Long-term prognosis of idiopathic ventricular fibrillation: An eighteen-year experience from a tertiary center.

INTRODUCTION AND OBJECTIVES: Idiopathic ventricular fibrillation (IVF) is diagnosed in patients who survive sudden cardiac arrest (SCA), preferably with documented ventricular fibrillation (VF), without any identifiable structural or electrical abnormality. Current evidence provides limited guidance on the diagnosis and follow-up of these patients. Our aim was to assess the clinical outcomes of survivors of an aborted SCA attributed to IVF. METHODS: We retrospectively collected clinical data from all patients who survived SCA and implanted a cardiac defibrillator (ICD) between 2005 and 2023. RESULTS: A total of 38 patients, 36.8% female, with a mean age of 44±14 years old were included. Median follow-up time was 8.7 years (interquartile range (IQR) 4.7-14.7 years). All patients underwent a comprehensive diagnostic evaluation that excluded structural and coronary disease. During follow-up, underlying diagnoses were established in 34.2% of the whole cohort. Genetic testing, performed in 37.2%, revealed underlying diagnoses in 57.1% of those tested, compared to only 26.3% of patients who did not undergo genetic testing [p=0.035, OR = 5.1 (95% confidence interval (CI) 1.2-21.5)]. Mortality was 10.5% (due to non-arrhythmic causes) and 36.8% patients received appropriate therapies with a median time to first ICD therapy of 39 [5.4-47.3] months. CONCLUSION(S): Etiological diagnosis and recurrence prediction in patients with IVF remains challenging, even with extensive diagnostic evaluation and long-term follow-up. In our study, genetic testing enhanced diagnostic yield. Consistent with previous findings, our cohort experienced a notable arrhythmic recurrence, with no cardiac deaths, underlining the pivotal role of ICD implantation in these patients.

ECMO for drug-refractory electrical storm without a reversible trigger: a retrospective multicentric observational study.

AIMS: Drug-refractory electrical storm (ES) is a life-threatening medical emergency. We describe the use of venoarterial extracorporeal membrane oxygenation (VA-ECMO) in drug-refractory ES without a reversible trigger, for which specific guideline recommendations are still lacking. METHODS AND RESULTS: Retrospective observational study in four Iberian centres on the indications, treatment, complications, and outcome of drug-refractory ES not associated with acute coronary syndromes, decompensated heart failure, drug toxicity, electrolyte disturbances, endocrine emergencies, concomitant acute illness with fever, or poor compliance with anti-arrhythmic drugs, requiring VA-ECMO for circulatory support. Thirty-four (6%) out of 552 patients with VA-ECMO for cardiogenic shock were included [71% men; 57 (44-62) years], 65% underwent cardiopulmonary resuscitation before VA-ECMO implantation, and 26% during cannulation. Left ventricular unloading during VA-ECMO was used in 8 (24%) patients: 3 (9%) with intraaortic balloon pump, 3 (9%) with LV vent, and 2 (6%) with Impella. Thirty (88%) had structural heart disease and 8 (24%) had an implantable cardioverter-defibrillator. The drug-refractory ES was mostly due to monomorphic ventricular tachycardia (VT) and ventricular fibrillation (VF) (59%), isolated monomorphic VT (26%), polymorphic VT (9%), or VF (6%). Thirty-one (91%) required deep sedation, 44% overdrive pacing, 36% catheter ablation, and 26% acute autonomic modulation. The main complications were nosocomial infection (47%), bleeding (24%), and limb ischaemia (21%). Eighteen (53%) were weaned from VA-ECMO, and 29% had heart transplantation. Twenty-seven (79%) survived to hospital discharge (48 (33-82) days). Non-survivors were older [62 (58-67) vs. 54 (43-58); P < 0.01] and had a higher first rhythm disorder-to-ECMO interval [0 (0-2) vs. 2 (1-11) days; P = 0.02]. Seven (20%) had rehospitalization during follow-up [29 (12-48) months], with ES recurrence in 6%. CONCLUSIONS: VA-ECMO bridged drug-refractory ES without a reversible trigger with a high success rate. This required prolonged hospital stays and coordination between the ECMO centre, the electrophysiology laboratory, and the heart transplant programme.

