Paediatric Pulmonary Hypertension (PH)

How Does Paediatric Pulmonary Hypertension Affect Children?

PH affects the heart, lungs, and overall health in the following ways:

• Lung Function: The narrowing of lung arteries makes it harder for blood to flow, reducing oxygen exchange. This can cause shortness of breath, fatigue, and difficulty with physical activities.
• Heart Strain: Since the heart must pump harder to push blood through the lungs, the right side of the heart can become thicker and weaker over time, leading to heart failure.
• Low Oxygen Levels: Reduced oxygen flow to the organs can cause cyanosis (a bluish tint to the skin, lips, and nails) and affect a child’s growth and development.
• Physical Symptoms: Children with PH may experience dizziness, fainting, rapid heartbeat, and chest pain, all of which limit their ability to participate in normal activities like play or exercise.

What Causes Paediatric Pulmonary Hypertension?

The causes of PH in children can vary. Some of the most common causes include:

• Congenital Heart Defects: Conditions like atrial septal defects (ASD), ventricular septal defects (VSD), and patent ductus arteriosus (PDA) increase blood flow to the lungs, causing higher pressure in the pulmonary arteries.
• Chronic Lung Diseases: Premature infants with lung issues, like bronchopulmonary dysplasia (BPD), often develop PH.
• Genetic Conditions: Certain genetic syndromes, like Down syndrome, increase the risk of developing PH.
• Blood Clots: Clots that block the lungs' arteries can raise pressure in these blood vessels.
• Hypoxic Conditions: If the lungs receive too little oxygen (like at high altitudes or in chronic sleep apnea), it can trigger PH.
• Connective Tissue Disorders: Autoimmune diseases like lupus or systemic sclerosis can cause arterial inflammation, leading to PH.

What Are the Symptoms of Paediatric Pulmonary Hypertension?

Symptoms of PH can develop slowly and may be hard to spot at first. Here’s what to watch for:

• Shortness of Breath: This may happen during physical activity or even at rest in severe cases.
• Tiredness and Fatigue: Children may seem more tired than usual and avoid physical play.
• Fast or Irregular Heartbeat (Palpitations): The heart may feel like it’s racing or pounding.
• Chest Pain: A pressure-like pain may be felt, especially during physical activity.
• Dizziness or Fainting (Syncope): Reduced oxygen flow to the brain can cause dizziness or fainting spells.
• Bluish Skin, Lips, or Nails (Cyanosis): Low oxygen levels cause a blue tint.
• Swelling (Edema): Swelling may be seen in the legs, ankles, or abdomen due to fluid buildup.
• Poor Growth and Delayed Development: Children with PH may grow slower or have trouble gaining weight.

If you notice any of these symptoms in your child, speak to your doctor immediately.

How is Paediatric Pulmonary Hypertension Diagnosed?

Doctors use a variety of tests to diagnose PH. Here’s what to expect:

• Physical Exam: The doctor will check for signs of cyanosis, swelling, and heart murmurs.
• Echocardiogram: This heart ultrasound shows how well the heart is pumping and can measure pressure in the pulmonary arteries.
• Electrocardiogram (ECG): This test records the heart’s electrical activity and looks for signs of heart strain.
• Pulse Oximetry: A small device on the child’s finger measures oxygen levels in the blood.
• Chest X-ray: An X-ray can show if the heart or lungs are enlarged.
• Cardiac Catheterization: A small tube is inserted into the heart to measure the exact pressure in the pulmonary arteries. This is considered the most accurate way to diagnose PH.
• Blood Tests: Blood tests check for infections, blood clotting issues, and underlying autoimmune diseases.
• MRI or CT Scan: Advanced imaging provides a clearer view of the heart and lung arteries.

Types of Paediatric Pulmonary Hypertension

PH is divided into five groups based on the cause:

• Pulmonary Arterial Hypertension (PAH): Caused by changes in the lungs' arteries, leading to narrowing and increased pressure. This type can be idiopathic (no known cause) or related to genetic conditions.
• Pulmonary Hypertension from Left Heart Disease: Pressure in the lungs rises when blood can't flow properly from the lungs to the left side of the heart due to valve problems or heart failure.
• Pulmonary Hypertension (PH) is caused by Lung Disease or Low Oxygen (Hypoxia). Conditions like bronchopulmonary dysplasia (BPD) or living at high altitudes cause low oxygen levels, which trigger PH.
• Chronic Blood Clot-Related Pulmonary Hypertension: Blood clots block the arteries in the lungs, increasing blood pressure in these vessels.
• Pulmonary Hypertension Due to Other Causes: This includes PH caused by blood diseases, connective tissue disorders, or other unknown conditions.

How is Paediatric Pulmonary Hypertension Treated?

The goal of treatment is to lower blood pressure in the lungs, reduce heart strain, and improve your child’s quality of life. Treatment options include:

• Medications:
• Vasodilators: These open (dilate) the lung’s arteries to reduce pressure.
• Endothelin Receptor Antagonists (ERAs): These block proteins that tighten blood vessels.
• Phosphodiesterase Type 5 Inhibitors (PDE5 inhibitors): These relax lung arteries to improve blood flow.
• Prostacyclin Analogues: These widen the arteries and prevent blood clotting.
• Anticoagulants: Blood thinners help prevent clots.
• Diuretics: These help remove excess fluid from the body, reducing heart strain.
• Oxygen Therapy: Oxygen may be given to ensure the child’s body gets enough oxygen to reduce strain on the heart.
• Lifestyle Changes:
• Encourage your child to rest and avoid intense physical activities that strain the heart.
• Provide a healthy diet and ensure proper hydration.
• Follow all medication schedules and doctor’s advice.
• Surgical Interventions:
• Atrial Septostomy: This creates a small hole between the heart’s upper chambers to reduce pressure on the right side of the heart.
• Heart-Lung Transplant: In rare, severe cases, a heart-lung transplant may be considered.



Living with Paediatric Pulmonary Hypertension

With proper treatment and regular care, children with PH can live full, active lives. Early diagnosis and ongoing management are key. If you have any concerns about your child’s heart or lung health, contact Dr. David Youssef, a specialist in Paediatric Pulmonary Hypertension, for expert advice and care.