A Case of Partial Congenital Pericardial Defect Presenting as Acute Coronary Syndrome

Congenital pericardial defects are rare and asymptomatic for both partial and complete defects. However, some patients can experience syncope, arrhythmia, and chest pain. When a patient experiences a symptom, it may be caused by herniation and dynamic compression or torsion of a heart structure including the coronary arteries. Diagnosis of a congenital pericardial defect may be difficult, especially in old patients with concomitant coronary artery disease. The clinical importance of congenital pericardial defect has not been stressed and congenital pericardial defects are regarded as benign, but in this case, pericardial defect was responsible for myocardial ischemia. The authors report a case of partial congenital pericardial defect causing herniation and dynamic compression of the coronary arteries, presenting as an acute coronary syndrome in an old man, with an emphasis on the unique features of the coronary angiogram that support the diagnosis of partial pericardial defects.

Surgical Treatment of Cardiac Myxoma: A 20 Years of Experiences / 대한흉부외과학회지

BACKGROUND: Myxoma makes up close to 50% of adult primary cardiac tumors, and this mainly occurs in the left atrium, and rarely in the right atrium or ventricle. The patients clinically present with symptoms of hemodynamic obstruction, embolization or constitutional changes. Diagnosis is currently established most appropriately with 2-D echocardiography. Surgical resection of myxoma is a safe and effective treatment. MATERIAL AND METHOD: We reviewed our clinical experience in the diagnosis and management of 57 cases of cardiac myxoma that were seen over a 20-year period from July 1984 to July 2004. RESULT: The mean age of the patients was 53.5+/-14.0 years (range: 12 to 76 years). There were 38 (67%) females and 19 (33%) males. The preoperative symptoms included dyspnea on exertion in 27 patients, palpitation in 4, chest pain in 9 and syncopal episode in 4. The diagnosis was made by echocardiography alone in 51, and by combination of echocardiography, CT and angiography in 6. The tumor attachment sites were the interatrial septum in 50, the mital valve annulus in 3 and the left atrial wall in cases. The tumor was excised successfully via biatriotomy in 33 (58%), left atriotomy in 15 (26%), the septal approach via right atriotomy in 3, Inverted T incision in 3 and the extended septal approach in 3. The follow-up time ranged from 1 to 229 months (mean follow-up: 84.0+/-71.3 months). There were no early and late deaths and no recurrence during the follow-up period except for follow-up loss in 5 patients. CONCLUSION: It's concluded that excision of cardiac myxoma is curative and the long-term survival is excellent. Immediate surgical treatment was indicated because of the high risk of embolization or of sudden cardiac death. Radical tumor excision may prevent recurrences.

Occlusion of the Right Coronary Artery Ostium due to Rheumatic Aortic Valve Stenosis / 대한흉부외과학회지

Occlusion of a coronary artery ostium and especially occlusion of the right by an aortic cusp is a rare condition. We experienced an adult patient with occlusion of the right coronary ostium that was due to fusion of the right coronary cusp to the aortic wall along with underlying rheumatic aortic valve stenosis. During the operation, the adherent right coronary cusp was excised. After confirming that the right coronary ostium was patent, the other cusps were removed, and this followed by replacement of the aortic valve with a mechanical valve. The postoperative course was uneventful.

Outcomes of the Modified Maze Procedure for Chronic Atrial Fibrillation Combined with Rheumatic Mitral Valve Disease / 대한흉부외과학회지

