Pulmonary Hypertension

Pulmonary hypertension (PHT)is a type of high blood pressure that affects he pulmonary arteries. Pulmonary arterial hypertension causes thickening and stiffening of the pulmonary artery walls, which leads to permanent damage of vessel wall. The consequence of this, is that the pressure rise in the pulmonary artery, and also in the RV. PHT is a pressure load for the right heart.



WHO classification
Category Subcategories
Pulmonary Artery Hypertension (PAH) • Idiopathische PAH (onbekende oorzaak)
• Familiaire PAH (genetische oorzaak)
• PAH door onderliggende oorzaken als; bindweefselziekten, aangeboren hartziekten, portale hypertensie, HIV-infectie, geneesmiddelen, drugs, giftige stoffen en andere factoren.
•PAH met significante veneuze en/of capillaire betrokkenheid
•Persisterende pulmonale hypertensie van pasgeborenen
Pulmonary hypertension at left sided heartdiseases • At left atrium and/or left ventricle disorders
• At valvediseases
Pulmonary hypertension at lungsiseases and/or hypoxemia • Chronic obstructive pulmonary diseases (COPD)
• Interstitial lungdiseases
• Sleep disorders; alveolar hypoventilation; chronic exposion to great hights.
• Congenital disorders /developmental
Pulmonary hypertension as a result of chronic trombotic and /or embolic processes • Trombo-embolic obstruction of proximal lungarteries
• Trombo-embolic obstruction of distal lungarteries
• Pulmonary embolism (e.g. tumor, parasite, corpus alienum, bone marrow)
Pulmonary hypertension as a result of other  syndromes and disorders • Sarcoïdosis
• Histiocytosis X
• Lymphangioleiomyomatosis
• Sickle cells
• Outside compression on lungarteries (e.g. by lymphadenopathy, tumor, fibrosing mediastinitis)
• Storage disease


RV pressure cavity and functional measuremenst that may indicate PHT
Parameter normal intermediate Abnormal
Right atrial pressure (mmHg) <5 5-10 >10
TR Vmax (m/s) <2.6 ≥2.6
RVSP (mmHg) <37 ≥37
RVOT AccT (ms) >110 105-110 <105
RA volume index (ml/m²) MALE ≤34 >34
RA volume index (ml/m²) FEMALE ≤27 >27
RA FAC (%) 32-60 ≤32
LV eccenctricty index 1 >1  *   **
RV MPI (Tei index) <0.28 0.28-0.32 >32
S' TV annulus (cm/s) >12 11.5-12 <11.5
IVRT (ms) <75 ≥75
TAPSE (mm) ≥20 16-20 <16
* >1 at end-diastole indicates volume loading of the RV, 
** >1 at end-systole indicates pressure loading of the RV


Flow in RVOT with a short acceleration time and a systolic notch


RIMP with prolonged isovolumetric times


Increased RVSP


Flattened septum



Calculation of pulmonary artery pressure
Parameter Formula
Systolic PA pressure 4 x (TR Vmax)² + estimated RA pressure
Diastolic PA pressure 4 x (PR Ved)² + estimated RA pressure
Mean PA pressure 0.3 x systolische PA-druk + 0.6 x diastolic PA pressure
90 - (0.62 x AccT) if Accelaration Time < 120ms
4 x (PR Vmax)² + estimated RA pressure
TR PGmean + estimated RA pressure