Research Progress on Aortic Root Aneurysms.

Aortic root aneurysms are one of the most common aortic root diseases, involving the aortic valve, aortic sinus, bilateral coronary arteries, and part of the ascending aorta. It is a life-threatening aortic disease with a high mortality rate of approximately 90%, due to aortic aneurysm rupture. Aortic valve insufficiency is one of the most common complications of aortic root aneurysms that can lead to acute left heart failure. The etiology of aortic root aneurysms is not yet completely clear and is mainly related to genetic diseases, such as Marfan syndrome and atherosclerosis. It can also occur secondary to aortic valve stenosis or a bivalve deformity. Surgery is the primary treatment for aortic root aneurysms, and aortic root replacement is a classic surgical method. However, the incidences of perioperative complications and mortality are relatively high, particularly in high-risk patients. In recent years, the anatomical structure of the aortic root has been gradually refined, and an in-depth understanding of root aneurysms has led to individualized treatment methods. Conservative drug therapy (ß-receptor blockers, angiotensin-converting enzyme inhibitors, or angiotensin receptor blockers), Bentall and modified Bentall surgeries (Button technology, Cabrol surgery, and modified Cabrol surgery), valve-sparing aortic root replacement (David and Yacoub), personalized external aortic root support, and endovascular intervention therapy have significantly improved the perioperative and long-term survival rates of patients with aortic root aneurysms. However, different treatment methods have their own advantages and disadvantages. This review aimed to summarize the current research progress and treatment of aortic root aneurysms.

Associations of Lifelong Exercise Characteristics With Valvular Function and Aortic Diameters in Patients With a Bicuspid Aortic Valve.

BACKGROUND: The potential impact of exercise on valvular function and aortic diameters in patients with a bicuspid aortic valve remains unclear. Therefore, we assessed the association between lifelong exercise characteristics, valvular dysfunction, and aortic dilatation in patients with a bicuspid aortic valve. METHODS AND RESULTS: In this cross-sectional study, exercise volume (metabolic equivalent of task minutes per week), exercise intensity, and sport type were determined from the age of 12 years to participation using a validated questionnaire. Echocardiography was used to assess aortic stenosis or aortic regurgitation and to measure diameters at the sinuses of Valsalva and ascending aorta. Aortic dilatation was defined as a Z-score ≥2. Four hundred and seven patients (42±17 years, 60% men) were included, of which 133 were sedentary (<500 metabolic equivalent of task minutes per week), 94 active (500-1000 metabolic equivalent of task minutes per week), and 180 highly active (≥1000 metabolic equivalent of task minutes per week). Moderate-to-severe aortic stenosis or aortic regurgitation was present in 23.7% and 20.0%, respectively. Sinuses of Valsalva and ascending aorta diameters were 34.8±6.6 and 36.5±8.1 mm, whereas aortic dilatation was found in 21.6% and 53.4%, respectively. Exercise volume was not associated with valve dysfunction or aortic dilatation. Vigorous intensity and mixed sports were associated with a lower prevalence of aortic stenosis (adjusted odds ratios, 0.43 [0.20-0.94] and adjusted odds ratios, 0.47 [0.23-0.95]). Exercise intensity and sport type were not associated with aortic regurgitation and aortic dilatation. CONCLUSIONS: We found no deleterious associations between lifelong exercise characteristics, valvular dysfunction, and aortic dilatation in patients with a bicuspid aortic valve. Vigorous intensity and exercise in mixed sports were associated with a lower prevalence of moderate-to-severe aortic stenosis. These observations suggest that lifelong exercise does not appear to induce adverse cardiovascular effects in patients with a bicuspid aortic valve.

Association Between Aortic Valve Calcification and Severity of Concomitant Aortic Regurgitation in Patients With Severe Aortic Stenosis.

BACKGROUND: Patients with severe aortic stenosis (AS) frequently have concomitant aortic regurgitation (AR), but the association between aortic valvular calcification (AVC) and the severity of AR remains unclear.Methods and Results: We retrospectively reviewed patients with severe AS who underwent transthoracic echocardiography and multidetector computed tomography (MDCT) within 1 month. The patients were divided into 3 groups according to the degree of concomitant AR. The association between AVC and the severity of concomitant AR was assessed in patients with severe AS. The study population consisted of 95 patients: 43 men and 52 women with a mean age of 82±7 years. Of the 95 patients with severe AS, 27 had no or trivial AR, 53 had mild AR, and 15 had moderate AR. The AVC score (AVCS) and AVC volume (AVCV) significantly increased as the severity of concomitant AR increased (P=0.014 for both), and similar findings were obtained for the AVCS and AVCV indexes (P=0.004 for both). CONCLUSIONS: The severity of AR correlated with AVCS and AVCV measured by MDCT in patients with severe AS. AVC may cause concomitant AR, leading to worsening of disease condition.

