Stent Implantation Across the Atrial Flow Regulator Device.

The Atrial Flow Regulator (AFR) is a self-expandable double-disc device with a central fenestration, intended to maintain a calibrated communication across the interatrial septum. We reported for the first time a stent implantation across an AFR device in an adolescent born with complex congenital heart disease with duct-dependent systemic circulation and severe combined pulmonary hypertension.

Transcatheter interventions in patients with a Fontan circulation: Current practice and future developments.

The Fontan operation represents the last of multiple steps that are offered a wide range of congenital cardiac lesions with a single ventricle (SV) physiology. Nowadays this surgical program consists of a total cavopulmonary connection (TCPC), by anastomosing systemic veins to the pulmonary arteries (PAs), excluding the right-sided circulation from the heart. As a result of imaging, surgical, percutaneous, and critical care improvements, survival in this population has steadily increased.

Congenital Heart Disease: Growth Evaluation and Sport Activity in a Paediatric Population.

(1) Objective: To evaluate: (i) the associations of age and disease severity with anthropometric indices and weight status, (ii) the difference in the frequency of sports activity among different levels of disease severity in paediatric patients with congenital heart disease (CHD). (2) Methods: Clinical data of Caucasian children (aged 2-18 years) diagnosed with CHD (2005-2018) were retrospectively collected from the electronic register of outpatient visits. Of the 475 children with CHD, 368 children and their 1690 complete anthropometric measurements were eligible for inclusion in our analysis.

Endomyocardial Biopsy in Pediatric Myocarditis and Dilated Cardiomyopathy: A Tool in Search for a Role.

Endomyocardial biopsy (EMB) is a well-known diagnostic tool for the investigation and treatment of myocardial diseases and remains the gold standard for the diagnosis of myocarditis. Due to its invasiveness, with a complication rate ranging from 1 to 15%, its role in the diagnostic work-up of pediatric heart failure is not well established. The aim of this review is to define the role of EMB as diagnostic technique in the work up of children presenting with severe left ventricular dysfunction with the support of our center experience.

Impact of hard lockdown on interventional cardiology procedures in congenital heart disease: a survey on behalf of the Italian Society of Congenital Heart Disease.

The Coronavirus disease 2019 (COVID-19) pandemic has thoroughly and deeply affected the provision of healthcare services worldwide. In order to limit the in-hospital infections and to redistribute the healthcare professionals, cardiac percutaneous intervention in Pediatric and Adult Congenital Heart Disease (ACHD) patients were limited to urgent or emergency ones. The aim of this article is to describe the impact of the COVID-19 pandemic on Pediatric and ACHD cath laboratory activity during the so-called 'hard lockdown' in Italy.

Bicuspid Aortic Valve Disease from Infancy to Older Age: A 25-Year Experience from an Italian Referral Center.

Aim: Bicuspid aortic valve (BAV) is the most common congenital heart defect, with considerable risk of morbidity and mortality. The purpose of the study was to analyze clinical and echocardiographic presentation of BAV in a large-volume tertiary Italian center and to test their interaction with full age span, sex, and first diagnosis versus second referral.

Methods: Consecutive patients of all ages diagnosed with BAV at our center from January 1988 to December 2012 were retrospectively included.

Transcatheter Valve-in-Mitral Homograft in Tricuspid Position: First-in-Human Report.

A young woman with concomitant symptomatic severe stenosis and regurgitation was managed by percutaneous valve implantation because of a failing mitral homograft in the tricuspid position. The implant procedure was performed using an Edwards S3 23-mm valve. Good hemodynamic results were achieved, and, at intermediate follow-up, she did not require hospitalization and was free of symptoms.

Acute Biventricular Interaction in Pediatric Patients Implanted with Continuous Flow and Pulsatile Flow LVAD: A Simulation Study.

Left ventricular assist devices (LVADs) are used to bridge pediatric patients till transplantation. However, the LVADs effects on right ventricular (RV) function are controversial. This work aims at studying the ventricular interdependency in the presence of continuous (c-) and pulsatile (p-) flow LVAD in pediatric patients using a lumped parameter model including the representation of the septum.

The Impact of Specific Viruses on Clinical Outcome in Children Presenting with Acute Heart Failure.

