Double outlet right ventricle.

This review article addresses the history, morphology, anatomy, medical management, and different surgical options for patients with double outlet right ventricle.

Early-Onset Cardiomyopathy After Pacemaker Implanted in a Preterm Infant With Congenital Complete Heart Block and Anti-Ro/SSA Antibodies.

Congenital complete heart block is a potentially fatal complication that can occur in neonates whose mothers have autoimmune disorders; it has rarely been reported in the presence of Sjögren syndrome. Pacemaker implantation is recommended to treat rhythm abnormalities in these neonates. We report the case of a late-preterm infant with Sjögren-syndrome-antibody-induced complete heart block who underwent temporary bipolar epicardial pacing as a bridge to permanent pacemaker implantation. Soon after the pacemaker was implanted, takotsubo cardiomyopathy developed. To our knowledge, this is the first report of reversible cardiomyopathy after pacemaker implantation in an infant.

Steroid-associated bradycardia in a newly diagnosed B precursor acute lymphoblastic leukemia patient with Holt-Oram syndrome.

Holt-Oram syndrome (HOS) (OMIM#142900) is a rare condition with upper extremity malformations as well as structural and conduction cardiac anomalies. There are sparse reports in the literature documenting malignancy in association with HOS. We report a pediatric patient clinically diagnosed with HOS (missing thumbs bilaterally, atrial septal defect, ventricular septal defect, and first-degree heart block), who also developed B precursor acute lymphoblastic leukemia. During induction of chemotherapy with steroids, she developed profound bradycardia without clinical symptoms. The bradycardia resolved without intervention, but this case highlights the challenges of managing chemotherapy side effects in a patient with congenital heart disease. A literature review pertinent to the associated findings in the case is also presented.

Chiari Network Associated with Hypoxemia in a Neonate: Case Report and Review of the Literature.

Chiari network is an embryonic remnant of the right sinus venosus. It appears as a thin, fenestrated membrane attached to two or more regions within the right atrium. Usually, a Chiari network has a benign course; however, rare complications associated with a Chiari network have been reported. We present the case of a neonate with hypoxemia and a Chiari network protruding into the right ventricular inflow tract associated with right-to-left shunting across the patent foramen ovale throughout the cardiac cycle. We noted spontaneous improvement after 3 weeks of life.

A Rare Report of Hypoplastic Coronary Arteries and Pulmonary Veins: A Case Report and Review of the Literature.

Congenital coronary artery anomalies are estimated to affect about 1% of the general population. Hypoplastic coronary artery disease is an uncommon subset associated with significant mortality regardless whether another major cardiac anomaly is present. In this case report, we present an extremely rare case of an infant with hypoplastic coronary artery disease and hypoplastic pulmonary veins. A literature review pertinent to the clinical findings is also contained herein.

A Proposed Etiology for Atrial Tachyarrhythmia in Neonates with Atrial Septal Aneurysms.

An atrial septal aneurysm (ASA) is an increasingly recognized entity that involves septal tissue significantly bulging into either atria instead of remaining in a relatively neutral position. ASAs may be described based on the length of the segment of atrial septal tissue involved as well as the distance and direction of excursion into the atria throughout the cardiac cycle. Complications associated with ASA include arrhythmias and thromboembolic events with the latter usually in the context of atrial shunting. While the presence of an ASA has been implicated in the development of atrial tachyarrhythmias, no clear mechanism has been elucidated to-date. In this case, we document one of the previously proposed mechanisms of atrial arrhythmia using echocardiography imaging.

National Survey of Medical Spanish Curriculum in U.S. Medical Schools.

BACKGROUND: Patients with limited English proficiency (LEP) may be at risk for medical errors and worse health outcomes. Language concordance between patient and provider has been shown to improve health outcomes for Spanish-speaking patients. Nearly 40 % of Hispanics, a growing population in the United States, are categorized as having limited English proficiency. Many medical schools have incorporated a medical Spanish curriculum to prepare students for clinical encounters with LEP patients. OBJECTIVE: To describe the current state of medical Spanish curricula at United States medical schools. METHODS: The Latino Medical Student Association distributed an e-mail survey comprising 39 items to deans from each U.S. medical school from July 2012 through July 2014. This study was IRB-exempt. RESULTS: Eighty-three percent (110/132) of the U.S. medical schools completed the survey. Sixty-six percent (73/110) of these schools reported offering a medical Spanish curriculum. In addition, of schools with no curriculum, 32 % (12/37) planned to incorporate the curriculum within the next two years. Most existing curricula were elective, not eligible for course credit, and taught by faculty or students. Teaching modalities included didactic instruction, role play, and immersion activities. Schools with the curriculum reported that the diverse patient populations in their respective service areas and/or student interest drove course development. Barriers to implementing the curriculum included lack of time in students' schedules, overly heterogeneous student language skill levels, and a lack of financial resources. Few schools reported the use of validated instruments to measure language proficiency after completion of the curriculum. CONCLUSIONS: Growing LEP patient populations and medical student interest have driven the implementation of medical Spanish curricula at U.S. medical schools, and more schools have plans to incorporate this curriculum in the near future. Studies are needed to reveal best practices for developing and evaluating the curriculum.

