EXPERT: DR. VIKAS KOHLI (PEDIATRIC CARDIOLOGIST & PH SPECIALIST)
Pulmonary hypertension (PH) can be difficult to diagnose in a routine medical exam because the most common symptoms of PH, such as breathlessness, fatigue and dizziness, are also associated with many other conditions.
Echocardiogram/ Echocardiography in this procedure, electrodes are placed on the patient’s skin, and a sonogram of the heart is taken. This painless procedure is often used to make a preliminary diagnosis by estimating the pressures in the right heart and assessing how well the heart is functioning. Other heart conditions that produce symptoms similar to PH may be diagnosed with an echocardiogram. In addition, an echocardiogram may be used to monitor a patient’s condition.
Chest X-rays can reveal an enlarged right ventricle or pulmonary arteries. Chest X-rays can also show signs of emphysema or scarring (interstitial fibrosis) of the lungs.
These tests measure how much air your lungs can hold, how much air moves in and out of them and the lungs’ ability to exchange oxygen. These tests may be performed to potentially identify its cause.
This diagnostic tool tests for blood clots in the lungs by producing a picture of air and blood flow to the lungs. A small dose of radioactive material is breathed in and another small dose is injected via a blood vessel into the lungs. The doctor will review the images that are produced to evaluate the health of the lungs.
Right-heart catheterization is one of the most accurate and useful tests to get a definitive diagnosis for pulmonary hypertension. This is the only test that directly measures the pressure inside the pulmonary arteries, and it should be done in all patients at least once to confirm a patient’s diagnosis of PH. During the test, doctors insert a catheter (a thin rubber tube) through a large vein in the patient’s groin or neck. They then pass the catheter up into the patient’s heart to measure the blood pressure in the right side of the heart and lungs.
Although there is currently no cure for Pulmonary Hypertension, there are treatment options available and more are on the horizon. Treatments include what health care providers call conventional medical therapies as well as oral, inhaled, subcutaneous (into the skin), and intravenous (into the vein) options. Depending on the severity of PH, heart or lung transplant may also be an option.
Table: Recommendations for efficacy of drug monotherapy for pulmonary arterial hypertension (group 1) according to World Health Organization functional class. The sequence is by pharmacological group, by rating and by alphabetical order.
The signs and symptoms of pulmonary hypertension develop slowly. You may not notice them for months or even years. Symptoms get worse as the disease progresses.
Pulmonary hypertension signs and symptoms include:
Transthoracic Echocardiography is used to image the effects of PH on the heart and estimate PAP from continuous wave Doppler measurements. Echocardiography should always be performed when PH is suspected and may be used to infer a diagnosis of PH in patients in whom multiple different echocardiographic measurements are consistent with this diagnosis.
The probability of PH may then be judged as high, intermediate or low. When interpreted in a clinical context, the echocardiographic result is required to decide the need for Cardiac Catheterization in individual patients. To facilitate and standardize assignment to the level of probability of PH, several additional echocardiographic signs are proposed in addition to criteria based on TRV.
These signs provide an assessment of the RV size and pressure overload, the pattern of blood flow velocity out of the RV, the diameter of the PA and an estimate of RAP. Their measurement has been defined in recommendations endorsed by the EACVI. Echocardiography can help detect the cause of suspected or confirmed PH. Two-dimensional, Doppler and contrast examinations can be used to identify CHD.
High pulmonary blood flow found on pulsed wave Doppler in the absence of a detectable shunt or significant dilatation of proximal PA despite only moderate PH may warrant transoesophageal examination with contrast or cardiac magnetic resonance (CMR) imaging to exclude sinus venous atrial septal defect and/or anomalous pulmonary venous return.
In cases of suspicion of LV diastolic dysfunction, Doppler echocardiographic signs should be assessed even if their reliability is considered low. RHC should be considered when the diagnosis remains uncertain after non-invasive investigations.
TABLE: Diagnostic management is suggested according to the echocardiographic probability of pulmonary hypertension in patients with symptoms compatible with pulmonary hypertension, with or without risk factors for pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension.
Appropriate diagnosis and analysis of the problem are necessary before starting any treatment. Treatment varies by patient, based on the different underlying causes, but generally includes:
Many different types of medications are available to treat pulmonary hypertension. Treatment choices, such as those listed below, depending on the underlying cause of pulmonary hypertension, how severe the pulmonary hypertension is, how likely it is to progress, and a patient’s drug tolerance.
Call us if you are having problems with:
Sign up to hear about upcoming shows, albums, and events.