Fate of Hypoplastic Pulmonary Arteries After Arterial Duct Stenting in Congenital Heart Disease With Duct-Dependent Pulmonary Circulation.

Objectives: This study sought to evaluate the impact of arterial duct (AD) stenting in promoting catch-up growth of hypoplastic pulmonary artery (PA) tree in congenital heart disease with duct-dependent pulmonary circulation (CHD-DPC).

Background: Significant and balanced PA growth following AD stenting has already been consistently reported in the literature. However, no data are so far available about the role of this approach in severe PA hypoplasia, which significantly impacts the risk of surgical repair.

Ten-years, single-center experience with arterial duct stenting in duct-dependent pulmonary circulation: early results, learning-curve changes, and mid-term outcome.

Objectives: To evaluate early results, learning-curve changes, and mid-term outcome of arterial duct (AD) stenting in congenital heart disease with duct-dependent pulmonary circulation (CHD-DPC) in a high-volume, tertiary referral center.

Background: In spite of wide acceptance as cost-effective alternative to surgical palliation, AD stenting indications, results and mid-term outcome still largely depend on small series, experiences in particular subsets of patients or multicenter series with different approaches and interventional philosophy.

Methods: Between April 2003 and December 2013, 119 patients underwent AD stenting as lower-risk palliation of CHD-DPC at our Institution.

Two decades of experience with the Ross operation in neonates, infants and children from the Italian Paediatric Ross Registry.

Objective: Children undergoing Ross operation were expected to have longer autograft, but shorter homograft durability compared with adults. In order to define the outcome in the second decade after Ross operation in children, a nationwide review of 23 years of experience was undertaken.

Methods: 305 children underwent Ross operation in 11 paediatric units between 1990 and 2012.

Aortic erosion during negative pressure therapy in a pediatric heart transplant recipient.

A happy-ending "series of unfortunate events" is reported of the successful emergency treatment of an erosion of the ascending aorta during negative pressure sternal wound therapy for a relapse of Berlin Heart driveline infection in a pediatric transplant recipient. Several key issues related to assist device-related infections and negative pressure complications are discussed in this peculiar setting.

Heterogeneity of ventricular repolarization in newborns with severe aortic coarctation.

Sudden death is a possible occurrence for newborns younger than 1 year with severe aortic coarctation (CoA) before surgical correction. Basic research and animal experiments have shown electrophysiologic changes during mechanical ventricular pressure overload. The current study aimed to evaluate the effect of severe CoA on the heterogeneity of ventricular repolarization by examining corrected QT and JT interval dispersion (respectively, QTc-D and JTc-D) and electrocardiographic parameters of spatial heterogeneity of ventricular repolarization in newborns with no associated congenital cardiac malformations.

Search of somatic GATA4 and NKX2.5 gene mutations in sporadic septal heart defects.

High prevalence of somatic mutations in the cardiac transcription factor genes NKX2.5 and GATA4 have been reported in the affected cardiovascular tissue of patients with isolated cardiac septal defects, suggesting a role of somatic mutations in the pathogenesis of these congenital heart defects (CHDs). However, all somatic mutations have been identified in DNA extracted from an archive of formalin-fixed cardiac tissues.

Pulmonary artery growth after palliation of congenital heart disease with duct-dependent pulmonary circulation: arterial duct stenting versus surgical shunt.

Objectives: The aim of this study was to compare the pulmonary artery (PA) growth after arterial duct (AD) stenting versus modified Blalock-Taussig shunt (MBTS) in neonates with congenital heart disease with duct-dependent pulmonary circulation (CHD-DPC).

Background: Arterial duct stenting is increasingly deemed a reliable alternative to surgical shunt in CHD-DPC. A stented duct might better adapt to the PA anatomy than a surgical conduit, thereby promoting a more uniform PA development.

Late percutaneous re-canalization of arterial duct-dependent isolated pulmonary artery.

A critical infant with unrestrictive ventricular septal defect and absence of the left pulmonary artery underwent transcatheter re-canalization of the occluded arterial duct-left pulmonary artery complex and surgical banding of the right pulmonary artery. After this hybrid approach, the baby was discharged under mild anticongestive therapy in view of a later surgical repair.

Hybrid transcatheter--surgical approach in complex pulmonary artery stenosis due to arterial tortuosity syndrome.

Arterial tortuosity syndrome is a connective tissue disorder characterized by elongation and tortuosity of large elastic arteries, potentially resulting in multiple vascular stenoses. This paper reports a novel hybrid strategy to treat 'complex' bilateral peripheral pulmonary artery stenoses consisting of transcatheter stent implantation via midline sternotomy and reductive angioplasty of the proximal pulmonary arteries.

Hybrid palliation in complex congenital heart malformation with duct-dependent isolated pulmonary artery.

A 2 month-old infant with severe congestive heart failure due to unrestrictive ventricular septal defect and absence of the left pulmonary artery was submitted to a hybrid transcatheter-surgical palliation consisting in percutaneous re-canalization of the occluded arterial duct-left pulmonary artery complex and surgical banding of the right pulmonary artery. The post-operative course was uneventful and the baby was discharged in a few days under mild anti-congestive therapy. This hybrid approach was highly effective in stabilizing this critical infant in view of a later and safer surgical repair.

