Search Results (14)

The electrocardiogram (ECG) remains a cornerstone of modern cardiology, providing rapid, non-invasive, and widely accessible diagnostic insights. While ECG interpretation is an essential skill for clinicians, certain patterns can be subtle or atypical, posing diagnostic challenges. In our previous review (doi.org/10.3390/jpm12111754), we explored several uncommon ECG syndromes with significant clinical implications. However, the spectrum of electrocardiographic abnormalities extends far beyond those initially discussed. In this second installment, we expand our discussion of rare and underrecognized ECG syndromes, including Long QT, Jervell and Lange-Nielsen, Romano–Ward, Andersen–Tawil, Timothy, Short QT, and Twiddler’s syndromes, as well as Noonan, Barlow’s, Bundgaard, BRASH, Carvajal, Naxos, and Danon disease. We highlight their clinical context, characteristic findings, and implications for diagnosis and management. These conditions range from acute, life-threatening emergencies requiring immediate intervention to chronic electrical disorders necessitating long-term monitoring and risk stratification. By broadening our focus, we aim to enhance awareness and recognition of these entities, ultimately improving patient outcomes through timely and accurate diagnosis. Full article

Objective: The aim was to assess the long-term outcomes, safety, and durability of total endoscopic mitral valve repair for Barlow mitral valve disease. Methods: A retrospective analysis of 98 patients undergoing minimal invasive total endoscopic mitral valve repair for Barlow mitral valve disease was conducted between May 2009 and December 2023. A non-resectional repair approach using artificial neochordae and/or ring annuloplasty was performed. Clinical and echocardiographic follow-ups were completed. Rates per patient-years with 95% confidence intervals (CI) for all time-to-event outcomes were calculated. Results: The mean age was 59 ± 12, and 43% were female. Minimally invasive mitral valve repair was successfully performed in all 98 patients with no conversions to sternotomy or mitral valve replacement. There was no mitral valve-related reoperation during the hospital stay. Procedural safety was as follows: no in-hospital mortality, no stroke, and no perioperative myocardial infarction. The mean follow-up was 4.1 ± 3.1 years. Survival at seven years was 87% (95% CI 63% to 96%). Freedom from myocardial infarction, stroke, and congestive heart failure was 89% (95% CI 60% to 97%), 93% (95% CI 82% to 97%), and 100%, respectively. Recurrent mitral valve insufficiency at Grade ≥ 2 was diagnosed in n = 4 (4.1%) of cases. Conclusions: Minimally invasive mitral valve repair using a non-resectional technique for Barlow disease can be performed with a low complication rate. The total endoscopic approach is safe in the long term, with minimal risk of reoperation and recurrent mitral valve insufficiency. Full article

Noncardiogenic pulmonary edema after cardiac surgery is a rare but severe complication. The etiology remains poorly understood; however, the issue may arise from multiple sources. Possible causes include a significant inflammatory response or an autoimmune process. Pulmonary edema resulting from noncardiac etiologies can necessitate extracorporeal membrane oxygenation (ECMO) because most of the cases present a substantial volume of fluid expelled from the lungs and the medical team must manage the inability to achieve effective ventilation. A 64-year-old patient with known heart disease was admitted to our clinic with acute pulmonary edema. His medical history included Barlow’s disease, severe mitral regurgitation (IIP2), moderate–severe tricuspid regurgitation, and moderate pulmonary hypertension. The patient had a coronary angiography performed in a prior hospitalization before the surgical intervention which indicated the absence of coronary lesions. Preoperative screening (nasal, pharyngeal exudate, inguinal pouch culture, and urine culture) was negative, with no active dental infections. The patient was stabilized, and 14 days post-admission, mitral and tricuspid valve repair was performed via a thoracoscopic approach. After being admitted to intensive care post-surgery, the patient quickly developed pulmonary edema, producing a large volume (4.5 L) of yellow secretions through the intubation tube followed by hemodynamic instability necessitating high doses of medications to support circulation but no cardiorespiratory arrest. Due to his worsening condition, the patient was urgently taken back to the operating room, where veno-venous extracorporeal membrane oxygenation (VV-ECMO) was initiated to support oxygenation and stabilize the patient. Full article

