Case report

Written informed consent for publication of patient information and images were not obtained to protect patient identity.

Postnatal echocardiography of a boy, born at 39 weeks of gestation and weighing 3692 g, revealed type 2 persistent truncus arteriosus with severe truncal valve regurgitation and uncontrollable heart failure. He underwent emergent surgical intervention on the second day of life. Pre-operative echocardiography revealed severe truncal valve regurgitation and moderate stenosis (peak pressure gradient = 45 mmHg). The truncal annulus diameter was 14.0 mm (z-value for aortic valve = 8.4) (Fig 1a).

Figure 1.

Pre-operative two-dimensional transthoracic echocardiographic image (a) and operative findings (b) demonstrating severe truncal valve regurgitation and moderate stenosis. aAo=ascending aorta; H=head side; L=left side; LA=left atrium; LV=left ventricle; R=right side; RV= right ventricle.

The procedure was performed using median sternotomy and standard cardiopulmonary bypass. Autologous pericardium was harvested to reconstruct three cusps. It was treated with 0.6% glutaraldehyde solution for 5 minutes, followed by rinsing in a saline solution for 5 × 3 minutes each.

After clamping the common arterial trunk, it was transected 12 mm above the valve annulus. The truncal valve was quadricuspid, and all leaflets had severe dysplastic and myxomatous changes with fused commissures. Since the geometric height of each leaflet was short and an adequate coaptation zone was not formed, there was a large gap at the centre (Fig 1b). Therefore, tricuspidization with leaflet resection and annuloplasty would not be adequate. Native leaflet-sparing Truncal valve repair (TrVR), such as leaflet extension, was considered ineffective because severe dysplasia and myxomatous changes extended to the base of all valve leaflets. Hence, three-leaflet reconstruction using autologous pericardium was decided.

In neonates, it is difficult to measure each intercommissural width using the original-sizing device such as that in the Ozaki technique. ^{Reference Baird, Cooney, Chávez, Sleeper, Marx and del Nido3} We measured the truncal annulus diameter using a standard sizing device and an original template that corresponded to the measured size. We created three neoleaflets of the same size. In the original template, the width reflected along the free margin length was approximately 1.4 times that of one-third of the circumference of the truncal annulus; further, leaflet height was designed to match the annulus diameter, which was one-third of the circumference of the truncal annulus. As a result, the coaptation height was expected to be approximately half the leaflet height.

All native leaflets were excised; the annulus diameter measured 13 mm. The new leaflets were trimmed using an original template of 13-mm annular size from the glutaraldehyde-treated autologous pericardium. We sutured three pericardial patches to the native annulus with 7-0 polypropylene running sutures. Three equal new leaflets were created based on the intercoronary commissure. The new leaflet was sewn to the top of the commissure and was designed to have deep coaptation that reached up to the same horizontal plane as the commissure. The top of each commissure was secured with additional sutures to adjust the coaptation height. The intraoperative view and new leaflet design are shown in Figure 2a. Finally, ventricular septal defect closure and right ventricular outflow tract reconstruction were performed. Finally, aortic cross-clamp time and cardiopulmonary bypass time were 165 and 252 minutes, respectively.

Figure 2.

(a) Three-leaflet reconstruction; intraoperative view and original leaflet template. Regarding original template details, the width reflected along the free margin length was approximately 1.4 times that of one-third of the circumference of truncal annulus; further, leaflet height was designed to match the annulus diameter, which is one-third of the circumference of the truncal annulus. (b) Two-dimensional transthoracic echocardiographic images (long- and short-axis view with diastolic phase) 1 year post-operatively demonstrating new leaflets with reduced mobility, reduced coaptation height, moderate/greater regurgitation, and mild stenosis and growth in neoaortic annular diameter. aAo=ascending aorta; C=circumference (= annulus diameter × 3.14); L=left coronary artery; LV=left ventricle; RCA=right coronary artery.

The patient’s post-operative haemodynamics was stable. Delayed sternal closure was performed on the fourth post-operative day. Dopamine and dobutamine administration was discontinued by the sixth post-operative day. He was extubated on the 10th post-operative day and subsequently transferred from the ICU to the general ward on the 18th post-operative day. Echocardiography at discharge revealed mild regurgitation and no stenosis (peak pressure gradient = 10 mmHg). The patient steadily gained weight, and the neoaortic annulus diameter had grown over time because of somatic growth (Fig 2b). One year post-operatively, echocardiography revealed that each neoaortic leaflet became less flexible due to sclerosis and calcification, and the coaptation height was reduced because of annular growth. This was accompanied by moderate regurgitation and mild stenosis (peak pressure gradient = 19 mmHg), but the patient’s general condition was stable (Fig 2b).