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Obstructive sleep apnoea (OSA) is a very common disease with a prevalence that ranges from 1% to 6% in children. It is characterized by intermittent partial or complete occlusion of the upper airway during sleep, leading to recurrent arousals and disturbed sleep architecture, to neurocognitive disorders and alterations in homeostatic gas exchange. Cardiovascular complications may develop in children with OSA through various mechanisms including activation and dysregulation of the sympathetic nervous system, induction of pro-inflammatory and pro-oxidant status and increased risk of systemic hypertension. As the deleterious effects of OSA on the cardio-vascular system may start early in life, in this brief review we focused our attention both on early and late cardiological changes induced by apnoeic events in the paediatric population, by reviewing recent findings in the literature.

Introduction. The treatment of congenital complete AV block (CCAVB) is burdened by RV-associated ventricular dysfunction at long-term in a subgroup of patients. Methods and Results. Two CCAVB adolescents with mild systolic dysfunction associated to VVIR pacing reached elective pacemaker replacement after 10±1 years. They were upgraded to physiologic stimulation by restoring AV synchrony and ventricular activation via His bundle pacing (HBP). At 9-months follow-up both had reverse left ventricular (LV) remodeling: LV end-diastolic volume index decreased from 89±4 to 70±7 ml/m2, LV end-systolic volume index decreased from 49±1 to 32 ml/m2, LVEF increased from 43±1% to 53±4%. Conclusions. HBP can improve LV function in CCAVB adolescents. It should be considered in the setting of LV dysfunction associated to RV pacing, and should be explored as first-choice treatment from late childhood onward.

Background The upside-down position is a little known modified Valsalva manoeuvre (VM). The aim of this study was to investigate the safety and the efficacy of the upside-down position for the treatment of paroxysmal SVT in children. Methods Twenty-four paediatric patients followed for SVT were enrolled. The patients were assigned (1:1) to a standard VM or to an upside-down position at the first episode of SVT at home. If no cardioversion occurred, a second attempt was undertaken with the other VM. At the patient's first relapse, the intervention protocol was applied in the opposite order at home. Results The upside-down position compared to standard VM reached 67% vs 33% rate of cardioversion at a first attempt, followed by 50% vs 0% rate of cardioversion in patients who had failed the first attempt. After having reversed the order of intervention in case of SVT recurrence, we recorded 67% vs 25% and 71% vs 42% success rates in favour of the upside-down position. There were no adverse events. Conclusion The upside-down position was safe and tended to be more effective than standard VM for out of hospital SVT treatment. Doctors and parents should be more aware of this effective but overlooked manoeuvre.

Background The novel “Chronicle of a death foretold” by Gabriel Garcia Marquez is a story of a sudden death which could have been prevented. In 1976, within the University of Maryland basketball program and only 8 weeks apart, two athletes died suddenly during physical exertion. They were affected by hypertrophic cardiomyopathy and Marfan syndrome and in both cases an echocardiogram would have prevented the tragic epilogue. This coincidence drew everyone's attention and experts' interest on sudden death in sports. Methods and results Even in recent Italian history, unexpected deaths continue to affect athletes but surprisingly any real knowledge regarding the numbers and the impact of those tragedies must take medical literature and non-medical press into consideration. Herein we report the clinical case of a 13-year-old patient with a bicuspid aortic valve, whose mother was alarmed by the news of a young boy who died because of an anomalous origin of coronary artery (AOCA) which had not been diagnosed at transthoracic echocardiography (TTE). Her obstinacy induced the physicians to repeat TTE and led to the same diagnosis in her son: actually, his right coronary artery originated from the opposite sinus of Valsalva. The suspicion was confirmed by coronary CT scan and, thanks to appropriate therapy, the boy now fares well. Conclusions AOCA is the second most common cause of sudden death in young athletes. Although AOCA is often undetectable at ECG, TTE increases sensitivity of preparticipation screening. It could therefore allow us to avoid such coincidences and prevent sudden juvenile death.

