1/19. The "surreptitious Staphylococcus": staphylococcus lugdunensis endocarditis in a child.A child with congenital heart disease developed infective endocarditis caused by staphylococcus lugdunensis. Despite an apparent excellent response to initial antibiotic treatment in clinical, inflammatory and echocardiographic indices, the patient's valve damage progressed silently and surgical intervention was required. This case highlights the potential for misidentification of S. lugdunensis, its usual susceptibility to penicillin and in particular the aggressive nature of endocarditis caused by this coagulase-negative staphylococcus. The epidemiology and treatment of endocarditis caused by this organism are reviewed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/19. Schinzel-Giedion syndrome: a further cause of West syndrome.Schinzel-Giedion syndrome (SGS) is a rare disorder with a likely autosomal recessive pattern of inheritance which is characterized by several facial dysmorphisms, midface hypoplasia, multiple skeletal anomalies including short and sclerotic skull base, short neck, and post-axial polydactyly. Cardiac and urogenital malformations are also present. Thirty-three cases have been described so far. We report on a boy affected by SGS in whom a long-term EEG follow-up showed a progressive deterioration of the background bioelectric activity ending, at the age of 19 months, with a hypsarrhythmic pattern clinically correlated with severe and refractory infantile spasms. EEG deterioration and neuroradiological findings, which showed progressive brain atrophy, confirm the neurodegenerative nature of SGS. We also re-evaluated all the published cases and found that 33% of patients with SGS experienced neonatal seizures and another 25% developed West syndrome in the following months. The seizures appeared extremely refractory to several anticonvulsive treatments. In conclusion, we believe that SGS should be included among the causes of secondary West syndrome.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/19. Positional cyanosis in infants: an unusual presentation of right-sided cardiac masses.Positional cyanosis is an uncommon finding in young patients. We report three infants who presented with positional cyanosis due to a pedunculated tumour in the right heart. Arterial desaturation was the result of right-to-left shunting at the level of the oval foramen caused by obstruction and/or insufficiency of the tricuspid valve. The obstruction at the level of the tricuspid valve was variable because of the pedunculated nature of the tumours, which gave them considerable mobility. Hence, the degree of right-to-left shunting was dependent on the position of the patient. In all the patients, surgical resection of the tumours resolved the cyanosis.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/19. Hypertrophic pulmonary osteoarthropathy: control of pain and symptoms with pamidronate.This case presents a patient with hypertrophic osteoarthropathy of the lower extremities that developed secondary to congenital cyanotic heart disease. The major clinical manifestation was severe bilateral leg pain. The pain that was debilitating in nature completely resolved following a single administration of 60 mg pamidronate. Hypertrophic osteoarthropathy (HOA) is an acquired, uncommon disorder of obscure etiology. It has been described mainly in association with chronic suppurative pulmonary diseases, bronchogenic carcinoma and lung metastases, cystic fibrosis, and cyanotic congenital malformations of the heart.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/19. The harlequin color change and association with prostaglandin E1.The harlequin color change is an unusual cutaneous phenomenon observed in newborn infants as transient, benign episodes of a sharply demarcated erythema on half of the infant, with simultaneous contralateral blanching. In this report, two newborns with congenital heart anomalies demonstrated the harlequin color change, one whose skin findings showed a course related to the dose of systemic prostaglandin E1, suggesting a possible association. The benign, self-limited nature of the color change mandates that prostaglandin E1 not be discontinued for this reason. The entity is likely more common than the paucity of reports in the world literature suggests, and all physicians should recognize its graphic appearance to avoid unnecessary exposure to agents in an effort to treat it.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/19. Isolated infundibuloarterial inversion.Isolated infundibuloarterial inversion is a rare conotruncal cardiac anomaly characterized by a leftward and posterior aortic valve in the face of ventriculoarterial concordance. It has previously been described only in cases associated with severe additional defects of substantial hemodynamic significance. We present a case of isolated infundibuloarterial inversion with a small, hemodynamically insignificant ventricular septal defect. The nature of this lesion, diagnostic methods of imaging, and features aiding its discrimination from the more commonly recognized anatomically corrected malposition of the great arteries are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/19. 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 = nature (Clic here for more details about this article) |
8/19. The cardio-facio-cutaneous (CFC) syndrome and noonan syndrome: are they the same?The distinction between the cardio-facio-cutaneous syndrome (CFC) and the Noonan syndrome (NS) has been based on the presence of a characteristic facies, abnormal hair and skin, and sporadic occurrence. However, all reports of the CFC syndrome comment on the similarity between it and NS, and its sporadic nature is now debatable. This report demonstrates the evolution of the clinical phenotype in a patient with the CFC syndrome and prompts us to question the validity of separating CFC from NS.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/19. Right ventricular failure: observations of the interrelationships affecting RV failure after aortocoronary bypass, valve surgery, and congenital heart defect repair.Right ventricular dysfunction in adult coronary artery bypass surgery with or without ventricular aneurysm repair has not been a source of hospital mortality in our institution, whereas it persists as a cause of death not infrequently after repair of congenital heart disease and, on occasion, after valvular surgery. Although we have strong clues as to the true nature of this dichotomy, there are so many factors involved with open congenital heart repairs that the explanation is not certain. Considering the coronary anatomy, the character of right ventricular infarction, the total lack of reports of post-operative angiography, and our clinical results, we have not found sufficient evidence at this time to recommend bypass to right ventricular coronary branches. We believe that the use of a checklist of right ventricular dysfunction after congenital heart repair may result in detection of a reversible cause of right ventricular dysfunction that could be fatal.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/19. Visceral larva migrans involving the myocardium: report of two cases and review of literature.Two cases of asymptomatic visceral larva migrans (VLM) involving the myocardium, discovered during open heart surgery, are reported. Only 7 cases of VLM myocarditis were found in recent English publications. All 7 cases involved children, with most of the patients acutely ill. Our 2 asymptomatic cases, coupled with the high rate of positive serologic tests in the general population, stress the widespread nature of toxocara canis infection in human and the large number of subclinical, undetected cases.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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