Associations of Home Monitoring Data to Interventional Catheterization for Infants with Recurrent Coarctation of the Aorta and Hypoplastic Left Heart Syndrome.

The post-Norwood interstage period for infants with hypoplastic left heart syndrome is a high-risk time with 10-20% of infants having a complication of recurrent coarctation of the aorta (RCoA). Many interstage programs utilize mobile applications allowing caregivers to submit home physiologic data and videos to the clinical team. This study aimed to investigate if caregiver-entered data resulted in earlier identification of patients requiring interventional catheterization for RCoA.

Impact of Udenafil on Echocardiographic Indices of Single Ventricle Size and Function in FUEL Study Participants.

Background: The FUEL trial (Fontan Udenafil Exercise Longitudinal) demonstrated statistical improvements in exercise capacity following 6 months of treatment with udenafil (87.5 mg po BID). The effect of udenafil on echocardiographic measures of single ventricle function in this cohort has not been studied.

Results of the FUEL Trial.

Background: The Fontan operation creates a total cavopulmonary connection, a circulation in which the importance of pulmonary vascular resistance is magnified. Over time, this circulation leads to deterioration of cardiovascular efficiency associated with a decline in exercise performance. Rigorous clinical trials aimed at improving physiology and guiding pharmacotherapy are lacking.

Pathophysiology, adaptation, and imaging of the right ventricle in Fontan circulation.

The Fontan procedure, which creates a total cavopulmonary anastomosis and represents the final stage of palliation for hypoplastic left heart syndrome, generates a unique circulation relying on a functionally single right ventricle (RV). The RV pumps blood in series around the systemic and pulmonary circulation, which requires adaptations to the abnormal volume and pressure loads. Here, we provide a complete review of RV adaptations as the RV assumes the role of the systemic ventricle, the progression of RV dysfunction to a distinct pattern of heart failure unique to this disease process, and the assessment and management strategies used to protect and rehabilitate the failing RV of Fontan circulation.

Design and rationale of the Fontan Udenafil Exercise Longitudinal (FUEL) trial.

The Fontan operation creates a circulation characterized by elevated central venous pressure and low cardiac output. Over time, these characteristics result in a predictable and persistent decline in exercise performance that is associated with an increase in morbidity and mortality. A medical therapy that targets the abnormalities of the Fontan circulation might, therefore, be associated with improved outcomes.

Development and Implementation of a Standardized Heparin Protocol for Left-Sided Pediatric Electrophysiology Procedures.

Heparin is used to decrease the risk of thromboembolic complications during electrophysiology studies (EPS); however, there is wide practice variation and minimal evidence to guide heparin dosing, particularly in pediatric patients. This study retrospectively analyzed heparin dosing and response, measured via activated clotting time (ACT), in patients undergoing EPS and used these data (pre-protocol cohort, n = 40), as well as guidance from available literature to implement a standardized heparin protocol (phase 1, n = 43). We utilized quality improvement methodology to refine this protocol (phase 2, n = 40) to improve therapeutic heparin response.

Infected Congenital Epicardial Cyst Presenting as Acute Abdomen.

A previously healthy 3-year-old boy presented to the emergency department with abdominal pain, fever, and emesis. Laboratory and radiologic evaluation for causes of acute abdomen were negative; however, review of the abdominal x-ray demonstrated cardiomegaly with the subsequent diagnosis of pericardial cyst by echocardiogram and computed tomography. The patient underwent surgical decompression and attempted removal of the cystic structure revealing that the cyst originated from the epicardium.

Total Anomalous Pulmonary Venous Connection: Preoperative Anatomy, Physiology, Imaging, and Interventional Management of Postoperative Pulmonary Venous Obstruction.

Total anomalous pulmonary venous connection refers to a spectrum of cardiac anomalies where the pulmonary veins fail to return to the left atrium and the pulmonary venous blood returns through a systemic vein or directly to the right atrium. There is a wide anatomical variety of venous connections and degrees of pulmonary venous obstruction that affect the presentation, surgical repair, and outcomes. In this review, we explore the preoperative physiology, echocardiographic diagnosis, and approach to postoperative complications.

Preoperative Physiology, Imaging, and Management of Transposition of the Great Arteries.

Transposition of the great arteries (TGA) refers to hearts with concordant atrioventricular connections but discordant ventriculoarterial connections. In this lesion, the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. As such, the pulmonary and aortic circulations run in parallel as opposed to in series, and this lesion is not compatible with survival without adequate mixing of these circulations.

Triiodothyronine facilitates weaning from extracorporeal membrane oxygenation by improved mitochondrial substrate utilization.

Background: Extracorporeal membrane oxygenation (ECMO) provides a bridge to recovery after myocardial injury in infants and children, yet morbidity and mortality remain high. Weaning from the circuit requires adequate cardiac contractile function, which can be impaired by metabolic disturbances induced either by ischemia-reperfusion and/or by ECMO. We tested the hypothesis that although ECMO partially ameliorates metabolic abnormalities induced by ischemia-reperfusion, these abnormalities persist or recur with weaning.

'Shovel-Ready' applications of stem cell advances for pediatric heart disease.

Purpose Of Review: The past decade has seen remarkable advances in the field of stem cell biology. Many new technologies and applications are passing the translational phase and likely will soon be relevant for the clinical pediatric cardiologist.

Recent Findings: This review will focus on two advances in basic science that are now translating into clinical trials.

A child with eosinophilia, Loeffler endocarditis, and acute lymphoblastic leukemia.

We present an 8-year-old male with Loeffler endocarditis and acute lymphoblastic leukemia with hypereosinophilia (ALL/Eo) who initially presented with a 3-month history of peripheral eosinophilia thought to be due to visceral larval migrans. Despite treatment for Toxocara, his leukocytosis persisted and he developed mitral valve insufficiency and congestive heart failure. Myocardial biopsy revealed fibrosis and thrombus formation indicative of Loeffler endocarditis, and a peripheral smear showed pre-B-cell acute lymphoid leukemia.