Pulmonary hypertension

Normally, as blood returns from supplying oxygen throughout the body, the heart’s right ventricle pumps the blood into the lungs through the pulmonary artery, where it picks up more oxygen. In pulmonary hypertension, there is high blood pressure in the lungs, specifically the pulmonary arteries of the lungs. When infants and children have pulmonary hypertension, the right ventricle has to work harder to pump blood to the lungs. This extra work can cause the right ventricle to get bigger, weaken, and even fail.

Children get pulmonary hypertension through a variety of different ways including over circulation from congenital heart defects, primary or idiopathic pulmonary hypertension, left sided heart defects that cause blood to back up into the lungs, chronic lung disease, and other rare conditions.

Infants, children, teenagers and adults can get pulmonary hypertension from overcirculation from congenital heart defects. This refers to having an unrepaired heart lesion that causes increased blood flow to the lungs. Over time, this causes damage to the pulmonary arteries and then pulmonary hypertension occurs. If nothing is done, it can continue to worsen and cause Eisenmenger syndrome. Certain structural congenital heart defects that left untreated that can cause pulmonary hypertension include:

  • Atrial septal defect: a hole between the two atria
  • Ventricular septal defect: a hole between the two ventricles
  • Atrioventricular canal defect: a large hole in the center of the heart creating a communication between the atria, ventricles, or both. There can also be valve abnormalities associated with this lesion.
  • Patent ductus arteriosus: a connection between the aorta and the pulmonary artery.

Primary pulmonary hypertension is a rare but serious form of pulmonary hypertension. It is also called idiopathic pulmonary hypertension because there is not a cause for it, meaning the patient was likely born with it because of their genetics.

Structural abnormalities on the left side of the heart may also cause pulmonary hypertension. These abnormalities can include aortic stenosis and mitral stenosis. The pulmonary hypertension results from blood backing up into the lungs from the left atrium. A very rare pulmonary vein problem with a poor prognosis is pulmonary vein stenosis which also causes blood to back up into the lungs, but the blockage is outside the heart, between the lung and the left atrium. Lastly, a poor functioning heart, as seen in severe cardiomyopathy or myocarditis, may also create pulmonary hypertension because of how poorly the heart pumps.

One of the more common causes of pulmonary hypertension is lung disease. Because of prematurity, many neonates are treated with ventilators and oxygen that while lifesaving, create the side effect of oxygen toxicity and micro tears in the lungs from the efforts of the ventilator. As the body tries to heal these injuries, pulmonary fibrosis occurs as a result of this healing process. This creates makes it harder to push blood through the lungs creating pulmonary hypertension. Any lung disease may do something similar, such as connective tissue lung conditions or diaphragmatic hernia lung compression.

The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute, affiliated with the University of Louisville, have the experience and skill to diagnose and provide care for pulmonary hypertension.

The Society of Thoracic Surgeons has ranked Norton Children’s Heart Institute’s pediatric heart care among the best in the region. Norton Children’s has a network of outreach diagnostic and treatment services conveniently located throughout in Kentucky and Southern Indiana.


Pulmonary hypertension symptoms often include difficulty breathing, which may get worse with exercise or activity. Other symptoms can include fainting or weakness when active; chest pain; bluish lips, hands and feet (cyanosis); and being more tired than usual.

A congenital (present at birth) heart defect is the most common cause of pulmonary hypertension in infants and children. Surgical repair of the congenital heart lesion often will improve the pulmonary hypertension over time. Another common cause is lung disease due to premature birth or a congenital condition. Chronic lung disease can also improve over a child’s first 6 years of life which can also improve their pulmonary hypertension.

Diagnosing pulmonary hypertension

Norton Children’s Heart Institute pediatric cardiologists may conduct a number of different tests to diagnose pulmonary hypertension. Some of these tests may include:

  • Chest X-ray: This shows a picture of the heart and surrounding organs.
  • Echocardiogram (echo): This test uses sound waves (ultrasound) to produce images of the heart and blood vessels’ structures on a screen. It can show the structure and function of the heart. Norton Children’s Heart Institute has 28 tele-echo locations throughout Kentucky and Southern Indiana.
  • Electrocardiogram (ECG or EKG): This test checks the heart’s electrical activity to show damage or irregular rhythms.
  • Heart catheterization: During this procedure, a thin, long tube called a catheter is inserted into a large blood vessel in the neck or groin and guided into the heart. This invasive procedure studies the structure, function, and provides direct pressure measurements of the heart and blood vessels around the heart, like the pulmonary artery. The interventional pediatric cardiologist performing the procedure can give different gases like oxygen and nitric oxide during the catheterization to see if the pulmonary hypertension improves. This will help decide what treatment is best for each patient.
  • A six-minute walking test can show how tired your child gets during exercise and how well he or she breathes. We’ll also make a record of your child’s oxygen levels over time to keep track of the progress of the disease.

Pulmonary hypertension treatment

While some forms of pulmonary hypertension can be cured, others will require the specialists at Norton Children’s Heart Institute to provide treatments to help ease symptoms and slow the progress of the disease.

Treatment for pulmonary hypertension may include:

Surgery for congenital cardiac lesions that are creating too much pulmonary blood flow or are causing left sided obstructions

Medications that make it easier for the heart to pump, make the heart contract better, and/or remove fluid from the body called diuretics

Oxygen, Nitric oxide, or medications that relaxes the vessels in the lungs

Anti-coagulants to prevent blood clots

Pulmonary hypertension is a serious condition that requires a pediatric cardiologist to provide a life-long treatment plan. Norton Children’s Heart Institute’s pulmonary hypertension program can provide this care in Norton Children’s Hospital and through our Norton Children’s Heart Institute 15 outreach clinics, including four Norton Children’s Hospital Outpatient Centers located in Bowling Green, Frankfort, Owensboro and Paducah.

Why Norton Children’s Heart Institute?

Norton Children’s Heart Institute, affiliated with the University of Louisville, is a comprehensive pediatric heart surgery, heart failure and heart transplant program serving Kentucky, Southern Indiana and beyond.

The goal of the full-service Norton Children’s Heart Institute is to provide care for the child and the whole family. Our specialists are prepared to repair even the most complex congenital and acquired heart conditions.

Our heart team includes:

  • Pediatric cardiothoracic surgeons
  • Pediatric transplant surgeons
  • Pediatric cardiologists
    • Fetal cardiologists
    • Adult congenital heart cardiologists
    • Heart failure/heart transplant cardiologists
    • Pediatric electrophysiologists
    • Pediatric cardiac catheterization cardiologists
  • Pediatric cardiovascular anesthesiologists
  • Pediatric intensive care physicians
  • Cardiac critical care nurses
  • Critical care pharmacists
  • Family support team
  • Child life specialists
  • Rehabilitation specialists
  • Social workers
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