The Condition


Pulmonary Hypertension, which essentially is high blood pressure in the lungs, is a serious group of diseases.

The blood carrying vessels in the lungs grow narrower causing the right side of the heart (that brings blood to the lungs for oxygen) to work harder. If chronic, over a period of time, the arteries of the lung harden and the right side of the heart grows bigger.

PH can be usually treated as long as the underlying cause can be. Eg.: plugging of a hole in the heart

Pulmonary Arterial Hypertension (PAH), a subset of the PH group bearing poor medical prognosis, is currently considered incurable and life-threatening. Idiopathic and Heritable forms of PAH, extremely rare, are considered to affect three times more women than men – especially, young women.

Statistics on PAH:

http://www.ncbi.nlm.nih.gov/pubmed/22798320

http://www.ncbi.nlm.nih.gov/pubmed/20558556

PAH can affect children too. In the years to come, it is believed that the incidence of PAH among children might be 40-50 per million with no approved therapies being applicable to them just yet.

PAH is designated as a critical illness in most medical insurance policies.


In PH, symptoms are not unique. They can bear a lot in common with other respiratory diseases. Symptoms can be:

  • Breathlessness with everyday activities (especially climbing stairs or going uphill)
  • Tiredness
  • Discomfort in the chest
  • Palpitating/ throbbing heart
  • Swelling of the abdomen, in the legs and around ankles
  • Dizzyness
  • Fainting spells
  • Bluish lips or skin

Check: http://www.phassociation.org/SometimesItsPH


Dana Point Clinical Classification of Pulmonary Hypertension

1. Pulmonary Arterial Hypertension (PAH)

  • 1.1 Idiopathic (IPAH)
  • 1.2 Heritable (HPAH)
    • 1.2.1 BMPR2 Gene
    • 1.2.2 ALK1, Endoglin Genes (with/ without Hereditary Haemorrhagic Telangiectasia - HHT)
    • 1.2.3 Unknown
  • 1.3 Induced by Drugs and Toxins
  • 1.4 Associated Type (APAH):
    • 1.4.1 Connective Tissue Diseases
    • 1.4.2 HIV Infection
    • 1.4.3 Portal Hypertension
    • 1.4.4 Congenital Heart Disease
    • 1.4.5 Schistosomiasis
    • 1.4.6 Chronic haemolytic anaemia
  • 1.5 Persistent Pulmonary Hypertension of the Newborn

2. Pulmonary Hypertension due to Left Heart disease

  • 2.1 Systolic Dysfunction
  • 2.2 Diastolic Dysfunction
  • 2.3 Valvular Disease

3. Pulmonary Hypertension due to Lung Diseases and/or Hypoxia

  • 3.1 Chronic Obstructive Pulmonary Disease (COPD)
  • 3.2 Interstitial Lung Disease
  • 3.3 Other Pulmonary Diseases, with mixed restrictive and obstructive pattern
  • 3.4 Sleep-disordered Breathing
  • 3.5 Alveolar Hypoventilation Disorders
  • 3.6 Prolonged Exposure to High Altitude
  • 3.7 Developmental Abnormalities

4. Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

5. Pulmonary Hypertension with unclear and/or multifactorial mechanisms

  • 5.1 Haemotological Disorders: Myeloproliferative Disorders, Splenectomy
  • 5.2 Systemic Disorders: Sarcoidosis, Pulmonary Langerhans Cell Histiocytosis, Lymphangioleiomyomatosis, Neurofibromatosis, Vasculitis
  • 5.3 Metabolic Disorders: Glycogen Storage Disease, Gaucher Disease, Thyroid Disorders
  • 5.4 Others: Tumoral Obstruction, Fibrosing Mediastinitis, Chronic Renal Failure (on dialysis)

 


Dr. Sean Gaine, a leading PH specialist, notes:

Pulmonary Hypertension has many causes. In some cases it is caused by clots that block the circulation and increase the pressure, but then the pressure comes down when the clots dissolve. In some cases it is a manifestation of acute inflammation in the body, and can come down when the inflammation is treated or resolves. In some cases, like HIV, it can have a dramatic response to current treatments. But in the majority of cases it is a fixed, progressive and incurable condition.

There is some evidence that lifestyle can play a role, but I believe that genetics are even more important.

Genes make someone susceptible to insults that would otherwise have no effect on the majority of people (e.g. thousands took diet pills but a small proportion developed fatal PH.).

Dr. Gaine provides more insights here:

http://www.pulmonaryhypertension.ie/wp-content/uploads/2012/11/Advances-in-pulmonary-hypertension-IMT-article.pdf