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3.
Medicina (Kaunas) ; 60(5)2024 May 16.
Article in English | MEDLINE | ID: mdl-38793002

ABSTRACT

Over the past decade, remote monitoring (RM) has become an increasingly popular way to improve healthcare and health outcomes. Modern cardiac implantable electronic devices (CIEDs) are capable of recording an increasing amount of data related to CIED function, arrhythmias, physiological status and hemodynamic parameters, providing in-depth and updated information on patient cardiovascular function. The extensive use of RM for patients with CIED allows for early diagnosis and rapid assessment of relevant issues, both clinical and technical, as well as replacing outpatient follow-up improving overall management without compromise safety. This approach is recommended by current guidelines for all eligible patients affected by different chronic cardiac conditions including either brady- and tachy-arrhythmias and heart failure. Beyond to clinical advantages, RM has demonstrated cost-effectiveness and is associated with elevated levels of patient satisfaction. Future perspectives include improving security, interoperability and diagnostic power as well as to engage patients with digital health technology. This review aims to update existing data concerning clinical outcomes in patients managed with RM in the wide spectrum of cardiac arrhythmias and Hear Failure (HF), disclosing also about safety, effectiveness, patient satisfaction and cost-saving.


Subject(s)
Heart Failure , Humans , Heart Failure/therapy , Heart Failure/diagnosis , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/therapy , Monitoring, Physiologic/methods , Telemedicine/trends , Defibrillators, Implantable/standards
4.
Europace ; 26(5)2024 May 02.
Article in English | MEDLINE | ID: mdl-38696701

ABSTRACT

AIMS: Women have been historically underrepresented in implantable cardioverter-defibrillator (ICD) trials. No data on sex differences regarding subcutaneous ICDs (S-ICD) carriers have been described. Aim of our study was to investigate sex-related differences among unselected S-ICD recipients. METHODS AND RESULTS: Consecutive patients enrolled in the multicentre, international i-SUSI registry were analysed. Comparisons between sexes were performed using a 1:1 propensity matching adjusted analysis for age, body mass index (BMI), left ventricular function, and substrate. The primary outcome was the rate of appropriate shocks during follow-up. Inappropriate shocks and other device-related complications were deemed secondary outcomes. A total of 1698 patients were extracted from the i-SUSI registry; 399 (23.5%) were females. After propensity matching, two cohorts of 374 patients presenting similar baseline characteristics were analysed. Despite similar periprocedural characteristics and a matched BMI, women resulted at lower risk of conversion failure as per PRAETORIAN score (73.4% vs. 81.3%, P = 0.049). Over a median follow-up time of 26.5 [12.7-42.5] months, appropriate shocks were more common in the male cohort (rate/year 3.4% vs. 1.7%; log-rank P = 0.049), while no significant differences in device-related complications (rate/year: 6.3% vs. 5.8%; log-rank P = 0.595) and inappropriate shocks (rate/year: 4.3% vs. 3.1%; log-rank P = 0.375) were observed. After controlling for confounders, sex remained significantly associated with the primary outcome (aHR 1.648; CI 0.999-2.655, P = 0.048), while not resulting predictor of inappropriate shocks and device-related complications. CONCLUSION: In a propensity-matched cohort of S-ICD recipients, women are less likely to experience appropriate ICD therapy, while not showing higher risk of device-related complications. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT0473876.


Subject(s)
Defibrillators, Implantable , Electric Countershock , Propensity Score , Registries , Humans , Female , Male , Middle Aged , Aged , Sex Factors , Electric Countershock/instrumentation , Electric Countershock/adverse effects , Risk Factors , Treatment Outcome , Arrhythmias, Cardiac/therapy , Risk Assessment , Europe , Time Factors , Death, Sudden, Cardiac/prevention & control
5.
Ther Umsch ; 81(2): 54-59, 2024 Apr.
Article in German | MEDLINE | ID: mdl-38780211

ABSTRACT

INTRODUCTION: Arrhythmias manifest frequently in individuals with heart failure, posing a notable threat of mortality and morbidity. While the prevention of sudden cardiac death through ICD therapy remains pivotal, accurate risk stratification remains a challenging task even in 2024. Recent data underscore the early consideration of catheter ablation for ventricular tachycardias. Although antiarrhythmic drug therapy serves as an ancillary measure for symptomatic patients, it does not confer prognostic advantages. The holistic management of arrhythmias in heart failure necessitates a systematic, multidimensional approach that initiates with evidence-based medical therapy for heart failure and integrates device-based and interventional therapies. Noteworthy clinical studies have illustrated the positive prognostic impact of early rhythm control strategies, particularly catheter ablation, in individuals managing heart failure and atrial fibrillation.


