Pulmonary Hypertension Flashcards
7 subtypes of group 1 PHTN
- Idiopathic
- Heritable
- Drug and Toxin induced
- PAH associated with
- Connective tissue disease
- HIV
- Portal HTN
- Congenital heart disease
- Schistosomiasis
- PAH long-term responders to CCBs
- PAH with overt features of venous/capillaries involvement (PVOD/PCH)
- Persistent PHTN of the newborn syndrome
4 subtypes of group 2 PHTN
- HFpEF
- HFrEF
- Valvular heart disease
- Congenital/Acquired cardiovascular conditions leading to post-capillary PHTN
5 subtypes of group 3 PHTN
- Obstructive lung disease
- Restrictive lung disease
- Mixed disease
- Hypoxia without lung disease
- Developmental lung disorders
2 subtypes of group 4 PHTN
- CTEPH
- Other pulmonary artery obstructions
4 subtypes of group 5 PHTN
- Hematologic disorders
- Systemic and metabolic disorders
- Others
- Complex congenital heart disease
Hemodynamics of pre and post capillary PHTN
Calculation for Transpulmonary Gradient (TPG)
TPG = MPAP - PCWP
PVR calculation formula
PVR = TPG/CO
Gene mutation responsible for 75% of heritable PAH
BMPR2
Genes associated with hereditary hemorrhagic telangiectasia and PAH
Alk-1
SMAD9
Endoglin
Gene mutation found in PAH from PVOD or PCH
EIF2AK4
Most common connective tissue disease in PAH
Scleroderma
DETECT algorithm for performing RHC in scleroderma
Get if any of the following:
- TTE changes
- FVC/DLCO ratio > 1.6
- > 2 fold upper limit of normal NT-Pro-BNP
PAH is an independent risk factor for death in what disease?
HIV
Echo criteria for RHC in portopulmonary HTN
- Tricuspid jet > 3.4
- RA enlargement
- RV enlargement or dysfunction