High Risk Of Sudden Death Research Articles

Sudden cardiac death in a patient with complicated peripartum cardiomyopathy, long QT syndrome and mutation in <I>FLNC</I> gene

Peripartum cardiomyopathy (PPCM) is a diagnosis of exclusion in women presenting with heart failure due to left ventricular (LV) systolic dysfunction. PPCM should be considered in case of unknown etiology of heart failure during pregnancy or after childbirth. Long QT syndrome is a primary electrical heart disease associated with a prolonged QT interval on the ECG, recurrent paroxysms of ventricular tachycardia, and a high risk of sudden death. Our aim was to demonstrate a case of cardiomyopathy in combination with long QT syndrome in a patient with a mutation in the FLNC gene. A 38-years-old woman was hospitalized 4,5 months after childbirth after sudden cardiac arrest and successful cardiopulmonary resuscitation. Long QT interval was revealed on the electrocardiogram. Echocardiography registered an akinesis of the apical and middle segments of the anterior wall of the left ventricle and interventricular septum, apex, left ventricular ejection fraction – 32%. Coronary angiography revealed no stenotic lesion of the coronary arteries. N-terminal precursor of brain natriuretic peptide (NTproBNP) was 33300 mg/l. Mass parallel sequencing of 17 genes revealed the nucleotide variant c.1609T>G (chr7:128480661T>G, NM_001488.4; rs760471547) in a heterozygous state in exon 10 of the FLNC gene (OMIM 102565), leading to the amino acid variant p.Y537D.The combination of peripartum cardiomyopathy and long QT syndrome may increase the likelihood of sudden cardiac death, especially in individuals with a genetic mutation of cardiomyopathy. Timely diagnosis of the described conditions is necessary to prevent complications and increase the life expectancy of patients.

DIAGNOSTIC UTILITY OF HOLTER MONITORING IN CATECHOLAMINERGIC POLYMORPHIC VENTRICULAR TACHYCARDIA

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare genetic arrhythmia predisposing to a high risk of sudden death during periods of adrenergic stress. The diagnosis hinges on the exercise stress test, which induces progressive polymorphic and bidirectional ventricular arrhythmias in affected patients. Genetic testing usually identifies a missense variant in the cardiac ryanodine receptor (RyR2). When arrhythmia symptoms are reported by patients, it is also common to perform a Holter monitor, which may identify ventricular arrhythmias.

Importance of Formula-Specific Centile Thresholds for Evaluation of Heart Rate-Corrected QT Prolongation in Williams Syndrome

Patients with Williams syndrome (WS) have a 25- to 100-fold higher risk of sudden death and prolonged heart rate-corrected QT (QTc). A recent study using the Fridericia formula for QT correction suggested that prolongation is principally an issue of heart rate. We used multiple published heart rate correction formulas to reevaluate the prevalence of QTc prolongation in our original dataset from our 2010 study at the Children's Hospital of Philadelphia. The ninety-eighth centile for QTc and corrected JT Interval (JTc) of the control population for each formula were used to set the threshold for prolongation. Prevalence comparison was done with Fisher's exact test. Predictors of longer QTc/JTc were assessed using linear regression models adjusting for age, gender, and heart rate. Adjusted odds of QTc/JTc prolongation were evaluated with conditional logistic regression models matched based on age and heart rate. There were 482 electrocardiograms from 188 patients with WS and 1,522 from normal controls. Patients with WS were younger, with higher heart rates and shorter RR and QRS intervals. WS was associated with longer QTc/JTc compared with controls. There were higher odds of prolonged QTc/JTc in patients with WS than controls using both Bazett and Fridericia formulas. In conclusion, this study confirms the higher prevalence of QTc prolongation in WS compared with controls and highlights the importance of setting appropriate formula-specific upper thresholds for QTc prolongation for accurate diagnosis.

The Case for Home AED in Children, Adolescents, and Young Adults NotMeeting Criteria for ICD.

