Floppy mitral valve/mitral valve prolapse syndrome: Beta-adrenergic receptor polymorphism may contribute to the pathogenesis of symptoms

AbstractBackgroundCertain patients with floppy mitral valve (FMV)/mitral valve prolapse (MVP) may have symptoms that cannot be explained on the severity of mitral valvular regurgitation (MVR) alone; hypersensitivity to adrenergic stimulation has been suggested in this group defined as the FMV/MVP syndrome.MethodsNinety-eight patients (75 men, 23 women) with mitral valve surgery for FMV/MVP were studied. Of those 41 (42%) had symptoms consistent with FMV/MVP syndrome [29 men (39%), 12 women (52%)]; median age of symptom onset was 30 years (range 10–63 years) and median duration of symptoms prior to valve surgery was 16 years (range 3–50 years). Ninety-nine individuals (70 men, 29 women) without clinical evidence of any disease were used as controls. Genotyping of β1 and β2 adrenergic receptors was performed.Resultsβ-Adrenergic receptor genotypes (β1 and β2) were similar between control and overall FMV/MVP patients. Subgroup analysis of patients, however, demonstrated that the genotype C/C at position 1165 resulting in 389 Arg/Arg of the β1 receptor was more frequent in women compared to those without FMV/MVP syndrome and to normal control women (p<0.025). This polymorphism may be related to hypersensitivity to adrenergic stimulation as reported previously in these patients.ConclusionThis study shows a large proportion of patients with FMV/MVP, predominantly women, had symptoms consistent with the FMV/MVP syndrome for many years prior to the development of significant MVR, and thus symptoms cannot be attributed to the severity of MVR alone. Further, women with FMV/MVP syndrome, symptoms at least partially may be related to β1-adrenergic receptor polymorphism, which has been shown previously to be associated with a hyperresponse to adrenergic stimulation

Spontaneous Dissection of Right Coronary Artery Manifested with Acute Myocardial Infarction

Spontaneous coronary artery dissection is a rare cause of acute ischemic coronary events and sudden cardiac death. It usually occurs in young women without traditional risk factors for coronary artery disease during pregnancy or postpartum period. However, it has also been reported in patients with atherosclerotic coronary disease. We present a case of spontaneous right coronary artery dissection in a 48-year male with recent myocardial infarction and previous percutaneous coronary intervention

Quinidine: an “Endangered Species” Drug Appropriate for Management of Electrical Storm in Brugada Syndrome

AbstractBrugada syndrome is an inherited channelopathy associated with an increased risk of syncope and sudden cardiac death. In rare cases it can be manifested with electrical storm. We report two cases of Brugada syndrome that presented with electrical storm and were treated successfully with oral quinidine, an "endangered species" drug

Quinidine: an “Endangered Species” Drug Appropriate for Management of Electrical Storm in Brugada Syndrome

Brugada syndrome is an inherited channelopathy associated with an increased risk of syncope and sudden cardiac death. In rare cases it can be manifested with electrical storm. We report two cases of Brugada syndrome that presented with electrical storm and were treated successfully with oral quinidine, an "endangered species" drug

Percutaneous repair of moderate‐to‐severe or severe functional mitral regurgitation in patients with symptomatic heart failure: Baseline characteristics of patients in the RESHAPE‐HF2 trial and comparison to COAPT and MITRA‐FR trials

© 2024 The Authors. European Journal of Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.Aim: The RESHAPE-HF2 trial is designed to assess the efficacy and safety of the MitraClip device system for the treatment of clinically important functional mitral regurgitation (FMR) in patients with heart failure (HF). This report describes the baseline characteristics of patients enrolled in the RESHAPE-HF2 trial compared to those enrolled in the COAPT and MITRA-FR trials. Methods and results: The RESHAPE-HF2 study is an investigator-initiated, prospective, randomized, multicentre trial including patients with symptomatic HF, a left ventricular ejection fraction (LVEF) between 20% and 50% with moderate-to-severe or severe FMR, for whom isolated mitral valve surgery was not recommended. Patients were randomized 1:1 to a strategy of delivering or withholding MitraClip. Of 506 patients randomized, the mean age of the patients was 70 ± 10 years, and 99 of them (20%) were women. The median EuroSCORE II was 5.3 (2.8-9.0) and median plasma N-terminal pro-B-type natriuretic peptide (NT-proBNP) was 2745 (1407-5385) pg/ml. Most patients were prescribed beta-blockers (96%), diuretics (96%), angiotensin-converting enzyme inhibitors/angiotensin receptor blockers/angiotensin receptor-neprilysin inhibitors (82%) and mineralocorticoid receptor antagonists (82%). The use of sodium-glucose cotransporter 2 inhibitors was rare (7%). Cardiac resynchronization therapy (CRT) devices had been previously implanted in 29% of patients. Mean LVEF, left ventricular end-diastolic volume and effective regurgitant orifice area (EROA) were 31 ± 8%, 211 ± 76 ml and 0.25 ± 0.08 cm2, respectively, whereas 44% of patients had mitral regurgitation severity of grade 4+. Compared to patients enrolled in COAPT and MITRA-FR, those enrolled in RESHAPE-HF2 were less likely to have mitral regurgitation grade 4+ and, on average, HAD lower EROA, and plasma NT-proBNP and higher estimated glomerular filtration rate, but otherwise had similar age, comorbidities, CRT therapy and LVEF. Conclusion: Patients enrolled in RESHAPE-HF2 represent a third distinct population where MitraClip was tested in, that is one mainly comprising of patients with moderate-to-severe FMR instead of only severe FMR, as enrolled in the COAPT and MITRA-FR trials. The results of RESHAPE-HF2 will provide crucial insights regarding broader application of the transcatheter edge-to-edge repair procedure in clinical practice.Open Access funding enabled and organized by Projekt DEALinfo:eu-repo/semantics/publishedVersio