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What Is Kyphoscoliotic Heart Disease?

Kyphoscoliosis or hunchback is a disease of the spine. It is a combination of kyphosis and scoliosis. Here the spine is bent sidewards, and there is also an abnormal front to backward curvature with a rounded back appearance. Kyphoscoliosis may be congenital which means it can be present at birth, or spontaneous which means it can occur any time in one's life. It may also be caused by diseases of the spine like tuberculosis or osteomalacia. Traumatic spine or brain injury, neuromuscular diseases like Friedreich's ataxia, cerebral palsy or poliomyelitis may also cause kyphoscoliosis. Kyphoscoliosis leads to a number of complications, one of which is heart disease.

  1. Pathogenesis

    • Kyphoscoliosis leads to right-sided heart failure when the external angle of the scoliosis is at a sidewards bend of more than 100 degrees, and the kyphosis, that is the front to backward curvature is more than 20 degrees. In such degrees of deformity, there is a restriction in chest growth and also lung complications. This leads to high pulmonary artery pressure, which in the long-run causes right-sided heart failure.

    Signs and Symptoms

    • Kyphoscoliotic heart disease results in right-sided heart failure, hence all the symptoms of right-sided heart failure are present. The symptoms include chest discomfort, shortness of breath, exercise intolerance and swelling of feet and ankles. The signs include distension of neck veins, fluid in the abdomen, bluish discoloration of the skin, swelling around ankles, liver enlargement and abnormal heart sounds. Other signs and symptoms of kyphoscoliosis itself, like backache and uneven appearance of shoulder and hips, are also present.

    Tests

    • Tests that aid in the diagnosis of kyphoscoliosis include chest X-ray, CT scan of the chest, ECG, lung function tests, catheterization of the right heart and ventilation/perfusion scan of the lungs. Scoliometer screening is used to diagnose kyphoscoliosis.

    Treatment of Kyphoscoliotic Heart Disease

    • In kyphoscoliotic heart disease, the outcome is not favorable. However, with proper treatment there is an improvement in survival rate. Positive pressure ventilation, intermittently, at night improves the survival rate significantly. This method is better than the more commonly used long-term oxygen therapy. Prevention of development of heart disease by recognizing and treating kyphoscoliosis at an early stage is a better option. Pharmacological agents like bosentan, sildenafil and calcium channel blockers are used in early cases of heart failure.

    Controlling Kyphoscoliasis

    • Early diagnosis and limiting the deformity of kyphoscoliosis by surgery or by wearing the appropriate body braces and receiving proper treatment from your doctor, will prevent or at least delay the onset of kyphoscoliotic heart disease.

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