Usefulness of exercise test in the diagnosis of short QT syndrome.

AIMS: Short QT syndrome (SQTS) is a rare arrhythmogenic inherited heart disease. Diagnosis can be challenging in subjects with slightly shortened QT interval at electrocardiogram. In this study we compared the QT interval behaviour during exercise in a cohort of SQTS patients with a control group, to evaluate the usefulness of exercise test in the diagnosis of SQTS. METHODS AND RESULTS: Twenty-one SQTS patients and 20 matched control subjects underwent an exercise test. QT interval was measured at different heart rates (HRs), at rest and during effort. The relation between QT interval and HR was evaluated by linear regression analysis according to the formula: QT = ß ×HR + α, where ß is the slope of the linear relation, and α is the intercept. Rest and peak exercise HRs were not different in the two groups. Short QT syndrome patients showed lower QT intervals as compared with controls both at rest (276 ± 27 ms vs. 364 ± 25 ms, P < 0.0001) and at peak exercise (228 ± 27 ms vs. 245 ± 26 ms, P = 0.05), with a mean variation from rest to peak effort of 48 ± 14 ms vs. 120 ± 20 ms (P < 0.0001). Regression analysis of QT/HR relationship revealed a less steep slope for SQTS patients compared with the control group, never exceeding the value of -0.90 ms/beat/min (mean value -0.53 ± 0.15 ms/beat/min vs. -1.29 ± 0.30 ms/beat/min, P < 0.0001). CONCLUSION: Short QT syndrome patients show a reduced adaptation of the QT interval to HR. Exercise test can be a useful tool in the diagnosis of SQTS.

Morbidity and mortality in adults with a Fontan circulation beyond the fourth decade of life.

AIMS: To evaluate the late outcomes of adults (above 35 years) with a Fontan-type circulation, for whom current data on morbidity and mortality are lacking. METHODS AND RESULTS: Data were collected retrospectively on consecutive patients with Fontan circulation above the age of 35 years followed in three European specialist centres. Overall, 115 Fontan patients were included [median age 35 (range 35-48) years, 47.8% female]. The most common underlying congenital heart disease diagnosis was tricuspid atresia (n = 58, 50.4%), and the age at first Fontan completion was 9.1 (interquartile range 5.0-15.8) years. Almost two-thirds (61.7%) of patients had undergone an atriopulmonary Fontan, and 23.5% had received a total cavopulmonary connection. One-third required repeat surgery or intervention. Most patients (55.9%) were in New York Heart Association functional class II or class I (30.6%), 76 (66.1%) patients had experienced at least one arrhythmia, and eight (7.0%) protein-losing enteropathy. At a median follow-up of 5.0 (2.4-10.3) years, 15 (13.0%) patients were referred for transplantation assessment and 19 (16.5%) patients died, mainly from heart failure (84.2%). Univariable predictors of death or transplantation included lower serum albumin level [hazard ratio (HR) 1.09 per g/L decrease, 95% confidence interval (CI): 1.04-1.15, P = 0.0009], prior heart failure admission (HR 4.28, 95% CI:1.75-10.44, P = 0.001), prior atrial tachycardia or flutter (HR 3.02, 95% CI: 1.23-7.38, P = 0.02), and baseline pulmonary vasodilator therapy (HR 8.59, 95% CI:1.05-70.13, P = 0.04). Lower serum albumin and prior atrial tachycardia or flutter remained significant on bivariable analysis. CONCLUSION: Our study highlights the significant morbidity and mortality in older adults with a Fontan-type circulation, emphasizing the need for lifelong specialist surveillance with frequent risk stratification, close monitoring, and early consideration for transplantation assessment.

