Malnutrition is becoming one of the most important determinants of worse clinical outcomes in patients with acute heart failure (AHF). However, appropriate tools for evaluating the nutritional status ...in patients aged ≥65 years with AHF remain unclear. We examined 490 consecutive patients aged ≥65 years with AHF. They were divided into 2 groups according to Geriatric Nutritional Risk Index (GNRI; cut-off value = 92). During a median period of 189 days, the mortality rate was significantly higher in the lower GNRI group than the higher GNRI group (p <0.001). In multivariate analyses, lower GNRI was an independent determinant of adverse events (HR 0.92, 95% CI 0.88 to 0.95, p <0.001). The GNRI showed the best prognostic value (C-statistic: 0.70) among other nutritional indexes. Adding GNRI to an existing outcome prediction model for mortality in AHF significantly increased the C-statistic from 0.68 to 0.74 (p = 0.017). The net reclassification improvement afforded by GNRI was 60% overall, 27% for events, and 33% for nonevents (p <0.001). In conclusion, lower GNRI on admission was independently associated with worse clinical outcomes in patients aged ≥65 years with AHF, and it was superior to other nutritional parameters. Furthermore, the assessment of nutritional status using GNRI is very helpful for risk stratification.
Andersen-Tawil syndrome (ATS) is an autosomal dominant genetic or sporadic disorder characterized by ventricular arrhythmias (VAs), periodic paralyses, and dysmorphic features. The optimal ...pharmacological treatment of VAs in patients with ATS remains unknown.
We evaluated the efficacy and safety of flecainide for VAs in patients with ATS with KCNJ2 mutations.
Ten ATS probands (7 females; mean age 27 ± 11 years) were enrolled from 6 institutions. All of them had bidirectional VAs in spite of treatment with β-blockers (n = 6), but none of them had either aborted cardiac arrest or family history of sudden cardiac death. Twenty-four-hour Holter recording and treadmill exercise test (TMT) were performed before (baseline) and after oral flecainide therapy (150 ± 46 mg/d).
Twenty-four-hour Holter recordings demonstrated that oral flecainide treatment significantly reduced the total number of VAs (from 38,407 ± 19,956 to 11,196 ± 14,773 per day; P = .003) and the number of the longest ventricular salvos (23 ± 19 to 5 ± 5; P = .01). At baseline, TMT induced nonsustained ventricular tachycardia (n = 7) or couplets of premature ventricular complex (n = 2); treatment with flecainide completely (n = 7) or partially (n = 2) suppressed these exercise-induced VAs (P = .008). In contrast, the QRS duration, QT interval, and U-wave amplitude of the electrocardiogram were not altered by flecainide therapy. During a mean follow-up of 23 ± 11 months, no patients developed syncope or cardiac arrest after oral flecainide treatment.
This multicenter study suggests that oral flecainide therapy is an effective and safe means of suppressing VAs in patients with ATS with KCNJ2 mutations, though the U-wave amplitude remained unchanged by flecainide.
Familial sick sinus syndrome (SSS) is often attributable to mutations in genes encoding the cardiac Na channel SCN5A and pacemaker channel HCN4. We previously found that SSS with SCN5A mutations ...shows early onset of manifestations and male predominance. Despite recent reports on the complications of atrial fibrillation (AF) and left ventricular noncompaction (LVNC) in patients with SSS caused by HCN4 mutations, their overall clinical spectrum remains unknown.
The purpose of this study was to investigate the clinical and demographic features of SSS patients carrying HCN4 mutations.
We genetically screened 38 unrelated SSS families and functionally analyzed the mutant SCN5A and HCN4 channels by patch clamping. We also evaluated the clinical features of familial SSS by a meta-analysis of 48 SSS probands with mutations in HCN4 (n = 16) and SCN5A (n = 32), including previously reported cases, and 538 sporadic SSS cases.
We identified two HCN4 and three SCN5A loss-of-function mutations in our familial SSS cohort. Meta-analysis of HCN4 mutation carriers showed a significantly younger age at diagnosis (39.1 ± 21.7 years) than in sporadic SSS (74.3 ± 0.4 years; P <.001), but a significantly older age than in SCN5A mutation carriers (20.0 ± 17.6 years; P = .003). Moreover, HCN4 mutation carriers were more frequently associated with AF (43.8%) and LVNC (50%) and with older age at pacemaker implantation (43.5 ± 22.1 years) than were SCN5A mutation carriers (17.8 ± 16.5 years; P <.001).
