Journal of Heart Valve Disease最新文献

The case is reported of a 62-year-old man with severe aortic regurgitation that was related to failed prior valve-sparing ascending aortic aneurysm repair, and who was successfully treated with a Perceval Sutureless valve.

Background: Aortic valve prolapse (AVP) and aortic regurgitation (AR) can develop in a subset of patients with ventricular septal defect (VSD). The incidence and progression of AVP and AR with VSD at long-term follow up was evaluated.

Methods: The records of 2,275 patients with isolated VSD who had been diagnosed using echocardiography at the present authors' institution between 1988 and 2014 were reviewed.

Results: AVP was detected in 178 patients (7.8%), using echocardiography. Of 178 patients with AVP, AR was detected in 124 (AR incidence 5.4%). A total of 142 patients was followed medically during a median of 10 years after AVP had appeared. Initially, no AR was noted in 66 of these patients, trivial AR in 41, mild AR in 30, moderate in three, and severe in two. Trivial AR developed in 20 and mild in eight of 66 patients who had no AR. In 18 of 61 patients (29.5%), trivial AR progressed to mild during a median of 3.6 years, and in five of 18 patients (27.7%) mild AR progressed to moderate during a median of 2.3 years. Postoperatively, AR improved in 17 patients, remained unchanged in 12, and worsened in four at between two months and 16.5 years of follow up (median 6.6 years).

Conclusions: Frequent (six-month) echocardiographic evaluation in patients with perimembraneous or muscular outlet VSD after AVP and AR development may be useful. In addition, surgical intervention in patients with perimembraneous or muscular outlet VSD, AVP and mild AR may prevent the worsening of AR.

Background: The MitraClip® system is a percutaneous treatment for mitral regurgitation (MR) that has shown promising results in patients who are inoperable or at high risk for mitral surgery. Data on the efficacy of the system over optimal medical therapy, above all in patients with functional MR, are scarce. The study aim was to assess the effect of MitraClip on the survival of patients with moderate/severe or severe MR compared to medical therapy, using meta-analytical techniques.

Methods: Independently, reviewers searched electronically for relevant articles based on predefined criteria and end-points. Only articles with a comparison between MitraClip and conservative therapy were included. Standard meta-analysis techniques were used. The primary outcomes were 30-day and one-year mortalities.

Results: Five observational reports were included that enrolled a total of 1,271 patients: 720 patients underwent percutaneous mitral valve repair (PMVR) with the MitraClip device, and 551 were managed conservatively. A total of 49 all-cause mortality events was reported at 30 days: 3.05% (22/720) in the PMVR arm, and 4.90% (27/510) in the conservative group, with no significant differences in all-cause mortality (OR 0.64; 95% CI 0.36-1.14). A total of 269 all-cause mortality events at one year was reported: 15.14% (109/720) in the PMVR arm, and 29.04% (160/551) in the conservative group. A significant difference favoring PMVR with the MitraClip system over medical therapy alone was observed (OR 0.44; 95% CI 0.30-0.64, p <0.0001). Neither significance between study heterogeneity (p = 0.18) nor publication bias was detected (p = 0.3).

Conclusions: PMVR with the MitraClip system may be associated with an improvement in one-year survival compared to stand-alone medical management.

Malignant primary tumors of the heart are among the most unusual forms of cancer. Cardiac malignancy accounts for about 20% of all primary cardiac tumors, with most arising from the atria and less frequently the ventricles; very unusual locations include the great vessels and the cardiac valves. A rare case is presented of a young female that arrived at the authors' emergency room in pulmonary edema and circulatory collapse secondary to a true tumor of the mitral leaflets that caused severe mitral valve stenosis. On pathological examination this proved to be a mitral leaflet angiosarcoma.

