CPC

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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

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Ventricular Dysfunction s/p Heart Transplantation

• Early graft dysfunction
• Late graft dysfunction

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Early Graft Dysfunction

• Hyperacute rejection
• Reperfusion injury
• Suboptimal donor

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Late Graft dysfunction(our differential
diagnosis)

• Original disease process
• Myocarditis
• Humoral rejection
• Cellular rejection
• Acclerated graft atherosclerosis

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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

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Dextrocardia with situs inversus

• Biliary atresia
• Kartagener syndrome

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Mirror Image Dextrocardia
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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

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Recurrence of original disease

• Amyloidosis
• Sarcoidosis
• Hereditary hemochromatosis

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Our Differential Diagnosis

• X -Recurrence of original disease process
• Myocarditis
• Humoral rejection
• Cellular rejection
• Accelerated graft atherosclerosis

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Myocarditis in Pediatric Heart Transplants

• Viruses CMV,EBV, varicella-zoster, respiratory
  viruses, herpes simplex
• Bacteria mycobacteria, gram positive, gram
  negative
• Toxoplasmosis
• Pneumocystis

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Myocarditis in our patient-unlikely

• No viral prodrome
• Afebrile
• WBC 8500
• Troponin I lt0.06
• Not found on biopsy
• Does have a history of CMV
• Is sexually active
• No longer on Bactrim prophylaxis

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Myocarditis-treatment

• IVIG
• Antivirals/antibiotics
• Support

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Our differential diagnosis

• X-Original disease process
• X-Myocarditis
• Humoral rejection
• Cellular rejection
• Accelerated graft atherosclerosis

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Humoral rejection

• Antibody directed against donor antigens located
  on the endothelial surface of the allograft
  coronary microvasculature

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Humoral rejection

• More common early after transplant but has been
  reported late
• More common in a sensitized patient

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Humoral rejectionTreatment

• Plasmapheresis
• Cytogam

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Our differential diagnosis

• X-Original disease process
• X-Myocarditis
• X-Humoral rejection
• Cellular rejection
• Accelerated graft atherosclerosis

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Cellular rejection

• Mononuclear inflammatory response, predominantly
  lymphocytic, directed against the cardiac
  allograft

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ISHLT Biopsy Grades
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Cellular Rejection- treatment

• 1R- no treatment
• 2R-steriod bolus
• 3R-steriods and antithymocyte globulin

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Cellular rejectionClinical manifestations

• Constitutional symptoms-malaise,fever,myalgias,
  flu-like symptoms
• Cardiac irritation-rub, arrhythmia
• Symptoms of low cardiac output-dyspnea,syncope,ort
  hopnea

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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

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Our differential diagnosis

• X-Original disease
• X-Myocarditis
• X-Humoral rejection
• ?-Cellular rejection
• Accelerated graft atherosclerosis

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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

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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)

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Accelerated Graft atherosclerosis by IVUS

• 25 by 1 year by single vessel IVUS60 by 3
  vessel IVUS
• 40 by 3 years by single vessel IVUS70 by 3
  vessel IVUS

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Risk factors for AGA

• Rejection
• CMV
• Black recipient
• Male donor
• Older recipient or donor

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Clinical presentation

• Discovered on routine surveillance
• Acute onset heart failure
• Arrhythmias
• Syncope
• Dyspnea
• Anginal-like chest pain uncommon
• Abdominal pain

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Our patient

• African American
• History CMV
• Dyspnea
• Abdominal pain/chest pain
• Borderline ecg
• Troponin I lt0.06 on admission

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Rejection vs Infarction

• Acute episode on floor- normal troponin I on
  admission
• No significant cellular rejection on biopsy
• Chest pain/ jaw pain

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Diagnosis

• Accelerated graft atherosclerosis with acute
  infarction