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Mortality in adults with congenital heart disease

Pavithra Naidu1, Leeanne Grigg1, Dominica Zentner1

  • 1Department of Cardiology, 300 Grattan Street, Parkville, Melbourne, Victoria, Australia.

International Journal of Cardiology
|September 7, 2017
PubMed

Insights

Most deaths in adult congenital heart disease patients were cardiac-related, primarily sudden cardiac death and heart failure. Identifying risk factors for sudden death may guide preventative therapies for this vulnerable population.

Area of Science:

  • Cardiology
  • Adult Congenital Heart Disease (ACHD)
  • Mortality Studies

Background:

  • The Royal Melbourne Hospital (RMH) maintains a registry of Adult Congenital Heart Disease (ACHD) patients, initiated in 1991.
  • Understanding mortality patterns in the growing ACHD population is crucial for clinical management and resource allocation.

Purpose of the Study:

  • To retrospectively determine the causes of mortality within the RMH ACHD cohort.
  • To analyze the frequency and specific causes of death across different congenital heart defect diagnoses.

Main Methods:

  • A retrospective analysis was conducted on deceased patients (n=73) from the RMH ACHD registry (1991-2015).
  • Data collected included patient age, gender, underlying congenital heart condition, and cause of death.
  • Causes of death were categorized as cardiac or non-cardiac, with specific sub-classifications.

Main Results:

  • Between 1991 and 2015, 3.3% of the ACHD cohort died, with a median age at death of 32 years.
  • The most frequent underlying conditions were Eisenmenger's syndrome (22%) and pulmonary atresia with VSD (12%).
  • Cardiac causes accounted for 67% of deaths, with sudden death (40%) and heart failure (13%) being most common. Sepsis was the leading non-cardiac cause (10%).

Conclusions:

  • Cardiac causes, particularly sudden death and heart failure, are the predominant causes of mortality in this ACHD cohort.
  • Further research into risk factors for sudden cardiac death is warranted to identify patients who could benefit from interventions like implantable cardioverter-defibrillators.
Abstract

Keywords:
Adult congenital heart diseaseHeart defectsMortality

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