Pulmonary venous hypertension (PVH) is associated with high blood pressure in the arteries of the lungs (pulmonary arteries) due to the heart being unable to efficiently carry blood away from the lungs. This type is part of  WHO Group 2 pulmonary hypertension and also may be called isolated postcapillary pulmonary hypertension.

Causes of PVH

PVH is one of the more common forms of pulmonary hypertension 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 effectively moved away from the lungs.

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, as well as preventing 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 leading from the lungs to the heart. Ultimately, this also results in the heart pumping less oxygenated blood around the body.

PVH can occur as a result of left heart failure, and this is one of the most common causes of pulmonary hypertension.

Symptoms of PVH

The symptoms of PVH are similar to those of other types of pulmonary hypertension. The main symptoms include shortness of breath, tiredness, and swelling of the legs and ankles. Patients with PVH often have sleep apnea or restricted breathing while sleeping or in a reclined position.

Diagnosis of PVH 

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

The patient’s medical history also will be considered during the diagnosis, such as whether they have a history of left heart failure or left heart disease. This may be combined with a physical exam, to check for known symptoms of PVH such as listening for 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 (PCWP), which can be used to estimate the left atrial pressure of the heart (elevated in PVH due to abnormalities in the left side of the heart). This is measured using a test called right heart catheterization. However, it can be very difficult to differentiate a diagnosis of PVH from WHO Group 1 pulmonary hypertension, or 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 treating 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 and angiotensin-converting enzyme (ACE) inhibitors may be used to prevent congestive heart failure. 

Beta blockers block the effects of the sympathetic innervation of the heart.  This decreases the heart’s workload, so it needs less blood and oxygen.  This aids in lowering blood pressure and helps to control abnormal beat rhythms that are either too fast or irregular. ACE inhibitors block the enzyme that is needed to form a molecule that constricts blood vessels.  This allows blood vessels to relax, allowing for easier blood flow while reducing blood pressure.

Other general therapies for pulmonary hypertension may be prescribed to treat PVH, such as diuretics, to remove excess fluid and reduce swelling, anticoagulants to prevent blood clots, or oxygen therapy, to improve oxygen supply to other organs and tissues.

There is some controversy as to whether medications used to treat PAH are effective and safe 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

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