[The last 50 years of pediatric cardiology (1971-2021): from Fontan operation to the present time].

Pediatric cardiology has matured profoundly over the last 50 years, paralleled by a similar development in pediatric cardiac surgery and cardiac anesthesia. This field of medicine provides structured and effective care for a very heterogeneous group of diseases including congenital heart disease, cardiomyopathies and heart failure, myocarditis, rheumatic heart disease, inherited and acquired arrhythmias, Kawasaki disease and more recently multisystemic inflammatory syndrome of children related to primary infection by SARS-CoV-2. This review summarizes achievements and results in selected topics of pediatric cardiology and cardiac surgery with focused attention to the diagnosis and management of congenital heart diseases.

Multimodality imaging, single center, cross-sectional study in adolescents or young adults with repaired tetralogy of Fallout.

Background: Proper integration of multiple imaging modalities in the routine follow-up of patients with repaired tetralogy of Fallout (TOF) is poorly supported by data. We report our single center comparative study between cardiac magnetic resonance (CMR) and echocardiography to assess equipoise in the clinical utility of these two imaging methods in an unselected consecutive cohort of TOF patients referred to our outpatient clinic.

Material And Methods: In this cross-sectional study, repaired TOF patients who underwent CMR and echocardiography within a 4-week period between 2010 and 2011 at our Center were included.

[Aortic root dilatation rate in pediatric patients with Marfan syndrome treated with losartan].

Background: Medical therapy with angiotensin II receptor blockers/angiotensin-converting enzyme inhibitors and/or beta-blockers was reported to reduce aortic root dilatation rates in pediatric patients with Marfan syndrome. No data are available in the literature on losartan effects after 3 years of therapy. The aim of our study was to establish whether losartan reduces aortic root dilatation rates in pediatric patients with Marfan syndrome in the mid and long term.

Diagnostic Accuracy of Aortic Root Cross-sectional Area/Height Ratio in Children and Young Adults with Marfan and Loeys-Dietz Syndrome.

Background: Accurate quantification of aortic dilatation is critical in children with syndromes associated with thoracic aortic aneurysm, yet classification of normality is difficult. Current methods of normalization use body surface area to account for growth, despite a nonlinear relationship of body surface area to aortic root dimensions. In contrast, height has a linear relationship with aortic root dimensions in normal children, is simple to measure and requires no secondary calculation.

Hydration properties of small hydrophobic molecules by Brillouin light scattering.

We study the relaxation of water molecules next to hydrophobic solutes with different functional groups by Brillouin light scattering. Evidence is given for (i) water activation energy in trimethylamine-N-oxide, proline and t-butyl alcohol diluted solutions which is comparable to that of neat water, almost independent from solute mole fraction and (ii) moderate slowdown of relaxation time of proximal water compared to the bulk, which is consistent with excluded volume models. Assuming that the main contribution to viscosity comes from bulk and hydration water, a rationale is given of the phenomenological Arrhenius' laws for the viscosity of diluted aqueous solutions.

Rasmussen's aneurysm in childhood: a case report.

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.

[Results and complications of permanent pacing in pediatric patients with congenital or acquired atrioventricular block].

Background: Currently there is no evidence to prefer an endocardial or epicardial approach for pacing in pediatric patients. This analysis was aimed at defining the complications of pacemaker implantation in a pediatric population with atrioventricular block according to a strategy of choosing an epicardial system for patients <10 kg and an endocardial system for patients >10 kg.

Methods: This is a retrospective study performed on 27 patients, with and without congenital heart disease, implanted with a permanent pacemaker because of idiopathic or acquired atrioventricular block at our Pediatric Cardiology and Cardiac Surgery Unit of S.

Exercise training improves exercise capacity in adult patients with a systemic right ventricle: a randomized clinical trial.

Objective: To assess whether exercise training in adult patients with a systemic right ventricle (RV) improves exercise capacity and quality of life and lowers serum N-terminal prohormone brain natriuretic peptide (NT-proBNP) levels.

Design: Multi-centre parallel randomized controlled trial.

Participants: Patients with a systemic RV due to congenitally or surgically corrected transposition of the great arteries.

An Unusual Case of Sinus of Valsalva Aneurysm in a GUCH Patient: An Unusual Side of the Aorto-Cardiac Fistula.

Sinus of Valsalva aneurysm can be congenital or acquired. The major complication is rupture: this can represent an evolution or an abrupt event. In case of rupture or of large aneurysm, a surgical approach is justified.

Calcified Thrombus in Right Atrium: Rare but Treatable Complication of Long-term Indwelling Central Venous Catheter.

Catheter-related central thrombosis is a rare complication of long-term central line. We describe the case of an asymptomatic boy who was diagnosed a calcified thrombus in right atrium eight years after the removal of a long-term central venous device. Although the most appropriate therapeutic approach for managing floating right heart thrombi remains to be determined, surgical removal is an effective and safe procedure for calcified long-standing thrombus and it is to be preferred in elective conditions especially in young asymptomatic patients without hemodynamic involvement, that are at low risk of surgery-related morbidity and mortality.

