Purpose: We assess whether cardiac catheterization and a biopsy is needed for registration of cardiac transplantation in a pediatric patient of dilated cardiomyopathy through our female case of dilated cardiomyopathy with onset at age 4 months.
Case: She needed ongoing intravenous inotropic support since 19 months. In response to her application for cardiac transplantation, the Japanese circulation society requested cardiac catheterization to rule out an anomalous origin of the left coronary artery from the pulmonary artery to accurately calculate pulmonary vascular resistance and to perform a cardiac biopsy to rule out metabolic disease as a cause of the cardiomyopathy. Because cardiac catheterization and a biopsy carried a considerable risk in this situation, we explained using video files of ultrasound cardiography to clearly show the normal origin of the left coronary artery. Tricuspid regurgitation showed almost normal right ventricular pressure under elevated left atrial pressure, not indicating the elevated pulmonary vascular resistance over exclusion criteria. No family history, no carnitine deficiency, normal hepatic and renal function, and no mental retardation did not indicate cardiomyopathy secondary to metabolic disease. Although these non-invasive assessments seemed to meet Japanese guidance for pediatric heart transplantation, cardiac catheterization and a biopsy had been requested until the end of her life.
Conclusion: We believe it is not absolutely necessary for the decision on the indication of pediatric cardiac transplantation if a non-invasive modality provided high-quality evidence. Instead, future refinement of the guidance is absolutely necessary to optimally guide the clinicians' and reviewers' evaluation process.