# Pulmonary Hypertension Group ***
-- ABC: need for ICU level care 2/2 resp failure or RV failure/cardiogenic shock? put on HFNC and avoid NIPPV or intubation if able
-- Chart Check: *** rheum dx, prior CT imaging, prior echos, cath reports
-- HPI Intake: ***
-- Can't Miss: RV failure or strain
-- Admission Orders: echo, trop, NT-proBNP, lactate, echo
-- Initial Treatment to Consider: oxygen to HFNC, diuresis if overloaded, avoid boluses more than 250cc; pulm dilators can help maintain CO and reduce RV afterload
-- History: *** pulm or cardiac co-morbidities, rheum dx
-- Clinical: *** DOE, fatigue, CP/angina, syncope; Rheum Intake - joint pain/swelling, rashes, vision changes, oral/nasal ulcers, raynaud's, cough, weight changes, dysphagia/reflux, frothy urine, new weakness or numbness
-- Exam: *** hypotension, cool extremities, JVP, edema, abd distention/hepatomegaly
-- Data: *** Echo, EKG, NT-proBNP, BMP, LFTs; RHC - mPAP, PVR, PCWP
-- 1 - PAH - idiopathic, heritable (BMPR2), CTD, congenital heart disease, PVOD, HIV, portal HTN, L→R shunt, drugs (dasatinib)
-- 2 - Left Heart Disease - (60%) - HFpEF, HFrEF, Valvular Dx (AS, MR, MS), LVOT, HCM
-- 3 - Lung Disease/Hypoxia - (20%) - COPD, ILD/IPF, OSA/OHS, high altitude
-- 4 - Obstructive - CTEPH, malignancy, arteritis
-- 5 - Misc - hemolytic anemia (ex: sickle cell), MPN, sarcoid, congenital dx
The patient's HPI is notable for ***. Exam showed ***. Labwork and data were notable for ***. Taken together, the patient's presentation is most concerning for ***, with a differential including ***.
-- f/u NT-proBNP, trop, echo for acute presentations
-- Group I - HIV, CTD (ANA, RF/CCP, ANCA, Scl-70, Ro/La), genetics screening if high concern
-- Group 2 - TTE
-- Group 3 - PFTs, CT dry scan, sleep study (look for chronic lung disease or OSA)
-- Group 4 - CTPE; if high concern, will need V/Q scan
-- RHC if unclear or need formal confirmation of the diagnosis - make sure patient is euvolemic
-- O2: currently ***, continuous pulse ox with goal >92%
-- pHTN Regimen: *** in general, start with PDE5i (sildenafil, tadalafil) and/or endothelin receptor antagonist (macitenatan, bosentan); if high risk, go staight to IV or inhaled prostacyclin pathway antagonist (epoprostenol, treprostinil) as bridge to other oral therapies; the goal of therapy is to get >3 low-risk criteria (CI >2.2, RAP <8, 6MWT >440m) - if cannot after 3 months, start prostacyclin antagonist
-- Volume: *** gentle fluid vs diuresis
-- Palliative: *** if patient not candidate for ECMO as bridge to transplant, cangive morphine and benzos for air hunger and anxiety
PDF coming soon!
Pulmonary hypertension, while simple in principle, is challenging to properly diagnose and manage. It can also be rapidly fatal if pressures begin to cause the RV to fail. Pulmonary hypertension is most commonly Group 2 (cardiac etiology), or Group 3 (chronic hypoxia as etiology); in these cases, the treatments will be centered on losing weight, using home O2 and CPAP, treating HTN, and trialing diuretics to achieve euvolemia. The rarer and more heterogeneous Group 1 (PAH) can be treated with endothelin receptor antagonists (macitentan, bosentan), PDE5i (sildenafil, tadalafil), and prostacyclin antagonists (epoprostenol). Do not stop these medications when patients are admitted even if they are hypotensive - the meds are usually selective for the pulmonary vasculature and their removal can cause rebound pulmonary hypertension and lead to rapid RV failure.
When patients are hypoxic or decompensating, prioritize HFNC over NIPPV - positive pressure leads to less venous return, lower preload, and can lead to lower CO. For the same reasons, such patients can also rapidly decompensate and arrest when intubated. You should always be mindful of volume status and shoot for euvolemia. Be careful with boluses and aggressive diuresis. Too much volume overloads the system leading to heart failure whereas too little volume leads to poor forward flow and cardiogenic shock (i.e. the patient is "pre-load dependent"). These patients should almost always be co-managed with a pulmonary hypertension specialist given their precarity.