Diagnosis of membranous supravalvular aortic stenosis with severe aortic valve insufficiency.

Although the clinical manifestations of membranous supravalvular aortic stenosis (SVAS) are distinctive, its diagnosis remains challenging. Failure to initiate surgical treatment in a timely manner greatly increases the risk of sudden cardiac death. We report a case of membranous SVAS, detailing the clinical presentation and imaging manifestations.

Long-term outcome of bicuspid aortic valve disease.

AIMS: Bicuspid aortic valve (BAV) is a common congenital condition that is frequently associated with aortic stenosis (AS) and aortic regurgitation (AR), as well as aortic aneurysms, but specific outcome data are scarce. The present study sought to assess outcomes in a large cohort of consecutive patients with BAV. METHODS AND RESULTS: A total of 581 consecutive patients (median age 29 years, 157 female) with BAV were included in the study and followed prospectively in a heart valve clinic follow-up programme. The overall survival rate after 10 years was 94.5%. During follow-up, 158 patients developed an indication for surgery. Event-free survival rates were 97%, 94%, 87%, and 73% at 1, 2, 5, and 10 years, respectively. In the multivariable analysis, event rates were independently predicted by AS [subdistribution hazard ratio (SHR) 2.3 per degree of severity], AR (SHR 1.5 per degree of severity), baseline aortic dilatation ≥ 40 mm (SHR 1.9), and age (SHR 1.3) (P < 0.001). CONCLUSION: BAV disease is associated with a high rate of cardiac events, but state-of-the-art care results in good survival with low rates of infective endocarditis, aortic dissection, and sudden death. Incremental degrees of AS and regurgitation, the presence of aortic dilatation, and age are predictive of cardiac events.

Left Atrial-Veno Arterial Extracorporeal Membrane Oxygenation as a Bridge to Surgery for Endocarditis-Related Acute Severe Aortic Regurgitation.

Mechanical circulatory support for cardiogenic shock complicated by acute severe aortic regurgitation poses a unique challenge for traditional veno arterial extracorporeal membrane oxygenation (ECMO) because of rapidly rising left ventricular pressures accentuated by the increased afterload from retrograde flow in femoral cannulation. This process necessitates rapid left ventricular unloading while also allowing for adequate native left ventricular function. Herein, we describe a case of cardiogenic and septic shock secondary to methicillin-resistant Staphylococcus aureus complicated by acute severe aortic regurgitation temporized by left atrial-veno arterial (LA-VA) ECMO via the Livanova TandemHeart system. Left ventricular unloading created a window of hemodynamic stability allowing for optimization of multiorgan failure and infectious source control before surgical aortic valve replacement.

Effects of intensive blood pressure control on left ventricular hypertrophy in aortic valve disease.

BACKGROUND: Hypertension adds to the pressure overload on the left ventricle (LV) in combination with aortic valve (AV) disease, but the optimal blood pressure (BP) targets for patients with AV disease remain unclear. We tried to investigate whether intensive BP control reduces LV hypertrophy in asymptomatic patients with aortic stenosis (AS) or aortic regurgitation (AR). METHODS: A total of 128 hypertensive patients with mild to moderate AS (n = 93) or AR (n = 35) were randomly assigned to intensive therapy, targeting a systolic BP <130 mm Hg, or standard therapy, targeting a systolic BP <140 mm Hg. The primary end point was the change in LV mass from baseline to the 24-month follow-up. Secondary end points included changes in severity of AV disease, LV volumes, ejection fraction and global longitudinal strain (GLS). RESULTS: The treatment groups were generally well balanced regarding the baseline characteristics. The mean (±SD) age of the patients was 68 ± 8 years and 48% were men. The mean BP was 145 ± 12/81 ± 10 mm Hg at baseline. Medication at baseline was similar between the 2 groups. The 2 treatment strategies resulted in a rapid and sustained difference in systolic BP (P < .05). At 24-month, the mean systolic BP was 129 ± 12 mm Hg in the intensive therapy group and 135 ± 14 mm Hg in the standard therapy group. No patient died or underwent AV surgery during follow-up in either of the groups. LV mass was changed from 189.5 ± 41.3 to 185.6 ± 41.5 g in the intensive therapy group (P = .19) and from 183.8 ± 38.3 to 194.0 ± 46.4 g in the standard therapy group (P < .01). The primary end point of change in LV mass was significantly different between the intensive therapy and the standard therapy group (-3.9 ± 20.2 g vs 10.3 ± 20.4 g; P = .0007). The increase in LV mass index was also significantly greater in the standard therapy group (P = .01). No significant differences in secondary end points (changes in severity of AV disease, LV volumes, ejection fraction and GLS) were observed between the treatment groups. CONCLUSIONS: Among hypertensive patients with AV disease, intensive hypertensive therapy resulted in a significant reduction in LV hypertrophy, although progression of AV disease was similar between the treatment groups. CLINICAL TRIAL REGISTRATION: http://ClinicalTrials.gov (Number NCT03666351).

