Striving for physiologic coaptation: An experimental and innovative approach towards designing and implanting aortic neo-leaflets.

Aortic valve repair has emerged as the treatment of choice for congenital aortic valvular disease, avoiding the need for a reoperation associated with stented prosthesis overgrowth. The introduction of a leaflet implant represents a recent advancement in a field that originated early techniques, such as simple commissurotomies. In our experimental approach, we assessed two established leaflet-sizing techniques by analysing their resultant coaptation areas.

Importance of Acute Anterior Angulation in Double Aortic Arch Needing Attention at Primary Surgery.

Background: Vascular rings are rare congenital abnormalities of the aortic arch. There are many embryological variants including a double aortic arch. In symptomatic children, division of ring and release of airway structures may be sufficient.

Diazoxide-induced pulmonary hypertension in hyperinsulinaemic hypoglycaemia: Recommendations from a multicentre study in the United Kingdom.

Objective: Diazoxide is first-line treatment for hyperinsulinaemic hypoglycaemia (HH) but diazoxide-induced pulmonary hypertension (PH) can occur. We aim to characterize the incidence and risk factors of diazoxide-induced PH in a large HH cohort to provide recommendations for anticipating and preventing PH in diazoxide-treated patients with HH.

Design And Patients: Retrospective cohort study involving four UK regional HH centres; review of case notes of HH patients on diazoxide.

Addison's disease presenting with perimyocarditis.

Background: Polyglandular autoimmune syndrome (PGA) and eosinophilic granulomatosis with polyangiitis (EGPA) do not seem to represent a coincidental association.

Case Presentation: A case of a 15-year-old boy is reported who presented with severe systemic inflammation, perimyocarditis and cardiogenic shock, in whom EGPA was initially suspected and later diagnosed with autoimmune adrenalitis with PGA.

Conclusions: The severity of the systemic inflammation and perimyocarditis suggests a more widespread autoimmune-mediated process.

Infective endocarditis in children: an update.

Purpose Of Review: Infective endocarditis in children remains a clinical challenge. Here, we review the impact of the updated 2015 American Heart Association and European Society of Cardiology guidelines on management as well as the significance of the new predisposing factors, diagnostic and treatment options, and the impact of the 2007-2008 change in prophylaxis recommendations.

Recent Findings: The new 2015 infective endocarditis guidelines introduced the endocarditis team, added the new imaging modalities of computer tomography and PET-computer tomography into the diagnostic criteria and endorsed the concept of safety of relatively early surgical treatment.

Granulomatosis with polyangiitis mimicking infective endocarditis in an adolescent male.

Granulomatosis with polyangiitis (GPA) is a rare but serious small vessel vasculitis with heterogeneous clinical presentation ranging from mainly localised disease with a chronic course, to a florid, acute small vessel vasculitic form characterised by severe pulmonary haemorrhage and/or rapidly progressive vasculitis or other severe systemic vasculitic manifestations. Cardiac involvement is, however, uncommon in the paediatric population. We report a case of a 16-year-old male who presented with peripheral gangrene and vegetation with unusual location on the supporting apparatus of the tricuspid valve, initially considered to have infective endocarditis but ultimately diagnosed with GPA.

Echocardiographic arterial measurements in complex congenital diseases before bidirectional Glenn: comparison with cardiovascular magnetic resonance imaging.

Aims: This study sought to investigate diagnostic accuracy of echocardiographic measures of great vessels in patients before bidirectional cavopulmonary connection (BCPC) compared with cardiovascular magnetic resonance (CMR).

Methods And Results: Seventy-two patients (61% after Norwood operation) undergoing BCPC between 2007 and 2012 were assessed pre-operatively using echocardiography and CMR. Bland-Altman analysis and correlation coefficients were used for comparison of echocardiography and CMR measurements.

Cor triatriatum dexter and coarctation of the aorta--a rare association in a 7-year-old child with type 1 neurofibromatosis.

Cor triatriatum dexter is an extremely rare congenital anomaly of the right atrium. It occurs because of the persistence of the right sinus venosus valve, resulting in partitioning of the right atrium. Most of the described cases of cor triatriatum dexter in the literature were incidental findings on echocardiogram or at necropsy.

Thoracoscopic pericardiectomy for constrictive pericarditis in a pediatric patient with mulibrey nanism.

Mulibrey nanism is a rare autosomal recessive disease with multisystem involvement. Clinical deterioration is most often related to cardiac involvement in the form of restrictive or constrictive disorders, and pericardiectomy may be required. We report a case of Mulibrey nanism in a patient of non-Finnish origin, where a thoracoscopic pericardiectomy helped in good palliation and clinical recovery.

Left ventricular and mitral valve function long after repair of left anomalous coronary artery from the pulmonary artery: recovery years after severe ischemia.

Background: The study evaluates the long-term results of surgery for anomalous left coronary artery from the pulmonary artery (ALCAPA) with special attention on the left ventricular (LV) function and mitral regurgitation.

Methods: Twenty-one children underwent surgery for ALCAPA over 23 years (1987-2010). All patients underwent establishment of a two-coronary system, by direct reimplantation (n = 13) or by intrapulmonary tunnel technique (n = 8), with concomitant mitral valve repair in one.

Aberrant subclavian artery origin in tetralogy of Fallot with pulmonary stenosis is associated with chromosomal or genetic abnormality.

We determined the relationship between aortic arch anatomy in tetralogy of Fallot with pulmonary stenosis and chromosomal or genetic abnormality, by performing analysis of 257 consecutive patients undergoing surgical repair from January, 2003 to March, 2011. Chromosomal or genetic abnormality was identified in 49 of the 257 (19%) patients. These included trisomy 21 (n = 14); chromosome 22q11.