Cardiomyopathy / Cardiac Transplant Donor & Recipient Selection

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1. Cardiomyopathy definition

Any myocardial disease process that leads to clinically significant myocardial dysfunction

2. Cardiomyopathy classification

Dilated cardiomyopathy

Hypertrophic cardiomyopathy

Restrictive cardiomyopathy

Arrhythmogenic right ventricular dysplasia

Dilated, characterized by dilation and impaired contraction of left or both ventricles

Idiopathic

Familial/genetic

Viral and/or immune

Alcoholic/toxic

Presentation with heart failure, often progressive, arrhythmias, thromboembolism, and sudden death

Hypertrophic, characterized by left and/or right ventricular hypertrophy

Usually asymmetric with normal or reduced LV volume

Systolic gradient common

Familial disease with predominantly autosomal dominant inheritance

Myocyte hypertrophy and disarray surrounding areas of increased loose connective tissue

Arrhythmias and premature sudden death are common

Restrictive, characterized by restrictive filling and reduced diastolic volume of either or both ventricles with normal or near-normal systolic function and wall thickness

Idiopathic

Associated with other disease (amyloidosis; endomyocardial disease with or without eosinophilia

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

Familial disease common, autosomal dominant inheritance and incomplete penetrance

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

Ischemic

Valvular

Hypertensive

Inflammatory (e.g., myocarditis, Chagas' disease, HIV, etc.)

Metabolic (e.g., thyrotoxicosis, hypothyroidism, storage diseases, etc.)

General system disease (e.g., SLE, sarcoidosis, etc.)

Muscular dystrophies (e.g., Duchenne, Becker-type, etc.)

Neuromuscular disorders (e.g., Friedreich's ataxia)

Sensitivity and toxic reactions (e.g., anthracyclines, irradiation, alcohol)

Peripartal

4. Prognosis

Factor Possibly Predictive Not Predictive
Factor	Predictive	Possibly Predictive	Not Predictive
Clinical	Symptoms	Acoholism, Peripartum, Family History	Age, Duration, Viral Illness
Hemodynamic	LVEF, CI	LV size, LAP, RAP	Viral Illness
Dysrhythmia	IVCD, Complex ectopy	AV block	Simple ectopy
Histologic		Myofibril volume
Neuroendocrine	PI, NE, ANF, Serum Na

Cumulative Mortality

Probability of Death

Probability of Survival

5. Pharmacological Treatment of Heart Failure

Digoxin*

Diuretics

Afterload Reduction

Isosorbine dinitrate/hydralazine**

Angiotensin Converting Enzyme Inhibitors

Enalapril**

Captoril**

Lisinopril

Angiotensin II Receptor Inhibitors

Losartan

Calcium Channel Blockers

Amlodipine

6. Beta Blockers

Carvedilol**

Metoprolol*

Inotropic Agents

Beta Agonists

Dopamine

Dobutamine

Phosphodiesterase Inhibitors

Amrinone

Milrinone

Anticoagulation

*Decreases risk of hospitalization or decompensation

**Decreases mortality

7. Pharmacologic Treatment of Heart Failure

Improves Survival	Decreases Hospitalization	Decreases Survival
Enalapril	Digoxin	Dobutamine
Captropril	Metoprolol	Milrinone
Isosorbide dinitrate		Vesnarinone
Carvedilol

8. Recipient Selection Process

Inclusion criteria

Exclusion criteria

Ongoing re-evaluation process

9. Inclusion Criteria

Absence of reversible or surgically amenable heart disease

NYHA Class III - IV symptoms despite optimal medical management

Maximal oxygen consumption < 14 ml/kg/minute

Estimated 1 year survival without transplant < 50%

10. Insufficient Indications for Cardiac Transplantation

Ejection fraction < 20%

History of NYHA Class III - IV symptoms

Low maximal oxygen consumption

11. Candidate exclusion criteria

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

12. Panel Reactive Antibody (PRA) Screen

AKA: HLA antibody or white blood cell antibody screen

Technique: Recipient sera placed in 40-60 wells containing lymphocytes with a wide variety of HLA antigens

Use: Determine presence of preformed antibodies

If > 10%: Prospective crossmatch

13. Management of Transplant Candidate While Waiting

Close follow-up

Low threshold for hospitalization

IV diuretics

Inotropic support

Mechanical assistance

Ongoing re-evaluation of candidacy

14. Ongoing Re-evaluation for Candidacy

Periodic assessment for degree of illness (VO2, EF, right heart pressures)

Periodic assessment of acceptability (development of a new or worsening of a pre-existing illness)

Periodic PRA determinations

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

HIV positivity

Significant ventricular arrhythmia

Echocardiographic abnormalities

Significant global hypokinesis

Significant valvular abnormality

Significant coronary disease by arteriography or documented previous myocardial infarct

Any acute malignancy, except primary brain cancer

Inadequately treated systemic infection

HbsAG positive, unless recipient is also positive

Hepatitis C positivity, unless recipient is also positive

Death from carbon monoxide poisoning, with carboxyhemoglobin level > 20%

Significant cardiac contusion

Severe left ventricular hypertrophy by echo

History of intravenous drug use

16. Donor-recipient Matching

Size: Greater than 80% of recipient body weight

Blood type: Identical or compatible

HLA-matching: Generally not done