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Potential for and timing of recovery in children with dilated cardiomyopathy

Matthew J Fenton1, Philippa Horne1, Jacob Simmonds1

  • 1Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Foundation Trust, Great Ormond Street, London WC1N 3JH, UK.

International Journal of Cardiology
|June 12, 2018
PubMed

Insights

Dilated cardiomyopathy (DCM) recovery is possible up to three years, with early signs including normalized NT-proBNP levels. Presentation to clinic offers better one-year survival, while ICU admission shows improved long-term outcomes despite initial severity.

Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Research
  • Clinical Outcomes

Background:

  • Dilated cardiomyopathy (DCM) diagnosis necessitates understanding clinical course and recovery timelines for effective management.
  • Guiding patient care and counseling relies on accurate prognostication following a DCM diagnosis.

Purpose of the Study:

  • To document outcomes and recovery timeframes in a cohort of pediatric patients with a dilated cardiomyopathy phenotype.
  • To identify predictors of recovery and the time scale for cardiac function restoration in DCM patients.

Main Methods:

  • Retrospective observational cohort study utilizing departmental database records.
  • Analysis of data including presentation mode, echocardiographic parameters, clinical management, and patient outcomes.
  • Investigation of predictors and time-to-recovery scales for dilated cardiomyopathy.

Main Results:

  • 209 new referrals with DCM were analyzed. Patients requiring intensive care (ICU) had 51% one-year and 45% five-year transplant-free survival.
  • Pediatric heart failure clinic patients (excluding neuromuscular disease) showed 85% one-year and 50% five-year transplant-free survival.
  • Normalized NT-proBNP preceded echocardiographic recovery; younger age, female sex, and smaller LV end-diastolic Z-scores predicted recovery.

Conclusions:

  • One-year transplant-free survival is better for clinic presenters, but ICU patients exhibit superior long-term survival due to late attrition in less severe cases.
  • Decreasing NT-proBNP levels serve as the earliest indicator of recovery in dilated cardiomyopathy.
  • Cardiac function recovery in DCM patients is achievable up to three years post-presentation.
Abstract

Keywords:
Heart failureHeart transplantMorbidityMortalityPediatric

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