Life-threatening Events In Patients Research Articles

Association Between Syncope Trigger Type and Risk of Subsequent Life-Threatening Events in Patients With Long QT Syndrome

Syncope is the most powerful predictor for subsequent life-threatening events (LTEs) in patients with congenital long QT syndrome (LQTS). Whether distinct syncope triggers are associated with differential subsequent risk of LTEs is unknown. To evaluate the association between adrenergic (AD)- and nonadrenergic (non-AD)-triggered syncopal events and the risk of subsequent LTEs in patients with LQT types 1 to 3 (LQT1-3). This retrospective cohort study included data from 5 international LQTS registries (Rochester, New York; the Mayo Clinic, Rochester, Minnesota; Israel, the Netherlands, and Japan). The study population comprised 2938 patients with genetically confirmed LQT1, LQT2, or LQT3 stemming from a single LQTS-causative variant. Patients were enrolled from July 1979 to July 2021. Syncope by AD and non-AD triggers. The primary end point was the first occurrence of an LTE. Multivariate Cox regression was used to determine the association of AD- or non-AD-triggered syncope on the risk of subsequent LTE by genotype. Separate analysis was performed in patients with β-blockers. A total of 2938 patients were included (mean [SD] age at enrollment, 29 [7] years; 1645 [56%] female). In 1331 patients with LQT1, a first syncope occurred in 365 (27%) and was induced mostly with AD triggers (243 [67%]). Syncope preceded 43 subsequent LTEs (68%). Syncopal episodes associated with AD triggers were associated with the highest risk of subsequent LTE (hazard ratio [HR], 7.61; 95% CI, 4.18-14.20; P < .001), whereas the risk associated with syncopal events due to non-AD triggers was statistically nonsignificant (HR, 1.50; 95% CI, 0.21-4.77; P = .97). In 1106 patients with LQT2, a first syncope occurred in 283 (26%) and was associated with AD and non-AD triggers in 106 (37%) and 177 (63%), respectively. Syncope preceded 55 LTEs (56%). Both AD- and non-AD-triggered syncope were associated with a greater than 3-fold increased risk of subsequent LTE (HR, 3.07; 95% CI, 1.66-5.67; P ≤ .001 and HR, 3.45, 95% CI, 1.96-6.06; P ≤ .001, respectively). In contrast, in 501 patients with LQT3, LTE was preceded by a syncopal episode in 7 (12%). In patients with LQT1 and LQT2, treatment with β-blockers following a syncopal event was associated with a significant reduction in the risk of subsequent LTEs. The rate of breakthrough events during treatment with β-blockers was significantly higher among those treated with selective agents vs nonselective agents. In this study, trigger-specific syncope in LQTS patients was associated with differential risk of subsequent LTE and response to β-blocker therapy.

A novel SLC5A6 homozygous variant in a family with multivitamin-dependent neurometabolic disorder: Phenotype expansion and long-term follow-up

The sodium-dependent multivitamin transporter (hSMVT) encoded by the SLC5A6 gene is required for the intestinal absorption of biotin, pantothenic acid and lipoate, three micronutrients essential for normal growth and development. Systemic deficiency of these elements, either occurring from nutritional causes or genetic defects, is associated with neurological disorders, growth delay, skin and hair changes, metabolic and immunological abnormalities. A few patients with biallelic variants of SLC5A6 have been reported, exhibiting a spectrum of neurological and systemic clinical features with variable severity. We describe three patients from a single family carrying a homozygous p.(Leu566Valfs*33) variant of SLC5A6 disrupting the frame of the C-terminal portion of the hSMVT. In these patients, we documented a severe disorder featuring developmental delay, sensory polyneuropathy, optic atrophy, recurrent infections, and repeated episodes of intestinal pseudo-obstruction. Two patients who did not receive multivitamin supplementation therapy died in early infancy. In a third patient, early supplementation of biotin and pantothenic acid stabilized the clinical picture changing the course of the disease. These findings extend genotype-phenotype correlations and show how a timely and lifelong multivitamin treatment may be crucial to reduce the risk of life-threatening events in patients with pathogenic variants of the SLC5A6 gene.

