Idiopathic dilatation of the right atrium: a not so benign entity.

Idiopathic dilatation of the right atrium is an isolated enlargement of the right atrium in the absence of other cardiac lesions. This rare anomaly has a clinical spectrum ranging from asymptomatic to heart failure or even sudden death. It can be associated with atrial arrhythmias and thrombus formation.

Comprehensive Functional Echocardiographic Assessment of Transposition of the Great Arteries: From Fetus to Newborn.

To improve the understanding of the pathophysiology of transposition of the great arteries with intact ventricular septum (TGA/IVS) and the cardiac remodeling occurring from fetal to neonatal life, we performed a morphometric and functional echocardiographic assessment in fetuses and newborns. This was a prospective case-control study performed in a tertiary referral center, which included fetuses and newborns with a diagnosis of TGA/IVS between 2011 and 2018. Morphometry and systolic and diastolic function parameters were compared with age and body surface-matched healthy controls.

Isolated aortic dilation without osteoarthritis: a case of SMAD3 mutation.

Aneurysm-osteoarthritis syndrome is a recently discovered inherited autosomal dominant connective tissue disease caused by SMAD3 mutations. Aneurysm-osteoarthritis syndrome is responsible for 2% of familial thoracic aortic aneurysms and dissections and is characterised by aneurysms, dissections, and tortuosity throughout the arterial tree in combination with osteoarthritis. Early-onset osteoarthritis is present in almost all patients.

Fetal Aortic Valvuloplasty: Experience and Results of Two Tertiary Centers in Spain.

Objective: Fetal aortic valvuloplasty (FAV) may avoid progression of critical aortic stenosis (CAS) to hypoplastic left ventricle, improving the options for biventricular circulation (BVC). We describe the results of FAV in 2 referral centers in Spain.

Methods: We analyzed all FAVs performed in the period 2007-2015.

Propranolol as a treatment for multiple coronary artery micro-fistulas.

We present a case of congenital multiple coronary artery-left ventricle micro-fistulas, which were treated with propranolol disappearing within 6 months. She had a malformative syndrome associated with a chromosomal abnormality. The treatment for coronary artery fistula includes surgical ligation and transcatheter closure, but they are not indicated in congenital micro-fistulas.

Single coronary artery-right ventricular fistula.

We report a rare case of a male child aged 4 years and 5 months who was diagnosed with a coronary artery fistula and left single coronary artery. Pre-operative evaluation with echocardiography and selective angiography showed a dilated and tortuous single coronary artery draining into the right ventricular outflow tract. The coronary fistula was ligated.

[Surgical treatment of anomalous left pulmonary artery].

We report our experience with the surgical treatment of anomalous origin of the left pulmonary artery in eight children between 2004 and 2009. The congenital heart disease most frequently associated with this condition was patent ductus arteriosus. Surgery was carried out with extracorporeal circulation in five children, and without, in three.

Percutaneous closure of a patent arterial duct in a newborn.

We report a successful percutaneous neonatal closure of haemodynamically significant patent arterial duct. The neonate was suffering multiorgan failure, and had complete atrioventricular block. Closure of the patent arterial duct, either with surgery or inhibitors of cyclooxygenase, was contraindicated.