Pulmonary Circulation Disorders Flashcards
1
Q
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
A
Increase diuretic therapy
- increased pulmonary venous pressure secondary to:
- chronic HTN
- LVH
- diastolic dysfunction
2
Q
What are two etiologies that must be evaluated and treated prior to further evaluation or treatment for PH?
A
left-heart disease
and
pulmonary disease
3
Q
What are high risk factors in assessment of patients with PAH?
A
- 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%
4
Q
What is the recommended treatment?
- Initial upfront presentation of PAH
- Seriously ill
A
Combination thearpy (Double or Triple)
- Epoprostenol
+ / -
- Ambrisetan
+ / -
- Tadalafil
5
Q
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
A
- 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
6
Q
What is the first line treatment for patients who respond to NO vasoreactivity testing?
A
High-dose CCB (diltiazem)
- recommended only for:
- idiopathic
- heritable
- drug-induced
7
Q
What are the three FDA-approved categories of PAH treatment?
A
- Prostacyclins
- Endothelin receptor antagonists
- Phosphodiesterase type 5 inhibitors and soluble gunaylate cyclase stimulators
- target NO pathway
8
Q
What are the prostacyclins that are commercially available for treatment?
A
- Epoprostenol (continuous IV)
- Treprostinil (continuous SQ, IV, intermittent inhaled, oral)
- Iloprost (intermittet inhaled)
9
Q
What is the MOA of prostacyclins?
A
- Prostacyclin synthase is reduced in PAH –>
- inadequate production of prostacyclin I2
- a vasodilator with antiproliferative effects
10
Q
What are the endothelin receptor antagonists that are commercially available for PAH treatment?
A
- Ambrisentan
- Bosentan
- Macietentan
11
Q
What should be evaluated on a monthly basis with Bosentan treatment?
A
LFT’s
- not required with ambrisentan
12
Q
What is the MOA in endothelin receptor antagonists?
A
Endothelin-1 is a potent vasoconstrictor and smooth muscle mitogen
13
Q
What is the MOA of phosphodiesterase inhibitors in PAH treatment?
A
- reduction in NO synthase in PAH –> derangements of the cyclic GMP pathway
- PDE5 inhibition –>
- inhibit the hydrolysis of cGMP
14
Q
What are the commercially available PDE5 inhibitors for treatment of PAH?
A
- Sildenafil
- Tadalafil
15
Q
What are the commercially available soluble guanylate cyclase stimulators in PAH treatment?
A
Riociguat
16
Q
What is the MOA for soluble guanylate cyclase stimulators?
A
- directly stimulates soluble guanylate cyclase independent of NO and
- increases the sensitivity of soluble guanylate cyclase to NO
17
Q
What is a contraindication to riociguat use?
A
Nitrates
18
Q
Describe the findings
A
- NSR ( +1 )
- PVC’s ( +1 )
- RBBB, complete ( +2 )
- Anterolateral MI, age recent or probably acute ( +4 )
19
Q
Describe the findings
A
- SVT ( +4 )
- RAD ( +1 )
- Electrical alternans ( +1 )
*****patient subsequently diagnosed with WPW / AVRT
20
Q
Describe the findings
A
- 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 )
21
Q
Describe features of the Two-minute assessment of hemodynamic profiles
- Evidence for congestion (elevated filling pressures)
A
Evidence for congestion / elevated filling pressures
- Increasing S3
- High JVP
- Orthopnea
- Valsalva square wave
- Ascites
- Loud P2
- Edema
- Abdominojugular refulx
- Rales (uncommon)
22
Q
Describe features of the Two-minute assessment of hemodynamic profiles
- Evidence for Low perfusion
A
- Cool forearms and legs
- Declining serum sodium level
- May be sleepy, obunded
- Pulsus Alteration
- ACE-related symptomatic hypotension
- Narrow pulse pressure
- Worsening renal function
23
Q
How is PAH defined?
A
- mPAP ► 25 mmHg
- PCWP / LVEDP « 15 mmHg
- left heart filling pressure
- PVR ► 3 Woods units
24
Q
Describe the classification system / types of PH?
A
- 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)
25
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
26
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
27
What are common etiologies of Group 3 PH?
* COPD
* ILD
* SDB
* Other restrictive lung diseases
28
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
29
Describe the PH Diagnostic Algorithm
30
What will blocked PACs in a pattern of bigeminy pattern show?
"pseudo-sinus bradycardia"
31
Describe the treatment algorithm for PH
32
Describe the treatment algorithm for PAH
33
What is considered "positive vasoreactivity testing" in PAH assessment?
fall in mPAP ► 10 mmHg
and
mPAP « 40 mmHg
and
unchanged / increased CO
34
What CCB is typically not utilized in PAH treatmet?
Verapamil
* potentinal negative inotropic effects
35
What is the survival rate for newly diagnosed PH patients?
* 1 year --\> 85%
* 2 year --\> 70%
* 3 year --\> 55%
36
What medication combination is contraindicated in PAH treatment?
Sildenafil + Riociguat
* PDE 5 and soluble guanylate cyclase stimulators combination is contraindicated due to hypotension
37
Define precapillary PH
mPAP \> 25 mmHg
and
PCWP « 15 mmHg
38
What agent is typically used to perform vasodilatory challenge in assessment of PAH?
inhaled nitric oxide
* very short half-life
39
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
40
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)
41
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)
* elevated PCWP + elevated TPG --\> postcapillary PH
42
Define postcapillary PH
mPAP ► 25 mmHg
+
PCWP \> 15 mmHg
+
TPG \> 12 mmHg
43
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
44
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
45
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)
46
What is a recommended therapy?
* CCB failure
* Group I (IPAH)
* WHO class 2 symptoms
**Ambrisentan + Tadalafil**
47
Describe the findings
* Atrial tachycardia ( +2)
* AV block, second degree, Mobitz I (Wenchkebach) ( +4 )
* Prolonged QT
48
Describe the findings
* NSR ( +2)
* SA exit block ( +4 )
* Grouped-beating with every 3rd P-wave dropped
49
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 )
50
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
51
What is the most common cause of a pause in sinus rhythm?
blocked PAC's
52
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
53