Pathophysiology and Clinical Impacts of Chronic Kidney Disease on Coronary Artery Calcification

The global prevalence of chronic kidney disease (CKD) has increased in recent years. Adverse cardiovascular events have become the main cause of life-threatening events in patients with CKD, and vascular calcification is a risk factor for cardiovascular disease. Vascular calcification, especially coronary artery calcification, is more prevalent, severe, rapidly progressive, and harmful in patients with CKD. Some features and risk factors are unique to vascular calcification in patients with CKD; the formation of vascular calcification is not only influenced by the phenotypic transformation of vascular smooth muscle cells, but also by electrolyte and endocrine dysfunction, uremic toxin accumulation, and other novel factors. The study on the mechanism of vascular calcification in patients with renal insufficiency can provide a basis and new target for the prevention and treatment of this disease. This review aims to illustrate the impact of CKD on vascular calcification and to discuss the recent research data on the pathogenesis and factors involved in vascular calcification, mainly focusing on coronary artery calcification, in patients with CKD.

Cerebrolysin Use in Patients with Liver Damage-A Translational Study.

The treatment of acute life-threatening events in patients suffering from chronic pathologies is problematic, as physicians need to consider multisystemic drug effects. Regarding Cerebrolysin, a Sonic Hedgehog signaling pathway amplifier and one of the few approved neurotrophic treatments for stroke patients, concerns of excessive Hedgehog pathway activation that could accelerate NAFLD progression to cirrhosis seem valid. We investigated stroke patients treated with Cerebrolysin that presented elevated levels of aspartate aminotransferase (AST) and/or alanine aminotransferase (ALT). We also investigated the efficiency of Cerebrolysin in reversing the neurogenesis inhibition within the hippocampus in a mouse model of NAFLD by evaluating behavior and histological outcomes. NeuN, BrdU and Iba1 positive signals in the cortex and hippocampus of the animals were also observed. Clinically, Cerebrolysin improved AST levels in a majority of stroke patients with hepatic damage. The same treatment in an experimental setup was able to reverse anxiety-like behavior in MCD mice, reducing their freezing time from 333.61 ± 21.81 s in MCD animals to 229.17 ± 26.28 in treated ones. The use of Cerebrolysin did not improve short-term memory nor rescued cell multiplication in the hippocampus after MCD food intake. Understanding the neuroprotective and neurotrophic effects that drugs have on NAFLD patients can significantly contribute to a suitable therapeutic approach.

Effect of nintedanib on acute exacerbations of fibrosing interstitial lung diseases: a national database study in Japan.

Acute exacerbation is a life-threatening event in patients with fibrosing interstitial lung diseases (ILDs). Although nintedanib reduces acute exacerbation incidence, its effectiveness during acute exacerbation is unclear. Using data from the Diagnosis Procedure Combination database (September 2015-March 2020) in Japan, we identified patients with fibrosing ILDs who received intravenous injection of high-dose corticosteroid within 3 days post-admission and analysed their first hospitalisation. We performed overlap propensity score weighting to compare in-hospital outcomes between patients who received nintedanib within 14 days post-admission and those who did not. The primary and secondary outcomes were in-hospital mortality and length of hospitalisation in the patients discharged alive, respectively. Among the 6235 identified patients, 353 patients received nintedanib within 14 days post-admission. In-hospital mortality occurred in 13.7% and 6.0% patients in the control (n=5882) and nintedanib-treated (n=353) patients, respectively. The mean length of hospitalisation was 39.9 and 30.4 days in the control and nintedanib-treated patients, respectively. After overlap propensity score weighting, nintedanib treatment was significantly associated with lower in-hospital mortality in the adjusted cohort (OR 0.43, 95% CI 0.27-0.70; p=0.001). The mean length of hospitalisation in nintedanib-treated patients (30.7 days) was significantly shorter than that in the control group (37.5 days; p<0.001). Nintedanib initiation during acute exacerbation was significantly associated with a lower risk of in-hospital death and shorter length of hospitalisation in patients with fibrosing ILDs. Our results elucidate the potential role of nintedanib in the treatment of acute exacerbation in patients with fibrosing ILDs. Further prospective studies are warranted.

