A Rare Case of Single Coronary Artery With Pulmonary Artery Fistula.

Background: Single coronary artery is a rare congenital anomaly. Its coexistence with coronary artery fistula is exceedingly uncommon.

Case Summary: A 61-year-old woman with no cardiovascular risk factors underwent her first cardiological evaluation after incidental detection of atrial fibrillation.

Yield of Genetic Testing in Pediatric Cardiomyopathies: Implications for Novel Therapeutic Options.

Pediatric cardiomyopathies are rare, heterogeneous, and challenging conditions, often with a genetic etiology. We estimated the yield of genetic testing in a pediatric cohort with cardiomyopathies and evaluated the potential candidacy to current or emerging treatments based on genetic results. Over one-third had a conclusive genetic test, including 25% of potential candidates for emerging precision therapies or developing pharmacological options.

[Management of arrhythmias during pregnancy: a literature review in light of the Heart Rhythm Society consensus document].

Maternal mortality represents a crucial indicator of the quality of care, as stated by the World Health Organization. Non-obstetric mortality in Italy is equally derived from cardiovascular and hypertensive problems with an upward trend. The increased incidence of cardiovascular risk factors, the rise in maternal age, and the use of assisted reproduction suggest a growing impact of arrhythmias on maternal morbidity and mortality.

Congenital Heart Disease After Mid-Age: From the "Grown-Up" to the Elderly.

Early surgery and improved medical care have led to the increased survival of neonates with congenital heart disease (CHD), who now commonly reach adulthood. Among adults with CHD, a growing subgroup is represented by middle-aged and even elderly patients. In this elderly population, acquired cardiac and extracardiac comorbidities represent the main cause of morbidity and mortality; the control and correction of cardiovascular risk factors or an appropriate check for extracardiac complications (such as malignancies) is therefore of paramount importance.

Family caregiver frailty in adult congenital heart disease is overlooked: A call to action.

The frailty of family caregivers (FCs) of adults with congenital heart disease (CHD) is an underrecognized yet critical issue in healthcare. As individuals with CHD live longer, their FCs, often aging alongside them, face escalating physical, emotional, and psychological challenges. This brief report examines frailty in FCs as a multidimensional issue stemming from prolonged caregiving demands and cumulative stress.

[Twin pregnancy in a young woman with Fontan circulation].

We report the case of a young woman with Fontan circulation who successfully completed a twin pregnancy. The key role of a multidisciplinary Pregnancy Heart Team for preconception counseling and pregnancy management is emphasized.

[Italian Society of Pediatric Cardiology (SICP) position paper on the prevention, diagnosis, treatment and follow-up of cardiotoxicity in pediatric patients with cancer].

The survival of pediatric cancer patients has significantly increased thanks to the improvement of oncological treatments. Therefore, it is of utmost importance to manage short- and long-term cardiovascular complications. In pediatric cardio-oncology, there are no recognized guidelines as in adults.

Borderline Ventricles: From Evaluation to Treatment.

A heart with a borderline ventricle refers to a situation where there is uncertainty about whether the left or right underdeveloped ventricle can effectively support the systemic or pulmonary circulation with appropriate filling pressures and sufficient physiological reserve. Pediatric cardiologists often deal with congenital heart diseases (CHDs) associated with various degrees of hypoplasia of the left or right ventricles. To date, no specific guidelines exist, and surgical management may be extremely variable in different centers and sometimes even in the same center at different times.

Hereditary Thoracic Aortic Diseases.

Advances in both imaging techniques and genetics have led to the recognition of a wide variety of aortic anomalies that can be grouped under the term 'hereditary thoracic aortic diseases'. The present review aims to summarize this very heterogeneous population's clinical, genetic, and imaging characteristics and to discuss the implications of the diagnosis for clinical counselling (on sports activity or pregnancy), medical therapies and surgical management.

Cardiac Involvement in Classical Organic Acidurias: Clinical Profile and Outcome in a Pediatric Cohort.

