Pulmonary Vascular Disease Program Conditions and Treatments

Pulmonary vascular disease (PVD) includes a spectrum of conditions or diseases in which the most serious and common complication is pulmonary hypertension (PH). As one of the largest pulmonary vascular disease programs in the nation, we treat mild to severe pulmonary hypertension of all types in children of all ages. We coordinate care among a team of specialized providers to treat children with advanced pulmonary hypertension who have a range of health needs, including heart failure, single ventricle heart conditions, lung failure, lung transplantation, Alagille syndrome, and others.

• Pulmonary hypertension is usually treated with medications and sometimes supplemental oxygen. We offer all types of therapies, including new and groundbreaking ones, and help you navigate access to these therapies.
• While we offer many treatments, there is no cure for some types of pulmonary hypertension and some lung diseases. We work closely with the Center for Advanced Lung (CEAL) Therapies to provide options for your child with advanced lung disease or severe pulmonary hypertension. Some children are candidates for the reverse Potts shunt, a surgery that redirects blood flow in the lungs and can improve symptoms and delay lung or heart-lung transplant. Other innovative therapies include mechanical devices that support your child’s lungs and heart prior to transplant.

About pulmonary hypertension

The heart and lungs are interconnected and work together to bring blood and oxygen to the body. The heart pumps blood into the lungs, where the blood is oxygenated, returned to the heart, and sent through major arteries throughout the body. With pulmonary hypertension, the blood vessels in the lungs either do not form correctly, or they become blocked or narrowed over time, causing high blood pressure in the lungs. When the lungs have high blood pressure, the heart has to work harder to pump blood through them, which can lead to heart failure. Pulmonary hypertension is often accompanied by shortness of breath, chest discomfort, fatigue, poor growth, lightheadedness, and fainting.

Pulmonary hypertension can occur in several ways. It can develop due to genetic abnormalities (sometimes inherited). It can also be related to one or more of the following conditions: congenital heart disease, lung disease, rheumatologic disease, sleep apnea, liver disease, prematurity, blood disorders, and exposure to certain drugs (typically in utero). In some cases, children have idiopathic pulmonary arterial hypertension, meaning no cause for the disease has been identified.

Pulmonary hypertension is often a chronic disease, but the course and severity varies widely and can be influenced by an associated diagnoses or cause.