Children, adolescents, and young adults with conditions such as cardiomyopathies and channelopathies are at higher risk of sudden cardiac death caused by lethal arrhythmias, especially ventricular fibrillation. Timely defibrillation saves lives. Patients thought to be at significantly high risk of sudden death typically undergo placement of an implantable cardioverter-defibrillator. Patients thought to be at lower risk are typically followed medically but do not undergo implantable cardioverter-defibrillator placement. However, low risk does not equal no risk. Compared with the general population, many of these patients are at significantly higher risk for lethal arrhythmias. We make the case that such individuals and families will benefit from having an at-home automatic external defibrillator. Used in conjunction with conventional measures such as training on cardiopulmonary resuscitation, an at-home automatic external defibrillator could lead to significantly shortened time to defibrillation with better overall and neurological survival. We recommend that the cost of such home automatic external defibrillators should be covered by medical insurance.

Establishment of an induced pluripotent stem cell line (ZJULLi004-A) from a hypertrophic cardiomyopathy patient carrying MYBPC3/c.3764C>A mutation

Hypertrophic cardiomyopathy (HCM) is an inherited cardiovascular disease characterized by left ventricular hypertrophy and a high risk of sudden death. In this study, a skin biopsy was obtained from a HCM patient harboring a heterozygous missense mutation (c.3764C>A; p.A1225D) in the myosin binding protein C3 (MYBPC3) gene. The isolated fibroblasts were reprogrammed using non-integrated Sendai viral method to establish the patient-specific induced pluripotent stem cell (iPSC) line. The established iPSC line displayed normal morphology and karyotype, expressed pluripotency markers, and can differentiate into three germ layers in vivo.

ARTIFICIAL INTELLIGENCE ANALYSIS OF AMBULATORY ECG MONITORING IN SYNCOPE INDICATED PATIENTS

Patients (pts) with a history of syncope are at high risk for sudden death with a reported 1-year mortality rate of 30%. Identifying arrhythmias (ars) in this patient population is critical for timely diagnosis and potential life-saving intervention. Recently, we reported a high incidence of atrial arrhythmia in the syncope indicated population receiving ambulatory ECG monitoring.

In Memoriam: Morton Mower, MD, January 31, 1933–April 25, 2022

On April 25, 2022, our profession lost Dr Morton M. Mower at the age of 89. He was one of cardiology’s preeminent inventors. In the 1960s Dr Mower, with his partner, Dr Michel Mirowski, conceptualized the need for an AICD to treat sudden death patients. They went on to prove the efficacy of this device in a series of carefully designed randomized clinical trials in patients at high risk of sudden death.

Звено патогенеза ночной внезапной смерти при сердечной недостаточности

Patients with chronic heart failure (CHF) constitute the bulk of the group at the highest risk of sudden death (SD). The majority of SDs occur at night. However, CHF grade and ejection fraction do not always determine the risk of SD in the outcome of the disease. The following view has been expressed based on the research on the topic and the described mechanisms underlying SD: impaired QT interval adaptation (“hyperadaptation”: QT/RR slope > 0.24) to HR in patients with CHF who show maximum QT interval prolongation during the night, capable of triggering life-threatening ventricular tachyarrhythmias that trigger the mechanism of SD associated with CHF, can play some role. It is possible that identification of QT interval hyperadaptation in patients with CHF makes it possible to form the group at high risk of SD associated with HF and can become an additional indication for implantation of cardioverter-defibrillator.

The long-term efficacy of left cardiac sympathetic denervation in long QT syndrome

