Besant Georgia, Bourque Pierre R, Smith Ian C, Chih Sharon, Lamacie Mariana M, Breiner Ari, Zwicker Jocelyn, Lochmüller Hanns, Warman-Chardon Jodi
Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada.
Department of Medicine, The Ottawa Hospital, University of Ottawa, Ottawa, ON, Canada.
Front Cardiovasc Med. 2022 Jun 3;9:899606. doi: 10.3389/fcvm.2022.899606. eCollection 2022.
Myotonic dystrophy type 1 (DM1) is a hereditary muscular dystrophy affecting ∼2.1-14.3/100,000 adults. Cardiac manifestations of DM1 include conduction disorders and rarely cardiomyopathies. DM1 increases the risk of obstetric complications, however, little is known about the relationship between pregnancy and cardiomyopathy in DM1 due to disease rarity.
A 23-year-old with DM1 developed cardiomyopathy during pregnancy. Despite initial medical stabilization, she subsequently developed multiple spontaneous coronary artery dissections postpartum, worsening cardiomyopathy and multiorgan failure. She died 5 months postpartum.
Though cardiomyopathy and arterial dissection are both known complications of pregnancy, this case suggests individuals with myotonic dystrophy type 1 may be at heightened risk for cardiac disease during the peripartum period. Physicians caring for women with suspected or proven DM1 should offer counseling and be alerted to the risk of cardiac complications with pregnancy and in the peripartum period. Pregnant and peripartum women with DM1 are likely to benefit from more frequent assessments of cardiac function including echocardiograms and early institution of heart failure management protocols when symptoms of cardiomyopathy present.
1型强直性肌营养不良（DM1）是一种遗传性肌肉营养不良症，影响约2.1 - 14.3/100,000成年人。DM1的心脏表现包括传导障碍，很少出现心肌病。DM1会增加产科并发症的风险，然而，由于该疾病罕见，关于DM1患者妊娠与心肌病之间的关系知之甚少。
一名23岁的DM1患者在怀孕期间发生了心肌病。尽管最初病情得到了医学稳定，但她随后在产后出现了多处自发性冠状动脉夹层，心肌病恶化并出现多器官功能衰竭。她在产后5个月死亡。
虽然心肌病和动脉夹层都是已知的妊娠并发症，但该病例表明，1型强直性肌营养不良患者在围产期患心脏病的风险可能更高。照顾疑似或确诊为DM1的女性的医生应提供咨询，并警惕妊娠和围产期心脏并发症的风险。患有DM1的孕妇和围产期妇女可能会从更频繁的心脏功能评估中受益，包括超声心动图，并且在出现心肌病症状时应尽早启动心力衰竭管理方案。
