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An 8-year-old male who had an orthotopic heart transplant at age 3 for idiopathic cardiomyopathy presents with noisy breathing, snoring at night, and daytime somnolence. He is found to have markedly enlarged adenoids and tonsils as well as cervical lymphadenopathy. He presents to the operating room for tonsillectomy/adenoidectomy and cervical lymph node biopsy. His last catheterization shows no signs of rejection and normal biventricular function.


Posttransplant lymphoproliferative disorder (PTLD) encompasses a wide range of disorders, from benign lymphoid tissue hyperplasia (lymph nodes, tonsils, and adenoids) to aggressive lymphomas. It is a complication of immunosuppression and is usually caused by Epstein-Barr virus (EBV) infection leading to B-cell (more common) or T-cell proliferation. Presentation is highly variable, including lymphadenopathy, fever, weight loss, tonsillar enlargement, gastrointestinal symptoms (diarrhea, vomiting, anorexia, abdominal pain), and rarely central nervous system symptoms. Fulminant disease can present as a mononucleosis-like illness followed by sepsis, disseminated intravascular coagulation, and multisystem organ failure. Excisional biopsy, CT scans, and bone marrow aspiration are done for staging. Treatment modalities include reduction of immunosuppression, local control with surgery and/or radiotherapy, rituximab (a monoclonal antibody that inhibits B-cell proliferation), chemotherapy, and antiviral agents.

Preoperative assessment should focus on the transplanted organ function, signs of rejection, current and recent infections, and evaluation for the following chemotherapy and immunosuppressant side effects:

  • Cyclosporine: nephrotoxicity, hepatotoxicity, neurotoxicity, hypertension, hirsutism, gum hyperplasia

  • Tacrolimus: nephrotoxicity, pancreatitis/diabetes, hypertension, hypertrophic cardiomyopathy

  • Azathioprine: myelosuppression, hepatotoxicity

  • Steroids: hypertension, diabetes, neurotoxicity


  • Continue all preoperative immunosuppressant agents.

  • Use antibiotic prophylaxis and aseptic technique to prevent infection.

  • Make a careful assessment of the airway and preparation for upper airway obstruction, including oral airways, laryngeal mask airway, and possibly preinduction IV placement.

  • If stridor is present, involvement of the epiglottis and trachea may be present. Spontaneous ventilation should be maintained, and advanced airway equipment such as rigid bronchoscopy should be available.

  • If vomiting and diarrhea are present, ensure adequate hydration and consider a rapid-sequence induction.

  • Cyclosporine can potentiate the effect of succinylcholine. Azathioprine can antagonize the effect of nondepolarizing muscle relaxants; however, the level of antagonism is probably clinically insignificant. As these drug interactions can be variable, twitch monitoring should be used.

  • Post-heart transplant patients have denervated hearts, causing a lack of a heart rate (HR) response to hypotension/hypertension, hypovolemia, light anesthesia, opioids, and atropine, as these are all mediated by the autonomic nervous system. Direct-acting medications such as epinephrine, norepinephrine, dopamine, and isoproterenol will cause an increase in HR, cardiac output, and blood pressure.

  • A five-lead electrocardiogram should be continuously monitored for heart transplant patients, as they are at risk for post transplant coronary artery disease, leading to perioperative myocardial infarction and arrhythmias. A double P wave may be noted, one from the remnant sinoatrial node that is not conducted.


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