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Pediatric Pulmonary Arterial Hypertension

Progressive vascular disease characterized by elevated mean pulmonary artery pressure causing right ventricular failure in children.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Pediatric Pulmonary Arterial Hypertension?

Pediatric pulmonary arterial hypertension (PAH) is defined hemodynamically by mean pulmonary artery pressure greater than 20 mmHg with elevated pulmonary vascular resistance index measured at right heart catheterization beyond three months of age.

Etiologies include idiopathic and heritable PAH (BMPR2, ALK1 mutations), congenital heart disease with systemic-to-pulmonary shunts, persistent pulmonary hypertension of the newborn, lung developmental disorders such as bronchopulmonary dysplasia and pulmonary vein stenosis, and connective tissue or hematologic diseases.

Children present with poor feeding, exertional dyspnea, syncope, chest pain, growth retardation and signs of right ventricular failure; older children may report exercise intolerance and palpitations.

Diagnostic evaluation combines echocardiography (estimated systolic pulmonary pressure, right ventricular size and function), six-minute walk testing in older children, cardiac magnetic resonance imaging, vasoreactivity testing during catheterization and genetic testing in selected cases.

Treatment includes general supportive measures, diuretics, anticoagulation in selected patients, calcium channel blockers in vasoreactive responders, endothelin receptor antagonists, phosphodiesterase-5 inhibitors and prostacyclin pathway agents; atrial septostomy and lung transplantation are considered in advanced disease.

Symptoms

Poor feeding and failure to thrive in infants
Exertional dyspnea and exercise intolerance
Syncope or near-syncope with exertion
Chest pain and palpitations
Cyanosis with effort
Hepatomegaly and peripheral edema (right heart failure)
Growth retardation
Loud P2 component of second heart sound on examination

Risk Factors

Family history of pulmonary hypertension (BMPR2, ALK1 mutations)
Congenital heart disease with left-to-right shunt
Bronchopulmonary dysplasia or chronic lung disease of prematurity
Persistent pulmonary hypertension of the newborn
Connective tissue disease (juvenile scleroderma)
Sickle cell disease
Down syndrome with associated heart disease
Portal hypertension

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent shortness of breath in a child
  • Exercise-related fainting or chest pain
  • Cyanosis with feeding or exertion
  • Growth failure with cardiopulmonary symptoms
  • Known congenital heart disease with worsening symptoms

Treatment Methods

01
Comprehensive evaluation at a pediatric pulmonary hypertension center
02
Diuretics for fluid overload and digoxin in selected cases
03
Calcium channel blockers in acute vasoreactivity responders
04
Endothelin receptor antagonists (bosentan, ambrisentan, macitentan)
05
Phosphodiesterase-5 inhibitors (sildenafil, tadalafil)
06
Prostacyclin pathway agents (epoprostenol, treprostinil, selexipag)
07
Anticoagulation in selected cases of idiopathic PAH
08
Atrial septostomy as palliative or bridge to transplant
09
Bilateral lung or heart-lung transplantation in advanced disease

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.