A case of severe aortic stenosis caused by unicuspid unicommissural aortic valve
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ABSTRACT:
SUBMITTER: Tanabe J
PROVIDER: S-EPMC7520516 | biostudies-literature | 2020 Jun
REPOSITORIES: biostudies-literature
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Project description:Unicuspid aortic valve (UAV) is an extremely rare form of congenital aortic valvular abnormality. Although UAV shows similar clinical characteristics to bicuspid aortic valve, the clinical symptoms develop at earlier age and progress at a faster pace in UAV. In this report, we are presenting a 42-year-old male with severe aortic stenosis associated with unicommissural UAV. The patients underwent a successful Bentall operation.
| S-EPMC5050315 | biostudies-literature
Project description:•UAV is a rare congenital anomaly that leads to severe symptomatic stenosis.•Echocardiography plays a critical role in the evaluation of aortic stenosis.•Correctly distinguishing between UAV and BAV is relevant in determining intervention.
| S-EPMC6098169 | biostudies-literature
Project description:The unicuspid aortic valve (UAV) is a very rare congenital anomaly, which usually presents as aortic stenosis, incompetence, or a combination of both. Here, we present a case of UAV with moderate aortic stenosis detected by transthoracic echocardiography in the infant.
| S-EPMC5353412 | biostudies-literature
Project description:The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objectives for all three competencies, see http://journals.sagepub.com/doi/10.1177/2374289517715040.
| S-EPMC7549171 | biostudies-literature
Project description:BackgroundA unicuspid aortic valve is a rare congenital cardiac abnormality. Despite its uncommon finding on an initial presentation, aortic insufficiency is accompanied with unicuspid aortic valve and this might reflect the natural history of progression in the morphology of unicuspid aortic valve.Case presentationWe describe a 65-year-old Japanese man who was evaluated for endocarditis and found to have a unicuspid aortic valve concomitant with moderate aortic insufficiency, which was, owing to the lack of evidence of valve membrane destruction, independent of underlying infectious endocarditis. In addition, aortic insufficiency was progressed because of nonbacterial thrombotic endocarditis on the ventricular side, in areas of high turbulence around the heart valve.ConclusionsOur case is unusual given the unicuspid aortic valve concomitant with aortic insufficiency, which was presumably independent of underlying infectious endocarditis because of the location of the vegetation and the lack of evidence of valve destruction. Therefore, attention should be paid to a variety of complications in the setting of unicuspid aortic valve.
| S-EPMC6753610 | biostudies-literature
Project description:Highlights•Myocardial fibrosis can occur in patients with severe asymptomatic aortic stenosis (AS).•Global longitudinal strain and cardiac magnetic resonance can detect myocardial fibrosis in AS.•Myocardial fibrosis could help to stratify the arrythmic risk of AS patients.•This could be useful to determine the timing of referral to surgery.
| S-EPMC6058217 | biostudies-other
Project description:BackgroundAortic stenosis is a progressive disease that frequently remains undiagnosed until late in the disease course. In patients that present with symptoms of heart failure and a systolic murmur at a young age, a congenital valvular abnormality must be on the differential. With patients that have accelerated symptoms of aortic stenosis and valvular dysfunction, a unicuspid aortic valve (UAV) could be present. A UAV is often difficult to distinguish from a bicuspid aortic valve (BAV) on transthoracic echocardiography. In patients with congenital valvular abnormalities an ascending aortic aneurysm can also be present. Aortic stenosis changes the jet of fluid emerging from the aortic valve leading to an increased risk for aortic aneurysm dissection and rupture. The gold standard treatment for aortic stenosis secondary to a congenital valvular abnormality is valve replacement. A known risk of aortic valve replacement is conduction abnormalities. In this case, we present a patient with a unicuspid valve who postoperatively develops complete heart block leading to pacemaker implantation.Case summaryWe present a case of a 46-year-old Caucasian male with no prior medical history who presented with progressively worsening exertional dyspnoea and palpitations for 7 months. Transthoracic echocardiogram showed a BAV, however, further work up confirmed a unicommissural aortic valve with severe aortic stenosis and moderate regurgitation along with an ascending aortic aneurysm. Aortic valve replacement and aortic aneurysm repair via the Bentall procedure was successfully completed with postoperative course being complicated by a complete heart block and subsequent permanent pacemaker placement.DiscussionWhen assessing patients with symptoms of heart failure with a systolic murmur that suggests aortic stenosis at a young age, a UAV must be kept on the differential. The symptoms of aortic stenosis and valvular dysfunction are accelerated in UAVs when compared with BAVs. Currently, the treatment for patients with congenital valvular abnormalities presenting with aortic stenosis is aortic valve replacement using traditional open surgery. A known sequelae of isolated aortic valve replacement is conduction abnormalities that can sometimes lead to permanent pacemaker placement. After the confirmation of unicuspid or bicuspid valve postoperatively, it is important to report any postoperative conduction abnormalities. This is because, currently, there is no literature that compares the incidence of conduction abnormalities after unicuspid replacement to that of other BAV syndromes.
