Advances in pediatric pulmonary arterial hypertension.

Purpose Of Review: Pulmonary arterial hypertension (PAH) is an important cause of morbidity and mortality in children. Approved medications for the treatment of adult PAH have been used to treat children, but evidence-based treatment algorithms for children are lacking.

Recent Findings: Pediatric PAH registries have begun to define the incidence and prevalence of idiopathic PAH and PAH associated with congenital heart disease.

Pediatric cardiac retransplant: differing patterns of primary graft failure by age at first transplant.

Objective: This study compared graft failure leading to retransplant in infants versus older children at initial heart transplant.

Methods: Twenty-six retransplant recipients were compared by age at first transplant: infant group (<1 year) and pediatric group (≥1 year).

Results: Early retransplant survival was 92%.

Twenty-year surgical experience with congenital supravalvar aortic stenosis.

Background: Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome and other elastin gene deletions. Our objectives were to review outcomes of congenital SVAS repair and to compare prosthetic patch repair techniques to all-autologous slide aortoplasty.

Methods: Congenital SVAS repairs from 1988 to 2008 were retrospectively reviewed.

Implications of incising the ventricular septum in double outlet right ventricle and in the Ross-Konno operation.

Objective: Incision into the ventricular septum in complex biventricular repair is controversial, and has been blamed for impairing left ventricular function. This retrospective study evaluates the risk of a ventricular septal incision in patients undergoing double outlet right ventricle (DORV) repair and Ross-Konno procedure.

Methods: From January 2003 to September 2007, 11 patients with DORV had a ventricular septum (VS) incision and 12 DORV patients did not.

Antiphospholipid syndrome leading to myocardial infarction in a Fontan patient 17 years after Norwood palliation.

Appropriate regimens for anticoagulation in the single ventricle population remain controversial and are usually based on individual practitioners' preferences. We present the case of a patient with a history of hypoplastic left heart syndrome and Norwood palliation who presented at 17 years of age with an acute myocardial infarction and a new diagnosis of antiphospholipid syndrome.

Outcomes of reparative and transplantation strategies for multilevel left heart obstructions with mitral stenosis.

Background: Conventional management for multilevel left heart obstructions and mitral stenosis (Shone's complex) involves multiple operations that carry additive risks. This study reviews our experience with reconstructive and transplantation approaches for Shone's complex.

Methods: Between 1987 and 2007, 43 patients with mitral stenosis and one or more left-sided obstructions were identified: supramitral ring (n = 13), subaortic stenosis (n = 25), aortic stenosis (n = 24), hypoplastic arch (n = 20), and coarctation (n = 38).

Intracardiac repair of double outlet right ventricle.

Intracardiac repair of double outlet right ventricle (DORV) remains controversial. DORV is a particular mode of ventriculo-arterial connection and not a specific congenital heart disease. It can exhibit a wide spectrum of anatomic and physiologic variations.

Comparison of the pattern of aortic dilation in children with the Marfan's syndrome versus children with a bicuspid aortic valve.

Marfan's syndrome (MS) and bicuspid aortic valves (BAVs) are associated with aortic dilation. Despite their histologic similarities, the 2 diseases differ with regard to the location of maximal aortic dilation. Echocardiographic analysis of aortic dimensions was performed in children with MS, children who had aortic dilation in the setting of nonstenotic BAVs (peak gradient <16 mm Hg), and healthy controls.

Birth weight and complexity are significant factors for the management of hypoplastic left heart syndrome.

Background: Classic options for treatment of hypoplastic left heart syndrome include the Norwood procedure (NW) and heart transplantation (HT). Recently off-pump palliative procedures were introduced in the management of these patients. Risk factors influencing the decision between the NW with staged reconstruction or off-pump palliation and HT were assessed.

Progression of aortic dilation in children with a functionally normal bicuspid aortic valve.

Adults with bicuspid aortic valves (BAVs) are at risk for progressive aortic dilation independent of valve function. The evolution of aortic dilation in children with functionally normal BAVs has not been studied. In this study, ascending aortic diameters were assessed in a group of children with functionally normal BAVs (peak gradient < or =16 mm Hg) to determine whether progressive dilation occurs during childhood.

The Aristotle method: a new concept to evaluate quality of care based on complexity.

Purpose Of Review: Evaluation of quality of care is a duty of the modern medical practice. A reliable method of quality evaluation able to compare fairly institutions and inform a patient and his family of the potential risk of a procedure is clearly needed. It is now well recognized that any method that purports to evaluate quality of care should include a case mix/risk stratification method.