The presence and type of viral genomes have been suggested as the main etiology for inflammatory dilated cardiomyopathy. Information on the clinical implication of this finding in a large population of children is lacking. We evaluated the prevalence, type, and clinical impact of specific viral genomes in endomyocardial biopsies (EMB) collected between 2001 and 2013 among 63 children admitted to our hospital for acute heart failure (median age 2.

The use of a numerical model to simulate the cavo-pulmonary assistance in Fontan circulation: a preliminary verification.

The lack of an established experience on the use of VAD for the cavo-pulmonary assistance leads to the need of dedicated VADs development and animal experiments. A dedicated numerical model could support clinical and experimental strategies design and new VADs testing. The aim of this work is to perform a preliminary verification of a lumped parameter model of the cardiovascular system to simulate Fontan physiology and the effect of cavo-pulmonary assistance.

Simulation of Ventricular, Cavo-Pulmonary, and Biventricular Ventricular Assist Devices in Failing Fontan.

Considering the lack of donors, ventricular assist devices (VADs) could be an alternative to heart transplantation for failing Fontan patients, in spite of the lack of experience and the complex anatomy and physiopathology of these patients. Considering the high number of variables that play an important role such as type of Fontan failure, type of VAD connection, and setting (right VAD [RVAD], left VAD [LVAD], or biventricular VAD [BIVAD]), a numerical model could be useful to support clinical decisions. The aim of this article is to develop and test a lumped parameter model of the cardiovascular system simulating and comparing the VAD effects on failing Fontan.

Natural history and clinical outcome of "uncorrected" scimitar syndrome patients: a multicenter study of the italian society of pediatric cardiology.

Introduction And Objectives: To analyze the clinical status of patients with "uncorrected" scimitar syndrome in a multicenter Italian study.

Methods: The natural history of scimitar syndrome was analyzed in 44 affected individuals (from 9 Italian centers).

Results: The median age at diagnosis was 1.

Coronary plaque composition assessed by intravascular ultrasound virtual histology: association with long-term clinical outcomes after heart transplantation in young adult recipients.

Objectives: To assess coronary plaque composition by virtual histology intravascular ultrasound (VH-IVUS) analysis in young adult recipients and to correlate these findings with time from heart transplant (HTx) and long-term outcomes.

Background: Rapid progression of coronary allograft vasculopathy after heart transplantation is a powerful predictor of mortality and clinical events at long-term.

Methods: Forty consecutive young adult recipients transplanted during childhood undergoing VH-IVUS during coronary surveillance have been prospectively included in this study.

Percutaneous treatment of abdominal coarctation in children using a covered stent.

Coarctation of the abdominal aorta is extremely rare. It generally involves a long segment of the descending aorta and causes uncontrolled and unexplainable hypertension in children. The therapeutic choice is very challenging because acute and chronic complications are reported for both the surgical and the percutaneous approaches.

Impact of donor quality on outcome of heart transplantation.

Objective: Over the last few years, there have been changes in both donor and recipient profiles in heart transplantation. Encouraging clinical outcome of marginal donors in candidates older than 60 years of age led us to allocate suboptimal donors for younger recipients as well. We reviewed our experience retrospectively so as to assess the impact of donor quality on heart transplantation.

The Ross-Yacoub procedure for aneurysmal autograft roots: a strategy to preserve autologous pulmonary valves.

Objectives: Autograft dilatation is leading to an increase in root reoperations late after the Ross procedure. A 14-year clinical experience was reviewed to define the feasibility and outcome of the autograft valve-sparing root reoperation.

Methods: One hundred twenty-six patients surviving an average of 7.

Arterial tortuosity syndrome in two Italian paediatric patients.

Background: Arterial tortuosity syndrome (ATS) (OMIM #208050) is a rare autosomal recessive connective tissue disorder characterized by tortuosity and elongation of the large and medium-sized arteries, propensity to aneurysms formation, vascular dissection, and pulmonary arteries stenosis. ATS is caused by mutations in SLC2A10 gene, encoding for the facilitative glucose transporter 10 (GLUT10). So far, 17 SLC2A10 mutations have been reported in 32 families, two of which were Italian with a total of five patients.