Atypical Shone's Complex Diagnosed at 70 Years Old: Presenting with Double-orifice Mitral Valve,Bicuspid Aortic Valve,and Aortic Coarctation

Atypical Shone''s complex is a rare congenital anomaly involving a left-sided obstructive lesion of two or three cardiovascular levels. A 70-year-old man with dyspnea on exertion was diagnosed with severe aortic stenosis (AS) with a bicuspid valve, complicated by severe aortic coarctation (CoA) and a double-orifice mitral valve. He underwent surgery for AS and CoA in one session. It is important to search for complicated malformations, even in cases of bicuspid aortic valve found in old age.     

High-Risk Congenital Coronary Abnormalities in Patients with Bicuspid Aortic Valve

Objective: Abnormal coronary artery origin (ACAO) from the opposite sinus with inter-arterial course of the ectopic proximal vessel is associated with the greatest potential for clinical manifestations, specifically sudden death. Data remain limited regarding the association between bicuspid aortic valve (BAV) and this potentially dangerous coronary variant reported in up to 0.6% in the general population. We investigated the frequency of this high-risk ACAO with inter-arterial course in our surgical series of BAV patients. Methods and Results: We conducted a retrospective study to identify BAV patients with ACAO and inter-arterial course who underwent elective aortic valve/root surgery between 2010 and 2019 in our tertiary center. A total of 279 consecutive patients with BAV were identified. Among these, four patients (1.4%) had ACAO with inter-arterial course. Three patients had abnormal right coronary artery arising from the theoretical opposite coronary sinus with intramural course. The fourth patient presented an abnormal left coronary artery from a single coronary artery arising from the theoretical right coronary sinus with a long inter-arterial course. Conclusion: Our results from a single-center experience support high-risk ACAOS may be more frequently diagnosed in BAV patients, especially in surgical series, justifying a careful preoperative assessment.     

Prevalence of Bicuspid Aortic Valve in Turner Syndrome Patients Receiving Cardiac MRI and CT: A Meta-Analysis

Turner syndrome (TS) is a rare disorder affecting 25–50 in 100000 female newborns. Bicuspid aortic valve (BAV)is assumed to be the most common congenital heart defect (CHD) in TS. In literature, reported BAV prevalence inTS ranges between 14% and 34%. The specific BAV prevalence in TS is still unknown. The aim of this study was togive a more precise estimation of BAV prevalence in TS by conducting a meta-analysis of TS-studies, whichdetected BAV by either cardiac magnetic resonance imaging (MRI) or cardiac computed tomography (CT).We searched PubMed, Cochrane Library, and Web of Science databases to collect observational studies includingthe prevalence of BAV identified by cardiac MRI or cardiac CT in TS patients up to June 4th, 2021. After screeningfor inclusion, data extraction, and quality assessment by two independent reviewers, the meta-analysis was performed with R 4.1.1 software. Results are shown as proportion and weighted mean difference with 95% confi-dence intervals (95% CI). In total, 11 studies involving 1177 patients were included. Pooled data showed thatthe prevalence of BAV in TS patients was 23.7% (95% CI: 21.3% to 26.1%). No high heterogeneity was foundbetween the included studies. The current meta-analysis reveals that BAV can be detected in 23.7% of TS patientsreceiving cardiac MRI or cardiac CT. Therefore, BAV can be considered as the most common CHD in TS. Compared to TTE, cardiac MRI and cardiac CT might represent superior imaging modalities in BAV assessment ofadult TS patients.     

Transthoracic Echo-Doppler Appearances of Perforation of a Bicuspid Aortic Valve by Bacterial Endocarditis

The echo-Doppler diagnosis of aortic valve perforation has seldom been made before. We describe a combination of findings in a patient with severe aortic regurgitation following endocarditis of a bicuspid aortic valve, which suggests the presence of aortic cusp perforation: (A) discontinuity of cusp contour in parasternal long-axis view; (B) emergence of aortic regurgitant jet through a cusp rather than between the two cusps; (C) fine flutter of a cusp, but not on the free apposed edges; (D) severe aortic regurgitation without flail cusp motion, following bacterial endocarditis.     

Association of Bicuspid Aortic Valve Morphology and Aortic Root Dimensions: A Substudy of the Aortic Stenosis Progression Observation Measuring Effects of Rosuvastatin (ASTRONOMER) Study

Background: Bicuspid aortic valve (BAV) is the leading cause of aortic stenosis in patients younger than the age of 50. A classification scheme of BAVs is based upon leaflet orientation: Type I (fusion of right and left coronary cusps) and Type II (fusion of right and noncoronary cusps). The correlation between BAV leaflet orientation and aortic root pathology however remains ill defined. Objective: The objective was to describe a potential relationship between BAV leaflet morphology and aortic root measurements in the ASTRONOMER study, a multicenter study to assess the effect of rosuvastatin on the progression of AS. Methods: BAV morphology was classified as Type I or Type II orientation based on the parasternal short‐axis view. Echo measurements including left ventricular and aortic root dimensions were obtained. Results: The study population included 89 patients (56 ± 11 years; 44 males). There were 63 patients with Type I and 26 patients with Type II BAV. Baseline demographics, hemodynamics, and left heart dimensions were similar between both groups. Patients with Type I BAV had larger aortic annulus and ascending root dimensions compared to those patients with Type II BAV (P < 0.05). Conclusion: In patients with mild to moderate aortic stenosis due to a BAV, the presence of Type I valve orientation was associated with significantly greater aortic root parameters compared to Type II valve orientation. (ECHOCARDIOGRAPHY 2010;27:174‐179)     

Prognostic Significance of LGE by CMR in Aortic Stenosis Patients Undergoing Valve Replacement

Background

Prior studies have shown that late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) can detect focal fibrosis in aortic stenosis (AS), suggesting that it might predict higher mortality risk.

