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New Delhi, May 19, 2022:
A 19 year female, R/O eastern UP was seen with history of shortness of breath, malaise, persistent nausea and palpitations. The parents correlated these complaints with her unwilling to attend school. They noticed that she would confined to the room without much activity. She was shown in a nearby hospital where her routine blood investigations and Chest X-ray were done and reported normal. As per the parents, patient also had decreased attention span in school and complained of persistent palpitations. Psychiatry opinion and regular counselling was done for the same as they attributed the illness towards hesitancy in attending her classes.
As there was not much improvement the parents decided to take opinion at Sir Ganga Ram Hospital. She was seen by Dr. Atul Kakar, Vice Chairman, Department of Medicine, SGRH who diagnosed that she was having symptoms suggestive of Pulmonary Artery Hypertension (PAH), based upon her clinical examination and echocardiography (ultrasound of the heart). Her pulmonary pressure (the artery which connects heart to the lung) was very high (65mmHg, normal <25mmHg). If not treated would have lead to death. Her symptoms were attributed towards rare condition – Primary Pulmonary Hypertension. Primary Pulmonary Artery Hypertension is a very rare identity and occurs in 1-2 patients every 10 lakh population.
The extensive other tests were also done to rule out the secondary causes of this condition and there was no cause found and the condition was labelled as idiopathic. In Primary Pulmonary Hypertension the blood vessels in the lung are narrowed, blocked or destroyed and the heart has to pump with lit of pressure through these damaged arteries. Slowly the shortness of breath increases to life threatening condition and with very quality of life.
Patient was started on drugs to decrease the PAH which included endothelin receptor antagonist along with phosphodiesterase 5 inhibitor. These drugs are now available for treatment of this rare disease. Over the last 5 years patient has symptomatically improved and her pulmonary pressures are under control.
Dr. Kakar diagnosed this condition and as the medication for this condition is expensive and life long, arranged for the funds from Uttar Pradesh Chief Minister Relief Fund. The diagnosis of PAH is usually missed because of non specific presentation. High index of suspicion is required to diagnose this among patients who present with dyspnoea (shortness of breath). Hence, timely diagnosis and treatment helps in prolonging the life expectancy otherwise it is fatal condition Patient is currently doing well and is on regular follow up.
Dr. Atul Gogia, Senior Consultant in the unit added that 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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