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

CategorySubcategories
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

ParameterNormalIntermediateAbnormal
RA Pressure (mmHg)<55 - 10>10
RV Syst Pressure (mmHg)<37 ≥37
TR Vmax (m/s)<2.8 2.8 - 3.4>3.4
AccT (ms)>110 110 - 105<105
RA volume index (ml/m²) MALE≤34>34
RA volume index (ml/m²) FEMALE≤27>27
RA FAC (%)32-60≤32
LV eccenctricty index1>1 * **
RV MPI (Pulsed)<0.280.28 - 0.32>0.32
RV MPI (TDI)<0.55
S' TV annulus (cm/s)>1211.5 - 12<11.5
IVRT (ms)<75≥75
TAPSE≥2016 - 20<16
Contour flow RVOTParabolicVariableTriangular
* >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

Calculation of pulmonary artery pressure

ParameterFormula
Systolic PA pressure4 x (TR Vmax)² + estimated RA pressure
Diastolic PA pressure4 x (PR Ved)² + estimated RA pressure
Mean PA pressure0.3 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

Eur Respir Rev 2012; 21: 125, 239-248

JASE 2015 Jan;28(1):1-39.e14

Pulm Circ. 2014 Jun;4(2):158-68