This study sheds light on the complex medical journey of adults living with the outcomes of Fontan surgery­a procedure performed in early childhood. These individuals have reached the milestone of their forties and beyond, yet they confront an array of significant health challenges that necessitate lifelong, individualized congenital heart disease care. The key findings are as follows: While adults with Fontan circulation are living longer, they are at high risk of death, mainly due to heart failure. They also face a host of other health issues, including the need for additional surgeries or interventions. Nearly two-thirds have experienced some form of heart rhythm problem, and a substantial number eventually require evaluation for a heart transplant.Heart transplants within this group were rare, which may be linked to the various barriers to transplantation in the Fontan population. Moreover, those with multiple indicators of advanced disease have a heightened risk of life-threatening events, reinforcing the critical need for personalized and continuous specialist care designed to meet their distinct health requirements.

The pathophysiology and complications of Fontan circulation.

The Fontan operation has been the final palliation for patients born with congenital heart defects with a functional single ventricle for more than 4 decades. The "normal" Fontan physiology is characterized by the loss of the sub-pulmonary ventricle with consequent elevated pressure in the caval system, non-pulsatile blood flow in the pulmonary circulation and at least mild reduction of the systemic output. When successful, this procedure is associated with a range of benefits including improved arterial saturation and abolishment of chronic volume overload, allowing a fairly normal life to the majority of patients through early adulthood. As we enter the 5th decade of caring for patients palliated with the Fontan procedure, it is evident that adult survivors face significant morbidity due to multiorgan dysfunction, early mortality and need for heart transplantation. Several late complications may occur: ventricular dysfunction, arrhythmia, cyanosis, exercise intolerance, elevated pulmonary vascular resistance, protein-losing enteropathy, plastic bronchitis, hepatic and renal complications. The mechanism of late Fontan failure is multifactorial and not completely understood, it depends on interactions between the ventricle, the pulmonary vascular bed, the venous and lymphatic compartments. Conclusions: the aim of this review is to describe the pathophysiology of Fontan circulation and the clinical and hemodynamic characteristics of early and late failing Fontan survivors, their association with morbidity and mortality, and the strategies for their management.

[Takotsubo syndrome complicated by ventricular thrombosis and pericarditis]. / Sindrome takotsubo complicata da trombosi ventricolare e pericardite.

Takotsubo syndrome is a heart disease characterized by transient ventricular dysfunction; although it is considered a benign pathology, it is not free from serious complications. Intraventricular thrombosis is a rare occurrence as well as pericarditis, and the simultaneous presence of both complications is very exceptional. Here we describe a case. Diagnosis and therapeutic management was successfully guided by multimodality imaging.

Catheterization laboratory activity before and during COVID-19 spread: A comparative analysis in Piedmont, Italy, by the Italian Society of Interventional Cardiology (GISE).

BACKGROUND: COronaVIrus Disease 19 (COVID-19) led to the reorganization of Cardiology Units in terms of working spaces and healthcare personnel. In this scenario, both outpatient visits and elective interventional cardiology procedures were suspended and/or postponed. We aimed to report the impact of COVID-19 on interventional coronary and structural procedures in Piedmont, Italy. METHODS: The number of coronary angiographies (CAG), percutaneous coronary interventions (PCI), primary PCI (pPCI), transcatheter aortic valve replacements (TAVR) and Mitraclip performed in Piedmont between March 1st and April 20th, 2020 (CoV-time) were collected from each catheterization laboratory and compared to the number of procedures performed the year before in the same months (NoCoV-time). RESULTS: Procedural data from 18 catheterization laboratories were collected. Both coronary (5498 versus 2888: difference: -47.5%; mean 305.4 VS 160.4; p = 0.002) and structural (84 versus 17: difference: -79.8%; mean 4.7 Vs 0.9; p < 0.001) procedures decreased during CoV-time compared to NoCoV-time. In particular, coronary angiographies (1782 versus 3460), PCI (1074 versus 1983), p PCI (271 versus 410), TAVR (11 versus 72) and Mitraclip (6 versus 12) showed a reduction of 48.5%, 45.7%, 33.7%, 84.7% and 50.0%, respectively (all p for comparison <0.05). CONCLUSIONS: Compared to the same time-period in 2019, both coronary and structural interventional procedures during COVID-19 epidemic suffered a dramatic decrease in Piedmont, Italy. Organizational change and structured clinical pathways should be created, together with awareness campaigns.

