We present a synthesis of 95 published investigations of the exceedingly rare tunnels that can exist between the aortic root and the left or right ventricles. From the 220 suitable cases included in these investigations, we reviewed the clinical presentations, modalities used for diagnosis, surgical approaches, and outcomes. Diagnostic information was provided by clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, magnetic resonance imaging, cardiac catheterization, and angiocardiography. These techniques elucidated the coronary arterial origins and associated defects and defined the disease before surgery. Patients occasionally present with an asymptomatic cardiac murmur and cardiomegaly, but most suffer cardiac failure in the first year of life when the tunnel enters the left ventricle. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks of gestation. Associated defects, involving the proximal coronary arteries or the aortic or pulmonary valves, are present in nearly half the cases. Prompt diagnosis and surgical repair are important for a favorable outcome. Overall, operative mortality has been cited to be between 3% and 8.3%. Associated congenital coronary arterial anomalies, residual severe aortic stenosis, poor left ventricular function, and rupture of an infected suture line have been the reported causes of death. Despite early surgical intervention, an incidence of 16% to 60% postoperative residual aortic regurgitation of varying severity has been reported. The requirement of further repair or replacement of the aortic valve ranges from 0% to 50%. We submit that an increased appreciation of these details relative to the tunnels will contribute to improved surgical management.

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Introduction: Role of transcranial Doppler in prevention of stroke in sickle cell children has been well appreciated. Studies are being done to develop the protocol in children. Since we don't find stroke very commonly in this part of the world, this study was done in order to see the prevalence of abnormal flow velocity in sickle children attending sickle cell clinic. The aims of this study were to measure mean flow velocity in different vessels in homozygous sickle cell patients using transcranial Doppler study, to compare the mean velocity in sickle children with age and sex matched controls and to correlate mean velocity with headache or stroke if any and also to correlate mean velocity with number of transfusions. Materials and Methods: The study was done in Paediatric wards. It was a prospective crosssectional comparative study. Twenty six children below 14yrs of age with homozygous sickle cell disease attending the Sickle cell clinic were selected as the cases. Forty cases of similar age and sex were recruited as normal control group. Transcranial Doppler was done in six different vessels in both the groups and mean flow velocity was measured. Mean flow velocity was correlated with symptoms and number of transfusions. Velocity was classified as normal (<170cm/sec), conditional (170-199cm/sec) or abnormal (>200cm/sec). Statistical analysis was done using SPSS 10 software. Results: In normal age and sex matched controls mean blood flow velocity was 50cm/sec where as in the cases of sickle cell disease was 180cm/sec. Maximum mean velocity was observed in middle and posterior cerebral artery. In two Sickle cell cases (8%) blood flow velocity was abnormal, these children had headache though received 5-10 transfusions/year. In only 4% sickle cell children flow velocity was normal and rest had conditional velocity. Among these children 39% received less than 5 and rest received 5-9 transfusions /yr and had no symptoms of stroke. Conclusions: Flow velocity measured by Transcranial Doppler is highest in middle cerebral artery and Posterior cerebral artery which appear to be the best arteries for this test in this region. Flow velocity was significantly high in children with sickle cell disease as compared to normal children. Prevalence of abnormal flow velocity in our children was 8% and children with abnormal mean flow velocity presented with headache.

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