Sickle cell disease and ventricular myocardial strain: A systematic review.

Cardiac disease is the primary cause of death in sickle cell disease (SCD). Cardiac abnormalities begin in childhood and progress throughout life. Right and left ventricular (RV, LV) myocardial strain are early markers of systolic dysfunction but are not well investigated among individuals with SCD. The objectives of this review were to (1) identify all published studies that have evaluated ventricular myocardial strain, (2) summarize their values, and (3) compare findings with those obtained from controls. From search results of four electronic databases-Medline, Embase, Scopus, and Web of Science-42 potential articles were identified, of which 18 articles and 17 studies met eligibility criteria for inclusion. The evaluated studies demonstrate that RV and LV myocardial strain are generally abnormal in individuals with SCD compared with controls, despite having normal ejection/shortening fraction. Myocardial strain has been inconsistently evaluated in this population and should be considered any time an echocardiogram is performed.

Ventricular global longitudinal strain is altered in children with sickle cell disease.

Cardiac disease is the primary cause of death in sickle cell disease (SCD). Right and left ventricular global longitudinal strain (RVGLS, LVGLS) are early markers of systolic dysfunction but are not well investigated among children with SCD. One hundred and forty-three patients with HbSS or HbSß0 -thalassaemia (median age 11 years, range 5-19 years) and 71 controls matched for age and sex were compared. RVGLS and LVGLS were measured and compared with conventional measures of echocardiography and markers of haemolysis and inflammation. RVGLS was higher in children with SCD than in controls (-25·72% ± 3·45% vs. -24·54% ± 2·41%, P = 0·005); LVGLS was not different. RVGLS decreased with older age in children with SCD (ρ = 0·338, P < 0·001) but not among controls. Decreased RVGLS was associated with increased left atrial end diastolic volume (ρ = 0·181, P = 0·04); RVGLS increased with cardiac output (r = -0·279, P = 0·01). RVGLS and LVGLS were not associated with disease-modifying therapies, degree of anaemia or haemolysis markers. Elevated RVGLS may indicate an early RV compensatory mechanism in response to upstream myocardial insults and elevated cardiac output. Global longitudinal strain may serve as an early marker of altered myocardial function in children with SCD.

Infective endocarditis prophylaxis: current practice trend among paediatric cardiologists: are we following the 2007 guidelines?

BACKGROUND: In 2007, the American Heart Association modified the infective endocarditis prophylaxis guidelines by limiting the use of antibiotics in patients with cardiac conditions associated with the highest risk of adverse outcomes after infective endocarditis. Our objective was to evaluate current practice for infective endocarditis prophylaxis among paediatric cardiologists. METHODS: A web-based survey focussing on current practice, describing the use of antibiotics for infective endocarditis prophylaxis in various congenital and acquired heart diseases, was distributed via e-mail to paediatric cardiologists. The survey was kept anonymous and was distributed twice. RESULTS: Data from 253 participants were analysed. Most paediatric cardiologists discontinued infective endocarditis prophylaxis in patients with simple lesions such as small ventricular septal defect, patent ductus arteriosus, and bicuspid aortic valve without stenosis or regurgitation; however, significant disagreement persists in prescribing infective endocarditis prophylaxis in certain conditions such as rheumatic heart disease, Fontan palliation without fenestration, and the Ross procedure. Use of antibiotic prophylaxis in certain selected conditions for which infective endocarditis prophylaxis has been indicated as per the current guidelines varies from 44 to 83%. Only 44% follow the current guidelines exclusively, and 34% regularly discuss the importance of oral hygiene with their patients at risk for infective endocarditis. CONCLUSION: Significant heterogeneity still persists in recommending infective endocarditis prophylaxis for several cardiac lesions among paediatric cardiologists. More than half of the participants (56%) do not follow the current guidelines exclusively in their practice. Counselling for optimal oral health in patients at risk for infective endocarditis needs to be optimised in the current practice.

Moya Moya cases treated with encephaloduroarteriosynangiosis.

OBJECTIVE: To study neurological outcome of Moya Moya disease treated surgically with Encephaloduroarteriosynengopsis (EDAS). DESIGN: Prospective observational study. SETTINGS: Community and General with tertiary care facility. SUBJECTS: Eight children diagnosed with Moya Moya disease by Magnetic Resonance Angiogramover 4 years of period were selected for EDAS. Children who were not able to sustain surgery excluded from study. METHODS: Treatment modality selected were surgery in form of EDAS. After surgery subjects were followed up for minimum of 2 year period to know neurological out come. Outcome was reported as poor, fair, good and excellent. No statistical analysis performed due to small sample size. RESULTS: After surgery no episode of stroke or TIA was observed in any patient during 2 year follow up period and all patients are living without any new neurological deficit. CONCLUSION: Long term outcome of EDAS is promising.

Teenage heart murmurs.

The first-time appearance of a murmur in an adolescent can create a substantial amount of anxiety in the parents and the teenager. The appropriate evaluation and diagnosis is very important in decision-making regarding sports participation in this population. Accurate identification of the innocent murmurs can obviate the need for echocardiography. Identification of a pathologic murmur may reduce morbidity and, possibly, mortality in critical lesions such as hypertrophic cardiomyopathy. This article discusses the physiology and characteristics of different murmurs, and outlines an approach to cardiac murmurs in adolescents.