Feeding Difficulties Following Vascular Ring Repair: A Contemporary Narrative Review.

Vascular rings are congenital abnormalities of the aortic arch vascular system that compress the trachea and esophagus. A review of long-term outcomes suggests that chronic feeding difficulties can persist following surgical repair of vascular rings. Previous reports of postoperative vascular ring division outcomes indicate that chronic esophageal symptoms may persist following repair, though most available data focuses on persistent respiratory symptoms. It is therefore the aim of this article to summarize and organize recent evidence reporting the frequency, presentation, and management of feeding difficulties following vascular ring repair in pediatric patients. Pathophysiologic mechanisms for postoperative esophageal symptoms may include residual compression from an unresected diverticulum of Kommerell or delayed repair leading to chronic esophageal dysmotility despite correction of esophageal compression. Guidance on the management of feeding difficulties following vascular ring repair is limited. The authors describe success in one case with nasogastric tube feeding and interdisciplinary evaluation. Consensus regarding the management of feeding difficulty following vascular ring repair is needed.

Longitudinal analysis of cardiac abnormalities in pediatric patients with sickle cell anemia and effect of hydroxyurea therapy.

Cardiac abnormalities such as left ventricular hypertrophy, left ventricular dilation, and pulmonary hypertension in sickle cell anemia have been previously described. Hydroxyurea, a disease-modifying therapy for sickle cell anemia, has been used for several decades. Longitudinal assessment of echocardiographic abnormalities in children and young adults with sickle cell anemia receiving hydroxyurea therapy is lacking. The goal of this retrospective study was to determine the prevalence of echocardiographic abnormalities in children and young adults with sickle cell anemia and to examine the effects of hydroxyurea on reverse cardiac remodeling. We reviewed the records of patients with sickle cell anemia who underwent routine cardiac screening at Cohen Children's Medical Center between 2010 and 2017, followed by retrospective longitudinal analysis of echocardiograms performed on patients receiving treatment with hydroxyurea. Data on a total of 100 patients with sickle cell anemia were analyzed; 60 (60%) were being treated with hydroxyurea. Twenty-five (41.6%) of the patients on hydroxyurea had been treated for <1 year; these patients had a significantly greater prevalence of left ventricular dilation compared with those who had been on treatment for >1 year. Serial echocardiograms of patients receiving hydroxyurea were then analyzed. Left ventricular dilation and hypertrophy improved significantly with hydroxyurea treatment. In addition, the left ventricular volume and mass correlated negatively with duration of treatment with hydroxyurea. Our study provides evidence that prolonged hydroxyurea therapy may lead to reverse cardiac remodeling. Future studies should attempt to follow up this patient cohort for a longer duration.

Neurodevelopmental outcomes in congenital heart disease through the lens of single ventricle patients.

PURPOSE OF REVIEW: This review aims to summarize and organize the current body of literature on this contemporary topic, alongside a more general discussion of neurodevelopmental complications of congenital heart disease. RECENT FINDINGS: It is theorized that the causes of the neurodevelopment disabilities are multifactorial resulting from structural central nervous system abnormalities, haemodynamic alterations and/or biochemical changes. It is therefore imperative that all patients with single ventricle anatomy and physiology receive long-term neurologic and developmental assessments in addition to their cardiac monitoring. SUMMARY: Advancements in surgical techniques and medical management have improved survivorship of these medically complex patients. Neurodevelopmental sequelae are one of the most common comorbidities affecting this patient population leading to long-term challenges in motor, language, social and cognitive skills.

Society for Cardiovascular Magnetic Resonance 2019 Case of the Week series.

The Society for Cardiovascular Magnetic Resonance (SCMR) is an international society focused on the research, education, and clinical application of cardiovascular magnetic resonance (CMR). The SCMR web site ( https://www.scmr.org ) hosts a case series designed to present case reports demonstrating the unique attributes of CMR in the diagnosis or management of cardiovascular disease. Each clinical presentation is followed by a brief discussion of the disease and unique role of CMR in disease diagnosis or management guidance. By nature, some of these are somewhat esoteric, but all are instructive. In this publication, we provide a digital archive of the 2019 Case of the Week series as a means of further enhancing the education of those interested in CMR and as a means of more readily identifying these cases using a PubMed or similar search engine.

Cardiac complications of malnutrition in adolescent patients: A narrative review of contemporary literature.

Eating disorders are common. Between 1% and 2% of adolescent females and 0.5% of males suffer from anorexia nervosa, bulimia nervosa, and binge eating disorder. Although suicide represents nearly half of the mortality in patients with eating disorders, a majority of the remainder is cardiac arrest, likely secondary to cardiovascular complications of eating disorders such as bradycardia, hypotension, QT interval changes, structural heart disease, and pericardial effusion. Bradycardia is suspected to be secondary to increased vagal tone and is a common finding in patients admitted with disordered eating. Similarly, hypotension and orthostatic abnormalities are common complications due to atrophy of peripheral muscles. Descriptive studies report prolongation of the corrected QT interval (QTc) in these patients relative to controls, albeit within the normal reference range. Structural heart disease is also common, with left ventricular mass reported as lower than predicted in several studies compared to healthy controls. Pericardial effusion is also commonly described, although it is possible that this is underestimated, as not all patients with eating disorders undergo echocardiograms. Further, refeeding syndrome as a result of treatment of eating disorders carries its own cardiac risks. Cardiac complications of malnutrition are common but reversible with appropriate management and recovery. It is imperative that providers are aware of the epidemiology of these complications, as it is only with a high clinical suspicion that proper evaluation including a thorough history and physical examination, electrocardiogram, and when necessary echocardiogram can be performed.

Surgical Angioplasty of Stenotic Left Main Coronary Artery 15 Years After ALCAPA Repair.

Studies regarding long-term results after repair of anomalous left main coronary artery from the pulmonary artery (ALCAPA) have mainly focused on survival rates, left ventricular function, and mitral valve function. The development of left main coronary stenosis following repair has been infrequently reported and its incidence unknown. Optimal therapy is also not known. Here we report the use of surgical angioplasty to achieve revascularization of a stenotic left main coronary artery in a patient with ALCAPA who had undergone coronary transfer 15 years earlier.