CardiomyopathyAmpDonor»Recipient Selection

Recipient Selection

Cardiomyopathy / Cardiac Transplant Donor & Recipient Selection

1. Cardiomyopathy definition

A. Any myocardial disease process that leads to clinically significant myocardial dysfunction

2. Cardiomyopathy classification

A. Dilated cardiomyopathy
B. Hypertrophic cardiomyopathy
C. Restrictive cardiomyopathy
D. Arrhythmogenic right ventricular dysplasia
E. Dilated, characterized by dilation and impaired contraction of left or both ventricles
1) Idiopathic
2) Familial/genetic
3) Viral and/or immune
4) Alcoholic/toxic
5) Presentation with heart failure, often progressive, arrhythmias, thromboembolism, and sudden death
F. Hypertrophic, characterized by left and/or right ventricular hypertrophy
1) Usually asymmetric with normal or reduced LV volume
2) Systolic gradient common
3) Familial disease with predominantly autosomal dominant inheritance
4) Myocyte hypertrophy and disarray surrounding areas of increased loose connective tissue
5) Arrhythmias and premature sudden death are common
G. Restrictive, characterized by restrictive filling and reduced diastolic volume of either or both ventricles with normal or near-normal systolic function and wall thickness
1) Idiopathic
2) Associated with other disease (amyloidosis; endomyocardial disease with or without eosinophilia
H. Arrhythmogenic right ventricular dysplasia, characterized by progressive fibrofatty replacement of right ventricular myocardium, initially with typical regional and later global right and some left ventricular involvement with relative sparing of the septum
1) Familial disease common, autosomal dominant inheritance and incomplete penetrance
2) Presentation with arrhythmias and sudden death is common, particularly in the young

3. Specific cardiomyopathies: heart muscle diseases that are associated with specific cardiac or systemic disorders

A. Ischemic
B. Valvular
C. Hypertensive
D. Inflammatory (e.g., myocarditis, Chagas' disease, HIV, etc.)
E. Metabolic (e.g., thyrotoxicosis, hypothyroidism, storage diseases, etc.)
F. General system disease (e.g., SLE, sarcoidosis, etc.)
G. Muscular dystrophies (e.g., Duchenne, Becker-type, etc.)
H. Neuromuscular disorders (e.g., Friedreich's ataxia)
I. Sensitivity and toxic reactions (e.g., anthracyclines, irradiation, alcohol)
J. Peripartal

4. Prognosis

Factor Possibly Predictive Not Predictive
FactorPredictivePossibly PredictiveNot Predictive
ClinicalSymptomsAcoholism, Peripartum, Family HistoryAge, Duration, Viral Illness
HemodynamicLVEF, CILV size, LAP, RAPViral Illness
DysrhythmiaIVCD, Complex ectopyAV blockSimple ectopy
Histologic Myofibril volume 
NeuroendocrinePI, NE, ANF, Serum Na  

5. Pharmacological Treatment of Heart Failure

A. Digoxin*
B. Diuretics
C. Afterload Reduction
D. Isosorbine dinitrate/hydralazine**
E. Angiotensin Converting Enzyme Inhibitors
F. Enalapril**
G. Captoril**
H. Lisinopril
I. Angiotensin II Receptor Inhibitors
1) Losartan
J. Calcium Channel Blockers
1) Amlodipine

6. Beta Blockers

A. Carvedilol**
B. Metoprolol*
C. Inotropic Agents
D. Beta Agonists
1) Dopamine
2) Dobutamine
E. Phosphodiesterase Inhibitors
1) Amrinone
2) Milrinone
F. Anticoagulation
1) *Decreases risk of hospitalization or decompensation
2) **Decreases mortality

7. Pharmacologic Treatment of Heart Failure

Improves SurvivalDecreases HospitalizationDecreases Survival
EnalaprilDigoxinDobutamine
CaptroprilMetoprololMilrinone
Isosorbide dinitrate Vesnarinone
Carvedilol  

8. Recipient Selection Process

A. Inclusion criteria
B. Exclusion criteria
C. Ongoing re-evaluation process

9. Inclusion Criteria

A. Absence of reversible or surgically amenable heart disease
B. NYHA Class III - IV symptoms despite optimal medical management
C. Maximal oxygen consumption < 14 ml/kg/minute
D. Estimated 1 year survival without transplant < 50%

10. Insufficient Indications for Cardiac Transplantation

A. Ejection fraction < 20%
B. History of NYHA Class III - IV symptoms
C. Low maximal oxygen consumption

11. Candidate exclusion criteria

CriteriaHigh RiskModerate Risk
Pulmonary Hypertension  
PVR > 8 Wood Units, unresponsive to nitroprussideX 
PVR > 8 Wood Units, decreasing in response to nitroprusside, but not below 3 Wood Units X
Pulmonary artery systolic pressure > 70 mmHg despite nitroprussideX 
Transpulmonary gradient > 15-20 mmHg (mean PAP - PCWP) X
Infection - active, untreatedX 
Irreversible hepatic diseaseX 
Irreversible renal diseaseX 
Irreversible pulmonary disease  
FEV1 < 1 LX 
FEV1 < 1.5 L X
Recent pulmonary infarction X
Age > 65 yearsX 
Diabetes mellitus, Type 1, with significant end-organ damage X
Cerebrovascular disease  
SymptomaticX 
Asymptomatic X
Peripheral vascular disease  
SymptomaticX 
Peptic ulcer disease  
Active bleeding X
Diverticulitis, recentX 
Chronic Active Hepatitis X
HIV positive X
Malignancy, recentX 
Malignancy, remote X
Psychiatric disease  
Acute, unresolvedX 
Recent, resolved on treatment X
Substance abuse  
Active, unresolvedX 
Recent,resolved X

12. Panel Reactive Antibody (PRA) Screen

A. AKA: HLA antibody or white blood cell antibody screen
B. Technique: Recipient sera placed in 40-60 wells containing lymphocytes with a wide variety of HLA antigens
C. Use: Determine presence of preformed antibodies
D. If > 10%: Prospective crossmatch

13. Management of Transplant Candidate While Waiting

A. Close follow-up
B. Low threshold for hospitalization
1) IV diuretics
2) Inotropic support
3) Mechanical assistance
C. Ongoing re-evaluation of candidacy

14. Ongoing Re-evaluation for Candidacy

A. Periodic assessment for degree of illness (VO2, EF, right heart pressures)
B. Periodic assessment of acceptability (development of a new or worsening of a pre-existing illness)
C. Periodic PRA determinations

15. Conditions Which Generally Preclude the Use of a Donor Heart

A. HIV positivity
B. Significant ventricular arrhythmia
C. Echocardiographic abnormalities
D. Significant global hypokinesis
E. Significant valvular abnormality
F. Significant coronary disease by arteriography or documented previous myocardial infarct
G. Any acute malignancy, except primary brain cancer
H. Inadequately treated systemic infection
I. HbsAG positive, unless recipient is also positive
J. Hepatitis C positivity, unless recipient is also positive
K. Death from carbon monoxide poisoning, with carboxyhemoglobin level > 20%
L. Significant cardiac contusion
M. Severe left ventricular hypertrophy by echo
N. History of intravenous drug use

16. Donor-recipient Matching

A. Size: Greater than 80% of recipient body weight
B. Blood type: Identical or compatible
C. HLA-matching: Generally not done