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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.
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.
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