Pulmonary Hypertension

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Pulmonary hypertension is a rare cause of high blood pressure (hypertension) in the pulmonary artery (pulmonary artery). When the blood vessels of the lungs becomes damaged, such as in pulmonary arterial hypertension, the thickening and stiffening of the pulmonary artery walls occur which causes deviations in the vessel wall. The consequence of this is that the pressure in the pulmonary artery rises and also in the RV. PH is a pressure load on the right heart. Click here to see an animation about PH.

WHO classification

Pulmonary hypertension is classified by the World Health Organization (WHO) as the place where the obstruction lies.

Classification Subcategories
Pulmonary Arterial Hypertension(PAH)
  • Idiopathic PAH(unknown cause)
  • Familial PAH(genetic cause)
  • PAH by underlying causes such as, connective tissue diseases, congenital heart disease, portal hypertension, HIV infection, drugs, drugs, toxins, and other factors.
  • PAH with significant venous and/or capillary involvement
  • Persistent pulmonary hypertension of newborn
Pulmonary hypertension with left heart disease
  • In disorders of left atrium and ventricle of the heart
  • With Valvular
Pulmonary hypertension in pulmonary disease and/or hypoxemia
  • Chronic obstructive pulmonary disease(COPD)
  • Interstitial lung disease
  • Sleep disorders, alveolar hypoventilation; chronic exposure to high altitude
  • Congenital malformations/developmental
Pulmonary hypertension due to chronic thrombotic and/or embolic processes
  • Thromboembolic obstruction of proximal pulmonary arteries
  • Thromboembolic obstruction of distal pulmonary arteries
  • Embolism of the lung (eg: tumor, parasites, foreign body, bone marrow)
Pulmonary hypertension as a result of other diseases and disorders
  • Sarcoidosis
  • Histiocytosis X
  • Lymphangioleiomyomatosis
  • Sickle Cells
  • Compression of the pulmonary vessels from outside(eg: by lymphadenopathy, tumor, fibrosing mediastinitis)
  • Storage disorders

 

Evidence of pulmonary hypertension

Unlikely Possible Likely
SPAP <36 mmHg 36-50 mmHg >50 mmHg
TR Vmax <2.8 m/s 2.9-3.4 m/s >3.4 m/s
AccT >120 ms 120-60 ms <60 ms
RV MPI (TCO-ET/ET) <0.36
RV MPI (TDI) <0:50

Calculations for estimating pressure in the pulmonary artery

 

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 systolic PA pressure + 0.6 x diastolic PA pressure
90 - (0.62 x ACCT) if Acct < 120ms
4 x(PR Vmax)2 + estimated RA pressure
TR PGmean + estimated RA pressure

Examples of severe pulmonary hypertension[1]

PVAccT02.jpg Video
Short AccT with "systolic notch" Flattened septum
SPAP01.jpg PAH01.jpg
Increased Systolic PA pressure Increased MPI, extension of isovolumetric times

References

  1. Howard LS, Grapsa J, Dawson D, Bellamy M, Chambers JB, Masani ND, Nihoyannopoulos P, and Simon R Gibbs J. Echocardiographic assessment of pulmonary hypertension: standard operating procedure. Eur Respir Rev. 2012 Sep 1;21(125):239-48. DOI:10.1183/09059180.00003912 | PubMed ID:22941889 | HubMed [1]