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Download Ventricular Dysfunction s/p Heart Transplantation
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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 rejection Treatment 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 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 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 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 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