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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)
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
“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
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
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 rejectionTreatment
Plasmapheresis Cytogam
Our differential diagnosis
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 treatment2R-steriod bolus3R-steriods and antithymocyte globulin
Cellular rejectionClinical manifestations
Constitutional symptoms-malaise,fever,myalgias, flu-like symptoms
Cardiac irritation-rub, arrhythmia Symptoms of low cardiac output-
dyspnea,syncope,orthopnea
Cellular rejection in our patient-possible
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
Our differential diagnosis
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 Atherosclerosis-detected 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
Rejection CMV Black recipient Male donor Older recipient or donor
Clinical presentation
Discovered on routine surveillance Acute onset heart failure Arrhythmias Syncope Dyspnea Anginal-like chest pain uncommon Abdominal pain
Our patient
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