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