日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

The function of the atrioventricular valve (AVV) has a great impact on the outcome of patients with congenital heart disease, especially those with single ventricular physiology. AVV regurgitation induces an elevation in atrial pressure, leading to an increase of both systemic and pulmonary venous pressure, and results in systemic and pulmonary congestion. There are three types of AVV: 1) mitral valve; 2) tricuspid valve; and 3) common AVV, all of which are morphologically very different. The AVV morphologies in patients with heterotaxy syndrome are extremely diversified, and thus are different from patient to patient. The function of the AVV is regulated not only by the valvar apparatus, but also by the subvalvar anatomy, ventricular, and atrial function. Although the precise preoperative anatomical assessment of the AVV still remains elusive, there are no other imaging modalities superior to echocardiography, including intraoperative transesophageal echocardiography or three-dimensional echocardiography. The tricuspid valve in patients with hypoplastic left heart syndrome or the common AVV with heterotaxy syndrome, are the most difficult AVVs to repair in single ventricles due to its complex anatomy. Thus, establishing the methodology for how to repair the AVV in this patient group would be a great contribution to prognostic improvement. Here an overview is presented on the usage of two-dimensional, three-dimensional, and transesophageal echocardiography to assess the anatomy of the AVV in pediatric patients.