Emergencies in Oncology

Oncological emergencies are defined as any acute possible morbid or life-threatening events in patients with cancer either because of the malignancy or because of their treatment. With advances in antitumor therapy, the prognosis of oncological diseases has improved, so the management of their complications, whether due to the tumors themselves or their respective treatments, becomes more important day by day. These events may occur at any time during malignancy, from symptoms present to end-stage disease. Metabolic syndromes include Tumor Lysis Syndrome and Hypercalcemia. Compression Syndromes includes Superior Vena Cava Syndrome and Spinal Cord Compression Syndrome.

Emergencies in Oncology

Oncological emergencies are defined as any acute possible morbid or life-threatening events in patients with cancer either because of the malignancy or because of their treatment. With advances in antitumor therapy, the prognosis of oncological diseases has improved, so the management of their complications, whether due to the tumors themselves or their respective treatments, becomes more important day by day. These events may occur at any time during malignancy, from symptoms present to end-stage disease. Metabolic syndromes include Tumor Lysis Syndrome and Hypercalcemia. Compression Syndromes includes Superior Vena Cava Syndrome and Spinal Cord Compression Syndrome.

Emergencies in Oncology

Oncological emergencies are defined as any acute possible morbid or life-threatening events in patients with cancer either because of the malignancy or because of their treatment. With advances in antitumor therapy, the prognosis of oncological diseases has improved, so the management of their complications, whether due to the tumors themselves or their respective treatments, becomes more important day by day. These events may occur at any time during malignancy, from symptoms present to end-stage disease. Metabolic syndromes include Tumor Lysis Syndrome and Hypercalcemia. Compression Syndromes includes Superior Vena Cava Syndrome and Spinal Cord Compression Syndrome.

Emergency catheter ablation: A feasible option for acute treatments of patients with unstable pre-excited atrial fibrillation.

Pre-excited atrial fibrillation (AF) is associated with increased risk of life-threatening events. However, at times, patients with pre-excited AF still repetitively suffer from hemodynamic disturbance, with resistance to acute treatments of antiarrhythmic therapy and cardioversion. To evaluate the feasibility in correcting hemodynamic disturbance, patients with pre-excited AF who underwent catheter ablation of accessory pathway as an emergency procedure, were retrospectively collected from two centers of China. The medical records of patients were analyzed and summarized in this case series. Five patients with pre-excited AF who received emergency catheter ablation of accessory pathway, were collected from two contributor centers and reported in this case series. All collected patients still repetitively suffered from hemodynamic disturbance induced by rapid anterograde conduction of AF via pathway, even guideline recommended acute interventions of intravenous antiarrhythmic therapy and cardioversion had been performed. Finally, as an emergency procedure, catheter ablation of accessory pathway was performed in collected patients. Correspondingly, the hemodynamic unstable status was greatly relieved. Meanwhile, all collected patients with high risk of pre-excited AF were combined with left-sided accessory pathway, with shortest RR interval of widened pre-excited QRS complex less than 250 ms. Thus, combination with left-sided pathway is proposed as an indicator for the increased risk of life-threatening events in patients with high risk of pre-excited AF. Emergency catheter ablation of accessory pathway is an effective option for the acute managements of patients with high risk of pre-excited AF in unstable hemodynamics, which is resistant to antiarrhythmic therapy and cardioversion.

Characteristics of Obese Patients with Acute Hypercapnia Respiratory Failure Admitted in the Department of Pneumology: An Observational Study of a North African Population.