Ventricular conduction delay as marker of risk in Brugada Syndrome. Results from the analysis of clinical and electrocardiographic features of a large cohort of patients.

BACKGROUND: Brugada Syndrome is a genetic arrhythmogenic disease with a variable clinical spectrum. The role of clinical and ECG parameters in the risk stratification is still uncertain. AIMS: In a large cohort of Brugada patients we analysed clinical and ECG features to determine the variables with prognostic value for the occurrence of a first documented arrhythmic event and for recurrences. METHODS: We enrolled 614 patients, subdivided into 3 groups according to their clinical presentation: 531 (88%) asymptomatic, 69 (10%) with previous unexplained syncope and 14 (2%) with aborted sudden death. We also compared the ECG characteristics of patients with a single documented arrhythmic event (either at presentation or at follow-up, 17 patients), with those of patients with arrhythmic recurrences (13 patients). RESULTS: The event rate was 1.3% in the asymptomatic patients and 15% among patients with unexplained syncope (median follow-up 6 years), p < 0.0001. In both groups a QRS duration ≥110 ms in lead II and/or V6 and/or S wave duration ≥40 ms in lead I and/or II were significant risk factors for the occurrence and timing of events at follow-up. The same ECG risk factors were also significantly associated with arrhythmic recurrences. CONCLUSIONS: The arrhythmic risk of Brugada patients is related not only to the symptoms at presentation, but also to the presence of a ventricular conduction delay (QRS duration ≥ 110 ms and/or S wave duration ≥ 40 ms). The ECG conduction parameters also affect the timing of events and recurrences.

Recurrent Pericarditis.

Recurrent pericarditis is the most common and troublesome complication of pericarditis affecting 20% to 50% of patients. Its pathogenesis is often presumed to be immune-mediated, but additional investigations are needed to clarify the pathogenesis in order to develop etiology-oriented therapies. Imaging with computed tomography and especially cardiac magnetic resonance holds promise to help in the identification of more difficult cases and improve their management. Refractory recurrent pericarditis with corticosteroid dependence and colchicine resistance remain still an unsolved issue in search of new therapies, although old drugs such as azathioprine, intravenous immunoglobulins, and biological agents seem promising, but new randomized clinical trials are needed to confirm their role. Despite compromising the quality of life, idiopathic recurrent pericarditis has an overall good long-term outcome without mortality and significant risk of constrictive pericarditis evolution. The risk of constriction, the most feared complication, is related to the etiology and not the number of recurrences.

Etiological diagnosis, prognostic significance and role of electrophysiological study in patients with Brugada ECG and syncope.

BACKGROUND: Syncope is considered a risk factor for life-threatening arrhythmias in Brugada patients. Distinguishing a benign syncope from one due to ventricular arrhythmias is often difficult, unless an ECG is recorded during the episode. Aim of the study was to analyze the characteristics of syncopal episodes in a large population of Brugada patients and evaluate the role of electrophysiological study (EPS) and the prognosis in the different subgroups. METHODS AND RESULTS: One hundred ninety-five Brugada patients with history of syncope were considered. Syncope were classified as neurally mediated (group 1, 61%) or unexplained (group 2, 39%) on the basis of personal and family history, clinical features, triggers, situations, associated signs, concomitant therapy. Most patients underwent EPS; they received ICD or implantable loop-recorder on the basis of the result of investigations and physician's judgment. At 62±45months of mean follow-up, group 1 showed a significantly lower incidence of arrhythmic events (2%) as compared to group 2 (9%, p<0.001). Group 2 patients with positive EPS showed the highest risk of arrhythmic events (27%). No ventricular events occurred in subjects with negative EPS. CONCLUSION: Etiological definition of syncope in Brugada patients is important, as it allows identifying two groups with different outcome. Patients with unexplained syncope and ventricular fibrillation induced at EPS have the highest risk of arrhythmic events. Patients presenting with neurally mediated syncope showed a prognosis similar to that of the asymptomatic and the role of EPS in this group is unproven.

