Rohit Shishodia

With correct and timely diagnosis, the doctors at Delhi’s Sir Ganga Ram Hospital (SGRH) have saved the life of 19-year-old UP girl who has Pulmonary Artery Hypertension (PAH), a very rare identity that occurs in 1-2 patients every 10 lakh population.

As per the hospital statement, the patient was started on drugs to decrease the PAH which included endothelin receptor antagonist along with phosphodiesterase 5 inhibitor.  The patient has symptomatically improved and her pulmonary pressures are under control.

The patient visited SGRH with a history of shortness of breath, malaise, persistent nausea and palpitations. The parents correlated these complaints with her unwillingness to attend school. They noticed that she would be confined to the room without much activity.

The hospital stated that 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.  

Dr. Atul Kakar, Vice Chairman, Department of Medicine, who treated the patient, diagnosed that she was having symptoms suggestive PAH, based upon her clinical examination and echocardiography (ultrasound of the heart).

Dr Kakar said that her pulmonary pressure (the artery which connects heart to the lung) was very high (65mmHg, normal <25mmHg). If not treated it would have led to death. Her symptoms were attributed towards a rare condition - PAH. 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 a lit of pressure through these damaged arteries. Slowly the shortness of breath increases to life threatening conditions and with very quality of life,” he added.

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 nonspecific 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 a fatal condition.

Dr Atul Gogia, Senior Consultant, Department of Medicine, SGRH, pointed out 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.

Patient is currently doing well and is on regular follow up.