Subject(s)
Catheter Ablation , Heart Failure , Heart Failure/therapy , Heart Failure/diagnosis , Humans , Catheter Ablation/methods , Anti-Arrhythmia Agents/therapeutic use , Defibrillators, Implantable , Arrhythmias, Cardiac/therapy , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Death, Sudden, Cardiac/etiology , Prognosis , Combined Modality Therapy , Atrial Fibrillation/therapy , Atrial Fibrillation/diagnosis , Atrial Fibrillation/complications , Evidence-Based Medicine , Tachycardia, Ventricular/therapy , Tachycardia, Ventricular/diagnosis
6.
J Bodyw Mov Ther ; 38: 417-424, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38763587

ABSTRACT

BACKGROUND: Heart disease is the leading cause of death in many countries around the world and is linked to numerous complications. In addition to conventional pharmacological treatments, complementary and alternative medicines like massage and dry cupping are employed to help manage the disease and its symptoms. This study aimed to compare the effects of massage and dry cupping on dysrhythmia in patients with heart diseases. METHODS: This randomized parallel controlled clinical trial study was conducted in two critical care units of Shafa hospital in Kerman, southeastern Iran, in 2019-2020. A total of 90 eligible patients were allocated into three groups: massage (n = 30), dry cupping (n = 30), and control (n = 30) using a stratified block randomization method. In the massage group, the head and face were massaged for three consecutive nights, while the dry cupping group received dry cupping between the fifth cervical vertebra and the second thoracic vertebra for the same duration. Each intervention session lasted 15 min. Data collection tools included a socio-demographic and clinical characteristics questionnaire, a form for hemodynamic parameters (systolic blood pressure, diastolic blood pressure, heart rate, respiratory rate, and peripheral oxygen saturation), and a form for assessing dysrhythmia using electrocardiogram readings. Dysrhythmia in the participants was evaluated after each session. RESULTS: The results within each group indicated a statistically significant difference in the prevalence of dysrhythmia in the dry cupping group after the intervention (P < 0.05), but this difference was not statistically significant in the massage and control groups. However, when comparing between the groups, no significant difference was found among the three groups. Additionally, there was no significant difference in the type of dysrhythmia between the groups (P > 0.05). CONCLUSION: While there was no difference in the type of dysrhythmia between the three groups, the additional reduction of dysrhythmia in the dry cupping group could hold clinical significance. Further studies are recommended to validate or refute the findings of the present study.


Subject(s)
Arrhythmias, Cardiac , Massage , Humans , Male , Female , Middle Aged , Massage/methods , Adult , Arrhythmias, Cardiac/therapy , Heart Rate/physiology , Cupping Therapy/methods , Aged , Heart Diseases , Iran , Blood Pressure/physiology
8.
Medicine (Baltimore) ; 103(18): e37990, 2024 May 03.
Article in English | MEDLINE | ID: mdl-38701276

ABSTRACT

Brugada syndrome (BS) is characterized by ST segment elevation in right precordial leads (V1-V3), ventricular tachycardia (VT), ventricular fibrillation (VF), and sudden cardiac death (SCD) in individuals without structural heart disease. The aim of this study is to contribute to the controversial issue of finding the most valuable marker that can predict poor prognosis during follow-up in patients with a diagnosis of BS. A total of 68 patients diagnosed with BS or had Brugada-type ECG change between January 1997 and July 2012 at the Department of Cardiology of Baskent University Faculty of Medicine, Ankara, Turkey, were included in this cohort study. Patients were screened every 6 months for arrhythmia-related syncope, SCD, appropriate and inappropriate defibrillation (shock), AF development and death; collectively defined as "arrhythmic events" and were the primary endpoints. Patients with and without arrhythmic events were compared. The mean age was 34.9 ±â€…12.2 years (9-71 years), and 52 (76.5%) patients were male. Mean follow-up was 49.6 ±â€…37.6 months (4-188 months). Univariate analysis showed that male sex (P = .004), type 1 electrocardiographic pattern (P = .008), SCD (P = .036), VT/VF history (P = .046), requirement for electrophysiological studies (P = .034), implantable cardioverter-defibrillator placement (P = .014) were found to demonstrate significant differences in patients with and without arrhythmic events. In multivariable analyzes, spontaneous type 1 ECG presence (HR = 8.54, 95% CI: 0.38-26.37; P = .003) and VT/VF history (HR = 9.21, 95% CI: 0.004-1.88; P = .002) were found to be independently associated with arrhythmic events. We found the presence of spontaneous type 1 ECG and a history of VT/VF to be associated with increased likelihood of overall arrhythmic events in BS. Given the higher risk of poor prognosis, we recommend additional measures in patients with BS who have these features.