The difference between men and women is clear even just by looking at an electrocardiogram: females present higher resting heart rate, a shorter QRS complex length and greater corrected QT interval. The development of these differences from pubertal age onward suggests that sexual hormones play a key role, although their effect is far from being completely understood. Different incidences between sexes have been reported for many arrhythmias, both ventricular and supraventricular, and also for sudden cardiac death. Moreover, arrhythmias are an important issue during pregnancy, both for diagnosis and treatment. Interestingly, cardiovascular structural and electrophysiological remodelling promoted by exercise training enhances this 'gender effect'. Despite all these relevant issues, we lack gender specific recommendations in the current guidelines for electrical therapies for heart rhythm disorders and heart failure. Even more, we continue to see that fewer women are included in clinical trials and are less referred than men for these treatments.

Vincent Van Gogh and Giovanni Pascoli were artists who shared many aesthetic and biographical affinities. Both were somehow intoxicated by digitalis, the painter literally in a pharmacological sense, the poet symbolically or in a literary sense. In the paper we propose an original theory on how digital affected the last works of the Dutch painter and in particular the portrait of doctor Gachet, which differs from the previous theories, that attribute the chromatic style of Van Gogh to the xanthopsia caused by digitalis overdose. The Italian poet dedicated a short poem to the Foxglove, inspired by the popular wisdom that centuries ago already recognized the poisonous power of Foxglove.

Objective To describe clinical presentation, electrocardiographic, and echocardiographic characteristics of carditis at the time of diagnosis of acute rheumatic fever (ARF) over a 13-year period. Study design A single-center retrospective chart analysis was conducted involving all consecutive patients diagnosed with ARF between 2003 and 2015. Patient age, sex, clinical characteristics, recent medical history for group A streptococcal pharyngotonsillitis and antibiotic treatment, and laboratory, echocardiographic, and electrocardiographic findings were recorded. Results Of 98 patients (62 boys, mean age 8.81 ± 3.04 years), 59 (60.2%) reported a positive history of pharyngotonsillitis; 48 (49%) had received antibiotic (mean duration of treatment of 5.9 ± 3.1 days), and, among these, 28 (58.3%) had carditis. Carditis was the second most frequent finding, subclinical in 27% of patients. Mitral regurgitation was present in 49 of 56 patients (87.5%) and aortic regurgitation in 36/56 (64.3%) no stenosis was documented. Conclusions ARF is still present in high-income countries and can develop despite primary prophylaxis, especially when given for a short course. Our findings highlight the need for 10 days of antistreptococcal treatment to prevent ARF. Echocardiography is important because 27% of cases with carditis were subclinical.

1. Identify a QRS complex that falls on a vertical line bordering a large square; the HR is calculated by dividing 300 by the number of large squares that separate it from the next QRS complex. 2. By dividing 60,000 (milliseconds in 1 min) by the interval—in milliseconds—between two P waves (atrial rate) or between two QRS complexes (ventricular rate) (Fig. 4.1).

There is one thing that neonatologists and one of the greatest Italian poets, Giacomo Leopardi, agree on: the first 48 h of man are the most dangerous—man is born with labor, and the risk of death is birth [1, 2]. Even the easiest childbirth involves dramatic adaptations. Dystocia, asphyxia, diaphragmatic hernia, and infectious, toxic, metabolic, or respiratory agents affect the pulmonary circulation, opposing the physiological decline in arterial resistance. The right ventricle is the first to pay the hypoxic-ischemic bill, but in severe cases the left is not much better off. We are talking in these cases of persistent pulmonary hypertension or neonatal transient ischemia. There are mild forms with slight cyanosis and tricuspid regurgitation and lethal forms with severe global dysfunction. The ECG reveals, in order, right overload beyond the norm or diffuse ischemia. Likewise, therapy ranges from just a whiff of oxygen to extracorporeal membrane oxygenation (ECMO); the latter drastic resolution is nowadays often obviated by sildenafil and nitric oxide [3].

Regular exercise activity, especially the aerobic type, is responsible for structural and functional cardiac remodeling with a variety of associated ECG patterns, known as athlete’s heart [1, 2].

