The prognosis and lifestyle restrictions for the adult patient will vary according to the causes and severity of the defect and symptoms.

Group 1 Idiopathic or heritable pulmonary hypertension is a serious and progressive disease, which may arise at any time in life. It presents numerous risks and complicates the treatment options for both cardiac and non-cardiac health concerns.

It often happens that pulmonary hypertension is well-advanced before it is recognized. Therefore, it is important that anyone suspected of having this disease be screened promptly at a specialized cardiac center.

Symptoms of pulmonary hypertension include cyanosis (blueness due to low levels of oxygen in the body's blood supply), a heart murmur, high pulmonary artery blood pressure, and right ventricular failure. Patients with a family history of pulmonary hypertension, connective tissue disease, or who have used certain appetite-suppressants (e.g. Aminorex fumarate, Fenfluramine) are at special risk for pulmonary hypertension and should be screened.

The screening process will usually include echocardiography, electrocardiography, chest radiography (x-ray), and certain respiratory tests. Angiography and cardiac catheterization may also be performed.

The prognosis for the patient with primary pulmonary hypertension is guarded. However, significant advances in understanding and treatment of the disease are currently being made. In some cases, the disease will advance rapidly; in others, more slowly. Rarely, the disease will go into spontaneous regression.