Pulmonary Hypertension and Congenital Heart Disease
PH associated with congenital heart disease can result from various heart and blood vessel abnormalities that develop before a person is born.
Pediatric Pulmonary Hypertension
Pediatric pulmonary hypertension, like adult PH, is high blood pressure in the blood vessels in the lungs. Causes of pediatric PH are often different, as are the types of treatments that can be used.
Pulmonary hypertension is high blood pressure in the lungs. This high blood pressure occurs in the pulmonary artery. The pulmonary artery carries blood from the right side of the heart to the lungs. The blood vessels in the lungs are damaged and become narrow and stiff. Over time, it may become difficult for the right side of the heart to pump blood through the lungs.
In pediatric PH, similar to adult PH, the disease process is the same. In both cases, damage occurs in the lung’s blood vessels leading to right heart stress. However, children are growing. This growth must be considered when diagnosing and treating PH. Additionally, some causes of pediatric PH are unique to children.
Common causes of pediatric PH
Pediatric PH has various causes. When your child is diagnosed, their doctor may be able to identify the cause, or it may be unknown. Children are also more likely to have a genetic cause of their PH. Common causes of pediatric PH include but are not limited to:
- Genetic causes
- Congenital heart disease
- Eisenmenger syndrome: This is a subclass of PH due to congenital heart disease. It can be seen in unrepaired shunts (bypass of blood inside the heart due to holes or abnormal blood vessels) such as ventricular septal defects (VSD) or patent ductus arteriosus (PDA). This creates PH that can cause low oxygen blood to cross the shunt and make a patient have lower body oxygen levels.
- Lung disease
- Unknown reasons
- Persistent pulmonary hypertension of the newborn (link to PPHN)
Learn more about the causes of pediatric PH
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- Group 1: Pulmonary Arterial Hypertension
Group 1 pulmonary hypertension is also known as pulmonary arterial hypertension. PAH occurs when the arteries in the lungs narrow and stiffen, leading to increased pressure in the lungs.
- Persistent Pulmonary Hypertension of the Newborn
PPHN is a type of pulmonary hypertension specific to infants less than 3 months of age.
- Group 3: PH Due to Lung Disease or Hypoxia
PH due to lung disease or hypoxia can occur in children as well as adults.
- Heritable Pulmonary Arterial Hypertension
Pulmonary arterial hypertension caused by gene variants inherited from a parent is called “heritable” or familial PAH.
- Eisenmenger Syndrome
Eisenmenger syndrome is a form of pulmonary arterial hypertension caused by congenital heart disease, although it is often diagnosed in teens or adults.
What are the types of pediatric pulmonary hypertension
Treating pediatric PH
Your child’s PH team will help determine the best treatment options. Treatment options could include targeted medications, surgical options and supplemental oxgen.
These drugs are approved by the Food and Drug Administration (FDA) for the treatment of pediatric PH. Learn more about these treatments:
Bosentan (Tracleer) for Pediatric Patients Sildenafil (Revatio) for Pediatric PatientsThese surgical options may be recommended by your doctor:
- Reverse Potts shunt palliation: This is a surgical connection between a lung artery and the main body artery (the aorta) that allows high pressure in the lung arteries to be “popped” off into the aorta. This helps lower the pressure in the lungs to the level of the body’s blood pressure which can protect the right ventricle for strain and failure.
- Lung transplantation: This is the final therapy for patients with severe PH not responding to medical or other surgical treatments. This involves using donor healthy lungs to replace disease PH lungs. Only a few specialized centers in the United States offer this procedure.
- Atrial septostomy: This is creation of a connection between the two upper chambers of the heart (the right and left atria) to allow blood to bypass the right ventricle and lungs to be pumped out the body through the left ventricle. This is used when pressures of the right ventricle are too high to adequately pump all of the blood into the lungs. This will make a patient to have lower oxygen levels but allows enough oxygen for the rest of the body.
Transplant and Procedures
Diuretics, blood thinners and treatment of iron deficiency are conventional therapies for PH that must be carefully addressed if the patient has a congenital heart defect. Oxygen therapy may improve symptoms for those with PH.
Targeted therapiesTreating pediatric PH
Your child’s PH team will help determine the best treatment options. Treatment options could include targeted medications, surgical options and supplemental oxgen.
These drugs are approved by the Food and Drug Administration (FDA) for the treatment of pediatric PH. Learn more about these treatments:
These surgical options may be recommended by your doctor:
- Reverse Potts shunt palliation: This is a surgical connection between a lung artery and the main body artery (the aorta) that allows high pressure in the lung arteries to be “popped” off into the aorta. This helps lower the pressure in the lungs to the level of the body’s blood pressure which can protect the right ventricle for strain and failure.
- Lung transplantation: This is the final therapy for patients with severe PH not responding to medical or other surgical treatments. This involves using donor healthy lungs to replace disease PH lungs. Only a few specialized centers in the United States offer this procedure.
- Atrial septostomy: This is creation of a connection between the two upper chambers of the heart (the right and left atria) to allow blood to bypass the right ventricle and lungs to be pumped out the body through the left ventricle. This is used when pressures of the right ventricle are too high to adequately pump all of the blood into the lungs. This will make a patient to have lower oxygen levels but allows enough oxygen for the rest of the body.
Diuretics, blood thinners and treatment of iron deficiency are conventional therapies for PH that must be carefully addressed if the patient has a congenital heart defect. Oxygen therapy may improve symptoms for those with PH.
What are the Differences between Pediatric and Adult Pulmonary Hypertension
Assembling your pediatric PH care team
Your pediatric PH care team may include:
- Pediatric PH Specialist
- Pediatric cardiologist or pulmonologist
- Nurse coordinator
- Social worker
- Child life specialist
Pediatric PH patients should see their doctors every three to six months to monitor symptoms and treatment.
If your child is newly diagnosed and you need to assemble a care team that specializes in treating pulmonary hypertension, PHA offers a directory of PHA–accredited pediatric medical centers. These centers have demonstrated exceptional knowledge and expertise in treating children with pulmonary hypertension. You may also use our Find a Doctor directory to identify a pediatric clinician who treats pulmonary hypertension near you.
- Find a PH Pediatric Care Center
- Find a pediatric PH specialist
What Parents and Caregivers Should Know About Pediatric PH
In this episode, Paul Critser, MD, PhD; Pediatric Cardiologist at Heart Institute Cincinnati Children’s Hospital Medical Center, provides an overview of pediatric pulmonary hypertension and how it may differ from adults living with PH. Critser shares the unique challenges a child living with PH may face from diagnosis to long-term management.
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