Prevalence of type 1 brugada electrocardiographic pattern evaluated by twelve-lead twenty-four-hour holter monitoring.

Patients with drug-induced type 1 Brugada electrocardiograms (BrECGs) are considered to have good prognosis. Spontaneous type 1 is, instead, considered a risk factor; however, it is probably underestimated because of the BrECG fluctuations. The aim of this study was to analyze, in a large population of patients with Br, the real prevalence of type 1 BrECG using 12-lead 24-hour Holter monitoring (12L-Holter) and its correlation with the time of the day. We recorded 303 12L-Holter in 251 patients. Seventy-five (30%) patients exhibited spontaneous type 1 BrECG at 12-lead ECG (group 1) and 176 (70%) had only drug-induced type 1 (group 2). Type 1 BrECG was defined as "persistent" (>85% of the recording), "intermittent" (<85%), or "absent." In group 1, 12% showed persistent type 1 at 12L-Holter, 57% intermittent type 1%, and 31% never had type 1; in group 2, none had persistent type 1, 20% had intermittent type 1%, and 80% never showed type 1. To evaluate the circadian fluctuations of BrECG, 4 periods in the day were considered. Type 1 BrECG was more frequent between 12-noon and 6 p.m. (52%, p <0.001). In conclusion, in patients with drug-induced type 1, spontaneous type 1 BrECG can be detected more frequently with 12L-Holter than with conventional follow-up with periodic ECGs and this has important implications in the risk stratification. 12L-Holter recording might avoid 20% of the pharmacological challenges with sodium channel blockers, which are not without risks, and should thus be considered as the first screening test, particularly in children or in presence of borderline diagnostic basal ECG.

Atrial fibrillation in a large population with Brugada electrocardiographic pattern: prevalence, management, and correlation with prognosis.

BACKGROUND: A high prevalence of atrial fibrillation/atrial flutter (AF/AFl) has been reported in small series of Brugada patients, with discordant data. OBJECTIVE: The purpose of this study was to analyze, in a large population of Brugada patients, the prevalence of AF/AFl, its correlation with prognosis, and the efficacy of hydroquinidine (HQ) treatment. METHODS: Among 560 patients with Brugada type 1 ECG (BrECG), 48 (9%) had AF/AFl. Three groups were considered: 23 patients with BrECG pattern recognized before AF/AFl (group 1); 25 patients first diagnosed with AF/AFl in whom Class IC antiarrhythmic drugs administered for cardioversion/prophylaxis unmasked BrECG (group 2); and 512 patients without AF/AFl (group 3). Recurrence of AF/AFl and occurrence of ventricular arrhythmias were evaluated at follow-up. RESULTS: Mean age was 47 ± 15 years, 59 ± 11 years, and 44 ± 14 years in groups 1, 2, and 3, respectively. Seven subjects (32%) in group 1 had syncope/aborted sudden death, 1 (4%) in group 2, and 122 (24%) in group 3. Ventricular arrhythmia occurred in three patients in group 1, none in group 2, and 10 in group 3 at median follow-up of 51, 68, and 41 months, respectively. Nine patients in group 1 and nine in group 2 received HQ for AF/AFl prophylaxis; on therapy, none had AF/AFl recurrence. CONCLUSION: Prevalence of AF/AFl in Brugada patients is higher than in the general population of the same age. Patients in group 1 are younger than those in group 2 and have a worse prognosis compared to both groups 2 and 3. HQ therapy has proved useful and safe in patients with AF/AFl and BrECG.