PULMONARY HYPERTENSION INDIA

PULMONARY HYPERTENSION INDIAPULMONARY HYPERTENSION INDIAPULMONARY HYPERTENSION INDIA

PULMONARY HYPERTENSION INDIA

PULMONARY HYPERTENSION INDIAPULMONARY HYPERTENSION INDIAPULMONARY HYPERTENSION INDIA
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TYPES OF PULMONARY HYPERTENSION

Group 1. Pulmonary Arterial Hypertension (PAH)

  • WHO Group 1 refers to pulmonary arterial hypertension (PAH), which is caused when the arteries in the lungs become narrowed, thickened or stiff. The right side of the heart must work harder to push blood through these narrowed arteries. This extra stress can cause the heart to lose its ability to pump enough blood through the lungs to meet the needs of the rest of the body.  
  • There are several types of PAH. Idiopathic PAH (IPAH) is PAH that occurs without a clear cause. Heritable PAH (HPAH is linked to genes that are inherited from family members. PAH can also develop in association with other medical conditions including congenital heart disease, liver disease, HIV and connective tissue diseases — such as scleroderma and lupus. PAH can even be associated with past or present drug use, such as the use of methamphetamine or certain diet pills. While there are treatment options for PAH, there is no known cure.

Recommendations for evaluation of the severity of Pulmonary Arterial Hypertension

CT: computed tomography; CTEPH: chronic thromboembolic pulmonary hypertension; DLCO: diffusing capacity of the lung for Carbon Monoxide; PAH: Pulmonary arterial hypertension; PH: pulmonary hypertension. aClass of recommendation. level of evidence. reference(s) supporting recommendations.

Group 2. Pulmonary Hypertension Due to Left Heart Disease

WHO Group 2 includes PH due to left heart disease. In this group of PH, the arteries and lungs are not as thick or stiff as WHO Group 1, but there are problems with how the heart squeezes or relaxes, or problems with the valves on the left side of the heart. Because of this, the left heart is unable to keep up with the blood returning from the lungs — causing a “backup” of blood which raises pressure in the lungs. WHO Group 2 is the most common form of PH.

Management of pulmonary hypertension in left heart disease

COPD: chronic obstructive pulmonary disease; CTEPH: chronic thromboembolic pulmonary hypertension; DPG: diastolic pressure gradient; LHD: left heart disease; LV: left ventricular; PE: pulmonary embolism; PH: pulmonary hypertension; PVR: pulmonary vascular resistance. aClass of recommendation. level of evidence. reference(s) supporting recommendations.

Group 3. Pulmonary Hypertension Due to Lung Disease

WHO Group 3 includes PH due to Chronic Lung disease and/or hypoxia (low oxygen levels). These lung diseases include obstructive lung disease where the lung airways narrow and make it harder to exhale (e.g. COPD or emphysema); restrictive lung disease in which the lungs have a tough time expanding when one inhales (e.g. interstitial lung disease or pulmonary fibrosis); sleep apnea; and living in an area of high altitude for a long period of time. Arteries in the lungs tighten so that blood can only go to areas of the lungs that are receiving the most air and oxygen. This tightening leads to high blood pressure throughout the lungs.

Recommendations for pulmonary hypertension due to lung diseases

CTEPH: chronic thromboembolic pulmonary hypertension; PAH: pulmonary arterial hypertension; PH: pulmonary hypertension; RHC: right heart catheterization. aClass of recommendation. level of evidence. reference(s) supporting recommendations. this recommendation does not apply to patients with end-stage lung disease who are not considered candidates for lung transplantation.

Group 4. Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

WHO Group 4 is called chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH can occur when the body is not able to dissolve a blood clot in the lungs. This can lead to scar tissue in the blood vessels of the lungs, which blocks normal blood flow and makes the right side of the heart work harder. This type of PH is unique because it can potentially be cured through pulmonary thromboendarterectomy (PTE) surgery to remove the blood clots. However, not all CTEPH patients are eligible for this surgery. A drug is also available for CTEPH patients if a doctor determines that a patient is not a candidate for the PTE surgery or if PH remains after the surgery.

Recommendations for chronic thromboembolic pulmonary hypertension

BPA: balloon pulmonary angioplasty; CTEPH: chronic thromboembolic pulmonary hypertension; PAH: pulmonary arterial hypertension; PE: pulmonary embolism; PEA: pulmonary endarterectomy. aClass of recommendation. level of evidence. reference(s) supporting recommendations.

Group 5. Pulmonary Hypertension with unclear and /or Multi-factorial mechanisms

WHO Group 5 is where PH is secondary to other diseases in ways that are not well understood. These associated conditions include, but are not limited to, sarcoidosis, sickle cell anemia, chronic hemolytic anemia, splenectomy (spleen removal) and certain metabolic disorders.

Recommendations for pulmonary hypertension referral centres

CTD: connective tissue disease; CTEPH: chronic thromboembolic pulmonary hypertension; DPAH: drug-induced pulmonary arterial hypertension; HPAH: heritable pulmonary arterial hypertension; IPAH: idiopathic pulmonary arterial hypertension; PAH: pulmonary arterial hypertension; PEA: pulmonary endarterectomy. aClass of recommendation. level of evidence. reference(s) supporting recommendations.

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