Advances and Innovation in Acute Type a Aortic Dissection.

The prompt and appropriate management of acute type A aortic dissections is imperative for patient survival. Advances in medical technology have broadened the adjuncts available to treat the spectrum of pathology within this population. The role of medical management prior to surgical intervention and the components of operative management, including cannulation strategies, neuroprotection, and the extent of aortic intervention, have been explored in-depth within the literature.

Mitral Valve Repair Is Effective When Performed With Valve-Sparing Aortic Root Replacement.

Background: Concurrent aortic and mitral valve repair presents specific technical challenges to surgeons. Here we evaluate the outcomes of patients undergoing combined valve-sparing aortic root reimplantation and mitral valve repair.

Methods: We performed a retrospective review of patients at our center between 2006 and 2021 who underwent concomitant valve-sparing aortic root replacement and mitral valve repair.

Impact of Ventricular Dysfunction and Atrioventricular Valve Regurgitation on Pre-Fontan Attrition.

Background: There is a significant incidence of pre-Fontan attrition-defined as failure to undergo Fontan completion-after superior cavopulmonary connection. This study investigated the impact of at least moderate ventricular dysfunction (VD) and atrioventricular valve regurgitation (AVVR) on pre-Fontan attrition.

Methods: This single-center retrospective cohort study included all infants who underwent Norwood palliation from 2008 to 2020 and subsequently underwent superior cavopulmonary connection.

Contemporary outcomes of open repair of acute complicated type B aortic dissection.

Objective: Open repair of acute complicated type B aortic dissection (ACTBAD), required when endovascular repair is not possible, is historically considered high-risk. We analyze our experience with this high-risk cohort compared with the standard cohort.

Methods: We identified consecutive patients undergoing descending thoracic or thoracoabdominal aortic aneurysm (TAAA) repair from 1997 to 2021.

Results of a Multimodal Approach for the Launch of a Ross Program.

Background: There has been renewed interest in the Ross procedure in young patients with aortic valve disease. We describe the details and results of a systematic strategy to launch a new Ross program.

Methods: Our department developed a multidisciplinary educational program that involved all practitioners involved in the care of potential Ross patients.

Balancing quality and quaternary care imperative using a high-risk case review committee in adults.

Objectives: Quaternary care centres have an imperative to serve as hospitals of last resort and must also meet professional quality targets. We developed a high-risk committee (HRC) to evaluate cases meeting pre-defined predicted risk cut-offs as a part of an overall quality improvement drive.

Methods: We describe the structure, outcomes and effects of the Penn HRC.

Noonan syndrome associated with hypoplastic left heart syndrome.

Noonan syndrome is an inherited disorder caused by alterations in the RAS-MAPK pathway. There have been several identified genotype-phenotype associations made with respect to congenital cardiac lesions and Noonan syndrome variants, but limited data exist regarding single ventricle disease in this population. Here, we report two patients with -related Noonan syndrome and hypoplastic left heart syndrome variants.

Radiographic and histologic characterisation of white matter injury in a sheep model of CHD.

Nearly one in five children with CHD is born with white matter injury that can be recognised on postnatal MRI by the presence of T1 hyperintense lesions. This pattern of white matter injury is known to portend poor neurodevelopmental outcomes, but the exact aetiology and histologic characterisation of these lesions have never been described. A fetal sheep was cannulated at gestational age 110 days onto a pumpless extracorporeal oxygenator via the umbilical vessels and supported in a fluid environment for 14.

Attrition between the superior cavopulmonary connection and the Fontan procedure in hypoplastic left heart syndrome.

Objective: We investigated the incidence and predictors of failure to undergo the Fontan in children with hypoplastic left heart syndrome who survived superior cavopulmonary connection.

Methods: The cohort consists of all patients with hypoplastic left heart syndrome who survived to hospital discharge after superior cavopulmonary connection between 1988 and 2017. The primary outcome was attrition, which was defined as death, nonsuitability for the Fontan, or cardiac transplantation before the Fontan.

Contrast-Enhanced Brain Ultrasound Perfusion Metrics in the EXTra-Uterine Environment for Neonatal Development (EXTEND): Correlation With Hemodynamic Parameters.

Objectives: Contrast-enhanced ultrasound (CEUS) can provide quantitative perfusion metrics and may be useful to detect cerebral pathology in neonates and premature infants, particularly in extrauterine environments. The effect of hemodynamics on cerebral perfusion metrics is unknown, which limits the clinical application of this technology. We aimed to determine associations between systemic hemodynamics and concurrently measured brain perfusion parameters in an animal model of extrauterine support.

Fetoscopic insufflation modeled in the extrauterine environment for neonatal development (EXTEND): Fetoscopic insufflation is safe for the fetus.

Background: Minimally invasive fetal surgery, or fetoscopy, is an alternative to open fetal surgery to repair common birth defects like myelomeningocele. Although this hysterotomy-sparing approach reduces maternal morbidity, the effects of in utero insufflation on the fetus are poorly understood. Our purpose was to determine the optimal fetal insufflation conditions.

Off-Pump Explant of a Left Ventricular Assist Device Using a Recovery Plug in a Pediatric Patient.

Though the numbers remain small, the use of continuous-flow left ventricular assist devices as a bridge to recovery in pediatric patients has been increasing. Select patients may have sufficient myocardial recovery to allow for device removal. Here, we describe a 13-year old requiring left ventricular assist device implantation for myocarditis who was referred for explant of the device after recovery.

The EXTrauterine Environment for Neonatal Development Supports Normal Intestinal Maturation and Development.

Background And Aims: The Extra-Uterine Environment for Neonatal Development (EXTEND) aims to avoid the complications of prematurity, such as NEC. Our goal was to determine if bowel development occurs normally in EXTEND-supported lambs, with specific emphasis on markers of immaturity associated with NEC.

Methods: We compared terminal ileum from 17 pre-term lambs supported on EXTEND for 2- 4 weeks to bowel from age-matched fetal lambs that developed in utero.

Neurologic outcomes of the premature lamb in an extrauterine environment for neonatal development.

Background/purpose: Neurologic injury remains the most important morbidity of prematurity. Those born at the earliest gestational ages can face a lifetime of major disability. Perinatal insults result in developmental delay, cerebral palsy, and other profound permanent neurologic impairments.

Inhaled Nitric Oxide Is Associated with Improved Oxygenation in a Subpopulation of Infants with Congenital Diaphragmatic Hernia and Pulmonary Hypertension.

Objectives: To determine which patients with congenital diaphragmatic hernia (CDH) and pulmonary hypertension (PH) benefit from inhaled nitric oxide (iNO) treatment by comparing characteristics and outcomes of iNO responders to nonresponders.

Study Design: We performed a retrospective chart review of infants with CDH treated at our center between 2011 and 2016. In a subset of patients, iNO was initiated for hypoxemia or echocardiographic evidence of extrapulmonary right to left shunting.

Prenatal hypoxemia alters microglial morphology in fetal sheep.

Objective: Neuroimmune cells, particularly microglia and astrocytes, play a critical role in neurodevelopment. Neurocognitive delays are common in children with congenital heart disease, but their etiology is poorly understood. Our objective was to determine whether prenatal hypoxemia, at levels common in congenital heart disease, induced neuroimmune activation to better understand the origins of neurobehavioral disorders in congenital heart disease.