A 36-year-old pregnant woman with a history of rheumatic heart disease and prior aortic valve replacement and mitral valve repair presented to an outside hospital with severe aortic stenosis. The patient had a cardiac arrest upon labor induction and underwent a transcatheter aortic valve replacement (TAVR), which dislodged two days later. Five months later, the patient underwent removal of the dislodged TAVR and a Ross procedure at the authors' institution. The patient was stable upon discharge, with minimal aortic and pulmonary regurgitation. To the authors' knowledge, the present report is the first of the Ross procedure being used under such circumstances. It is suggested that caution be taken when using bioprosthetic and transcatheter aortic valves in young patients, and primary use of the Ross procedure is encouraged at experienced centers.

Background: Myxomatous mitral valve disease (MMVD) is one of the most prevalent valvular heart diseases, while back pain, neck pain and upper-extremity numbness are some of the most common complaints in outpatient settings. Decreased thoracic kyphosis (straight back) is a known cause of hastening back or neck problems, radiculopathy, or even myelopathy. The study aim was to examine the relationship between MMVD, straight back, and the need for cervical fusion.

Methods: In this single-center retrospective study, patients who underwent mitral valve repair or replacement due to MMVD (cases) based on age, gender and body mass index (BMI), were matched with patients who underwent coronary artery bypass grafting (CABG) surgery and had no history of mitral valve disease (controls). The number of patients in each group who required cervical fusion was also noted. Patients included were aged <65 years at the time of surgery, which was performed between January 2014 and December 2015. Thoracic kyphosis curvature was measured from the length of a perpendicular line drawn from the middle of the anterior border of T8 vertebral body to a vertical line connecting anterior superior T4 and anterior inferior T12 on a lateral chest radiograph (AP distance). An AP distance <12 mm was defined as straight back. A Wilcoxon rank-sum test was used to compare the AP distance between cases and controls, and a chi-square test was used to compare the prevalence of straight back in the two groups.

Results: The study cohort included 75 patients in the MMVD group and 225 patients in the CABG group. Straight back was present in 27% of the MMVD group versus only 6.7% of the CABG group (p <0.0001) (Odds ratio 5.1; 95% CI 2.4-10.6). The mean AP distance in the MMVD group was 19.8 ± 8 mm, compared to 22.4 ± 6 mm for CABG cohort (p = 0.017). Of those patients with MMVD who had straight back, 10% required cervical fusion, compared to none in the CABG group.

Conclusions: MMVD is associated with straight back and a relatively high requirement for cervical fusion. Patients with MMVD should be screened for straight back and, if the condition is identified, should consider preventive measures to obviate the need for cervical fusion.

Background: A genetic component to familial mitral valve prolapse (MVP) has been proposed for decades. Despite this, very few genes have been linked to MVP. Herein is described a four-generation pedigree with numerous individuals affected with severe MVP, some at strikingly young ages.

Methods: A detailed clinical evaluation performed on all affected family members demonstrated a spectrum of MVP morphologies and associated phenotypes.

Results: Linkage analysis failed to identify strong candidate loci, but revealed significant regions, which were investigated further using whole-exome sequencing of one of the severely affected family members. Whole-exome sequencing identified variants in this individual that fell within linkage analysis peak regions, but none was an obvious pathogenic candidate. Follow up segregation analysis of all exome-identified variants was performed to genotype other affected and unaffected individuals in the family, but no variants emerged as clear pathogenic candidates. Two notable variants of uncertain significance in candidate genes were identified: p.I1013S in PTPRJ at 11p11.2 and FLYWCH1 p.R540Q at 16p13.3. Neither gene has been previously linked to MVP in humans, although PTPRJ mutant mice display defects in endocardial cushions, which give rise to the cardiac valves. PTPRJ and FLYWCH1 expression was detected in adult human mitral valve cells, and in-silico analysis of these variants suggests they may be deleterious. However, neither variant segregated completely with all of the affected individuals in the family, particularly when 'affected' was broadly defined.

Conclusions: While a contributory role for PTPRJ and FLYWCH1 in this family cannot be excluded, the study results underscored the difficulties involved in uncovering the genomic contribution to MVP, even in apparently Mendelian families.

