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Anomalous right coronary artery origin from pulmonary artery associated with aortic stenosis, mitral regurgitation, and Vieussens' arterial ring: a very rare association and case.

ABSTRACT: We report a very rare case of anomalous origin of the right coronary artery from the pulmonary artery associated with severe aortic stenosis, severe mitral regurgitation, and Vieussens' arterial ring (VAR). Diagnosis was made accidentally during preoperative coronary angiography and confirmed by multidetector computed tomography. Surgery was performed with aortic valve replacement, mitral valve annuloplasty, right coronary artery reimplantation into the ascending aorta, and VAR resection. <Learning objectives: The association of severe aortic stenosis, severe mitral regurgitation, anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA), and Vieussens' arterial ring (VAR) is extremely rare. Multidetector computed tomography can confirm the diagnosis of ARCAPA and VAR, exclude a bicuspid aortic valve, and provide comprehensive preoperative anatomic information. Surgical correction with aortic valve replacement, mitral valve annuloplasty, right coronary artery reimplantation into the ascending aorta, and VAR resection should be preferred in patients with deemed acceptable surgical risk. A conservative treatment limited to transcatheter aortic-valve replacement might be considered in patients with a very high surgical risk.>.

SUBMITTER: Musuraca G

PROVIDER: S-EPMC7817905 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature

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Anomalous right coronary artery origin from pulmonary artery associated with aortic stenosis, mitral regurgitation, and Vieussens' arterial ring: a very rare association and case.

Musuraca Gerardo G Imperadore Ferdinando F Terraneo Clotilde C Agostoni Pierfrancesco P Tezza Michela M Fornasa Francesca F Albiero Remo R

Journal of cardiology cases 20201108 2

We report a very rare case of anomalous origin of the right coronary artery from the pulmonary artery associated with severe aortic stenosis, severe mitral regurgitation, and Vieussens' arterial ring (VAR). Diagnosis was made accidentally during preoperative coronary angiography and confirmed by multidetector computed tomography. Surgery was performed with aortic valve replacement, mitral valve annuloplasty, right coronary artery reimplantation into the ascending aorta, and VAR resection. <<b>Le ...[more]

PMID: 33520033

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Project description:AimsConcurrent mitral regurgitation (MR) influences treatment considerations in patients with severe aortic stenosis (sAS). Limited information exists regarding haemodynamic effects of sAS on MR severity and outcome of these patients. We assessed the impact of aortic valve replacement (AVR) on MR according to mechanism in patients with sAS and MR.Methods and resultsIn patients with sAS who received surgical or transcatheter AVR from 2008 to 2017, those with effective mitral regurgitant orifice area (ERO) ≥ 10 mm2 prior to AVR were evaluated. The change in MR after AVR was considered significant when there was at least one grade difference. We compared the all-cause mortality of patients with and without improvement in MR. Of 234 patients with sAS and MR (age 80 ± 9 years, 52% male, ERO 19 ± 7 mm2 ), organic and functional MR were present in 166 (71%) and 68 (29%), respectively. MR improved in 136 (58%); improvement occurred with similar frequency in organic versus functional MR (59% and 57%, P = 0.88). Associated determinants were absence of atrial fibrillation in organic MR [odds ratio (OR) 2.09, 95% confidence interval (CI) 1.00-4.37; P = 0.049] and indexed aortic valve area (iAVA) ≤ 0.40 cm2 in functional MR (OR 3.28, 95% CI 1.13-9.47; P = 0.028). In the overall cohort, mitral annulus diameter < 3 cm (OR 1.74, 95% CI 1.02-2.97; P = 0.041) and QRS duration < 115 ms (OR 1.73, 95% CI 1.00-2.98; P = 0.049) were independently associated with improvement in MR. During median follow-up of 3.5 years, lack of improvement in MR was not associated with higher mortality in the overall cohort of patients with ERO ≥ 20 mm2 [adjusted hazard ratio (HR) 1.71, 95% CI 0.90-3.27; P = 0.10, adjusted for age, New York Heart Association III or IV, diabetes, and creatinine ≥ 2.0 mg/dL]. Lack of improvement in organic MR was associated with higher mortality (adjusted HR 3.36, 95% CI 1.40-8.05; P < 0.01). In patients with functional MR, change in MR was not associated with mortality (HR 1.24, 95% CI 0.44-3.47; P = 0.68).ConclusionsIn nearly 60% of patients with sAS and MR, MR improved after AVR, even in the majority of patients with organic MR. Absence of atrial fibrillation in organic MR, iAVA ≤ 0.40 cm2 in functional MR, and mitral annulus diameter < 3 cm and QRS duration < 115 ms in the overall population were associated with MR improvement. Post-operative improvement in organic MR was associated with better survival.

Project description:AimThe objective of this study was to investigate the prognostic importance of right ventricular dysfunction (RVD) and tricuspid regurgitation (TR) in patients with moderate-severe functional mitral regurgitation (FMR) receiving MitraClip procedure. RVD and TR grade are associated with cardiovascular mortality in the general population and other cardiovascular diseases. However, there are limited data from observational studies on the prognostic significance of RVD and TR in FMR receiving MitraClip procedure.Methods and resultsA systemic review and meta-analysis were performed using MEDLINE, Scopus, and Embase to assess the prognostic value of RVD and TR grade for mortality in patients with functional mitral regurgitation (FMR) receiving MitraClip procedure. Hazard ratios were extracted from multivariate models reporting on the association of RVD and TR with mortality and described as pooled estimates with 95% confidence intervals. A total of eight non-randomized studies met the inclusion criteria with seven studies having at least 12 months follow-up with a mean follow-up of 20.9 months. Among the aforementioned studies, a total of 1112 patients (71.5% being male) were eligible for being included in our meta-analysis with an overall mortality rate of 28.4% (n = 316). Of the enrolled patients, RVD was present in 46.1% and moderate-severe TR in 29.2%. RVD was significantly associated with mortality compared to normal RV function (HR, 1.79, 95% CI, 1.39-2.31, P < 0.001, I2 = 0). Patients with moderate-severe TR showed increased risk of mortality compared with those in the none-mild TR group (HR, 1.61. 95% CI, 1.11-2.33, P = 0.01, I2 = 14).ConclusionsThis meta-analysis demonstrates the prognostic importance of RVD and TR grade in predicting all-cause mortality in patients with significant FMR. RV function and TR parameters may therefore be useful in the risk stratification of patients with significant FMR undergoing MitraClip procedure.

Dataset Information

Anomalous right coronary artery origin from pulmonary artery associated with aortic stenosis, mitral regurgitation, and Vieussens' arterial ring: a very rare association and case.

Publications

Anomalous right coronary artery origin from pulmonary artery associated with aortic stenosis, mitral regurgitation, and Vieussens' arterial ring: a very rare association and case.

Similar Datasets

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