Objective: To investigate the long-term efficacy and safety of left cardiac sympathetic denervation(LCSD) for long QT syndrome(LQTS) patients with either recurrence on drug therapy intolerance/refusal. Methods: This study was a retrospective cohort study. The cases selected from 193 patients with LQTS who were enrolled in the Chinese Channelopathy Registry Study from November 1999 to November 2012. This study selected 28 LQTS patients with either recurrence on drug therapy intolerance/refusal and underwent LCSD surgery in the Peking University People's Hospital or Beijing Tongren Hospital. The patients were allocated into 3 groups: high-risk group(n=13, baseline QTc ≥550 ms or symptomatic in the first year of life or highly malignant genetics); intermediate-risk group(n=10, 500 ms≤baseline QTc<550 ms, symptomatic after the first year and without highly malignant genetics); low-risk group(n=5, baseline QTc<500 ms, symptomatic after the first year and without highly malignant genetics). LCSD was performed with the traditional supraclavicular approach or video assisted thoracoscopic surgery (VATS). Patients were regularly followed up until 20 years after the surgery. Data were collected before and 1 year after surgery and at the last follow-up. Patients' electrocardiograph(ECG), cardiac events and surgery-related complications were recorded. Kaplan-Meier survival analysis was used to determine the cardiac event-free survival based on different risk stratification and genotypes. Results: A total of 28 LQTS patients, aged 20.5 (15.0, 37.5) and underwent LCSD surgery, were enrolled in this study, including 23(82.1%) women. There were 11(39.3%) patients treated with traditional approach while 17(60.7%) with VATS-LCSD. There were 19(67.9%) patients had positive genetic test results, including 4 LQT1, 12 LQT2, 1 LQT1/LQT2 mixed type, and 2 Jervell-Lange-Nielsen (JLN) syndrome. The median follow-up period was 189.3(138.7, 204.9) months. The dropout rate was 10.7%(3/28) while 3 patients in the intermediate-risk group were lost to follow-up. Horner syndrome occurred in 1 patient (in the high-risk group). Sudden cardiac deaths were observed in 3 (12.0%) patients (all in the high-risk group), and 12 patients (48.0%) had syncope recurrences (2 in low-risk group, 3 in intermediate-risk group and 7 in high-risk group). A significant reduction in the mean yearly episodes of cardiac events was observed, from (3.5±3.3) before LCSD to(0.2±0.1) at one year after LCSD and (0.5±0.8) at last follow up(P<0.001). The mean QTc was shortened from (545.7±51.2)ms before the surgery to (489.0±40.1)ms at the last follow-up (P<0.001). Among the 20 patients with basic QTc ≥500 ms and completing the follow-up, the QTc intervals of 11(55.0%) patients were shortened to below 500 ms. The event free survival rates for any cardiac events after LCSD decreased sequentially in the low-, intermediate- and high-risk groups, and the difference was statistically significant (χ²=7.24, log-rank P=0.026). No difference was found in the event free survival rates among LQT1, LQT2 and undefined gene patients (χ²=5.20, log-rank P>0.05). Conclusions: LCSD surgery can reduce the incidence of cardiac events and shorten the QTc interval in patients with LQTS after the long-term follow-up. LCSD surgery is effective and safe for patients with LQTS ineffective or intolerant to drug therapy. However, high-risk patients are still at a high risk of sudden death after surgery and should be actively monitored and protected by combined therapies.

P22 USE OF THE LIFE–VEST IN PATIENTS WITH HIGH ARRHYTHMIC RISK: EXPERIENCE OF A SPOKE CENTER IN THE COVID–19 PERIOD

Abstract Background Patients with newly found dilated heart disease have a high risk of sudden death. During the COVID period, the follow–up of these patients was difficult due to the limitation of access to the hospital and the impossibility of performing tests with high decision–making power (cardiac MRI) at third–level hospitals. Purpose To evaluate the use of the Life–Vest in patients with newly found dilated heart disease as a protection system for early discharge and the related cost/benefit ratio in relation to an early ICD implant. Methods In the COVID period, a Life–Vest was applied to 18 patients with newly found dilated heart disease (4 post ischemic and 16 without coronary artery disease), to monitoring ventricular arrhythmias and to protect them against any life–threatening ventricular tachycardias. These patients showed an high arrhythmic risk for ventricular tachycardias, (VT found on monitoring) and an unfavorable echocardiographic aspect. Each week, the patient‘s telemetry was remotely viewed and a telephone assessment was performed for clinical conditions. A control echocardiogram was performed at 30 days to evaluate the FE and the possible continuation of monitoring. Results Of the 18 patients (mean age 59 years) analyzed, 5 (28%) underwent ICD implantation for persistent severe reduction in FE during 3 months after diagnosis and 13 (72%) normalized FE (duration average follow–up 50 days); there are no significant differences between the postischemic and non–postischemic DCM groups. The cost of renting the Life–Vest is about 4000 euros for 40 days and the average cost for an ICD implant, considering the device and the costs related to the days of hospitalization and use of human resources/advanced technological support, amounts to about 20700 euros. Considering these data, we observed a saving of approximately 261900 euros for 18 observed patients. To this saving must be added the costs related to the reduction of the days of hospitalization (average 5 days) and the costs to any future replacement of the ICD. Conclusion In patients with newly found dilated heart disease at high arrhythmic risk, the use of the Life–Vest reduces the days of hospitalization, allows patients to be discharged safely and generates substantial savings for the National Health System.

