Congenital heart disease
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Congenital heart disease · May 2017
Incidence, morphology, and progression of bicuspid aortic valve in pediatric and young adult subjects with coexisting congenital heart defects.
Bicuspid aortic valve (BAV) occurs both as an isolated cardiac lesion and in association with congenital heart defects (CHD). Their aim was to identify the incidence and morphology of BAV in patients with coexisting CHD and compare their disease progression to patients with isolated BAV. ⋯ The morphology of BAV, the pattern and progression of aortic dilatation, and the severity of aortic valve disease vary in pediatric and young adult patients with BAV and coexisting CHD. However, there was no significant BAV disease progression when associated with these CHD.
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Congenital heart disease · Mar 2017
ReviewSurveillance for liver complications after the Fontan procedure.
The physiological consequences of the Fontan circulation impose risk for hepatic dysfunction and may culminate in hepatic fibrosis, cirrhosis, and hepatocellular carcinoma. Consensus regarding appropriate surveillance modalities to diagnose liver disease in Fontan patients is lacking, in part due to the relative lack of strong evidence and prospective studies in this patient population. The goal of this paper is to critically review the current evidence and provide recommendations for the surveillance of hepatic complications in the post-Fontan patient population.
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Congenital heart disease · Mar 2017
ReviewPeri-procedural risk stratification and management of patients with Williams syndrome.
Williams syndrome (WS) is a congenital, multisystem disorder affecting the cardiovascular, connective tissue, and central nervous systems in 1 in 10 000 live births. Cardiovascular involvement is the most common cause of morbidity and mortality in patients with WS, and noninvasive and invasive procedures are common. ⋯ The goal of this review is to provide thoughtful, safe and effective management strategies for the peri-procedural care of patients with WS with careful consideration of hemodynamic impacts of anesthetic strategies. In addition, an expanded risk stratification system for anesthetic administration is provided.
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Congenital heart disease · Mar 2017
Cerebral tissue oxygenation index and lactate at 24 hours postoperative predict survival and neurodevelopmental outcome after neonatal cardiac surgery.
There are no well-established noninvasive biomarkers for identifying patients at risk for poor outcome after surgery for congenital heart disease. Few studies have assessed prognostic accuracy of cerebral tissue oxygenation index (cTOI) measured by near infrared spectroscopy (NIRS). ⋯ Cerebral TOI combined with lactate at 24 hours postoperative are accurate non-invasive predictive biomarkers of patient survival and neurodevelopmental outcome in neonates with CHD undergoing cardiac surgery.
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Congenital heart disease · Mar 2017
Appropriateness and diagnostic yield of inpatient pediatric echocardiograms.
Multiple reports have shown echocardiograms for certain indications are neither cost-effective nor of high diagnostic yield. Given the ease with which tests can be obtained at a tertiary academic children's hospital, our aims were to: (1) determine the diagnostic yield of inpatient studies by in-hospital location; (2) evaluate inpatient echocardiograms to determine indications and level of appropriateness; and (3) evaluate the frequency of cardiology involvement prior to those echocardiograms. ⋯ The diagnostic yield of inpatient, first-time pediatric echocardiograms is relatively low. The majority of studies that identified pathology were performed on patients located in higher acuity units. General floor echocardiograms for murmurs had a low diagnostic yield, raising the question of cardiology consultation versus direct echocardiogram ordering for subjective physical exam signs.