Endocardial fibroelastosis

Endocardial fibroelastosis refers to a diffuse fibroelastic proliferative disease of the endocardium. It may be accompanied by myocardial degeneration. All four cardiac chambers can be involved individually or in combination, but the left ventricle is most commonly affected. Clinically, it is divided into fulminant, acute, and chronic types, mainly manifested as congestive heart failure; the fulminant type can present as cardiogenic shock. The disease has no obvious regional distribution. Domestic reports indicate a higher incidence in males than in females. Its clinical manifestations mainly include the following aspects: 1. Irritability, pallor, cold sweats, and refusal to feed. 2. Cough, tachypnea, cyanosis, and rales or wheezing sounds audible in both lungs. 3. Thready and rapid pulse, precordial bulge, enlarged cardiac border, muffled heart sounds, and in a few cases, a systolic murmur of grade 2 or above can be heard at the apex. 4. Hepatomegaly. The electrocardiogram of endocardial fibroelastosis mostly shows left ventricular hypertrophy, with a few cases showing right ventricular hypertrophy or combined left and right ventricular hypertrophy, which may be accompanied by ST-segment and T-wave changes as well as atrioventricular block. X-ray changes show marked left ventricular hypertrophy, weakened pulsation of the left heart border, and increased lung markings. If necessary, left heart catheterization can be performed; left ventricular end-diastolic pressure is elevated, and its waveform has diagnostic significance. The main treatment for endocardial fibroelastosis is to control heart failure. Acute heart failure requires intravenous digoxin or cedilanid for rapid digitalization, and long-term maintenance doses of digoxin should be continued for 2-3 years or even longer, until the heart returns to normal size. Premature discontinuation of the drug can lead to deterioration of the condition. For those with severe cardiac enlargement, severely reduced ejection fraction, and poor response to drug therapy, heart transplantation should be considered. The condition is generally critical after onset and requires prompt hospital treatment. The disease is prone to inducing heart failure due to pulmonary infection or complicating pulmonary infection due to heart failure. Therefore, in infants under 1 year of age with recurrent heart failure or heart failure that is difficult to control, the possibility of endocardial fibroelastosis should be considered. If diagnosed and treated early, with regular follow-up, cooperation with the physician, and long-term adherence to medication, a considerable number of patients can recover.