Pulmonary Circulation Disorders Flashcards

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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
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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

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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%
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4
Q

What is the recommended treatment?

  • Initial upfront presentation of PAH
  • Seriously ill
A

Combination thearpy (Double or Triple)

  • Epoprostenol

+ / -

  • Ambrisetan

+ / -

  • Tadalafil
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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
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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
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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
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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)
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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
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10
Q

What are the endothelin receptor antagonists that are commercially available for PAH treatment?

A
  • Ambrisentan
  • Bosentan
  • Macietentan
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11
Q

What should be evaluated on a monthly basis with Bosentan treatment?

A

LFT’s

  • not required with ambrisentan
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12
Q

What is the MOA in endothelin receptor antagonists?

A

Endothelin-1 is a potent vasoconstrictor and smooth muscle mitogen

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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
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14
Q

What are the commercially available PDE5 inhibitors for treatment of PAH?

A
  • Sildenafil
  • Tadalafil
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15
Q

What are the commercially available soluble guanylate cyclase stimulators in PAH treatment?

A

Riociguat

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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
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17
Q

What is a contraindication to riociguat use?

A

Nitrates

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18
Q

Describe the findings

A
  • NSR ( +1 )
  • PVC’s ( +1 )
  • RBBB, complete ( +2 )
  • Anterolateral MI, age recent or probably acute ( +4 )
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19
Q

Describe the findings

A
  • SVT ( +4 )
  • RAD ( +1 )
  • Electrical alternans ( +1 )

*****patient subsequently diagnosed with WPW / AVRT

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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 )
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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)
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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
Q

Which parameters on initial EKG obtained independently predict 30-day all-acuse mortality in acute MI?

A
  • Sinus tachycardia
  • sum of absolue ST segment deviations (elevation and/or depression)
  • QRS > 100 ms
26
Q

What are common etiologies of Group 1 PAH?

A
  • Drug / Toxin induced
  • Idiopathic
  • Connective tissue diseases (Scleroderma)
  • Congenintal heart disease
    • Eisenmenger’s syndrome
  • Portal Hypertesion
  • Heritable
  • HIV
  • Schistosomiasis
27
Q

What are common etiologies of Group 3 PH?

A
  • COPD
  • ILD
  • SDB
  • Other restrictive lung diseases
28
Q

What are common etiologies of Group 5 PH?

A
  • Hematologic disorders
    • chronic hemolytic anemia, myeloproliferative disorders, splenectomy
  • Sarcoidosis
  • Glycogen storage diseases
  • Thyroid disorders
  • Chronic renal failure
29
Q

Describe the PH Diagnostic Algorithm

A
30
Q

What will blocked PACs in a pattern of bigeminy pattern show?

A

“pseudo-sinus bradycardia”

31
Q

Describe the treatment algorithm for PH

A
32
Q

Describe the treatment algorithm for PAH

A
33
Q

What is considered “positive vasoreactivity testing” in PAH assessment?

A

fall in mPAP ► 10 mmHg

and

mPAP « 40 mmHg

and

unchanged / increased CO

34
Q

What CCB is typically not utilized in PAH treatmet?

A

Verapamil

  • potentinal negative inotropic effects
35
Q

What is the survival rate for newly diagnosed PH patients?

A
  • 1 year –> 85%
  • 2 year –> 70%
  • 3 year –> 55%
36
Q

What medication combination is contraindicated in PAH treatment?

A

Sildenafil + Riociguat

  • PDE 5 and soluble guanylate cyclase stimulators combination is contraindicated due to hypotension
37
Q

Define precapillary PH

A

mPAP > 25 mmHg

and

PCWP « 15 mmHg

38
Q

What agent is typically used to perform vasodilatory challenge in assessment of PAH?

A

inhaled nitric oxide

  • very short half-life
39
Q

When is vasodilatory testing in the assessment of PAH contraindicated?

A

significantly elevated left heart filling pressures

  • acute reduction in pulmonary resistance with
  • high downstream pressures may result in pulmonary edema
40
Q

What is the recommended therapy (and supportive therapy) in patients with positive vasoreactivity testing?

A
  • High-dose CCB (Diltiazem, Amlodipine, Nifedipine)
  • Supportive therapy:
    • diuretics
    • oxygen
    • oral anticoagulants (often)
41
Q

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
A

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
Q

Define postcapillary PH

A

mPAP ► 25 mmHg

+

PCWP > 15 mmHg

+

TPG > 12 mmHg

43
Q

What is the best method to determine efficacy of thearpy in PH?

A

6-minute walk distance > 380 meters

  • absolute increase in distance dose not correlate with long-term outcomes
  • absolute value / threshold distance –> predicts survival
44
Q

What are predictors of efficacy in PH treatment?

A
  • 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
Q

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
A

Group I - IPAH

CCB (high dose) + Warfarin

  • improvement in survival with use of Warfarin (goal INR 1.5-2.5)
46
Q

What is a recommended therapy?

  • CCB failure
  • Group I (IPAH)
  • WHO class 2 symptoms
A

Ambrisentan + Tadalafil

47
Q

Describe the findings

A
  • Atrial tachycardia ( +2)
  • AV block, second degree, Mobitz I (Wenchkebach) ( +4 )
  • Prolonged QT
48
Q

Describe the findings

A
  • NSR ( +2)
  • SA exit block ( +4 )
    • Grouped-beating with every 3rd P-wave dropped
49
Q

Describe the findings

A
  • 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
Q

Describe the findings:

A
  • 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
Q

What is the most common cause of a pause in sinus rhythm?

A

blocked PAC’s

52
Q

Describe coding strategy in setting of ventricular arrhythmias that originate away from the normal conduciton system

A

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
Q
A