Pulmonary Venous Hypertension (PVH)

Pulmonary venous hypertension (PVH) is due to the heart being unable to efficiently carry blood away from the lungs. This type is part of WHO Group 2 pulmonary hypertension (PH) and also may be called isolated post-capillary PH.

Causes of PVH

Pulmonary hypertension due to left heart disease is the most common form of PH and is generally caused by conditions affecting the left side of the heart. Normally, deoxygenated blood is pumped from the right side of the heart through the lungs to pick up oxygen, and is transported back through the left side of the heart where it can be dispersed throughout the body. When there is an issue with the left side of the heart, blood cannot be moved away from the lungs effectively.

PVH can occur as a common complication of any type of left heart disease. One example is as a complication of mitral valve disease. The mitral valve controls blood flow between the two chambers of the left side of the heart, and prevents blood from flowing backward, also known as mitral regurgitation.

When the mitral valve is not functioning correctly, the oxygen-filled blood can move backward into the left atrium (the top chamber of the heart) instead of leaving the heart to deliver oxygen to the rest of the body. This results in an increased volume of blood, which can increase the blood pressure in the left side of the heart and in the blood vessels connecting the lungs to the heart. Ultimately, this also can result in the heart pumping less oxygenated blood through the body.

Symptoms of PVH

The symptoms of PVH are similar to those of other types of PH. Patients may experience shortness of breath, tiredness, chest pain, and fainting during normal activity, but are comfortable at rest. Swelling of the legs and ankles also may occur.

Patients with PVH often have sleep apnea, which is repeated stops and starts of breathing while sleeping.

Diagnosis of PVH

The initial diagnosis of PVH involves the same tests used in other forms of PH. These include imaging scans to confirm any abnormalities in blood vessels and heart, as well as tests to measure lung function and assess the pressure and blood flow in the pulmonary arteries.

The patient’s medical history also is considered during the diagnosis, such as whether there is a history of left heart disease. This may be combined with a physical exam, to check for known symptoms of PVH such as left-sided heart murmurs, which can indicate blood flowing backward through an ineffective valve.

One of the main diagnostic factors for PVH is an increase in pulmonary capillary wedge pressure, used to estimate the left atrial pressure of the heart. This is measured with a procedure called right heart catheterization.

However, it can be difficult to differentiate a diagnosis of PVH from pulmonary arterial hypertension (PAH) because the pulmonary arterial pressure can rise as a consequence of issues with the left side of the heart.

Treatment of PVH

With PVH, treatment is focused on the underlying cause of the disease. For example, surgery to repair or replace a faulty mitral valve can be an option. Medications such as beta blockers may be used to treat heart failure, as they reduce the heart’s workload.

Angiotensin-converting enzyme (ACE) inhibitors also may be used to treat PVH. ACE inhibitors target the enzyme needed to form a molecule, angiotensin II, that constricts blood vessels. This allows blood vessels to relax, allowing for easier blood flow while reducing blood pressure.

Other general therapies for PH may be prescribed to treat PVH, such as diuretics to remove excess fluid and reduce swelling.

There is some controversy as to whether medications used to treat PAH are effective for patients with PVH, due to a lack of evidence from large, controlled clinical trials. Currently, they are not approved to treat PVH, but are sometimes used off-label. 

 

Last updated: June 29, 2021

***

Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.


A Conversation With Rare Disease Advocates