Pregnancy and Progression of Cardiomyopathy in Women With LMNA Genotype‐Positive

We aimed to assess the association between number of pregnancies and long‐term progression of cardiac dysfunction, arrhythmias, and event‐free survival in women with pathogenic or likely pathogenic variants of gene encoding for Lamin A/C proteins  (LMNA+).  
In our cohort of women with LMNA+, pregnancy did not seem associated with long‐term adverse disease progression or event‐free survival. Likewise, women with LMNA+ generally well‐tolerated pregnancy, with a small proportion of patients experiencing arrhythmias.

Clinical Perspective

What Is New?

  • In women with LMNA+, number of previous pregnancies was not associated with long‐term worsening of electrical disease and occurrence of sustained ventricular arrhythmias, and did not accelerate cardiac dilatation.
  • Number of deaths, left ventricular assistance device implantations, and heart transplantations did not differ significantly between nulliparous women and women with previous pregnancy in our cohort.
  • Pregnancy was mostly well tolerated with a low number of maternal and fetal complications, but risk of arrhythmias during pregnancy cannot be excluded.

What Are the Clinical Implications?

  • Women with LMNA+, without overt electric/structural cardiomyopathy, should not be suggested to refrain pregnancy.
  • Pre‐pregnancy counseling in high experience cardiomyopathy centers, to assess disease status and pregnancy‐related risks, should be considered in these patients.
  • During pregnancy, because of lack of systematic data on triggering of arrhythmic events, it can be reasonable to use ambulatory ECG monitoring in women with known LMNA+, independently of current phenotype.

To our knowledge, this is the largest study exploring the association between previous pregnancies, long‐term development of cardiomyopathy, and outcomes in patients with LMNA+. Number of pregnancies was not associated with long‐term worsening of electrical disease and occurrence of sustained VA and was not associated with worse primary outcome. Furthermore, pregnancy did not accelerate the progression of cardiac dysfunction in our cohort. The majority of women retrospectively reported uncomplicated pregnancies without increase in serious obstetric or fetal adverse events. A few selected patients followed during pregnancy experienced increased arrhythmic symptoms.

Pregnancy and Progression of Cardiomyopathy in Women With LMNA Genotype‐Positive | Journal of the American Heart Association (ahajournals.org)

J Am Heart Assoc. 2022 Apr 19;11(8):e024960.
Anna I Castrini, Eystein Skjølsvik, Mette E Estensen, Vibeke M Almaas, Helge Skulstad, Erik Lyseggen, Thor Edvardsen, Øyvind H Lie, Kermshlise C I Picard, Neal K Lakdawala, Kristina H Haugaa
PMID: 35434999
PMCID: PMC9238471
DOI: 10.1161/JAHA.121.024960 

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