Mitral regurgitation after previous aortic valve surgery for bicuspid aortic valve insufficiency

Girdauskas, Evaldas; Disha, Kushtrim; Espinoza, Andres; Misfeld, Martín; Reichenspurner, Hermann; Borger, Michael A.; Kuntze, Thomas

doi:10.23736/s0021-9509.16.09311-3

Cited by 5 publications

(5 citation statements)

References 9 publications

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“…Among patients with AS, MR grade has been shown to improve overtime following both surgical [18] and transcatheter [19] AVR, while TR worsening has been observed in up to 17% of cases post-procedure, inflicting lower survival [20][21][22][23]. In patients with AR, mild MR has deteriorated in 4% of patients after SAVR according to one report [24] and moderate or severe MR has occurred in 9.4% according to another [25]. Notably, the former study spanned 3.2 years of follow-up and found a direct correlation between the follow-up time and MR progression, whereas the latter, representing 10 ± 4 years of follow-up, analyzed 97 patients, all with bicuspid AV.…”

Section: Discussionmentioning

confidence: 97%

Progression of Non-Significant Mitral and Tricuspid Regurgitation after Surgical Aortic Valve Replacement for Aortic Regurgitation

Kazum,

Vaturi,

Yedidya

et al. 2023

JCM

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Little is known about the natural history of non-significant mitral and tricuspid regurgitation (MR and TR) following surgical aortic valve replacement (SAVR) for aortic regurgitation (AR). We retrospectively analyzed 184 patients (median age 64 (IQR, 55–74) years, 76.6% males) who underwent SAVR for AR. Subjects with significant non-aortic valvulopathies, prior/concomitant valvular interventions, or congenital heart disease were excluded. The cohort was evaluated for MR/TR progression and, based on the latter’s occurrence, for echocardiographic and clinical indices of heart failure and mortality. By 5.8 (IQR, 2.8–11.0) years post-intervention, moderate or severe MR occurred in 20 (10.9%) patients, moderate or severe TR in 25 (13.5%), and either of the two in 36 (19.6%). Patients who developed moderate or severe MR/TR displayed greater biventricular disfunction and functional limitation and were less likely to be alive at 7.0 (IQR, 3.4–12.1) years compared to those who did not (47.2 vs. 79.7%, p < 0.001). The emergence of significant MR/TR was associated with preoperative atrial fibrillation/flutter, symptomatic heart failure, and above-mild MR/TR as well as concomitant composite graft use, but not with baseline echocardiographic measures of biventricular function and dimensions, aortic valve morphology, or procedural aspects. In conclusion, among patients undergoing SAVR for AR, significant MR/TR developed in one fifth by six years, correlated with more adverse course, and was anticipated by baseline clinical and echocardiographic variables.

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Section: Discussionmentioning

confidence: 97%

Progression of Non-Significant Mitral and Tricuspid Regurgitation after Surgical Aortic Valve Replacement for Aortic Regurgitation

Kazum,

Vaturi,

Yedidya

et al. 2023

JCM

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“…[16][17][18] It has been established that "root phenotype," a special type of aortopathy predominantly found in young males and characterized by prominent aortic root dilation over the tubular portion of ascending aorta, harbors an elevated genetic risk and correlates closely with additional cardiac anomaly such as mitral valve prolapse. 8,19) For patients with TAAD, it is recommended that genetic risk stratification based on the panel of at least 30 genes be performed to provide appropriate and personalized management, as prophylactic surgical intervention is suggested under an aortic dimension of 45 mm among patients carrying pathogenic TGFBR1, TGFBR2, and SMAD3 variants and 50 mm among patients carrying FBN1, TGFB2, COL3A1, ACTA2, MYH11, MYLK, and PRKG1 variants. 7,16) Under these circumstances, genetic testing for FLNA loss-of-function variants could play a pivotal role in the personalized management of SVA and contribute to the proposal of a diseasetailored screen panel.…”

Section: Discussionmentioning

confidence: 99%

Sinus of Valsalva Aneurysm in Females

Wang

et al. 2022

Int. Heart J.

