1/15. Striae and acne following cardiac surgery in a child.We report a 13-year-old girl with extensive striae and an acneiform eruption following surgery for complex congenital heart disease. These findings were associated with elevated serum and urinary cortisol levels with loss of diurnal rhythm. The resolution of the eruption and the fading of her striae coincided in time with normalization of her blood parameters on day 72 postoperatively. We conclude that the cause of steroid excess in our patient was stress induced by the cardiac surgery and a complicated and protracted postoperative course. To our knowledge, this is the first report in the English language literature of skin changes due to endogenous hypercortisolaemia caused by intense physical and emotional stress.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
2/15. Pulmonary vein atresia with Shone's anomaly in an infant: a case report.We report a case of individual pulmonary vein atresia associated with multiple levels of left heart obstruction, including aortic coarctation, valvular aortic stenosis, and parachute mitral valves with stenosis. The diagnosis of pulmonary vein obstruction is likely to be missed in patients who also have other left heart obstructive diseases, since the latter usually dominates the clinical presentation. We diagnosed the existence of individual pulmonary vein atresia preoperatively via cardiac catheterization. The pulmonary artery angiograms revealed back and forth motion of the dye with no visualization of either a capillary or venous phase on the lesion side. The pulmonary capillary wedge pressure was unevenly elevated and highest on the lesion side. The results were later confirmed by operation and autopsy. Thus, selective pulmonary artery catheterization and angiography remains a good diagnostic tool to rule out the existence of pulmonary vein obstruction in cases which have multiple levels of left heart obstruction.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
3/15. cor triatriatum: echocardiograhic findings.The echocardiographic findings in a patient with cor triatriatum are presented. The preoperative echocardiogram demonstrated an unusual structure behind the mitral valve that moved anteriorly during atrial systole. There was also echocardiographic evidence of abnormalities of mitral valve motion, right ventricular and left atrial enlargement, and pulmonary hypertension. Postoperatively, the unusual echo behind the mitral valve had disappeared and the mitral valve appeared more normal. Left atrial and right ventricular dimension had decreased and the pulmonary valve appeared more normal. Fine diastolic mitral valve fluttering, an abnormal echo behind the anterior mitral valve leaflet moving anteriorly with atrial systole, an abnormal pulmonary valve echogram suggesting pulmonary hypertension, and marked right ventricular and left atrial enlargement were the main echocardiographic findings in our patient.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
4/15. prenatal diagnosis of transposition-like double-outlet right ventricle with mitral valve atresia in heterotaxy syndrome.We present a case of heterotaxy syndrome with right isomerism detected in a twin fetus, with the sonographic findings that established this diagnosis. At 24 weeks' menstrual age, ultrasound detected anomalies in the heart suggestive of heterotaxy syndrome. Intrauterine fetal echocardiography demonstrated that the heart of the affected twin was located on the left side with a normal cardiac axis. The right ventricle was much larger than the left ventricle. The appearance of the mitral valve was dysplastic with restricted motion. The ascending aorta exited from the right ventricle, anterior to, right to, and parallel to the smaller pulmonary trunk, which also arose from the right ventricle. Double-outlet right ventricle (DORV) was clearly demonstrated. cesarean section was performed at 38 weeks' menstrual age. Both twins were healthy and survived. Postnatal echocardiography and MRI confirmed the prenatal findings. At the age of 4 months, the affected infant had no evidence of cardiac decompensation. This report shows that conventional ultrasound can be used to detect heterotaxy syndrome and that intrauterine fetal echocardiography can delineate the cardiac complex in heterotaxy syndrome. Postnatal surgery with complex Fontan procedures was planned for the affected twin.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
5/15. dexmedetomidine sedation in a pediatric cardiac patient scheduled for MRI.PURPOSE: To describe the use of dexmedetomidine for sedation in a critically ill infant undergoing magnetic resonance imaging (MRI). CLINICAL FEATURES: A nine-month-old 5.1 kg infant was to have an MRI study of the thorax. The infant had multiple congenital cardiac anomalies which had been partially corrected surgically. After administration of atropine, 0.1 mg iv, a loading dose of dexmedetomidine (1 microg.kg(-1) iv) was administered over ten minutes followed by a continuous infusion of 0.5 microg.kg(-1).hr(-1) for maintenance. propofol 5 mg iv were administered after the loading dose of dexemedetomidine to produce somnolence. Anesthetic conditions for performing the MRI were excellent. The infant remained motionless, breathing spontaneously. hemodynamics remained stable throughout the procedure. Recovery was rapid and uneventful. CONCLUSION: dexmedetomidine and a small dose of propofol were used successfully to sedate a critically ill infant for MRI. More studies are required to determine the role of this unique drug in the pediatric population.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