Background Acute hypercapnic respiratory failure (AHRF) is a common life-threatening event in patients with obesity hypoventilation syndrome (OHS). Objectives To study the clinical pattern, noninvasive ventilatory support, as well as the short- and long-term outcomes of patients with OHS admitted in a ward because of AHRF. Methods We conducted a retrospective cohort study including all adults with OHS aged ≥ 18 − year − old, admitted in a 90-bed-ward for AHRF. Results A total of 44 patients were included. Fifteen (34.1%) and 29 (65.9%) patients were diagnosed with malignant OHS (mOHS) and nonmalignant OHS (non-mOHS), respectively, while 36 (81.8%) had coexisting obstructive sleep apnea hypopnea syndrome (OSAHS). Patients with mOHS had a significantly higher rate of heart failure (100% vs. 31%; p < 0.001), chronic renal insufficiency (CRI) (73.3% vs. 41.4%; p = 0.04), and dyslipidemia (66.7% vs. 34.5%; p = 0.04) than those with non-mOHS. The mean forced vital capacity (FVC) in our patients was of 59.5% ± 18.5 of the predicted value, lower than what is usually reported in stable patients with OHS. At hospital admission, more than two-thirds (n = 34, 77.3%) were misdiagnosed as having asthma exacerbation (n = 4, 4.9.1%), chronic obstructive pulmonary disease (COPD) exacerbation (n = 12, 27.3%) and/or heart failure (n = 29, 65.9%). Acute pulmonary oedema (ACPE) (n = 16, 36.4%) and acute viral bronchitis (n = 12, 27.3%) were the main identified causal factors, while no cause could be determined in 5 (11.4%) patients. Noninvasive positive pressure ventilation (NIPPV) using bilevel positive airway pressure (BIPAP) was very highly effective to treat AHRF, with only 2.27% of patients failing the modality. Median overall duration of ventilation was 9 hours per day (1.3–20) and was significantly longer in patients with mOHS than in those with non-mOHS (10 [6–18] vs. 8 [1.3–20], respectively; p = 0.01). Forty two of the forty-three patients discharged alive were treated with BIPAP or continuous positive airway pressure (CPAP) in 26 and 16 patients, respectively. The probability of survival was 90% at 12 months, while the probability of readmission for a new episode of AHRF was 56% at 6 months and 22% at 12 months, respectively. Conclusion AHRF in OHS patients is a life-threatening event which can be successfully and safely treated with BIPAP, with a low long-term mortality even in patients with mOHS.

Oncological Emergencies from Pathophysiology and Diagnosis to Treatment: A Narrative Review

ABSTRACT Oncological emergencies are defined as any acute possible morbid or life-threatening events in patients with cancer either because of the malignancy or because of their treatment. These events may occur at any time during malignancy, from symptoms present to end-stage disease. The aim of this study is the review of urgent conditions results from cancer or cancer treatment side effects that need to be addressed immediately. In this study, a comprehensive and in-depth narrative review was carried out by searching the databases of PubMed, Scopus, Science Direct, Google Scholar with the keywords of “cancer, emergency, metabolic emergency, neutropenic fever” along with the words, “tumor lysis syndrome, chemotherapeutic emergency, diagnosis, treatment “ in last two decades. Patients suffering from cancer mostly face the challenges that we are classified in different categories, including metabolic, hematologic, cardiovascular, neurologic, respiratory, infectious, and chemotherapeutic emergencies. These patients mostly complain of headaches, nausea, pain, and fever. In conclusion, knowledge of oncology emergencies and palliative care as part of a team approach is critical for treating cancer patients. In this light, it is pivotal for physicians to focus on the early detection of oncological emergencies. Moreover, training programs for cancer patients help them to timely recognize and report the oncologic emergency symptoms, leading to avoid deleterious consequences and unnecessary healthcare costs as well as improve the quality of life in these patients.

Prevalence of Acute Kidney Injury in Patients with Liver Cirrhosis.

Acute kidney injury is a common and life-threatening event in patients with liver cirrhosis occurring in approximately 20-50% of hospitalized patients of liver cirrhosis. Pre-renal acute kidney injury, the hepatorenal syndrome type of acute kidney injury and acute tubular necrosis represent the common causes. The aim of this study was to study the profile of acute kidney injury in patients with liver cirrhosis. Consecutive patients of liver cirrhosis admitted in Liver unit of Bir Hospital were studied to see the presence of acute kidney injury in this hospital based descriptive cross-sectional study. Clinical and laboratory parameters along with various clinical outcome were compared between different groups categorized by the severity of liver disease and renal dysfunction. Out of 302 liver cirrhosis patients, 56 (18.5%) had acute kidney injury among which 23 (46%) were found to have pre-renal acute kidney injury, 15 (30%) with hepatorenal syndrome- acute kidney injury and 12 (24%) with intrinsic renal disease. Patients with higher stages of acute kidney injury had longer duration of hospital stay and hepatorenal syndrome-acute kidney injury was seen in patients with higher grade of ascites and with hyponatremia. Acute kidney injury is a common occurrence in patients with advanced liver cirrhosis with pre-renal acute kidney injury being the commonest cause. Median hospital stay is directly affected by the severity of acute kidney injury and hepatorenal syndrome-acute kidney injury was seen in patients with higher grade of ascites and hyponatremia. Early identification of patients at high risk for acute kidney injury may help to reduce mortality and contain costs.