Prognostic impact of the E/e' ratio in patients with chronic severe aortic regurgitation undergoing aortic valve replacement.

OBJECTIVES: The study objective was to evaluate the clinical implication of left ventricular diastolic dysfunction in patients with chronic severe aortic regurgitation undergoing aortic valve replacement. METHODS: We reviewed the medical records of 323 patients (age, 56.3 ± 14.1 years; 111 female) who underwent aortic valve replacement for chronic severe aortic regurgitation between 2005 and 2019. Left ventricular diastolic dysfunction was assessed by the ratio of peak left ventricular inflow velocity over mitral annular velocity (E/e'). The study end point was the composite of death and heart failure requiring hospital admission. RESULTS: The E/e' ratio was significantly correlated with age, left atrial dimension, left ventricular end-diastolic volume, mitral regurgitation grade, and tricuspid regurgitation grade (all P < .001). During follow-up (1748.3 patient-years), death and heart failure occurred in 36 patients (2.06/patient-year) and 9 patients (0.53/patient-year), respectively. In multivariable analysis, E/e' ratio (per 5 increment, hazard ratio, 1.32; 95% confidence interval, 1.02-1.71; P = .03), age (hazard ratio, 1.06; 95% confidence interval, 1.03-1.10; P < .001), and left ventricular ejection fraction (hazard ratio, 0.94; 95% confidence interval, 0.90-0.98; P = .002) were independent predictors of death and heart failure. The 5-year heart failure-free survival was 94.9% ± 1.7% in patients with E/e' less than 15% and 84.2% ± 4.2% in patients with E/e' 15 or greater (P < .001). CONCLUSIONS: The E/e' ratio was significantly associated with adverse outcomes in patients with chronic severe aortic regurgitation undergoing aortic valve replacement and may be useful as a prognostic marker in such patients.

Infective Endocarditis Complicated With a Large Left Coronary Cusp Aneurysm: A Condition With Undefined Natural History and Treatment.

The natural history and treatment of an aortic cusp aneurysm with or without rupture because of native aortic valve infective endocarditis (NAV-IE) have not been well defined. This may explain why current guidelines for the management of valvular heart disease do not include this complication as an indication for surgical aortic valve replacement or repair or transcatheter aortic valve replacement (TAVR). We describe herein the first case of a man aged 76 years with multiple co-morbidities with a NAV-IE associated large left coronary cusp aneurysm with subsequent rupture and consequent severe aortic regurgitation and heart failure for which he underwent an off-label successful TAVR. This patient's scenario suggests that a cusp aneurysm because of NAV-IE poses a high risk for subsequent rupture, severe aortic regurgitation, and heart failure. In conclusion, TAVR may be a reasonable alternative to high-risk surgical aortic valve replacement in patients with NAV-IE associated cusp aneurysms with or without but impending rupture.

Ventricular septal defect associated with aortic regurgitation and ascending aortic aneurysm: a case report.

INTRODUCTION: Ventricular septal defect (VSD) is one of the most common congenital cardiac anomalies. Patients with perimembranous VSD may have aortic regurgitation (AR) secondary to prolapse of the aortic cusp. CASE PRESENTATION: We present a case of 23-year-old White man with VSD, AR and ascending aortic aneurysm. The patient presented to outpatient clinic with weakness and gradual worsening shortness of breath for the past 5 years. Clinical examination revealed regular heart rhythm and loud continuous systolic-diastolic murmur (Lewin's grade 6/6), heard all over the precordium, associated with a palpable thrill. The ECG showed right axis deviation, fractionated QRS in V1 and signs of biventricular hypertrophy. The chest X-ray showed cardiomegaly. Transthoracic and transesophageal echocardiograms showed a perimembranous VSD with moderate restrictive shunt (Qp/Qs = 1.6), aortic regurgitation (AR), and ascending aortic aneurysm. Other clinical and laboratory findings were within normal limits. CONCLUSIONS: Perimembranous VSD, may be associated with aortic regurgitation and ascending aortic aneurysm as secondary phenomenon if it is not early diagnosed and successfully treated.

