Thoracic Aortic, Aortic Valve, and Mitral Valve Surgery in Pediatric and Young Adult Patients With Marfan Syndrome: Characteristics and Outcomes.

Patients with Marfan syndrome (MFS) often require surgical intervention on the mitral valve (MV), aortic root or valve (AV), or thoracic aorta (TA) during childhood and adolescence. We aim to utilize a national database to evaluate outcomes in pediatric and young adult patients with MFS undergoing MV, AV, and aortic surgical procedures, and describe factors associated with increased mortality. The Pediatric Hospital Information System (PHIS) database, a multi-institutional administrative database of 48 pediatric hospitals, was queried for patients less than 25 years of age with a diagnosis of MFS (ICD-9 759.82) who underwent MV, AV, or thoracic aortic surgery between January 2004 and October 2015. We assessed comorbidities and complications, and performed univariate analysis to evaluate factors associated with inpatient mortality. Included were 321 hospital encounters in 294 patients. Fifty-one patients underwent 54 MV surgeries, 213 patients underwent 224 aortic/AV surgeries, and 43 patients underwent both MV and aortic/AV surgery in the same encounter. Postoperative complications were common for all surgeries (46.3% for MV procedures and 45.5% for aortic/AV procedures). Overall in-hospital mortality was 2.2% (3.7% for MV procedures, 1.8% for AV/aortic procedures, and 2.3% in the combined MV and aortic/AV procedure group). Aortic dissection or rupture was reported in 3.4%, with no in-hospital mortalities. Death after MV as well as after aortic/AV surgery was associated with younger age. Postoperative complications are common in pediatric and young adult patients with MFS after intervention on the MV, AV, and TA, although mortality is relatively low.

In Utero Aortic Arch Thrombosis Masquerading as Interrupted Aortic Arch: A Case Report and Review of the Literature.

Aortic arch thrombosis is an extremely rare but life-threatening diagnosis that is often misdiagnosed in the neonatal period. Strategies including surgical intervention, systemic anticoagulation, and thrombolysis have been previously described in the treatment of these neonates. We describe the case of a neonate who presented with concern for interrupted aortic arch and was diagnosed with an in utero aortic arch thrombosis. To our knowledge, this is the first reported case with evidence of aortic arch thrombosis in fetal life. The patient underwent successful treatment with systemic thrombolysis with tissue plasminogen activator. A brief review of the literature regarding the diagnosis, treatment, and management of neonatal aortic arch thrombosis is also presented.

Strength in numbers: Crowdsourcing the most relevant literature in pediatric cardiology.

OBJECTIVE: The growing body of medical literature in pediatric cardiology has made it increasingly difficult for individual providers to stay abreast of the most current, meaningful articles to help guide practice. Crowdsourcing represents a collaborative process of obtaining information from a large group of individuals, typically from an online or web-based community, and could serve a potential mechanism to pool individual efforts to combat this issue. This study aimed to utilize crowdsourcing as a novel way to generate a list of the most relevant, current publications in congenital heart disease, utilizing input from an international group of professionals in the field of pediatric cardiology. DESIGN AND SETTING: All members of the PediHeartNet Google group, an international email distribution list of medical professionals with an interest in pediatric cardiology, were queried in 2017 to submit literature that they considered to be most relevant to their current practice. A Google Form submission platform was used. The articles were evaluated by a multi-institutional panel of four experts in pediatric cardiology using the Delphi method via an electronic evaluation form until a consensus was reached regarding whether the article merited inclusion in the final list. RESULTS: In total, 260 articles were submitted by members of the PediHeartNet Google group. Expert review using the Delphi method resulted in a list of 108 articles. The final collection of articles was published on a publicly available educational website. CONCLUSIONS: Crowdsourcing represents a novel approach for generating a high-yield, comprehensive, yet practical list of the most relevant recent publications in pediatric cardiology. The same techniques could be easily applied to any medical subspecialty. By enlisting the input of frontline providers, the value and relevance of such a list will be significant. A web-based platform for publication of the list allows for real-time updates to ensure continued relevance.

Utility and safety of the SafeSept™ transseptal guidewire for electrophysiology studies with catheter ablation in pediatric and congenital heart disease.

PURPOSE: The atrial transseptal procedure is used in catheter ablation of left-sided arrhythmias. Studies in adult patients have shown the SafeSept™ transseptal guidewire (SSTG) to be effective in atrial transseptal procedures. We analyzed our 5-year experience with SSTG use in pediatric and congenital heart disease patients undergoing catheter ablation. METHODS: This is a single-center retrospective analysis of patients undergoing catheter ablation from 2009 to 2014. We identified all procedures where SSTG was used for atrial transseptal or trans-baffle access. Success of transseptal access and complications were recorded and compared to the standard transseptal approach without the SSTG. RESULTS: One hundred twenty-seven patients underwent 132 attempted atrial transseptal or trans-baffle procedures using SSTG. Median age was 14 (1.2-38) years. Arrhythmia substrates included AV reentrant tachycardia (90.2%), atrial tachycardia (4.5%), ventricular tachycardia (2.3%), and AV nodal reentrant tachycardia (2.3%). Transseptal or trans-baffle access was successful in 96.2% of the SSTG cases compared to 98.9% in the standard transseptal group without SSTG (p = NS). The youngest patient with successful atrial transseptal procedure using SSTG was 4 years old. SSTG was used to successfully cross a surgically created atrial baffle in a patient who had undergone the Mustard procedure. There was one major complication in both groups, 0.8% in the SSTG group compared to the standard transseptal group without SSTG, 1.1% (p = NS). The major complication in the SSTG group occurred when the SSTG crossed the aorta into the coronary artery system and mimicked placement in the left atrial appendage, with subsequent placement of a transseptal sheath into the aorta, requiring sternotomy and surgical intervention. CONCLUSIONS: SSTG is effective for use in atrial transseptal and surgical trans-baffle access in pediatric and congenital heart disease patients. Placement of the SSTG into the pulmonary vein is necessary to avoid major complications, and if not achieved requires additional methods to determine appropriate left atrial placement.

Social interactions and the circadian rhythm in locomotor activity in the cockroach Leucophaea maderae.

The role of social interactions in entrainment has not been extensively studied in the invertebrates. Leucophaea maderae is a gregarious species of cockroach that exhibits extensive social interactions. Social interactions associated with copulation between the sexes have been shown to be regulated by the circadian system. We show here that social interactions between males are also under circadian control. We examined the question of whether or not these rhythmic social contacts could function as zeitgebers capable of regulating circadian phase and period. Animals initially in phase that were housed as groups or pairs of single sex or mixed sex in constant darkness for 2-7 weeks were found to drift out of phase. Their behavior was not significantly different from individual animals maintained in isolation. Further, animals that were initially out of phase by 12 h housed as groups or pairs were not significantly different in phase from animals that were isolated. The results show the circadian clocks of cockroaches are remarkably insensitive to the extensive social interactions that occur between individuals.