Truncal valve insufficiency in neonates is a major risk factor for postoperative mortality. Reference Russell, Pasquali and Jacobs1 Mechanical valve replacement is technically challenging; thus, native leaflet-sparing repair is generally preferred. However, fragile, dysplastic leaflets and annular dilatation make repair difficult. In adult aortic valve repair, studies emphasise the importance of root geometry, including the sinotubular junction and annulus, for durable leaflet coaptation. Reference Pettersen, Du, Skeens and Humes2 Despite this, precise root reconstruction has rarely been applied in neonatal truncal valve repair. Here, we report a case of severe neonatal truncal valve insufficiency successfully treated with geometry-based root reconstruction using body-weight–adjusted reference values.
Case report
Written informed consent for publication of patient information and images was not obtained because anonymised data were used.
A male neonate, born at 39 weeks of gestation and weighing 2908 g, was diagnosed postnatally with type I persistent truncus arteriosus accompanied by severe truncal valve regurgitation and refractory heart failure. Despite intensive medical therapy, cardiac dysfunction worsened owing to excessive pulmonary blood flow, and bilateral pulmonary artery banding was performed on day 4. However, distortion of the truncal root and increased afterload aggravated valve regurgitation, necessitating emergent truncal valve repair on day 7 after birth. Because the patient was a small neonate with severe regurgitation and pre-existing ventricular dysfunction shortly after bilateral pulmonary artery banding, we were concerned that substantially prolonging the aortic cross-clamp time to perform complete intracardiac repair at the index operation might increase the risk of postoperative low cardiac output and myocardial injury. We therefore adopted a staged strategy, prioritising truncal valve and root reconstruction at 7 days of age and deferring ventricular septal defect closure until 2 months of age under more stable conditions.
Preoperative echocardiography and computed tomography findings of the truncal valve and root are shown in Figures 1(a) and 1(b). Severe truncal valve regurgitation was noted, with leaflet geometric heights ranging from 6.6 to 8.2 mm. Measured diameters were 13.0 mm at the annulus (z score for aortic valve = 6.8), 13.0 mm at the sinuses of Valsalva, and 14.4 mm at the sinotubular junction.
Preoperative echocardiography ( a ) computed tomography scan ( b ) and intraoperative findings ( c ) demonstrating severe truncal valve regurgitation and an enlarged truncal root with a severely dysplastic quadricuspid valve. The incomplete central commissure and dysplastic small leaflets (red arrow) are indicated by direct labels. (a = annulus, 8.6 mm; b = sinuses, 11.1 mm; c = sinotubular junction, 9.7 mm). LCA = Left Coronary Artery; RCA = Right Coronary Artery.
Based on reference values for aortic root dimensions corresponding to the patient’s body weight, Reference Pettersen, Du, Skeens and Humes2 we planned root reconstruction targeting diameters of 8 mm at the annulus, 13 mm at the sinuses of Valsalva, and 10 mm at the sinotubular junction.
The procedure was performed through a median sternotomy under standard cardiopulmonary bypass. After clamping the common arterial trunk, it was transected 5 mm above the sinotubular junction. The truncal valve was quadricuspid, with an incomplete central commissure between the left coronary and noncoronary leaflets. The right coronary leaflet was mobile but markedly thickened with myxomatous change (Figure 1(c)). The left coronary leaflet was bifid and short, the noncoronary leaflet was similarly shortened, and the right coronary leaflet had restricted mobility; as a result, adequate central coaptation could not be achieved, and a residual central gap became the main mechanism of regurgitation. Because trileafletization would have required patch augmentation and additional commissural creation in this fragile neonatal valve, we excised the incomplete central commissure and sutured the left coronary and noncoronary leaflets together, creating a bicuspid valve opposed by the right coronary leaflet and maximising the coaptation area without leaflet patching.
Following excision of the incomplete commissure, the left and noncoronary leaflets were sutured together to form a bicuspid valve with the right coronary leaflet. Each reconstructed leaflet had a geometric height of 9 mm. The main pulmonary artery arising from the left coronary sinus was divided from the truncal root, and aortotomies were made across the annulus at this site and at the centre of the right coronary sinus. Annular plication was performed at both sites to achieve an annular diameter of 8 mm, followed by resection of excess truncal wall to reconstruct the sinuses of Valsalva and sinotubular junction (Figure 2(a)). The truncal wall resection was limited to segments remote from both coronary ostia under direct visualisation, allowing reduction of the annular and sinotubular diameters while maintaining a safe margin from the coronary arteries. Coronary mobilisation or reimplantation was not required in this neonate.
