Impact of pregnancy on progression of preclinical autoimmune disorders: a prospective cohort study.

Objectives: The objective of this study was to evaluate the role of pregnancies in the progression from the preclinical phase of autoimmune disorder to a definite rheumatic disease.

Methods: A cohort study of women with symptoms and laboratory findings suggestive for autoimmune disorder were enrolled during the first trimester of pregnancy and followed-up for 5 years with clinical and laboratory assessment. Multinomial logistic regression was used to compute the risk of progression to definite autoimmune disease correcting for confounders.

Late outcome of extracardiac Fontan patients: 32 years of follow-up.

Objectives: We sought to evaluate the long-term survival and risk factors for morbidity and mortality in our cohort of patients after extracardiac (EC) Fontan.

Methods: We collected and analysed follow-up data until September 2020 for all patients who underwent EC Fontan operation at our institution from November 1988 to November 2015.

Results: Out of 406 patients treated with EC Fontan at a mean age of 5.

Health-related quality of life in Italian children and adolescents with congenital heart diseases.

Background: Congenital heart disease (CHD) is the most common congenital anomaly at birth, affecting approximately 1% of live births. In recent decades great medical and surgical advances have significantly increased life expectancy, shifting healthcare professionals' and researchers' interests in patients' Quality of Life (QoL). The main aims of our study were to evaluate generic and condition-specific QoL in a group of Italian children and adolescents with CHD and their parents and examine the level of agreement and directional disagreement between child/adolescent and parents reports on generic and condition-specific QoL.

Validity and reliability of the Italian version of the cardiac quality of life questionnaire for pediatric patients with heart disease (PedsQLTM).

Background: Congenital heart disease (CHD) accounts for nearly a third of all major congenital anomalies. Advances in pediatric cardiology shifted attention from mortality to morbidity and health-related quality of life (HRQOL) of patients with CHD and impact on their families. The purposes of this study were to assess the validity and reliability of the Italian version of the Pediatric Quality of Life (PedsQL) Cardiac Module and to create normative data for the Italian population.

Placental pathologic features in thyroid autoimmunity.

Introduction: Data on placental pathologic features associated with thyreoperoxidase antibodies (TPO Ab) and/or hypothyroidism are limited. The objective of the study was to analyze placental pathologic features of women with TPO Ab positivity.

Methods: Prospective case-control observational study of pregnancy outcome among women screened for TPO Ab positivity and/or isolated hypothyroidism (TSH>4mU/L) during the first trimester of pregnancy.

Applicability and Effectiveness of Laparoscopic Procedures in Pediatrics.

Three-dimensional (3D) laparoscopic surgery in pediatrics is still uncommon and few studies assessed in clinical practice advantages and disadvantages. Applicability and effectiveness of 3D versus two-dimensional (2D) laparoscopic procedures in congenital and acquired conditions in children are still unknown. We assessed applicability and effectiveness of 3D compared with 2D laparoscopic procedures in a pediatric setting.

Non-Alcoholic Fatty Liver Disease is Associated with Higher Metabolic Expenditure in Overweight and Obese Subjects: A Case-Control Study.

Non-alcoholic fatty liver disease (NAFLD) is a common condition in Western countries. However, their metabolic characteristics are poorly known even though they could be important. Therefore, the objective of this study was to measure resting metabolic parameters in overweight/obese adults with hepatic steatosis compared to controls, matched for age, sex, and obesity level.

Women in LOVe: Lacto-Ovo-Vegetarian Diet Rich in Omega-3 Improves Vasomotor Symptoms in Postmenopausal Women. An Exploratory Randomized Controlled Trial.

Objectives: In the postmenopausal period, most women suffer vasomotor symptoms (VMS). It is well-known that VMS can worsen the quality of life. Diet seems to play a relevant role in the development of VMS, but the effect of diet on VMS is mainly limited to observational studies, and analyses of nutritional supplements.

Impact of complex congenital heart disease on the prevalence of arterial hypertension after aortic coarctation repair.

Objectives: This study was designed to evaluate the difference in the prevalence of long-term arterial hypertension among patients with corrected aortic coarctation according to the existence of associated cardiac congenital lesions.

Methods: We identified 235 patients who had undergone surgery for aortic coarctation and classified them into 2 groups: patients with isolated coarctation of the aorta (CoA) and patients with aortic coarctation associated with complex congenital heart disease. Data were retrospectively analysed.

Long-term survival and phenotypic spectrum in heterotaxy syndrome: A 25-year follow-up experience.

Background: Heterotaxy syndrome (HS) is a group of congenital disorders characterized by abnormal arrangement of thoraco-abdominal organs across the left-right axis of the body, classified as right (RAI) and left atrial isomerism (LAI)·We investigated the long-term survival and phenotypic spectrum in our HS cohort. Results are compared to literature data.

Methods: This is a single centre, observational, both retro and prospective study.

Bradyarrhythmias in Repaired Atrioventricular Septal Defects: Single-Center Experience Based on 34 Years of Follow-Up of 522 Patients.

Atrioventricular Septal Defect (AVSD) is a rare congenital heart defect (CHD) often associated with genetic syndromes, most commonly Down syndrome (DS). Over the last four decades, surgical repair has increased survival and improved quality of life in these patients. The prevalence of bradyarrhythmias namely, atrioventricular block (AVB) and sinus node dysfunction (SND) in AVSD is partially known.

Long-Term Outcomes After Extracardiac Fontan Takedown to an Intermediate Palliative Circulation.

