SFGH morning report pearls 7/8/15: pulmonary hypertension

  • Pulmonary Hypertension pneumonic (to remember the WHO groups): AVRT other
    1. Arterial (primary PAH)
    2. Venous (back up from left heart disease)
    3. Respiratory and rheum (Lung disease)
    4. Thromboembolic
    5. Other
  • Pulmonary tumor thrombotic microangiopathy:
    • rare entity causing severe pulmonary hypertension with rapidly progressive dyspnea
    • widespread tumor emboli with fibrocellular intimal proliferation and thrombus formation in small pulmonary arteries and arterioles
    • most frequently caused by gastric cancer
  • PVOD (WHO group 1′) –
    • diffuse occlusion of the pulmonary veins
    • triad of severe PAH, radiographic evidence of pulmonary edema, normal pulmonary artery occlusion pressure
  • Echo may be misleading in assessment of patients with suspected pulmonary hypertension, especially with there is an inadequate TR jet. The TR in pulmonary hypertension is not from an abnormality of the TV, but secondary to dilation of the tricuspid annulus and right ventricle
  • Schistosomiasis associated pulmonary hypertension is the most prevalent cause of pulmonary hypertension worldwide; WHO group 2 is the most common cause in the US/Europe
Chinen K, Tokuda Y, Fujiwara M, Fujioka Y. Pulmonary tumor thrombotic microangiopathy in      patients with gastric carcinoma: An analysis of 6 autopsy cases and review of the literature. Pathol Res Pract. 2010;206:682–9.
Fisher MR, Forfia PR, Chamera E, et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 2009; 179:615.
Montani D, Price LC, Dorfmuller P, et al. Pulmonary veno-occlusive disease. Eur Respir J 2009; 33:189.
Simonneau G, Gatzoulis MA, Adatia I, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2013; 62:D34.

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