Methanosarcina acetivorans C2A topoisomerase IIIα, an archaeal enzyme with promiscuity in divalent cation dependence.

Topoisomerases play a fundamental role in genome stability, DNA replication and repair. As a result, topoisomerases have served as therapeutic targets of interest in Eukarya and Bacteria, two of the three domains of life. Since members of Archaea, the third domain of life, have not been implicated in any diseased state to-date, there is a paucity of data on archaeal topoisomerases. Here we report Methanosarcina acetivorans TopoIIIα (MacTopoIIIα) as the first biochemically characterized mesophilic archaeal topoisomerase. Maximal activity for MacTopoIIIα was elicited at 30-35°C and 100 mM NaCl. As little as 10 fmol of the enzyme initiated DNA relaxation, and NaCl concentrations above 250 mM inhibited this activity. The present study also provides the first evidence that a type IA Topoisomerase has activity in the presence of all divalent cations tested (Mg(2+), Ca(2+), Sr(2+), Ba(2+), Mn(2+), Fe(2+), Co(2+), Ni(2+), Cu(2+), Zn(2+) and Cd(2+)). Activity profiles were, however, specific to each metal. Known type I (ssDNA and camptothecin) and type II (etoposide, novobiocin and nalidixic acid) inhibitors with different mechanisms of action were used to demonstrate that MacTopoIIIα is a type IA topoisomerase. Alignment of MacTopoIIIα with characterized topoisomerases identified Y317 as the putative catalytic residue, and a Y317F mutation ablated DNA relaxation activity, demonstrating that Y317 is essential for catalysis. As the role of Domain V (C-terminal domain) is unclear, MacTopoIIIα was aligned with the canonical E. coli TopoI 67 kDa fragment in order to construct an N-terminal (1-586) and a C-terminal (587-752) fragment for analysis. Activity could neither be elicited from the fragments individually nor reconstituted from a mixture of the fragments, suggesting that native folding is impaired when the two fragments are expressed separately. Evidence that each of the split domains plays a role in Zn(2+) binding of the enzyme is also provided.

Enhancing calstabin binding to ryanodine receptors improves cardiac and skeletal muscle function in heart failure.

Abnormalities in intracellular calcium release and reuptake are responsible for decreased contractility in heart failure (HF). We have previously shown that cardiac ryanodine receptors (RyRs) are protein kinase A-hyperphosphorylated and depleted of the regulatory subunit calstabin-2 in HF. Moreover, similar alterations in skeletal muscle RyR have been linked to increased fatigability in HF. To determine whether restoration of calstabin binding to RyR may ameliorate cardiac and skeletal muscle dysfunction in HF, we treated WT and calstabin-2-/- mice subjected to myocardial infarction (MI) with JTV519. JTV519, a 1,4-benzothiazepine, is a member of a class of drugs known as calcium channel stabilizers, previously shown to increase calstabin binding to RyR. Echocardiography at 21 days after MI demonstrated a significant increase in ejection fraction in WT mice treated with JTV519 (45.8 +/- 5.1%) compared with placebo (31.1 +/- 3.1%; P < 0.05). Coimmunoprecipitation experiments revealed increased amounts of calstabin-2 bound to the RyR2 channel in JTV519-treated WT mice. However, JTV519 did not show any of these beneficial effects in calstabin-2-/- mice with MI. Additionally, JTV519 improved skeletal muscle fatigue in WT and calstabin-2-/- mice with HF by increasing the binding of calstabin-1 to RyR1. The observation that treatment with JTV519 improved cardiac function in WT but not calstabin-2-/- mice indicates that calstabin-2 binding to RyR2 is required for the beneficial effects in failing hearts. We conclude that JTV519 may provide a specific way to treat the cardiac and skeletal muscle myopathy in HF by increasing calstabin binding to RyR.