Surgical correction of severe mitral regurgitation (MR) can reverse left ventricular (LV) remodeling in patients with mitral valve prolapse (MVP). However, whether this process is similar to the case in Barlow’s Disease (BD) and Fibro-elastic Deficiency (FED) is currently unknown. The aim of this study is to evaluate post-operative LV reverse remodeling and function in patients with BD versus FED. In this study, 100 MVP patients (BD = 37 and FED = 63) with severe MR who underwent mitral valve surgery at three Belgian centers were retrospectively included. Transthoracic echocardiography was used to assess MR severity, LV volumes and function before surgery and 6 months thereafter. Baseline MR severity, LV ejection fraction (LVEF), indexed LV end-diastolic (LVEDVi) and end-systolic volumes (LVESVi) were not different between the groups. After a median follow-up of 278 days, there was a similar decrease in LVEDVi, but a trend towards a smaller decrease in LVESVi in BD compared to FED (−3.0 ± 11.2 mL/m² vs. −5.3 ± 9.0 mL/m²; p = 0.154). This resulted in a significantly larger decrease in LVEF in BD (−8.3 ± 9.6%) versus FED (−3.9 ± 6.9%) after adjusting for baseline LVEF (p < 0.001) and type of surgical intervention (p = 0.01). These findings suggest that LV (reverse) remodeling in BD could be affected by other mechanisms beyond volume overload, potentially involving concomitant cardiomyopathy. Full article

Both the MitraClip and PASCAL systems offer transcatheter edge-to-edge repair (TEER) solutions for mitral regurgitation. Evidence indicates a lower technical success rate for TEER in complex degenerative mitral regurgitation (DMR) cases. We conducted a retrospective analysis of patients who underwent transcatheter edge-to-edge therapy for primary mitral regurgitation with advanced anatomy, defined as mitral regurgitation effective regurgitant orifice area (MR-EROA) ≥0.40 cm² or large flail gap (≥5 mm) or width (≥7 mm) or Barlow’s disease, that completed follow-up after 1 year. Our criteria were met by 27 patients treated with PASCAL and 18 with MitraClip. All patients exhibited a significant, equivalent short-term reduction in MR-EROA, mitral regurgitation vena contracta diameter (MR-VCD), regurgitant volume, and clinical status. At 1 year follow-up, reductions in MR-VCD, regurgitant volume, and MR-EROA remained significant for both groups without significant differences between groups. MR-Grade ≤ 1+ was achieved in 18 (66.7%) and 10 (55.6%) patients, respectively. At follow-up, no difference in hospitalization for cardiac decompensation was observed. Overall death was similar in both groups. Our study suggests that both the PASCAL and MitraClip systems significantly reduce mitral regurgitation even in advanced degenerative diseases. Within our limited data, we found no evidence of inferior performance of the PASCAL system. Full article

Developmental dysplasia of the hip, previously known as congenital dislocation of the hip, is the most common congenital disease of the musculoskeletal system in newborns. It corresponds to a complete or partial displacement of the femoral head out of the acetabulum. The incidence is around 1:1000 births and it depends on multiple factors (region, family history, gender, mechanical factors, or some specific neonatal conditions). Screening may be by universal neonatal clinical examination (Ortolani or Barlow maneuvers) with the addition of sonographic imaging of the hip (selecting the patients who present abnormalities detected by physical examination or risk factors, and universal screening in the neonate). This screening is recommended at the discharge of newborns in most maternity hospitals in Romania. Full article