Subject(s)
Brugada Syndrome , Death, Sudden, Cardiac , Electrocardiography , Humans , Brugada Syndrome/therapy , Brugada Syndrome/complications , Brugada Syndrome/physiopathology , Male , Female , Adult , Middle Aged , Risk Factors , Follow-Up Studies , Death, Sudden, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Death, Sudden, Cardiac/epidemiology , Adolescent , Young Adult , Aged , Child , Turkey/epidemiology , Prognosis , Defibrillators, Implantable , Ventricular Fibrillation/therapy , Ventricular Fibrillation/etiology , Ventricular Fibrillation/complications , Arrhythmias, Cardiac/etiology , Arrhythmias, Cardiac/therapy
9.
Europace ; 26(5)2024 May 02.
Article in English | MEDLINE | ID: mdl-38693772

ABSTRACT

AIMS: Arrhythmia-induced cardiomyopathy (AiCM) represents a subtype of acute heart failure (HF) in the context of sustained arrhythmia. Clear definitions and management recommendations for AiCM are lacking. The European Heart Rhythm Association Scientific Initiatives Committee (EHRA SIC) conducted a survey to explore the current definitions and management of patients with AiCM among European and non-European electrophysiologists. METHODS AND RESULTS: A 25-item online questionnaire was developed and distributed among EP specialists on the EHRA SIC website and on social media between 4 September and 5 October 2023. Of the 206 respondents, 16% were female and 61% were between 30 and 49 years old. Most of the respondents were EP specialists (81%) working at university hospitals (47%). While most participants (67%) agreed that AiCM should be defined as a left ventricular ejection fraction (LVEF) impairment after new onset of an arrhythmia, only 35% identified a specific LVEF drop to diagnose AiCM with a wide range of values (5-20% LVEF drop). Most respondents considered all available therapies: catheter ablation (93%), electrical cardioversion (83%), antiarrhythmic drugs (76%), and adjuvant HF treatment (76%). A total of 83% of respondents indicated that adjuvant HF treatment should be started at first HF diagnosis prior to antiarrhythmic treatment, and 84% agreed it should be stopped within six months after LVEF normalization. Responses for the optimal time point for the first LVEF reassessment during follow-up varied markedly (1 day-6 months after antiarrhythmic treatment). CONCLUSION: This EHRA Survey reveals varying practices regarding AiCM among physicians, highlighting a lack of consensus and heterogenous care of these patients.


Subject(s)
Arrhythmias, Cardiac , Cardiomyopathies , Humans , Arrhythmias, Cardiac/therapy , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/physiopathology , Female , Male , Cardiomyopathies/therapy , Cardiomyopathies/diagnosis , Cardiomyopathies/physiopathology , Middle Aged , Adult , Europe , Surveys and Questionnaires , Stroke Volume , Health Care Surveys , Anti-Arrhythmia Agents/therapeutic use , Practice Patterns, Physicians'/statistics & numerical data , Ventricular Function, Left , Catheter Ablation , Cardiologists
10.
J Am Coll Cardiol ; 83(22): 2214-2232, 2024 Jun 04.
Article in English | MEDLINE | ID: mdl-38811098