Ventricular Arrhythmia and Life-Threatening Events in Patients With Repaired Tetralogy of Fallot

Risk stratification for malignant arrhythmias and risk of sudden cardiac death in tetralogy of Fallot (TOF) remains challenging. We aimed to ascertain factors associated with life-threatening arrhythmic events. A multicenter retrospective case-control study including 72 TOF patients with documented cardiac arrest and/or sustained ventricular tachycardia, compared with 216 controls matched for era of surgery. The mean age at event in the cases was 27.3 ± 12.5 years. The majority (57%) presented with sustained ventricular tachycardia. Fatal events occurred in 9. Random forest analysis and a decision tree demonstrated surgical era specific risk factors (< vs ≥ 1980). For both eras, arrhythmic symptoms and left ventricular dysfunction were strongly associated with malignant arrhythmias. In addition, right ventricular dysfunction and age at repair ≥ 6.5 years preceded by a shunt were associated with a higher risk group in the early era, whereas a trans-annular patch type repair was associated with a lower risk group in the recent era. For the moderate and high-risk groups, the decision tree showed a sensitivity of 88.4% and specificity of 68.1%. An "importance factor" was calculated for each predictor, creating a risk score and 4 risk categories. In conclusions, this risk stratification scheme, based on clinical history and noninvasive testing, allows categorization of TOF patients at high risk of malignant arrhythmia. A multicenter prospective evaluation of the accuracy of this scoring system is now being planned.

Additive prognostic significance of ejection fraction for ESC risk model in hypertrophic cardiomyopathy patients.

The European Society of Cardiology (ESC) clinical risk model is reported in predicting sudden death of hypertrophic cardiomyopathy (HCM). We examined the validity of this model and investigated the significance of ejection fraction (EF) in predicting the prognosis using ESC risk model in HCM patients. 305 HCM patients (198 males) were followed (median follow-up 4.8years) for life-threatening arrhythmic events (sudden death, aborted sudden death, sustained VT/VF, appropriate ICD intervention for VT/VF) and were divided using ESC risk model into low- (Group L), intermediate- (Group I) and high- (Group H) risk groups. There was a significant difference in the events rate among the 3 groups (L, 0.9%/year; I, 3.9%/year; H, 6.8%/year; log-rank p < 0.001) in all study patients. Reduced EF (<50%) was identified in 27 (8.9%) cases. There was a significant difference in the events rate among the 3 groups in patients with reduced EF (L, 2.4%/year; I, 4.9%/year; H, 16.1%/year; log-rank p = 0.025). There was a significant difference in the events rate among 2 groups in patients stratified as Group H (preserved EF, 3.1%/year vs. reduced EF, 16.1%/year; log-rank p = 0.041). ESC risk model precisely predicts life-threatening events in patients with HCM. Adding EF to ESC risk model are useful for further risk stratification of life-threatening arrhythmic events.

Surveillance of bloodstream infections in pediatric cancer centers - what have we learned and how do we move on?

Pediatric patients receiving conventional chemotherapy for malignant disease face an increased risk of bloodstream infection (BSI). Since BSI may represent an acute life-threatening event in patients with profound immunosuppression, and show further negative impact on quality of life and anticancer treatment, the prevention of BSI is of paramount importance to improve and guarantee patients’ safety during intensive treatment. The great majority of all pediatric cancer patients (about 85%) have a long-term central venous access catheter in use (type Broviac or Port; CVAD). Referring to the current surveillance definitions a significant proportion of all BSI in pediatric patients with febrile neutropenia is categorized as CVAD-associated BSI. This state of the art review summarizes the epidemiology and the distinct pathogen profile of BSI in pediatric cancer patients from the perspective of infection surveillance. Problems in executing the current surveillance definition in this patient population are discussed and a new concept for the surveillance of BSI in pediatric cancer patients is outlined.