SSS with HCN4 mutations may form a distinct SSS subgroup characterized by early clinical manifestation after adolescence and frequent association with AF and LVNC.
Prednisolone (PSL) therapy is the gold standard treatment in patients with cardiac sarcoidosis (CS). However, clinicians often have difficulty in deciding whether to discontinue PSL therapy in ...long-term management. Sixty-one consecutive patients with CS were divided into 2 groups based on the discontinuation of PSL during the median follow-up period of 9.9 years. PSL was discontinued in 12 patients because of improvement of clinical findings. There were no significant differences between the 2 groups in age, gender, left ventricular ejection fraction (LVEF), findings of imaging techniques, incidence of fatal arrhythmias and heart failure, and dose of PSL. After discontinuation of PSL, 5 patients had cardiac death, and discontinuation of PSL was significantly associated with higher cardiac mortality compared with continuation (p = 0.035). Although patients with discontinuation had improvement of LVEF after PSL treatment, LVEF decreased after discontinuation of PSL. Furthermore, discontinuation of PSL was associated with greater percent decrease in LVEF compared with continuation (p = 0.037) during the follow-up period. In conclusion, in the long-term management of patients with CS, discontinuation of PSL was associated with poor clinical outcomes and decreased LVEF, suggesting the importance of PSL maintenance therapy.
Little is known about the clinical and prognostic impact of early repolarization (ER) on patients with Brugada syndrome (BrS), especially those with documented ventricular fibrillation (VF).
To ...investigate the prevalence and prognostic significance of ER in inferolateral leads in patients with BrS and documented VF.
We investigated 10 different 12-lead electrocardiograms (ECGs) recorded on different days to identify the presence of ER, which was defined as J-point elevation ≥0.1 mV in inferior (II, III, aVF) or lateral leads (I, aVL, V₄-V₆), in 49 individuals (46 men; age 46 ± 13 years) with a type 1 ECG of BrS and previous history of VF.
ER was observed persistently (in all ECGs) in 15 patients (31%; P group), intermittently (in at least one but not in all ECGs) in 16 patients (33%; I group), and not observed in 18 patients (37%; N group), yielding an overall ER incidence of 63% (31/49). During the follow-up period (7.7 years), recurrence of VF was documented in all 15 patients (100%) in the P group, and less in 12 patients (75%) in the I group and in 8 patients (44%) in the N group. The P group showed a worse prognosis than N group (P = .0001) by Kaplan-Meier analysis. Either persistent or intermittent ER in an inferolateral lead was an independent predictor of fatal arrhythmic events (hazard ratio 4.88, 95% confidence interval 2.02-12.7, P = .0004; and hazard ratio 2.50, 95% confidence interval 1.03-6.43, P = .043, respectively).
The prevalence of ER in inferolateral leads was high and an especially persistent form of ER was associated with a worse outcome in BrS patients with documented VF.
Implantable cardiac monitors (ICMs) help investigate the cause of unexplained syncope, but the probability and predictors of needing a pacing device thereafter remain unclear.
We retrospectively ...analyzed the data of patients who received ICM insertion for unexplained syncope with suspected arrhythmic etiology. The data were obtained from a nationwide database obtained between April 1, 2012 and March 31, 2020. Multivariable mixed-effects survival analysis was performed to identify predictors of pacing device implantation (PDI), and a risk score model was developed accordingly.
In total, 2905 patients (age: 72 years range: 60-78) implanted with ICMs to investigate the cause of syncope were analyzed. During the median follow-up period of 128 days (range: 68–209) days, 473 patients (16%) underwent PDI. Older age, history of atrial fibrillation, bundle branch block (BBB), and diabetes were independent predictors of PDI in multivariable analysis. A risk score model was developed with scores ranging from 0 to 32 points. When patients with the lowest quartile score (0–13 points) were used as a reference, those with higher quartiles had a higher risk of PDI (second quartile: 14–15 points, hazard ratio HR: 3.86, 95% confidence interval CI: 2.62–5.68; third quartile: 16–18 points, HR: 4.67, 95% CI: 3.14–6.94; fourth quartile: 19–32 points, HR: 6.59, 95% CI: 4.47–9.71).
The 4 identified predictors are easily assessed during the initial evaluation of patients with syncope. They may help identify patients with a higher risk of requiring permanent PDI.