Severe aortic insufficiency-induced cardiogenic shock treated with left atrial VA-ECMO and emergent valve-in-valve TAVR.

Conventional venoarterial extracorporeal membrane oxygenation (VA-ECMO) places a functional afterload burden on the left ventricle. In the setting of acute severe aortic insufficiency-induced cardiogenic shock, the utility of VA-ECMO in combination with a failing valve may result in catastrophic haemodynamic consequences. This challenge is compounded when the culprit is a failing surgical bioprosthetic valve. We present a case of severe rapid-onset bioprosthetic aortic insufficiency-induced cardiogenic shock successfully resuscitated with left atrial VA-ECMO promptly followed by emergent percutaneous valve-in-valve transaortic valve replacement. We discuss the logistics, implications, and associated haemodynamic manifestations in utilizing this strategy for such disease processes.

Association of Mild-to-Moderate Aortic Regurgitation With Outcomes in Heart Failure With Preserved Ejection Fraction.

OBJECTIVE: To assess aortic regurgitation (AR) prevalence, its hemodynamic effect, and long-term prognostic implications in patients admitted with de novo or worsened heart failure with preserved ejection fraction (HFpEF). METHODS: Consecutive patients hospitalized with de novo or worsened HFpEF between 2014 and 2020 were enrolled. Patients with more than moderate aortic and/or mitral valve disease were excluded. Based on the presence and degree of AR, patients were divided into those without AR, those with mild, and those with moderate AR. Data on cardiovascular death, heart failure (HF) rehospitalization, and their composite (major adverse cardiovascular events) were collected. RESULTS: The final study population consisted of 458 HFpEF patients: 156 (34.1%) with mild-AR, 153 (33.4%) with moderate-AR, and the remaining 149 (32.5%) with no AR. Mild-to-moderate AR patients were older, with larger left atrium-left ventricle (LV) volumes, greater LV mass index, higher filling pressure, and prevalence of diastolic dysfunction compared with the no-AR group (all P<.05). During 5-year follow-up, 113 patients died of cardiovascular causes, 124 patients were rehospitalized for HF, whereas 196 experienced the composite endpoint. Mild-to-moderate AR was identified as an independent predictor of all-cause death (HR, 1.62; 95% CI, 1.14 to 1.58; P=.04) and major adverse cardiovascular event occurrence (HR, 1.48; 95% CI, 1.05 to 2.09; P=.02). A total of 126 (35.5%) of 355 patients showed progression of AR at follow-up echocardiography. CONCLUSION: Mild-to-moderate AR is common among patients hospitalized for HFpEF. It is associated with adverse LV remodeling and worse long-term outcomes. These findings warrant further prospective studies addressing the importance of AR in prognostic stratification and exploring therapeutic strategies to mitigate its hemodynamic effect on HF.

Pancycle aortic incompetence and double alternans in pediatric heart failure.

A 12-year-old boy presented with bicuspid aortic valve, severe aortic regurgitation, and dilated dysfunctional left ventricle in heart failure. He underwent aortic valve replacement with a 23 mm TTK Chitra heart valve prosthesis (tilting disk). He was gradually weaned off milrinone and noradrenaline in the intensive care. Echocardiography showed severe left ventricular dysfunction with an ejection fraction of 24%. The radial pulse was regular and of normal volume but exactly half that of the heart rate. Evaluation of the rhythm and echocardiography revealed an interesting hemodynamic phenomenon with double alternans.

Left-sided valvular heart disease and survival in out-of-hospital cardiac arrest: a nationwide registry-based study.

Survival in left-sided valvular heart disease (VHD; aortic stenosis [AS], aortic regurgitation [AR], mitral stenosis [MS], mitral regurgitation [MR]) in out-of-hospital cardiac arrest (OHCA) is unknown. We studied all cases of OHCA in the Swedish Registry for Cardiopulmonary Resuscitation. All degrees of VHD, diagnosed prior to OHCA, were included. Association between VHD and survival was studied using logistic regression, gradient boosting and Cox regression. We studied time to cardiac arrest, comorbidities, survival, and cerebral performance category (CPC) score. We included 55,615 patients; 1948 with AS (3,5%), 384 AR (0,7%), 17 MS (0,03%), and 704 with MR (1,3%). Patients with MS were not described due to low case number. Time from VHD diagnosis to cardiac arrest was 3.7 years in AS, 4.5 years in AR and 4.1 years in MR. ROSC occurred in 28% with AS, 33% with AR, 36% with MR and 35% without VHD. Survival at 30 days was 5.2%, 10.4%, 9.2%, 11.4% in AS, AR, MR and without VHD, respectively. There were no survivors in people with AS presenting with asystole or PEA. CPC scores did not differ in those with VHD compared with no VHD. Odds ratio (OR) for MR and AR showed no difference in survival, while AS displayed OR 0.58 (95% CI 0.46-0.72), vs no VHD. AS is associated with halved survival in OHCA, while AR and MR do not affect survival. Survivors with AS have neurological outcomes comparable to patients without VHD.

