A Clinical Diagnostic Test for Calcium Release Deficiency Syndrome.

Importance: Sudden death and cardiac arrest frequently occur without explanation, even after a thorough clinical evaluation. Calcium release deficiency syndrome (CRDS), a life-threatening genetic arrhythmia syndrome, is undetectable with standard testing and leads to unexplained cardiac arrest. Objective: To explore the cardiac repolarization response on an electrocardiogram after brief tachycardia and a pause as a clinical diagnostic test for CRDS. Design, Setting, and Participants: An international, multicenter, case-control study including individual cases of CRDS, 3 patient control groups (individuals with suspected supraventricular tachycardia; survivors of unexplained cardiac arrest [UCA]; and individuals with genotype-positive catecholaminergic polymorphic ventricular tachycardia [CPVT]), and genetic mouse models (CRDS, wild type, and CPVT were used to define the cellular mechanism) conducted at 10 centers in 7 countries. Patient tracings were recorded between June 2005 and December 2023, and the analyses were performed from April 2023 to December 2023. Intervention: Brief tachycardia and a subsequent pause (either spontaneous or mediated through cardiac pacing). Main Outcomes and Measures: Change in QT interval and change in T-wave amplitude (defined as the difference between their absolute values on the postpause sinus beat and the last beat prior to tachycardia). Results: Among 10 case patients with CRDS, 45 control patients with suspected supraventricular tachycardia, 10 control patients who experienced UCA, and 3 control patients with genotype-positive CPVT, the median change in T-wave amplitude on the postpause sinus beat (after brief ventricular tachycardia at ≥150 beats/min) was higher in patients with CRDS (P < .001). The smallest change in T-wave amplitude was 0.250 mV for a CRDS case patient compared with the largest change in T-wave amplitude of 0.160 mV for a control patient, indicating 100% discrimination. Although the median change in QT interval was longer in CRDS cases (P = .002), an overlap between the cases and controls was present. The genetic mouse models recapitulated the findings observed in humans and suggested the repolarization response was secondary to a pathologically large systolic release of calcium from the sarcoplasmic reticulum. Conclusions and Relevance: There is a unique repolarization response on an electrocardiogram after provocation with brief tachycardia and a subsequent pause in CRDS cases and mouse models, which is absent from the controls. If these findings are confirmed in larger studies, this easy to perform maneuver may serve as an effective clinical diagnostic test for CRDS and become an important part of the evaluation of cardiac arrest.

Easily available ECG and echocardiographic parameters for prediction of left atrial remodeling and atrial fibrillation recurrence after pulmonary vein isolation: A multicenter study.

BACKGROUND: The assessment of noninvasive markers of left atrial (LA) low-voltage substrate (LVS) enables the identification of atrial fibrillation (AF) patients at risk for arrhythmia recurrence after pulmonary vein isolation (PVI). METHODS: In this prospective multicenter study, 292 consecutive AF patients (72% male, 62 ± 11 years, 65% persistent AF) underwent high-density LA voltage mapping in sinus rhythm. LA-LVS (<0.5 mV) was considered as significant at 2 cm2  or above. Preprocedural clinical electrocardiogram and echocardiographic data were assessed to identify predictors of LA-LVS. The role of the identified LA-LVS markers in predicting 1-year arrhythmia freedom after PVI was assessed in 245 patients. RESULTS: Significant LA-LVS was identified in 123 (42%) patients. The amplified sinus P-wave duration (APWD) best predicted LA-LVS, with a 148-ms value providing the best-balanced sensitivity (0.81) and specificity (0.88). An APWD over 160 ms was associated with LA-LVS in 96% of patients, whereas an APWD under 145 ms in 15%. Remaining gray zones improved their accuracy by introduction of systolic pulmonary artery pressure (sPAP) of 35 mmHg or above, age, and sex. According to COX regression, the risk of arrhythmia recurrence 12 months following PVI was twofold and threefold higher in patients with APWD 145-160 and over 160 ms, compared to APWD under 145 ms. Integration of pulmonary hypertension further improved the outcome prediction in the intermediate APWD group: Patients with APWD 145-160 ms and normal sPAP had similar outcome than patients with APWD under 145 ms (hazard ratio [HR] 1.62, p = .14), whereas high sPAP implied worse outcome (HR 2.56, p < .001). CONCLUSIONS: The APWD identifies LA-LVS and risk for arrhythmia recurrence after PVI. Our prediction model becomes optimized by means of integration of the pulmonary artery pressure.

