Endocardial fibroelastosis

Endocardial fibroelastosis. Endocardial fibroelastosis is a heart condition characterized by diffuse thickening of the left ventricular endocardium, giving it a milky white appearance with a relatively smooth lining. Often, in addition to the left ventricle, the left atrium is also affected.

Microscopically, he has hyperplasia of fibrous and elastic tissue. At the junction of the endocardium with the myocardium, it shows some degeneration of the muscle fibers with infiltration of round cells and vacuolization of the same area. Associated with interstitial myocarditis, necrosis, fibrosis, or calcification. Embolic complications have been described.

Summary

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  • 1 Clinical picture
  • 2 Radiological examination
  • 3 Electrocardiographic examination
    • 1 Atypical EKGs
  • 4 Diagnosis and differential diagnosis
    • 1 With myocarditis
    • 2 With cardiac glycogenosis
  • 5 Calcification of the coronary artery
  • 6 Diffuse myocardial fibrosis
  • 7 Others
  • 8 Source

Clinical picture

Endocardial fibroelastosis predominates over the female sex, but when it is associated with heart disease, the male sex predominates. It generally occurs in children of mothers between 25 and 30 years of age. Some authors report family cases.

Fibroelastosis is associated in 6% with aortic atresia; in 4% with aortic coarctation and in 1% with persistent ductus arteriosus. The first symptoms are due to congestive heart failure, which can progress chronically. Cyanosis rarely occurs and when it appears it is due to severe myocardial failure or the final stage of the disease.

The initial clinical picture is tachypnea, gallop rhythm, hepatomegaly, cardiomegaly, systolic murmur in the apex or in the area of ​​auscultation of the aorta.

Radiological examination

Radiologically 95% of cases have cardiomegaly and congestive lungs when they evolve with heart failure.

The apex is sunk into the diaphragm when left ventricular growth predominates. The heart may be globular in shape and the upper mediastinum narrow. Almost all patients have left atrial growth. The pulmonary hypertension occurs without exceed systemic. In the terminal phase of the disease, cardiomegaly is massive.

Electrocardiographic examination

The authors AR. Mastreiter and Elizabeth A. Fischer describe four types of left ventricular abnormalities:
1.Typical left overload pattern with negative T waves and ST segment depression in the left precordial leads.
2. Symmetric and inverted T waves in the left precordial without ST segment depression. This layout persists for weeks and exceptionally for years.
3. Flattened or diphasic T waves in the left precordial and with an evolution similar to the previous one.
4. Positive T waves in 5 to 10% of cases with a prolonged state of the disease.
Signs of left ventricular hypertrophy may be present in the first months of the disease.

Atypical electrocardiograms

1.Low voltage without overload criteria in any of the ventricles, which occurs in 5% of cases of endocardial fibroelastosis; it is transitory at the beginning of the disease.
2. Symmetric and inverted T waves without depression of the ST segment in the left precordial leads, which persist for a few weeks or months.
3. Flattened or isodiphasic T waves in left precordial.
4.The pattern of injury or infarction is more frequent in myocarditis and has been described by some authors associated with extensive fibrosis.

The front axis of QRS between +20 and +150 degrees with an average of + 65 degrees. First-degree AV block in some patients receiving digitalis treatment. Left bundle branch block may or may not occur, and right bundle branch block is very rare. WPW syndrome has been reported in various statistics.

Diagnosis and differential diagnosis

The diagnostic suspicion in every child who in the first year of life shows signs of congestive heart failure, cardiomegaly and on the electrocardiogram signs of left ventricular growth with overload of that cavity.

There are two types of endocardial fibroelastosis, one associated with other heart defects and a second isolated form. In the first group, it can be suspected with the presence of aortic atresia, which has this endocardial injury in 60% of cases. In coarctation of the aorta with endocardial fibroelastosis, he has left ventricular hypertrophy, but the clinical picture of coarctation dominates.

The differential diagnosis of isolated endocardial fibroelastosis should be made with the

With myocarditis

When the mother suffers from a Coxsackie virus infection in late pregnancy, the baby can be born with the same infection as the mother and suffer from myocarditis. After the neonatal period, myocarditis appears irregularly. Electrocardiographic examination is useful for the differential diagnosis between myocarditis and fibroelastosis, which has a high R voltage in left precordial and deep S in V1, while myocarditis has a low QRS voltage.

Inverted and deep T waves in the left precordial in endocardial fibroelastosis, while it is flat and shallow in myocarditis. The infarction pattern is rare in endocardial fibroelastosis and is observed in 10% in myocarditis. The Q wave in V6 is 1 mm or more in fibroelastosis and rare in myocarditis.

With cardiac glycogenosis

Glycogen storage heart disease is characterized by congestive heart failure, generalized hypotonia, macroglossia, cretinoid appearance, and repeated respiratory infections, more frequent in the left lung than in the right lung and cardiomegaly. On the electrocardiogram, a syndrome of preexcitation and negative T waves and left ventricular growth. These clinical, radiological, and electrocardiographic data distinguish Pompe disease from endocardial fibroelastosis. The left coronary artery emerging from the pulmonary artery

Children with this abnormality suffer from dyspnea, hepatomegaly, and congestive heart failure. This anomaly is accompanied by electrocardiographic signs of interseptal infarction, manifested by negative T waves in Dl, in aVl, V5, and V6 with positive ST-segment elevation, although he may be depressed.

Coronary artery calcification

This disease is rare with radiological evidence of calcification of the systemic arteries and without electrocardiographic signs of heart attack.

 

Diffuse myocardial fibrosis

Endemic disease in some regions of Africa, similar to endocardial fibroelastosis.

Others

When fibroelastosis is accompanied by mitral regurgitation or aortic valve injury, it is necessary to make a differential diagnosis with all those pathologies that affect these valves. Other entities such as asymmetric septal hypertrophy, polyarteritis nodosa, severe anemia, hypertension, supraventricular tachycardia, aortic stenosis, coarctation of the aorta should be considered. Complete the study with the echocardiogram.

 

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