( a ) Schematic illustration and postoperative views of the reconstructed truncal valve and root showing wall resection and annular plication (target diameters: annulus, 8 mm; sinuses, 13 mm; sinotubular junction, 10 mm). Red-shaded areas represent the portions of the aortic wall that were resected, followed by annular plication. The commissures and abnormal leaflets are directly labelled (red arrow). ( b , c ) Computed tomography and echocardiography at discharge demonstrating trivial regurgitation, no stenosis, and a reconstructed truncal root of appropriate size (a = annulus 8.6 mm; b = sinuses 11.1 mm; c = sinotubular junction 9.7 mm). LCA = Left Coronary Artery; RCA = Right Coronary Artery.
Finally, right ventricular outflow tract reconstruction was performed using a 5-mm expanded polytetrafluoroethylene graft (GORE-TEX®, W. L. Gore & Associates, Flagstaff, AZ, USA), leaving the ventricular septal defect open to minimise cardioplegic arrest time for later staged closure.
The postoperative course was uneventful. Echocardiography and computed tomography at discharge revealed trivial regurgitation, no significant stenosis (peak pressure gradient = 16 mmHg), and a reconstructed truncal root of appropriate size (annulus = 8.6 mm, sinuses of Valsalva = 11.1 mm, sinotubular junction = 9.7 mm) (Figures 2(b), 2(c)).
At 2 months of age, intracardiac repair with VSD closure was successfully performed, and the postoperative course was uneventful. At 1-year follow-up (body weight, 7.8 kg), transthoracic echocardiography demonstrated appropriate growth of the reconstructed root, with an annular diameter of 10.5 mm (Z score = 0.6), a peak Doppler gradient of 14 mmHg across the truncal valve, and only mild regurgitation. The two reconstructed cusps showed symmetric mobility with good excursion, and no prolapse, restriction, or tethering was observed.
Discussion
Enlargement of the truncal annulus and root, combined with severely dysplastic and fragile leaflets, typically results in severe truncal valve insufficiency in neonates. In adult aortic valve repair, restoring root geometry—particularly the annulus and sinotubular junction—is critical for durable valve function. Reference ElZein, Roberson and Hammad3–Reference Igarashi, Matsushima and Shimizu5 Adult procedures often employ prosthetic materials such as rings or grafts to stabilise dimensions. However, in neonates, prosthetic materials are generally undesirable because of somatic growth and tissue mismatch, requiring reconstruction using only native tissue.
The valve leaflets in neonates are extremely fragile, making complex reconstruction technically challenging. In contrast, the truncal annulus and root have greater structural integrity, allowing safe incision and suturing. Leaflet-based techniques, including tricuspidisation, limited leaflet resection, and subcommissural annuloplasty, have been reported to preserve growth potential. Reference Aybala, Mahmut and Manan6,Reference Naimo, Fricke, Lee and d’Udekem7 Nonetheless, these methods alone may not adequately reduce the annulus or restore physiologic root geometry, potentially compromising leaflet coaptation and long-term valve function.
In this case, we performed root reconstruction guided by reference values for aortic root dimensions adjusted for body weight, further refined intraoperatively according to leaflet geometric height. We acknowledge that these Z scores were originally derived from normal aortic roots and have not been specifically validated for truncal anatomy; therefore, they were used as an approximate surrogate to avoid both marked oversizing and undersizing rather than as exact targets. This represents an inherent limitation of our strategy in the absence of robust normative data for truncal root dimensions in neonates.
For this patient’s body weight, we used the Pettersen equations as reference normal values for annular, sinus, and sinotubular diameters and selected target diameters of 8 mm for the annulus, 13 mm for the sinuses of Valsalva, and 10 mm for the sinotubular junction. These targets were intended to downsize the markedly dilated annulus (13.0 mm, Z score 6.8) toward a near-normal Z score while preserving relatively large sinuses for leaflet mobility and an appropriate sinotubular junction to support the commissures, thereby approximating physiologic root geometry. Intraoperatively, the geometric height of the reconstructed cusps was approximately 9 mm. Based on adult aortic valve repair concepts linking geometric and effective heights to durable coaptation, we considered an annular diameter of 8 mm appropriate to ensure that cusp height exceeded the annular radius. Although bicuspidisation was expected to yield a somewhat shorter effective geometric height, intraoperative assessment confirmed that the resulting coaptation height was acceptable.
Excess truncal wall tissue was resected from the two sinuses not involving the coronary arteries, with the incisions extended below the annular level to allow controlled plication and remodelling. This strategy allowed us to reduce annular and sinotubular diameters while maintaining a safe distance from both coronary ostia, and coronary mobilisation or reimplantation was not required in this neonate.
This approach achieved an anatomically appropriate root configuration and enabled leaflet-sparing repair while maintaining maximal leaflet height and mobility.
Although this report describes a single case with limited follow-up, the technique demonstrates a feasible, growth-preserving, native-tissue approach for neonatal truncal valve repair. It allows physiologic restoration of root geometry, preserves the patient’s own valve tissue, and may contribute to long-term durability and adaptability as the patient grows.
Financial support
This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.
Competing interests
None.
Ethical standard
This article does not contain any studies with human participants performed by any of the authors.