Objectives

This study was conducted to evaluate whether LGE-CMR can predict post-operative survival in patients with severe AS undergoing aortic valve replacement (AVR).

Methods

We prospectively evaluated survival (all-cause and cardiovascular disease related) according to LGE-CMR status in 154 consecutive AS patients (96 men; mean age: 74 ± 6 years) without a history of myocardial infarction undergoing surgical AVR and in 40 AS patients undergoing transcatheter aortic valve replacement (TAVR).

Results

LGE was present in 29% of patients undergoing surgical AVR and in 50% undergoing TAVR. During a median follow-up of 2.9 years, 21 patients undergoing surgical AVR and 20 undergoing TAVR died. In surgical AVR, the presence of LGE predicted higher post-operative mortality (odds ratio: 10.9; 95% confidence interval [CI]: 1.2 to 100.0; p = 0.02) and worse all-cause survival (73% vs. 88%; p = 0.02 by log-rank test) and cardiovascular disease related survival (85% vs. 95%; p = 0.03 by log-rank test) on 5-year Kaplan-Meier estimates of survival after surgical AVR. Multivariate Cox analysis identified the presence of LGE (hazard ratio: 2.8; 95% CI: 1.3 to 6.9; p = 0.025) and New York Heart Association functional class III/IV (hazard ratio: 3.2; 95% CI: 1.1 to 8.1; p < 0.01) as the sole independent predictors of all-cause mortality after surgical AVR. The presence of LGE also predicted higher all-cause mortality (p = 0.05) and cardiovascular disease related mortality (p = 0.03) in the subgroup of patients without angiographic coronary artery disease (n = 110) and higher cardiovascular disease related mortality in 25 patients undergoing transfemoral TAVR.

Conclusions

The presence of LGE indicating focal fibrosis or unrecognized infarct by CMR is an independent predictor of mortality in patients with AS undergoing AVR and could provide additional information in the pre-operative evaluation of risk in these patients.     

Matrix Metalloproteinase‐2 and ‐9 Levels in Patients with Dilated Ascending Aorta and Bicuspid Aortic Valve

Background: Predictors of aortic dilatation are not well‐described in patients with bicuspid aortic valve (BAV). Changes in extracellular matrix composition in the aortic wall may play an important role. Our study aimed to examine the relationship between ascending aortic dilatation and biochemical markers for collagen metabolism, such as matrix metalloproteinase‐2 (MMP‐2) and matrix metalloproteinase‐9 (MMP‐9) levels in patients with BAV. Methods: All patients underwent cardiac echocardiography using a standard protocol, and aortic measurements were made in end‐diastole. One hundred twelve BAV patients with no or mild valvular impairment were recruited and grouped according to the aortic dimensions corrected for body surface area (BSA) and age. There were 54 patients with dilated ascending aorta (Group 1) and 58 patients with nondilated ascending aorta (group 2). The plasma levels of MMP‐2 and MMP‐9 were determined by ELISA. Results: The mean ascending aorta diameter was 4.49 ± 0.49 mm in group 1 and 3.51 ± 0.46 mm in group 2 (P < 0.001). There were no significant difference in gender, BSA, presence of hypertension, diabetes mellitus, hyperlipidemia, and smoking between the 2 groups. Nevertheless, no significant difference was observed in the levels of MMP‐2 and MMP‐9 between the 2 groups. The ascending aorta diameter correlated significantly with age (r = 0.438 P < 0.001). No significant correlation was observed between plasma MMP‐2 and MMP‐9 concentration and ascending aorta diameter, respectively (r = ?0.005 P = 0.58, r = ?0.106 P = 0.07). Multivariate analysis showed that age was independent predictor of aortic dilatation (P ≤ 0.001). Conclusion: Age was an independent predictor of aortic dilatation in patients with BAV, whereas MMP‐2 and 9 levels were not relevant by aortic dilatation.     

The Role of Multimodality Cardiac Imaging for the Assessment of Sports Eligibility in Patients with Bicuspid Aortic Valve

Bicuspid aortic valve (BAV) cannot be considered an innocent finding, but it is not necessarily a life-threatening condition. Athletes with BAV should undergo a thorough staging of the valve anatomy, taking into consideration hemodynamic factors, as well as aortic diameters and looking for other associated significant cardiovascular anomalies by use of a multimodality cardiac imaging approach. Furthermore an accurate follow-up is mandatory with serial cardiological controls in those allowed to continue sports.