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.
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Project description:BackgroundThe aetiology of secondary complete atrioventricular blocks includes ischaemia, cardiac sarcoidosis, electrolyte imbalance, drug use, rheumatic fever, and infections such as Lyme disease and endocarditis. Diagnosis is important since some of these causes are reversible. Although several studies have reported on aortic valve calcification causing complete atrioventricular blocks, no study has described improvement of complete atrioventricular blocks by removal of the calcification.Case summaryA 42-year-old man with syncope had a Mobitz type II atrioventricular block, an alternating bundle branch block, and severe aortic stenosis. We identified a 10 s paroxysmal complete atrioventricular block with pre-syncope and performed pacemaker implantation. Electrocardiography-gated computed tomography confirmed that the calcification had reached the muscular septum. 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) revealed significant FDG uptake with high CT value of calcification in basal interventricular septum. The calcification in the septum was removed carefully, and aortic valve replacement was performed. The atrioventricular conduction capacity improved post-surgery. During the 1-year follow-up, the patient reported dramatic improvement in exercise capacity. We also noted an improvement of <0.1% in the right ventricular pacing burden.DiscussionComplete atrioventricular blocks occur in patients with aortic stenosis accompanied by severe calcification of the aortic valve, which are visualized comprehensively by echocardiography. Electrocardiography-gated computed tomography and FDG-PET enabled detailed evaluation of the extent of calcification and pre- and post-operative tissue inflammation. Hence, we suspected that the calcification in the septum was causing complete atrioventricular block. Moreover, clinicians should recognize that aortic valve calcification with aortic stenosis can cause complete atrioventricular blocks.
| S-EPMC11020220 | 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: Not available
| S-EPMC6058217 | biostudies-literature
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:Aortic valve calcium scoring by cardiac computed tomographic (CT) has been recommended as an alternative to classify the AS (aortic stenosis) severity, but it is unclear that whether CT findings would have additional value to discriminate significant AS subtypes including high gradient severe AS, classic low-flow, low gradient (LF-LG) AS, paradoxical LF-LG AS, and moderate AS. In this study, we examined the preoperative clinical and cardiac CT findings of different subtypes of AS in patients with surgical aortic valve replacement (AVR) and evaluated the subtype classification as a factor affecting post-surgical outcomes. This study included 511 (66.9 ± 8.8 years, 55% men) consecutive patients with severe AS who underwent surgical AVR. Aortic valve area (AVA) was obtained by echocardiography (AVAecho) and by CT (AVACT) using each modalities measurement of the left ventricular outflow tract. Patients with AS were classified as (1) high-gradient severe (n = 438), (2) classic LF-LG (n = 18), and (3) paradoxical LF-LG (n = 55) based on echocardiography. In all patients, 455 (89.0%) patients were categorized as severe AS according to the AVACT. However, 56 patients were re-classified as moderate AS (43 [9.8%] high-gradient severe AS, 5 [27.8%] classic LF-LG AS, and 8 [14.5%] paradoxical LF-LG AS) by AVACT. The classic LF-LG AS group presented larger AVACT and aortic annulus than those in high-gradient severe AS group and one third of them had AVACT ≥ 1.2 cm2. After multivariable adjustment, old age (hazard ratio [HR], 1.04, P = 0.049), high B-type natriuretic peptide (BNP) (HR, 1.005; P < 0.001), preoperative atrial fibrillation (HR, 2.75; P = 0.003), classic LF-LG AS (HR, 5.53, P = 0.004), and small aortic annulus on CT (HR, 0.57; P = 0.002) were independently associated with major adverse cardiac and cerebrovascular events (MACCE) after surgical AVR.
| S-EPMC9079063 | 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