Authors' Response.

Dear Editor, We would like to thank Dr. Madias for his valuable comment on our original article entitled "QT interval prolongation in Takotsubo Syndrome: a frightening feature with no major prognostic impact" published in Monaldi Archives for Chest Disease on December 6, 2023...

QT interval prolongation in Takotsubo syndrome: a frightening feature with no major prognostic impact.

Despite the frequent and often severe repolarization abnormalities seen in Takotsubo syndrome (TTS), the underlying mechanism of life-threatening arrhythmias is incompletely understood, and the risk remains uncertain. TTS is considered a potential cause of acquired long QT syndrome; however, there is no robust evidence that QT prolongation has a major prognostic impact on these patients' outcomes. Our aim was to assess the prevalence and clinical implications of acquired long QT during TTS events and compare in-hospital and long-term outcomes between patients with and without corrected QT interval (QTc) prolongation. This is a retrospective cohort study that included 113 patients admitted to our tertiary care hospital with a diagnosis of TTS. The cohort was divided into two groups: a long QTc group (QTc≥460 milliseconds in any electrocardiogram at admission or during hospitalization) and a normal QTc group. Baseline characteristics, occurrences during hospitalization, and outcome data were obtained from the revision of medical registries and hospital visits. Of the 113 patients, 107 (94.7%) were female. The mean age was 67.6±11.7 years. QTc prolongation was found in 38%. Demographic characteristics, relevant chronic medication, prevalence of cardiovascular risk factors, and other comorbidities were similar between the groups, except for history of atrial fibrillation, which was more common in the long QTc group. Syncope was more prevalent in the long QTc group. In-hospital complications were not statistically different between patients with long and normal QTc (48.8% versus 44.2%, p=0.637), including ventricular arrhythmias and complete atrioventricular block (both 4.7% versus 1.4%, p=0.556). In-hospital mortality was 0.9%, corresponding to one patient in the long QTc group. The mean follow-up time was 4.8±3.8 years. 5-year all-cause mortality and the occurrence of the composite endpoint of major adverse cardiac and cerebrovascular events did not differ between the groups (p=0.511 and p=0.538, respectively). Our study found no association between prolonged QTc interval during TTS events and adverse prognosis, since in-hospital and long-term outcomes were similar between the groups. Our findings suggest that, although QT prolongation is usually a frightening feature on ECG, this repolarization abnormality during the acute phase has no major prognostic implications in the TTS population.

Dependência de Pacing a Longo-Prazo e Preditores de Implante de Pacemaker após Implante Percutâneo de Prótese Valvular Aórtica - 1 Ano de Seguimento / Long-Term Ventricular Pacing Dependency and Pacemaker Implantation Predictors after Transcatheter Aortic Valve Replacement - A 1-Year Follow-Up

Resumo Fundamento Os distúrbios de condução (DC) são a complicação mais frequente após a substituição da válvula aórtica transcateter (TAVR) e ainda não há consenso sobre seu tratamento. Objetivo Avaliar novos DC e implante de marca-passo definitivo (MPD) após a TAVR e avaliar a porcentagem de estimulação ventricular (EV) até 1 ano de acompanhamento. Métodos Pacientes submetidos a TAVR de outubro de 2014 a novembro de 2019 foram cadastrados; pacientes com MPD anterior foram excluídos. Dados clínicos, do procedimento, do ECG e do MPD foram coletados até 1 ano após o implante. O nível de significância adotado para a análise estatística foi 0,05%. Resultados Um total de 340 indivíduos foram submetidos a TAVR. O DC mais comum foi bloqueio de ramo esquerdo novo (BRE; 32,2%), sendo que 56% destes foram resolvidos após 6 meses. O bloqueio do ramo direito (BRD) foi o maior fator de risco para bloqueio atrioventricular avançado (BAV) [RC=8,46; p<0,001] e implante de MPD [RC=5,18; p<0,001], seguido de BAV de baixo grau prévio [RC=2,25; p=0,016 para implante de MPD]. Em relação às características do procedimento, válvulas de gerações mais recentes e procedimentos de válvula-em-válvula foram associados a menos DC. No total, 18,5% dos pacientes tiveram MPD implantado após a TAVR. Na primeira avaliação do MPD, pacientes com BAV avançado tinham uma porcentagem mediana de EV de 80%, e, após um ano, de 83%. Em relação aos pacientes com BRE e BAV de baixo grau, a EV mediana foi mais baixa (6% na primeira avaliação, p=0,036; 2% após um ano, p = 0,065). Conclusão O BRE foi o DC mais frequente após a TAVR, com mais da metade dos casos se resolvendo nos primeiros 6 meses. O BRD foi o principal fator de risco para BAV avançado e implante de MPD. O BAV avançado foi associado a uma porcentagem mais alta de EV no acompanhamento de 1 ano.

