Pulmonary Hypertensive Crisis

Pulmonary hypertensive crises are moments of stress during which the pulmonary pressure increases drastically, resulting in an overload of pressure on the right heart ventricle and drop in cardiac output.

Due to these episodes, patients are at higher risk of suffering hypoxia, which is consistent with poor levels of oxygen in the tissues and can cause an even more severe increase in pulmonary vascular resistance, and even death.

Pulmonary hypertensive crises are closely related to pulmonary hypertension, particularly in children.

Persistent pulmonary hypertension of the newborn (PPHN) is a rare and rapidly developing condition that affects babies’ hearts at birth. In newborns, the most common cause for the development of the disease is a failure to develop a normal fall in pulmonary vascular resistance, while older children usually have congenital heart defects or idiopathic pulmonary hypertension.

Pulmonary hypertensive crises are moments of pulmonary vascular constriction acute rising that occur in these patients, and treatment is not very different from the treatment of pulmonary hypertension.

Symptoms and diagnosis

During a pulmonary hypertensive crisis, the enlargement of the right ventricular causes a shift in the interventricular septum toward the left heart ventricle, which pressures it and prevents it from filling. The cardiac output becomes compromised, which can be identified through symptoms like irregular heartbeat, racing pulse, passing out or dizziness, progressive shortness of breath particularly during exercise or activity, and difficulty in breathing at rest.

Due to the severity of pulmonary hypertensive crises, patients who are more likely to suffer them need to be particularly aware of the symptoms and early signs.

According to a study, “Diagnosis and management of postoperative pulmonary hypertensive crisis,” patients submitted to surgeries to treat pulmonary hypertension are particularly at risk of suffering crises. Similarly to paying attention to early signs, preoperative evaluation, including invasive hemodynamics is one of the ways of detecting the possibility of crises.

Treatment and prognosis

There is no cure for pulmonary hypertension, but there are treatments to manage the disease and help patients deal with the burden of its symptoms.

To address pulmonary hypertensive crises, there are a combination of routine emergency medication and medical practices designed to decrease the pulmonary arterial pressure and grant support to the right heart ventricle.

While patients who suffer from pulmonary hypertension face a survival rate of about five years after diagnosis, patients with pulmonary hypertensive crises have a mortality rate two times higher, and the same is also true for patients who suffer from pulmonary hypertensive crises in the perioperative setting. To prevent further consequences and improve survival, fast recognition and intervention are determinant.

Note: Pulmonary Hypertension News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

A Conversation With Rare Disease Advocates