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European Heart Journal. Case Reports Jun 2024Tricuspid annular disjunction (TAD) is an annular disjunction of the right-sided heart. Although TAD is often concomitant with mitral annular disjunction (MAD), it often...
BACKGROUND
Tricuspid annular disjunction (TAD) is an annular disjunction of the right-sided heart. Although TAD is often concomitant with mitral annular disjunction (MAD), it often presents as mitral regurgitation (MR), rather than tricuspid regurgitation (TR). While the clinical significance of MAD has been well-established, there is still little data on TAD. This is a rare case of severe TR due to TAD that appears to be isolated from MAD.
CASE SUMMARY
A 63-year-old female complaining of pre-syncope and dyspnoea on exertion was referred to our department. Initial transthoracic echocardiography showed MR and TR due to tricuspid valve prolapse (TVP). On transoesophageal echocardiography, the TVP consisted of an excessively redundant anterior leaflet, where the annular disjunction and severe regurgitation were formed. She recently underwent mitral and tricuspid valve plasties for symptomatic primary severe TR.
DISCUSSION
This case report emphasizes the clinical significance of TAD as a potential cause of severe TR, even without significant MR. Tricuspid annular disjunction progresses more gradually compared with MAD. This case suggests that remodelling of the right atrium, particularly in chronic atrial fibrillation, may contribute to the development of TR. Despite diagnostic challenges due to the flexible and dynamic nature of the tricuspid annulus, this is the first report of TAD-induced severe TR necessitating surgical intervention. Accurately diagnosing TAD remains challenging with current imaging modalities, emphasizing the need for improved diagnostic tools to optimize treatment strategies.
PubMed: 38912119
DOI: 10.1093/ehjcr/ytae270 -
Methodist DeBakey Cardiovascular Journal 2024Apical hypertrophic cardiomyopathy (HCM) is a rare variant of HCM. A 43-year-old female with a past medical history significant for hypertension and kidney...
Apical hypertrophic cardiomyopathy (HCM) is a rare variant of HCM. A 43-year-old female with a past medical history significant for hypertension and kidney transplantation presented with recurrent syncopal episodes and dyspnea on exertion. Electrocardiogram showed characteristic diffuse giant T-waves inversion, and cardiac magnetic resonance showed HCM with circumferential apical thickening. This case highlights the rapid development of apical HCM and its challenging diagnostic characteristics.
Topics: Humans; Female; Cardiomyopathy, Hypertrophic; Adult; Electrocardiography; Disease Progression; Magnetic Resonance Imaging; Predictive Value of Tests; Magnetic Resonance Imaging, Cine; Apical Hypertrophic Cardiomyopathy
PubMed: 38911827
DOI: 10.14797/mdcvj.1386 -
Pulmonary Circulation Apr 2024Pulmonary hypertension (PH) adds a substantial disease burden, including higher mortality, when associated with interstitial lung disease (ILD), a severe, chronic,...
Pulmonary hypertension (PH) adds a substantial disease burden, including higher mortality, when associated with interstitial lung disease (ILD), a severe, chronic, progressive condition. Yet little is known of the lived experiences, perspectives, priorities, and viewpoints of patients and carers living with PH-ILD. The Voice of the Patient meeting at the center of this qualitative research study aims to provide these difficult-to-obtain insights from a European perspective for the first time. The multistakeholder approach brought together four PH-ILD patients, three primary caregivers, two patient associations, clinical experts, sponsor representatives, and a facilitator. Of the six major themes identified in the thematic analysis, symptoms, and physical limitations were the most impactful. Shortness of breath was the most bothersome symptom affecting patients daily. Further symptoms included fatigue, cough, dizziness, syncope, edema, and palpitations. Physical limitations focused on reduced mobility, impacting patients' ability to perform daily tasks, hobbies, sports, and to enjoy travel. Existing antifibrotic and pulmonary arterial hypertension-targeted treatments were perceived as beneficial. However, despite advances in treatment, severe disease burdens and high unmet medical needs persist from the perspectives of patients. Most meaningful to patients' daily wellbeing was supplemental oxygen, enabling greater mobility. Patients and carers reported difficulties and barriers in navigating the healthcare system and obtaining adequate information to reduce their considerable uncertainties, documenting the substantial challenges that rare and complex conditions such as PH-ILD pose for routine clinical practice beyond PH expert centers and indicating an urgent need for high-quality patient- and clinician-directed information to support patient-centered care.