Background. There are few data on the mechanism of recurrent neurological events after transcatheter closure of patent foramen ovale (PFO) in cryptogenic stroke or TIA. Methods. We retrospectively reviewed PFO closure procedures for the secondary prevention of cryptogenic stroke/TIA performed between 1999 and 2014 in Bologna, Italy. Results. Written questionnaires were completed by 402 patients. Mean follow-up was 7 ± 3 years. Stroke recurred in 3.2% (0.5/100 patients-year) and TIA in 2.7% (0.4/100 patients-year). Ninety-two percent of recurrent strokes were not cryptogenic. Recurrent stroke was noncardioembolic in 69% of patients, AF related in 15% of patients, device related in 1 patient, and cryptogenic in 1 patient. AF was diagnosed after the procedure in 21 patients (5.2%). Multivariate Cox’s proportion hazard model identified age ≥ 55 years at the time of closure (OR 3.16, p=0.007) and RoPE score p=0.03) as predictors of recurrent neurological events. Conclusion. Recurrent neurological events after PFO closure are rare, usually noncryptogenic and associated with conventional vascular risk factors or AF related. Patients older than 55 years of age and those with a RoPE score

The value of electrocardiography for diagnosing congenital heart disease has decreased considerably since the introduction of fetal ultrasound as routine antenatal screening. In the past, brilliant cardiologists used to deduce the diagnosis of tetralogy of Fallot and the relative degree of oxygen desaturation by judging the S wave in lead V6. Nowadays, an accomplished gynecologist, who suddenly has lots of ultrasound equipment and no ECG experience, can grapple quite successfully with a cardiologic diagnosis. So, univentricular hearts and complex cardiac defects are now expected to be known before the ECG is performed. Nevertheless, some conditions left undetected by antenatal screening come to light in early infancy or even later in life, following a symptomatic or random ECG recording. Right ventricular hypertrophy (RVH) beyond the expected prevalence for the age, “adult” conduction intervals, and abnormal axis deviations could all suggest an underlying CHD (Tables 1.1, 3.4, 18.1, 18.2, and 18.3): left axis deviation of at least −30° is suggestive of atrioventricular canal (AVC) in a “rosy pink” newborn or toddler—which is certain in trisomy 21—or of tricuspid atresia (TA) in a cyanotic baby. The ECG is quite informative in the case of complex cardiac defects and right ventricular overload, while it is much less sensitive in detecting left ventricular overload. Surprisingly, the ECG could be perfect in the transposition of the great arteries (TGA)—a CHD with an absolute ductus dependency—while an asymptomatic CCTGA may leave a number of signs on the ECG even in the neonatal period: the former has a clear clinical presentation with no need of an ECG; the latter could occasionally be encountered in an ECG recording performed due to detection of a “not-quite-innocent” heart murmur or bradycardia. Post-tricuspid shunts, such as ventricular septal defect (VSD) and patent ductus arteriosus (PDA), initially cause left ventricular overload, and, if untreated, can eventually lead to Eisenmenger syndrome with predominant right ventricular hypertrophy. Ebstein anomaly, CCTGV, and isomerism-associated arrhythmias are related to specific cardiac conduction system anomalies. Children who undergo congenital heart treatment procedures are at increased risk of various hyper- and hypokinetic postoperative “incisional” arrhythmias, e.g., atrial reentry or atypical flutter (atrial septal defect, Mustard/Senning correction for the transposition of the great vessels, Fontan procedure for univentricular heart) and ventricular tachycardia (tetralogy of Fallot).

We did not start from the action potential, and we have deliberately not got bogged down in vectorcardiographic analysis. So we can say that Willem Einthoven is our notable absentee. The “child” vision requires an intuitive approach which nevertheless seeks to translate the deep, unconscious electrophysiology into a concept of surface: the vector and its symbol, the arrow shot by ions that travel across the membrane proteins.

Premature ventricular complexes (PVCs) have always been a matter of debate among physicians and a cause for concern for family members of affected patients. The available literature on isolated PVCs in children is limited to case reports and small single-center retrospective series that are consistent in demonstrating the benign course of PVCs and their frequent disappearance during childhood, though many questions remain unanswered. Nevertheless, two key actions should be undertaken in pediatric patients with documented PVCs: 1) to rule out the presence of structural heart disease; 2) to determine whether symptoms are due to PVCs, or whether PVC frequency is responsible for left ventricular dysfunction. This document aims to provide concise and easy to understand recommendations on the diagnostic work-up of healthy children with simple PVCs and the appropriate clinical and therapeutic approach.