Life-threatening events in patients with pheochromocytoma.

Pheochromocytomas are rare chromaffin cell-derived tumors causing paroxysmal episodes of headache, palpitation, sweating and hypertension. Life-threatening complications have been described in case reports and small series. Systematic analyses are not available. We took an opportunity of a large series to make a survey. We analyzed records of patients diagnosed with pheochromocytomas in three geographically spread German referral centers between 2003 and 2012 (n=135). Eleven percent of the patients (ten women, five men) required in-hospital treatment on intensive care units (ICUs) due to complications caused by unsuspected pheochromocytomas. The main reasons for ICU admission were acute catecholamine induced Tako-Tsubo cardiomyopathy (n=4), myocardial infarction (n=2), acute pulmonary edema (n=2), cerebrovascular stroke (n=2), ischemic ileus (n=1), acute renal failure (n=2), and multi organ failure (n=1). One patient required extracorporeal membrane oxygenation due to a hypertensive crisis with lung edema occurring during delivery (n=1). Two patients died of refractory shock and pheochromocytomas were found postmortem. Two patients were treated by emergency surgery. Compared to pheochromocytoma patients without life-threatening events (n=120), patients with complications had a significant larger maximal tumor diameter (7.0 vs 4.5 cm, P<0.01), higher levels of catecholamines (20- vs ninefold upper limit of normal, P<0.01), and tended to be younger (42 vs 51 years, P=0.05). Although pheochromocytomas are rare, they are likely to be associated with a life-threatening situation. Clinicians have to be aware of these situations and perform a timely diagnosis.

Incidence of adrenal crisis in patients with adrenal insufficiency.

An adrenal crisis (AC) is a potential life-threatening event in patients with adrenal insufficiency (AI). This study aims to determine the incidence, causes, and risk factors of AC in AI. Patients with AI diagnosed and treated at the University Medical Center Utrecht for the past 30 years were identified, and all medical records were assessed by two independent investigators. The observed frequency of AC was determined as incidence rate, calculated as the number of AC divided by person-years (PY). In addition, precipitating factors and risk factors were assessed. We observed an incidence rate of 5·2 AC (95% CI 4·3-6·3) per 100 PY in primary adrenal insufficiency (PAI, a total of 111 patients), and 3·6 AC (95% CI 3·1-4·1) per 100 PY in secondary adrenal insufficiency (SAI a total of 319 patients). Patients with an established diagnosis of tertiary (glucocorticoid-induced) adrenal insufficiency (a total of 28 patients) had 15·1 AC (95% CI 11·0-19·9) per 100 PY. The most important risk factor was the existence of comorbidity. Gastro-enteritis and other infections were the most common precipitating factors for AC. AC still occurs relatively frequent in patients with AI, mostly precipitated by infections and particularly in patients with high comorbidity. This should be taken into account in the education and follow-up of patients with AI.

Clinical utility of a panfungal polymerase chain reaction assay for invasive fungal diseases in patients with haematologic disorders

Invasive fungal diseases (IFDs) are life-threatening events in patients with haematologic disorders, and the spectrum of the aetiological pathogens continues to expand. This study aimed to evaluate the clinical utility of a panfungal polymerase chain reaction (PCR) assay for the management of IFDs in such patients. We prospectively analysed 273 consecutive blood samples from 64 risk episodes in 51 patients with haematologic disorders at high risk for IFD who were treated at our hospital between April 2007 and October 2010. PCR-positive results were obtained in 18 of 64 risk episodes (35.3%). IFD was documented in 14 episodes (21.9%, 9 probable IFDs and 5 possible IFDs) according to the revised criteria of the European Organization for Research and Treatment of Cancer/Mycoses Study Group. PCR was positive in all of these 14 episodes, and in 4 of the 50 episodes with no IFD category. Sensitivity, specificity, positive predictive value, and negative predictive value of our assay were 100%, 92%, 78% and 100% respectively. A considerable number of fungi (44.4%) that are less common than Aspergillus and Candida species were positive by PCR. Molecular diagnoses of Cunninghamella species, Aspergillus ustus, Fusarium species, Scedosporium apiospermum, Rhodotorula species and Rhizopus species were beneficial in selecting suitable treatments. Our panfungal PCR approach allows for the highly sensitive and specific detection and identification of a wide spectrum of fungal pathogens, which provides indispensable information for managing IFDs, especially refractory or breakthrough IFDs during antifungal therapy in high-risk patients with haematologic disorders.

