Group 1 Pulmonary Hypertension – This is due to issues with the pulmonary arteries themselves.  Multiple different issues can affect the arteries.  Congenital heart defects can cause elevated pulmonary artery pressures. Connective tissue diseases such as scleroderma, lupus, and rheumatoid arthritis may also cause pulmonary hypertension.  As part of the workup for this disease, we may order labwork and an echocardiogram. The only way to definitely diagnose this type of pulmonary hypertension is with a right heart catheterization.

 

Group 2 Pulmonary Hypertension – This occurs due to issues with the left side of the heart.  The most common cause of right-sided heart issues is left-sided heart issues.  Often this can be due to a very weak left heart muscle wall (called systolic dysfunction, or heart failure with reduced ejection fraction).  Another very common cause is stiffness of the left heart wall (called diastolic dysfunction).  Another very common cause is valve regurgitation, or leaky valves.  All of these result in a heart that cannot pump fluid forward effectively…and so the heart spills backwards into the organ behind…the lungs. We work with the cardiologists to manage this type of PH, which usually requires an echocardiogram and sometimes a stress test, transesophageal echocardiogram, and sometimes a right heart catheterization to diagnose.

 

Group 3 Pulmonary Hypertension – This type of pulmonary hypertension is due to issues with the lungs.  When the lungs become scarred, due to lung fibrosis or smoking-related diseases such as COPD (chronic obstructive pulmonary disease) or emphysema, the pressures of the pulmonary arteries inside the lungs also can increase.  As part of the diagnostic workup, we will obtain a CT scan of the chest, do pulmonary function tests (PFTs), and do a right heart catheterization to check the pressures.

 

Group 4 Pulmonary Hypertension – One of the main jobs of the pulmonary arteries is to filter clots out of the body.  Sometimes these clots can start to accumulate and cause blockages in the pulmonary arteries, leading to elevated pressures. In some instances CT scans with contrast do not identify these clots, and we will order a more accurate scan called a ventilation/perfusion (V/Q) scan.  We then may apply direct contrast to the pulmonary arteries during a diagnostic right heart catheterization to evaluate where these clots are.

 

Group 5 Pulmonary Hypertension – Multiple other miscellaneous issues such as sarcoidosis, end-stage renal disease, can lead to Group 5 PH.