Pulmonary Hypertension

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Pulmonary hypertension in babies and older children refers to high blood pressure in the pulmonary arteries in the lungs. This causes the heart’s right ventricle to work harder, which can lead to it getting bigger and weaker, and possibly failing.

Pulmonary hypertension in babies can be caused by congenital heart defects that allow increased blood flow to the lungs. Congenital heart defects also can cause pulmonary hypertension as children grow older.

Interventional cardiologists at Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, were some of the first in the world to use a modified Medtronic Micro Vascular Plug to restrict excessive pulmonary artery blood flow that can cause pulmonary hypertension in newborns.

Causes of Pulmonary Hypertension in Babies

Certain structural congenital heart defects left untreated can cause pulmonary hypertension in babies, including:

Over time, a congenital defect can damage the pulmonary arteries, leading to pulmonary hypertension. If the condition continues to worsen, it can cause Eisenmenger syndrome, a condition in which high blood pressure causes the blood flow between heart chambers to reverse.

Rarely, blood vessels in the lungs narrow for no known reason, a condition called primary or idiopathic pulmonary hypertension.

Structural abnormalities on the left side of the heart also may cause pulmonary hypertension in babies. 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. The blockage is found between the lung and the left atrium.

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 in babies is lung disease caused by ventilators. Many babies born pre-term are treated with ventilators and oxygen that, while lifesaving, can create the side effect of oxygen toxicity and microtears in the lungs. 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.

Pulmonary hypertension in babies and older children is a serious and complex condition. The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute have the experience and skills to diagnose and provide care for pulmonary hypertension.

The Society of Thoracic Surgeons has rated 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.

Symptoms of Pulmonary Hypertension in Children

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.

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

Diagnosing Pulmonary Hypertension in Children

Norton Children’s Heart Institute pediatric cardiologists may conduct a number of different tests to diagnose pulmonary hypertension in babies and older children. 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 procedure studies the heart’s structure and function, and provides direct pressure measurements of the heart and nearby blood vessels, 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 provide direction on what treatment is best for the child.
  • Stress test: A six-minute walking test can show how tired your child gets during exercise and breathing capability. 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 in babies and older children can be cured, others will require the specialists at Norton Children’s Heart Institute to provide treatments to help ease symptoms and slow 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 lifelong 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 five Norton Children’s Hospital Outpatient Centers located in Bowling Green, Frankfort, Owensboro, Paducah and Shelbyville, Kentucky.

Why Choose Norton Children’s Heart Institute

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

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