We report the case of a boy who was first addressed to medical attention when he was 2-years-old because of an episode of Salmonellosis. His cardiologic evaluation was normal. There were no clinical signs of myocarditis. Three years later he was admitted to the hospital due to a prolonged episode of flu with persistent fever. At that time, his electrocardiogram (ECG) showed junctional rhythm (JR) at 35 bpm. Further ECGs revealed sinus rhythm (SR) with first degree atrio-ventricular block, episodes of sinoatrial (SA) exit blocks, sinus arrests and phases of JR, supporting the diagnosis of sick sinus syndrome (SSS) [1]. Although the boy did not complain of symptoms, Holter monitoring showed frequent pauses of sinus arrest (max interval = 5.4 s) and chronotropic incompetence. At the age of 8 years, he underwent permanent pacemaker (PM) implantation. Although ventricular pacing is less indicated in SSS [2], the patient was treated with VVIR modality because an attempt at right atrial catheter positioning failed, due to inexcitability of the atrium. Since then, the boy has been asymptomatic. Further Holter monitoring showed episodes of paroxysmal atrial fibrillation (Fig. 1) and showed rate responsive pacing with an acceptable mean heart rate of 66 bpm

Kawasaki shock syndrome (KSS) is a rare manifestation of Kawasaki disease (KD) characterized by systolic hypotension or clinical signs of poor perfusion. The objectives of the study are to describe the main clinical presentation, echocardiographic, and laboratory findings, as well as the treatment options and clinical outcomes of KSS patients when compared with KD patients. This is a retrospective study. All children referred to two pediatric rheumatology units from January 1, 2012, to December 31, 2014, were enrolled. Patients were divided into patients with or without KSS. We compared the two groups according to the following variables: sex, age, type of KD (classic, with less frequent manifestations, or incomplete), clinical manifestations, cardiac involvement, laboratory findings, therapy administered, response to treatment, and outcome. Eighty-four patients with KD were enrolled. Of these, five (6 %) met the criteria for KSS. Patients with KSS had higher values of C-reactive protein (p = 0.005), lower hemoglobin levels (p = 0.003); more frequent hyponatremia (p = 0.004), hypoalbuminemia (p = 0.004), and coagulopathy (p = 0.003); and increase in cardiac troponins (p = 0.000). Among the KSS patients, three had a coronary artery involvement, but none developed a permanent aneurysm. Intravenous immunoglobulin resistance was more frequent in the KSS group, although not significantly so (3/5, 60 % vs. 23/79, 30 %, P = NS). None of the five cases was fatal, and all recovered without sequelae. KSS patients are more likely to have higher rates of cardiac involvement. However, most cardiovascular abnormalities resolved promptly with therapy.

In medical literature, few cases of Rasmussen's aneurysm have been reported until now, and to the best of our knowledge, there has been no description of a case of Rasmussen's aneurysm in childhood. The child described in the case report had a restrictive cardiomyopathy, complicated by pulmonary hypertension, in association with miliary tuberculosis.

In medical literature, few cases of Rasmussen's aneurysm have been reported until now, and to the best of our knowledge, there has been no description of a case of Rasmussen's aneurysm in childhood. The child described in the case report had a restrictive cardiomyopathy, complicated by pulmonary hypertension, in association with miliary tuberculosis.

Syncope is common in pediatric population, carrying a significant health and psychological impact. The incidence of syncope in the pediatric age that requires medical intervention is estimated at 125 of 100,000 subjects (0.125%). An incidence peak occurs around the age of 15 years, with females having more than twice the incidence of males. A lower peak occurs in older infants and toddlers, most commonly referred to as “breath-holding spells.†In the pediatric age, the most common etiology is certainly neurally mediated syncope (61—80%), followed by neurological—neuropsychiatric loss of consciousness (LOC) (11—19%), and cardiac syncope (6—11.5%). The etiology remains undetermined in 15—20% of cases. © Springer International Publishing Switzerland 2015.

