Cardiology in the young
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Cardiology in the young · Jan 2017
ReviewAdvances in the diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia.
Since the sentinel description of exercise-triggered ventricular arrhythmias in 21 children, our recognition and understanding of catecholaminergic polymorphic ventricular tachycardia has improved substantially. A variety of treatments are now available, but reaching a diagnosis before cardiac arrest remains a challenge. Most cases are related to variants in the gene encoding for ryanodine receptor-2 (RyR2), which mediates calcium-induced calcium release. ⋯ Limited data have suggested that genotype may predict phenotype in catecholaminergic polymorphic ventricular tachycardia, including a higher risk of life-threatening cardiac events in subjects with variants in the C-terminus of ryanodine receptor-2 (RyR2). At present, international efforts are underway to better understand this condition through large prospective registries. The recent publication of gene therapy in an animal model of the recessive form of the disease highlights the importance of improving our understanding of the genetic underpinnings of the disease.
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Cardiology in the young · Jan 2017
Interstage somatic growth in children with hypoplastic left heart syndrome after initial palliation with the hybrid procedure.
Introduction The hybrid procedure is one mode of initial palliation for hypoplastic left heart syndrome. Subsequently, patients proceed with either the "three-stage" pathway - comprehensive second stage followed by Fontan completion - or the "four-stage" pathway - Norwood procedure, hemi-Fontan, or Fontan completion. In this study, we describe somatic growth patterns observed in the hybrid groups and a comparison primary Norwood group.
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Cardiology in the young · Jan 2017
Case ReportsCardiogenic unilateral pulmonary oedema in an infant with severe residual mitral regurgitation.
An infant with residual severe mitral regurgitation following mitral commissurotomy developed cardiogenic unilateral pulmonary oedema and subsegmental atelectasis that resolved with mechanical mitral valve replacement.
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Cardiology in the young · Jan 2017
Effects of chronic treprostinil treatment on experimental right heart hypertrophy and failure.
Right heart function is an important predictor of morbidity and mortality in pulmonary arterial hypertension and many CHD. We investigated whether treatment with the prostacyclin analogue treprostinil could prevent pressure overload-induced right ventricular hypertrophy and failure. ⋯ In the pulmonary trunk banding model of pressure overload-induced right ventricular hypertrophy and failure, moderate- and high-dose treatment with treprostinil did not improve right ventricular function neither in compensated nor in decompensated right heart failure.
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The development of new graduate medical education programmes provides both opportunities and challenges. Efforts to address physician workforce shortages as well as a realisation that curricula need to be updated to adjust to our rapidly changing healthcare environment have resulted in more educators considering the "how to" and "what's new" of programme development. Understanding the Next Accreditation System, an accreditation system introduced by the Accreditation Council of Graduate Medical Education in 2012, is critical to the success of new as well as existing residency and fellowship programmes. ⋯ We provide a series of insights and perspectives based on our experience relative to what priorities we saw outlined from both the programmatic and the institutional perspective to have our graduate medical education programmes reviewed for accreditation. During this discussion, the following objectives are outlined: to overview the Next Accreditation System as a framework and priorities, to discuss the opportunities and challenges that may exist in developing new programmes, and to discuss future directions in the evaluation of trainees and assessment of training competency. Although challenges are outlined, we hope to relay the continued excitement and opportunities that exist relative to enhancing training curricula for future graduate medical education programme builders.