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Sinus of Valsalva aneurysm (SVA) is a rare cardiovascular disease with male predominance. Recently, an association with aortic aneurysm and SVA has been revealed in periventricular nodular heterotopia patients with loss-of-function Filamin A (FLNA) mutations, which were located on chromosome X and almost exclusively affect females.Among patients hospitalized for aortic surgery with aortic root diameter over 4.0 cm, next-generation sequencing was performed to investigate 30 candidate genes related to inherited aortic aneurysm syndromes and familial thoracic aortic aneurysm and dissection. The present report reviewed an electronic case database and identified two female cases of unruptured SVA with heterozygous FLNA truncating mutations.Case 1 displaying a rare SVA phenotype involving left and noncoronary sinus harbored a nonsense variant p.Tyr1720Ter/c.5160C > G. Case 2 displayed right and noncoronary SVA with predominantly enlarged right coronary sinus, posterior mitral valve prolapse, and harbored a frameshift variant p.Val1724fs*68/c.5171_5172 delTG. Both novel mutations resulted in the premature termination of filamin A with the loss of functional Rod 2 and dimerization region.The present report raised the possibility of the presence of a cardiovascular onset form in the spectrum of FLNA hereditary diseases. The association between SVA and loss-of-function FLNA mutations indicates a unique etiology and pathogenesis among female patients, which requires further investigation to establish the linkage between FLNA variants and a wide spectrum of phenotypes.

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“…BAV-AI is an independent predictor for adverse aortic events even after AVR, with patients showing a 10-fold higher risk of dissection than BAV-AS patients post AVR (2.8% pooled estimate of dissection rate vs 0.2%), with increasing risk seen with smaller aortic diameters in BAV-AI patients [ 42 ]. Despite these findings, both groups demonstrated similar long-term survival [ 51 ], likely due to the overall low numbers of observed adverse aortic events.…”

Section: Clinical Patterns Of Taa Depend On Valvular Dysfunctionmentioning

confidence: 99%

“…As outlined in this review, the presence of a BAV is associated with serious long-term health risks including progressive aortic valve disease and thoracic aortopathy, with approximately 30–40% of BAV patients undergoing TAA repair [ 14 , 99 ]. When compared to TAV patients, BAV patients (with or without aneurysms) are at increased risk of future aortic dilation and dissection [ 33 , 70 ], and display faster rates of aneurysmal growth [ 20 , 51 ]. These associations are so strong that, even after aortic valve replacement, BAV patients still require lifelong surveillance of the aorta [ 20 , 51 ].…”

Section: Future Research Perspectivementioning

confidence: 99%

“…When compared to TAV patients, BAV patients (with or without aneurysms) are at increased risk of future aortic dilation and dissection [ 33 , 70 ], and display faster rates of aneurysmal growth [ 20 , 51 ]. These associations are so strong that, even after aortic valve replacement, BAV patients still require lifelong surveillance of the aorta [ 20 , 51 ].…”

Section: Future Research Perspectivementioning

confidence: 99%

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Implications of Bicuspid Aortic Valve Disease and Aortic Stenosis/Insufficiency as Risk Factors for Thoracic Aortic Aneurysm

Jabagi¹,

Levine²,

Gharibeh³

et al. 2023

Rev. Cardiovasc. Med.

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Bicuspid Aortic Valves (BAV) are associated with an increased incidence of thoracic aortic aneurysms (TAA). TAA are a common aortic pathology characterized by enlargement of the aortic root and/or ascending aorta, and may become life threatening when left untreated. Typically occurring as the sole pathology in a patient, TAA are largely asymptomatic. However, in some instances, they are accompanied by aortic valve (AV) diseases: either congenital BAV or acquired in the form of Aortic Insufficiency (AI) or aortic stenosis (AS). When TAA are associated with aortic valve disease, determining an accurate and predictable prognosis becomes especially challenging. Patients with AV disease and concomitant TAA lack a widely accepted diagnostic approach, one that integrates our knowledge on aortic valve pathophysiology and encompasses multi-modality imaging approaches. This review summarizes the most recent scientific knowledge regarding the association between AV diseases (BAV, AI, AS) and ascending aortopathies (dilatation, aneurysm, and dissection). We aimed to pinpoint the gaps in monitoring practices and prediction of disease progression in TAA patients with concomitant AV disease. We propose that a morphological and functional analysis of the AV with multi-modality imaging should be included in aortic surveillance programs. This strategy would allow for improved risk stratification of these patients, and possibly new AV phenotypic-specific guidelines with more vigilant surveillance and earlier prophylactic surgery to improve patient outcomes.

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Mitral regurgitation after previous aortic valve surgery for bicuspid aortic valve insufficiency

Cited by 5 publications

References 9 publications

Progression of Non-Significant Mitral and Tricuspid Regurgitation after Surgical Aortic Valve Replacement for Aortic Regurgitation

Progression of Non-Significant Mitral and Tricuspid Regurgitation after Surgical Aortic Valve Replacement for Aortic Regurgitation

Sinus of Valsalva Aneurysm in Females

Implications of Bicuspid Aortic Valve Disease and Aortic Stenosis/Insufficiency as Risk Factors for Thoracic Aortic Aneurysm

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