In recent years, medical and surgical therapy has progressed such that even children with the most complex cardiac disease may reach adulthood with an acceptable quality of life. However, apart from this minority, pediatricians and cardiologists deal with diseases such as cardiomyopathies, arrhythmias, channelopathies and other acquired heart diseases. The majority of patients can be problematic 'cause of a cardiac murmur or in obtaining a certificate of sports eligibility. Following recent regulations, in Italy the electrocardiogram (ECG) must be performed also in 6-year-old children who want to practice sport. Although the ECG is a simple and inexpensive tool with good diagnostic accuracy, there remains the issue of false positives that results in additional costs and alarms. The modern era is facing a pandemic, that is, the spread of digital lifestyle and obesity. The only vaccine against this plague is exercise. Denying sport to children for a false positive test may expose them to obesity, hypertension, diabetes and other bad habits. For some, it may be preferable to accept the infinitesimal risk of sudden death rather than being condemned to a chronic life. Like all therapies, sports can have side effects and overdoses. If this happens in the most dramatic way - cardiac arrest - there is the antidote (i.e., the automated external defibrillator). More than 100 years since its birth, the ECG retains a sustainable and irreplaceable lightness. Nevertheless, the ECG seems to suffer from a sort of collective dyslexia. As cardiologists, we should learn to read pediatric ECG and minimize the false positive rate to prevent a healthy child from having a worse quality of life than cardiac patients saved from modern cardiac surgery.

The aim of the study was to evaluate the time course of atrial and ventricular function improvement following internal atrial cardioversion in patients with structural heart disease. Twenty-nine patients with chronic persistent atrial fibrillation (AF) and underlying structural heart disease were followed by serial echocardiograms performed at 1 and 6 hours, 1 day, 1, 2, and 3 weeks, and 1, 2, 3, and 6 months after successful cardioversion. Sinus rhythm was maintained at 6 months in 24 patients. Following cardioversion the time course of left atrial mechanical function (peak A wave, percent A wave filling) differed from that of left ventricular ejection fraction: peak A wave values (cm/s) increased significantly at 1 week (51 +/- 23 vs 35 +/- 15 at 1 hour, P < 0.05), percent A wave filling (%) increased significantly at 2 weeks (34 +/- 12 vs 22 +/- 9 at 1 hour, P < 0.05), whereas left ventricular ejection fraction (%) increased later (at 1 month 60 +/- 14 vs 55 +/- 14 at baseline, P < 0.05 and at 2 months 60 +/- 14 vs 56 +/- 14 at 1 hour, P < 0.05). In conclusion, restoration of sinus rhythm results in an improvement in left ventricular ejection fraction during follow-up, even in patients with structural heart disease without fast ventricular rates at baseline. The dissociation between the time course of atrial and ventricular function improvement suggests that the latter was partly due to regression of a concealed form of cardiomyopathy and/or of a ventricular dysfunction due to chronic AF.

Objective The persistence of a left superior vena cava(LSVC) has been observed in 0.3% of the general population asestablished by autopsy. In the adult population, it is an importantanatomic finding if a left superior approach to the heart isconsidered. The aim of the study was to evaluate the prevalence of aLSVC in patients undergoing pacemaker (PM) andcardioverter-defibrillator (CD) implantation. Design We observed the prevalence of LSVC during a 10-year period; eachpatient undergoing PM or transvenous CD implantation received a leftcephalic/left subclavian venous approach to the heart. With thistechnique, LSVC persistence is easily diagnosed during leadplacement. Results A total of 1,139 patientsconsecutively underwent PM implantation during 10 years: 4 patients hadpersistent LSCV (0.34%). Among 115 patients undergoing CDimplantation, 2 patients with LSVC (1.7%) were observed. Overall LSVCpersistence was found in 6 of 1,254 patients (0.47%). Two patients, one of whom had no right superior vena cava (RSVC), received aleft-sided PM, whereas two other patients received right-sided devices. Both CD patients received a left-sided active-can device: the firstpatient with a right-sided lead tunneled to the left pectoral pocket, as a result of poor catheter handling through the LSVC and coronarysinus, and the second patient with a screw-in lead from LSVC. Long-termfollow-up of these patients (average ± SD, 41 ± 26 months)revealed absence of lead dislodgment and appropriate device functionregardless of lead implantation site. Conclusions Persistence of LSVC in adults undergoing PM/CD implantation is similarto that of the general population (0.47% in our study). The left-sidedimplant can be achieved by stylet shaping and by use of active fixationleads in most patients, with a reliable outcome at short term inaddition to appropriate device performance at follow-up. Assessment ofthe RSVC is advisable when planning a right-sided implantation, since aminority of patients lacks this vessel.

