Pulmonary Hypertension

Pulmonary hypertension (PH) is abnormally high blood pressure that occurs in the arteries of the lungs (the pulmonary arteries). Some cases of PH have no identifiable cause (idiopathic pulmonary hypertension). However, genetic defects and immune system disease may be contributing factors to the development of this disease.

Another type of PH, called secondary pulmonary hypertension, occurs when the arteries in the lungs become too narrow for blood to flow through the vessels normally or because there are too few vessels to begin with. As a result, the heart works harder to pump blood through the lungs, causing pressure in the pulmonary artery to rise. Narrowing of the vessels in secondary hypertension can be caused by an associated medical condition such as blood clots, congenital heart defects and liver disease.

The first symptoms of PH are typically shortness of breath during mild physical activity, although some patients may have fainting spells, especially with exercise.  However, because the symptoms of PH aren’t specific to the condition, it can be difficult to diagnose.

While for most patients there is no “cure” for pulmonary hypertension, certain medications can help lessen symptoms and improve lung and heart function.  Careful monitoring by a cardiologist can help your child live as normal life as possible.

How Boston Children’s Hospital approaches pulmonary hypertension

Here at Boston Children’s, the mission of the Pulmonary Hypertension Program is threefold:

  • Provide the best possible care for our patients. Ours is a multidisciplinary approach using experienced physicians and other clinical professionals who have expertise in specific areas. Your child’s care may also involve consultation with specialists at other institutions, to make sure that we bring the best collective knowledge to each case.
  • Advance our understanding of pulmonary hypertension and develop new therapies. This serves both current and future patients: Our clinical trials regularly bring new treatments to our patients sooner than would be otherwise possible.
  • Train new practitioners. As a program with enormous experience in treating children with pulmonary hypertension, it’s our responsibility to help train the next generation of professionals dedicated to curing this disease.