Tables
- Table 1– Haemodynamic definitions of pulmonary hypertension (PH)
Definition Characteristics Clinical group(s)# PH P̄pa ≥25 mmHg All Pre-capillary PH P̄pa ≥25 mmHg 1. PAH Ppcw ≤15 mmHg 3. PH due to lung diseases CO normal or reduced¶ 4. CTEPH 5. PH with unclear and/or multifactorial mechanisms Post-capillary PH P̄pa ≥25 mmHg 2. PH due to left heart disease Ppcw >15 mmHg CO normal or reduced¶ Passive TPG ≤12 mmHg Reactive (out of proportion) TPG >12 mmHg All values measured at rest. P̄pa: mean pulmonary arterial pressure; Ppcw: pulmonary capillary wedge pressure; CO: cardiac output; TPG: transpulmonary pressure gradient (P̄pa – mean Ppcw); PAH: pulmonary arterial hypertension; CTEPH: chronic thromboembolic PH. #: according to table 3; ¶: high CO can be present in cases of hyperkinetic conditions, such as systemic-to-pulmonary shunts (only in the pulmonary circulation), anaemia and hyperthyroidism, etc.
- Table 2– Arbitrary criteria for estimating the likelihood of pulmonary hypertension (PH) based on tricuspid regurgitation peak velocity, correspondent Doppler-calculated pulmonary arterial systolic pressure at rest (assuming a normal right atrial pressure of 5 mmHg) and additional echocardiographic variables suggestive of PH
PH present? Peak tricuspid regurgitant velocity m·s−1 Pulmonary artery systolic pressure mmHg Additional echocardiographic signs of PH Unlikely ≤2.8 ≤35 No Possible ≤2.8 ≤35 Yes 2.9–3.4 36–50 No/yes Likely >3.4 >50 No/yes - Table 3– Updated clinical classification of pulmonary hypertension (PH)
1. PAH 1.1. Idiopathic PAH 1.2.Heritable 1.2.1 BMPR2 gene mutation 1.2.2 ALK1, endoglin (with or without hereditary haemorrhagic telangiectasia) gene mutation 1.2.3 Unknown 1.3. Drug- and toxin-induced 1.4. APAH 1.4.1. Connective tissue diseases 1.4.2. HIV infection 1.4.3. Portal hypertension 1.4.4. Congenital heart disease 1.4.5. Schistosomiasis 1.4.6. Chronic haemolytic anaemia 1.5. Persistent PH of the newborn 1′. Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis 2. PH due to left heart disease 2.1. Systolic dysfunction 2.2. Diastolic dysfunction 2.3. Valvular disease 3. PH due to lung diseases and/or hypoxia 3.1. Chronic obstructive pulmonary disease 3.2. Interstitial lung disease 3.3. Other pulmonary diseases with mixed restrictive and obstructive pattern 3.4. Sleep-disordered breathing 3.5. Alveolar hypoventilation disorders 3.6. Chronic exposure to high altitude 3.7. Developmental abnormalities 4. CTEPH 5. PH with unclear and/or multifactorial mechanisms 5.1. Haematological disorders: myeloproliferative disorders and splenectomy 5.2. Systemic disorders, sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis and vasculitis 5.3. Metabolic disorders: glycogen storage disease, Gaucher disease and thyroid disorders 5.4. Others: tumoural obstruction, fibrosing mediastinitis and chronic renal failure on dialysis PAH: pulmonary arterial hypertension; BMPR2: bone morphogenetic protein receptor, type 2; ALK1: activin receptor-like kinase 1; APAH: associated PAH; CTEPH: chronic thromboembolic PH.
- Table 4– Clinical probability of pulmonary arterial hypertension (PAH) diagnosis and suggested diagnostic work-up according to the likelihood of the presence of pulmonary hypertension (PH) by Doppler echocardiography (table 2), occurrence of symptoms and risk factors, and associated conditions (table 3)
PH likelihood by echocardiography Symptoms Risk factors/AC PAH probability Work-up Unlikely No Yes/no Low No Yes Yes Low Echocardiographic follow-up Yes No Low Other causes Possible No No Intermediate Echocardiographic follow-up Yes Yes Intermediate Right-heart catheterisation Yes No Intermediate Echocardiographic follow-up# Likely Yes Yes/no High Right-heart catheterisation No Yes/no High Right-heart catheterisation AC: associated conditions. #: consider also other causes and, if symptoms at least moderate, also right heart catheterisation.
Vol 36 Issue 5
Table of Contents
Pulmonary hypertension and pulmonary arterial hypertension: a clarification is needed
Jump To
- Article
- PH IS A HAEMODYNAMIC AND PATHOPHYSIOLOGICAL CONDITION
- DOPPLER ECHOCARDIOGRAPHY CAN PROVIDE AN ESTIMATE OF THE LIKELIHOOD OF PH
- PH CAN BE FOUND IN AT LEAST 37 SYNDROMES CLASSIFIED INTO SIX CLINICAL GROUPS
- PAH IS A CLINICAL GROUP OF RARE CONDITIONS
- THE FINAL CLINICAL DIAGNOSIS IN THE INDIVIDUAL PATIENT WITH PH REQUIRES AN APPROPRIATE DIAGNOSTIC ALGORITHM
- CONCLUSIONS
- Footnotes
- REFERENCES
- Figures & Data
- Info & Metrics