• Journal of Chest Surgery
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• v.19 no.3
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• pp.464-469
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• 1986

Pulmonary atresia with intact ventricular septum, and with VSD were uncommon congenital anomalies with high mortality in the neonatal period. Those survivals depend on an adequate interatrial communication or interventricular communication and pulmonary flow via large aortopulmonary collateral including PDA. Recently we experienced surgical correction of 2 cases pulmonary atresia with intact ventricular septum and with VSD. On case 1, 10-years old male patient was confirmed as pulmonary valvular membranous atresia with intact ventricular septum combined with large functioning PFO and mild tricuspid incompetence. So we performed total correction under the E.C.C, that was PDA ligation, RVOT reconstruction with monocusp valved outflow patch [16mm], repair of tricuspid insufficiency and closure of PFO. Post-operative hemodynamic result was good and there was no event during hospital course. On case 2, 16-years old female patient was diagnosed as pulmonary atresia with VSD and PDA. MPA was absent, remained fibrous cord like remnant and type of VSD was subaortic defect [3cm by 3cm in the size]. PDA was located at the usual site. Under the E.C.C. VSD patch closure through the right ventriculotomy, anastomosis between the right ventricular outflow tract and the pulmonary bifurcated site with the extra-cardiac Hancock valved conduit [22cm] and PDA ligation were performed.

• Clinical and Experimental Pediatrics
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• v.61 no.7
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• pp.210-216
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• 2018

Purpose: The present study aimed to evaluate progression and prognosis according to the palliation method used in neonates and early infants aged 3 months or younger who were diagnosed with pulmonary atresia with ventricular septal defect (PA VSD) or tetralogy of Fallot (TOF) with severe pulmonary stenosis (PS) in a single tertiary hospital over a period of 12 years. Methods: Twenty with PA VSD and 9 with TOF and severe PS needed initial palliation. Reintervention after initial palliation, complete repair, and progress were reviewed retrospectively. Results: Among 29 patients, 14 patients underwent right ventricle to pulmonary artery (RV-PA) connection, 11 palliative BT shunt, 2 central shunt, and 2 ductal stent insertion. Median age at the initial palliation was 13 days (1-98 days). Additional procedure for pulmonary blood flow was required in 5 patients; 4 additional BT shunt operations and 1 RV-PA connection. There were 2 early deaths among patients with RV-PA connection, one from RV failure and the other from severe infection. Finally, 25 patients (86%) had a complete repair. Median age of total correction was 12 months (range, 2-31 months). At last follow-up, 2 patients had required reintervention after total correction; 1 conduit replacement and 1 right ventricular outflow tract (RVOT) patch enlargements. Conclusion: For initial palliation of patients with PA VSD or TOF with severe PS, not only shunt operation but also RV-PA connection approach can provide an acceptable outcome. To select the most proper surgical strategy, we recommend thorough evaluation of cardiac anomalies such as RVOT and PA morphologies and consideration of the patient's condition.

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

• Journal of Chest Surgery
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• v.42 no.1
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• pp.87-91
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• 2009

The surgical management of complete transposition of the great arteries, ventricular septal defect, and pulmonary stenosis still remain a significant challenge. The Rastelli (REV procedure) remains the most widely applied procedure for surgical repair of these lesions. Although the Rastelli procedure can be performed with good early results, the intermediate- and long-term results have been less than satisfactory because of deterioration of the hemodynamic performance of the LVOT or RVOT. We performed a modified Nikaidoh procedure as an alternative surgical procedure in a 19-month-old boy weighing 10.4 kg with this anomaly. Aortic translocation with biventricular outflow tract reconstruction resulted in a more "normal" anatomic repair and postoperative echocardiography showed straight, direct, and unobstructed ventricular outflow.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.270-274
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• 1997