Objective: The natural history of pediatric patients with severely symptomatic neurocardiogenic syncope is poorly defined respect to the likelihood of remission or symptomatic recurrence along time. We undertook this study to investigate the likelihood of clinical relapse, and to assess the effect of prophylactic pharmacological treatment in the most symptomatic patients. Methods: Twenty-nine patients with neurocardiogenic syncope were studied at our Institution: 14 (12 ± 3.6 years) highly symptomatic received prophylactic therapy with β-blockers guided by head up tilt (HUT), whereas 15 (12.2 ± 2.7 years) moderately symptomatic received only education to avoid triggering of the vasovagal reflex and to abort forthcoming syncope. Patients were then followed respectively for 33.7 ± 9.0 and 33.3 ± 8.7 months (p = NS). Results: The average duration of symptoms before HUT was 9.0 ± 4.3 months (range 3–17) for treated patients, and 6.2 ± 2.5 months (range 2–11) for those untreated ( p < 0.05). Treated patients had also a greater number of symptomatic events: 6 ± 2 vs. 2 ± 1 (p < 0.001). During follow up, 9/15 untreated and 6/14 treated patients had at least 1 recurrence, with an odds ratio of 2 (95% CI 0.72–5.49). Clinical events were greatly reduced in both groups at follow up, but treated patients had a significantly greater reduction either of syncopal (p < 0.001) or near syncopal events (p < 0.02). Time to the first recurrence, syncope or near syncope, was shorter for untreated vs treated patients: 5 ± 2 vs. 25 ± 12 months (p < 0.001). Looking at the time course of all clinical recurrences, 23/26 occurred in untreated patients, whereas 7/10 occurred in treated patients within 24 months. An attempt to therapy discontinuation was made after 30 months in 4 patients, and resulted in half of them being asymptomatic, and half with a single minor recurrence. Conclusions: Spontaneous reduction of symptoms occurs along time in pediatric patients with neurocardiogenic syncope, so that recurrences are very unlikely after 24 months from first diagnosis. Tiered prophylactic therapy may be guided by HUT in selected highly symptomatic patients; β-blockers appear a very effective intervention. Larger, prospective controlled studies are required to investigate the role of any intervention in moderately symptomatic patients.

Introduction Electrical shocks delivered for atrial cardioversion (CV) may cause myocardial damage. The aim of this study was to assess the extent of myocardial injury caused by repeated intracardiac shocks delivered for low-energy internal atrial CV. Methods and results Thirty-five patients with chronic persistent atrial fibrillation (AF) of different etiologies underwent CV with delivery of synchronized biphasic shocks (3.0/3.0 ms) between two catheters positioned in the right atrium and the coronary sinus. Shocks were delivered according to a step-up protocol (50 V, 180 V, then steps of 40 to 56 V up to 500 V, if necessary). In 23 patients, AF was reinduced after baseline CV, and CV was repeated. Myocardial injury was monitored by measuring cardiac troponin I (cTnI) serum concentrations in blood samples taken at baseline and at 2, 4, 8, 12, and 24 h after the procedure, by means of an immunoenzymologic assay (normal values, ≤ 0.6 ng/mL). A mean (± SD) of 6.9 ± 3.4 shocks per patient were delivered (range, 2 to 17). Shocks delivered in each patient had a maximal energy of 7.3 ± 4.0 J (range, 1.7 to 15.7). In 20 patients (57%), no evidence of myocardial injury (cTnI level, ≤ 0.6 ng/mL) was found. In 13 patients (37%), mildly elevated cTnI levels (range, 0.7 to 1.4 ng/mL) in samples taken 4 to 12 h after CV suggested minor myocardial injury. In two patients (6%), higher cTnI levels were found in samples taken 4 to 8 h after CV (peak, 1.7 and 2.4 ng/mL), indicating a necrotic damage. Patients with no cTnI elevation, with mild cTnI elevation, or with cTnI levels≥ 1.5 ng/mL did not differ significantly with respect to the total number of shocks delivered, the mean amount of energy delivered, and the cumulative amount of energy delivered. No clinical complications were observed. Conclusions Following internal CV with the delivery of repeated shocks, minor elevations of cTnI serum levels could be detected in a significant proportion of patients, and this suggests subtle asymptomatic minor myocardial injury. The elevations of cTnI levels do not appear to be related to the number of shocks or to the amount of energy delivered.