Canine myxomatous mitral valve disease (MMVD) is similar to Barlow’s form of MMVD in humans. These valvulopathies are complex, with varying speeds of progression. We hypothesized that the relative abundances of serum proteins would help identify the consecutive MMVD stages and discover new disease pathways on a systemic level. To identify distinction-contributing protein panels for disease onset and progression, we compared the proteomic profiles of serum from healthy dogs and dogs with different stages of naturally occurring MMVD. Dogs were divided into experimental groups on the basis of the left-atrium-to-aorta ratio and normalized left ventricular internal dimension in diastole values. Serum was collected from healthy (N = 12) dogs, dogs diagnosed with MMVD in stages B1 (N = 13) and B2 (N = 12) (asymptomatic), and dogs diagnosed with MMVD in chronic stage C (N = 13) (symptomatic). Serum biochemistry and selected ELISAs (galectin-3, suppression of tumorigenicity, and asymmetric dimethylarginine) were performed. Liquid chromatography–mass spectrometry (LC–MS), tandem mass tag (TMT) quantitative proteomics, and statistical and bioinformatics analysis were employed. Most of the 21 serum proteins with significantly different abundances between experimental groups (p < 0.05, FDR ˂ 0.05) were classified as matrix metalloproteinases, protease inhibitors, scaffold/adaptor proteins, complement components, anticoagulants, cytokine, and chaperone. LC–MS TMT proteomics results obtained for haptoglobin, clusterin, and peptidase D were further validated analytically. Canine MMVD stages, including, for the first time, asymptomatic B1 and B2 stages, were successfully distinguished in dogs with the disease and healthy dogs on the basis of the relative abundances of a panel of specific serum proteins. Most proteins with significantly different abundances were involved in immune and inflammatory pathways. Their role in structural remodeling and progression of canine MMVD must be further investigated. Further research is needed to confirm the resemblance/difference with human MMVD. Proteomics data are available via ProteomeXchange with the unique dataset identifier PXD038475. Full article

Mitral annular calcification is a chronic and degenerative process of the fibrous base of the mitral valve. Surgical management of mitral valve dysfunction with severe annular calcification remains technically demanding and, to date, the preferred approach is still a standard full sternotomy. We aimed to analyze and report our experience with mitral valve surgery addressing annular calcification via the minimally invasive approach through a right mini-thoracotomy. Data of patients with mitral valve disease and underlying annular calcification undergoing minimally invasive surgery from 2018 to 2022 were prospectively collected and retrospectively analyzed. The severity of mitral annular calcification was categorized with an angio-computerized tomography scan analysis as mild, moderate or severe according to calcium thickness, calcium distribution, and trigone and leaflet involvement using the Mitral Annular Calcification Computerized Tomography-score. During the study period, 27 patients with mitral valve disease and associated mitral annular calcification were enrolled. The most common etiology was advanced Barlow’s disease, which was encountered in 18 cases (67%). Mitral valve replacement was performed in 21 patients (78%). No intraoperative death, atrioventricular disruption, or circumflex coronary artery injury occurred. Conversion to sternotomy was necessary in a single case. Residual moderate periprosthetic leak requiring early reoperation and permanent heart block mandating permanent pacemaker implantation were reported in one and in three patients, respectively. No cases of stroke were reported. Two patients died, accounting for a 7.4% perioperative mortality. At a median follow-up of 9 months, one patient had residual moderate mitral regurgitation, whereas two patients required short-term reoperation and prosthetic valve (re)replacement. Minimally invasive mitral valve surgery via right mini-thoracotomy should be considered an and effective approach to be indicated also in patients with mild-to-severe mitral annular calcification. Routine angio-computerized tomography scan during work-up is a mandatory step to stratify the anatomical extension and severity of the mitral annular calcification. Full article

Backround: Mitral valve (MV) repair in the case of a large anterior-posterior diameter and redundant valve tissue remains challenging and favors repair with a ring that exhibits a large anterior-posterior diameter. Compared to other available rings, the Medtronic Simulus annuloplasty ring shows the largest anterior-posterior diameter. This study reports for the first time mid-term results using this annuloplasty ring. Methods: Between 11/2015 and 12/2019, a total of 378 patients underwent MV repair for degenerative mitral regurgitation using the Medtronic Simulus ring, according to the following selection criteria: large MV annuli, abundant leaflet tissue (i.e., Barlow disease), and risk for SAM. Results: Overall survival after 5 years was 90.8 ± 4.6%. Five patients required valve-related reoperations because of ring dehiscence (n = 1), progression of native valve disease (n = 2), dehiscence of quadrangular resection suture (n = 1), and endocarditis (n = 1). The cumulative incidence of valve-related reoperation at 5 years was 1.3 ± 0.5%. At latest follow-up, echocardiography demonstrated excellent valve function with no/mild MR in 299 patients (94.6%). Two patients (0.6%) had more than moderate MR. No patient developed SAM after repair. Conclusion: Repair of MV with large annuli and abundant leaflet tissue with the Medtronic Simulus annuloplasty ring shows excellent mid-term results regarding reoperation rates and recurrent MR. Full article

