1/7. 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 = physician (Clic here for more details about this article) |
2/7. prenatal diagnosis of Shone's syndrome: parental counseling and clinical outcome.OBJECTIVE: To describe a series of fetuses diagnosed as having Shone's syndrome, which includes four cardiac defects and for which there is a wide variety of clinical presentations, surgical treatments and outcomes, and to discuss the counseling strategy. methods: We reviewed retrospectively the records of four babies who were suspected prenatally to have Shone's syndrome. RESULTS: The mean age at diagnosis was 27.5 (range, 22-35) weeks. A small left ventricle, mitral and aortic annulus and ascending aorta were detected in three cases. In three fetuses there was an abnormal mitral valve apparatus and in two fetuses a sub-aortic membrane was detected. Coarctation of the aorta was an impending diagnosis in three babies. Following counseling, all parents decided to continue the pregnancy to term. Echocardiographic evaluation was performed soon after birth. The mean mitral valve annulus diameter was 8.2 (range, 7-10) mm, and that of the aortic valve annulus was 6 (range, 5-7) mm. The aortic valve was bicuspid in all babies with an additional sub-aortic membrane in two babies. Doppler ultrasound examination revealed high-velocity flow through the mitral valve in three babies; two of them had a parachute mitral valve. Coarctation repair was performed in two babies during the first week of life. One patient underwent mitral valvuloplasty followed by later mitral valve replacement. All patients were alive after a mean of 7.8 (range, 3.3-10.5) years' follow-up. CONCLUSION: When counseling families regarding fetal Shone's syndrome, it is important to emphasize the wide variety of clinical presentations and possible outcomes. Differentiation between hypoplastic left ventricle and Shone's complex is crucial and may influence the physician's attitude, the presentation of the case to the family and the family's final decision.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
3/7. scoliosis in CHARGE: a prospective survey and two case reports.charge syndrome was first identified as a cluster of congenital anomalies in 1979 and has since undergone diagnostic criteria modifications to include the major and minor characteristics that occur during infancy and childhood. As the individuals with charge syndrome have aged into their adolescents and adulthood, it has become increasingly common for them to develop scoliosis. This article presents an older population of individuals with charge syndrome and describes the prevalence of scoliosis, and identifiable risk factors for scoliosis. Two case reports demonstrate the variability of scoliosis in charge syndrome. A survey of adults and adolescents with charge syndrome was completed to collect information about late onset medical issues, and those identifying scoliosis as an issue, were further followed for more information. The total population (n=31) and then the subgroup of individuals with scoliosis (n=19) were analyzed. Sixty one percent (19 of 31) of this population was diagnosed with scoliosis. The age of charge syndrome diagnosis was later in the scoliosis population (6.3 years compared to 3.7 years in the no scoliosis population). growth hormone use was reported in 7 of 31 of the individuals; 6 of these subsequently were diagnosed with scoliosis (32% of the scoliosis group). Of the scoliosis subgroup, most were mild scoliosis but eight were diagnosed with moderate to severe scoliosis, and all of these were treated with either a brace (n=5) or with surgical fusion (n=2) and one individual had both. scoliosis in charge syndrome individuals is more common than previously reported, and the age of onset is earlier than when routine monitoring for scoliosis is recommended. The prevalence of scoliosis in the charge syndrome population is higher than in the general population therefore, it is very important for physicians to carefully monitor the spine for the development of scoliosis in children with charge syndrome, especially if they are being treated with growth hormone.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
4/7. Evaluation and management of supraventricular tachycardia in children.Emergency physicians may be called on to resuscitate acute complications in pediatric patients with congenital heart disease. Supraventricular tachycardia, with or without hemodynamic decompensation, is one of the most serious complications. We present the case of a 22-month-old boy with a history of single ventricle who presented to our institution with a history of syncope and hemodynamically stable supraventricular tachycardia. Initial attempts at pharmacologic conversion with propranolol and verapamil failed. The arrhythmia was terminated in response to an IV fluid bolus and dopamine infusion and probably resulted from a combination of anemia, hypovolemia, and impaired contractility. Appropriate evaluation and management relating to the cre of acute supraventricular tachycardia in children are discussed.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
5/7. association of ocular, cervical, and cardiac malformations.association of ocular, cervical, and cardiac anomalies is discussed. A case with severe congenital heart disease, unusual Duane's retraction syndrome, classified here as atypical typical, and Klippel-Feil anomaly is described. To alert the physicians to such an association of congenital malformations and for nosological purposes this entity may be called oculo-cervico-cardiac syndrome. Importance of the association of heart and eye abnormalities is stressed and syndromes with associated ocular and cardiac anomalies are briefly discussed.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
6/7. Cardiovascular evaluation of the young athlete.heart murmurs and cardiovascular defects, elevated blood pressure, and arrhythmias cause considerable concern to many physicians evaluating or certifying students for competitive athletic participation. The purpose of this article is to present a practical approach to the evaluation and management of these problems.- - - - - - - - - - ranking = 1keywords = physician (Clic here for more details about this article) |
7/7. adult patients with congenital heart disease.adult patients with congenital heart disease present complex problems that require individualized management. Unfortunately, few physicians who care for adults with congenital heart disease have received adequate specific training to deal with their problems. Pediatric cardiologists who are familiar with congenital heart disease often refer patients to adult cardiologists after adolescence. And adult cardiologists have not traditionally had much training or experience in dealing with congenital heart disease. The cardiac needs of these patients are often best managed through the collaborative efforts of adult and pediatric cardiologists, cardiac surgeons, specialized nurse clinicians, and social workers. Of course, it is crucial to their optimal care that there be involvement of a primary care physician who can help with their many medical, surgical, and psychosocial problems.- - - - - - - - - - ranking = 2keywords = physician (Clic here for more details about this article) |