PubMed: 38911184
DOI: 10.1002/pul2.12405 -
Bundesgesundheitsblatt,... Jun 2024The prevalence of cardiovascular diseases increases with age. Common symptoms such as dyspnea, chest pain, dizziness, or syncope can impact driving fitness. Due to... (Review)
Review
The prevalence of cardiovascular diseases increases with age. Common symptoms such as dyspnea, chest pain, dizziness, or syncope can impact driving fitness. Due to a growing number of private drivers aged 65 and older and an increasing prevalence of cardiovascular diseases, questions regarding driving fitness restrictions for cardiological patients are gaining prominence in clinical settings. This article aims to summarize current recommendations for driving fitness in the context of cardiovascular diseases. The basis for the guidelines includes the Driving License Ordinance, the expert assessment guidelines of the Federal Highway Research Institute, and the guidelines of the German Society of Cardiology on driving fitness. Original literature on this topic is limited.Emphasizing an individualized assessment, clear guidelines for driving fitness in cardiac diseases or their symptoms and treatments are formulated. Regardless of the cardiac condition, the symptoms and likelihood of sudden loss of consciousness play a leading role in driving fitness assessment. Resulting impairments can range from a few weeks to a complete revocation of driving fitness. Regular examinations and differentiated assessments by medical professionals are prerequisites for maintaining driving fitness.The driving fitness of older private drivers is a significant and practical topic in cardiology. Current guidelines support the treating physicians in providing appropriate recommendations.
PubMed: 38904775
DOI: 10.1007/s00103-024-03912-7 -
Cureus May 2024Intralesional steroid injections avoid potential side effects associated with systemic administration, such as hypothalamus-pituitary-adrenal axis suppression,...
Intralesional steroid injections avoid potential side effects associated with systemic administration, such as hypothalamus-pituitary-adrenal axis suppression, endocrine changes, allergic reactions, syncope, and blindness, but do not spare local side effects, such as pain, hemorrhage, ulceration, atrophy, hypopigmentation, calcification, secondary infection, granuloma formation, and allergic reaction. Linear leukoderma following intralesional steroid is a rare but known complication. Here, we report a case of a 23-year-old female presented with cutaneous linear depigmentation along the volar aspect of her left forearm developed three months following a single episode of injection triamcinolone acetonide for ganglion cyst.
PubMed: 38903338
DOI: 10.7759/cureus.60783 -
Radiology. Cardiothoracic Imaging Jun 2024Purpose To use unsupervised machine learning to identify phenotypic clusters with increased risk of arrhythmic mitral valve prolapse (MVP). Materials and Methods This...
Purpose To use unsupervised machine learning to identify phenotypic clusters with increased risk of arrhythmic mitral valve prolapse (MVP). Materials and Methods This retrospective study included patients with MVP without hemodynamically significant mitral regurgitation or left ventricular (LV) dysfunction undergoing late gadolinium enhancement (LGE) cardiac MRI between October 2007 and June 2020 in 15 European tertiary centers. The study end point was a composite of sustained ventricular tachycardia, (aborted) sudden cardiac death, or unexplained syncope. Unsupervised data-driven hierarchical -mean algorithm was utilized to identify phenotypic clusters. The association between clusters and the study end point was assessed by Cox proportional hazards model. Results A total of 474 patients (mean age, 47 years ± 16 [SD]; 244 female, 230 male) with two phenotypic clusters were identified. Patients in cluster 2 (199 of 474, 42%) had more severe mitral valve degeneration (ie, bileaflet MVP and leaflet displacement), left and right heart chamber remodeling, and myocardial fibrosis as assessed with LGE cardiac MRI than those in cluster 1. Demographic and clinical features (ie, symptoms, arrhythmias at Holter monitoring) had negligible contribution in differentiating the two clusters. Compared with cluster 1, the risk of developing the study end point over a median follow-up of 39 months was significantly higher in cluster 2 patients (hazard ratio: 3.79 [95% CI: 1.19, 12.12], = .02) after adjustment for LGE extent. Conclusion Among patients with MVP without significant mitral regurgitation or LV dysfunction, unsupervised machine learning enabled the identification of two phenotypic clusters with distinct arrhythmic outcomes based primarily on cardiac MRI features. These results encourage the use of in-depth imaging-based phenotyping for implementing arrhythmic risk prediction in MVP. MR Imaging, Cardiac, Cardiac MRI, Mitral Valve Prolapse, Cluster Analysis, Ventricular Arrhythmia, Sudden Cardiac Death, Unsupervised Machine Learning © RSNA, 2024.
