Case presentation controversies in Cardiomyopathy

1. Controversies in
Cardiomyopathy.
A Case Presentation
Howie Tran, MD
Heart Failure Fellow
University of California, San Diego

2. Disclosures: None

3. Case 1.0
Spring —Transfer for local San Diego hospital—
CC: Inotrope-dependent young lady with dilated cardiomyopathy
19F w/o significant PMH p/w progressive dyspnea.
- Viral prodrome reported 2 months ago
- Since then have experienced CP, palpitations and DOE.
Brief outside hospital course.
- Dx: myocarditis (LVEF 25% + severely dilated LV+ mildly dilated RV+ at
least mod MR)
- Steady introduction of neurohormonal blockade with vascillatory
hemodynamics: Lisinopril + Carvedilol + Spironolactone
- Transferred to UC San Diego Sulpizio Cardiovascular Center on
Dobutamine 2.5mcg/kg/min (off beta-blockade)

4. 138 16
3.5 0.9
Medications
on transfer
Aspirin 81mg daily
Spironolactone 25mg daily
Furosemide 10mg bid
Dobutamine 2.5mcg/kg/min
Physical HR 112, regular
BP 85/51
JVD 12 cm (at 30o)
Grade 3/6 holosystolic murmur heard at apex without
respiratory variation
Labs 13.2
39.5
8.0
TnT 0.02
AST 20 ALT 18 Tbili 2.1

5. HD
2 RA—8
PA—38/27
mPAP—31
W—27
SvO2 62%
CO/CI (Td)—2.73/1.56
CO/CI (F)—3.20/1.83
RVSWI—4.5-5 g/m2/beat
Right Heart Catheterization:
DBA
increased to
6mcg/kg/min
ECG
I
II
III
aVR
aVL
aVF
V1
V2
V3
V4
V5
V6
V1
II
V5
25mm/s 10mm/mV 150Hz 8.0.1 12SL 239 CID: 104 EID:
aVR
aVL
aVF
V1
V2
V3
V4
V5
V6
Confirmed by BLANCHARD MD, DANIEL (84) on 6/9/2014 9:23:21 PM
10mm/mV 150Hz 8.0.1 12SL 239 CID: 104
Referred by: SCRIPPS HOSPITAL-ENCINITAS Confirmed By: DANIEL BLANCHARD MD
23810349P-R-T axes
Technician: LS
Test ind:CHEST PAIN
Page 1 of 1
EID:84 EDT: 21:23 09-JUN-2014 ORDER:

6. HD
3-6
DBA steadily decreased due to worsening tachycardia (SGC
removed) until off HD 4
Started Hydralazine 25mg TID with Isosorbide Dinitrate 5mg
BID. Discussions undertaken regarding MRI.
LVEF 14% and LVEDD 6.9cm

7. HD
7
HR 130s, regular, worsening difficulty with breathing, cool
extremities. Repeat Echo: LVEF 10%, slightly dilated RV
up-titration Milrinone 0.375 mcg/kg/min  CvO2 up to 49%
Creatinine 1.4  1.9
Stopped above meds, started Milrinone 0.25 mcg/kg/min
CvO2 29% (PICC) after several hours of inodilation
BP 80s/40s, HR 120-130s, regular

8. HD
7
RA—23
PA—45/31
mPAP—38
W—33
SvO2 35%
CO/CI (Td)—2.2/1.27
CO/CI (F)—2.7/1.50
RVSWI—2 g/m2/beat
Right Heart Catheterization
repeated on Mil 0.375 mcg/kg/min
HR 140 bpm
BP 100/58
Increased Milrinone 0.50 mcg/kg/min, kept SGC in
Considerations for Endomyocardial Biopsy?
Considerations for Advanced Therapies?

9. The issue: Timing symptom onset

11. RA—21
PA—45/31
mPAP—38
W—35
SvO2 35%
CO/CI (Td)—2.2/1.27
CO/CI (F)—2.7/1.50
RVSWI—2 g/m2/beat
Right Heart Catheterization repeated
HR 150 bpm
BP 100/58
Increased Milrinone 0.50 mcg/kg/min
Ad hoc meeting. Listed 1A for UNOS transplantation.
Strongly entertained mechanical circulatory support (concerns of BiV process)
Gostman I, Keren A. Fulminant lymphocytic myocarditis versus giant cell myocarditis. Oct 2008 www.escardio.org
HD
7 Endomyocardial Biopsy: on Mil 0.375 mcg/kg/min
Myocarditis, remember is a global process

12. RV FAILURE AFTER LVAD
PLACEMENT

13. HD 8
POD 0
HVAD LV implantation
With attempted gradual wean off cardiopulmonary bypass
RV appeared markedly dilated. MvO2 mid 40s.
Considerations for Right Ventricular Support?
Unable to wean off bypass
due to tenuous
hemodynamic status

14. Study Year Baseline Lab Imaging Hemodynamics
Ochiai et al
(N 245; RVAD 23)
Cleveland Clinic
2002 Female gender,
NICM,
Vent support,
Preoperative MCS
----- ----- Low mean and diastolic
PAPs
Low RVSWI
Fitzpatrick et
al
(N 266; RVAD 99)
UPenn
2008 Prior cardiac surgery,
SBP < 96
Cr > 1.9 Severe pre-VAD
RV failure
Cardiac index < 2.2
Low RVSWI
Matthews et
al
(N 197; RVF 68)
Michigan
2008 Vasporessor
requirement
AST > 80
Bili > 2.0
Cr > 2.3
----- -----
Drakos et al
(N 175; RVF 77)
Utah
2010 DT, IABP, Inotrope
dependence,
Obesity, BB;
ACEI/ARB (neg)
----- ----- PVR (four quartiles)
Kormos et al
(N 484; RVAD 30,
RVF 68)
Pittsburgh
2010 Vent support WBC >
10.4
Hct < 31
AST > 49
----- CVP/PCWP ratio > 0.63
RVSWI < 300
Alturi et al
(N 191; RVAD 51)
UPenn
2012 Vent support
HR > 100
----- Severe RV
dysfunction
Severe TR
CVP > 15
Composite RV failure risk factors

15. Indication: Myocarditis
Percutaneous 0 Surgical 3

17. HD 8
POD 0
HVAD LV implantation
HVAD RA implantation
With attempted gradual wean off cardiopulmonary bypass
RV appeared markedly dilated
LV HVAD
2700 rpm
4.6 L/min
3.7W
Pulsatility: 4.2 L
RA HVAD
2000 rpm
4.5 L/min
1.9W
Pulsatility:
minimal
MAPs improved and weaned off
bypass
Dobutamine 5 mcg/kg/min,
Norepiphrine 5 mcg/kg/min,
Vasopressin 0.03 U/min
Inhaled Nitric Oxide 20ppm,
MvO2 84%

18. POD
1
Weaned of iNO and NE, and extubated POD 1
Prolonged wean off Dobutamine
POD
16
Orthotopic Heart Transplantation.
Subsequent post-op course was uneventful