Background: Acute failure of the Fontan circulation is rare but remains associated with high morbidity and mortality rates. Little is known about the long-term outcome of patients who underwent Fontan takedown to an intermediate palliative circulation and their potential candidacy for redo Fontan completion.

Methods: Patients followed up at a single institution who underwent takedown of a Fontan circulation to an intermediate palliative circulation within 2 months of extracardiac Fontan completion were reviewed.

Role of right ventricular three-dimensional electroanatomic voltage mapping for arrhythmic risk stratification of patients with corrected tetralogy of Fallot or other congenital heart disease involving the right ventricular outflow tract.

Background: The post-surgical history of repaired congenital heart disease (rCHD), in particular tetralogy of Fallot (TOF), is often complicated by sudden death. Electrical myocardial abnormalities could be a substrate for malignant ventricular arrhythmias.

Methods And Results: 146 patients with TOF or other rCHD involving a subpulmonary right ventricle, considered to be at high arrhythmic risk, underwent right ventricular (RV) electroanatomic voltage mapping (EVM).

The Ross procedure in patients aged less than 18 years: the midterm results.

Objective: This study reviews a single-center experience with the Ross procedure in infants and young children.

Methods: From November 1993 to March 2012, 55 children aged less than 17 years underwent a Ross procedure. The patients ranged in age from 2 days to 17 years (median, 5.

No thyroid abnormalities in patients submitted to cardiac catheterization in the first eighteen months of life.

Background: No data are available about the risk of thyroid disturbance after exposure to low-dose radiation due to the use of cardiac catheterization in the first years of life.

Aim: To determine the risk of functional and morphological thyroid abnormalities in a homogeneous cohort of patients who underwent diagnostic low-dose radiation for heart catheterization during the first 18 months of life.

Subjects And Methods: Fifty-five patients, submitted to cardiac catheterization during the first 18 months of life, underwent evaluation of the thyroid function and structure after a median period of 13 yr since the first radiation exposure.

Mid-term results, and therapeutic management, for patients suffering hypertension after surgical repair of aortic coarctation.

We designed our study to investigate the efficacy of a new therapeutic approach to late onset hypertension in patients after surgical repair of aortic coarctation. Several studies have shown a higher incidence of hypertension during daily activities, and during exercise, in patients after surgical correction of coarctation. To the best of our knowledge, however, no data exists concerning haemodynamics, the response of arterial pressures, and the effects of medications for lowering blood pressure during exercise or during daily activities.

Clinical outcome of 193 extracardiac Fontan patients: the first 15 years.

Objectives: We sought to evaluate the mid-term outcome of hospital survivors with extracardiac Fontan circulation.

Background: Few data exist about the mid-term and long-term results of the extracardiac Fontan operation.

Methods: From November 1988 to November 2003, 221 patients underwent an extracardiac Fontan procedure as primary (9 patients) or secondary (212 patients) palliation, at a mean age of 72.

The influence of different surgical procedures on hypertension after repair of coarctation.

We measured resting and exercise haemodynamics, as well as 24-hour ambulatory blood pressure, so as to study the influence on development of hypertension in children after repair of coarctation by either construction of a subclavian flap or end-to-end anastamosis. The patients in both groups were studied a mean time of 13 years after surgery. Thus, we divided 43 children who had undergone surgical repair of coarctation, and who were not on antihypertensive therapy, into a group of 22 patients who had undergone subclavian flap repair, with a mean age of 14 plus or minus 2.

Use of DDDRP pacing device in prevention and treatment of tachy-brady syndrome after Mustard procedure.

A 13-year-old male patient, who underwent Mustard operation for a very complex congenital heart disease (CHD), after palliation presented a decrease of the sinus node function, developing a tachy-brady syndrome and a mild dysfunction of atrioventricular (AV) conduction. He was successfully treated using a DDDRP pacemaker, which ensured a suitable atrial rhythm and was able to interrupt supraventricular tachycardia episodes. Until now, hospitalization related to episodes of heart failure or symptomatic arrhythmia, has not been necessary.

Exercise blood pressure response, cardiac output and 24-hour ambulatory blood pressure monitoring in children after aortic coarctation repair.

Background: The aim of this study was to assess blood pressure at rest, the response to exercise and the 24-hour ambulatory blood pressure monitoring (ABPM) profile in children operated for aortic coarctation.

Methods: Twenty children were operated upon for aortic coarctation. The patients' data were compared with those obtained from 19 healthy controls of the same age.

The beneficial vortex and best spatial arrangement in total extracardiac cavopulmonary connection.

Objective: Total extracardiac cavopulmonary connection is an established procedure, but the best spatial arrangement remains controversial. On the basis of our clinical experience with total extracardiac cavopulmonary connection, we performed quantitative and qualitative flow analysis on total extracardiac cavopulmonary connection models simulating the two most frequent arrangements applied to our patients to determine the most favorable hydrodynamic pattern.

Methods: We selected two main groups among 110 patients who underwent total extracardiac cavopulmonary connection, those with left-sided inferior vena cava anastomosis (type 1) and those with facing superior and inferior vena cava anastomoses (type 2).

Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions.

Objectives: To evaluate late outcome of non-isomeric total anomalous pulmonary venous connection (TAPVC) repair, controlling for anatomic subtypes and surgical technique.

Methods: Between 1983 and 2001, 89 patients (median age 54 days) underwent repair for supracardiac (38), cardiac (26), infracardiac (16) or mixed (nine) TAPVC. Ten patients (11.