ABSTRACT
Pulmonary atresia with intact ventricular septum(PA/IVS) is one of the complicated cyanotic congenital heart diseases with high mortality,which needs early intervention after birth.With further understanding of the disease and the development of surgery,the operative plan of the disease is gradually changing and some fundamental changes have taken place.The hemodynamic awareness of the disease is an important factor in the selection of surgery and the improvement of prognosis.In addition,it is also important for the selection of surgery and the improvement of prognosis to evaluate the development degree of the right ventricle comprehensively and accurately.At present,it is generally believed that the most effective treatment strategy can be obtained by classifying the development degree of the right ventricle in morphology.The surgical treatment has developed from early surgical pulmonary bypass to the interventional and hybrid surgery,which greatly improves prognosis and reduces surgical trauma.Among them,the pereutaneous pulmonary valve radiofrequency perforation plus balloon dilation and PDA stent implantation can be used as the preferred treatment for PA/IVS with dysplasia of the right ventricle.Particularly,PA/IVS can be cured with the interventional surgery alone for children with mild or even moderate dysplasia of the right ventricle.Hybrid surgery that significantly reduces trauma compared with traditional surgery is also an important choice for the areas where interventional surgery is relatively outdated.This article reviews and summarizes the progress of recognition and surgical treatment of PA/IVS,in order to provide reference for the clinical diagnosis and treatment.
ABSTRACT
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+/-2.80(-8.4--0.45), and tricuspid/mitral valvular annulus size ratio 0.68+/-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.