Abstract Background Conduction disturbances (CD) are the most frequent complication after transcatheter aortic valve replacement (TAVR), and there continues to be a lack of consensus on their management. Objective To assess new CD and permanent pacemaker (PPM) implantation after TAVR and to evaluate the ventricular pacing percentage (VP) up to 1 year of follow-up. Methods Patients who underwent TAVR from October 2014 to November 2019 were enrolled; patients with previous PPM were excluded. Clinical, procedure, ECG, and PPM data were collected up to 1 year after implantation. The significance level adopted in the statistical analysis was 0.05. Results A total of 340 patients underwent TAVR. The most frequent CD was the new left bundle branch block (LBBB; 32.2%), which 56% resolved after 6 months. Right bundle branch block (RBBB) was the biggest risk factor for advanced atrioventricular block (AVB) [OR=8.46; p<0.001] and PPM implantation [OR=5.18, p<0.001], followed by previous low-grade AVB [OR=2.25; p=0.016 for PPM implantation]. Regarding procedure characteristics, newer generation valves and valve-in-valve procedures were associated with fewer CDs. Overall, 18.5% of patients had a PPM implanted post-TAVR. At first PPM evaluation, patients with advanced AVB had a median percentage of VP of 80% and 83% at one year. Regarding patients with LBBB plus low-grade AVB, median VP was lower (6% at first assessment, p=0.036; 2% at one year, p = 0.065). Conclusion LBBB was the most frequent CD after TAVR, with more than half being resolved in the first six months. RBBB was the major risk factor for advanced AVB and PPM implantation. Advanced AVB was associated with a higher percentage of VP at 1 year of follow-up.

Long-Term Ventricular Pacing Dependency and Pacemaker Implantation Predictors after Transcatheter Aortic Valve Replacement - A 1-Year Follow-Up. / Dependência de Pacing a Longo-Prazo e Preditores de Implante de Pacemaker após Implante Percutâneo de Prótese Valvular Aórtica ­ 1 Ano de Seguimento.

BACKGROUND: Conduction disturbances (CD) are the most frequent complication after transcatheter aortic valve replacement (TAVR), and there continues to be a lack of consensus on their management. OBJECTIVE: To assess new CD and permanent pacemaker (PPM) implantation after TAVR and to evaluate the ventricular pacing percentage (VP) up to 1 year of follow-up. METHODS: Patients who underwent TAVR from October 2014 to November 2019 were enrolled; patients with previous PPM were excluded. Clinical, procedure, ECG, and PPM data were collected up to 1 year after implantation. The significance level adopted in the statistical analysis was 0.05. RESULTS: A total of 340 patients underwent TAVR. The most frequent CD was the new left bundle branch block (LBBB; 32.2%), which 56% resolved after 6 months. Right bundle branch block (RBBB) was the biggest risk factor for advanced atrioventricular block (AVB) [OR=8.46; p<0.001] and PPM implantation [OR=5.18, p<0.001], followed by previous low-grade AVB [OR=2.25; p=0.016 for PPM implantation]. Regarding procedure characteristics, newer generation valves and valve-in-valve procedures were associated with fewer CDs. Overall, 18.5% of patients had a PPM implanted post-TAVR. At first PPM evaluation, patients with advanced AVB had a median percentage of VP of 80% and 83% at one year. Regarding patients with LBBB plus low-grade AVB, median VP was lower (6% at first assessment, p=0.036; 2% at one year, p = 0.065). CONCLUSION: LBBB was the most frequent CD after TAVR, with more than half being resolved in the first six months. RBBB was the major risk factor for advanced AVB and PPM implantation. Advanced AVB was associated with a higher percentage of VP at 1 year of follow-up.