Impact Of Left Atrial Volume Index On Syncope In Patients With Hypertrophic Cardiomyopathy

Introduction Syncope commonly occurs in hypertrophic cardiomyopathy patients (HCM), usually without warning or prodrome and is associated with high risk for sudden death. Moreover, the exact mechanism of syncope cannot be identified making it difficult to predict which patients are at greatest risk for syncope. Prior studies have shown left atrial size to be associated with sudden death with markers of left atrial (LA) remodeling seen in advanced stages of HCM. However, it is unclear if LA structural changes correlate with incidence of syncope. Our aim was to evaluate if there is a correlation between left atrial volume index (LAVI) and occurrence of syncope in HCM patients. Methods This was a retrospective study of 123 HCM patients identified at our institution from 2016-2019. Diagnosis of syncope was identified by chart review. Left atrial volume index was determined by tracing the LA endocardial border in both the apical and four and two chamber view by two-dimensional echocardiography. HCM subtypes were defined as obstructive (presence of systolic anterior motion of the mitral valve—SAM), and non-obstructive (apical variant). Results Of 123 patients, 29 (23.6%) had syncope: 25 of the 100 SAM subjects and 4 of the 23 non-obstructive subjects. No difference in age, gender, or BMI was found between those with syncope and without syncope. LAVI showed a correlation with syncope in HCM patients (p< 0.05). In patients with a diagnosis of syncope, mean LAVI was larger compared to those without history of syncope (43.8±12.6 mL/m2 vs. 37.7±14.3 mL/m2). Each 1 mL/m2 increase in LAVI corresponded to a 1.03 OR risk for syncope. ROC analysis showed a LAVI threshold of 31.8mL/m2 for predicting syncope in all HCM (sensitivity 89.7%, specificity 40.4%) (Figure 1). Conclusion Syncope showed a strong correlation in those with larger LAVI with incremental risk of syncope for each 1 mL/m2 added to LAVI size. Our findings suggest LAVI may have utility as a marker to stratify HCM patients at risk for syncope. Further studies in HCM patients are needed to assess the impact of changes in LAVI size over time on cardiovascular outcomes. Syncope commonly occurs in hypertrophic cardiomyopathy patients (HCM), usually without warning or prodrome and is associated with high risk for sudden death. Moreover, the exact mechanism of syncope cannot be identified making it difficult to predict which patients are at greatest risk for syncope. Prior studies have shown left atrial size to be associated with sudden death with markers of left atrial (LA) remodeling seen in advanced stages of HCM. However, it is unclear if LA structural changes correlate with incidence of syncope. Our aim was to evaluate if there is a correlation between left atrial volume index (LAVI) and occurrence of syncope in HCM patients. This was a retrospective study of 123 HCM patients identified at our institution from 2016-2019. Diagnosis of syncope was identified by chart review. Left atrial volume index was determined by tracing the LA endocardial border in both the apical and four and two chamber view by two-dimensional echocardiography. HCM subtypes were defined as obstructive (presence of systolic anterior motion of the mitral valve—SAM), and non-obstructive (apical variant). Of 123 patients, 29 (23.6%) had syncope: 25 of the 100 SAM subjects and 4 of the 23 non-obstructive subjects. No difference in age, gender, or BMI was found between those with syncope and without syncope. LAVI showed a correlation with syncope in HCM patients (p< 0.05). In patients with a diagnosis of syncope, mean LAVI was larger compared to those without history of syncope (43.8±12.6 mL/m2 vs. 37.7±14.3 mL/m2). Each 1 mL/m2 increase in LAVI corresponded to a 1.03 OR risk for syncope. ROC analysis showed a LAVI threshold of 31.8mL/m2 for predicting syncope in all HCM (sensitivity 89.7%, specificity 40.4%) (Figure 1). Syncope showed a strong correlation in those with larger LAVI with incremental risk of syncope for each 1 mL/m2 added to LAVI size. Our findings suggest LAVI may have utility as a marker to stratify HCM patients at risk for syncope. Further studies in HCM patients are needed to assess the impact of changes in LAVI size over time on cardiovascular outcomes.

