R2 Talk

Rob Windisch, MD

Mentor: Alicia Gerke, MD

Pulmonary Hypertension 

Pulmonary hypertension (PH) – general term used to describe elevated pulmonary arterial blood pressure

Pulmonary arterial hypertension (PAH)specific condition of the pulmonary arteries themselves which causes pulmonary hypertension

Disease Process

Increased pulmonary vasculature resistance or pulmonary venous pressure  pulmonary artery hypertension  right ventricular overload, hypertrophy, ischemia  RV failure

WHO Groups

1.     Pulmonary Arterial Hypertension

2.     Left heart disease

3.     Lung disease

4.     Chronic thromboembolic

5.     Other


–       Elevated BNP

–       EKG w/ right axis deviation

–       PFT’s with decreased DLCO

–       Chest XR w/ prominent pulmonary vasculature

–     Chest CT w/ dilated PA, enlarged RV       

–       Echocardiogram

§  Cannot be used to diagnose PH; used as a screening tool

§  Visual clues: D-sign, enlarged RA and RV

§  Tricuspid annular plane systolic excursion (TAPSE) may be decreased

§  PAP = 4 x (TRV)2 + RAP to estimate pulmonary arterial pressure (PAP)

–       Right heat catherization

–       For Chronic Thromboembolic (CTEPH) specifically

§  Echo  Right heat cath  V/Q scan  CT pulmonary angiography

§  Then determine if need additional MR-PA, CT angiogram, PET CT, etc


–       In general, treat the underlying disease process that is causing the PH

–       Pulmonary rehabilitation, fluid restriction, diuretics, and oxygen are a part of most regiments

–       For CTEPH specifically:

§  Anticoagulation

§  Riociguat is only PO approved med

§  Thromboendarterectomy if conservative treatment failure

§  Transplant



Feldman, Jeremy. “Pulmonary Hypertension.” Pulmonary Hypertension RN, Inspire Pulmonary Media, pulmonaryhypertensionrn.com/about-us/.

Gopalan D, Blanchard D, Auger WR. Diagnostic Evaluation of Chronic Thromboembolic Pulmonary Hypertension. Ann Am Thorac Soc. 2016 Jul;13 Suppl 3:S222-39. doi: 10.1513/AnnalsATS.201509-623AS. PMID: 27571004.


Gopalan D, Delcroix M, Held M. Diagnosis of chronic thromboembolic pulmonary hypertension. Eur Respir Rev. 2017 Mar 15;26(143):160108. doi: 10.1183/16000617.0108-2016. PMID: 28298387.

Rubin, Lewis, and William Hopkins. “Clinical Features and Diagnosis of Pulmonary Hypertension of Unclear Etiology in Adults.” UpToDate, 17 May 2019, www.uptodate.com/contents/clinical-features-and-diagnosis-of-pulmonary-hypertension-of-unclear-etiology-in-adults.

Simonneau G, Montani D, Celermajer DS, Denton CP, Gatzoulis MA, Krowka M, Williams PG, Souza R. Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J. 2019 Jan 24;53(1):1801913. doi: 10.1183/13993003.01913-2018. PMID: 30545968; PMCID: PMC6351336.