FUNDAMENTO: Os distúrbios de condução (DC) são a complicação mais frequente após a substituição da válvula aórtica transcateter (TAVR) e ainda não há consenso sobre seu tratamento. OBJETIVO: Avaliar novos DC e implante de marca-passo definitivo (MPD) após a TAVR e avaliar a porcentagem de estimulação ventricular (EV) até 1 ano de acompanhamento. MÉTODOS: Pacientes submetidos a TAVR de outubro de 2014 a novembro de 2019 foram cadastrados; pacientes com MPD anterior foram excluídos. Dados clínicos, do procedimento, do ECG e do MPD foram coletados até 1 ano após o implante. O nível de significância adotado para a análise estatística foi 0,05%. RESULTADOS: Um total de 340 indivíduos foram submetidos a TAVR. O DC mais comum foi bloqueio de ramo esquerdo novo (BRE; 32,2%), sendo que 56% destes foram resolvidos após 6 meses. O bloqueio do ramo direito (BRD) foi o maior fator de risco para bloqueio atrioventricular avançado (BAV) [RC=8,46; p<0,001] e implante de MPD [RC=5,18; p<0,001], seguido de BAV de baixo grau prévio [RC=2,25; p=0,016 para implante de MPD]. Em relação às características do procedimento, válvulas de gerações mais recentes e procedimentos de válvula-em-válvula foram associados a menos DC. No total, 18,5% dos pacientes tiveram MPD implantado após a TAVR. Na primeira avaliação do MPD, pacientes com BAV avançado tinham uma porcentagem mediana de EV de 80%, e, após um ano, de 83%. Em relação aos pacientes com BRE e BAV de baixo grau, a EV mediana foi mais baixa (6% na primeira avaliação, p=0,036; 2% após um ano, p = 0,065). CONCLUSÃO: O BRE foi o DC mais frequente após a TAVR, com mais da metade dos casos se resolvendo nos primeiros 6 meses. O BRD foi o principal fator de risco para BAV avançado e implante de MPD. O BAV avançado foi associado a uma porcentagem mais alta de EV no acompanhamento de 1 ano.

Does the type of seizure influence heart rate variability changes?

OBJECTIVE: Heart 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. METHODS: We 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. RESULTS: We 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/CONCLUSION: This 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.

Heart rate variability in patients with refractory epilepsy: The influence of generalized convulsive seizures.

OBJECTIVE: Patients with epilepsy, mainly drug-resistant, have reduced heart rate variability (HRV), linked to an increased risk of sudden death in various other diseases. In this context, it could play a role in SUDEP. Generalized convulsive seizures (GCS) are one of the most consensual risk factors for SUDEP. Our objective was to assess the influence of GCS in HRV parameters in patients with drug-resistant epilepsy. METHODS: We prospectively evaluated 121 patients with refractory epilepsy admitted to our Epilepsy Monitoring Unit. All patients underwent a 48-hour Holter recording. Only patients with GCS were included (n = 23), and we selected the first as the index seizure. We evaluated HRV (AVNN, SDNN, RMSSD, pNN50, LF, HF, and LF/HF) in 5-min epochs (diurnal and nocturnal baselines; preictal - 5 min before the seizure; ictal; postictal - 5 min after the seizure; and late postictal - >5 h after the seizure). These data were also compared with normative values from a healthy population (controlling for age and gender). RESULTS: We included 23 patients, with a median age of 36 (min-max, 16-55) years and 65% were female. Thirty percent had cardiovascular risk factors, but no previously known cardiac disease. HRV parameters AVNN, RMSSD, pNN50, and HF were significantly lower in the diurnal than in the nocturnal baseline, whereas the opposite occurred with LF/HF and HR. Diurnal baseline parameters were inferior to the normative population values (which includes only diurnal values). We found significant differences in HRV parameters between the analyzed periods, especially during the postictal period. All parameters but LF/HF suffered a reduction in that period. LF/HF increased in that period but did not reach statistical significance. Visually, there was a tendency for a global reduction in our patients' HRV parameters, namely AVNN, RMSSD, and pNN50, in each period, comparing with those from a normative healthy population. No significant differences were found in HRV between diurnal and nocturnal seizures, between temporal lobe and extra-temporal-lobe seizures, between seizures with and without postictal generalized EEG suppression, or between seizures of patients with and without cardiovascular risk factors. SIGNIFICANCE/CONCLUSION: Our work reinforces the evidence of autonomic cardiac dysfunction in patients with refractory epilepsy, at baseline and mainly in the postictal phase of a GCS. Those changes may have a role in some SUDEP cases. By identifying patients with worse autonomic cardiac function, HRV could fill the gap of a lacking SUDEP risk biomarker.

