Right heart test for the right HF patient // Mexico ...

©2013 MFMER | 3248567-1©2013 MFMER | 3248567-1

Right heart test for the right HF

patient // Mexico, October 2016

Hector I. Michelena MD FASE FACCAssociate Professor of Medicine

©2013 MFMER | 3248567-2

DISCLOSURE

No Relevant Financial Relationship(s)

No Off Label Usage

©2013 MFMER | 3248567-3

Case

• 50 year old white female, dilated CMP

• Hx Mv repair, severe functional MR

• Presented with syncopal event

• Noted to have 3rd Degree Heart Block

• PPM implanted

• Father died at age 59 of “heart disease”

• Needed AICD for VT later

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Case

• Blood work

• Hgb 14.8 WBC 5.7 Plt 190

• NA 142 K 4.2 Cr 1.1

• Physical exam

• Normotensive, Normal Weight

• Paradoxically Split S2, otherwise Nl

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Case

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Case

A. Coronary artery Disease

B. Cardiac Sarcoid

C. Hypertrophic Cardiomyopathy

D. Dilated Cardiomyopathy

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Case

A. Coronary artery Disease

B. Cardiac Sarcoid (Isolated)

C. Hypertrophic Cardiomyopathy

D. Dilated Cardiomyopathy

Left atrial wall from Mv repair surgery: pathologic confirmation

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Cardiac Sarcoidosis

**

*Extensive Transmural

fibrosis non-coronary distribution

non-necrotizing granulomatous

inflammation, giant multinucleated cells,

and epithelioid hystiocytes

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Sarcoidosis

• Granulomatous inflammatory disease of unknown etiology involving multiple organs (immune system disorder)

• Main manifestations caused by pulmonary involvement

• pulmonary fibrosis

• right-sided heart failure

• pulmonary hypertension

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PulmonaryNodules

SarcoidCXR

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Cardiac SarcoidosisClinical Cardiac Features

• Can affect any portion of the heart

• Can produce a myriad clinical problems that may simulate other more common disorders (palpitations, atypical chest pain, syncope, etc)

• Syncope or sudden death

• Heart Block, VT, VF

• CHF

• Can also be asymptomatic

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Cardiac SarcoidosisEchocardiographic Features

• Early: LV walls may be thick

• Late LV findings:

• Segmental thinning

• Regional wall motion abnormalities

• LV aneurysms

• LV enlargement, reduced function

• Diastolic dysfunction

• Pericardial effusion

• Valvular dysfunction(MR)

• With pulmonary involvement

• RVE, decreased RV function and RVH

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Cardiac SarcoidosisIn Who??

• <55 yo ECG BLOCK

• Known extracardiac sarcoidwith heart symptoms

• <55 yo

• Monomorphic VT

• Dilated CMP

• HCM

• ARVD

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MRICardiac Sarcoidosis

• Findings can overlap with other causes of myocarditis

• Direct cardiac involvement:• Clinical – 5%• Subclinical – about 70%

• Typical findings:• Wall thinning• Wall motion abnormalities• Edema in acute phase• Nonischemic delayed

enhancement

©2011

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3160621-15

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©2011

MFMER |

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• Nonischemic delayed enhancement

• Basal and mid septal predilection

• RV enhancement common

• Amount of delayed enhancement correlates inversely with EF

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• May see pulmonary findings

• Helps distinguish from other causes of myocarditis

©2011

MFMER |

3160621-17

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Greulich et al: J Am Coll Cardiol Img 6:501, 2013

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Cardiac MRI in SarcoidDeath, AICD Discharge

0.0

0.2

0.4

0.6

0.8

1.0

0 365 730 1,095 1,460 1,825 2,190 2,555 2,920


Days after CMR

Cu

mu

lative

su

rviv

al fr

ee

of a

bo

rte

d S

CD

an

d IC

D s

ho

cks No LGE

LGE

P<0.0001

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Cardiac MRI in SarcoidDeath, AICD Discharge, VT

0.0

0.2

0.4

0.6

0.8

1.0

0 365 730 1,095 1,460 1,825 2,190 2,555 2,920


Days after CMR

Cu

mu

lative

su

rviv

al fr

ee

of

ab

ort

ed

IC

D s

ho

cks, V

T a

nd

nsV

T

Patients at risk

No LGE 89 63 44 29 20 15 10 5

LGE 23 20 17 11 5 4 4 2

No LGE

LGE

P<0.0001

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Survival Free of Death or VT Stratifiedby Cardiac PET Examination Results

0.00

0.25

0.50

0.75

1.00

0 200 400 600 800

Blankstein et al: J Am Coll Cardiol 63:329, 2014

Time (days)

Su

rviv

al fr

ee

of d

ea

th/V

T

P<0.01

Normal perfusion and FDG

Abnormal perfusion or FDG

Abnormal perfusion and FDG

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Diagnostic and Treatment Algorithmfor a Patient With Extracardiac Sarcoid


Patient withproven

extra-cardiacsarcoid

1. H&P2. ECG based testing

3. TTE

Any of the above abnormal&

no severe CAD by angiography

A. CMR or 18F-FDG PET orB. Rest/stress sestamibi or 201-thallium plus 67-gallium

Treat with steroids& answer below:

1. Type II 2nd degree or complete AV block?2. Sustained VT?

3. EF <50%?

Place ICD +/- CRT SAECG

PES No ICD +/- CRT

Follow-up

in 6 months

Look for

other

causes

Follow-up

in 3 months

Not suggestive

Normal

Negative

Positive

Positive Negative

NoYes (at least one)

Abnormal

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Diagnostic Algorithm for a Patient With Suspicion for Isolated Cardiac Sarcoidosis


No prior historyof sarcoidosisand less than60 years old

1. Type II 2nd degree or complete AV block2. Sustained VT

3. Unexplained diastolic or systolic heart failure

Suspect cardiac sarcoidosis andrefer for two types of imaging

Type 1A. CMR or 18F-FDG PET or

B. Rest/stress sestamibi or 201-thalliumplus 67-galliumType 2

High resolution, CT chest #

Type 1) or 2) is positive Type 1) or 2) are negative

Consider image guided biopsy before

steroid initiation

Low probability forcardiac sarcoidosis

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Cardiac Sarcoid

• CMR may have a higher specificity for diagnosis of cardiac sarcoid

• PET appears to be more sensitive

• Both PET and CMR appear predictive of outcomes

• Local expertise and patient factors will determine optimal testing

• RV Biopsy is gold standard

• Low sensitivity in 20% range (Patchy distribution)---Guided

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Cardiac SarcoidTreatment

• Steroids remain treatment of choice

• No Randomized Trials

• Steroid therapy

• Appears to decrese mortality (non randomized)

• Appears to maintain LV function if initiated in patients with normal function at time of diagnosis

• LVEF improves in patients with mild to moderate LV dysfunction at time of diagnosis (our case)

• No improvement in LVEF in patients with severe LV dysfunction (<30%) at time of diagnosis

Right heart test for the right HF patient // Mexico ...

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