ABSTRACT

Arrhythmias frequently accompany heart failure and left ventricular dysfunction. Tachycardias, atrial fibrillation, and premature ventricular contractions can induce a reversible form of dilated cardiomyopathy (CM) known as arrhythmia-induced CM (AiCM). The intriguing question is why certain individuals are more susceptible to AiCM, despite similar arrhythmia burdens. The primary challenge is determining the extent of arrhythmias' contribution to left ventricular systolic dysfunction. AiCM should be considered in patients with a mean heart rate of >100 beats/min, atrial fibrillation, or a PVC burden of >10%. Confirmation of AiCM occurs when CM reverses upon eliminating the responsible arrhythmia. Therapy choice depends on the specific arrhythmia, patient comorbidities, and preferences. After left ventricular function is restored, ongoing follow-up is essential if an abnormal myocardial substrate persists. Accurate diagnosis and treatment of AiCM have the potential to enhance patients' quality of life, improve clinical outcomes, and reduce hospital admissions and overall health care costs.


Subject(s)
Arrhythmias, Cardiac , Humans , Arrhythmias, Cardiac/etiology , Arrhythmias, Cardiac/therapy , Arrhythmias, Cardiac/physiopathology , Cardiomyopathies/etiology , Cardiomyopathies/therapy , Cardiomyopathies/physiopathology , Cardiomyopathies/diagnosis , Cardiomyopathy, Dilated/therapy , Cardiomyopathy, Dilated/physiopathology , Cardiomyopathy, Dilated/etiology
12.
J Am Heart Assoc ; 13(11): e031632, 2024 Jun 04.
Article in English | MEDLINE | ID: mdl-38804208

ABSTRACT

BACKGROUND: Pregnancy in patients with pulmonary hypertension (PH) is associated with a heightened risk of medical complications including right heart failure, pulmonary edema, and arrhythmias. Our study investigated the association between PH and these complications during delivery. METHODS AND RESULTS: The National Inpatient Sample was used to identify delivery hospitalizations from 2011 to 2020. Multivariable logistic regression was performed to study the association of PH with the primary outcomes of in-hospital medical and obstetric complications. A total of 37 482 207 delivery hospitalizations in women ≥18 years of age were identified, of which 9593 patients had PH. Pregnant patients with PH had higher incidence of complications during delivery including preeclampsia/eclampsia, arrhythmias, and pulmonary edema among others, compared with those without PH. Pregnant patients with PH also had a higher incidence of in-hospital mortality compared with those without PH (0.51% versus 0.007%). In propensity-matched analyses, PH was still significantly associated with a higher risk of in-hospital mortality (odds ratio [OR], 5.02 [95% CI, 1.82-13.90]; P=0.001), pulmonary edema (OR, 9.11 [95% CI, 6.34-13.10]; P<0.001), peripartum cardiomyopathy (OR, 1.85 [95% CI, 1.37-2.50]; P<0.001), venous thromboembolism (OR, 12.60 [95% CI, 6.04-26.10]; P<0.001), cardiac arrhythmias (OR, 6.11 [95% CI, 4.97-7.53]; P<0.001), acute kidney injury (OR, 3.72 [95% CI, 2.86-4.84]; P<0.001), preeclampsia/eclampsia (OR, 2.24 [95% CI, 1.95-2.58]; P<0.001), and acute coronary syndrome (OR, 2.01 [95% CI, 1.06-3.80]; P=0.03), compared with pregnant patients without PH. CONCLUSIONS: Delivery hospitalizations in patients with PH are associated with a high risk of mortality, pulmonary edema, peripartum cardiomyopathy, venous thromboembolism, arrhythmias, acute kidney injury, preeclampsia/eclampsia, and acute coronary syndrome.


Subject(s)
Hospital Mortality , Hospitalization , Hypertension, Pulmonary , Pregnancy Complications, Cardiovascular , Humans , Female , Pregnancy , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/mortality , Hypertension, Pulmonary/therapy , Adult , United States/epidemiology , Hospitalization/statistics & numerical data , Hospitalization/trends , Pregnancy Complications, Cardiovascular/epidemiology , Pregnancy Complications, Cardiovascular/therapy , Hospital Mortality/trends , Incidence , Young Adult , Risk Factors , Retrospective Studies , Delivery, Obstetric/statistics & numerical data , Delivery, Obstetric/adverse effects , Pulmonary Edema/epidemiology , Pulmonary Edema/etiology , Pulmonary Edema/mortality , Arrhythmias, Cardiac/epidemiology , Arrhythmias, Cardiac/therapy , Arrhythmias, Cardiac/mortality , Risk Assessment
13.
J Am Heart Assoc ; 13(11): e032465, 2024 Jun 04.
Article in English | MEDLINE | ID: mdl-38804218

