[Tachyarrhythmias in congenital heart disease patients: diagnosis, clinical presentation and treatment]. / Tachiaritmie nel paziente con cardiopatia congenita: diagnosi, presentazione clinica e trattamento.

Tachyarrhythmias are a major cause of morbidity and mortality in adult congenital heart disease patients. There is a strong association between tachyarrhythmias and the specific congenital heart disease and the patient's surgical history. Health care for adults with congenital heart disease and arrhythmias should be coordinated by adult congenital heart disease centers of excellence that serve as a resource for consultation and referral. A complete and multidisciplinary evaluation of patients with tachyarrhythmias, including a detailed hemodynamic and electrophysiological evaluation, is mandatory to define the best treatment strategy.

Diagnosis and treatment of fetal and pediatric age patients (0-12 years) with Wolff-Parkinson-White syndrome and atrioventricular accessory pathways.

Overt or concealed accessory pathways are the anatomic substrates of ventricular preexcitation (VP), Wolff-Parkinson-White syndrome (WPW) and paroxysmal supraventricular tachycardia (PSVT). These arrhythmias are commonly observed in pediatric age. PSVT may occur at any age, from fetus to adulthood, and its symptoms range from none to syncope or heart failure. VP too can range from no symptoms to sudden cardiac death. Therefore, these arrhythmias frequently need risk stratification, electrophysiologic study, drug or ablation treatment. In this review of the literature, recommendations are given for diagnosis and treatment of fetal and pediatric age (≤12 years) WPW, VP, PSVT, and criteria for sport participation.

Nutrient intakes in adult and pediatric coeliac disease patients on gluten-free diet: a systematic review and meta-analysis.

Celiac Disease (CD) continues to require a strict lifetime gluten-free diet (GFD) to maintain healthy status. Many studies have assessed the GFD nutritional adequacy in their cohorts, but an overall picture in adults and children would offer a lifetime vision to identify actionable areas of change. We aimed at assessing the nutrient intakes of adult and pediatric CD patients following a GFD diet and identifying potential areas of improvement. Systematic review was carried out across PubMed, Scopus and Scholar up to October 2022, including full-text studies that assessed the nutrient intakes of CD patients on GFD, in terms of macro- and/or micronutrients (absolute or percentage daily average). Random-effect meta-analysis and univariable meta-regression were applied to obtain pooled estimates for proportions and influencing variables on the outcome, respectively. Thirty-eight studies with a total of 2114 patients were included. Overall, the daily energy intake was 1995 (CI 1884-2106) Kcal with 47.8% (CI 45.7-49.8%) from carbohydrates, 15.5% (CI 14.8-16.2%) from proteins, and 35.8% (CI 34.5-37.0%) from fats. Of total fats, 13.2% (CI 12.4-14.0%) were saturated fats. Teenagers had the highest consumption of fats (94.9, CI 54.8-134.9 g/day), and adults presented insufficient dietary fiber intake (18.9 g, CI 16.5-21.4 g). Calcium, magnesium, and iron intakes were particularly insufficient in adolescence, whereas vitamin D was insufficient in all age groups. In conclusion, GFD may expose CD patients to high fat and low essential micronutrient intakes. Given GFD is a lifelong therapy, to prevent the occurrence of diseases (e.g. cardiovascular or bone disorders) dietary intakes need to be assessed on long-term follow-ups.

Sudden cardiac death related to left coronary artery anomalies including hypoplasia and anomalous origin with retro-aortic course.

Congenital anomalies of the coronary arteries are a rare condition with an incidence of 0.3-1.3% in the general population. Clinically, sometimes these anomalies increase the risk of myocardial ischemia, which can present with a wide spectrum of symptoms, from angina to sudden cardiac death (SCD). This case report is about the SCD of an 8-year-old male, in apparent good health, during a football training. Although basic life support maneuvers were performed timely from bystanders and medical staff, the automated external defibrillator (AED) was not used. Autopsy revealed multiple left coronary artery (LCA) anomalies: origin from a separate ostium in the right sinus of Valsalva, slit-like shape of the ostium, acute angle take-off of the LCA from the aorta, retro-aortic course and focal coronary hypoplasia of some branches of the LCA. Microscopic examination revealed diffuse ischemic consequences at a different stage of tissue repair and mild multifocal lymphocytic infiltration. No other significant elements were detected at post-mortem examination. We discuss the forensic evaluation about the cause and the manner of death, considering also the modality of the resuscitation attempts and the claimed malpractice, as often occurs in case of sudden unexpected death in young athletes.

Cardiovascular issues in obstructive sleep apnoea in children: A brief review.

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.

Chronicle of a death foretold. It is time for echocardiographic screening in young athletes.

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.

Carditis in Acute Rheumatic Fever in a High-Income and Moderate-Risk Country.

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.

[Vincent Van Gogh and Giovanni Pascoli, foiled by the foxglove.] / Il giallo digitale di Van Gogh e Pascoli.

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.

Upside-down position for the out of hospital management of children with supraventricular tachycardia.

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.

Premature ventricular complexes in children with structurally normal hearts: clinical review and recommendations for diagnosis and treatment.

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.

The effects of gender on electrical therapies for the heart: physiology, epidemiology, and access to therapies.

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.

Describing Kawasaki shock syndrome: results from a retrospective study and literature review.

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.

Recurrent Stroke after Transcatheter PFO Closure in Cryptogenic Stroke or Tia: Long-Term Follow-Up.

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 < 7 (OR 3.21, 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 < 7 are likely to get less benefit from PFO closure. After transcatheter PFO closure, lifelong strict vascular risk factor control is warranted.

[The heart between the risk of sudden death and chronic life]. / Il cuore tra morte improvvisa e vita cronica.

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.