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 can get pulmonary hypertension through a variety of ways, including overcirculation 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 caused by congenital heart defects. This refers to having an unrepaired heart lesion that allows 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 left untreated can cause pulmonary hypertension, including:

Primary pulmonary hypertension is a rare but serious form of pulmonary hypertension. It also is 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 also may 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 condition with a poor prognosis is pulmonary vein stenosis, which also causes blood to back up into the lungs, but the blockage is outside of the heart, between the lung and the left atrium. Lastly, a poorly functioning heart, as seen in severe cardiomyopathy or myocarditis, may create pulmonary hypertension because of how poorly the heart pumps.

One of the more common causes of pulmonary hypertension is lung disease. Many premature babies 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. This makes it harder to push blood through the lungs, creating pulmonary hypertension. Any lung disease may cause a similar issue, 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 UofL School of Medicine, have the experience and skills to diagnose and provide care for pulmonary hypertension.

Norton Children’s has a network of outreach diagnostic and treatment services conveniently located throughout in Kentucky and Southern Indiana.

Symptoms

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 six years of life, which also can improve 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: 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 heart’s structure and function, and provides direct pressure measurements of the heart and blood vessels around the heart, such as 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.
  • Stress test: 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 (diuretics)
  • Oxygen, nitric oxide or medications that relax the vessels in the lungs
  • Anticoagulants 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 Norton Children’s Heart Institute’s 15 outreach clinics, including four Norton Children’s Hospital Outpatient Centers located in Bowling Green, Frankfort, Owensboro and Paducah, Kentucky.

Why Choose Norton Children’s Heart Institute

The Adult Congenital Heart Association has recognized Norton Children's Heart Institute for its expertise treating adult congenital heart disease.

  • Norton Children’s Hospital has been a pioneer in pediatric cardiothoracic surgery, performing Kentucky’s first pediatric heart transplant in 1986 and becoming the second site in the United States to perform an infant heart transplant.
  • The American Board of Thoracic Surgery has certified our cardiothoracic surgeons in congenital heart surgery.
  • The Adult Congenital Heart Association has accredited Norton Children’s Heart Institute’s Adult Congenital Heart Program as the only comprehensive care center in Kentucky and Indiana treating adults born with a heart defect.
  • More than 5,000 children a year visit Norton Children’s Heart Institute for advanced heart care.
  • Norton Children’s Heart Institute has offices across Kentucky and Southern Indiana to bring quality pediatric heart care closer to home.
  • The Jennifer Lawrence Cardiac Intensive Care Unit (CICU) at Norton Children’s Hospital is the largest dedicated CICU in Kentucky, equipped with 17 private rooms and the newest technology available for heart care.
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