Left ventricular pacing with a temporary pacemaker: Case report.

We report a case of temporary pacemaker lead malposition in the left ventricle crossing the interventricular septum (IVS). The majority of described cases occur due to a patent foramen ovale and are frequently incidental findings. A course across the IVS is rarely found and this complication with temporary leads is not even reported in the literature. This very rare location entails a risk of dangerous complications associated with left-to-right flow after lead removal. Echocardiography was an essential tool to diagnose the lead's course inside the heart and enabled secure removal of the lead with cardiac surgery backup.

Myocardial dysfunction in Takotsubo syndrome: More than meets the eye?

INTRODUCTION: Takotsubo syndrome (TTS) is characterized by transient left ventricular (LV) dysfunction, typically mimicking an anterior wall myocardial infarction (MI), without obstructive coronary artery disease. In the few published reports assessing myocardial deformation in TTS and MI, no consistent differences have been described between the two entities. We sought to characterize global and regional function in TTS and to compare it with a population with MI. METHODS: Clinical data, including echocardiography, were gathered from 17 TTS patients and 20 anterior wall ST-segment elevation myocardial infarction (STEMI) controls. Peak systolic longitudinal strain was determined for each LV segment using speckle tracking imaging, and global and mean apical, midventricular and basal longitudinal strain were calculated from these. RESULTS: Both TTS and STEMI patients presented significant LV systolic dysfunction, and there were no significant differences in ejection fraction or global longitudinal strain. Regional longitudinal strain was more severely impaired in basal inferolateral and mid anterolateral segments in the TTS group and in apical anteroseptal segments in the STEMI group. Mean longitudinal strain was worse in the basal segments of TTS patients (-9.8±2.9 vs. -12.4±4.1%, p=0.010), with no significant differences in mid and apical segments. The basal/apical ratio was significantly lower in this group as well (1.51±0.86 vs. 2.94±1.88, p=0.006). CONCLUSIONS: While both TTS and STEMI feature significantly impaired global systolic function, we found a regional pattern of worse basal longitudinal strain and a lower basal/apical ratio in the former. These suggest generalized myocardial impairment in TTS, providing new clues about its pathophysiology and possible specific echocardiographic changes.

Enfermedad relacionada con IgG4 que se presenta como parada cardiaca / IgG4-related disease presenting as cardiac arrest

No disponible

Percutaneous Closure of a Fistulous Giant Coronary Aneurysm.

Giant coronary artery aneurysms larger than 50 mm are rare and associated with important complications: namely, rupture. Its workup requires comprehensive imaging and standard treatment is surgical exclusion. We present a 60-year-old patient with previous ostium secundum atrial septal defect surgical closure diagnosed with a giant proximal right coronary artery aneurysm (70 x 62 mm) fistulizing into the right atrium. Percutaneous closure of its aortic origin with an atrial septal occluder was successfully performed, and thrombosis of the aneurysm confirmed on angiography and echocardiogram. This case depicts an innovative, minimally invasive approach to this worrisome entity.