Pediatric cardiology
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Pediatric cardiology · Jan 2006
Case ReportsCoincidence of long QT syndrome and propionic acidemia.
Propionic acidemia and long QT syndrome (LQTS) are rare disorders. In addition, both conditions are potentially lethal. ⋯ Although the patient was asymptomatic and arrhythmia free, we started her on atenolol. This is the first report of the association between LQTS and propionic acidemia.
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To investigate the origin of the pulmonary systolic ejection innocent flow murmur (IFM), echocardiographic examinations were undertaken in 30 children with IFM and in a control group consisting of 28 healthy children without murmur. Compared to the controls, the diameters of the left ventricular outflow tract (LVOT) and aortic valve annulus and aortic valve area tended to be smaller, whereas stroke volume (SV) and cardiac output were slightly greater in children with IFM, but they were not statistically significant. Mean fractional shortening was significantly higher in children with IFM. ⋯ The variables of left-sided flow velocities in the same individuals with IFM were significantly higher compared to those derived from the right heart. The ratios of the SV to the LVOT diameter and to the aortic valve area were found to be significantly greater. It was concluded that IFM originates from higher blood flow velocities in the region of LVOT and aortic valve annulus, and that the increased flow velocity results from the larger SV passing through the relatively narrow LVOT and aortic valve in children with IFM.