Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome.

Objectives: Absent pulmonary valve syndrome is a rare congenital heart disease with severe airway compression due to dilatation of the pulmonary arteries (PAs). We investigated risk factors for death and prolonged mechanical ventilation (>7 days) and a threshold PA size for these outcomes.

Methods: This retrospective 2-centre cohort study included 68 patients with complete repair between January 1996 and December 2015.

Percutaneous pulmonary Melody valve implantation in small conduits.

Background: The Melody pulmonary valve has received approval for the treatment of dysfunctional right ventricular (RV) outflow tract conduits ≥16mm.

Aims: To investigate technical and clinical outcomes in patients who underwent percutaneous pulmonary valve implantation (PPVI) in conduits ≤16mm.

Methods: Eleven patients were enrolled retrospectively between 2000 and 2015 in a cardiac referral centre.

Discordances Between Pre-Natal and Post-Natal Diagnoses of Congenital Heart Diseases and Impact on Care Strategies.

Background: Pre-natal diagnosis of congenital heart disease (CHD) allows anticipation of urgent neonatal treatment and provides adequate information to the parents on cardiac outcomes.

Objectives: This study sought to analyze the discordances between expert fetal cardiac diagnosis and final diagnosis of CHD and their impact on neonatal and long-term care strategies.

Methods: We included 1,258 neonates with a pre-natally diagnosed CHD and 189 fetopsies following termination of pregnancy at our tertiary center over a 10-year period.

Neonatal management and outcomes of prenatally diagnosed CHDs.

Objectives: The aim of this study was to determine the probability of intervention at birth after prenatal diagnosis of CHD.

Methods: A 10-year retrospective study including all foetuses with a prenatally diagnosed CHD and those delivered in a tertiary-care cardiac centre between January, 2002 and December, 2011 was carried out. Patients were classified into eight groups according to the anticipated risk of neonatal intervention.

Associated genetic syndromes and extracardiac malformations strongly influence outcomes of fetuses with congenital heart diseases.

Background: Congenital heart disease (CHD) is often associated with extracardiac malformations (ECMs) and genetic syndromes.

Aims: To determine the effect of cytogenetic anomalies and/or ECMs associated with CHD on parental decision to choose termination of pregnancy (TOP) or compassionate care (CC), as well as on the outcome of children born alive.

Methods: This 10-year retrospective study included all prenatally diagnosed cases of CHD in a single tertiary referral centre.