Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders is the Holt-Oram syndrome, which is characterized by septal defects of the heart and preaxial radial ray abnormalities. Its incidence is one in 100,000 live births. Approximately three out of four patients have some cardiac abnormality with common associations being either an atrial septal defect or ventricular septal defect. Herein, we report a rare sporadic case of Holt-Oram syndrome with atrial septal defect with symptoms of heart failure in a forty-five-year-old lady who underwent emergency cardiac surgery for the symptoms.
Triple differential cross sections (TDCS) are estimated for the ionization of metastable 3d-state hydrogen atoms by electron at 250 eV for various kinematic conditions pursuing a multiple scattering theory. The present new results are compared with the theoretical results of hydrogenic different metastable states as well as the hydrogenic ground state experimental data. Obtained new finding results are in good qualitative agreement with those of compared theories. The present results give an immense opportunity for experimental trial in the field of ionization problems.
The surgical management of d-transposition of great arteries (d-TGAs) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) is ever evolving and still remains a challenge because of wide anatomic variability, age of presentation, surgical options available, and their variable long-term results in different series. We describe a patient with d-TGA, VSD, and LVOTO who presented to us at 13 years of age and underwent an arterial switch operation along with neoaortic valve replacement with a mechanical prosthesis. The postoperative course was uneventful, and at hospital discharge, the echocardiogram was satisfactory. We present the pros and cons of this hitherto undescribed treatment option.
The purpose of this study was to assess the effective dose received by children during cardiac procedures. 6 children from 3 to 14 years old who underwent coronary angiogram, cardiac catheterization and pulmonary valvuloplasty treatment were included in the present study. Measurement of effective doses of the paediatric cardiac patients was performed in four catheterization laboratories in three hospitals. Harshaw TLD badges (TLD-100, LiF: Mg, Ti) were used on patients to measure effective dose in the catheterization laboratory. The TLD badges were calibrated from Secondary Standard Dosimetery Laboratory. It is found that the highest dose was 2.01 mSv in 5.4 minute fluoroscopic time during peripheral angiogram and primary pace maker procedure and the lowest effective dose was 0.044 mSv in 1.2 minute fluoroscopic time during coronary angiography performed in the same hospital. The highest fluoroscopic time (18:14 min) was taken to perform a cardiac catheterization and pulmonary valvuloplasty procedure and the dose was 0.781 mSv. During cardiac catheterization children were exposed to high levels of radiation but there was a variant in dosage. Careful consideration should be given to minimize dosage by practicing ALARA principle. This type of study may lead cardiologist and scientist to improve necessary safety measures to be taken to reduce the exposure to patients and occupational worker. DOI: http://dx.doi.org/10.3329/bjmp.v4i1.14704 Bangladesh Journal of Medical Physics Vol.4 No.1 2011 145-147
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