• Clinical and Experimental Pediatrics
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• v.46 no.5
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• pp.484-489
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• 2003

Purpose : To understand morphologic and hemodynamic variations in patients with pulmonary atresia with intact ventricular septum(PA/IVS), and to decide the best treatment modalities, we measured right ventricular volume, inflow, and outflow valvular annulus size in these patients and compared them with those of normal newborns. Methods : Eight patients with PA/IVS diagnosed by echocardiography from January to December 2001 were enrolled in this study. Among the total eight patients, five were male and three were female. The mean age of patients was 6.9 days(1-34 days), and the mean body weight was 3,343 gm (2,970-4,000 gm). Ten fullterm newborn infants with sepsis or hyperbilirubinemia without heart disease were enrolled as a control group. Echocardiographic and Doppler studies using Acuson Aspen (7Mh probe) were recorded on super-VHS videotape and later on, with review mode. We measured volumes of right and left ventricles, aortic, pulmonic, mitral and tricuspid valvular annulus sizes using an installed program, and then these parameters were compared with those of the control group. Results : Mean Z-value of tricuspid valvular annulus in PA/IVS was $-3.69{\pm}2.80$(-8.4--0.45), and tricuspid/mitral valvular annulus size ratio $0.68{\pm}0.15$(0.43--0.84). The more the tricuspid/mitral valvular annulus size ratio, the more Z-value of tricuspid valvular annulus(P=0.003, r=0.885). Those patients who underwent pulmonary valvuloplasty(balloon or surgical) had a tendency toward larger volume of the right ventricle, more Z-value of pulmonic and tricuspid valvular annulus, and more tricuspid/mitral valvular annulus size ratio than those patients who underwent a shunt operation. Conclusion : Compared to a measurement of the volume of the right ventricle, measurements of tricuspid/mitral valvular annulus size ratio and Z-value of tricuspid valvular annulus may be easier and better parameters to decide the treatment method and to predict prognosis in PA/IVS patients.