6/15. Abnormal sympathetic innervation of the heart in a patient with Emery-Dreifuss muscular dystrophy.A 33-year-old man was admitted for general malaise and vomiting. An electrocardiogram showed a complete atrioventricular block and an echocardiogram showed right atrial dilatation and normal wall motion of left ventricle (LV). Gene analysis showed nonsense mutation in the STA gene, which codes for emerin, and Emery-Dreifuss muscular dystrophy was diagnosed. An endomyocardial biopsy of right ventricle showed mild hypertrophy of myocytes. Myocardial scintigraphic studies with Tc-99m methoxyisobutylisonitrile (MIBI) and I-123-betamethyl-p-iodophenylpentadecanoic acid (BMIPP) scintigrams showed no abnormalities. In contrast, I-123 metaiodobenzylguanidine (MIBG) scintigrams showed a diffuse and severe decrease in accumulation of MIBG in the heart. Six months later, his LV wall motion on echocardiograms developed diffuse hypokinesis. These results suggest that the abnormality on I-123 MIBG myocardial scintigrams may predict LV dysfunction in Emery-Dreifuss muscular dystrophy.- - - - - - - - - - ranking = 2keywords = motion (Clic here for more details about this article) |
7/15. Transthoracic real-time three-dimensional echocardiography in the diagnosis and description of noncompaction of ventricular myocardium.BACKGROUND: Numerous modalities have been used to diagnose and characterize noncompaction of ventricular myocardium (NCVM) including magnetic resonance imaging, two-dimensional echocardiography (2DE), contrast-enhanced 2DE, and angiography. The current case series examines the use of real-time three-dimensional transthoracic echocardiography (RT-3DE) in four such cases of NCVM. methods: From December 2003 to March 2004, we performed RT-3DE using a Philips Sonos 7500 echocardiographic scanner equipped with a 2-4 MHz 3D matrix array transthoracic probe, to evaluate four patients with NCVM. The real-time 3D transthoracic probe allows for dataset acquisition in an ultrasound wedge, which can be manipulated instantaneously. In addition, complete 3D volume rendering is acquired, allowing for volumetric imaging. RESULTS: The age range of the patients was 2 months to 42 years. One patient had the codiagnoses of coarctation of the aorta and bicuspid aortic valve. In all four patients, RT-3DE enabled diagnosis and provided detailed characterization of the affected myocardium. Entire trabecular projections and intertrabecular recesses were easily visualized simultaneously, and endocardial borders were clearly demarcated. Wall motion abnormalities of the affected myocardium were clearly visualized. The compacted and noncompacted portions of the myocardium could be differentiated well. CONCLUSIONS: Our study provides preliminary data highlighting the utility and feasibility of RT-3DE in the clinical characterization of NCVM. The complex 3D nature of this disorder and the endocardial hypertrabeculation were more readily visualized than with 2DE.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
8/15. Abnormal systolic atrioventricular flow related to incoordinate motion of the ventricular wall after the Fontan operation.The development of abnormal flow has been described in patients with incoordinate motion of the ventricular wall. In all cases so far documented, these abnormal patterns of flow have been restricted to the ventricular cavity, and the atrioventricular valve remained closed. We identified a patient after a Fontan operation in whom the degree of ventricular mural incoordination was of such severity that the atrioventricular valve remained open and atrioventricular flow occurred for 140 msec after the onset of QRS complex. As far as we know, this phenomenon has not previously been described.- - - - - - - - - - ranking = 5keywords = motion (Clic here for more details about this article) |
9/15. Isotopic findings in anomalous origin of the left coronary artery from the pulmonary artery: report of an adult case.Anomalous origin of the left coronary artery from the main pulmonary trunk results in myocardial ischemia or infarction, and may be a cause of death in the first months of life. Some patients, however, develop satisfactory coronary collateral circulation and remain asymptomatic into adulthood. In these patients, myocardial perfusion and left ventricular function are not well understood. We report the case of a 17-yr-old female patient, suffering from anomalous origin of the left coronary artery from the main pulmonary trunk, who underwent reimplantation of the left coronary artery to the aorta. The preoperative permanent 201Tl defect of the left antero-lateral ventricular wall and the abnormal regional wall motion induced by stress exercise testing were fully reversed after the operation.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
10/15. Ethical dilemmas in fetal echocardiography.Recent technology permits detection of congenital heart diseases as early as the 17th or 18th week of gestation. These detectable defects include hypoplastic left heart syndrome, ebstein anomaly, and atrioventricular canal malformation. Surgical treatments for these problems raise ethical as well as medical questions regarding patients whose prenatal diagnosis reveals congenital heart disease. We report six cases of in utero diagnosis of hypoplastic left heart syndrome and other complex congenital heart anomalies. Empirical factors relevant to our management were gestational age, severity of the anomaly, degree of certainty of diagnosis and prognosis, possibilities for treatment, and parental wishes regarding the fetus or newborn. Each of these factors was examined in the context of the reported cases and correlated with ethical principles applicable to obstetrics and neonatology: 1) respect for parental autonomy, 2) promotion of the infant's welfare, and 3) fairness in distributing scarce medical resources. We maintain that the first two principles are paramount in ethical decisions regarding individual patients, while the third is foremost in policy decisions affecting others as well.- - - - - - - - - - ranking = 1keywords = motion (Clic here for more details about this article) |
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