Topics: Humans; Mitral Valve Prolapse; Female; Male; Middle Aged; Unsupervised Machine Learning; Retrospective Studies; Phenotype; Registries; Magnetic Resonance Imaging, Cine; Arrhythmias, Cardiac; Adult; Magnetic Resonance Imaging
PubMed: 38900026
DOI: 10.1148/ryct.230247 -
Heart Rhythm Jun 2024
PubMed: 38897334
DOI: 10.1016/j.hrthm.2024.06.024 -
Journal of Clinical Medicine May 2024Syncope is a common condition encountered in the emergency department (ED), accounting for about 0.6-3% of all ED visits. Despite its high frequency, a widely accepted... (Review)
Review
Syncope is a common condition encountered in the emergency department (ED), accounting for about 0.6-3% of all ED visits. Despite its high frequency, a widely accepted management strategy for patients with syncope in the ED is still missing. Since syncope can be the presenting condition of many diseases, both severe and benign, most research efforts have focused on strategies to obtain a definitive etiologic diagnosis. Nevertheless, in everyday clinical practice, a definitive diagnosis is rarely reached after the first evaluation. It is thus troublesome to aid clinicians' reasoning by simply focusing on differential diagnoses. With the current review, we would like to propose a management strategy that guides clinicians both in the identification of conditions that warrant immediate treatment and in the management of patients for whom a diagnosis is not immediately reached, differentiating those that can be safely discharged from those that should be admitted to the hospital or monitored before a final decision. We propose the mnemonic acronym RED-SOS: Recognize syncope; Exclude life-threatening conditions; Diagnose; Stratify the risk of adverse events; Observe; decide on the Setting of care. Based on this acronym, in the different sections of the review, we discuss all the elements that clinicians should consider when assessing patients with syncope.
PubMed: 38892942
DOI: 10.3390/jcm13113231 -
International Journal of Molecular... Jun 2024Arrhythmogenic cardiomyopathy (ACM) is a rare genetic cardiac disease characterized by the progressive substitution of myocardium with fibro-fatty tissue. Clinically,... (Review)
Review
Arrhythmogenic cardiomyopathy (ACM) is a rare genetic cardiac disease characterized by the progressive substitution of myocardium with fibro-fatty tissue. Clinically, ACM shows wide variability among patients; symptoms can include syncope and ventricular tachycardia but also sudden death, with the latter often being its sole manifestation. Approximately half of ACM patients have been found with variations in one or more genes encoding cardiac intercalated discs proteins; the most involved genes are plakophilin 2 (), desmoglein 2 (), and desmoplakin (). Cardiac intercalated discs provide mechanical and electro-metabolic coupling among cardiomyocytes. Mechanical communication is guaranteed by the interaction of proteins of desmosomes and adheren junctions in the so-called , whereas electro-metabolic coupling between adjacent cardiac cells depends on gap junctions. Although ACM has been first described almost thirty years ago, the pathogenic mechanism(s) leading to its development are still only partially known. Several studies with different animal models point to the involvement of the Wnt/β-catenin signaling in combination with the Hippo pathway. Here, we present an overview about the existing murine models of ACM harboring variants in intercalated disc components with a particular focus on the underlying pathogenic mechanisms. Prospectively, mechanistic insights into the disease pathogenesis will lead to the development of effective targeted therapies for ACM.
Topics: Animals; Humans; Disease Models, Animal; Arrhythmogenic Right Ventricular Dysplasia; Plakophilins; Desmoplakins; Wnt Signaling Pathway; Desmoglein 2; Desmosomes; Mice
PubMed: 38892395
DOI: 10.3390/ijms25116208 -
Open Heart Jun 2024Neurocardiogenic syncope is a common condition with significant associated psychological and physical morbidity. The effectiveness of therapeutic options for... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Neurocardiogenic syncope is a common condition with significant associated psychological and physical morbidity. The effectiveness of therapeutic options for neurocardiogenic syncope beyond placebo remains uncertain.
METHODS
The primary endpoint was the risk ratio (RR) of spontaneously recurring syncope following any therapeutic intervention. We also examined the effect of blinding on treatment efficacy. We identified all randomised trials which evaluated the effect of any pharmacological, device-based or supportive intervention on patients with a history of syncope. A systematic search was conducted on Medline, Embase, PubMed databases and Cochrane Central Register for Controlled Trials from 1950 to 25 April 2023. Event rates, their RRs and 95% CIs were calculated, and a random-effects meta-analysis was conducted for each intervention. Data analysis was performed in R using RStudio.
RESULTS
We identified 47 eligible trials randomising 3518 patients. Blinded trials assessing syncope recurrence were neutral for beta blockers, fludrocortisone and conventional dual-chamber pacing but were favourable for selective serotonin reuptake inhibitors (SSRIs) (RR 0.40, 95% CI 0.26 to 0.63, p<0.001), midodrine (RR 0.70, 95% CI 0.53 to 0.94, p=0.016) and closed-loop stimulation (CLS) pacing (RR 0.15, 95% CI 0.07 to 0.35, p<0.001). Unblinded trials reported significant benefits for all therapy categories other than beta blockers and consistently showed larger benefits than blinded trials.
CONCLUSIONS
Under blinded conditions, SSRIs, midodrine and CLS pacing significantly reduced syncope recurrence. Future trials for syncope should be blinded to avoid overestimating treatment effects.
PROSPERO REGISTRATION NUMBER
CRD42022330148.
Topics: Humans; Syncope, Vasovagal; Randomized Controlled Trials as Topic; Treatment Outcome; Recurrence
PubMed: 38890128
DOI: 10.1136/openhrt-2024-002669