Seiichi Tazawa

Anomalous mitral and tricuspid arcade in a patient with stenotic double-orifice tricuspid valve.

A 2910-g cyanotic male infant born at 39 weeks of gestation via normal delivery was diagnosed with severe tricuspid valve stenosis, pulmonary valve stenosis, a hypoplastic right ventricle (RV), and an atrial septal defect (Fig. 1). The size of the RV and tricuspid valve was too small and inadequate for biventricular repair (Fig. 2). Therefore, at the age of 8 months, a bidirectional Glenn (BDG) procedure and transection of the main pulmonary artery were performed as palliative surgery. After the BDG procedure, tricuspid regurgitation (TR) was gradually increased, followed by RV dilatation, the pressure of which was elevated beyond the systemic pressure over time (Fig. 3a). Considering the unfavorable interaction of the left ventricle and Fontan circulation, at the age of 2 years, we decided to perform a Starnes-like procedure, i.e., closure of the tricuspid valve with a fenestrated patch and RV plication to reduce the volume and pressure of the RV. Transthoracic echocardiography prior to surgery revealed an anomalous mitral arcade with the bilateral papillary muscles directly connected to the mitral leaflet (Fig. 3a); however, the detailed morphology of the tricuspid valve could not be visualized. Transpericardial three-dimensional echocardiography (3-DE) during the Starnes-like surgery showed the anomalous tricuspid arcade, in which both the anterior papillary muscle and another abnormal papillary muscle in the RV were directly connected to the tricuspid leaflet without choldae tendinae, shaping the anomalous arcade (Fig. 4a, Video 1). In addition, a small orifice divided in two by fibrous tissue was observed (Fig. 4b, Video 2). Afterwards, TR improved from severe to mild, the RV end-diastolic volume decreased from 18 to 11 ml, and the RV pressure decreased from 112 % of the systemic pressure to 40 %. As a result, we were able to plan the final Fontan completion without any concerns.

Imaging of aortico-left ventricular tunnel by three-dimensional echocardiography

Aortico-left ventricular tunnel (ALVT) is a rare congenital heart disease presenting as congestive heart failure in the neonatal or early infantile period due to severe aortic regurgitation (AR). We presented a 1-month-old boy with ALVT, originally diagnosed by two-dimensional echocardiography; however, the detailed anatomical features were not ascertained. Real-time three-dimensional echocardiography (RT3DE) could provide clear images of the three-dimensional structures of ALVT, just the same as those of surgical findings. ALVT originated from the left coronary cusp and ran an oblique path to the subvalvular orifice, having a narrow segment in the middle. RT3DE is a clinically useful diagnostic tool to clarify the detailed anatomy of ALVT.