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Original article
Pregnancy course and outcomes in women with arrhythmogenic right ventricular cardiomyopathy
  1. Anke R Hodes1,2,
  2. Crystal Tichnell1,
  3. Anneline S J M te Riele1,3,
  4. Brittney Murray1,
  5. Judith A Groeneweg3,4,
  6. Abhishek C Sawant1,
  7. Stuart D Russell1,
  8. Karin Y van Spaendonck-Zwarts5,
  9. Maarten P van den Berg2,
  10. Arthur A Wilde6,
  11. Harikrishna Tandri1,
  12. Daniel P Judge1,
  13. Richard N W Hauer4,
  14. Hugh Calkins1,
  15. J Peter van Tintelen2,4,5,
  16. Cynthia A James1
  1. 1Division of Cardiology, Department of Medicine, Johns Hopkins University, Baltimore, Maryland, USA
  2. 2Department of Cardiology/Genetics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands
  3. 3Department of Cardiology, University Medical Center Utrecht, Utrecht, The Netherlands
  4. 4Interuniversity Cardiology Institute of the Netherlands (ICIN), Utrecht, The Netherlands
  5. 5Department of Genetics, Academic Medical Center, Amsterdam, The Netherlands
  6. 6Department of Clinical and Experimental Cardiology, Heart Centre, Academic Medical Centre, Amsterdam, The Netherlands
  1. Correspondence to Cynthia A James, Division of Cardiology, Department of Medicine, The Johns Hopkins Hospital, Carnegie 568D, 600 North Wolfe Street, Baltimore MD 21287, USA; cjames7{at}


Objectives To characterise pregnancy course and outcomes in women with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C).

Methods From a combined Johns Hopkins/Dutch ARVD/C registry, we identified 26 women affected with ARVD/C (by 2010 Task Force Criteria) during 39 singleton pregnancies >13 weeks (1–4 per woman). Cardiac symptoms, treatment and episodes of sustained ventricular arrhythmias (VAs) and heart failure (HF) ≥ Class C were characterised. Obstetric outcomes were ascertained. Incidence of VA and HF were compared with rates in the non-pregnant state. Long-term disease course was compared with 117 childbearing-aged female patients with ARVD/C who had not experienced pregnancy with ARVD/C.

Results Treatment during pregnancy (n=39) included β blockers (n=16), antiarrhythmics (n=6), diuretics (n=3) and implantable cardioverter defibrillators (ICDs) (n=28). In five pregnancies (13%), a single VA occurred, including two ICD-terminated events. Arrhythmias occurred disproportionately in probands without VA history (p=0.045). HF, managed on an outpatient basis, developed in two pregnancies (5%) in women with pre-existing overt biventricular or isolated right ventricular disease. All infants were live-born without major obstetric complications. Caesarean sections (n=11, 28%) had obstetric indications, except one (HF). β Blocker therapy was associated with lower birth weight (3.1±0.48 kg vs 3.7±0.57 kg; p=0.002). During follow-up children remained healthy (median 3.4 years), and mothers were without cardiac mortality or transplant. Neither VA nor HF incidence was significantly increased during pregnancy. ARVD/C course (mean 6.5±5.6 years) did not differ based on pregnancy history.

Conclusions While most pregnancies in patients with ARVD/C were tolerated well, 13% were complicated by VA and 5% by HF.

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