Background: Segmental analysis of diseased mitral valves is important to predict a successful surgical valve repair. An assessment was made of the comparative accuracy of intraoperative three-dimensional (3D) and two-dimensional (2D) transesophageal echocardiography (TEE) in the evaluation of mitral valve lesions when compared with intraoperative surgical segmental analysis.

Methods: A total of 42 consecutive patients (12 females, 30 males; mean age 70.5 ± 14 years) with severe mitral valve regurgitation due to degenerative disease and who underwent mitral valve repair was enrolled in the study. Complete 2D- and 3D-TEE were performed before surgery. The findings obtained using the different echocardiographic techniques were compared with intraoperative segmental analysis performed by a single operator who was blinded to the 2D- and 3D-TEE findings until the end of the inspection. The sensitivity and specificity of echocardiographic evaluations of involved scallops were compared with surgical inspection.

Results: 3D-TEE allowed an accurate identification of all mitral lesions. Thirty-three patients had simple lesions at 3D-TEE and underwent a simple surgical procedure, while nine patients had complex lesions; in these latter cases complex surgical procedures were performed. 3D-TEE showed more sensitivity than 2D-TEE in the analysis of the anterior leaflet (A), in particular for A3 lesion (100% versus 25%, p <0.001) and for complex lesion (100% versus 33.3%, p <0.009).

Conclusions: 3D-TEE allowed a more accurate identification of mitral valve lesions compared with 2D-TEE. The greatest accuracy was achieved for analysis of the anterior leaflet. 3D-TEE should be regarded as an important adjunct to standard 2D-TEE in decisions regarding mitral valve repair.

Mitral annuloplasty ring thrombosis is an extremely infrequent pathology, for which no evidence-based antithrombotic management has yet been described. Herein is presented a case of heterozygous Factor V Leiden (FVL) and hyperhomocysteinemia with homozygous methylenetetrahydrofolate reductase (MTHFR) mutations that caused early thrombosis of the mitral annuloplasty ring. The clinical management and antithrombotic treatment of the patient, and the implications of hyperhomocysteinemia, are discussed. Video 1: Mobile 9-mm thrombus of mitral annuloplasty ring on two-dimensional (2D) transesophageal echocardiography. Video 2: Mitral annuloplasty ring thrombus on real time three-dimensional (RT-3D) transesophageal echocardiography. Video 3: 2D video showing the morphologic change of mitral annuloplasty ring thrombus after prolonged UFH infusion.

Background: Transthoracic echocardiography (TTE) is the standard method for evaluating the severity of aortic stenosis (AS), while transesophageal echocardiography (TEE) is useful for morphologic characterization. The study aim was to assess the impact of complementary TEE to TTE in refining hemodynamic assessment of AS severity.

Methods: A retrospective analysis was conducted of sequential TTE and TEE studies performed in 100 patients with moderate or severe AS confirmed on prior TTE. The left ventricular outflow tract (LVOT) velocity-time-integral (VTI1) and the aortic valve velocity-time integral (VTI2) were measured for both modalities. The highest values of VTI1 and VTI2 and mean gradients and peak velocities were selected from the sequential TTE/TEE study for indexed aortic valve area (AVAi) calculation and AS severity determination through an integrated assessment.

Results: AVAi determined by TTE was not significantly different from that determined by TEE (mean difference -0.008 cm2; p = 0.38). The dimensionless velocity index (DVI) was higher when assessed by TTE than by TEE (mean difference 0.0126 ± 0.04; p = 0.003). Using the integrated AS assessment, six of 13 patients with a TTE-based diagnosis of moderate AS were re-classified as severe AS. A slight lowering of the recommended TTE-derived DVI threshold for severe AS, from 0.25 to 0.24, improved the ability to discriminate moderate versus severe AS, as determined by the integrated assessment.

Conclusions: TEE integrated with TTE may improve the detection of severe AS, particularly in patients with moderate AS criteria assessed by TTE, but with a DVI ratio ≤0.24.