Les moniteurs cardiaques implantables (MCI) aident à déterminer la cause d’une syncope inexpliquée, mais la probabilité et les facteurs prédictifs du besoin d’un dispositif de stimulation cardiaque par la suite demeurent incertains.
Nous avons analysé de façon rétrospective les données de patients s’étant fait implanter un MCI après une syncope inexpliquée et chez lesquels une étiologie d’arythmie était soupçonnée. Les données proviennent d’une base de données nationale et s’étendent du 1er avril 2012 au 31 mars 2020. Une analyse de survie multivariable à effets mixtes a été effectuée pour cibler les facteurs prédictifs de l’implantation d’un dispositif de stimulation cardiaque (IDSC), et un modèle de score de risque a été conçu en conséquence.
Au total, les cas de 2905 patients (âge : 72 ans écart interquartile (ÉI) : 60-78) ayant reçu un MCI pour déterminer la cause de la syncope ont été analysés. Durant la période de suivi médiane de 128 jours (ÉI : 68-209), 473 patients (16 %) ont subi une IDSC. L’âge avancé, les antécédents de fibrillation auriculaire, le bloc de branche et le diabète étaient des facteurs prédictifs indépendants de l’IDSC dans l’analyse multivariable. Un modèle de score de risque a été conçu, les scores allant de 0 à 32 points. Lorsque les patients ayant un score dans le quartile inférieur (0 à 13 points) étaient utilisés à titre de référence, ceux ayant un score dans les quartiles supérieurs avaient un risque plus élevé d’IDSC (deuxième quartile : 14-15 points, rapport des risques instantanés RRI : 3,86, intervalle de confiance IC à 95 % de 2,62 à 5,68; troisième quartile : 16-18 points, RRI : 4,67, IC à 95 % de 3,14 à 6,94; quatrième quartile : 19-32 points, RRI : 6,59, IC à 95 % de 4,47 à 9,71).
Les quatre facteurs prédictifs ciblés sont faciles à évaluer durant l’évaluation initiale des patients ayant subi une syncope. Ils peuvent aider à repérer les patients présentant un risque plus élevé d’avoir besoin d’un dispositif de stimulation cardiaque permanent.
Objectives The aim of this study was to investigate the significance of non-type 1 anterior early repolarization (NT1-AER) combined with inferolateral early repolarization syndrome (ERS). Background ...Inferolateral ERS might be a heterogeneous entity, although it excludes type 1 Brugada syndrome (BS). Methods Of 84 patients with spontaneous ventricular fibrillation, 31 ERS patients were divided into 2 groups. The ERS(A)-group consisted of inferolateral ER and NT1-AER—that is, notching or slurring with J-wave ≥1 mm at the end of QRS to early ST segment in any of V1 to V3 leads, in which the ST-T segment did not change to a coved pattern in the standard and high costal (second and third) electrocardiographic recordings even after drug provocation tests (n = 12). The other, ERS(B)-group, showed only inferolateral ER (n = 19). Clinical characteristics and outcomes were compared between the ERS groups, 40 patients with type-1 BS (BS-group), and 13 patients with idiopathic ventricular fibrillation lacking J-wave (IVF-group). Results Ventricular fibrillation occurred during sleep or near sleep in 10 of 12 patients in ERS(A)-group and in 22 of 40 patients in BS-group but in 2 of 19 patients in ERS(B)-group and in 1 of 13 patients in IVF-group (ERSA vs. ERSB, p < 0.0001). Ventricular fibrillation recurrence was significantly higher in ERS(A)-group (58%), particularly in patients with J waves in the high lateral lead, and BS-group (55%), compared with ERS(B)-group (11%) and IVF-group (15%) (ERSA vs. ERSB, p = 0.012). Conclusions Inferolateral ERS comprises heterogeneous ER subtypes with and without NT1-AER. Coexistence of NT1-AER was a key predictor of poor outcome in patients with ERS.