In recent years, the use of allograft conduits in repair of congenital cardiac disease is widely accepted. However, the supply of homograft. is currently limiting their increased clinical application, especially small cryopreserved homografts for use in neonates and inf'ants. We used a technique to surgically reduce the size of the more readily available large-diameter allografts, making them suitable for right ventricular outflow tract reconstruction in small infants and children. From December 1994 to March 1996, a total of 11 patients ranging in age from 10 months to 6 years (mean age, 27.3 months) and ranging in weight from 5.6 to 18.5 kg (mean 11.5 kg) underwent reconstruction of the right ventricular outflow tract using this surgical technique (pulmo ary atresia with ventricular septal defect, 9 cases ; tetralogy of Falloff, 2 cases). The diameter after downsizing ranged from 14 to 19 mm with a mean of 16.8 mm. There was one operative death due to rupture of the infected homograft. Evaluation of these patients between 2 and 15 months (mean 6.9 months) after homograft implantation reveals excellent clinical and echocardiographic results. There were no significant homograft insufficiency and RVOT obstructions. Although a longer follow-up is certainly required to evaluate the long term fate of the surgically modified bicuspid homografts, we believe that this technique may represent a valuable therapeutic alternative, at least in the short term, to the use of synthetic grafts when an appropriately sized homograft is not available.

• Journal of Chest Surgery
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• v.24 no.7
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• pp.674-684
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• 1991

The surgical treatment of tetralogy of Fallot [TOF] was initiated by Blalock and Taussig in 1945 with the establishment of the subclavian artery to pulmonary artery anastomosis. In an imaginative and daring effort, in 1954, Lillehei and collaborators [1955] using controlled cross-circulation, carried out the first intracardiac repair of TOF by closing the ventricular septal defect [VSD] and relieving the pulmonary stenosis under direct vision. Nowadays, total correction is the ideal operation for treatment of TOF and is accomplished with extracorporeal circulation. And the results of surgery for TOF have steadily improved over the years, thanks to important contributions of many surgeons. Nevertheless because of its protean physiologic and anatomic presentation, TOF continues to offer challenges to cardiologist and cardiac surgeons. Thirty two cases of TOF have undergone total corrective surgery using extracorporeal circulation in the Department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, Inje University, from Oct. 1985 to Feb. 1990. Clinical considerations were applied to these cases and the results were obtained as follows. 1. The heart lung machine used for extracorporeal circulation was SarnsO 7000, 5-head roller pump, and the number and type of oxygenators were 10 of bubble type and 22 of membrane type. The mean bypass time was 148.9 minutes and the mean aortic cross clamp time was 123.8 minutes. The GIK [glucose-insulin-potassium] solution was used as cardioplegic solution for myocardial protection during operation. 2. 20 cases were male and 12 were female, the mean age was 8 years old and the mean body weight was 25Kg. 3. The preoperative symptoms were cyanosis [29 cases], squatting [27 cases] and etc. The mean values of preoperative Hb., Hct., and SaO2 were 16.5 gm /dl, 50.3%, and 78.5%. 4. Combined anomalies were noticed in 16 cases [50%]. Among them 10 cases were PFO and 6 cases were ASD. 5. The degree of aorta overriding were 25% in 5 cases, 25 ~ 50% in 22 cases and above 50% in 5 cases. The dPA/Ao [ratio of diameter of pulmonary artery trunk to ascending aorta] were below 25% in 5 cases, 25 ~ 50% in 10 cases, 50 ~ 70% in 6 cases and above 75% in 11 cases. 6. The types of RVOT [right ventricular outflow tract] stenosis were valvular and infundibular in 14 cases [43.6%], diffuse hypoplastic type in 12 cases [37.5%], infundibular in 5 cases, and valvular and supravalvular in 1 case. 7. One stage radical corrective surgery was applied to the all cases. In widening of the RVOT, 3 types of patches were used: MVOP [monocusp ventricular outflow patch, Polystan BioprosthesesO] in 3 cases, knitted Dacron vessel patches in 2 cases, and double layer with bovine pericardium and woven Dacron prosthesis in 26 cases. 8. Postoperative complications were occurred in 15 cases. Among them, low output syndrome were occurred in 10 cases [31.3%] and 2 of them were expired postoperatively.

