Pregnancies, ventricular arrhythmias, and substrate progression in women with arrhythmogenic right ventricular cardiomyopathy in the Nordic ARVC Registry

Platonov, Pyotr G.; Castrini, Anna Isotta; Svensson, Anneli; Christiansen, Morten Krogh; Gilljam, Thomas; Bundgaard, Henning; Madsen, Triene; Heliö, Tiina; Christensen, Alex Hørby; Astrom, M A; Carlson, Jonas; Edvardsen, Thor; Jensen, Henrik Kjærulf; Haugaa, Kristina H; Svendsen, Jesper Hastrup

doi:10.1093/europace/euaa136

Cited by 11 publications

(16 citation statements)

References 18 publications

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“…Platonov and Castrini et al [13,48] recently published similar results in their latest series from the Scandinavian Nordic Registry, including 261 pregnancies in 120 women with AC. They reported two maternal SCD, five aborted SCD, five heart transplantations, and a total of 41 events during long-term follow-up.…”

Section: Maternal Mortality and Complications (Va Hf)mentioning

confidence: 60%

“…The study cohort differed significantly from other publications by a much longer follow-up and a substantially higher risk profile, including a history of cardiac arrest (40%), sustained VT/VF (74%), HF (33%), heart transplantation (17%), catheter ablation (50%), and ICD implantation (17%) [69]. Similar to the studies by Castrini and Platonov [13,48], the majority of pregnancies (in 76% of women) occurred long before the diagnosis of AC was made.…”

Section: Maternal Mortality and Complications (Va Hf)mentioning

confidence: 62%

“…Impact of multiple pregnancies on VA and HF. Platonov et al [48] retrospectively analyzed the course of 261 pregnancies in 120 women with at least one childbirth. Of these, 58 patients were previously reported [13].…”

Section: Maternal Mortality and Complications (Va Hf)mentioning

confidence: 99%

“…Only case reports [ 1 , 3 , 15 , 22 , 23 , 30 , 33 , 34 , 54 , 61 ] and small series [ 5 , 7 , 9 , 36 ] with a total of 30 pregnancies in 30 women with AC had been reported prior to the first larger case studies and registry reports on pregnancy in AC that were recently published ([ 13 , 27 , 31 , 48 , 69 ]; Tables 1 and 2 ). All reported pregnancies in AC were singleton.…”

Section: Outcomes Of Pregnancy In Women With Acmentioning

confidence: 99%

“…a Reference[48] includes 58 patients b Higher event rates are caused by very long intervals between last pregnancy and diagnosis of arrhythmogenic cardiomyopathy (see text for details)…”

mentioning

confidence: 99%

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Pregnancy in arrhythmogenic cardiomyopathy

Wichter

Milberg²,

Wichter³

et al. 2021

Herzschr Elektrophys

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Arrhythmogenic cardiomyopathy (AC) is a rare heart muscle disease with a genetic background and autosomal dominant mode of transmission. The clinical manifestation is characterized by ventricular arrhythmias (VA), heart failure (HF) and the risk of sudden cardiac death (SCD). Pregnancy in young female patients with AC represents a challenging condition for the life and family planning of young affected women. In addition to genetic mechanisms that influence the complex pathophysiology of AC, experimental and clinical data have confirmed the pathogenetic role of strenuous exercise and competitive sports in the early onset and rapid progression of AC symptoms and complications. Pregnancy and exercise share a number of physiological aspects of adaptation. In AC, both result in ventricular volume overload and myocardial stretch. Therefore, pregnancy has been postulated as a potential risk factor for HF, VA, SCD, and pregnancy-related obstetric complications in patients with AC. However, the available evidence on pregnancy in AC does not confirm this hypothesis. In most women with AC, pregnancies are well tolerated, uneventful, and follow a benign course. Pregnancy-related symptoms (VA, syncope, HF) and mortality, as well as obstetric complications, are uncommon in AC patients and range in the order of background populations and cohorts with AC and no pregnancy. The number of completed pregnancies is not associated with an acceleration of AC pathology or an increased risk of VA or HF during pregnancy and follow-up. Accordingly, there is no medical indication to advise against pregnancy in patients with AC. Preconditions include stability of rhythm and hemodynamics at baseline, as well as clinical follow-ups and the availability of multidisciplinary expert consultation during pregnancy and postpartum. Genetic counseling is recommended prior to pregnancy for all couples and their families affected by AC.

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Section: Maternal Mortality and Complications (Va Hf)mentioning

confidence: 60%

Section: Maternal Mortality and Complications (Va Hf)mentioning

confidence: 62%

Section: Maternal Mortality and Complications (Va Hf)mentioning

confidence: 99%

Section: Outcomes Of Pregnancy In Women With Acmentioning

confidence: 99%

“…a Reference[48] includes 58 patients b Higher event rates are caused by very long intervals between last pregnancy and diagnosis of arrhythmogenic cardiomyopathy (see text for details)…”

mentioning

confidence: 99%

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Pregnancy in arrhythmogenic cardiomyopathy

Wichter

Milberg²,

Wichter³

et al. 2021

Herzschr Elektrophys

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Disease features and management of cardiomyopathies in women

Aimo,

Morfino,

Arzilli

et al. 2024

Heart Fail Rev

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Over the last years, there has been a growing interest in the clinical manifestations and outcomes of cardiomyopathies in women. Peripartum cardiomyopathy is the only women-specific cardiomyopathy. In cardiomyopathies with X-linked transmission, women are not simply healthy carriers of the disorder, but can show a wide spectrum of clinical manifestations ranging from mild to severe manifestations because of heterogeneous patterns of X-chromosome inactivation. In mitochondrial disorders with a matrilinear transmission, cardiomyopathy is part of a systemic disorder affecting both men and women. Even some inherited cardiomyopathies with autosomal transmission display phenotypic and prognostic differences between men and women. Notably, female hormones seem to exert a protective role in hypertrophic cardiomyopathy (HCM) and variant transthyretin amyloidosis until the menopausal period. Women with cardiomyopathies holding high-risk features should be referred to a third-level center and evaluated on an individual basis. Cardiomyopathies can have a detrimental impact on pregnancy and childbirth because of the associated hemodynamic derangements. Genetic counselling and a tailored cardiological evaluation are essential to evaluate the likelihood of transmitting the disease to the children and the possibility of a prenatal or early post-natal diagnosis, as well as to estimate the risk associated with pregnancy and delivery, and the optimal management strategies.

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