Bevacizumab is a recombinant humanized monoclonal antibody and a key drug for treatment of various types of cancer. Bevacizumab is associated with the occurrence of heart failure, but its risk factors remain unknown. A 55-year-old woman was diagnosed with cervical cancer, which was completely treated by bevacizumab-incorporated chemotherapy. During the 9-month bevacizumab therapy, she suffered from hypertension requiring multiple antihypertensive agents. She was admitted to our hospital due to acute heart failure with afterload mismatch and severe mitral regurgitation. A transesophageal echocardiography showed Barlow’s disease with a degenerated and widely prolapsed mitral valve. She received a scheduled surgical mitral valve repair. Post-operative cause was uneventful, but metastatic dissemination developed later. The existence of mitral valve regurgitation, even when sub-clinical, might be a risk of worsening heart failure during bevacizumab therapy. Careful follow-up at an onco-cardiology clinic is highly encouraged particularly for such a cohort during bevacizumab therapy. Full article

Degenerative mitral valve disease causing mitral valve prolapse is the most common cause of primary mitral regurgitation, with two distinct phenotypes generally recognized with some major differences, i.e., fibroelastic deficiency (FED) and Barlow’s disease. The aim of this review was to describe the main histological, clinical and echocardiographic features of patients with FED and Barlow’s disease, highlighting the differences in diagnosis, risk stratification and patient management, but also the still significant gaps in understanding the exact pathophysiology of these two phenotypes. Full article

The development of transcatheter mitral valve replacement therapies requires accurate post-processing analysis tools to provide D-shaped mitral annulus dimensions from 3-dimensional (3D) data. The agreement between two semi-automated, software packages to process 3D transesophageal echocardiography (TEE) data for the measurement of the mitral valve annulus dimensions was evaluated. 3DTEE data of patients with moderate–severe mitral regurgitation (MR) were postprocessed with semi-automated, vendor-independent (VI) software and vendor-specific (VS) software. Both post-processing software provided key measurements for the selection of transcatheter valve prosthesis size: annulus area, annulus circumference and the septal-to-lateral distance of the annulus. The intertrigonal distance was provided only by the VS software. The inter- and intra-observer agreements were assessed with Bland–Altman analysis. Of 105 patients (63.8 ± 11 years, 66% male) with MR, 28 had secondary MR, 45 fibroelastic deficiency, and 32 Barlow’s disease. Using VS software, the dimensions for the overall population were 16.1 ± 4.6 cm² for annulus area, for circumference 14.4 ± 1.9 cm, intertrigonal distance 3.4 ± 0.5 cm and septal-to-lateral distance 3.8 ± 0.6 cm. Similar dimensions were obtained using VI software: 15.7 ± 4.6 cm² for annulus area, 14.5 ± 2.0 cm for circumference, and 4.1 ± 0.6 cm for septal-to-lateral distance. The inter- and intra-observer agreement for both software programs was excellent. In conclusion, current post-processing software programs for 3DTEE data of the mitral valve annulus provide good reproducibility of key measurements to select the transcatheter prosthesis size. Full article

Myxomatous mitral valve disease is the single most important mitral valve disease in both dogs and humans. In the case of the dog it is ubiquitous, such that all aged dogs will have some evidence of the disease, and for humans it is known as Barlow’s disease and affects up to 3% of the population, with an expected increase in prevalence as the population ages. Disease in the two species show many similarities and while both have the classic myxomatous degeneration only in humans is there extensive fibrosis. This dual pathology of the human disease markedly affects the valve transcriptome and the difference between the dog and human is dominated by changes in genes associated with fibrosis. This review will briefly examine the comparative valve pathology and then, in more detail, the transcriptomic profiling and gene expression reported so far for both species. Full article

We describe the case of a 46-year old female with a Barlow’s disease (MVP) characterized by systolic curling of posterior left ventricular (LV) wall + significant mitral annular disjunction + complex ventricular arrhythmias + syncope + inverted T waves in inferolateral leads in whom a successful surgical mitral valve rapair determined the disappearance not only of the echocardiographic but also the electrocardiographic abnormalities (in particular the inferolateral T waves inversion on basal electrocardiogram and the complex basal arrhythmic pattern). This case demonstrates that electrocardiographic abnormalities may disappear after the surgical correction of the mechanical stretch imposed on the inferior LV free wall by the prolapsing mitral valve leaflets. Electrocardiographic changes remain an important and easy marker to recognize for the identification of a high-risk subgroup of MVP patients. Full article