[COVID-19 and myocardial injury]. / Covid-19 et atteintes myocardiques.

SARS-CoV-2 affects the cardiovascular system triggering a proinflammatory response which results in direct and indirect myocardial injury. The objective of this article is to describe the underlying mechanisms and clinical implications.

Le système cardiovasculaire est largement affecté par le coronavirus SARS-CoV-2. La maladie à coronavirus 2019 (Covid-19) peut déclencher une réaction inflammatoire intense, responsable de lésions myocardiques directes et indirectes chez environ un quart des patients hospitalisés. L'objectif de cet article est de décrire les mécanismes sous-jacents aux atteintes cardiovasculaires et de discuter les potentielles implications cliniques.

Postcardiotomy effusive-constrictive pericarditis in a 65-year-old man: Noteworthy echocardiographic signs.

Effusive-constrictive pericarditis (eCP) is a rare entity where constriction of the heart occurs through the visceral pericardium, with concomitant presence of a tense effusion in the pericardial space. We present the case of a 65-year-old patient with shortness of breath and pericardial effusion, 4 months after aortic and mitral valve replacement for Streptococcus agalactiae endocarditis. The present report focuses on the echocardiographic features of constrictive pericarditis (CP) and provides a brief overview of the pathophysiology, work-up, and management guidelines.

Overuse of plain abdominal radiography in emergency departments: a retrospective cohort study.

BACKGROUND: Plain abdominal radiography (PAR) is routinely performed in emergency departments (EDs). This study aimed to (1) identify the indications for PAR in EDs and compare them against international guidelines, (2) uncover predictors of non-compliance with guidelines, and (3) describe the use of additional radiological examinations in EDs. METHODS: Retrospective cohort study in the EDs of two hospitals in Geneva, Switzerland, including all adult patients who underwent PAR in the EDs. Indications were considered "appropriate" if complying with guidelines. Predictors of non-compliance were identified by univariate and multivariate analyses. RESULTS: Over 1 year, PAR was performed in 1997 patients (2.2% of all admissions). Their mean age was 59.7 years, with 53.1% of female patients. The most common indications were constipation (30.8%), suspected ileus (28.9%), and abdominal pain (15.3%). According to the French and American guidelines, only 11.8% of the PARs were indicated, while 46.2% of them complied with the Australian and British guidelines. On multivariate analysis, admission to the private hospital ED (odds ratio [OR] 3.88, 95% CI 1.78-8.45), female gender (OR 1.95, 95% CI 1.46-2.59), and an age >  65 years (OR 2.41, 95%CI 1.74-3.32) were associated with a higher risk of inappropriate PAR. Additional radiological examinations were performed in 73.7% of patients. CONCLUSIONS: Most indications for PAR did not comply with guidelines and elderly women appeared particularly at risk of being exposed to inappropriate examination. PAR did not prevent the need for additional examinations. Local guidelines should be developed, and initiatives should be implemented to reduce unnecessary PARs. TRIAL REGISTRATION: ClinicalTrials.gov , identifier NCT02980081 .

[Management and orientation of severely infected patients : relevance of a « Sepsis Fast-Track ¼]. / Trajectoire de prise en charge des patients avec infections sévères : intérêt de l'implémentation d'une filière sepsis.

Sepsis is a syndrome defined by a life-threatening organ dysfunction caused by a dysregulated host response to an infection. The early recognition of this syndrome in the emergency department (ED) can lead to a better prognosis, when associated with a standardized management focusing on identification of the infectious source, its treatment, and appropriate organ support. Therefore, the implementation of a « Sepsis Fast Track ¼, by analogy with similar protocols regarding stroke or ST-elevated myocardial infarct, deserves interest. The aim of this article is to review the available evidences that support an implementation of such an initiative, and to identify the key elements that permit its integration in the ED setting of a secondary-care hospital.

Le sepsis est un syndrome associant une infection à une dysfonction organique significative et est grevé d'une mortalité élevée. Une identification précoce aux urgences, associée à une prise en charge systématisée, centrée sur l'identification et le contrôle de la source infectieuse et sur le soutien des organes, peut permettre d'améliorer le pronostic. Dans ce contexte, la mise en place d'une « filière sepsis ¼ par analogie aux filières STEMI ou AVC pourrait s'avérer intéressante. Cet article discute les évidences disponibles pour soutenir une telle initiative, en identifiant les éléments-clés nécessaires à une telle mise en place dans un service d'urgence et son intégration dans le fonctionnement d'un hôpital périphérique.