• Journal of Chest Surgery
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• v.36 no.4
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• pp.255-260
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• 2003

Background: Stenosis of the left pulmonary artery (LPA) after repair of tetralogy of Fallot (TOF) is troublesome. A new technique of LPA angioplasty using an autologous MPA flap was performed in patients with TOF Material and Method: From October 1998 to January 2001, 24 patients (median age; 10 months, range; 4 to 145 months) underwent total correction of TOF with LPA angioplasty using the autologous MPA flap. Five patients underwent pulmonary angioplasty without any patch over the MPA and LPA. The patches were required to enlarge only the MPA in 4 patients, and transannular RVOT widening was performed in 15. Result: There were no operative or late deaths. During follow-up (range: 6~42 months), reoperation for LPA stenosis was not required in any patients, but balloon angioplasty for branch pulmonary artery stenosis was performed in 3 patients. Echocardiography and CT angiography at the recent follow-up showed an obtuse angle between the MPA and LPA. Conclusion: Although further follow-up is needed, the angioplasty using the autologous MPA flap can be easily performed, avoiding patch-related complications, and allowing growth of the MPA flap. This angioplasty technique creates a more natural and obtuse angle between the MPA and LPA, which can minimize kinking of the LPA, especially in the patients who underwent transannular patch widening.

• Journal of Chest Surgery
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• v.29 no.6
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• pp.639-645
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• 1996

A 14-year-old male patient with previous surgical repair of tetralogy of Fallot was admitted with hemodynamically significant ventricular tachycardia (VT). On preoperative electrophysiologic study (EPS), the morphology of documented VT was RBBB of vertical axis with 320 msec cycle length. The endocardial mapping during VT delineated the origin of VT at right ventricular outflow tract (RVOT), where the patch was attached. The clinical VT had a clockwise reentry circuit around the patch with the earliest activation at the same site seen during the preoperative EPS. The previously placed right ventricular outflow patch and fibrous tissue were removed. During a postoperative EPS, it was no longer possible to induce the VT. Ventricular tachycardia following repair of tetralogy of Fallot seen in this patient was caused by a macro-reentry around the right ventricular outflow patch. We were able to ablate the VT with the aid of a detailed mapping of its epicardial activation sequence.

• Journal of Chest Surgery
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• v.30 no.9
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• pp.869-875
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• 1997

Perimembranous ventri ular septal defects(PMVSDS) are the most common type of ventricular septal defects(VSDs) and consist morphologically of deficiency of the membranous septum and variable portions of the adjacent muscular septum. Repair of VSD has begun via a right ventriculotomy. Even with this exposure, however, it mght lead to ventricular dysfunction. Transatrial exposure of VSDs is luiown to a versatile approach to PMVSDS and even malaligunent defects can be repaired by this method. Although transatrial exposure can be improved by taking down'the atrioventricular valve at the annulus, surgeons have been hesitant to do so because of concern for valvular competence. Therefore, this study was undertaken to clarity the effects of transamlular approach of tricuspid valve (TATV) at operation of PMVSD. During last 5 years, twenty eight cases from 96 patients of PMVSD were closed by TATV and follow up study was done from 3 months to 33 months and results were obtained as follows. 1. Age at operation was fr m 4 months to 38 years and most patients(17, 62%) were above 5 years. 2. Preoperative pulmonary-systemic flow ratio(QPIQS) was ranged from 1 to 2.8 and 22 patients(79%) were less than 2. 3. Peak systolic pulmonary artery pressure was below 30mmHg in 8, 30-50mmHg in 17, above 50mmHg in 3 patients and 25 patients(89%) were less than 50mmHg. 4. Preoperative tricuspid regurgitation(TR) is none in 12, trivial in 6, mild in 3, moderate in 5, severe in 2 patients but postoperative TR was none in 18, trivial in 6, mild in 4 patients, so TR in most patients had decreased or not. 5. Indications for operation were based on the presence of a significant shunt. However, in patients with small shunts, indications for operation were included additional factors, tricuspid valve pouch, RVOT obstruction(right ventricular outflow tract obstruction), subacute bacterial endocarditis and associated anomalies. 6. There were no hospital deaths and residual shunts in postoperative echocardiography. Therefore TATV is especially a good method in PMVSn where patients have trcuspid valve pouch. And it is a safe and effective technique that improves exposure for PMVSD repair and does not adversely affect tricuspid valvular competence.