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Pediatric Pulmonary Hypertension

Diagnosis and management of pulmonary hypertension in children

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

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 Hypertension?

Elevated mean pulmonary arterial pressure above 20 mmHg with PVR over 3 Wood Units.

Idiopathic, familial, associated with CHD, lung disease, or post-capillary causes.

Affects infants through adolescents with age-specific risk stratification.

Goal-directed therapy with escalation based on WHO functional class and risk score.

Symptoms

Exercise intolerance and fatigue
Syncope on exertion
Dyspnea and cyanosis
Right heart failure with hepatomegaly
Loud P2 and right ventricular heave
Chest pain and palpitations

Risk Factors

Congenital heart disease with shunt
Bronchopulmonary dysplasia and chronic lung disease
Family history of PAH and BMPR2 mutation
Connective tissue disease
Prematurity and neonatal hypoxia
HIV, portal hypertension, schistosomiasis

When to See a Doctor?

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

  • Seek urgent evaluation for unexplained dyspnea or syncope.
  • Annual follow-up with PH specialist for diagnosed patients.
  • Report worsening symptoms or syncope promptly.

Treatment Methods

01
Right heart catheterization for definitive diagnosis and vasoreactivity.
02
Calcium channel blockers for responders in vasoreactivity testing.
03
PDE5 inhibitors (sildenafil) as first-line oral therapy.
04
Endothelin receptor antagonists (bosentan, macitentan) as combination therapy.
05
Intravenous prostanoids (epoprostenol, treprostinil) for severe disease.
06
Atrial septostomy or Potts shunt for refractory disease; consider transplant.

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

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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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.