Review

Evaluating suspected pulmonary hypertension: A structured approach

Akshay Bhatnagar, MD, Jonathan Wiesen, MD, Raed Dweik, MD and Neal F. Chaisson, MD
Cleveland Clinic Journal of Medicine June 2018, 85 (6) 468-480; DOI: https://doi.org/10.3949/ccjm.85a.17065

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  • TABLE 1

    Updated World Health Organization classification of pulmonary hypertension

    Group 1:
    Pulmonary arterial hypertension
    Idiopathic
    Heritable
     BMPR2 mutation
     Other mutations
    Drug- and toxin-induced
    Associated with:
     Connective tissue disease
     Human immunodeficiency virus (HIV) infection
     Portal hypertension
     Congenital heart disease
     Schistosomiasis
    Pulmonary veno-occlusive disease or pulmonary capillary hemangiomatosis
     Idiopathic
     Heritable
      EIF2AK4 mutation
      Other mutations
     Drug-, toxin-, and radiation-induced
     Associated with:
      Connective tissue disease
      HIV infection
    Persistent pulmonary hypertension of the newborn
    Group 2:
    Pulmonary hypertension due to left heart disease
    Left ventricular systolic dysfunction
    Left ventricular diastolic dysfunction
    Valvular disease
    Congenital or acquired left heart inflow or outflow tract obstruction and congenital cardiomyopathies
    Congenital or acquired pulmonary vein stenosis    		Group 3:
    Pulmonary hypertension due to lung diseases, hypoxia, or both
    Chronic obstructive pulmonary disease
    Interstitial lung disease
    Other pulmonary diseases with mixed restrictive and obstructive pattern
    Sleep-disordered breathing
    Alveolar hypoventilation disorders
    Chronic exposure to high altitude
    Developmental lung diseases
    Group 4:
    Chronic thromboembolic pulmonary hypertension and other pulmonary artery obstructions
    Chronic thromboembolic pulmonary hypertension
    Other pulmonary artery obstructions
     Angiosarcoma
     Other intravascular tumors
     Arteritis
     Congenital pulmonary artery stenosis
     Parasites (hydatidosis)
    Group 5:
    Pulmonary hypertension with unclear or multifactorial mechanisms
    Hematologic disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy
    Systemic disorders, sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis
    Metabolic disorders: glycogen storage disease,
    Gaucher disease, thyroid disorders
    Others: pulmonary tumoral thrombotic microangiopathy, fibrosing mediastinitis, chronic renal failure (with or without dialysis), segmental pulmonary hypertension

    • Reproduced with permission of the European Society of Cardiology and the European Respiratory Society. European Respiratory Journal Oct J 2015; 46(4):903–975. doi:10.1183/13993003.01032-2015

  • TABLE 2

    Echocardiographic features supporting pulmonary hypertension

    Right atrial dilation (area > 18 cm2)
    Elevated right atrial pressure based on inferior vena cava (IVC) assessment (IVC > 21 mm and collapses < 50% with inspiratory sniff)
    Ratio of right ventricle to left ventricle basal diameter > 1
    Interventricular septal flattening or bowing towards the left ventricle
    A “mid-systolic notch” across the right ventricular outflow tract
    Reduced right ventricular outflow Doppler acceleration time < 105 msec
    Increased early diastolic pulmonary regurgitation velocity > 2.2 m/sec
    Pulmonary artery diameter > 25 mm

    • Based on information in Galiè et al, reference 1.

  • TABLE 3

    Risk assessment in pulmonary arterial hypertension

    Determinants of prognosisEstimated 1-year mortality
    Low-risk (< 5%)Intermediate risk (5%–10%)High risk (> 10%)
    Clinical signs of right heart failureAbsentAbsentPresent
    Progression of symptomsNoSlowRapid
    SyncopeNoOccasionalRepeated
    WHO functional classI, IIIIIIV
    6-minute walk distance> 440 m165–440 m< 165 m
    Natriuretic peptide levelsBNP < 50 ng/L
    NT-proBNP < 300 ng/mL    		BNP 50–300 ng/L
    NT-proBNP 300–1,400 ng/L    		BNP > 300 ng/L
    NT-proBNP > 1,400 ng/L
    Imaging
    (echocardiography, CMRI)    		RA area < 18 cm2
    No pericardial effusion    		RA area 18–26 cm2
    No or minimal pericardial effusion    		RA area > 26 cm2
    Pericardial effusion
    HemodynamicsRA pressure < 8 mm Hg
    CI ≥ 2.5 L/min/m2
    SvO2 > 65%    		RA pressure 8–14 mm Hg
    CI 2.0–2.4 L/min/m2
    Svo2 60%–65%    		RA pressure > 14 mm Hg
    CI < 2.0 L/min/m2
    Svo2 < 60%

    • CI = cardiac index; CMRI = cardiac magnetic resonance imaging; NT-proBNP = N-terminal pro-B-type natriuretic peptide; RA = right atrium; Svo2 = saturation of venous oxygen; WHO = World Health Organization. Reproduced with permission of the European Society of Cardiology and the European Respiratory Society. European Respiratory Journal Oct 2015; 46(4):903–975. doi:10.1183/13993003.01032-2015

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