Pulmonary hypertension is a complex disorder with multiple pathologies. This is the syndrome resulting from restricted flow through the pulmonary arterial circulation causing high pulmonary arterial resistance which has significant negative impact on right heart (RV) and eventually right ventricle failure.
This is a serious condition with poor prognosis. There are several new therapeutic modalities available but still has 14-16% mortality within one year. Physicians can assess who has poor prognosis. Among these indicators and most important are 1. Poor exercise capacity, 2. Poor RV function by echocardiography, 3. High BNP in blood, enzyme indicative of heart failure. Primary pulmonary hypertension is much more common in females.
Primary cause, although still not completely known, but it has some genetic roots. It is manifested as autosomal dominant disorder but incomplete penetration, i.e. can skip one or two generations. However, it is commonly associated with liver dysfunctions, connective tissue disorders (scleroderma, lupus, rheumatoid arthritis, etc.), HIV, drugs & toxins. Patients who have a history of blood clots in the lungs can also develop pulmonary hypertension. People who have COPD are also prone to this disorder.
Diagnosis heavily relies on a good history and physical exam. Then EKG and echocardiogram with pulmonary function tests are essential for initial screening. Confirmation is done by right heart catheterization. Again, we must rule out other causes like COPD, left heart failure, HIV and connective tissue disorders. At Cardiac Center, we can perform all these screening tests and we have pulmonary Hypertension specialists who are board certified interventional cardiologists, to perform right heart cath to confirm diagnosis. These kinds of testing should only be done by qualified vascular specialists.
In early stages, patients may not have any symptoms. The most common symptom is shortness of breath. Patients also feel more tired and fatigued. They may have chest pain with or without activity. They also feel dizziness and a feeling of passing out. Palpitations are also common in these patients. When disease is advanced, they start having swelling of legs and then fluid accumulation in upper parts of the body.
Management requires multi-disciplinary collaboration. Basic treatment includes oxygen therapy, careful diet, and tailored exercise. All patients should be on blood-thinners like Coumadin. Those patients should have a very tight preventative schedule for vaccinations and avoid pregnancy at all costs. Diuretics are the first line of therapy to reduce fluid retention. Some patients also benefit from calcium channel blockers.
Specific therapies are emerging and include so for these class of drugs to tackle this condition.
- Prostaglandis, which is usually given by IV and its main therapy for critically ill patients. First drug showed mortality benefits was epoprosterol give continuous IV infusion. This is followed by Treprostinil – can give s.c., Iloprost – aerosol rx 6 times per day
- Endothelial receptor antagonist
- Phosphodiesterase inhibitors
The last two are given in mild to moderate conditions. We can combine the above medications to maximize benefits. These patients need to be followed closely by pulmonary hypertension specialist in properly designated centers for pulmonary hypertension prescriptions. They need to be seen for 3-6 month follow ups.