Is Pulmonary Hypertension a Rare Disease?

Is Pulmonary Hypertension a Rare disease?

No, pulmonary hypertension (PH) is not at all a rare disease. The reason is very simple that it is associated with lots of diseases and hence because of so many associations its occurrence is quite common.

It is seen that isolated pulmonary hypertension is rare to occur. It is usually accompanied by heart diseases. So while diagnosing any pulmonary abnormality it is rule of thumb to check for cardiac abnormalities as any abnormalities in heart have a direct impact on the pulmonary system so as a wise clinician never forget to check cardiovascular system while diagnosing a case of pulmonary origin.

It is always said that a doctor is none less than a detective who needs to unveil the hidden mystery and this is the fact what is to be followed while making a diagnosis.

Pulmonary Hypertension PH is Quite Common

So considering pulmonary hypertension, it is a common disease which can occur when there are any cardiac abnormalities. To be a precise increase in pulmonary hypertension can be divided into arterial and venous causes.

Pulmonary artery hypertension can be associated with Collagen vascular disorder, the presence of congenital shunting which leads to transfer of blood from systemic to pulmonary circulation.

Other causes which can occur from pulmonary vein hypertension include left-sided valvular heart disease, pulmonary venous obstruction, and pulmonary venoocclusion disease.

The most commonly encountered problem in many patients is thromboembolism. It is said that chronic thromboembolism for more than 3 months is a risk factor for pulmonary hypertension.

Many people consider that pulmonary hypertension is an adult disease but this is really untrue. Many developmental anomalies which include patent ductus arteriosus and septal defect which increases pressure on right atrium can cause pulmonary artery hypertension. So according to the defect, one can divide into pulmonary artery or venous hypertension.

Pulmonary hypertension can also occur in various diseases of lungs which include interstitial lung disease, associated with fibrosis of lung parenchyma. This imparts resistance to pulmonary circulation and thus there is increased pressure in both arterial and venous system. Therefore pulmonary hypertension is associated with multiple diseases ranging from congenital diseases to adulthood diseases. The disease present with dyspnoea and most of the time associated with any heart abnormality.


In many developing countries pulmonary hypertension can occur from an infectious cause which includes mostly HIV in developing countries and schistosomiasis, histoplasmosis, sarcoidosis in developing countries. So accordingly it varies.

So a disease with so many aetiologies can never be rare. Instead of it is mostly seen in cardiac patients. So being a rare disease it should be diagnosed early and treated appropriately by every clinician. So treatment depends upon the etiological factor but it should be kept in mind that the primary cause must be treated first so as to prevent the progression of the disease. Like in case of cardiac abnormalities which can be a septal defect, it should be treated early as if it is not treated early there is no use of treating pulmonary hypertension as it may reoccur because the underlying defect is still not corrected.

So what Drugs should be given in Pulmonary Hypertension?

Calcium channel blockers are the preferred drugs as these causes vasodilation and reduce pulmonary hypertension. Nifedipine 240 mg/dl is the preferred drug as it reduces the resistance and decreases hypertension. Another class of drug include endothelin receptor antagonist which includes bosentan as even approved for the treatment of pulmonary hypertension. Phosphodiesterase-5 inhibitor includes sildenafil can also be given but certain precautions to take while giving this drug in hypotension patient.

There are few other drugs which can be used which include iloprost a prostacyclin analogue. In severe cases, lung transplant can be done.

Special emphasis should be taken while treating a patient of refractory hypertension as these may not respond to medical therapy hence another class of drugs should be used in such patients

Also Read:

Pramod Kerkar, M.D., FFARCSI, DA
Pramod Kerkar, M.D., FFARCSI, DA
Written, Edited or Reviewed By: Pramod Kerkar, M.D., FFARCSI, DA Pain Assist Inc. This article does not provide medical advice. See disclaimer
Last Modified On:August 2, 2018

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