCM with a favorable outcome and a full recovery.3 Both sisters presented dilated cardiomyopathy in the peripartum period with severe systolic dysfunction (left ventricular ejection fraction respectively 20% and 40%). They were aggressively supported in a timely manner, including inotropic support and extracorporeal membrane oxygenation for 1 of them and long-term medical treatment including a β-blocker agent and angiotensin-converting enzyme inhibitor. Full recovery was assessed by cardiac magnetic resonance imaging and transthoracic echocardiography, respectively, at 12- and 3-month follow-ups.In the current era of heart failure management, PPCM prognosis appears to be better than previously reported, with a left ventricular function normalization rate of 54%.4 Our observation shows that full recovery does not authorize elimination of a familial covert dilated cardiomyopathy.Therefore we suggest that cardiological screening be recommended to all first-degree relatives of PPCM patients, regardless of gender, even in the case of normalization of left ventricular size and function. It should allow presymptomatic diagnosis of unknown familial dilated cardiomyopathy in relatives. Despite the fact that it was recommended 10 years ago by the National Institutes of Health on PPCM,5 the impact of such a screening strategy remains to be assessed in large-scale studies.Alban-Elouen Baruteau, MDErwan Donal, MD, PhDJean-Claude Daubert, MD, PhD CHU Rennes Service de Cardiologie et Maladies Vasculaires Université de Rennes 1 Rennes, FranceDisclosuresNone.