Congenital heart disease
-
Congenital heart disease · Nov 2014
Review Historical ArticlePublic health research in congenital heart disease.
Public health research is an integral part of the study of congenital heart disease. While this type of research has become more popular, particularly over the past decade, it has a history that stretches back to almost the beginnings of pediatric cardiology as a field. This review aims to introduce the concepts and methodologies of public health and how they relate to congenital heart disease, describe some of the challenges of traditional research methods in congenital heart disease, describe the history of public health research, and demonstrate the relevance of public health research, particularly databases, to pediatric cardiology fellows.
-
Congenital heart disease · May 2014
Review Case ReportsSevere left main coronary artery stenosis with abnormal branching pattern in a patient with mild supravalvar aortic stenosis and Williams-Beuren syndrome.
Williams-Beuren syndrome (WBS) is a multisystem genetic disorder comprising of craniofacial, developmental, and cardiac malformations. The most common cardiac defects found are supravalvar aortic stenosis and peripheral pulmonary stenosis. However, WBS should be regarded as a general arteriopathy consisting of stenoses of medium- and large-sized arteries including the coronary arteries. ⋯ This case report highlights the necessity of careful cardiology assessment without delay in patients with a suspicion of WBS. Isolated coronary stenosis though rare in WBS should be considered in the presence of ischemia or reduced ventricular function. Larger case series are needed to further characterize the correlation between WBS and acute coronary events.
-
Congenital heart disease · Nov 2013
ReviewHemodynamic monitoring in the cardiac intensive care unit.
Hemodynamic monitoring is central to the management of critically ill patients in the cardiac intensive care unit (CICU). The goals of hemodynamic monitoring are to anticipate threats and complications before they arise, to gauge the effectiveness of interventions, and to avoid progression to a decompensated shock state. Although there are numerous modalities of hemodynamic monitoring in the CICU, discordance exists between assessments based on physical exam and standard hemodynamic parameters and those based on measurements of cardiac output. This article will review both the standard and advanced hemodynamic monitoring strategies employed in the CICU.
-
Congenital heart disease · Sep 2012
ReviewResuscitation and perioperative management of the high-risk single ventricle patient: first-stage palliation.
Infants born with hypoplastic left heart syndrome or other lesions resulting in a single right ventricle face the highest risk of mortality among all forms of congenital heart disease. Before the modern era of surgical palliation, these conditions were universally lethal; recent refinements in surgical technique and perioperative management have translated into dramatic improvements in survival. ⋯ Herein, resuscitation and perioperative management of infants with hypoplastic left heart syndrome are reviewed. Basic neonatal and pediatric life support recommendations are summarized, and perioperative first-stage clinical management strategies are reviewed.
-
Congenital heart disease · Jan 2012
ReviewCan "inoperable" congenital heart defects become operable in patients with pulmonary arterial hypertension? Dream or reality?
The decision whether to repair congenital heart defects in patients with raised pulmonary vascular resistance to alleviate pulmonary hypertension is a complex one. The degree of pulmonary vascular disease is of paramount importance. Operating on patients with pulmonary vascular resistance above a certain threshold runs the risk of postoperative persistent pulmonary hypertension and a worse long-term prognosis. This review focuses on patients deemed "borderline inoperable" or "inoperable" due to pulmonary vascular disease and asks whether they can be "converted to an operable status" with pulmonary arterial hypertension-specific drugs that potentially modify the pulmonary vascular lesions and resistance.