Arrhythmia prevalence and sudden death risk in adults with the m.3243A>G mitochondrial disorder

AimsTo define the prevalence of non-sustained tachyarrhythmias and bradyarrhythmias in patients with the m.3243A>G mitochondrial genotype and a previously defined, profile, associated with ‘high sudden-death risk’.Methods and resultsPatients at high...

Primary Prevention Implantable Cardioverter-Defibrillators in Adults With Congenital Heart Disease: Recommendations From Professional Societies.

Identifying adults with congenital heart disease (CHD) at high risk for sudden death who stand to benefit from primary prevention implantable cardioverter-defibrillators (ICDs) remains a major challenge. Inconsistencies among evidence-based recommendations by various professional societies can be a source of confusion to practicing clinicians. This article summarizes and compares recommendations from the Canadian Cardiovascular Society management guidelines on adults with CHD (2009), expert consensus from the Pediatric and Congenital Electrophysiology Society and Heart Rhythm Society (2014), European Society of Cardiology (ESC) guidelines on sudden death (2015), position statement from the European Heart Rhythm Association, Association for European Paediatric and Congenital Cardiology, and ESC working group on grown-up CHD (2018), and ESC guidelines on adult CHD (2020). Most recommendations are fundamentally similar, particularly with regards to: 1) recommending or considering primary prevention ICDs in adults with CHD and a systemic left ventricular ejection fraction ≤35%, biventricular physiology, and functional class II or III symptoms despite optimal medical therapy; 2) considering ICDs in those with unexplained syncope of suspected arrhythmic etiology and either advanced ventricular dysfunction or inducible sustained ventricular arrhythmias; and 3) considering ICDs in selected patients with tetralogy of Fallot and multiple risk factors for sudden death, such as left ventricular dysfunction, non-sustained ventricular tachycardia, QRS duration ≥180 ms, right ventricular scarring, or inducible sustained ventricular tachycardia. Areas of continued clinical uncertainty, which offer important opportunities for future research, include improving patient selection for primary prevention ICDs in the context of a systemic right ventricle, univentricular heart, or Eisenmenger syndrome.

The Effect of the Door to Needle Time of Streptokinase Administration on the QTc Interval and the Incidence of Life-Threatening Arrhythmia in Patients With Anterior Myocardial Infarction

Early administration of thrombolytic agents is standard for patients presenting with acute myocardial infarction (MI). Also, prolonged QT intervals indicate a higher risk for sudden death in patients with MI. This study was conducted to evaluate the door to needle time of streptokinase administration and the incidence of life-threatening arrhythmia in patients with anterior MI. This study was a prospective, single-center study on participants with anterior MI, who were divided into streptokinase and non-streptokinase groups. After administration of streptokinase, QTc was measured in hyper-acute, acute, and recent phases of anterior MI in the group and compared with acute and recent phases in the non-streptokinase group. The incidence of life-threatening arrhythmia was measured and compared in two groups. The data were analyzed by descriptive statistics method and variance analysis in the SPSS software, version 22. The level of significance was considered to be 0.05. Among 87 participants, there was a significant relationship between the door to needle time of 30 minutes and QTc interval in the hyperacute phase (P=0.005). Also, QTc in the streptokinase group was significantly lower than the non-streptokinase group in the acute phase (P=0.003 vs. P=0.205) and recent phase (P=0.007 vs. P=0.228). The incidence of fatal arrhythmias in the streptokinase group was lower than in the others. The relationship between the incidence of VT/VF and TIMI flow grade was insignificant (P=0.089). Reduction of the door to needle time after anterior MI has significant effects on QTc and incidence of threatening arrhythmia.

