Clinical presentation

Key Symptoms

Pulmonary hypertension is a chimera. Symptoms and signs are often vague and nonspecific, particularly in the early stage of disease. Symptoms at rest are reported only in very advanced cases. The mean time from onset of the first symptom to diagnosis is about 2 years with a maximum of up to 20 years.

The classical triad consists of

  • increasing dyspnea on exertion
  • without gross abnormalities on chest X-ray
  • without gross abnormalities in lung function testing

Initially patients may present with

  • fatigue
  • depression
  • or weight gain only

One third complain about

  • chest pain
  • palpitations
  • syncope
  • peripheral edema

Less common symptoms include

  • cough
  • hemoptysis
  • hoarseness (due to compression of the left recurrent laryngeal nerve by a dilated pulmonary artery = Ortner’s syndrome)


The physical signs of PAH include:

  • left parasternal lift
  • an accentuated pulmonary component of second heart sound
  • a pansystolic murmur of tricuspid regurgitation
  • a diastolic murmur of pulmonary insufficiency
  • an RV third sound

Jugular vein distension, hepatomegaly, peripheral oedema, ascites and cool extremities characterise patients in more advanced state. Lung sounds are usually normal.

The examination may also provide clues as to the cause of PH

  • telangiectasia, digital ulceration and sclerodactyly are seen in scleroderma
  • inspiratory crackles may point interstitial lung disease
  • the stigmata of liver disease such as spider naevi, testicular atrophy and palmar erythema should be considered
  • if digital clubbing is encountered in "IPAH", an alternative diagnosis such as CHD or PVOD should be sought.