Over time, the right ventricle becomes more involved, leading to right ventricular failure.

This may cause immediate right ventricular failure, since it may not be able to pump the blood against the pulmonary hypertension.

However, by the time the individual has signs of overt right ventricular failure, there will be histological involvement of the left ventricle.

This may lead to right ventricular failure (dilatation and decreased systolic function of the right ventricle).

For the remainder with pre-fibrotic lung infiltration, about 7% will progress to respiratory failure, right ventricular failure, and death within 10 to 15 years.

Nitric oxide is also administered as salvage therapy in patients with acute right ventricular failure secondary to pulmonary embolism.

Others may have symptoms and signs related to right ventricular failure, such as lower extremity edema, or liver congestion with elevated hepatic enzymes.

During fetal development, this shunt protects the right ventricle from pumping against the high resistance in the lungs, which can lead to right ventricular failure if the DA closes in-utero.

If right ventricular failure develops, right atrial pressure will increase, and this may result in reopening of the foramen ovale, shunting of unoxygenated blood into the left atrium, and systemic cyanosis.

In the absence of isolated right ventricular failure, seen in some patients with right ventricular infarction, a positive abdominojugular test suggests a pulmonary artery wedge pressure of 15 mm Hg or greater.

The NIH IPAH registry from the 1980s showed an untreated median survival of 2-3 years from time of diagnosis, with the cause of death usually being right ventricular failure (cor pulmonale).

In particular sinus node dysfunction, atrial arrhythmias, ventricular arrhythmias including sudden cardiac arrhythmic death, heart failure due to anatomically right ventricular failure or venous obstruction at the level of the baffle or caval anatomy have been described.

Rupture of the intraventricular septum (the muscle separating the left and right ventricles) causes a ventricular septal defect with shunting of blood through the defect from the left side of the heart to the right side of the heart, which can lead to right ventricular failure as well as pulmonary overcirculation.