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Evaluation, Diagnosis, and Classification of Pulmonary Hypertension Cover

Evaluation, Diagnosis, and Classification of Pulmonary Hypertension

Open Access
|Jul 2021

Figures & Tables

Table 1

Updated clinical classification of pulmonary hypertension. Reproduced with permission of ©ERS 2020; DOI: 10.1183/13993003.01913-2018.3 PAH: pulmonary arterial hypertension; HIV: human immunodeficiency virus; CCB: calcium channel blockers; PVOD: pulmonary veno-occlusive disease; PCH: pulmonary capillary hemangiomatosis; LVEF: left ventricular ejection fraction; PH: pulmonary hypertension

GROUP 1: PAHGROUP 2: PH DUE TO LEFT HEART DISEASE
  • 1.1 Idiopathic PAH

  • 1.2 Heritable PAH

  • 1.3 Drug- and toxin-induced PAH

  • 1.4 PAH associated with:

    • 1.4.1 Connective tissue disease

    • 1.4.2 HIV infection

    • 1.4.3 Portal hypertension

    • 1.4.4 Congenital heart disease

    • 1.4.5 Schistosomiasis

  • 1.5 PAH long-term responders to CCB

  • 1.6 PAH with overt features of PVOD/PCH

  • 1.7 Persistent PH of the newborn

  • 2.1 PH due to heart failure with preserved LVEF

  • 2.2 PH due to heart failure with reduced LVEF

  • 2.3 Valvular heart disease

  • 2.4 Congenital/acquired cardiovascular conditions leading to postcapillary PH

GROUP 3: PH DUE TO LUNG DISEASE AND/OR HYPOXIAGROUP 4: PH DUE TO PULMONARY ARTERY OBSTRUCTIONS
  • 3.1 Obstructive lung disease

  • 3.2 Restrictive lung disease

  • 3.3 Other lung disease with mixed restrictive/obstructive pattern

  • 3.4 Hypoxia without lung disease

  • 3.5 Developmental lung disorders

  • 4.1 Chronic thromboembolic PH

  • 4.2 Other pulmonary artery obstructions

GROUP 5: PH WITH UNCLEAR AND/OR MULTIFACTORIAL MECHANISMS
  • 5.1 Hematological disorders

  • 5.2 Systemic and metabolic disorders

  • 5.3 Others

  • 5.4 Complex congenital heart disease

Table 2

Updated classification of drugs and toxins associated with pulmonary hypertension. Reproduced with permission of the ©ERS 2020: DOI: 10.1183/13993003.01913-2018.3 HCV: hepatitis C virus

DEFINITEPOSSIBLE
Aminorex
Fenfluramine
Dexfenfluramine
Benfluorex
Methamphetamines
Dasatinib
Toxic rapeseed oil
Cocaine
Phenylpropanolamine
L-tryptophan
St John’s wort
Amphetamines
Interferon alpha and beta
Alkylating agents
Bosutinib
Direct-acting anti-HCV agents
Leflunomide
Idarubicin (Chinese herb Qing-Dai)
Figure 1

Algorithm for diagnosis of pulmonary hypertension and identifying its cause(s). Reproduced with permission of the © ERS 2020: DOI: 10.1183/13993003.01904-2018.7 PH: pulmonary hypertension; V/Q: ventilation/perfusion; CTEPH: chronic thromboembolic PH

Table 3

Echocardiographic probability of pulmonary hypertension (PH) in symptomatic patients with a suspicion for PH. Reproduced with permission of the ©ERS 2020: DOI: 10.1183/13993003.01904-2018.7

PEAK TRICUSPID REGURGITANT VELOCITY M/SPRESENCE OF OTHER ECHOCARDIOGRAPHIC “PH SIGNS”ECHOCARDIOGRAPHIC PROBABILITY OF PH
≤ 2.8 or not measurableNoLow
≤ 2.8 or not measurableYesIntermediate
2.9–3.4No
2.9–3.4YesHigh
> 3.4Not required
Table 4

Echocardiographic signs that are used with tricuspid regurgitant velocity measurements in Table 3 to assess the probability of pulmonary hypertension (PH). Reproduced with permission of the ©ERS 2020: DOI: 10.1183/13993003.01904-2018.7 Echocardiographic signs from at least two different categories (A/B/C) should be present to alter the level of echocardiographic probability of PH. RA: right atrium; RV: right ventricle; LV: left ventricle; PA: pulmonary artery.

A: THE VENTRICLESB: PULMONARY ARTERYC: INFERIOR VENA CAVA AND RA
RV/LV basal diameter ratio > 1
 
Flattening of the interventricular septum (LV eccentric index > 1.1 in systole and/or diastole)
RV outflow Doppler acceleration time < 105 ms and/or mid-systolic notching
 
Early diastolic pulmonary regurgitation velocity > 2.2 m/s
 
PA diameter > 25 mm
Inferior vena cava diameter > 21 mm with decreased inspiratory collapse (< 50% with a sniff or < 20% with quiet inspiration)
 
RA area (end systole) > 18 cm2
DOI: https://doi.org/10.14797/OCDF4453 | Journal eISSN: 1947-6108
Language: English
Page range: 86 - 91
Accepted on: Jan 7, 2021
Published on: Jul 1, 2021
Published by: Houston Methodist DeBakey Heart & Vascular Center
In partnership with: Paradigm Publishing Services

© 2021 Sarah Beshay, Ashrith Guha, Sandeep Sahay, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.