Background: Cardiac involvement is reported in a significant proportion of patients with classical organic acidurias (OAs), contributing to disability and premature death. Different cardiac phenotypes have been described, among which dilated cardiomyopathy (DCM) is predominant. Despite recent progress in diagnosis and treatment, the natural history of patients with OAs remains unresolved, specifically with regard to the impact of cardiac complications.

Focus on Paediatric Restrictive Cardiomyopathy: Frequently Asked Questions.

Restrictive cardiomyopathy (RCM) is characterized by restrictive ventricular pathophysiology determined by increased myocardial stiffness. While suspicion of RCM is initially raised by clinical evaluation and supported by electrocardiographic and echocardiographic findings, invasive hemodynamic evaluation is often required for diagnosis and management of patients during follow-up. RCM is commonly associated with a poor prognosis and a high incidence of heart failure, and PH is reported in paediatric patients with RCM.

Overcoming Underpowering in the Outcome Analysis of Repaired-Tetralogy of Fallot: A Multicenter Database from the CMR/CT Working Group of the Italian Pediatric Cardiology Society (SICPed).

Managing repaired tetralogy of Fallot (TOF) patients is still challenging despite the fact that published studies identified prognostic clinical or imaging data with rather good negative predictive accuracy but weak positive predictive accuracy. Heterogeneity of the initial anatomy, the surgical approach, and the complexity of the mechanism leading to dilation and ventricular dysfunction explain the challenge of predicting the adverse event in this population. Therefore, risk stratification and management of this population remain poorly standardized.

Management of Residual Risk in Chronic Coronary Syndromes. Clinical Pathways for a Quality-Based Secondary Prevention.

Chronic coronary syndrome (CCS), which encompasses a broad spectrum of clinical presentations of coronary artery disease (CAD), is the leading cause of morbidity and mortality worldwide. Recent guidelines for the management of CCS emphasize the dynamic nature of the CAD process, replacing the term "stable" with "chronic", as this disease is never truly "stable". Despite significant advances in the treatment of CAD, patients with CCS remain at an elevated risk of major cardiovascular events (MACE) due to the so-called residual cardiovascular risk.

[Arrhythmias in adult congenital heart disease at the emergency department: ANMCO Tuscany clinical pathway].

Arrhythmias are a common complication in the adult population with congenital heart disease (ACHD). Arrhythmias often lead to hemodynamic instability and, on the other hand, may be a marker of hemodynamic impairment in ACHD patients, both in natural history and after cardiac surgery. Treatment requires knowledge of basic anatomy and any previous cardiac surgery; the availability of patient's health records, if possible, is therefore crucial for therapeutic choices.

[Heart involvement in multisystem inflammatory syndrome in children correlated with SARS-CoV-2 infection: a review by ANMCO/SICP].

Acute clinical manifestations of COVID-19 are generally less severe in childhood, however a proportion of them can develop a severe systemic hyperinflammatory syndrome after SARS-CoV-2 infection, known as the multisystem inflammatory syndrome (multisystem inflammatory syndrome in children, MIS-C). Cardiovascular manifestations in MIS-C are frequent (34-82%), including myocardial dysfunction, coronary artery dilation or aneurysms, arrhythmias, conduction abnormalities, pericarditis and valvulitis. The most affected cases can develop cardiogenic shock needing intensive care unit admission, inotropic support and sometimes even mechanical circulatory support.

Aortic Dilatation in Pediatric Patients with Bicuspid Aortic Valve: How the Choice of Nomograms May Change Prevalence.

Background: Aortic dilation (AoD) is commonly reported in patients with bicuspid aortic valve (BAV) and has been related to flow abnormalities and genetic predisposition. AoD-related complications are reported to be extremely rare in children. Conversely, an overestimate of AoD related to body size may lead to excess diagnoses and negatively impact quality of life and an active lifestyle.