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

• Journal of Chest Surgery
• /
• v.33 no.6
• /
• pp.476-486
• /
• 2000

Background: Pulmonary atresia with intact ventricular septum(PA/IVS) is an anatomically heterogenous anomaly with a variety of surgical strategies possible. The purpose of the study is to evaluate the influence of right ventricular size on the early and midterm results of surgical repair of PA/IVS. Material and method: Medical records of 20 consecutive patients with PA/IVS operated on between January 1993 and August 1999 were retrospectively reviewed. There were 12 boys and 8 girls whose ages ranged from 2 days to 14.5 months (median 6 days). Their body weight ranged from 2.52kg to 9.35 kg(median 3.18kg). The preoperative Z-value of the diameter of the tricuspid valve(T-valve) was less than or -4 in 5 patients, between -4 and -2 in 1, between -2 and 0 in 7, between 0 and 2 in 6, and greater than or 2 in 1. All patients who had z-value of tricuspid valve greater than -2.05 were attempted biventricular repair(n=15) and all patients who had it smaller than -4.4 underwent systemic-pulmonary shunt operation only(n=3) or bidirectinal cavopulmonary shunt with right ventricular reconstruction(n=2). Result: Two early deaths(2/20, 10%) occurred. Both were infants who underwent transannular patch with shunt. One of these two had huge right ventricle(Z-value of tricuspid valve = 5). There were 2 late non-cardiac deaths 3 and 7 months after operations respectively. Follow-up was completed in all children at a mean of 35.3 months(range, 5 to 54 months). 10 of 11 survivors who underwent transannular patch or valvotomy with or without shunt procedure were in NYHA functional class I even though some of them had small interatrial communication or patent shunt. All three patients who had shunt procedure only at initial palliation completed Fontan procedures with no death. Two patients who underwent right ventricular outflow reconstruction with bidirectional cavopulmonary shunt were also in good condition. Conclusion: The transanular RVOT patch or valvotomy with or without systemic-pumonary shunt as an initial palliative procedure to achieve biventricular repair for the patients who had neither too small nor too large right ventricle(-2.05$\leq$Z-value of T-value of T-valve$\leq$2) could be performed at low operative risk(1/14 7.1%). Systemic-pulmonary shunt procedure and bidirectional cavopulmonary shunt procedure for the patients who had small right ventricle(Z-value of T-valve$\leq$4.4) could be also performed with low risk. But a patient with huge right atrium and ventricle(Z-value of t-valve=5) had poor operative result.

• Clinical and Experimental Pediatrics
• /
• v.46 no.3
• /
• pp.250-258
• /
• 2003

Purpose : The actual clinical examples of co-appliance of catheter intervention with surgical procedures in the treatment of pulmonary atresia with an intact ventricular septum(PA/IVS) which we have experienced in our institution are here shown, and the anatomical and hemodynamical profiles between each method is compared. Methods : Medical records of 33 patients with PA/IVS who underwent various treatment from January, 1995 to December, 2000 were reviewed for a retrograde study. Results : In three out of 10 patients who underwent percutaneous balloon pulmonary valvotomy (PPV), residual pulmonary stenosis were observed in their out patient department(OPD) follow-ups, eventually necessitatig balloon pulmonary valvuloplasty(BPV). One out of three patients exhibited deterioration of tricuspid regurgitation after BPV, requiring surgical tricuspid annuloplasty(TAP). Two out of the seven patients who received primarily surgical right ventricle outlet tract(RVOT) repair without any systemic-pulmonary shunt or intervention needed additional intervention employing cardiac catheterization after operation. Two patients received interventional catheterization before surgical RVOT repair. In five out of 11 cases of Fontan type operation, coil embolization of collateral circulation was done before total cavo-pulmonary connection(TCPC), and in three cases, interventional catheterization was needed after TCPC. Conclusion : Both medical and surgical treatment modalities are widely used in management of PA/IVS patients, and recent results prove that medico-surgical cooperative treatment is essential.

• Journal of Chest Surgery
• /
• v.29 no.8
• /
• pp.828-835
• /
• 1996

From January 1993 to April 1995, 27 neonates (under age of 30 days underwent open heart surgery in the Department of Thoracic and Cardiovascular Surgery, Dong-A Medical Center. Mean age and weight were 12.1 days(2days∼306ays) and 3.29 kg(2.6kg∼4.1 kg) respectively. Cardiac anomalies were simple complete transposition of great arteries(TGA) in 11 neonates, TGA with coarctation of aorta(COA) in 1 , total anomalous pulmonary venous connection(TAPVC) in 5, double inlet right ventricle with TAPVC in 1, interrupted aortic arch(IAA) with ventricular septal defect(VSD) in 3, pulmonary atresia(PA) with intact ventricular septum(IVS) in 3, pulmonary stenosis with IVS in 1, Taussig-Bing anomaly with IAA in 1, and hypoplastic left heart syndrome(HLHS) in 1 . Postoperative complications were myocardial and/or pulmonary edema which caused open sternum in 13 patients(54.2%), acute renal failure( RF) in 10(37.0%), Intractable low cardiac output syndrome (LCOS) including weaning failure from cardiopulmonary bypass in 7(25.9%), bronchopulmonary dysplasia in 1, wound infection in 1, and paroxysmal supraventricular tachycardia in 1. Nine of 13 patients with postoperative open sternum were recovered with delayed sternal closure, and seven of 10 patients survived postoperative ARF with peritoneal dialysis. There were 8 operative deaths(29.6%): 3 in the patients with simple complete TGA, 1 In TCA with COA, 1 in PA with IVS, 1 in Taussig-Bing anomaly with IAA, 1 in DIRV with TAPVC, and 1 in HLHS. One late death occurred after arterial switch operation in simple TGA. The mosts common cause of death was low cardiac output syndrome. Our initial experience of open heart surgery in neonates showed high operative mortality and morbidity, especially in complex anomalies.