ABSTRACT

BACKGROUND: New methods to identify patients who benefit from a primary prophylactic implantable cardioverter-defibrillator (ICD) are needed. T-wave alternans (TWA) has been shown to associate with arrhythmogenesis of the heart and sudden cardiac death. We hypothesized that TWA might be associated with benefit from ICD implantation in primary prevention. METHODS AND RESULTS: In the EU-CERT-ICD (European Comparative Effectiveness Research to Assess the Use of Primary Prophylactic Implantable Cardioverter-Defibrillators) study, we prospectively enrolled 2327 candidates for primary prophylactic ICD. A 24-hour Holter monitor reading was taken from all recruited patients at enrollment. TWA was assessed from Holter monitoring using the modified moving average method. Study outcomes were all-cause death, appropriate shock, and survival benefit. TWA was assessed both as a contiguous variable and as a dichotomized variable with cutoff points <47 µV and <60 µV. The final cohort included 1734 valid T-wave alternans samples, 1211 patients with ICD, and 523 control patients with conservative treatment, with a mean follow-up time of 2.3 years. TWA ≥60 µV was a predicter for a higher all-cause death in patients with an ICD on the basis of a univariate Cox regression model (hazard ratio, 1.484 [95% CI, 1.024-2.151]; P=0.0374; concordance statistic, 0.51). In multivariable models, TWA was not prognostic of death or appropriate shocks in patients with an ICD. In addition, TWA was not prognostic of death in control patients. In a propensity score-adjusted Cox regression model, TWA was not a predictor of ICD benefit. CONCLUSIONS: T-wave alternans is poorly prognostic in patients with a primary prophylactic ICD. Although it may be prognostic of life-threatening arrhythmias and sudden cardiac death in several patient populations, it does not seem to be useful in assessing benefit from ICD therapy in primary prevention among patients with an ejection fraction of ≤35%.


Subject(s)
Death, Sudden, Cardiac , Defibrillators, Implantable , Electrocardiography, Ambulatory , Primary Prevention , Humans , Primary Prevention/methods , Male , Female , Death, Sudden, Cardiac/prevention & control , Death, Sudden, Cardiac/etiology , Middle Aged , Aged , Prospective Studies , Electrocardiography, Ambulatory/methods , Electric Countershock/instrumentation , Electric Countershock/adverse effects , Risk Assessment/methods , Risk Factors , Arrhythmias, Cardiac/therapy , Arrhythmias, Cardiac/physiopathology , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/prevention & control , Arrhythmias, Cardiac/mortality , Treatment Outcome , Predictive Value of Tests , Time Factors , Europe/epidemiology , Prognosis , Heart Rate/physiology
16.
Europace ; 26(4)2024 Mar 30.
Article in English | MEDLINE | ID: mdl-38558121

ABSTRACT

AIMS: Recently, a genetic variant-specific prediction model for phospholamban (PLN) p.(Arg14del)-positive individuals was developed to predict individual major ventricular arrhythmia (VA) risk to support decision-making for primary prevention implantable cardioverter defibrillator (ICD) implantation. This model predicts major VA risk from baseline data, but iterative evaluation of major VA risk may be warranted considering that the risk factors for major VA are progressive. Our aim is to evaluate the diagnostic performance of the PLN p.(Arg14del) risk model at 3-year follow-up. METHODS AND RESULTS: We performed a landmark analysis 3 years after presentation and selected only patients with no prior major VA. Data were collected of 268 PLN p.(Arg14del)-positive subjects, aged 43.5 ± 16.3 years, 38.9% male. After the 3 years landmark, subjects had a mean follow-up of 4.0 years (± 3.5 years) and 28 (10%) subjects experienced major VA with an annual event rate of 2.6% [95% confidence interval (CI) 1.6-3.6], defined as sustained VA, appropriate ICD intervention, or (aborted) sudden cardiac death. The PLN p.(Arg14del) risk score yielded good discrimination in the 3 years landmark cohort with a C-statistic of 0.83 (95% CI 0.79-0.87) and calibration slope of 0.97. CONCLUSION: The PLN p.(Arg14del) risk model has sustained good model performance up to 3 years follow-up in PLN p.(Arg14del)-positive subjects with no history of major VA. It may therefore be used to support decision-making for primary prevention ICD implantation not merely at presentation but also up to at least 3 years of follow-up.


