PH is a chronic debilitating disease associated with increase in pulmonary artery pressures. It may be caused by congenital heart disease, lung problems or idiopathic. The disease is progressive and is associated with right ventricular dilation, failure and early death.
What is PH clinic?
PH clinic is a multi-disciplinary clinic with single point of contact to diagnose, investigate and treat patients with this disease.
Why is a separate clinic required for PH?
Patients with PH require extensive work up to define the cause of PH. It requires a multidisciplinary team including a PH specialist, adult and pediatric cardiologist, pulmonologist, rheumatologist and a cardiac surgeon.
Once accurate diagnosis is made, the patient needs to be followed up in a systematic manner with echocardiogram, six minute walk test and blood investigations. Cardiac catheterization by experts trained in this is an important aspect of diagnosis and for guiding therapy.
Drug therapy is constantly improving and needs expert knowledge and monitoring.
Who is the target patient population?
Children and adults with congenital heart disease.
Patients having suffered from pulmonary embolism
Patients with rheumatological diseases
Patients with lung disease
Patients with portal hypertension
Patients with chronic renal diseases on hemodialysis
Idiopathic where none of the above exist
Location and timing of the clinic?
Kokilaben Dhirubai Ambani Hospital, Childrens Heart Centre
2nd Floor, Wednesday and Friday 2pm to 5 pm.
What are the treatment options available in India?
Since the number of drugs available in India to treat PH are currently limited accurate diagnosis of the etiology is crucial to therapy.
Calcium channel blocker (Diltiazem/Nifidepine): Should only be administered to a select group of patients after performing the cardiac catheterization with vasodilator therapy.
Protacyclin analogues (Ilioprost, trepostinil and epoprostenolol): even though very effective, these drugs are not available in India. However they can be imported by the patient for individual use.
Lung/Heart lung transplant
What extra benefit has the PH clinic provided so far?
First Potts shunt for refractory idiopathic PAH
Pulmonary endarterectomy for CTEPH
Diagnosis and therapy of rare causes of PAH like Abernathy malformation
What does the future hold for PAH clinic?
Introduction of prostacyclin for therapy of refractory PAH. (Currently not marketed in India, but can be exclusively made available at our hospital). Prostacyclins are the only drugs which have shown mortality benefits in patients with PAH.
Ample opportunities for research in this rare disease
Patients with refractory PAH would be candidates for heart lung transplant.
Pulmonary hypertension specialist: Dr. Prashant Bobhate
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