Heterotaxy is the failure of the developing embryo to establish normal left-right asymmetry, which is often associated with multiple malformations. Previous studies have identified different mutations in the cytoplasmic tail of the connexin 43 (cx 43) gene in six patients from a series of six sporadic cases with defects of laterality and severe heart malformations. These cases showed that of the genes involved in lateralization defects with autosomal recessive transmission, cx 43 was the most important.
View Article and Find Full Text PDFA ten-year-old girl who presented with a continuous murmur was diagnosed with a right coronary to right ventricular fistula with colored Doppler echocardiography and selective arteriography. She underwent traditional treatment-ligation of the fistula by sternotomy. Afterwards, the systolic component of the murmur persisted and repeat arteriography showed a residual shunt through the fistula, with no change in the diameter of the right coronary artery.
View Article and Find Full Text PDFTranscatheter occlusion of persistent patent ductus arteriosus (PDA) was attempted in 32 patients (22 female and 10 male, mean age 5.12 +/- 3.98 years, range 9 months to 19.
View Article and Find Full Text PDFA 3-year-old girl with a continuous murmur on auscultation was found to have left coronary artery to right atrial fistula simulating cor triatriatum sinister on echocardiography. Coronary angiography of the patient was also insufficient to clarify the exact pathologic anatomy. Magnetic resonance imaging showed that the main drainage of the aneurysmal coronary artery was to the left superior vena cava, resulting in a diffuse arteriovenous fistula in the lower lobe of the left lung by compressing its pulmonary vein.
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