BACKGROUNG: The aim of this study was to investigate the mid-term outcomes of our modifications to the maze procedure using cryoablation for treating atrial fibrillation associated with rheumatic mitral valve disease. MATERIAL AND METHOD: Between March 2000 and February 2004, 177 consecutive patients underwent the modified maze procedure with the use of cryoablation concomitant with mitral valve surgery for atrial fibrillation associated with rheumatic mitral valve disease, and were divided into three groups: (1) modified Cox-maze III (CM group, n=88); (2) modified Kosakai-maze (KM group, n=63); and (3) left atrial maze procedure (LA group, n=26). The postoperative and follow- up results were analyzed and compared between the groups. RESULT: There were three hospital deaths (1.7%) and no significant differences in the incidence of postoperative complications between the three groups. The operative time, such as the cardiopulmonary bypass and aortic crossclamp time, were significantly longer in the CM group than in the KM and LA groups, respectively (p<0.0001). The mean follow-up was 22.4+/-15.1 months (1~52.6 months) for all patients. One late death developed in the CM group (0.6%). At last follow-up, 139 patients exhibited sinus rhythm (79.9%), which was also regained in 67 patients (77.9%) in the CM group, 50 (80.7%) in the KM group and 22 (84.6%) in the LA group (p=0.743). The actuarial freedom from stroke at 4 years was 84.6+/-9.4% in the CM group, 95.0+/-4.9% in the KM group, and 92.9+/-6.9% in the LA group (p=0.916). CONCLUSION: The modified maze procedure using cryoablation is safe and effective in treating chronic atrial fibrillation associated with rheumatic mitral valve disease.

Proximal Coronary Artery Stenosis after Direct Coronary Artery Ostial Perfusion : Report of 3 Cases / 대한흉부외과학회지

Proximal coronary artery stenosis after direct coronary artery ostial perfusion is an infrequent but life-threatening complication. We had been experienced 3 cases of proximal coronary artery stenosis related to direct ostial perfusion since September, 2000. And now we report the cases.

Surgical Treatment of Anomalous Origin of Coronary Artery from the Pulmonary Artery: Postoperative Changes of Ventricular Dimensions and Mitral Regurgitation / 대한흉부외과학회지

BACKGROUND: The aims of this study are to verify the result of the surgical treatment of ALCAPA and to identify the postoperative changes of left ventricular dimensions and mitral regurgitation (MR). MATERIAL AND METHOD: Fifteen patients operated on since 1985 were included in the study. The patients operated on before 1998 (n=9) showed heterogeneous properties with various surgical strategies and cardiopulmonary bypass techniques. However, six patients were operated on with the established surgical strategy since 1998; 1) Dual perfusion and dual cardioplegic solution delivery through ascending aorta and main pulmonary artery, 2) Coronary transfer by rolled-conduit made of pulmonary artery wall flap, and 3) Additional mitral valvular procedure was not performed. RESULT: Median age of the study group was 6 months (1 month to 34 years). The operative methods were left subclavian artery to left coronary artery anastomosis in 1, simple ligation in 2, Takeuchi operation in 2, and coronary reimplantation in 10 pateints. The mean follow up period was 5.5<5.8 years (2 months 14 years). There were one early death (6.7%) and one late death. Overall 5-year survival rate was 85.6+/-9.6%. The Z-value of left ventricular end-diastolic and end-systolic dimensions were 6.4+/-3.0 and 5.1+/-3.6 preoperatively, and decreased to 1.7+/-1.9 and 0.8+/-1.6 in 3 months (p<0.05). Significant preoperative MR was identified in 6 patients (40%) and all the patients showed immediate improvement of MR within 1 month postoperatively. There were 3 cases of reoperation due to coronary anastomosis site stenosis and recurrence of MR. However, there was no mortality nor late reoperation in the patients operated on after 1998. CONCLUSION: The surgical treatment of ALCAPA showed favorable survival and early recovery of ventricular dimensions and mitral valvular function. Although long-term reintervention was required in some cases of earlier period, all the cases after 1998 showed excellent surgical outcome without long-term problem.

