Successful Total Pericardiectomy for Constrictive Pericarditis in the First Series of Japanese Patients With Mulibrey Nanism

Can J Cardiol. 2018 May;34(5):690.e5-690.e8. doi: 10.1016/j.cjca.2018.02.008. Epub 2018 Feb 13.

Abstract

Patients with Mulibrey nanism (MUL) present with growth failure and multiple organ manifestations, and MUL is caused by mutations in TRIM37. In this article, we report on the first case series of Japanese patients with MUL who developed congestive heart failure due to constrictive pericarditis. Our case series suggests that early diagnosis and total pericardiectomy before adherence of the pericardium might provide clinical benefit and better prognosis for MUL.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Child, Preschool
  • Early Diagnosis
  • Echocardiography / methods
  • Female
  • Genetic Testing
  • Heart Failure* / diagnosis
  • Heart Failure* / etiology
  • Humans
  • Magnetic Resonance Imaging, Cine / methods
  • Mulibrey Nanism* / diagnosis
  • Mulibrey Nanism* / genetics
  • Mulibrey Nanism* / physiopathology
  • Mulibrey Nanism* / therapy
  • Mutation
  • Nuclear Proteins / genetics*
  • Pericardiectomy / methods*
  • Pericarditis, Constrictive* / complications
  • Pericarditis, Constrictive* / diagnosis
  • Pericardium / pathology
  • Prognosis
  • Treatment Outcome
  • Tripartite Motif Proteins
  • Ubiquitin-Protein Ligases

Substances

  • Nuclear Proteins
  • Tripartite Motif Proteins
  • TRIM37 protein, human
  • Ubiquitin-Protein Ligases