Pulmonary Circulation Disorders Flashcards
What is the next step in management?
- 65 year old with SOB
- PMH: PAF, HTN
- PE: HR 75 (regular), BP 155/88, O2 sat 95% (RA)
- CV exam: JVP 10, crackles at bilateral lung bases, bilateral LE edema
- Echo: LVEF 65%, LVH, mild-moderate MR, PASP 48 mmHg
- CT chest: mild bibasilar pulmonary infiltrates, no PE
- PFT’s: mild reduction in diffusion capacity
- BNP 135
- Meds: Metoprolol XL 50mg, Amiodarone 200mg, Lasix 20mg daily, Warfarin
Increase diuretic therapy
- increased pulmonary venous pressure secondary to:
- chronic HTN
- LVH
- diastolic dysfunction
What are two etiologies that must be evaluated and treated prior to further evaluation or treatment for PH?
left-heart disease
and
pulmonary disease
What are high risk factors in assessment of patients with PAH?
- Syncope
- NYHA / WHO Class IV
- 6MWD
- < 300 m
- CPET
- Peak VO2 < 12 mL/kg/min
- Echo:
- Pericardial effusion
- TAPSE < 1.5 cm
- Hemodynamics:
- RAP > 15 mmHg
- CI « 2 L/min/m2
- CMR
- RVEF < 35%
What is the recommended treatment?
- Initial upfront presentation of PAH
- Seriously ill
Combination thearpy (Double or Triple)
- Epoprostenol
+ / -
- Ambrisetan
+ / -
- Tadalafil
What is the diagnosis and next best step?
- 51 year old female with systemic sclerosis and worsening DOE with minimal activity
- PE: elevated JVP, prominent P2 and a right sided precordial heave
- Echo: PASP 70 mmHg
- RHC with pulmonary vasoreactivity testing
- RA 14
- RVSP 68, RVEDP 14
- PASP 68/28
- PCWP 12
- CO 4.8
- CI 2
- NO administration –> no significant change
- Precapillary PAH (high risk features)
- High RA pressure
- low CI
- WHO functional class 4
-
IV Epoprostenol and Oral Ambrisentan
- combination has been studied and appears to have hemodynamic advantages upfront for severe, high-risk PAH
- ESC guidelines - Class IIa recommendation
What is the first line treatment for patients who respond to NO vasoreactivity testing?
High-dose CCB (diltiazem)
- recommended only for:
- idiopathic
- heritable
- drug-induced
What are the three FDA-approved categories of PAH treatment?
- Prostacyclins
- Endothelin receptor antagonists
- Phosphodiesterase type 5 inhibitors and soluble gunaylate cyclase stimulators
- target NO pathway
What are the prostacyclins that are commercially available for treatment?
- Epoprostenol (continuous IV)
- Treprostinil (continuous SQ, IV, intermittent inhaled, oral)
- Iloprost (intermittet inhaled)
What is the MOA of prostacyclins?
- Prostacyclin synthase is reduced in PAH –>
- inadequate production of prostacyclin I2
- a vasodilator with antiproliferative effects
What are the endothelin receptor antagonists that are commercially available for PAH treatment?
- Ambrisentan
- Bosentan
- Macietentan
What should be evaluated on a monthly basis with Bosentan treatment?
LFT’s
- not required with ambrisentan
What is the MOA in endothelin receptor antagonists?
Endothelin-1 is a potent vasoconstrictor and smooth muscle mitogen
What is the MOA of phosphodiesterase inhibitors in PAH treatment?
- reduction in NO synthase in PAH –> derangements of the cyclic GMP pathway
- PDE5 inhibition –>
- inhibit the hydrolysis of cGMP
What are the commercially available PDE5 inhibitors for treatment of PAH?
- Sildenafil
- Tadalafil
What are the commercially available soluble guanylate cyclase stimulators in PAH treatment?
Riociguat
What is the MOA for soluble guanylate cyclase stimulators?
- directly stimulates soluble guanylate cyclase independent of NO and
- increases the sensitivity of soluble guanylate cyclase to NO
What is a contraindication to riociguat use?
Nitrates
Describe the findings
- NSR ( +1 )
- PVC’s ( +1 )
- RBBB, complete ( +2 )
- Anterolateral MI, age recent or probably acute ( +4 )
Describe the findings
- SVT ( +4 )
- RAD ( +1 )
- Electrical alternans ( +1 )
*****patient subsequently diagnosed with WPW / AVRT
Describe the findings
- NSR ( +1 )
- AV junctional rhythm/tachycardia ( +2 )
- 3rd degree AV block ( +4 )
- ST and/or T wave abnormalities suggesting myocardial ischemia ( +2 )
- VVI, normally functioning ( +2 )
Describe features of the Two-minute assessment of hemodynamic profiles
- Evidence for congestion (elevated filling pressures)
Evidence for congestion / elevated filling pressures
- Increasing S3
- High JVP
- Orthopnea
- Valsalva square wave
- Ascites
- Loud P2
- Edema
- Abdominojugular refulx
- Rales (uncommon)