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Two novel presentations of KCNMA1-related pathology--Expanding the clinical phenotype of a rare channelopathy.

Jotte Rodrigues BentoCandice FebenMarlies KempersMaartje van RijMallory WoiskiKoenraad DevriendtLuc De CatteMarcella BaldewijnsMaaike AlaertsJosephina MeesterAline VerstraetenWilly HendsonBart Loeys
Published in: Molecular genetics & genomic medicine (2021)
The current report presents the first antenatal presentation of a pathogenic KCNMA1 mutation and confirms the specific association of the p.Gly375Arg variant with early onset aortic root dilatation, gingival hypertrophy, and neonatal overgrowth.
Keyphrases
  • early onset
  • late onset
  • pregnant women
  • aortic valve
  • left ventricular
  • preterm birth
  • pulmonary artery
  • case report
  • aortic dissection
  • heart failure
  • coronary artery
  • pulmonary arterial hypertension
  • atrial fibrillation