The Quadricuspid aortic valve

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Familial non-syndromal thoracic aortic aneurysms and dissections - incidence and family screening outcomes

Background: Non-syndromal thoracic aortic aneurysm and dissection (ns-TAAD) is a genetic aortopathy, with uncertain incidence. This study documented the incidence of ns-TAAD and outcomes of family screening over 15years. Methods: Consecutive series of 2385 patients with aortic disease in prospective registry (2000 to 2014), including 675 undergoing surgery. Diagnosis of ns-TAAD included family history, aortic imaging, tissue pathology and mutation testing. Screening was offered to relatives of ns-TAAD probands, with follow-up for affected individuals. Results: There were 270 ns-TAAD probands (74% males), including 116 (43%) presenting with aortic dissection. Among surgical cases, a diagnosis of ns-TAAD was established for 116 (17%). Age of probands was 50.4 ± 14.1years, with aortic diameter of 51 ± 12mm. Screening of 581 at-risk relatives identified 216 new ns-TAAD cases (detection rate = 37%). Among 71 probands with known family history, screening identified 130 new affected relatives and among 53 probands with no family history, screening identified 86 new affected relatives. Mean age of new affected relatives at diagnosis was 44±18years, with aortic diameter of 42 ± 7mm, including 42 with diameter>50mm. Ten-year mortality was similar for probands without dissection (7.7 ± 3.1%) and new affected relatives (11.4 ± 4.0%) but greater for probands surviving initial dissection (27.6 ± 7.8%, p=0.003). Conclusions: Up to 1 in 6 patients undergoing aortic surgery have features of ns-TAAD, frequently presenting as aortic dissection but at later age than other genetic aortopathies. Family screening identifies affected relatives in up to half of ns-TAAD probands, many of whom already have significant aortic dilatation.9 page(s

Clinical utility of magnetic resonance imaging in the follow-up of chronic aortic type B dissection

Several imaging modalities are utilised in the assessment of disease progression in chronic aortic dissection. We present the case of a 66 year-old male who underwent ascending aorta repair for Stanford type A aortic dissection. On follow-up the persisting dissection of the descending thoracic aorta was observed to regress on magnetic resonance imaging (MRI). MRI has several advantages over computed tomography (CT) scanning and echocardiography in the follow-up phase of this disease.3 page(s

The Freestyle aortic bioprosthesis : a systematic review

Background: The Medtronic Freestyle bioprosthesis (FSB) provides an alternative to other prostheses for both aortic valve and aortic root surgery. This paper is a systematic review of the post-operative outcomes in patients with aortic valve and/or aortic root disease following FSB implantation. Methods: Electronic databases were searched for primary analysis, prospective randomised studies comparing the FSB with an alternative aortic prosthesis wereluded. Additionally, case series thatluded data for at least 100 individual operated patients were used for secondary analysis. Results: Among three identified randomised studies, 199 FSB cases were compared with homografts, and stented and an alternative stentless bioprosthesis. The FSB showed comparable hospital mortality (4.5% vs 5.3%) and eight-year actuarial survival (80 ± 5.0% versus 77 ± 6.0%) with the homograft (respectively) and comparable reduction in left ventricular mass index relative to other prosthesis types. Over 6000 individual patients wereluded in the selected 15 case series. Weighted mean operative mortality, neurological event rate and five-year actuarial survival was 5.2%, 5.5% and 77.8%, respectively. Conclusion: The FSB performed comparably against alternative prostheses regarding in-hospital mortality, long-term survival and reduction in left ventricular mass index.luded case series demonstrated robust post-operative outcomes in both the short and long term.8 page(s

Long term outcomes following Freestyle stentless aortic bioprosthesis implantation : an Australian experience

Background: The Freestyle stentless bioprosthesis (FSB) has been demonstrated to be a durable prosthesis in the aortic position. We present data following Freestyle implantation for up to 10 years post-operatively and compare this with previously published results. Methods: A retrospective cohort analysis of 237 patients following FSB implantation occurred at five Australian hospitals. Follow-up data included clinical and echocardiographic outcomes. Results: The cohort was 81.4% male with age 63.2±13.0 years and was followed for a mean of 2.4±2.3 years (range 0-10.9 years, total 569 patient-years). The FSB was implanted as a full aortic root replacement in 87.8% patients. The 30-day all cause mortality was 4.2% (2.0% for elective surgery). Cumulative survival at one, five and 10 years was 91.7±1.9%, 82.8±3.8% and 56.5±10.5%, respectively. Freedom from re-intervention at one, five and 10 years was 99.5±0.5%, 91.6±3.7% and 72.3±10.5%, respectively. At latest echocardiographic review (mean 2.3±2.1 years post-operatively), 92.6% had trivial or no aortic regurgitation. Predictors of post-operative mortality included active endocarditis, acute aortic dissection and peripheral vascular disease. Conclusions: We report acceptable short and long term outcomes following FSB implantation in a cohort of comparatively younger patients with thoracic aortic disease. The durability of this bioprosthesis in the younger population remains to be confirmed.7 page(s