Heart rate variability in Doberman

Purposes: to study the daily rhythms of the heart rate in 20 healthy Doberman dogs; obtaining reference values of time indicators of heart rate variability; study and determination of the type, identification of the predominance of the electrocardiological syndrome in this group of animals.Materials and methods. Due to the high prevalence of the disease: dilated cardiomyopathy (DCM) in Dobermans and a high risk of sudden death associated with the development of ventricular tachycardia, we conducted a study of heart rate variability in 20 clinically healthy Doberman dogs in order to study the electrical activity of the heart, heart rate variability, identify reference values of HRVi parameters, to study and determine the prevalence of which electrocardiological syndrome in this group of animals. The study of circadian rhythm was carried out using 24-hour Holter ECG monitoring on an outpatient basis.Results. When analyzing the daily fluctuations of the intervalogram for every 2 hours, it was found that the maximum heart rate of 90 beats/min is in the range of 16:00–18:00, the minimum is 71 beats/min in the range of 4:00–6:00. Received temporal parameters of heart rate variability. It was registered that the parameters responsible for the general autonomic tone (SDNN, SDANN, HRVi) are higher during the daytime. Parameters indicating high frequency variation and tone of the parasympathetic nervous system (SDNN, rMSSD, SDSD) are higher at night. The predominance of normocardial syndrome in Doberman breed dogs was established during the assessment of the daily intervalogram.

A Case-Control Study of the Dose-Response Relationship Between Thrombin Activatable Fibrinolysis Inhibitor and Acute Myocardial Infarction.

BackgroundAcute myocardial infarction (AMI) is considered an acute coronary syndrome (ACS), which is caused by the death of myocardial cells after prolonged ischemia, and there is a high risk of sudden death during AMI. Therefore, the purpose of this study is to explore the relationship between thrombin activatable fibrinolysis inhibitor (TAFI) and AMI and provide evidence for their association and potentially the prevention of AMI.MethodsThere were 228 subjects included in this retrospective study, which included 78 AMI patients and 150 controls. The immune turbidimetry was used to measure TAFI concentration in the serum. Mann–Whitney U test was used to compare serum TAFI levels. The logistic regression analysis was used to construct a model of influencing factors of AMI. The dose-response relationship between serum TAFI level and AMI was explored by using the restricted cubic spline (RCS) functions combined with logistic regression analysis.ResultsThe serum TAFI levels of the AMI group were higher than the control group’s (P = 0.003). The risk of AMI in the high-TAFI level group was 2.24 times higher than the low-TAFI level group (P = 0.007) and it was 2.74 times higher after adjustment of other risk factors (P = 0.025). According to the dose-response curve, the risk of AMI increased significantly with an increase of serum TAFI concentration (P = 0.0387).ConclusionAcute myocardial infarction patients had higher serum TAFI levels, and TAFI was an independent risk factor for AMI patients. Serum TAFI levels demonstrated a dose- dependent response to the risk of AMI. Our study provides evidence that TAFI could be used for risk stratification of AMI patients.

Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy-The Role of Cardiac MRI.

Arrhythmogenic right ventricular dysplasia (ARVD) is a rare genetic condition of the myocardium, with a significantly high risk of sudden death. Recent genetic research and improved understanding of the pathophysiology tend to change the ARVD definition towards a larger spectrum of myocardial involvement, which includes, in various proportions, both the right (RV) and left ventricle (LV), currently referred to as ACM (arrhythmogenic cardiomyopathy). Its pathological substrate is defined by the replacement of the ventricular myocardium with fibrous adipose tissue that further leads to inadequate electrical impulses and translates into varies degrees of malignant ventricular arrythmias and dyskinetic myocardium movements. Particularly, the cardio-cutaneous syndromes of Carvajal/Naxos represent rare causes of ACM that might be suspected from early childhood. The diagnostic is sometimes challenging, even with well-established rTFC or Padua criteria, especially for pediatric patients or ACM with LV involvement. Cardiac MRI gain more and more importance in ACM diagnostic especially in non-classical forms. Furthermore, MRI is useful in highlighting myocardial fibrosis, fatty replacement or wall movement with high accuracy, thus guiding not only the depiction, but also the patient’s stratification and management.