| S-EPMC6458860 | biostudies-literature
Project description:BackgroundCirculatory efficiency reflects the ratio between total left ventricular work and the work required for maintaining cardiovascular circulation. The effect of severe aortic valve stenosis (AS) and aortic valve replacement (AVR) on left ventricular/circulatory mechanical power and efficiency is not yet fully understood. We aimed to quantify left ventricular (LV) efficiency in patients with severe AS before and after surgical AVR.MethodsCirculatory efficiency was computed from cardiovascular magnetic resonance (CMR) imaging derived volumetric data, echocardiographic and clinical data in patients with severe AS (n?=?41) before and 4 months after AVR and in age and sex-matched healthy subjects (n?=?10).ResultsIn patients with AS circulatory efficiency was significantly decreased compared to healthy subjects (9?±?3% vs 12?±?2%; p?=?0.004). There were significant negative correlations between circulatory efficiency and LV myocardial mass (r?=?-?0.591, p?<?0.001), myocardial fibrosis volume (r?=?-?0.427, p?=?0.015), end systolic volume (r?=?-?0.609, p?<?0.001) and NT-proBNP (r?=?-?0.444, p?=?0.009) and significant positive correlation between circulatory efficiency and LV ejection fraction (r?=?0.704, p?<?0.001). After AVR, circulatory efficiency increased significantly in the total cohort (9?±?3 vs 13?±?5%; p?<?0.001). However, in 10/41 (24%) patients, circulatory efficiency remained below 10% after AVR and, thus, did not restore to normal values. These patients also showed less reduction in myocardial fibrosis volume compared to patients with restored circulatory efficiency after AVR.ConclusionIn our cohort, circulatory efficiency is reduced in patients with severe AS. In 76% of cases, AVR leads to normalization of circulatory efficiency. However, in 24% of patients, circulatory efficiency remained below normal values even after successful AVR. In these patients also less regression of myocardial fibrosis volume was seen. Trial Registration clinicaltrials.gov NCT03172338, June 1, 2017, retrospectively registered.
| S-EPMC7919094 | biostudies-literature
Project description:BackgroundTranscatheter aortic valve implantation (TAVI) is a minimally invasive, life-saving treatment for patients with severe aortic valve stenosis that improves quality of life. We examined cardiac output and cerebral blood flow in patients undergoing TAVI to test the hypothesis that improved cardiac output after TAVI is associated with an increase in cerebral blood flow.DesignProspective cohort study.SettingEuropean high-volume tertiary multidisciplinary cardiac care.ParticipantsThirty-one patients (78.3 ± 4.6 years; 61% female) with severe symptomatic aortic valve stenosis.MeasurementsNoninvasive prospective assessment of cardiac output (L/min) by inert gas rebreathing and cerebral blood flow of the total gray matter (mL/100 g per min) using arterial spin labeling magnetic resonance imaging in resting state less than 24 hours before TAVI and at 3-month follow-up. Cerebral blood flow change was defined as the difference relative to baseline.ResultsOn average, cardiac output in patients with severe aortic valve stenosis increased from 4.0 ± 1.1 to 5.4 ± 2.4 L/min after TAVI (P = .003). The increase in cerebral blood flow after TAVI strongly varied between patients (7% ± 24%; P = .41) and related to the increase in cardiac output, with an 8.2% (standard error = 2.3%; P = .003) increase in cerebral blood flow per every additional liter of cardiac output following the TAVI procedure.ConclusionFollowing TAVI, there was an association of increase in cardiac output with increase in cerebral blood flow. These findings encourage future larger studies to determine the influence of TAVI on cerebral blood flow and cognitive function.
| S-EPMC7894507 | biostudies-literature
Project description:Sutureless aortic valve replacement (SU-AVR) has been developed as an alternative surgical treatment for patients with symptomatic severe aortic stenosis (AS). The aim of this study was to evaluate the clinical outcomes of SU-AVR through an assessment of hemodynamic performance and safety.From December 2014 to June 2016, a total of 12 consecutive patients with severe AS underwent SU-AVR. The endpoints were overall survival and valve-related complications (paravalvular leakage, valve thrombosis, migration, endocarditis, and permanent pacemaker implantation). The mean follow-up duration was 18.1±8.6 months.The mean age of the patients was 77.1±5.8 years and their mean Society of Thoracic Surgeons score was 9.2±17.7. The mean cardiopulmonary bypass and aortic cross-clamp times were 94.5±37.3 minutes and 54.9±12.5 minutes, respectively. Follow-up echocardiography showed good prosthesis function with low transvalvular pressure gradients (mean, 13.9±8.6 mm Hg and peak, 27.2±15.0 mm Hg) at a mean of 9.9±4.2 months. No cases of primary paravalvular leakage, valve thrombosis, migration, or endocarditis were reported. A new permanent pacemaker was implanted in 1 patient (8.3%). The 1-year overall survival rate was 83.3%±10.8%.Our initial experience with SU-AVR demonstrated excellent early clinical outcomes with good hemodynamic results. However, there was a high incidence of permanent pacemaker implantation compared to the rate for conventional AVR, which is a problem that should be solved.
| S-EPMC5796611 | biostudies-literature