Pulmonary Arterial Hypertension Initial Assessment

Last updated: 17 March 2026
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Clinical Presentation

Typical Symptoms

  • Cardinal symptom is progressive dyspnea on minor exertion
  • Dyspnea when bending forward (bendopnea)
  • Exercise-induced abdominal distention and nausea
  • Fatigue and rapid exhaustion
  • Hemoptysis
  • Palpitations
  • Syncope during or shortly after physical exertion
  • Weight gain due to fluid retention
  • Late and less common symptoms due to PA dilation include:
    • Exertional chest pain due to dynamic compression of the left main coronary artery
    • Hoarseness or dysphonia due to compression of the left laryngeal recurrent nerve
    • Shortness of breath, wheezing, cough, lower respiratory tract infection and atelectasis due to compression of the bronchi

Physical Examination

Signs of Increased Pulmonary Artery Pressure (PAP)

  • Accentuated pulmonary component of the S2 sound audible at the apex
  • Central, peripheral or mixed cyanosis
  • Diastolic murmur of pulmonary insufficiency and right ventricular (RV) S3
  • Early systolic ejection click from sudden interruption of pulmonary valve opening
  • Mid-systolic ejection murmur
  • Palpable left parasternal lift
  • Pansystolic murmur of tricuspid regurgitation
  • Prominent jugular “a” wave which suggests increased RV filling pressure
  • RV S4 gallop
  • Lung sounds are usually normal


Signs of Right Ventricular Forward Failure

  • Cool extremities
  • Dizziness
  • Pallor
  • Peripheral cyanosis (blue lips and tips)
  • Prolonged capillary refill


Signs of Right Ventricular Backward Flow

  • Abdominal distention
  • Ascites
  • Ascites
  • Jugular venous distention
  • Peripheral edema


Signs Pointing towards Underlying Cause of Pulmonary Hypertension

  • Auscultatory findings of crackles, wheezing or murmurs: Lung or heart disease
  • Digital clubbing: Bronchiectasis, cyanotic CHD, fibrotic lung disease, liver disease or pulmonary veno-occlusive disease (PVOD)
  • Differential clubbing or cyanosis: Eisenmenger’s syndrome or patent ductus arteriosus (PDA)
  • Sclerodactyly, Raynaud’s phenomenon, digital ulceration, gastroesophageal reflux disease (GERD): Systemic sclerosis
  • Sequelae of deep venous thrombosis (DVT), venous insufficiency: Chronic thromboembolic pulmonary hypertension (CTEPH)
  • Telangiectasia: Hereditary hemorrhagic telangiectasia (HHT) or systemic sclerosis

Diagnosis or Diagnostic Criteria

A series of non-invasive diagnostic tests may be done initially to support the diagnosis of pulmonary hypertension.  Complete right heart catheterization (RHC) should be performed to establish the diagnosis of PAH.

Screening

The goals of screening of at-risk individuals include early diagnosis of HPAH, early initiation of treatment and improve prognosis.  

Screening of asymptomatic, high-risk groups such as patients diagnosed with systemic sclerosis, BMPR2 mutation carriers, first-degree relatives of patients with HPAH, and patients who will undergo liver transplant is recommended. Prevalence of PAH in patients with systemic sclerosis ranges from 9-15% with annual incidence of PAH development between 0.7-1.5%. Patients who are BMPR2 mutation carriers have approximately 20% lifetime risk of developing PAH with penetrance higher in females compared to males. Approximately 1-2% of patients with liver disease and portal hypertension develop porto-pulmonary hypertension.  

Screening for HPAH in at-risk individuals should be done annually but intervals may vary between 6 months to 3 years depending on assessment results, gene, gender and family history. Screening should include medical history, physical examination, WHO-FC, ECG, PFT, BNP/NT-proBNP and echocardiography.  

Annual screening for PAH is recommended for patients with systemic sclerosis. The DETECT algorithm is recommended in adult patients with systemic sclerosis with disease duration >3 years, an FVC ≥40% and a DLCO <60% to identify asymptomatic patients with PAH. RHC is recommended in patients with systemic sclerosis where breathlessness remains unexplained after non-invasive assessment to exclude PAH. Assessment of risk of having PAH based in an evaluation of breathlessness in combination with echocardiogram or PFTs and BNP/NT-proBNP should be considered in patients with systemic sclerosis.  

Patients with liver disease and portal hypertension who are considered for liver transplantation or transjugular portosystemic shunting are recommended to have annual echocardiography even in the absence of symptoms to screen for PAH.  

Further evaluation to assess for CTEPH is recommended in patients with persistent or new onset dyspnea or exercise limitation after pulmonary embolism. The optimal timing for assessing symptoms to aid early detection of CTEPH may be 3 to 6 months after acute pulmonary embolism. Echocardiography is the preferred first-line diagnostic tool to detect CTEPH. Asymptomatic relatives testing positive for PAH-causing mutations should undergo annual screening with echocardiography.