Hybrid transcatheter-surgical strategy in arterial tortuosity syndrome.

Arterial tortuosity syndrome is a rare connective tissue disorder characterized by elongation and tortuosity of large elastic arteries resulting in multiple vascular stenoses. This article reports on a novel hybrid transcatheter-surgical strategy to treat bilateral peripheral pulmonary artery stenoses due to this syndrome. Operation consisted of stent implantation by midline sternotomy and reductive reconstruction of the proximal pulmonary arteries.

Prosthesis-patient mismatch in the elderly: survival, ventricular mass regression, and quality of life.

Background: Evaluation of the impact of prosthesis-patient mismatch (PPM) on long-term outcome and quality of life (QOL) in elderly patients who underwent implantation of small size bileaflet prostheses for aortic stenosis.

Methods: Between September 1988 and September 2006, 377 patients aged greater than 70 years underwent aortic valve replacement with a small size bileaflet prosthesis (17, 19, and 21 mm) in one Institution. The study population's survivors (345 patients) were divided into three groups according to the indexed effective orifice area (EOAI): Group A included patients with EOAI less than 0.

Bilateral arterial duct 'stenting' in a low-weight neonate with complex congenital heart defect.

Bilateral arterial ducts feeding discontinuous pulmonary arteries are a very rare anatomic arrangement in complex heart malformations with pulmonary atresia. In this setting, neonatal ductal closure may result in abrupt pulmonary hypoperfusion and life-threatening systemic hypoxia. In high-risk patients, percutaneous arterial duct stenting might be an engaging and cost-effective alternative to surgery.

Changing spectrum and outcome of 705 fetal congenital heart disease cases: 12 years, experience in a third-level center.

Introduction: Congenital heart diseases are the most common prenatal and postnatal malformations. Nowadays, fetal echocardiography is a widely practiced technique; however, the impact of prenatal diagnosis on prognosis of the newborns affected by congenital heart disease remains uncertain.

Objective: To assess the outcome and the changes in the spectrum of prenatally detected congenital heart disease in our tertiary care centre in 12 years of activity (1995-2006).

Aortic coarctation with persistent fifth left aortic arch.

A neonate with severe aortic coarctation showed a double lumen transverse aorta (persistent fifth aortic arch) with both channels joining at the isthmus where the obstruction was confirmed by echocardiography and cardiac catheterization. Surgical repair was performed with a pantaloon-shaped patch. Persistent fifth aortic arch does not result in a vascular ring and, per se, is not hemodynamically significant unless associated with other cardiac malformations.

Hybrid approach in a case of arterial tortuosity syndrome.

Arterial tortuosity syndrome is a rare connective tissue disorder characterised by elongation, tortuosity, stenosis and aneurysms of the large and middle-sized arteries. The symptomatology is correlated to the artery affected by the pathology with correlated stenosis. We describe our hybrid surgical procedure in the treatment of a case of kinking of the pulmonary branches with significant gradient and hypertension.

Tissue versus mechanical prostheses: quality of life in octogenarians.

Background: The aim of this study was to determine whether changes in prognosis and quality of life (QOL) after aortic valve replacement (AVR) in octogenarians differ depending on the choice of mechanical (MP) or tissue (BP) valves.

Methods: Between July 1992 and September 2006, 160 consecutive octogenarians underwent AVR with (18.8%) or without concomitant coronary artery bypass grafting.

Transcatheter ductal stenting in critical neonatal Ebstein's anomaly.

Background: Symptomatic neonatal Ebstein's anomaly shows a very poor outcome, most frequently due to duct-dependent pulmonary circulation caused by functional pulmonary valve atresia. In this setting, percutaneous ductal stenting using high-flexibility coronary stents might be a highly cost-effective alternative to systemic-to-pulmonary shunt.

Methods: Three newborns (age 4.

Congenital heart disease in live-born children: incidence, distribution, and yearly changes in the Campania Region.

Objectives: Congenital heart disease (CHD), the most common cardiac malformation in the fetal and neonatal period, represents a heterogeneous group of defects with little known cause. The aim of this study was to investigate the incidence rate (IR) of CHD without spontaneous resolution in the first year of life, the age at diagnosis, the most common type of CHD, and the IR changes in severe and nonsevere CHD during the study period.

Methods: From January 1997 to December 2002, 4559 live births with CHD (diagnosed until December 2004) were enrolled in the Campania Region.

Stenting of bilateral arterial ducts in complex congenital heart disease.

Bilateral arterial ducts serving nonconfluent pulmonary arteries is a very rare pattern of pulmonary blood flow in congenital heart disease with pulmonary atresia. In this setting, neonatal ductal closure might result in abrupt pulmonary hypoperfusion and life-threatening systemic hypoxia, thereby indicating emergent surgical palliation or repair. However, percutaneous arterial duct stenting might be an alternative to surgery, especially in high-risk patients.