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Congenital aortic valvar disease represents a heterogeneous population with suboptimal surgical repair or replacement outcomes. We assess our approach and short-term outcomes in this population using cardiac CT evaluation for personalised surgical planning and execution.
Methods:
We assessed patients who underwent aortic valvar surgery from February 2022 to August 2024. Pre-surgical evaluation included cardiac CT with quantitative assessment of the leaflet geometry and measures of leaflet coaptation. A standardised approach towards surgical execution guided by this assessment was established and followed.
Results:
Seventy-three patients underwent surgery at a median age of 26.0 years (interquartile range 19–44), 65.8% males. Forty-eight patients (65.8%) underwent some form of aortic valvar repair, with 22 of these 48 patients undergoing a valve-sparing aortic root replacement. The remaining 25 patients (34.2%) underwent some form of aortic valvar replacement. Mean post-surgical follow-up was 4.2 ± 6.1 months. Moderate or greater aortic regurgitation was present in 45 patients (61.6%) pre-operatively versus 2 patients (2.7%) post-operatively (p-value < 0.001). The peak and mean gradient improved from 33.2 ± 31.3 mmHg and 16.9 ± 10.7 mmHg pre-operatively, to 16.9 mmHg±10.7 mmHg and 9.5 ± 6.4 mmHg post-operatively (p-value < 0.001).
Conclusion:
The heterogeneity and complexity of the dysfunctional and/or dilated (neo-)aortic root encountered in those presenting for surgery necessitates a methodical, detailed three- and four-dimensional assessment. By applying such an approach, we have aimed to standardise not only the assessment, but also description and surgical execution in this challenging patient population. Excellent short-term results have been achieved, necessitating long-term follow-up to understand the potential benefits towards this personalised approach.
Loeys–Dietz syndrome is a connective tissue disorder known to cause aggressive aortopathy in paediatric patients, but it is extremely rare for cardiovascular events to present during infancy. We report the first successful aortic repair in a neonate with LDS presenting in extremis with an early onset, massive aortic aneurysm.
A 10-month-old girl with type I Loeys–Dietz syndrome developed a conspicuous aortic root aneurysm that was well demonstrated on chest X-ray/CT reconstruction. She underwent successful valve-spare repair of the ascending aorta.
Truncus arteriosus and tetralogy of Fallot with pulmonary atresia may be difficult to differentiate prenatally. We present a case that, on newborn echocardiography, angiography, and intraoperative inspection, shared features of both diagnoses.
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