Cardiac alterations in 36 consecutive patients with idiopathic haemochromatosis: polygraphic and echocardiographic...

Area of Science:

• Cardiology
• Cardiovascular Medicine
• Hepatology

Background:

• Idiopathic haemochromatosis (IH) is a genetic disorder characterized by excessive iron absorption and deposition.
• Iron overload in the myocardium can lead to cardiac dysfunction and heart failure.
• Understanding the early cardiac manifestations of IH is crucial for timely intervention.

Purpose of the Study:

• To investigate the cardiac involvement in patients with idiopathic haemochromatosis using comprehensive echocardiographic assessments.
• To evaluate the impact of iron depletion therapy on cardiac structure and function in IH patients.
• To identify early, potentially reversible cardiac alterations associated with iron deposition in the myocardium.

Main Methods:

• Thirty-six idiopathic haemochromatosis patients underwent electrocardiography (ECG), polygraphy, and various echocardiographic techniques (M-mode, 2D, Doppler).
• Echocardiographic parameters were analyzed for correlations with ECG findings and iron overload severity.
• Ten patients were assessed before and after iron-depleting therapy to evaluate treatment effects.

Main Results:

• No significant correlations were found between ECG parameters and echocardiographic measurements.
• Left ventricular enlargement with impaired systolic function was observed in only 5.5% of patients.
• Echocardiographic abnormalities were more pronounced in patients with higher iron overload. Iron depletion therapy led to significant reductions in interventricular septum thickness and left ventricular mass.

Conclusions:

• Increased ventricular wall thickness without impaired systolic function may represent the initial, reversible cardiac change in IH.
• Progressive iron overload can lead to impaired left ventricular function and dilated cardiomyopathy.
• Early diagnosis and treatment of IH cardiopathy are essential to prevent irreversible cardiac damage.