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Pulmonary Arterial Hypertension — Group 1 (PAH)

A rare progressive pulmonary vascular disease driven by remodeling of small pulmonary arteries.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

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

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What is Pulmonary Arterial Hypertension — Group 1 (PAH)?

Group 1 PAH is a precapillary pulmonary hypertension caused by intrinsic remodeling of small pulmonary arteries (intimal proliferation, medial hypertrophy, plexiform lesions). It is hemodynamically distinct from Groups 2–5, which are due to left heart disease, lung disease, chronic thromboembolism, or multifactorial mechanisms.

Subgroups include idiopathic PAH, heritable PAH (BMPR2, ALK1, ENG, SMAD9 mutations), drug- or toxin-induced PAH (amphetamines, methamphetamine, fenfluramine), connective tissue disease-associated PAH (systemic sclerosis, lupus, MCTD), HIV-associated, portopulmonary, congenital heart disease (Eisenmenger), and pulmonary veno-occlusive disease.

Diagnosis follows a stepwise algorithm: clinical suspicion, echocardiogram screening, right heart catheterization for confirmation, and comprehensive work-up to assign group. Risk stratification (REVEAL 2.0, ESC/ERS four-strata) guides therapy intensity.

Symptoms

Progressive exertional dyspnea (most common initial symptom)
Fatigue and exercise intolerance
Syncope or near-syncope on exertion
Chest pain, palpitations
Peripheral edema, ascites, hepatomegaly (right heart failure)
Loud P2, parasternal heave, tricuspid regurgitation murmur, jugular venous distension

Risk Factors

Female sex (2–4× higher prevalence), peak in 30–40s
Family history of PAH (BMPR2 mutation in 20–25% of heritable cases)
Connective tissue diseases (systemic sclerosis is highest risk)
Congenital heart disease (left-to-right shunts)
Portal hypertension and chronic liver disease
Anorexigens, methamphetamine, dasatinib exposure
HIV infection
Schistosomiasis (most common cause worldwide)

When to See a Doctor?

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

  • Unexplained progressive exertional dyspnea or fatigue
  • Exertional syncope or pre-syncope
  • Suspected systemic sclerosis or other CTD with new respiratory symptoms
  • Worsening symptoms in a previously diagnosed PAH patient
  • Family member with PAH and new symptoms in relatives

Treatment Methods

01
Risk-based combination therapy is now standard, including upfront combination for intermediate/high risk
02
Endothelin receptor antagonists (macitentan, ambrisentan, bosentan)
03
Phosphodiesterase-5 inhibitors (sildenafil, tadalafil) or sGC stimulator (riociguat — also CTEPH)
04
Prostacyclin pathway: oral selexipag, inhaled iloprost/treprostinil, IV/SC epoprostenol/treprostinil for high-risk patients
05
Sotatercept (activin signaling inhibitor) — newly approved adjunct for PAH
06
Anticoagulation in selected idiopathic PAH (controversial)
07
Diuretics for right heart failure, oxygen for hypoxemia, supervised exercise rehabilitation
08
Vaccination, contraception (pregnancy strongly contraindicated), psychological support
09
Atrial septostomy and lung/heart-lung transplantation for refractory disease

Which Department to Visit?

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

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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.