Transcript Ventricular Dysfunction s/p Heart Transplantation

CPC #6
 17yr female 2 years s/p orthotopic
heart transplant
 New onset SOB, chest pain,
incontinence, weakness of arms and
legs
 Decreased ventricular function
 Normal troponin I on admission
 Elevated pro-BNP
Ventricular Dysfunction s/p Heart
Transplantation
 Early graft dysfunction
 Late graft dysfunction
Early Graft Dysfunction
 Hyperacute rejection
 Reperfusion injury
 Suboptimal donor
Late Graft dysfunction
(our differential diagnosis)
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Original disease process
Myocarditis
Humoral rejection
Cellular rejection
Acclerated graft atherosclerosis
Dextrocardia with situs inversus
Congenital heart disease
incidence similar to that of
the general population
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“He does not seem to be left handed
more than his fellows. He is apt to live his
life unmarked by any peculiarity and die
of the same disease that carry off the rest
of mankind……” Cleveland 1926
Dextrocardia with situs inversus
 Biliary atresia
 Kartagener syndrome
Mirror Image Dextrocardia
Polysplenia
 Multiple small spleens –frequently
functionally asplenia
 More commonly seen in patients with
heterotaxy (i.e dextrocardia with situs
solitus) than dextrocardia with situs
inversus
Recurrence of original disease
 Amyloidosis
 Sarcoidosis
 Hereditary hemochromatosis
Our Differential Diagnosis
 X -Recurrence of original disease
process
 Myocarditis
 Humoral rejection
 Cellular rejection
 Accelerated graft atherosclerosis
Myocarditis in Pediatric Heart
Transplants
 Viruses –CMV,EBV, varicella-zoster,
respiratory viruses, herpes simplex
 Bacteria – mycobacteria, gram
positive, gram negative
 Toxoplasmosis
 Pneumocystis
Myocarditis in our patient-unlikely
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No viral prodrome
Afebrile
WBC 8500
Troponin I <0.06
Not found on biopsy
Does have a history of CMV
Is sexually active
No longer on Bactrim prophylaxis
Myocarditis-treatment
 IVIG
 Antivirals/antibiotics
 Support
Our differential diagnosis
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X-Original disease process
X-Myocarditis
Humoral rejection
Cellular rejection
Accelerated graft atherosclerosis
Humoral rejection
 Antibody directed against donor
antigens located on the endothelial
surface of the allograft coronary
microvasculature
Humoral rejection
 More common early after transplant
but has been reported late
 More common in a sensitized patient
Humoral rejection
Treatment
 Plasmapheresis
 Cytogam
Our differential diagnosis
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X-Original disease process
X-Myocarditis
X-Humoral rejection
Cellular rejection
Accelerated graft atherosclerosis
Cellular rejection
 Mononuclear inflammatory response,
predominantly lymphocytic, directed
against the cardiac allograft
ISHLT Biopsy Grades
Cellular Rejection- treatment
1R- no treatment
2R-steriod bolus
3R-steriods and antithymocyte globulin
Cellular rejection
Clinical manifestations
 Constitutional symptomsmalaise,fever,myalgias, flu-like
symptoms
 Cardiac irritation-rub, arrhythmia
 Symptoms of low cardiac outputdyspnea,syncope,orthopnea
Cellular rejection in our patientpossible
Shortness of breath
Tachycardia
Initially hypertensive then hypotensive
Not seen on biopsy but this does not
eliminate it entirely
 Risk factors-female,teenager,CMV,
African-American,?induction
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Our differential diagnosis
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X-Original disease
X-Myocarditis
X-Humoral rejection
?-Cellular rejection
Accelerated graft atherosclerosis
Accelerated Graft Atherosclerosis
 Concentric narrowing or focal
obstruction of the coronary arteries in
the transplanted heart
 Leading cause of death in long term
follow up
 Progression very variable
Accelerated Graft Atherosclerosisdetected by coronary angiography
 10% during first year
 20% by the second year
 50% by the fifth year (only 10%
severe enough to cause graft loss)
Accelerated Graft atherosclerosis
by IVUS
 25% by 1 year by single vessel
IVUS;60% by 3 vessel IVUS
 40% by 3 years by single vessel
IVUS;70% by 3 vessel IVUS
Risk factors for AGA
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Rejection
CMV
Black recipient
Male donor
Older recipient or donor
Clinical presentation
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Discovered on routine surveillance
Acute onset heart failure
Arrhythmias
Syncope
Dyspnea
Anginal-like chest pain uncommon
Abdominal pain
Our patient
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African American
History CMV
Dyspnea
Abdominal pain/chest pain
Borderline ecg
Troponin I <0.06 on admission
Rejection vs Infarction
 Acute episode on floor- normal
troponin I on admission
 No significant cellular rejection on
biopsy
 Chest pain/ jaw pain
Diagnosis
 Accelerated graft atherosclerosis with
acute infarction