Rob Windisch, MD
Mentor: Alicia Gerke, MD
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
Increased pulmonary vasculature resistance or pulmonary venous pressure pulmonary artery hypertension right ventricular overload, hypertrophy, ischemia RV failure
1. Pulmonary Arterial Hypertension
2. Left heart disease
3. Lung disease
4. Chronic thromboembolic
– 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
§ 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:
§ Riociguat is only PO approved med
§ Thromboendarterectomy if conservative treatment failure
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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.
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