Pulmonary Hypertension

Diagnosis

Suspicion: Dyspnea on exertion, lower extremity edema/abdominal distension, angina, parasternal RV “heave”, tricuspid regurgitation, pulmonic insufficiency (1)

Echocardiography: Can estimate pulmonary arterial pressure, pulmonary arterial resistance, right atrial pressure, RV volume, and RV contractility – all of which can provide increasing evidence for pulmonary hypertension (2)

The above findings are based on measurement of tricuspid and pulmonic regurgitant jet velocities, TAPSE (tricuspid annular plane systolic excursion), RV size, RV relaxation time, RVOT acceleration time, and IVC size (3)

Definitive Diagnosis: Right heart catheterization (RHC) with mean pulmonary artery pressure findings exceeding 20 mmHg (4)

RHC also allows for additional testing (5)

  • Vasodilator challenge: Assesses pulmonary arterial pressure response to calcium channel blockers
  • Exercise challenge: Assesses for worsening of pulmonary pressures with physical exertion
  • Fluid challenge: Assesses for worsening of pulmonary pressures with administration of fluid volume
  • Pulmonary angiogram: Assesses for pulmonary blood flow obstruction

World Health Organization (WHO) Classification

Group 1

  • Idiopathic
  • Heritable
    • Drug/toxin associated
    • Connective tissue disease
    • HIV
    • Portal hypertension
    • Schistosomiasis
  • Evaluation: ANA, Rheumatoid Factor, Anti-SSA, Anti-SSB, HIV, Hepatitis panel/Hepatic function panel

Group 2

  • Cardiogenic (Left ventricular systolic or diastolic dysfunction, valvular abnormalities)
  • Evaluation: Echocardiography

Group 3

  • Intraparenchymal Disease
    • Chronic obstructive pulmonary disease
    • Interstitial lung disease
    • Obstructive Sleep Apnea
  • Evaluation: Pulmonary Function Testing, polysomnography

Group 4

  • Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
  • Evaluation: Pulmonary angiogram or V/Q (Ventilation/Perfusion) scan

Group 5

  • Other (6)
    • Hematologic disorders
    • Systemic disorders (sarcoidosis, pulmonary histiocytosis, Lymphangioleiomyomatosis)
    • Metabolic disorders (thyroid dysfunction, lysosomal storage diseases)
    • Others: Fibrosing mediastinitis, chronic renal failure

Complications of Pulmonary Hypertension

  • Right Heart Failure (“cor pulmonale”)
  • Arrhythmias
  • Hypoxemia
  • Pulmonary thromboembolism
  • Pulmonary artery dissection/diffuse alveolar hemorrhage
  • Pregnancy complications

Treatment

If calcium channel blockers lower pulmonary arterial pressure during right heart catheterization, these may be useful in treatment (7)

IV Epoprostenol is first-line therapy (8)

Second line in either monotherapy or combination therapy

  • Endothelin receptor antagonists (ambrisentan, bosentan, macitentan)
  • Phosphodiesterase-5 inhibitors (sildenafil, tadalafil)
  • Soluble guanylate cyclase stimulators (riociguat)
  • Prostanoids (iloprost, treprostinil)

Lung transplant

References

  1. Jonathan D. Rich and MD Stuart RichMD, Rich, J., Jonathan D. Rich From the Department of Medicine, Rich, S., Stuart Rich From the Department of Medicine, & Rich, C. (2014, November 11). Clinical diagnosis of pulmonary hypertension. Retrieved February 25, 2021, from https://www.ahajournals.org/doi/full/10.1161/CIRCULATIONAHA.114.006971
  2. DS;, C. (n.d.). State-of-the-art review: Echocardiography in pulmonary hypertension. Retrieved February 25, 2021, from https://pubmed.ncbi.nlm.nih.gov/31109891/
  3. Howard, L., Grapsa, J., Dawson, D., Bellamy, M., Chambers, J., Masani, N., . . . Gibbs, J. (2012, September 01). Echocardiographic assessment of PULMONARY hypertension: Standard operating procedure. Retrieved February 25, 2021, from https://err.ersjournals.com/content/21/125/239
  4. Hoeper, M., & Humbert, M. (2019, March 01). The new haemodynamic definition of pulmonary hypertension: Evidence prevails, finally! Retrieved February 25, 2021, from https://erj.ersjournals.com/content/53/3/1900038
  5. Vera Moulton Wall Centerfor Pulmonary Vascular Disease. (n.d.). RHC for PH. Retrieved February 25, 2021, from https://med.stanford.edu/wallcenter/patient_care/patient-resources/articles-external-and-videos/rhc-for-ph.html
  6. Prins, K., & Thenappan, T. (2016, August). World health Organization Group I Pulmonary Hypertension: Epidemiology and Pathophysiology. Retrieved February 26, 2021, from https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4959804/
  7. Medarov, B. I., & Judson, M. A. (2014, January 1). The role of calcium channel blockers for the treatment of pulmonary arterial hypertension. Retrieved February 25, 2021, from https://www.resmedjournal.com/article/S0954-6111(15)00007-4/fulltext
  8. Corris, P., & Degano, B. (2014, December 01). Severe pulmonary arterial hypertension: Treatment options and the bridge to transplantation. Retrieved February 26, 2021, from https://err.ersjournals.com/content/23/134/488

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