Long-term outcomes of percutaneous closure of ventricular septal defects in children using different devices: A single centre experience from Egypt.

BACKGROUND: The feasibility of percutaneous closure ventricular septal defects (VSD) in children has been previously proven. However, data on long-term outcomes are limited. We aim to evaluate the long-term outcome of our experience with percutaneous closure of VSD using various occluders. METHODS: Retrospective institutional analysis of children who underwent transcatheter closure of perimembranous and muscular VSDs between September 2012 and February 2020. Patient demographics, procedural, and long-term follow-up data were comprehensively analyzed. Patients who lost to follow-up within two years post-procedure were excluded. RESULTS: We identified 75 patients (54.7% males) with a median of 66 months (IQR, 46-96). The closure success rate at one year was 95.7%. Complete heart block was detected in two patients early post-procedure and resolved with steroids. The VSDs were perimembranous (52%), muscular (33.33%), and residual (14.67%). Implanted devices were Pfm Nit-Occlud LeˆVSD Coil (42.7%), HyperionTM VSD Muscular Occluder (28%), Amplatzer VSD muscular occluder (10.7%), Amplatzer Duct Occluder (14.7%), Occlutech Muscular VSD Occluder (2.7%), and Amplatzer Duct Occluder II (1.3%). No new arrhythmia or valve regurgitation was detected after two years post-procedure. Persisted complications on long-term follow-up included: residual shunting in 3(4%), mild tricuspid regurgitation in 2(2.7%), and aortic regurgitation in 2(2.7%), with one immediate post-catheterization mild aortic regurgitation worsened during follow-up, requiring surgical repair of VSD three years after device implantation. No deaths were reported. CONCLUSION: Long-term outcomes of pediatric transcatheter VSD closure using different devices are satisfactory. Post-procedural adverse events are limited, but long-term surveillance is necessary to monitor their progression.

Gender Effects on Left Ventricular Responses and Survival in Patients with Severe Aortic Regurgitation: Results from a Cohort of 756 Patients with up to 22 Years of Follow-Up.

OBJECTIVES: We sought to evaluate the effect of gender on biology, therapeutic decisions, and survival in patients with severe aortic regurgitation (AR). BACKGROUND: Gender affects adaptive response to the presence of valvular heart diseases and therapeutic decisions. The impact of these on survival in severe AR patients is not known. METHODS: This observational study was compiled from our echocardiographic database which was screened (1993-2007) for patients with severe AR. Detailed chart reviews were performed. Mortality data were obtained from the Social Security Death Index and analyzed as a function of gender. RESULTS: Of the 756 patients with severe AR, 308 (41%) were women. Over a follow-up of up to 22 years, there were 434 deaths. Women compared to men were older (64 ± 18 vs. 59 ± 17 years, p = 0.0002). Women also had smaller left ventricular (LV) end diastolic dimension (5.2 ± 1.1 vs. 6.0 ± 1.0 cm, p < 0.0001), higher EF (56% ± 17% vs. 52% ± 18%, p = 0.003), higher prevalence of diabetes mellitus (18% vs. 11%, p = 0.006), and higher prevalence of ≥2+ mitral regurgitation (52% vs. 40%, p = 0.0008) despite a smaller LV size. Women were also less likely to receive aortic valve replacement (AVR) (24% vs. 48%, p < 0.0001) compared to men and had a lower survival on univariate analysis (p = 0.001). However, after adjusting for group differences including AVR rates, gender was not an independent predictor of survival. However, the survival benefit associated with AVR was similar in both women and men. CONCLUSIONS: This study strongly suggests that female gender is associated with different biological responses to AR compared to men. There is also a lower AVR rate in women, but women derive similar survival benefit as men with AVR. Gender does not seem to affect survival in an independent fashion in patients with severe AR after adjusting for group differences and AVR rates.