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)
Describe features of the Two-minute assessment of hemodynamic profiles
- Evidence for Low perfusion
- Cool forearms and legs
- Declining serum sodium level
- May be sleepy, obunded
- Pulsus Alteration
- ACE-related symptomatic hypotension
- Narrow pulse pressure
- Worsening renal function
How is PAH defined?
- mPAP ► 25 mmHg
- PCWP / LVEDP « 15 mmHg
- left heart filling pressure
- PVR ► 3 Woods units
Describe the classification system / types of PH?
- Group 1 - PAH
- Group 2 - Left sided heart disease
- Group 3 - Lung disease and/or hypoxia
- Group 4 - Chronic thromboembolic (CTEPH)
- Group 5 - Multifactorial mechanisms (unclear)
Which parameters on initial EKG obtained independently predict 30-day all-acuse mortality in acute MI?
- Sinus tachycardia
- sum of absolue ST segment deviations (elevation and/or depression)
- QRS > 100 ms
What are common etiologies of Group 1 PAH?
- Drug / Toxin induced
- Idiopathic
- Connective tissue diseases (Scleroderma)
-
Congenintal heart disease
- Eisenmenger’s syndrome
- Portal Hypertesion
- Heritable
- HIV
- Schistosomiasis
What are common etiologies of Group 3 PH?
- COPD
- ILD
- SDB
- Other restrictive lung diseases
What are common etiologies of Group 5 PH?
- Hematologic disorders
- chronic hemolytic anemia, myeloproliferative disorders, splenectomy
- Sarcoidosis
- Glycogen storage diseases
- Thyroid disorders
- Chronic renal failure
Describe the PH Diagnostic Algorithm
What will blocked PACs in a pattern of bigeminy pattern show?
“pseudo-sinus bradycardia”
Describe the treatment algorithm for PH
Describe the treatment algorithm for PAH
What is considered “positive vasoreactivity testing” in PAH assessment?
fall in mPAP ► 10 mmHg
and
mPAP « 40 mmHg
and
unchanged / increased CO
What CCB is typically not utilized in PAH treatmet?
Verapamil
- potentinal negative inotropic effects
What is the survival rate for newly diagnosed PH patients?
- 1 year –> 85%
- 2 year –> 70%
- 3 year –> 55%
What medication combination is contraindicated in PAH treatment?
Sildenafil + Riociguat
- PDE 5 and soluble guanylate cyclase stimulators combination is contraindicated due to hypotension
Define precapillary PH
mPAP > 25 mmHg
and
PCWP « 15 mmHg
What agent is typically used to perform vasodilatory challenge in assessment of PAH?
inhaled nitric oxide
- very short half-life
When is vasodilatory testing in the assessment of PAH contraindicated?
significantly elevated left heart filling pressures
- acute reduction in pulmonary resistance with
- high downstream pressures may result in pulmonary edema
What is the recommended therapy (and supportive therapy) in patients with positive vasoreactivity testing?
- High-dose CCB (Diltiazem, Amlodipine, Nifedipine)
- Supportive therapy:
- diuretics
- oxygen
- oral anticoagulants (often)
What is the next best step?
- 40 year old female seen 6 weeks post discharge for PH with continued exertional fatigue and mild orthopnea
- PMH: Tobacco abuse, HTN, obesity
- Meds: Furosemide, Lisinopril
- VS: 132/78, HR 72, BMI 33, JVP 10, clear lungs
- RHC:
- RA 15, RVSP 90/15, PASP 90/25 (mean = 47)
- PCWP 22, CO 3.9, CI 1.8
VQ scan
- to exclude thromboembolic disease
- tobacco abuse and obesity may contribute to precapillary disease process
- Calculations:
- TPG 25 / CO 3.9 –> PVR 6.4
- implies possibly a secondary cause besides left sided disease (elevated PCWP)
- TPG 25 / CO 3.9 –> PVR 6.4
- elevated PCWP + elevated TPG –> postcapillary PH
Define postcapillary PH
mPAP ► 25 mmHg
+
PCWP > 15 mmHg
+
TPG > 12 mmHg
What is the best method to determine efficacy of thearpy in PH?
6-minute walk distance > 380 meters
- absolute increase in distance dose not correlate with long-term outcomes
- absolute value / threshold distance –> predicts survival
What are predictors of efficacy in PH treatment?
- 6MWT > 380 meters
- BNP (normal or near normal)
- RV function (normal or near normal)
- WHO functional class I or II symptoms
- CI 2.5
- VO2 ► 15 mL/min/kg
What is the diagnosis and next best step in treatment?
- 35 year old AAF with 6 months of worsening DOE
- PMH: denies HTN, DM, Dyslipidemia, OA, h/o PE, tobacco abuse
- VS 135/72, HR 86, +JVD, clear lung fields, no edema
- RHC: RA 6, RV 73/9, PA 73/18 (mean 38), PCWP 13, + reactivity to NO challenge
Group I - IPAH
CCB (high dose) + Warfarin
- improvement in survival with use of Warfarin (goal INR 1.5-2.5)
What is a recommended therapy?
- CCB failure
- Group I (IPAH)
- WHO class 2 symptoms
Ambrisentan + Tadalafil
Describe the findings
- Atrial tachycardia ( +2)
- AV block, second degree, Mobitz I (Wenchkebach) ( +4 )
- Prolonged QT
Describe the findings
- NSR ( +2)
- SA exit block ( +4 )
- Grouped-beating with every 3rd P-wave dropped
Describe the findings
- NSR ( +1 )
- AV block, second degree, Mobitz I (Wenchkebach) ( +4 )
- Lengthening of PR-interval –> Mobitz I (Wenckebach)
- AV block, 2:1 ( +2 )
- slight morphology variation in the T-wave following the 4th QRS complex (best seen in lead II) –> P on T wave
- Marching out P-waves from that point demonstrates NSR with AV block 2:1
- LAD ( +1 )
- RBBB, complete ( +2 )
Describe the findings:
- Dual-chamber PPM, normally functioning ( +2 )
- Paced morphology consistent with Bi-V pacing/cardiac resynchronization ( +4 )
- RBBB morphology + dual-chamber pacing –> Bi-V device
What is the most common cause of a pause in sinus rhythm?
blocked PAC’s
Describe coding strategy in setting of ventricular arrhythmias that originate away from the normal conduciton system
AVIR
- result in abnormal ventricular activation, usually results in:
- wide QRS
- axis shift
- altered QRS voltage
- Should not be coded:
- LVH / RVH
- Axis deviation
- BBB
