SUBENDOCARDIAL SCLEROSIS

SUBENDOCARDIAL SCLEROSIS

Overview and Facts

Subendocardial sclerosis is a rare disorder of the cardiovascular system that affects both infants and children. Its characteristic feature is a thickening within the cardiac muscle lining of the atria and ventricles due to the increase in the quantity of inelastic collagen and elastic fibers.

The human heart has four chambers. These are the atria which act as holding chambers for blood, and the ventricles which pump blood out of the heart. Both of these chambers are separated by a partition called a septum. Valves connect the atria to the ventricles.

Subendocardial sclerosis is marked by diffuse endocardial thickening and myocardial dysfunction. Endocardial thickening is supposed to be caused by constant and heightened wall tension in the ventricles, perhaps secondary to damaged myocardium, mitral regurgitation, or both. This, however, changes with age progression. The disease may occur for no apparent reason (sporadic).

Types and Symptoms

SYMPTOMS

The symptoms of subendocardial sclerosis often have an immediate onset at ages four to 12 months. These symptoms occur because of increased growth of fibrous tissue and thickening of the endocardium and subendocardium. In extraordinary cases of subendocardial sclerosis, the left ventricle is hypoplastic and the right ventricle is enlarged.

Common symptoms of subendocardial sclerosis include:

  • Dyspnea
  • Breathlessness
  • Grunting sounds while breathing
  • Coughing
  • Irritability
  • Pallor
  • Malaise

 

Life-threatening complications of subendocardial sclerosis may also develop, these include:

  • Tachycardia
  • Arrhythmia
  • Congestive heart failure

Diagnosis & Medications

DIAGNOSIS

The diagnosis of this is verified through a thorough clinical evaluation which includes a physical examination. This may help reveal signs of respiratory distress and irregular heart rhythms. Roentological findings of the chest may help reveal cardiomegaly. Damage to the heart may be exhibited by performing an ECG (electrocardiogram) which measures the electrical activity of the heart. It may reveal minute changes that vehemently suggest damage to the heart due to subendocardial sclerosis. ECGs must be repeated to monitor changes in heart function.

TREATMENT

Infants who receive an early diagnosis during the runoff of the disease respond more favorably to treatment. This is antithetical to those who receive a diagnosis when considerable heart damage has already occurred. The treatment approach for subendocardial sclerosis is technically the same for patients with chronic heart failure. A myriad of drugs may be used to manage congestive heart failure associated with subendocardial sclerosis to reduce heart rate and improve the heart’s ability to contract. Drugs such as diuretics, antiarrhythmics, and anticoagulants may be prescribed by the doctor. Prolonged bed rest is also highly recommended to reduce the workload of the heart.

For some children with advanced illness, the only recourse will be heart transplantation, as the illness has already run its course.

Genetic counseling may be essential for patients and their families. Other forms of treatment are symptomatic and palliative.

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