Endomyocardial fibrosis in children.

Area of Science:

• Pediatric Cardiology
• Cardiovascular Surgery
• Pediatric Cardiac Surgery

Background:

• Endomyocardial fibrosis (EMF) is a rare, restrictive cardiomyopathy predominantly affecting children.
• Surgical intervention is considered for advanced stages of EMF, often presenting as end-stage heart failure.
• Limited data exists on the long-term outcomes of surgical treatment for pediatric EMF.

Purpose of the Study:

• To evaluate the surgical outcomes and long-term results in children with endomyocardial fibrosis.
• To assess the efficacy and limitations of surgical treatment for pediatric EMF.

Main Methods:

• Retrospective analysis of 10 children with EMF who underwent surgical treatment between 1978 and 1999.
• Detailed review of patient demographics, disease extent, surgical procedures, and postoperative follow-up.
• Assessment of mortality, morbidity, reoperation rates, and functional status.

Main Results:

• 30% (3/10) in-hospital mortality due to low cardiac output.
• 50% (5/10) long-term survival rate, with two late deaths from heart failure and endocarditis.
• Significant morbidity including reoperations for prosthetic valve dysfunction and persistent heart failure symptoms in survivors.

Conclusions:

• Surgical treatment for pediatric endomyocardial fibrosis is primarily palliative with suboptimal outcomes.
• High rates of mortality and morbidity necessitate further research into alternative or improved therapeutic strategies.
• Current surgical approaches for pediatric EMF yield results that are largely unsatisfactory.