general Flashcards
High risk features of PAH
- New York Heart Association class 4 dyspnea
- Syncope
- RA pressure >15 mm Hg
- 6-minute walk distance <300 m
- Peak VO2 <12 ml/kg/min
- Cardiac index ≤2 L/min/m2
- Right ventricular ejection fraction <35% on cardiac magnetic resonance.
This therapy should be included in all its with severe PAH
epoprostenol should be included in initial treatment plan of high risk patients
expert opinion favors upfront combination therapy of two to three drugs, including epoprostenol.
Classified as Group 1 PAH
- idiopathic PAH
- heritable PAH (the most common mutation being bone morphogenetic protein receptor type 2 [BMPR2])
- drug- or toxin-induced PAH (particularly by appetite suppressant drugs)
- those associated with connective tissue disorders (particularly scleroderma)
- human immunodeficiency virus (HIV) infection
- portal hypertension
- schistosomiasis
- related to congenital heart disease
- other illnesses such as hemoglobinopathies, glycogen storage diseases, hereditary hemorrhagic telangiectasia, and chronic myeloproliferative disorder.
used to assess response and need for repeat endarterectomy in patients with CTEPH
6 min walk test
appropriate vasodilator response during RHC
Fall in mean pulmonary artery systolic pressure of at least 10 mm Hg to a value less than 40 mm Hg, without a drop in cardiac output
goals for patient on therapy for PAH
Achieving 6-minute walk distances above this range is a generally accepted goal for patients on therapies for pulmonary artery hypertension.
- Normal or near normal BNP
- Normal or near normal right ventricular function
- WHO functional class I or II symptoms
- Cardiac index = 2.5 L/min/m2, or
- VO2 ≥15 ml/min/kg.
when is RHC indicated
RHC is indicated in patients with suspected pulmonary arterial HTN as part of the work up of elevated right heart pressures detected on echocardiography
systemic anticoagulation in PAH? What is the recommendation?
Most guidelines recommend warfarin anticoagulation titrated to an international normalized ratio of 1.5-2.5 in patients with IPAH.
What is the gold standard for diagnosis of CTEPH
V/Q scan
Contraindications to acute vasodilator testing
- Elevated left side filling pressures
- Pts with markedly decompensated RHF and RV dysfunction where calcium channel blockers would not be an appropriate long-term therapy
what conditions fall under WHO group 5 PH
Miscellenous group
- Sarcoidosis
- lymphangiomatosis
- Compression of pulmonary vessels
Characteristic of CREST syndrome
CREST syndrome a variant of Systemic Sclerosis a limited cutaneous form which is characterized by;
- Calcinosis
- Raynaud’s
- Esophageal dysfunction
- Sclerodactaly
- Telangectasias)