Computed tomography anatomic predictors of outcomes in patients undergoing tricuspid transcatheter edge-to-edge repair.

AIM: To identify anatomical computed tomography (CT) predictors of procedural and clinical outcomes in patients undergoing tricuspid transcatheter edge-to-edge repair (T-TEER). METHODS AND RESULTS: Consecutive patients undergoing T-TEER between March 2018 to December 2022 who had cardiac CT prior to the procedure were included. CT scans were automatically analyzed using a dedicated software that employs deep learning techniques to provide precise anatomical measurements and volumetric calculations. Technical success was defined as successful placement of at least one implant in the planned anatomic location without single leaflet device attachment. Procedural success was defined as tricuspid regurgitation reduction to moderate or less. Procedural complexity was assessed by measuring the fluoroscopy time. The clinical endpoint was a composite of death, heart failure hospitalization, or tricuspid re-intervention throughout two years. A total of 33 patients (63.6% male) were included. Procedural success was achieved in 22 patients (66.7%). Shorter end-systolic (ES) height between the inferior vena cava (IVC) and tricuspid annulus (TA) (r â€‹= â€‹- 0.398, p â€‹= â€‹0.044) and longer ES RV length (r â€‹= â€‹0.551, p â€‹= â€‹0.006) correlated with higher procedural complexity. ES RV length was independently associated with lower technical(adjusted Odds ratio [OR] 0.812 [95% CI 0.665-0.991], p â€‹= â€‹0.040) and procedural success (adjusted OR 0.766, CI [0.591-0.992], p â€‹= â€‹0.043). Patients with ES right ventricular (RV) length of >77.4 â€‹mm had a four-fold increased risk of experiencing the composite clinical endpoint compared to patients with ES RV length ≤77.4 â€‹mm (HR â€‹= â€‹3.964 [95% CI, 1.018-15.434]; p â€‹= â€‹0,034]). CONCLUSION: CT-derived RV length and IVC-to-TA height may be helpful to identify patients at increased risk for procedural complexity and adverse outcomes when undergoing T-TEER. CT provides valuable information for preprocedural decision-making and device selection.

Transient central hypoxemia due to intermittent high-degree atrioventricular block in a heart-transplanted patient diagnosed during routine electroencephalography: a case report.

BACKGROUND: Bradycardia frequently occurs in heart-transplanted patients, mainly as a temporally restricted manifestation early after transplantation and often without symptoms. A high-degree atrioventricular block is mostly symptomatic through cerebral hypoxia induced through cerebral hypoperfusion. Only a few published cases show this specific electroencephalography result in this context. The purpose of this case is to bring attention to atypical manifestations of typical cardiac complications after heart transplantation and the importance of perseverance in the diagnostic. CASE PRESENTATION: A Central European man in his 50s with history of heart transplantation 31 years previously was admitted to the internal medicine ward for short-lived recurrent episodes of generalized weakness with multiple falls but without loss of consciousness. During routine electroencephalography, the patient perceived this recurrent sensation. This episode coincided with a transient third-degree atrioventricular block followed 8-10 seconds later by a generalized slowing of the electroencephalography, reflecting cerebral hypoxia due to cerebral hypoperfusion. Holter monitoring confirmed the diagnosis. A pacemaker was implanted, consequently resolving the episodes. CONCLUSION: This case report illustrates the pathophysiological central hypoxemic origin of episodes of generalized weakness caused by a high-degree atrioventricular block in a patient surviving 29 years after heart transplant. It highlights the benefit of electroencephalography as a diagnostic tool in well-selected patients.

Fulminant Influenza A myocarditis in a patient presenting with cardiogenic shock and biventricular thrombi: a case report.

