Cardiology in the young
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In the normal heart, the right and left coronary arteries arise from the aortic valvar sinuses adjacent to the pulmonary trunk. The right coronary artery then directly enters the right atrioventricular groove, whereas the main stem of the left coronary artery runs a short course before dividing to become the anterior interventricular and circumflex arteries. These arteries can have an anomalous origin from either the aorta or pulmonary trunk; their branches can have various anomalous origins relative to arterial pedicles. ⋯ In the most common variant of an anomalous origin from the pulmonary trunk, the main stem of the left coronary artery arises from the sinus of the pulmonary trunk adjacent to the anticipated left coronary arterial aortic sinus. The artery can, however, arise from a pulmonary artery, or the right coronary artery can have an anomalous pulmonary origin. The key feature in the anomalous aortic origin is the potential for squeezing of the artery, produced by either the so-called intramural origin from the aorta, or the passage of the abnormal artery between the aortic root and the subpulmonary infundibulum.
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The Fontan operation, originally described for the surgical management of tricuspid atresia, is now the final surgery in the strategy of staged palliation for a number of different forms of congenital cardiac disease with a functionally univentricular heart. Despite the improved technical outcomes of the Fontan operation, staged palliation does not recreate a normal physiology. Without a pumping chamber delivering blood to the lungs, the cardiovascular system is less efficient; cardiac output is generally diminished, and the systemic venous pressure is increased. ⋯ Despite the staged palliation resulting in remarkable survival, the possible complications for this group of patients are complex, involve multiple organ systems, and can be life threatening. Identifying the mechanisms associated with each of the rare complications, and developing strategies to treat them, requires the work of many people at many institutions. Continued collaboration between sub-specialists and between institutions will be required to optimise the care for this group of survivors with functionally univentricular hearts.
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Cardiology in the young · Dec 2010
The registry of anomalous aortic origin of the coronary artery of the Congenital Heart Surgeons' Society.
The anomalous aortic origin of a coronary artery from the wrong sinus of Valsalva with interarterial, intramural, and/or intraconal course is a rare congenital anomaly that is associated with a high risk of sudden death in children. The Congenital Heart Surgeons' Society established the Registry of Anomalous Aortic Origin of the Coronary Artery to help determine the outcome of children and young adults managed with surgical intervention versus observation and to test the hypothesis that subsets of patients with anomalous aortic origin of a coronary artery can be identified in whom the risk of intervention is less than the risk of observation. All institutional members of the Congenital Heart Surgeons' Society were recruited for participation. ⋯ The overall purpose of the Registry of Anomalous Aortic Origin of the Coronary Artery is to determine the outcome of surgical intervention versus observation in children and young adults with anomalous aortic origin of a coronary artery, and to describe the natural and "unnatural" history of these patients over the course of their lifetime. In this report, we describe the creation and design of the Registry of Anomalous Aortic Origin of the Coronary Artery. Data from the registry will be published at a later date.
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Cardiology in the young · Dec 2010
Asymptomatic rhythm and conduction abnormalities in children with acute rheumatic fever: 24-hour electrocardiography study.
Some rhythm and conduction abnormalities can occur in children with acute rheumatic fever. These abnormalities have been defined based on standard electrocardiography; however, the real prevalence of these abnormalities has not been investigated previously by the evaluation of long-term electrocardiographic recordings. In this study, we evaluated the asymptomatic rhythm and conduction abnormalities in children with acute rheumatic fever by evaluating the 24-hour electrocardiography. ⋯ Absence of carditis was found to be related to the presence of accelerated junctional rhythm (p > 0.05), and the presence of carditis was found to be related to the presence of premature contractions (p = 0.000). In conclusion, our results suggest that in children with acute rheumatic fever, the prevalence of rhythm and conduction abnormalities may be much higher than determined on standard electrocardiography. Further studies are needed to clarify whether or not these abnormalities are specific to acute rheumatic fever.
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Dysfunction of the autonomic nervous system, or dysautonomia, is an uncommon disease. Postural orthostatic tachycardia syndrome is one of the several types of dysautonomia. Postural orthostatic tachycardia syndrome, also known as chronic orthostatic intolerance, is the most common but least severe of the dysautonomic disorders; it will serve as the model for evaluation and management of the other dysautonomias. ⋯ As postural orthostatic tachycardia syndrome is underdiagnosed and poorly understood, it is a disease that provides an excellent opportunity to perform research. The most important studies would be those that aim to elucidate an aetiology and a pathophysiology of postural orthostatic tachycardia syndrome. In the final analysis, the role of the cardiologist in the evaluation and management of a patient with dysautonomia is to help a patient with severe disability to feel as if they are normal, or much closer to it.