Successful treatment of pulmonary arterial hypertension in a 2-month-old female infant with incontinentia pigmenti: A case report.

Incontinentia pigmenti (IP) is a rare X-linked dominant neuroectodermal dysplasia affecting almost exclusively females. It is caused by loss-of-function mutations in the inhibitor of kappa light polypeptide gene enhancer in B cells, kinase gamma gene, formerly known as NF-κB essential modulator. The disorder is typically identified by peculiar skin findings that develop throughout the 1 year of life.

A Double Lane for a Better Circulation: Percutaneous Duplication of the Thoracic Aorta in a Child.

Aortic arch interruption or atresia consists in a complete loss of anatomical and luminal continuity between the ascending and descending thoracic aorta. It is usually diagnosed in newborn babies but may also be present in young children, adolescents, adults and elderly patients. In these cases, it can be congenital or more frequently acquired.

Transfemoral Valve-In-Valve in a Degenerated Small Aortic Bioprosthesis: Five-Year Follow Up.

A small-sized bioprosthesis can limit transcatheter valve-in-valve (V-in-V) implantations. The case is reported of a 91-year-old woman who had successfully undergone a V-in-V procedure with a 26 mm CoreValve in a previously implanted Mitroflow 19 mm valve. At the five-year follow up the prosthesis showed good echocardiographic function and the patient was alive and free from major symptoms.

Long-term outcome after percutaneous closure of persistent left superior caval vein draining into the left atrium: a contrast-enhanced CT study.

Background: Data regarding long-term outcome after percutaneous closure of left superior caval vein draining into the left atrium are lacking. The aim of the present study was to report the long-term follow-up by using contrast-enhanced CT.

Methods: In all, three patients underwent percutaneous closure of left superior caval vein draining into the left atrium between 2005 and 2015.

[Preliminary results and future perspectives of the Piedmont Adult Congenital Heart Disease Registry].

Background: Adults with congenital heart disease are a relatively new population that progressively increases in size and complexity. In Italy, there are no accurate data concerning the distribution of congenital defects and the long-term outcome relating to both congenital heart disease per se and comorbidities, due to the aging process.

Methods: The Piedmont Adult Congenital Heart Disease Registry has been designed to investigate these aspects and to support a high quality healthcare development for grown-up congenital heart patients.

Fontan circulation causes early, severe liver damage. Should we offer patients a tailored strategy?

Background: In patients with Fontan circulation, the liver is profoundly affected by chronic venous stasis. Little is known about early hepatic changes in this population.

Methods: We performed echocardiography, abdominal ultrasound, liver elastography, cardiac catheterization, esophago-gastro-duodenoscopy and calculated MELD-XI score in 64 Fontan patients (69% minors), at an interval of 1-15years since Fontan.

Off-label use of the amplatzer ductal occluder II additional size for percutaneous closure of congenital and acquired coronary fistulae.

We report two cases in which congenital and acquired fistulae (CAFs) have been successfully closed by using the Amplatzer Ductal Occluder (ADO) II AS in children. Because of the flexibility, small profile and short length of ADO II AS, this device proved to be an excellent option in case of tortuous and short CAFs with coronary branches next to the fistulous orifice. © 2015 Wiley Periodicals, Inc.

Iatrogenic "aortopulmonary window": percutaneous rescue closure as a bridge to surgical repair.

We present the case of a 15-year-old boy who underwent arterial switch operation due to transposition of the great arteries with severe scoliosis, obstruction of the right coronary ostium, and severe stenosis of the pulmonary trunk. Balloon angioplasty caused a large aortopulmonary shunt provoking myocardial ischaemia and pulmonary hypertension. The traumatic "aortopulmonary window" was percutaneously occluded using an Amplatzer Septal Occluder device as a bridge to surgical repair.

Percutaneous closure of atrial septal defect in adults: very long-term clinical outcome and effects on aortic and mitral valve function.

Aim: To investigate the very long-term clinical outcomes of atrial septal defect (ASD) percutaneous closure in adult patients and to evaluate the 12-month effects of the device on aortic and mitral valve function.

Methods: Over a 12-year period, a total of 110 consecutive patients underwent percutaneous ASD closure. A yearly clinical follow-up was conducted and any adverse event was recorded.

Impact of diabetes mellitus on early and midterm outcomes after transcatheter aortic valve implantation (from a multicenter registry).

Several clinical and procedural factors have been identified as predictors of early and midterm events after transcatheter aortic valve implantation (TAVI), but incidence and prognostic impact of diabetes mellitus (DM), especially insulin treated, on short- and midterm outcomes remain to be defined. All consecutive patients who underwent TAVI at our institutions were enrolled and stratified according to DM status. All-cause mortality at 30 days or in hospital and at follow-up was the primary end point, whereas periprocedural complications, rates of myocardial infarction, stroke, and reintervention at follow-up were the secondary ones.

Association among oral health, apical periodontitis, CD14 polymorphisms, and coronary heart disease in middle-aged adults.

Introduction: There is evidence to suggest that an association exists between oral infections and coronary heart disease (CHD). Subjects presenting lesions of endodontic origin (LEOs) or pulpal inflammation had an increased risk of developing CHD. However, findings concerning systemic manifestations of apical periodontitis (AP) remain controversial.

Altered nitric oxide/cGMP platelet signaling pathway in platelets from patients with acute coronary syndromes.

This study was aimed at evaluating whether the nitric oxide (NO)/cyclic GMP (cGMP) signaling pathway is altered in platelets from patients with an acute coronary syndrome (unstable angina and acute myocardial infarction). We investigated 10 patients with unstable angina (UA), 14 with acute myocardial infarction (AMI) and 14 age and sex-matched healthy subjects. The serum markers of platelet activation (sP-selectin), inflammation (TNF-alpha and erythrocyte sedimentation rate), thrombotic state (fibrinogen) and plaque disruption were significantly higher in both UA and AMI patients compared to the healthy controls.