Pediatr Crit Care Me
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Pediatr Crit Care Me · Aug 2016
Shunt Lesions Part I: Patent Ductus Arteriosus, Atrial Septal Defect, Ventricular Septal Defect, and Atrioventricular Septal Defect.
This review summarizes the current understanding of the pathophysiology and perioperative management of patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect. ⋯ The four congenital cardiac lesions that are the subject of this review, patent ductus arteriosus, atrial septal defect, ventricular septal defect, and atrioventricular septal defect, are the most commonly found defects causing a left-to-right shunt. These defects frequently warrant transcatheter or surgical intervention. Although the perioperative care is relatively straightforward for many of these patients, there are a number of management strategies and complications associated with each intervention. The treatment outcomes for all of these lesions are very good in the current era.
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Pediatr Crit Care Me · Aug 2016
Hypoplastic Left Heart Syndrome and Other Shunt-Dependent Single Ventricles.
In this review, we discuss the pathophysiology, treatment, and outcomes of patients with the hypoplastic left heart syndrome and other single ventricle variants prior to and following surgery. ⋯ Patients with shunted single ventricle physiology are at increased risk for acute hemodynamic decompensation owing to the increased myocardial workload, the dynamic balance between systemic and pulmonary circulations, and the potential for shunt obstruction. Understanding of the physiology and anticipatory management are critical to prevent hemodynamic compromise and cardiac arrest.
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Pediatr Crit Care Me · Aug 2016
Aortic and Mitral Valve Disease and Left Ventricular Dysfunction in Children.
In this review, we will discuss aortic stenosis, aortic regurgitation, mitral regurgitation, and mitral stenosis. We will review the etiology, anatomy, pathophysiology, presentation, and treatment of aortic and mitral valve disease. Age and lesion specific treatments are outlined based on the severity of valve disease with an aim at long-term preservation of left ventricular function. ⋯ Mitral and aortic valve disease leads to unique hemodynamic burdens that can impact left ventricular function, quality of life, and longevity. The primary challenge in the management of mitral and aortic valve disease is to apply appropriate medical management and identify that point in time at which the surgery is necessary. Although guidelines have been established for the management of aortic and mitral valve disease in adults, the challenges of early presentation, maintenance of growth potential, and apparent increased tolerance of hemodynamic burden in children makes decision making challenging.
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Pediatr Crit Care Me · Aug 2016
Clinical-Physiological Considerations in Patients Undergoing Staged Palliation for a Functionally Single Ventricle.
The objectives of this review are to discuss the pathophysiology of the circulation with a functionally univentricular heart, with a focus on the unique physiologic characteristics, which provide the underpinnings for the management of these complex patients. ⋯ The circulation of the patient with a functionally univentricular heart displays unique physiologic characteristics, which are quite different from those of the normal biventricular circulation. There are profound differences within the heart itself in terms of ventricular function, interventricular interactions, and myocardial architecture, which are likely to have significant implications for the efficiency of ventricular ejection and metabolism. The coupling between the systemic ventricle and the aorta also displays unique features. The 3D orientation of the Fontan anastomosis itself can profoundly impact cardiac output, although the "portal" pulmonary arterial bed is a crucial determinant of overall cardiovascular function. As a result, disease-specific approaches to improve cardiovascular function are required at all stages during the care of these complex patients.
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The objectives of this review are to discuss the physiology, perioperative management, surgical correction, and outcomes of infants with transposition of the great arteries and common variants undergoing the arterial switch operation. ⋯ The widespread adoption of the arterial switch operation for transposition of great arteries has been one of the more gratifying advances in pediatric cardiovascular care, and represents the simultaneous improvements in diagnostics, surgical and bypass techniques, anesthesia in the neonate, improvements in intensive care technology, nursing strategies, and system-wide care delivery. Many of the strategies adopted for the neonate with transposition of the great arteries have been translated to neonatal care for other congenital heart lesions. Continued work is necessary to investigate the effects of perioperative care on long-term neurodevelopmental outcomes, as well as collaboration between centers to spread "best practices" for outcome, cost, and morbidity reduction.