Histological guided treatment in paediatric patients presenting with severe left ventricular dysfunction

Area of Science:

• Pediatric Cardiology
• Cardiovascular Pathology

Background:

• Diagnosing pediatric dilated cardiomyopathy with severe cardiac dysfunction is complex.
• Endomyocardial biopsy (EMB) is crucial for diagnosing myocarditis and guiding treatment.
• A hospital protocol uses EMB results to direct therapy in selected pediatric patients.

Purpose of the Study:

• To evaluate the diagnostic and therapeutic utility of EMB in pediatric patients with dilated cardiomyopathy and severe cardiac dysfunction.
• To identify factors associated with treatment response in this patient population.

Main Methods:

• A unicenter, ambispective study included pediatric patients with dilated cardiomyopathy (LVEF < 35%) undergoing EMB.
• Treatment was prescribed based on EMB findings.
• Patients were analyzed for recovery based on biopsy results.

Main Results:

• Of 23 patients (24 episodes), 15 achieved complete recovery, 5 had partial response, and 4 showed no response.
• Complete recovery correlated with intense inflammation, necrosis, edema on EMB, negative genetic testing, high troponin levels, and positive myocardial viral PCR.
• No significant differences were found in sex, age, clinical presentation, or ECMO need between recovery groups.

Conclusions:

• EMB is a valuable tool for diagnosing and treating pediatric dilated cardiomyopathy.
• Favorable treatment response is associated with specific EMB findings (inflammation, edema, necrosis), viral PCR positivity, high troponins, and negative genetic testing.
• Genetic testing should be considered for all patients with severe cardiac dysfunction.