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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Diffuse, concentric coronary artery narrowing on angiography due to a panarteritis

    • Maximal intimal thickness on intravascular ultrasound > 0.5 mm

    • Angina pectoris rare due to denervation of the allograft

GENERAL CONSIDERATIONS

  • Incidence is 8% at 1 year, 30% at 5 years, and 50% at 10 years

  • It is the main cause of late posttransplantation deaths and is the main factor limiting long-term survival

  • Noninvasive tests are less useful in assessing this disease

  • Recipient characteristics (eg, hypertension, hyperlipidemia, insulin resistance, and cytomegalovirus infection) and donor characteristics (eg, preexisting coronary disease, donor ischemic time) may play a role

  • Thought to be a form of chronic rejection, but the pathophysiology is complex

  • When heart failure is present, it is more likely due to a restrictive myocardial physiology rather than left ventricular systolic dysfunction

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Dyspnea

  • Sudden death

  • Ventricular arrhythmia

  • Angina is rare despite advanced graft vasculopathy

PHYSICAL EXAM FINDINGS

  • Features of heart failure may be present

DIFFERENTIAL DIAGNOSIS

  • Acute rejection

  • Cytomegalovirus infection

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • No specific lab tests have proven useful

  • Cardiac biomarker elevation if there is acute myocardial infarction (MI)

ELECTROCARDIOGRAPHY

  • ECG may indicate acute MI

  • Ventricular arrhythmias may signify allograft vasculopathy

IMAGING STUDIES

  • Echocardiography may show mitral valve E/A wave amplitude ratio > 2 in restrictive cardiomyopathy

  • Doppler echocardiography may show early mitral valve inflow velocity deceleration time < 150 ms in restrictive cardiomyopathy

  • Dobutamine echocardiography (DSE) may offer reasonable sensitivity and specificity

  • DSE may also provide prognostic value

diagnostic PROCEDURES

  • Right heart catheterization evidence of restrictive cardiomyopathy:

    • – Right atrial pressure > 12 mm Hg

    • – Pulmonary capillary wedge pressure > 25 mm Hg

    • – Cardiac index < 2 L/min/m2

  • Coronary angiography:

    • – The disease is diffuse and concentric and easy to miss on angiogram

    • – Diameter comparison with prior angiogram is critical in recognizing the condition

    • – Collateral vessel formation is uncommon

  • Intravascular ultrasound is a promising tool for early recognition of allograft vasculopathy

TREATMENT

CARDIOLOGY REFERRAL

  • All patients are usually followed by a transplantation cardiologist

HOSPITALIZATION CRITERIA

  • Heart failure

  • Acute MI

  • Symptomatic arrhythmia

MEDICATIONS

  • Pravastatin useful for prevention regardless of blood lipid levels

  • Calcium blockers plus angiotensin-converting enzyme inhibitors useful for hypertension

  • Vitamins C and E have shown efficacy for prevention

    • Everolimus plus cyclosporine may be better than azathioprine plus cyclosporine

THERAPEUTIC PROCEDURES

  • Percutaneous coronary intervention may be useful in some cases if discrete proximal lesions are noted

SURGERY
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