Correlating Severity of Pulmonary Hypertension by Echocardiogram with Mortality in Premature Infants with Bronchopulmonary Dysplasia.

Objective:  Bronchopulmonary dysplasia (BPD) is the most common complication of preterm birth. Infants with BPD are at increased risk for pulmonary hypertension (PH). Cardiac catheterization is the gold standard for diagnosing PH, but cardiac catheterization is challenging to perform in small, sick, premature infants.

Causes of Death in Infants and Children with Congenital Heart Disease.

With improved surgical outcomes, infants and children with congenital heart disease (CHD) may die from other causes of death (COD) other than CHD. We sought to describe the COD in youth with CHD in North Carolina (NC). Patients from birth to 20 years of age with a healthcare encounter between 2008 and 2013 in NC were identified by ICD-9 code.

Overcoming underpowering: Trial simulations and a global rank end point to optimize clinical trials in children with heart disease.

Background: Randomized controlled trials (RCTs) in children with heart disease are challenging and therefore infrequently performed. We sought to improve feasibility of perioperative RCTs for this patient cohort using data from a large, multicenter clinical registry. We evaluated potential enrollment and end point frequencies for various inclusion cohorts and developed a novel global rank trial end point.

Agreement of an echocardiogram-based diagnosis of pulmonary hypertension in infants at risk for bronchopulmonary dysplasia among masked reviewers.

Objective: To evaluate the agreement of an echocardiogram-based pulmonary hypertension diagnosis in premature infants at risk for bronchopulmonary dysplasia (BPD).

Study Design: Echocardiograms from infants born ≤28 weeks post menstrual age were retrospectively reviewed with a standardized reading protocol by three pediatric cardiologists masked to patient's clinical history to determine the presence of pulmonary hypertension.

Results: A total of 483 echocardiograms from 49 unique patients were each reviewed by three pediatric cardiologists.

PRKAG2 mutations presenting in infancy.

PRKAG2 encodes the γ2 subunit of AMP-activated protein kinase (AMPK), which is an important regulator of cardiac metabolism. Mutations in PRKAG2 cause a cardiac syndrome comprising ventricular hypertrophy, pre-excitation, and progressive conduction-system disease, which is typically not diagnosed until adolescence or young adulthood. However, significant variability exists in the presentation and outcomes of patients with PRKAG2 mutations, with presentation in infancy being underrecognized.

G Protein-coupled Receptor Biased Agonism.

G protein-coupled receptors are the largest family of targets for current therapeutics. The classic model of their activation was binary, where agonist binding induced an active conformation and subsequent downstream signaling. Subsequently, the revised concept of biased agonism emerged, where different ligands at the same G protein-coupled receptor selectively activate one downstream pathway versus another.

Coarctation of the aorta: Management from infancy to adulthood.

Coarctation of the aorta is a relatively common form of congenital heart disease, with an estimated incidence of approximately 3 cases per 10000 births. Coarctation is a heterogeneous lesion which may present across all age ranges, with varying clinical symptoms, in isolation, or in association with other cardiac defects. The first surgical repair of aortic coarctation was described in 1944, and since that time, several other surgical techniques have been developed and modified.

Transcatheter pulmonary embolectomy after fontan.

Pulmonary embolism (PE) is a significant cause of morbidity and mortality among hospitalized patients, and thrombolytics are the mainstay of treatment. However, for patients who fail or have contraindications to thrombolytic therapy, catheter-based therapies, such as the AngioVac aspiration system, have emerged as an alternative to surgical embolectomy. Here, we present the novel case of a 22 year-old woman with Fontan circulation who developed severe postpartum cardiomyopathy and a saddle PE.

Thoracoscopic resection of the left atrial appendage after failed focal atrial tachycardia ablation.

Background: This case series describes 3 patients with the unusual location of focal atrial tachycardia in the left atrial appendage who failed catheter ablation but were successfully treated by left atrial appendage resection by a totally thoracoscopic surgical technique.

Methods: In all 3 cases, left atrial appendage resection was carried out by video-assisted thoracoscopic surgery using only 3 5- to 10-mm incisions, eliminating the need for median sternotomy or thoracotomy. An endoscopic stapler was used to resect the left atrial appendage at its base, successfully eliminating the source of the patients' focal atrial tachycardia.

Tinea capitis mimicking dissecting cellulitis.

Tinea capitis is a common disease of childhood that typically follows one of several clinical patterns. Our patient and several previously reported cases demonstrate the existence of a dissecting cellulitis-like presentation of tinea capitis. This variant should be recognized to prevent misdiagnosis of dissecting cellulitis and allow proper treatment to prevent scarring alopecia.