Significance of Microembolic Signals during Oxygen Inhalation in Patients with Prosthetic Mechanical Heart Valve / 대한흉부외과학회지

BACKGROUND: Transcranial Doppler ultrasonography (TCD) can detect microembolic signals (MES) in the patients with a potential embolic source. Clinical significance of MES has not been demonstrated in patients with prosthetic mechanical heart valves. We studied the correlation between cerebral thromboemoblic events after the mechanical heart valve surgery (MHVS) and residual MES during TCD monitoring with 100% oxygen inhalation in patients with mechanical heart valves. MATERIAL AND METHOD: Twenty patients with previous cerebral thromboemoblic events after MHVS and a sex- and age-matched control group (n=30) were studied. TCD monitoring was performed from unilateral middle cerebral artery. After baseline monitoring for 20 minutes, 6L of oxygen was inspired for 40 minutes. RESULT: The site of valve and the duration after MHVS of the patients did not differ from those of controls. During baseline monitoring, there was no significant difference in MES prevalence or counts compared to controls. During oxygen inhalation, patients showed a higher MES prevalence (55%, 27.6%, p=0.045) and a more frequent MES counts (p=0.027) compared to controls. CONCLUSION: TCD monitoring with oxygen inhalation may be useful to differentiate clinically significant MES in patients with mechanical heart valve.

Outcome of Patch Angioplasty for Left Main Coronary Ostial Stenosis / 대한흉부외과학회지

BACKGROUND: Patch angioplasty is an alternative surgical procedure to coronary artery bypass grafting (CABG) for left main coronary ostial stenosis. The purpose of this study is to evaluate the outcome of patch angioplasty by analyzing the short-term and long-term results. MATERIAL AND METHOD: Twenty nine patients who had undergone patch angioplasty due to left main coronary ostial stenosis between July 1991 and May 2003 were enrolled in the study. The mean age of the patients was 53.1+/-12.5 years. There were 8 males and 21 females, and there were 12 female patients who had no risk factor for atherosclerosis. Twenty six (89.7%) patients showed isolated coronary ostial stenosis without any distal coronary lesion. RESULT: Anterior approach was used in 28 patients and superior approach was used in one patient. Transsection of the main pulmonary artery was used in one patient. Concomitant CABG was performed in 4 patients because of left anterior descending artery lesions in 3 patients and unstable postoperative hemodynamic status in one patient. Hospital mortality had occurred in one patient (3.4%) and late mortality also in one patient, therefore the overall 5 year survival rate was 91.2+/-6.1%. Seventeen coronary angiographies were done in 13 patients (44.8%) postoperatively. Two distal patch stenoses, 1 proximal patch stenosis, and 1 new right coronary ostial lesion were identified and 3 percutaneous interventions and 1 CABG were performed during the follow-up period. The overall 5 year freedom from reintervention rate was 82.4+/-8.5%. Aortic regurgitation less than grade I had developed postoperatively in 4 patients and one patient showed progression of preexisting aortic regurgitation from grade II to III. CONCLUSION: Patch angioplasty in left main coronary ostial lesion showed acceptable short-term and long-term results in this study. However, restenosis at the patch anastomosis site and aortic regurgitation should be carefully investigated during the follow-up period.

Biventricular Repair of Double Outlet Right Ventricle with Non-Committed Ventricular Septal Defect by Arterial Switch: Report of 1 cases / 대한흉부외과학회지

Although surgical options for double outlet right ventricle (DORV) with non-committed ventricular septal defect (VSD) are vary in accordance to the morphological characteristics, it is very difficult to use biventricular repair technique when there is tricuspid chordae originating from conal septum or when the distance between the tricuspid valve and the pulmonic valve is too short. We report our clinical experience of biventricular repair of DORV with non- committed VSD by VSD rerouting to the pulmonary artery and arterial switch in case of a presence of conal tricuspid chordae and short distance between the tricuspid valve and the pulmonic valve.