Objectives The aim of the study was to evaluate the effects of intravenous (IV) flecainide on defibrillation energy requirements in patients treated with low-energy internal atrial cardioversion. Background Internal cardioversion of atrial fibrillation is becoming a more widely accepted therapy for acute episode termination and for implantable atrial defibrillators. Methods Twenty-four patients with atrial fibrillation (19 persistent, 5 paroxysmal) underwent elective transvenous cardioversion according to a step-up protocol. After successful conversion in a drug-free state, atrial fibrillation was induced by atrial pacing; IV flecainide (2 mg/kg) was administered and a second threshold was determined. In patients in whom cardioversion in a drug-free state failed notwithstanding a 400- to 550-V shock, a threshold determination was attempted after flecainide. Results Chronic persistent atrial fibrillation was converted in 13/19 (68%) patients at baseline and in 16/19 (84%) patients after flecainide. Paroxysmal atrial fibrillation was successfully cardioverted in all the patients. A favorable effect of flecainide was observed either in chronic persistent atrial fibrillation (13 patients) or in paroxysmal atrial fibrillation (5 patients) with significant reductions in energy requirements for effective defibrillation (persistent atrial fibrillation: 4.42 ± 1.37 to 3.50 ± 1.51 J, p < 0.005; paroxysmal atrial fibrillation: 1.68 ± 0.29 to 0.84 ± 0.26 J, p < 0.01). In 14 patients not requiring sedation, the favorable effects of flecainide on defibrillation threshold resulted in a significant reduction in the scores of shock-induced discomfort (3.71 ± 0.83 vs. 4.29 ± 0.61, p < 0.005). No ventricular proarrhythmia was observed for any shock. Conclusions Intravenous flecainide reduces atrial defibrillation threshold in patients treated with low-energy internal atrial cardioversion. This reduction in threshold results in lower shock-induced discomfort. Additionally, flecainide may increase the procedure success rate in patients with chronic persistent atrial fibrillation.

Giustetto et al .1 have reported the largest available study of patients affected by short QT syndrome, an extremely rare genetic ion channel disease that has only recently been identified and described.2,3 The results of this multicentre study reinforce concepts gleaned from previous reports. Short QT syndrome is a genetic arrhythmogenic disease with a high risk of both syncope and sudden death throughout life. The study was based on data from 25 patients belonging to eight affected families, plus a further four sporadic cases. Although syncopes were reported, cardiac arrest was the most common event and often prompted the initial diagnosis of short QT syndrome. Notably, sudden death events occurred in the first year of life, as well as in adolescent age and adulthood. Moreover, other cases of sudden death were traced in apparently healthy family members (usually younger than 40 years) for whom no ECG was available. Thus, this new study reinforces the concept that sudden death is the most relevant clinical manifestation of short QT syndrome and that these events may occur in infants and other children. Short QT syndrome is now included among the set of arrhythmogenic diseases that can lead to sudden death during infancy and childhood ( Table 1 ). In all these diseases, an implantable cardioverter-defibrillator (ICD) is currently proposed for prevention of sudden death in high-risk patients.4 In most of the diseases (those with purely electrical abnormalities and otherwise normal systolic ventricular function), sudden death due to ventricular tachyarrhythmias is the dominant cause of premature death and alone accounts for almost all the difference in life expectancy … *Corresponding author. Tel: +390 51349858; fax: +390 51344859. E-mail address : cardio1{at}med.unibo.it