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Signs of pulmonary arterial hypertension

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"pulmonary arterial hypertension"[MeSH Terms] AND "diagnosis"

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pulmonary hypertension chest radiograph enlarged pulmonary arteries right ventricular hypertrophy

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Signs of pulmonary arterial hypertension (PAH) fall into three groups. Early disease is often clinically silent, so signs usually appear late.

Symptoms that usually come first

  • Exertional dyspnea and reduced exercise capacity are the earliest and most common symptoms. Dyspnea is present in over 50% of patients (Tintinalli's Emergency Medicine).
  • Fatigue, light-headedness, chest pain (angina) and palpitations also occur.
  • Syncope is often exertional. It is an ominous sign because it means the right ventricle cannot raise cardiac output with activity (Goldman-Cecil Medicine).
  • The average delay from symptom onset to diagnosis is about 2 years (Tintinalli's Emergency Medicine).

Physical signs

Raised pulmonary pressure
  • Loud or palpable P2. A loud P2 is a sign of PAH, and in severe cases P2 is palpable (Fuster and Hurst's The Heart, "Abnormalities of S2 Components").
  • Tachypnea.
Right ventricular overload
  • Palpable right ventricular (parasternal) heave (Harrison's, Goldman-Cecil Medicine).
  • Pulmonic regurgitation murmur (Graham Steell) and tricuspid regurgitation murmur. The tricuspid murmur is holosystolic.
Right heart failure (late)
  • Elevated jugular venous pressure.
  • Hepatomegaly, ascites and dependent edema.
  • Murray & Nadel's lists the pulmonary vascular picture as jugular venous distension, a prominent pulmonic S2, a tricuspid regurgitation murmur, ascites and lower-limb edema.
Signs pointing to the underlying cause
  • Cyanosis and clubbing suggest congenital heart disease with shunt or lung disease.
  • Telangiectasia and Raynaud phenomenon suggest systemic sclerosis. About 15% of systemic sclerosis patients develop PAH (Goldman-Cecil Medicine).

Supporting test findings

  • ECG: right axis deviation and right ventricular hypertrophy or strain.
  • Chest X-ray: enlarged central pulmonary arteries with peripheral pruning, and right heart enlargement. I did not find a matching image in the library.
  • Echocardiography: tricuspid regurgitant jet velocity above 3 m/s, or estimated pulmonary artery systolic pressure above 40 mmHg at rest, suggests PAH (Harrison's, 22nd ed.). Echocardiography can over- or underestimate pressure.
  • Reduced DLCO and a raised NT-proBNP also support the diagnosis.
  • Right heart catheterization is required to confirm it. The definition quoted in Goldman-Cecil is mean pulmonary artery pressure of 25 mmHg or more with wedge pressure of 15 mmHg or less. Newer guidelines lower the mean pressure threshold to above 20 mmHg, with pulmonary vascular resistance above 2 Wood units. That update comes from my own knowledge, not the library.

Poor prognostic signs

Syncope, right heart failure signs and WHO functional class III-IV are all poor prognostic signs.

Recent literature

A quick PubMed check of the last 2 years found only systematic reviews on diagnosis. They cover PH associated with lung disease (PMID 40222881) and machine learning for diagnosis (PMID 39257851). Neither contradicts the clinical signs above.
If you want, I can go through the diagnostic algorithm or the WHO functional classes next.
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