Abnormal liver function test results are often observed in acute decompensated heart failure (ADHF). However, the prognostic value of bilirubin fractionation has not been elucidated. The prognostic ...value of direct bilirubin (DB), in comparison with total bilirubin (TB), was examined in 556 consecutive patients with ADHF. Patients with elevated DB showed mostly similar patient characteristics including signs of elevated right-sided pressure (frequent hepatomegaly, jugular venous distention, dilated inferior vena cava, and elevated gamma-glutamyltransferase) and decreased cardiac output (cold extremities, decreased pulse pressure, and lower blood pressure) and other parameters of heart failure (HF) severity (increased plasma renin activity, decreased sodium, total cholesterol, and ejection fraction) to elevated TB; however, only patients with elevated DB showed a significant difference in the frequency of HF history and alkaline phosphatase value. Kaplan-Meier analysis showed that patients with elevated DB had a significantly higher rate of the composite end point of all-cause mortality or HF readmission (p = 0.021) compared with those with normal DB, whereas patients with elevated TB did not show a statistically significant difference compared with those with normal TB (NS). A multivariate Cox hazards model showed that DB was an independent predictor of adverse events (adjusted hazard ratio 1.052, 95% confidence interval 1.001 to 1.099, p = 0.034), whereas TB was not (adjusted hazard ratio 1.017, 95% confidence interval 0.985 to 1.046, p = 0.27). Adding DB to existing prognostic variables resulted in higher C-statistics than adding TB (C-statistics: 0.670 to 0.675, 0.670 to 0.674, respectively). In conclusion, elevated DB in ADHF was an independent prognostic predictor that was superior to TB. DB may be useful for further risk stratification in ADHF.
The pathophysiological mechanism of J wave in anterior leads (A-leads) and inferolateral leads (L-leads) remains unclear.
We investigated the onset mode and circadian distribution of ventricular ...tachyarrhythmia (VTA) episodes between patients with early repolarization syndrome (ERS) and Brugada syndrome (BrS).
The study enrolled 35 patients with ERS and 52 patients with type 1 BrS with spontaneous ventricular fibrillation who were divided into 4 groups: ERS(A+L) (n = 15), patients with ERS who had a non-type 1 Brugada pattern electrocardiogram in any A-leads (second to fourth intercostal spaces) in control and/or after drug provocation tests; ERS(L) (n = 20), patients with ERS with J wave only in L-leads; BrS(A) (n = 24), patients with BrS without J wave in L-leads; and BrS(A+L) (n = 28), patients with BrS with J wave in L-leads. The onset mode of 206 VTAs obtained from electrocardiograms or implantable cardioverter-defibrillators and the circadian distribution of 352 VTAs were investigated in the 4 groups.
Three groups with J wave in A-leads, ERS(A+L), BrS(A), and BrS(A+L), had higher incidences of nocturnal (63%, 43%, and 47%, respectively) and sudden onset VTAs (67%, 97%, and 86%, respectively) with longer coupling intervals of premature ventricular contractions (388.8, 397.3, and 385.6 ms, respectively) than the ERS(L) group with J wave only in L-leads (25%, P = .0019; 19%, P < .0001; and 330.6 ms, P = .0004, respectively), the last of which mainly displayed VTAs with a short-long-short sequence.
The onset mode of VTAs was different between patients with J wave in A-leads and patients with J wave in only L-leads. The underlying mechanism of J wave may differ between A-leads and L-leads.
A high incidence of early repolarization (ER) pattern in the inferolateral leads has been reported in patients with idiopathic ventricular fibrillation (IVF). Brugada syndrome (BS) is characterized ...by J-point or ST-segment elevation in the right precordial leads and ventricular fibrillation, and some patients with BS also have ER in the inferolateral leads.
To compare the clinical characteristics and effects of sodium-channel blockade on ER between IVF patients with ER (early repolarization syndrome ERS) and BS patients with or without ER.
Fourteen patients with ERS and 21 patients with BS were included in this study. ER was defined as an elevation of at least 0.1 mV from baseline in the QRS-T junction in the inferorolateral leads. Provocative tests with sodium-channel blockers were conducted in all patients with ERS to distinguish ERS from BS.
In the ERS group, all patients were male and most patients experienced ventricular fibrillation during sleep or low activity (79%). ER was attenuated by sodium-channel blockers in most patients with ERS (13/14, 93%) and BS (5/5, 100%), whereas ST-segment elevation was augmented in the right precordial leads in the BS group. The rates of positive late potentials were significantly higher in the BS group (60%) than in the ERS group (7%) (P <.01).
Some similarities were observed between ERS and BS, including gender, arrhythmia triggers, and response of ER to sodium-channel blockers. Unlike the ST segment in the right precordial leads in BS, ER was attenuated in patients with both ERS and BS, suggesting a differential mechanism between ER in the inferolateral leads and ST elevation in the right precordial leads.
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