Cabrol Technique Application in Off-pump Coronary Artery Bypass Grafting Using Radial Artery / 대한흉부외과학회지

In off-pump coronary artery bypass grafting (CABG), multiple proximal anastomosis may increase the risk of cerebral embolism (air, debris) and aortic injury (dissection, pseudoaneurysm). Radial artery (RA) has no intraluminal valve such as saphenous vein. We applied Cabrol technique using aortic root replacement for proximal anastomosis in off-pump CABG. Cabrol technique using RA graft can reduce numbers of proximal anastomosis and reduce number of aortic manipulation in off-pump CABG. We report a Cabrol technique for proximal anastomosis in off-pump CABG with RA graft.

Immediate Reoperation for Failed Mitral Valve Repair / 대한흉부외과학회지

BACKGROUND: We analysed the surgical outcomes of immediate reoperations after mitral valve repair. MATERIAL AND METHOD: Eighteen patients who underwent immediate reoperation for failed mitral valve repair from April 1995 through July 2001 were reviewed retrospectively. There were 13 female patients. The mitral valve disease was regurgitation (MR) in 12 patients, stenosis (MS) in 3, and mixed lesion in 3. The etiologies of the valve disease were rheumatic in 9 patients, degenerative in 8, and endocarditis in 1. The causes of reoperation was residual MR in 13 patients, residual MS in 4, and rupture of left ventricle in 1. Fourteen patients had rerepair for residual mitral lesions (77.8%) and four underwent replacement. RESULT: There was no early death. After mean follow-up of 33 months, there was one late death. Echocardiography revealed no or grade I of MR (64.3%) in 9 patients and no or mild MS in 11 patients (78.6%). Reoperation was done in one patient. The cumulative survival and freedom from valve-related reoperation at 6 years were 94% and 90%, respectively. The cumulative freedom from recurrent MR and MS at 4 years were 56% and 44%, respectively. CONCLUSION: This study suggests that immediate reoperation for failed mitral valve repair offers good early and intermediate survival, and mitral valve rerepair can be successfully performed in most of patients. However, because mitral rerepair have high failure rate, especially in rheumatic valve disease, adequate selections of valvuloplasty technique and indication are important to reduce the failure rate of mitral rerepair.

Changes of Microembolic Signals after Heart Valve Surgery / 대한흉부외과학회지

BACKGROUND: The detection of circulating microemboli by transcranial Doppler ultrasonography (TCD) has the potential to select the patients with high risk for future symptomatic brain embolism. We prospectively evaluated the positive rate and the frequency of microembolic signals (MES) before and after the heart valve surgery (HVS). MATERIAL AND METHOD: Fifty in-patients with heart valve disease were enrolled in this study. Patients with history of previous stroke or heart valve surgery were excluded. Two unilateral TCD monitoring sessions were performed from middle cerebral artery for 1-hour, before and after HVS. RESULT: Mechanical Heart valves were implanted in 28 patients, tissue valves were implanted in 10 patients, and remaining 12 patients received mitral valve repair. Positive rate of MES was significantly increased after HVS (50%), compared to that of before HVS (8%, p=0.00). There was no relation between MES after HVS and intensity of anticoagulation, cardiac rhythm, patients' age, and history of hypertension. The positive rate of MES after implantation of mechanical heart valve (71.4%) was significantly higher than those after implantation of tissue valve or mitral valve plasty (p=0.002). CONCLUSION: Positive rate of MES was increased significantly after the implantation of HVS. The changes of MES in those with mechanical prosthesis may be related to the increased risk of embolism after HVS.

Outcomes of Combined Mitral Valve Repair and Aortic Valve Replacement / 대한흉부외과학회지