[The ECG in pediatric patients: what the cardiologist needs to know].

During life ECG is always changing according to postnatal circulation adaptation, position of the heart in the chest, and body mass. Reference values and normal morphologies of P wave, PR interval, QRS complex, ST segment, T wave, and QTc interval are reported. Some clues to detect congenital and acquired heart diseases are also provided.

[The "big heart" of carnitine].

Fat is the primary energy source for heart muscle. In the heart, carnitine is essential for normal fatty acid beta-oxidation and even partial deficiency could lead to organ dysfunction. Dilated cardiomyopathy is one of the cardinal manifestations of carnitine deficiency in children.

[Percutaneous aortic valvuloplasty in congenital aortic valve stenosis performed in patients older than one month: a good alternative to surgery].

Background: Percutaneous aortic valvuloplasty is an effective means of treatment for congenital aortic valve stenosis. The aim of this study was to evaluate the immediate results of valvuloplasty, to analyze the medium to long-term outcome and to assess its efficacy in preventing or postponing a new percutaneous valvuloplasty or aortic valve surgery.

Methods: We retrospectively analyzed the reports of 37 patients aged > 1 month (mean age 6.

Noncoronary aortic cusp rupture in an adult patient with ventricular septal defect: echocardiographic diagnosis.

Aortic regurgitation is a common complication of ventricular septal defects. The most common mechanism is right or noncoronary cusp prolapse. Other mechanisms are right or noncoronary cusp fibrosis resulting in thickening and restricted motion of the leaflets or infective endocarditis leading to cusp perforation.

Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients.

Aims: previous studies have established an association between exercise intolerance and increased morbidity and mortality in congenital heart disease patients. We aimed to clarify if exercise intolerance is associated with poor outcome in Fontan patients and to identify risk factors for mortality, transplantation, and cardiac-related hospitalization.

Methods And Results: a total of 321 Fontan patients (57% male, mean age 20.

Dilated cardiomyopathy presenting in childhood: aetiology, diagnostic approach, and clinical course.

Objective: To determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification.

Methods: Retrospective study of 41 consecutive children affected by dilated cardiomyopathy - aged 0-14 years; median 33.4 plus or minus 49.

Long-term coronary artery outcome after arterial switch operation for transposition of the great arteries.

Objective: To analyse the long-term patency of coronary arteries after neonatal arterial switch operation (ASO).

Methods: A retrospective study of the operative reports, follow-up and postoperative catheterisation data of 119 patients, who underwent the great arteries (TGA) repair since 1991, has been carried out.

Patient Population: Among the 133 survivors of the 137 ASOs performed between 1991 and 2007, 119 patients have been studied by routine control cardiac catheterisation and form the study population.

Are nasal decongestants safer than rhinitis? A case of oxymetazoline-induced syncope.

Derivatives of Imidazoline usually act to stimulate peripheral alpha2 receptors causing vasoconstriction. In young children, however, they can also stimulate alpha2receptors in the cardiovascular and central nervous systems, possibly causing cardiovascular, neurological, and respiratory depression. These medications do not require medical prescriptions, so often parents use them, bypassing paediatricians.

Determinants of exercise capacity after arterial switch operation for transposition of the great arteries.

Patients who undergo the arterial switch operation for transposition of the great arteries (TGA) are at risk of reduced exercise capacity, with most reports focusing on chronotropic incompetence as the cause. Residual right ventricular outflow tract (RVOT) obstruction is relatively common after the arterial switch operation, but its effect on exercise capacity is unknown. We studied 60 patients (44 males, age 13.

Ventilatory efficiency and aerobic capacity predict event-free survival in adults with atrial repair for complete transposition of the great arteries.

Objectives: The goal of this study was to assess the prognostic value of the cardiopulmonary exercise test (CPET) in patients who received a Mustard and Senning (M/S) operation.

Background: Patients who received an M/S operation have increased long-term risk of cardiovascular morbidity and mortality. Limited information is available on how to stratify risk in this population.

Correlations of phenotype and genotype in relation to morphologic remodelling of the aortic root in patients with Turner's syndrome.

Background: Patients with Turner's syndrome are at risk of aortic dilation and dissection. Currently, it is not known whether such dilation is related to associated cardiovascular abnormalities, or to the genetic anomaly itself.

Methods: We studied echocardiographically 107 patients with genetically proven Turner's syndrome, with heterogeneous underlying karyotypes, and without associated cardiac lesions.

Resolution of atrial thrombosis with heparin in a newborn with atrial flutter.

Unlabelled: Atrial thrombosis is a relatively rare event in children. We report a case of a newborn with AFI who after restoration of sinus rhythm, developed atrial thrombus on a prominent Chiari network floating between the right and left atrium through the patent foramen ovale. The thrombus was resolved following treatment with heparin without events.

Incompatibility between intravenous amiodarone and heparin in an infant.

Amiodarone is an effective antiarrhythmic agent and represents the drug of choice in the treatment of severe arrhythmias, especially in the setting of ventricular dysfunction. Amiodarone has the potential for interaction with many cardiac and non-cardiac drugs. Nonetheless few incompatibilities have been reported.