Subject(s)
Arrhythmias, Cardiac , Defibrillators, Implantable , Female , Humans , Male , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/genetics , Arrhythmias, Cardiac/therapy , Calcium-Binding Proteins/genetics , Death, Sudden, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Reproducibility of Results , Risk Factors , Adult , Middle Aged
17.
Ital J Pediatr ; 50(1): 67, 2024 Apr 14.
Article in English | MEDLINE | ID: mdl-38616285

ABSTRACT

BACKGROUND: Carnitine palmitoyltransferase II (CPT II) deficiency is a rare inborn error of mitochondrial fatty acid metabolism with autosomal recessive pattern of inheritance. Its phenotype is highly variable (neonatal, infantile, and adult onset) on the base of mutations of the CPT II gene. In affected subjects, long-chain acylcarnitines cannot be subdivided into carnitine and acyl-CoA, leading to their toxic accumulation in different organs. Neonatal form is the most severe, and all the reported patients died within a few days to 6 months after birth. Hereby, we report on a male late-preterm newborn who presented refractory cardiac arrhythmias and acute multiorgan (hepatic, renal, muscular) injury, leading to cerebral hemorrhage, hydrocephalus, cardiovascular failure and early (day 5 of life) to death. Subsequently, extended metabolic screening and target next generation sequencing (NGS) analysis allowed the CPT II deficiency diagnosis. CASE PRESENTATION: The male proband was born at 36+ 4 weeks of gestation by spontaneous vaginal delivery. Parents were healthy and nonconsanguineous, although both coming from Nigeria. Family history was unremarkable. Apgar score was 9/9. At birth, anthropometric measures were as follows: weight 2850 g (47th centile, -0.07 standard deviations, SD), length 50 cm (81st centile, + 0.89 SD) and occipitofrontal circumference (OFC) 35 cm (87th centile, + 1.14 SD). On day 2 of life our newborn showed bradycardia (heart rate around 80 bpm) and hypotonia, and was then transferred to the Neonatal Intensive Care Unit (NICU). There, he subsequently manifested many episodes of ventricular tachycardia, which were treated with pharmacological (magnesium sulfate) and electrical cardioversion. Due to the critical conditions of the baby (hepatic, renal and cardiac dysfunctions) and to guarantee optimal management of the arrythmias, he was transferred to the Pediatric Cardiology Reference Center of our region (Sicily, Italy), where he died 2 days later. Thereafter, the carnitines profile evidenced by the extended metabolic screening resulted compatible with a fatty acid oxidation defect (increased levels of acylcarnitines C16 and C18, and low of C2); afterwards, the targeted next generation sequencing (NGS) analysis revealed the known c.680 C > T p. (Pro227Leu) homozygous missense mutation of the CPTII gene, for diagnosis of CPT II deficiency. Genetic investigations have been, then, extended to the baby's parents, who were identified as heterozygous carriers of the same variant. When we meet again the parents for genetic counseling, the mother was within the first trimester of her second pregnancy. Therefore, we offered to the couple and performed the prenatal target NGS analysis on chorionic villi sample, which did not detect any alterations, excluding thus the CPT II deficiency in their second child. CONCLUSIONS: CPTII deficiency may be suspected in newborns showing cardiac arrhythmias, associated or not with hypertrophic cardiomyopathy, polycystic kidneys, brain malformations, hepatomegaly. Its diagnosis should be even more suspected and investigated in cases of increased plasmatic levels of creatine phosphokinase and acylcarnitines in addition to kidney, heart and liver dysfunctions, as occurred in the present patient. Accurate family history, extended metabolic screening, and multidisciplinary approach are necessary for diagnosis and adequate management of affected subjects. Next generation sequencing (NGS) techniques allow the identification of the CPTII gene mutation, essential to confirm the diagnosis before or after birth, as well as to calculate the recurrence risk for family members. Our report broads the knowledge of the genetic and molecular bases of such rare disease, improving its clinical characterization, and provides useful indications for the treatment of patients.


