Hypoplastic left heart syndrome (HLHS) with a restrictive atrial septal defect (ASD) is a form of congenital heart disease with considerable morbidity and mortality. This morphologic analysis assesses the pulmonary vasculature in this patient population. Pulmonary arteries, the persistence of high-resistance fetal arterioles, pulmonary veins, and lymphatics from multiple lung sections from each of five patients with HLHS and a restrictive ASD were compared to those of five patients with HLHS and nonrestrictive ASD. Lung sections from each patient were qualitatively graded in severity of pathology from 0 to 3 for each of the structures described previously, with the pathologist blinded to the status of the ASD. Patients with a restrictive ASD exhibited more significant pulmonary venous thickening and lymphatic dilatation (p = 0.02), with a tendency toward persistence of high-resistance fetal vessels (p = 0.2), compared to patients with a nonrestrictive ASD. These findings imply that patients with HLHS and a restrictive ASD possess pulmonary vascular abnormalities that place them at higher risk for the current surgical interventions available compared to patients with a nonrestrictive ASD.
Current outcomes of hypoplastic left heart syndrome with restrictive atrial septum: a single-center experience
Hypoplastic left heart syndrome with intact atrial septum--attempt of an interventional palliation by ductal and interatrial stent implantation
Effect of a selection and postoperative care protocol on survival of infants with hypoplastic left heart syndrome
Preoperative pulmonary hemodynamics and assessment of operability: is there a pulmonary vascular resistance that precludes cardiac operation?
Altered lymphatics in an ovine model of congenital heart disease with increased pulmonary blood flow
Vasoreactive response to maternal hyperoxygenation in the fetus with hypoplastic left heart syndrome
The use of reconstructive surgery to improve quality of life and survival in prenatal hypoplastic left heart syndrome
Pediatric plastic bronchitis: case report and retrospective comparative analysis of epidemiology and pathology
Technical challenges of atrial septal stent placement in fetuses with hypoplastic left heart syndrome and intact atrial septum
Hypoplastic left heart syndrome and the nutmeg lung pattern in utero: a cause and effect relationship or prognostic indicator?
Differing lung function development in infants with univentricular hearts compared with healthy infants
Prenatal diagnosis of atrial restriction in hypoplastic left heart syndrome is associated with decreased 2-year survival
Pulmonary overcirculation following left atrial decompression in hypoplastic left heart syndrome with intact/restrictive atrial septum
Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: surgical experience from a single center
Fetal critical aortic stenosis with natural improvement of hydrops fetalis due to spontaneous relief of severe restrictive atrial communication
KLF2-mediated disruption of PPAR-γ signaling in lymphatic endothelial cells exposed to chronically increased pulmonary lymph flow
Hypoplastic left heart syndrome with intact or highly restrictive atrial septum: outcome after neonatal transcatheter atrial septostomy
Percutaneous fetal cardiac catheterization technique for stenting the foramen ovale in a midgestation lamb model
Predictive value of fetal pulmonary venous flow patterns in identifying the need for atrial septoplasty in the newborn with hypoplastic left ventricle
Creation of an atrial septal defect in utero for fetuses with hypoplastic left heart syndrome and intact or highly restrictive atrial septum
Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association
Stent implantation for recurrent interatrial obstruction in an infant with hypoplastic left heart syndrome
Catheter-based decompression of the left atrium in patients with hypoplastic left heart syndrome and restrictive atrial septum is safe and effective
Disrupted NOS signaling in lymphatic endothelial cells exposed to chronically increased pulmonary lymph flow
Impact of malnutrition on pediatric risk of mortality score and outcome in Pediatric Intensive Care Unit
Risk stratification of patients with hypoplastic left heart syndrome and intact atrial septum using fetal MRI and echocardiography.
Pulmonary venous Doppler patterns and midterm outcomes in fetuses with left-sided obstructive lesions and restrictive atrial septum.
Guidelines for the management of neonates and infants with hypoplastic left heart syndrome: The European Association for Cardio-Thoracic Surgery (EACTS) and the Association for European Paediatric and Congenital Cardiology (AEPC) Hypoplastic Left Heart Syndrome Guidelines Task Force.
Hypoplastic left heart syndrome with prenatally diagnosed foramen ovale restriction: diagnosis, management and outcome
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CSF & Lymphatic System
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