BACKGROUND: Acute myocarditis is a common condition, with viral infections being the most common aetiology in North America and Europe. Influenza A myocarditis is however rare. As clinical manifestation may be fulminant, early recognition and management are paramount and may impact overall prognosis by hindering complications such as thromboembolism. A brief review of the literature, diagnostic modalities, work-up and treatment are discussed. CASE SUMMARY: We present the case of a 42-year-old, previously healthy woman with recent flu-like symptoms, developing decompensated heart failure (HF) and cardiogenic shock within a week, due to Influenza A myocarditis. Biventricular thrombi were identified. Pharmacological haemodynamic support, followed by HF therapy, allowed full recuperation of heart function. Intracavitary thrombi disappeared under unfractionated heparin with bridging to rivaroxaban. DISCUSSION: Fulminant myocarditis due to Influenza A is rare and, to the best of our knowledge, has not been associated with intracardiac thrombi formation. Echocardiography is the essential first-line imaging modality. Cardiac magnetic resonance plays a major role in the diagnosis of myocarditis and may preclude the need for an endomyocardial biopsy in selected cases. Coronary angiography may be required to rule out ischaemic aetiology. First-line therapy in fulminant disease is pharmacological and, if required, mechanical haemodynamic support. Standard HF therapy complete the therapeutic options and should be introduced as soon as possible. Complications such as intracardiac thrombi formation, require targeted treatment. Specific drug therapies targeting Influenza A have no proven benefit in myocarditis.

Rapidly progressive heart failure after dual-chamber pacemaker implantation.

Pacing-induced cardiomyopathy (PICM) consists of heart failure (HF) associated with a drop in the left ventricular ejection fraction (LVEF) in the setting of high-burden right ventricular pacing, with presentation that may range from subclinical to severe. Time to manifestation can go from weeks to years after device implantation. Treatment typically consists in an upgrade to a cardiac resynchronisation therapy (CRT) or His bundle pacing (HisP). Several risk factors for PICM have been described and should be considered before pacemaker (PM) implantation, as thorough patient selection for de novo CRT or HisP, may preclude its manifestation. We present the case of an 82-year-old patient presenting with acute congestive HF and new severely reduced LVEF, 30 days following dual chamber PM implantation for high-grade atrioventricular block. Treatment with HF medication and upgrade to a CRT permitted rapid resolution of the symptoms and normalisation of the LVEF at 1-month follow-up.

Elderly woman presenting with multivessel coronary artery disease and Takotsubo syndrome.

Takotsubo syndrome is an acute and often reversible condition, with initial presentation mimicking acute coronary syndrome. Typically, patients present with left ventricular regional wall motion abnormalities, without a corresponding coronary artery obstruction on angiography. Coexistence of a coronary artery disease is possible and may render the distinction between the two entities particularly challenging. We report the case of a 94-year-old woman with chest pain after an emotional upset and acute myocardial injury. Transthoracic echocardiogram (TTE) revealed a severely reduced left ventricular ejection fraction (LVEF) with apical ballooning. Coronary angiogram showed significant stenosis of the distal left main coronary artery and of the mid-left anterior descending artery, as well as a 30%-50% stenosis of the mid-distal right coronary artery. Revascularisation was deferred and antiplatelet as well as heart failure therapy begun. A repeat TTE 6 days later revealed a quasi-normalised LVEF. Ultimately, percutaneous coronary revascularisation of the left main and left anterior descending artery was performed, with favourable outcome at 6-month follow-up.

An incidental finding of a bicuspid aortic valve and pseudocoarctation of the descending aorta in a patient presenting with an acute coronary syndrome: a case report.

BACKGROUND: Aortic pseudocoarctation (PsCoA) is an elongation of the supra-isthmic aorta with kinking and low-grade narrowing. Consequently, no collateral circulation is found. It is frequently associated with other congenital heart anomalies, such as bicuspid aortic valve (BAV). CASE SUMMARY: We report the case of 60-year-old patient who presented to the ED with acute chest pain. Physical examination was remarkable for an elevated blood pressure (BP) and the presence of a systolic murmur on the left lower sternal border. An acute coronary syndrome was suspected and the patient underwent urgent coronary angiogram which showed an occluded obtuse marginal artery. Additionally, the presence of an aortic anomaly was noted during the aortography, and additional work-up, including a thoracic computed tomography angiography and transoesophageal echocardiography, revealed a BAV and a PsCoA of the descending aorta. The patient was discharged after optimizing BP control with oral medication and periodical follow-up was arranged. DISCUSSION: Aortic PsCoA may mimic true coarctation (CoA), but the absence of a haemodynamically significant descending aortic narrowing and of the typical clinical findings associated with aortic CoA, distinguishes both entities. Treatment is therefore conservative and based on the control of cardiovascular risk factors and in particular of arterial hypertension. Work-up should include imaging of the entire aorta and the search for associated congenital cardiac anomalies, which, if present, should be managed in consequence.