pulmonary hypertension

• related: Pulmonary Diseases, Cardiology and Hemodynamics
• tags: #literature #cardiology #pulmonology

---

Cause

• pulmonary hypertension clinical classifications
• genetic mutations in pulmonary hypertension

Symptoms

• When to suspect pulmonary hypertension

Labs and CT

• Suspected pulmonary hypertension initial workup
• pulmonary hypertension initial labs include BNP, CBC, TSH, LFT, ANA
• presence of RVH and P pulmonale can suggest pulmonary hypertension

Echocardiogram

• echo findings in pulmonary hypertension includes, high RVSP, TR, and RV dysfunction
• large PA size on CT can suggest pulmonary hypertension but does not tell severity

Right Heart Cath

• normal right heart catheterization pressures
  • pulmonary artery catheter
• pulmonary hypertension pre and post capillary pressures
• evaluate for shunt in pulmonary hypertension with RHC
• vasoreactivity testing for pulmonary hypertension

Work up

• hemodynamics in portopulmonary hypertension
• PVOD pulmonary venoocclusive disease and PCH pulmonary hemangiomatosis

Other pathophysiology

• higher PVR is higher predictor for mortality in pulmonary HTN
• High PCWP in pulmonary hypertension can be beneficial
• cor pulmonale is right heart failure from pulmonary hypertension

Treatment

• Generally figure out if pH is dominant driver of disease or comorbidity is dominator of disease
• pulmonary hypertension treatment overview
• use endothelin antagonist to treat pulmonary hypertension in sickle cell patients
• pulmonary vasodilators can worsen group 2 PH
• ILD and pulmonary hypertension ILD-PH
  • avoid Riociguat: worse outcome
  • avoid endothelin receptor
  • prostacyclins: Tyvaso approved for ILD-PH but not COPD-PH
• Group 3 PH treatment
  • PDEi: can start low dose sildenafil but watchout for worsening hypoxemia. Can go up to 40 but not up to 80¹
  • Can start tadalafil 20 for group 3 PH and go up to 40 mg if tolerated
  • Tyvaso: used for ILD-PH. Not effective in CPFE subgroup
  • avoid endothelin receptor: ERA, Winrevair: not recommended in group 3 PH

Literature Notes

• ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
• pulmonary hypertension 1 31 2023 lecture
• RVEDP = surrogate for RAP?
• sotatercept: stellar trial
• redox trial for o2

Links to this note

• Pulmonary Diseases

  • pulmonary hypertension

• Cardiology and Hemodynamics

  • pulmonary hypertension

• pulmonary artery catheter

  • related: Cardiology and Hemodynamics, pulmonary hypertension

• normal right heart catheterization pressures

  • related: cardiac hemodynamics and pressures, pulmonary hypertension

• ILD and pulmonary hypertension ILD-PH

  • related: ILD Interstitial Lung Disease or Diffuse Parenchymal Lung Disease, pulmonary hypertension

• When to suspect pulmonary hypertension

  • related: pulmonary hypertension

• read CVP and PCWP at end expiration

  • related: pulmonary hypertension, CVP tracing

• echo findings in pulmonary hypertension includes, high RVSP, TR, and RV dysfunction

  • related: pulmonary hypertension

• pulmonary hypertension clinical classifications

  • related: pulmonary hypertension

• evaluate for shunt in pulmonary hypertension with RHC

  • related: pulmonary hypertension

• genetic mutations in pulmonary hypertension

  • related: pulmonary hypertension

• hemodynamics in portopulmonary hypertension

  • related: pulmonary hypertension

• higher PVR is higher predictor for mortality in pulmonary HTN

  • related: pulmonary hypertension

• large PA size on CT can suggest pulmonary hypertension but does not tell severity

  • related: pulmonary hypertension

• pulmonary hypertension pre and post capillary pressures

  • related: pulmonary hypertension

• pulmonary hypertension treatment overview

  • related: pulmonary hypertension

• pulmonary vasodilators can worsen group 2 PH

  • related: pulmonary hypertension

• PVOD pulmonary venoocclusive disease and PCH pulmonary hemangiomatosis

  • related: pulmonary hypertension

• Suspected pulmonary hypertension initial workup

  • related: pulmonary hypertension

• use endothelin antagonist to treat pulmonary hypertension in sickle cell patients

  • related: pulmonary hypertension

• vasoreactivity testing for pulmonary hypertension

  • related: pulmonary hypertension

• pulmonary hypertension associated medications

  • related: pulmonary hypertension

• sickle cell disease and pulmonary hypertension

  • related: pulmonary hypertension

Footnotes

1. NCTS lecture 11 2026 ↩