The Presentation and Diagnosis of Coronary Allograft Vasculopathy in Pediatric Heart Transplant Recipients

One of the most important causes for long-term graft failure in pediatric heart transplant recipients is coronary allograft vasculopathy (CAV). Graft survival is approximately 50% at 5 years postdiagnosis of CAV. CAV can be difficult to detect largely because of its variability in presentation and in the definition of CAV. Making the diagnosis of CAV can be challenging, and to date, the current gold standard test is angiography, which is an expensive and invasive procedure. A number of studies in the pediatric and adult heart transplant literature exist for noninvasive methods of diagnosing CAV, ranging from biochemical markers and echocardiographic techniques to computed tomography and magnetic resonance imaging. In addition, there is evidence to show that hemodynamic and function evaluation of the coronary arteries in transplanted patients may provide diagnostic clues to the potential development of CAV. These methodologies add to the armamentarium that can compliment angiography for the purposes of diagnosis. Clinical suspicion for CAV should be heightened in patients who have had recurrent rejection, new onset arrhythmias, unexplained recurrent chest or abdominal pain, and/or the development of systolic or diastolic dysfunction in the absence of rejection. This review strives to highlight the current literature with respect to the investigations available for the diagnosis of CAV, recognizing that our understanding of this disease process is still currently in evolution.