Prognostic Significance of J Wave in Patients Who Underwent Device Therapy for Ventricular Defibrillation

Background: J wave has been reported to be associated with higher incidences of life-threatening events in patients with either structural or nonstructural heart disease. The risk of J wave in patients who implanted a defibrillator with/without cardiac-resynchronization therapy device is unclear.Methods: The relationship between J-wave appearance and defibrillator interventions was evaluated by analyzing electrograms stored in devices retrospectively with proportional hazards models.Results: A total of 150 patients were enrolled and followed for a mean period of 38.8±25.0 months. Underlying heart diseases for device implantation were: myocardial infarction in 40; dilated cardiomyopathy in 30; hypertrophic cardiomyopathy in 16; ARVC in 3; Brugada syndrome in 22; idiopathic VF in 13; and others in 26 patients. Of these, 30 patients (20.0%) showed J wave at the time of implant. Patients with J wave had higher incidences of device intervention for VT/VF than those without (56.7% vs. 35.8%; P=0.0390). Multivariate analysys revealed that J-wave appearance and documented VT were independently associated with life-threatening events in patients with device implantation, even after the adjustment of age, gender, and left ventricular ejection fraction (HR, 2.20; 95% CI, 1.14–4.06; P=0.0193 and HR, 4.13; 95% CI, 2.15–8.46; P<0.0001, respectively).Conclusion: J wave at the time of device implantation identifies the risk of future device interventions.

Risk for Life-Threatening Cardiac Events in Patients With Genotype-Confirmed Long-QT Syndrome and Normal-Range Corrected QT Intervals

This study was designed to assess the clinical course and to identify risk factors for life-threatening events in patients with long-QT syndrome (LQTS) with normal corrected QT (QTc) intervals. Current data regarding the outcome of patients with concealed LQTS are limited. Clinical and genetic risk factors for aborted cardiac arrest (ACA) or sudden cardiac death (SCD) from birth through age 40 years were examined in 3,386 genotyped subjects from 7 multinational LQTS registries, categorized as LQTS with normal-range QTc (≤ 440 ms [n = 469]), LQTS with prolonged QTc interval (> 440 ms [n = 1,392]), and unaffected family members (genotyped negative with ≤ 440 ms [n = 1,525]). The cumulative probability of ACA or SCD in patients with LQTS with normal-range QTc intervals (4%) was significantly lower than in those with prolonged QTc intervals (15%) (p < 0.001) but higher than in unaffected family members (0.4%) (p < 0.001). Risk factors ACA or SCD in patients with normal-range QTc intervals included mutation characteristics (transmembrane-missense vs. nontransmembrane or nonmissense mutations: hazard ratio: 6.32; p = 0.006) and the LQTS genotypes (LQTS type 1:LQTS type 2, hazard ratio: 9.88; p = 0.03; LQTS type 3:LQTS type 2, hazard ratio: 8.04; p = 0.07), whereas clinical factors, including sex and QTc duration, were associated with a significant increase in the risk for ACA or SCD only in patients with prolonged QTc intervals (female age > 13 years, hazard ratio: 1.90; p = 0.002; QTc duration, 8% risk increase per 10-ms increment; p = 0.002). Genotype-confirmed patients with concealed LQTS make up about 25% of the at-risk LQTS population. Genetic data, including information regarding mutation characteristics and the LQTS genotype, identify increased risk for ACA or SCD in this overall lower risk LQTS subgroup.