Outcomes of TAVR in Bicuspid Aortic Valve Stenosis
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The most common congenital cardiac anomaly, affecting an estimated 0.4–2.25% of the general population, is the bicuspid aortic valve. The “pure” bicuspid aortic valve (non-raphe-type or bicuspid aortic valve type 0) is composed of 2 cusps, morphologically and functionally. The shape of the bicuspid aortic valve annulus is often elliptical, is relatively larger than the tricuspid aortic valves, and probably shows severe eccentric calcification. This situation contributes to the difficulties in selecting the correct type and size of transcatheter heart valve when treating bicuspid aortic valve stenosis. Furthermore, it is often associated with a dilated, horizontal ascending aorta and effaced sinuses. The goal of our video tutorial is to present the contemporary circle method used in preoperative sizing during TAVI procedures in patients with a bicuspid aortic valve as well as certain technical considerations and useful advice. Although annular sizing is the main focus for most patients with a bicuspid aortic valve, some patients may need the supra-annular level of sizing. For a dedicated sizing and positioning approach for the SAPIEN 3 Ultra valve, experts in the field propose the circle method.
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Hereditary factors may have a part to play in the development of bicuspid aortic valve. This report concerns a pair of identical twins, probably monozygotic, who required aortic valve replacement as a result of aortic stenosis occurring in bicuspid aortic valves. This supports the contention that there may be a genetic influence in the development of bicuspid aortic valve, though environmental factors may be of greater importance.
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Bicuspid aortic valve ( BAV ) is the most common congenital heart valve abnormality. Different BAV phenotypes determine procedural and clinical success with transcatheter aortic valve replacement ( TAVR ). BAV has consistently been an exclusion criterium in the pivotal randomized controlled TAVR trials. Patients with BAVs are typically younger and at lower surgical risk. Registry data confirmed favorable clinical outcome after TAVR with contemporary THV platforms in selected cohorts of BAV patients. Still, TAVR in the context of BAV seems associated with a higher rate of stroke and more than mild paravalvular leakage. Optimized patient selection and continued THV iterations may further improve TAVR outcomes in BAV.
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The bicuspid aortic valve is the most frequent congenital cardiac malformation; it may be isolated or associated with other congenital heart disease. The present investigation consists of a study of bicuspid aortic valves in 1022 heart specimens belonging to the anatomical collection of the Institute of Pathological Anatomy of the University of Padua. A bicuspid aortic valve was observed in 95 specimens. It occurred as an isolated congenital cardiac defect in 28 cases, seven of which had spontaneous laceration of the aortic valve (aortic dissection). It was associated with other congenital cardiac malformations in 67 out of the remaining 994 specimens (6.7%), 41 of which (61.2%) showed obstruction of the aortic arch. The frequency of bicuspid aortic valve in specimens with complete transposition of great arteries was only 1%. Bicuspid aortic valve was particularly frequent in association with ventricular septal defect and was significantly more frequent in cases with (51.1%) than in cases without (20.5%) aortic arch obstruction (p < 0.001). There was no significant relationship between the occurrence of bicuspid aortic valves and left ventricular outflow tract obstructions or mitral valve malformations. The morphology of the pulmonary valve was also examined. Concurrence of a bicuspid aortic and pulmonary valve was detected in 11 specimens, five of these had trisomy-18. Our findings cast doubt on the assumption that altered fetal blood flow through the aortic valve may be the main factor producing the bicuspid condition. Indeed, they rather support the hypothesis that most bicuspid aortic valves are expressions of a developmental complex that affects the aortic arch and the wall of the ascending aorta as well as the aorta valve.
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Bicuspid aortic valve is the most common congenital cardiac defect in adults, and symptom typically develops in adulthood. In the majority of cases, bicuspid aortic valve disease progress with ages and surgical aortic valve replacement is performed with excellent operative outcomes. However, with the relatively slow progression of disease, surgical aortic valve replacement is required in elderly patients but the surgical risk often deemed extremely high due to old age and multiple comorbidities. Transcatheter aortic valve replacement (TAVR) has evolved from a novel technology to an established therapy for intermediate- and high-risk patients with symptomatic severe aortic valve stenosis (AS). Numerous studies have demonstrated the safety and efficacy of TAVR, and more than 250,000 patients have been treated with this technology. Although randomized trials have established TAVR as the standard treatment, these trials excluded congenital bicuspid AS due to its unique morphological features. Nevertheless, the growing experience, accumulated knowledge, and advancements of new technology lead to the expand use of TAVR to other pathologies or other populations such as bicuspid AS. With integration of imaging multimodalities (computed tomography and echocardiography), the diagnosis and classification of bicuspid aortic valve has been changing. Due to unfavorable anatomic features of bicuspid AS, the outcomes of TAVR in bicuspid AS was suboptimal, particularly when using the first-generation transcatheter valves. However, the newer-generation transcatheter valves significantly improved the outcomes of TAVR in bicuspid AS. Nonetheless, several issues still remain to be resolved. Given longer life expectancy in patients with bicuspid AS undergoing TAVR, durability of transcatheter valves is concerned. In addition, patients with bicuspid aortic valves often have concomitant dilatation of proximal part of ascending aorta (aortopathy), but limited data exist about the clinical prognosis of bicuspid aortic valve with concomitant aortopathy in elderly patients. Considering the expanding indication of TAVR to lower surgical risk and younger population, these issues should be evaluated in future studies.
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There are little data on the efficacy and safety of the Edwards Sapien valve (Edwards Lifesciences, Irvine, California, USA) in congenital, bicuspid aortic valve stenosis. Patients with bicuspid valves were excluded from the randomised PARTNER trial.1 Bicuspid valves differ from tricuspid aortic valves in that the orifice is elliptical and the leaflets are typically bulky and very heavily calcified. Bicuspid valves are considered …
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