Doctors at Delhi hospital save life of woman with rare pulmonary disorder

A 19-year-old woman who suffered from primary pulmonary hypertension -- a rare condition found in just one or two persons per 10 lakh people -- was saved from the clutches of death by doctors at the Sir Ganga Ram Hospital SGRH here.

A 19-year-old woman who suffered from primary pulmonary hypertension -- a rare condition found in just one or two persons per 10 lakh people -- was saved from the clutches of death by doctors at the Sir Ganga Ram Hospital (SGRH) here. According to the hospital administration, the woman, a resident of eastern Uttar Pradesh, had a history of shortness of breath, malaise, persistent nausea, and palpitation which her parents correlated with her unwillingness to attend school.

She would complain of decreased attention at school and confine herself to her room, they said. Doctors at a nearby hospital conducted routine blood investigations and chest x-ray which came out normal. Psychiatrists attributed her illness to her hesitancy in attending classes. When her condition did not improve, the parents sought help from doctors at SGRH. Based on a clinical examination and echocardiography (ultrasound of the heart), Dr. Atul Kakar, Vice Chairman, Department of Medicine, diagnosed that the woman had symptoms suggestive of pulmonary artery hypertension (PAH). ''The pressure in the artery that connects the heart to the lungs was very high -- 65mmHg as against the normal level of 25mmHg and below. Had it not been treated, it would have led to her death,'' Dr. Kakar said.

Primary pulmonary artery hypertension (PAH) is a very rare condition that affects only one or two persons per 10 lakh people. In such patients, the blood vessels in the lungs get blocked, narrowed, or destroyed and the heart has to pump blood with a lot of pressure through these damaged arteries. More tests were done to rule out the secondary causes of this condition and the condition was described as idiopathic. ''The patient was started on drugs to decrease PAH which included endothelin receptor antagonist along with phosphodiesterase 5 inhibitor. These drugs are now available for the treatment of this rare disease,'' Dr. Kakar said.

The patient has symptomatically improved over the last five years and her pulmonary pressures are under control, he said.

As the treatment for this condition is expensive and lifelong, Dr. Kakar also arranged for the funds from Uttar Pradesh Chief Minister Relief Fund, the hospital said.

Dr. Atul Gogia, the senior consultant in the unit, said the treatment of this condition has improved over the years with the availability of newer drugs. These patients are living longer with better quality of life. 

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