Subject(s)
Arrhythmias, Cardiac , Carnitine O-Palmitoyltransferase , Carnitine O-Palmitoyltransferase/deficiency , Metabolism, Inborn Errors , Infant, Newborn , Adult , Infant , Child , Female , Pregnancy , Humans , Male , Carnitine O-Palmitoyltransferase/genetics , Fatal Outcome , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/genetics , Arrhythmias, Cardiac/therapy , Fatty Acids , Sicily
18.
BMC Cardiovasc Disord ; 24(1): 218, 2024 Apr 23.
Article in English | MEDLINE | ID: mdl-38654151

ABSTRACT

BACKGROUND: The coexistence of cardiac arrhythmias in patients with acute myocardial infarction (AMI) usually exhibits poor prognosis. However, there are few contemporary data available on the burden of cardiac arrhythmias in AMI patients and their impact on in-hospital outcomes. METHODS: The present study analyzed data from the China Acute Myocardial Infarction (CAMI) registry involving 23,825 consecutive AMI patients admitted to 108 hospitals from January 2013 to February 2018. Cardiac arrhythmias were defined as the presence of bradyarrhythmias, sustained atrial tachyarrhythmias, and sustained ventricular tachyarrhythmias that occurred during hospitalization. In-hospital outcome was defined as a composite of all-cause mortality, cardiogenic shock, re-infarction, stroke, or heart failure. RESULTS: Cardiac arrhythmia was presented in 1991 (8.35%) AMI patients, including 3.4% ventricular tachyarrhythmias, 2.44% bradyarrhythmias, 1.78% atrial tachyarrhythmias, and 0.73% ≥2 kinds of arrhythmias. Patients with arrhythmias were more common with ST-segment elevation myocardial infarction (83.3% vs. 75.5%, P < 0.001), fibrinolysis (12.8% vs. 8.0%, P < 0.001), and previous heart failure (3.7% vs. 1.5%, P < 0.001). The incidences of in-hospital outcomes were 77.0%, 50.7%, 43.5%, and 41.4%, respectively, in patients with ≥ 2 kinds of arrhythmias, ventricular tachyarrhythmias, bradyarrhythmias, and atrial tachyarrhythmias, and were significantly higher in all patients with arrhythmias than those without arrhythmias (48.9% vs. 12.5%, P < 0.001). The presence of any kinds of arrhythmia was independently associated with an increased risk of hospitalization outcome (≥ 2 kinds of arrhythmias, OR 26.83, 95%CI 18.51-38.90; ventricular tachyarrhythmias, OR 8.56, 95%CI 7.34-9.98; bradyarrhythmias, OR 5.82, 95%CI 4.87-6.95; atrial tachyarrhythmias, OR4.15, 95%CI 3.38-5.10), and in-hospital mortality (≥ 2 kinds of arrhythmias, OR 24.44, 95%CI 17.03-35.07; ventricular tachyarrhythmias, OR 13.61, 95%CI 10.87-17.05; bradyarrhythmias, OR 7.85, 95%CI 6.0-10.26; atrial tachyarrhythmias, OR 4.28, 95%CI 2.98-6.16). CONCLUSION: Cardiac arrhythmia commonly occurred in patients with AMI might be ventricular tachyarrhythmias, followed by bradyarrhythmias, atrial tachyarrhythmias, and ≥ 2 kinds of arrhythmias. The presence of any arrhythmias could impact poor hospitalization outcomes. REGISTRATION: Clinical Trial Registration: Identifier: NCT01874691.


Subject(s)
Arrhythmias, Cardiac , Hospital Mortality , Registries , Humans , Male , Female , China/epidemiology , Middle Aged , Aged , Arrhythmias, Cardiac/epidemiology , Arrhythmias, Cardiac/diagnosis , Arrhythmias, Cardiac/mortality , Arrhythmias, Cardiac/therapy , Risk Factors , Risk Assessment , Time Factors , Myocardial Infarction/mortality , Myocardial Infarction/epidemiology , Myocardial Infarction/diagnosis , Myocardial Infarction/therapy , Myocardial Infarction/complications , Hospitalization , Prognosis , Recurrence , ST Elevation Myocardial Infarction/therapy , ST Elevation Myocardial Infarction/mortality , ST Elevation Myocardial Infarction/diagnosis , ST Elevation Myocardial Infarction/epidemiology , ST Elevation Myocardial Infarction/complications , Aged, 80 and over
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