A neonate with acquired long QT from placental transfer of medications

Long QT Syndrome (LQTS) can present in the neonate with bradycardia and AV block and confers significant risk for life threatening ventricular arrhythmias. We report a case of a neonate presenting with complex bradyarrhythmias secondary to acquired LQTS after maternal overdose of QT-prolonging medications (haloperidol and sertraline). When the mother was brought in to the delivery center unconscious, fetal bradycardia was noted and the baby was emergently delivered prematurely. The newborn’s initial ECG showed bradycardia with AV node conduction block, rate related His-Purkinje conduction aberration with varying QRS morphology and a QTc &gt;600 ms. She continued to be bradycardic through her first day of life with echocardiogram showing mild cardiac dysfunction. With the elimination of the offending medication from her circulation, the infant’s rate and function abnormalities resolved and QTc normalized. Both haloperidol and sertraline are known to be strong suppressors of the HERG potassium channel that plays an important role in myocardial repolarization and both medications can cross the placenta. Maternal overdose can cause transient QT-prolonging effect similar to that of inherited LQTS type 2. Given the high risk for sudden death in affected newborns, reporting this case is aimed to raise the awareness of acquired LQTS by placental transfer of medication. Early identification of neonates at risk should prompt ECG testing. If long QT is identified, the newborn should be closely monitored with care to avoid any further QT prolonging medications or conditions, until the toxic medication effect resolves.

Are SIDS, SUDC and SUDEP the different masks of the same great mystery?

The cases of sudden, unexpected child death against the background of relative clinical well-being, i.e., in the absence of apparent reasons take a special place in the structure of infant mortality. Sudden Infant Death Syndrome (SIDS), which is recognized as one of the leading causes of postnatal infant mortality in most developed countries, is the most common cause of unexpected out-ofhospital death of a child. Today SIDS remains one of the most mysterious problems in medicine. The lack of identifiable mechanisms causing SIDS has led to a large number of theories about the mechanisms responsible for death due to this syndrome. Also, sudden unexplained death in childhood (SUDC), which is the unexplained death of children over one- year-old, is currently distinguished among cases of unexpected death. The main clinical features of SUDC include: more common in boys; death occurs at night time, children are found in the early morning in a prone position, face down; children often have convulsions, including febrile ones in the clinical symptom complex during life. Several authors have noted that in some cases, the death due to SUDC resembles Sudden Death in Epilepsy (SUDEP), which is the leading cause of death in epilepsy. To date, it has already been shown that SUDEP is one of the forms of canalopathies characteristic of young children and it is associated with a high risk of sudden death. The mechanisms of thanatogenesis in SUDEP remain unknown. SIDS, SUDC, and SUDEP are a series of fatal syndromes united by multifactorial pathophysiological mechanisms, the causes of which are not fully understood. In fact, these syndromes represent a catastrophic multisystem failure, which is caused by an extremely unfavorable combination of autonomic, respiratory and cardiogenic disorders.

Does the type of seizure influence heart rate variability changes?

ObjectiveHeart rate variability (HRV), an index of the autonomic cardiac activity, is decreased in patients with epilepsy, and a low HRV is associated with a higher risk of sudden death. Generalized tonic-clonic seizures are one of the most consistent risk factors for SUDEP, but the influence (and relative risk) of each type of seizure on cardiac function is still unknown. Our objective was to assess the impact of the type of seizure (focal to bilateral tonic-clonic seizure – FBTCS – versus non-FBTCS) on periictal HRV, in a group of patients with refractory epilepsy and both types of seizures. MethodsWe performed a 48-hour Holter recording on 121 patients consecutively admitted to our Epilepsy Monitoring Unit. We only included patients with both FBTCS and non-FBTCS on the Holter recording and selected the first seizure of each type to analyze. To evaluate HRV parameters (AVNN, SDNN, RMSSD, pNN20, LF, HF, and LF/HF), we chose 5-min epochs pre- and postictally. ResultsWe included 14 patients, with a median age of 36 (min–max, 16–55) years and 64% were female. Thirty-six percent had cardiovascular risk factors, but no previously known cardiac disease.In the preictal period, there were no statistically significant differences in HRV parameters, between FBTCS and non-FBTCS. In the postictal period, AVNN, RMSSD, pNN20, LF, and HF were significantly lower, and LF/HF and HR were significantly higher in FBTCS.From preictal to postictal periods, FBTCS elicited a statistically significant rise in HR and LF/HF, and a statistically significant fall in AVNN, RMSSD, pNN20, and HF. Non-FBTCS only caused statistically significant changes in HR (decrease) and AVNN (increase). Significance/conclusionThis work emphasizes the greater effect of FBTCS in autonomic cardiac function in patients with refractory epilepsy, compared to other types of seizures, with a significant reduction in vagal tonus, which may be associated with an increased risk of SUDEP.