BACKGROUND: The long-term results of combined mitral valve repair and aortic valve replacement (AVR) have not been well evaluated. This study was performed to investigate the early and long-term results of mitral valve repair with AVR. MATERIAL AND METHOD: We retrospectively reviewed 45 patients who underwent mitral valve repair and AVR between September 1990 and April 2002. The average age was 47 years; 28 were men and 17 women. Twelve patients had atrial fibrillation and three had a previous cardiac operation. The mitral valve disease consisted of pure insufficiency (MR) in 34 patients, mitral stenosis (MS) in 3, and mixed lesion in 8. Mitral valve disease was due to rheumatic origin in 24 patients, degenerative in 11, annular dilatation in 8, and ischemia or endocarditis in 2. The functional anatomy of mitral valve was annular dilatation in 31 patients, chordal elongation in 19, leaflet thickening in 19, commissural fusion in 13, chordal fusion in 10, chordal rupture in 6, and so on. Aortic prostheses used included mechanical valve in 32 patients, tissue valve in 12, and pulmonary autograft in one. The techniques of mitral valve repair included annuloplasty in 32 patients and various valvuloplasty of 54 techniques in 29 patients. Total cardiopulmonary bypass and aortic cross clamp time were 204+/-62 minute and 153+/-57 minutes, respectively. RESULT: Early death was in one patient due to low output syndrome (2.2%). After follow up of 57+/-37 months, late death was in one patient and the actuarial survival at 10 years was 96+/-4%. Recurrent MR developed grade II or III in 11 patients and moderate MS in 3. Three patients required reoperation for valve-related complications. The actuarial freedom from recurrent MR, MS, and reoperation were 64+/-11%, 86+/-8%, and 89+/-7% respectively. CONCLUSION: Combined mitral valve repair with AVR offers good early and long-term survival, and adequate techniques and selection of indication of mitral valve repair, especially in rheumatic disease, are prerequisites for better long-term results.

Aortic Root Replacement in Patient of Right Ventricular Hypoplasia with Annuloaortic Ectasia, Ventricular Septal Defect and Aortic Regurgitation: Report of 1 case / 대한흉부외과학회지

Annuloaortic ectasia, aortic regurgitation (AR), and ventricular septal defect (VSD) in patients with right ventricular hypoplasia is a very rare condition. We report a patient who underwent aortic root replacement with a composite graft for annuloaortic ectasia associated with VSD and AR in right ventricular hypoplasia. The patient was a 19 year-old male. Transthoraic echocardiogram and cardiac catheterization revealed a perimembranous VSD (2 cm in diameter), severe AR, annuloaortic ectasia, bipartite right ventricle with hypoplasia, and hypoplastic tricuspid valve. Operative findings showed that free margins of the right and noncoronary cusps were markedly elongated, thickened, and retracted, and commissure between the right coronary cusp and the noncoronary cusp was fused and calcified. VSD was closed with an autologous pericardial patch and composite graft aortic root replacement using direct coronary button reimplantation was performed, and the hypertrophic muscle of the right ventricular outflow tract was resected. The patient had transient weaning failure of cardiopulmonary bypass and was discharged at the postoperative 14 days without any problems.

Anticoagulant Therapy for Left Ventricular Thrombosis after Dor Procedure / 대한흉부외과학회지

Left ventricular thrombosis is a frequent and potentially dangerous complication in acute myocardiac infarction, but its occurrence and adequate therapy has not been known in patients with Dor procedure for the ischemic cardiomyopathy. We report a patient, 45 year-old male, who had a new left ventricular thrombus developed after coronary arterial bypass graft, Dor procedure, and removal of the left ventricular thrombus for ischemic cardiomyopathy. Left ventricular thrombus was disappeared on the follow-up cardiac MRI following intravenous heparin injection and oral coumadin therapy. This case suggest that anticoagulation therapy may prevent patients with the severe left ventricular dysfunction and apical aneurysm and dyskinesia from developing the left ventricular thrombus, and that thrombi will resolve without clinical evidence of systemic embolism.

Fontan Conversion with Arrhythmia Surgery in a Jehovah's Witnesses / 대한흉부외과학회지

The conversion of failing Fontan circuit to total cavopulmonary connection(TCPC) is recommended as a therapeutic option in patients with late Fontan complications such as atrial arrhythmia, atrial enlargement, pulmonary venous obstruction, and ventricular dysfunction. Combined TCPC with extracardiac conduit and cryoablation of arrhythmia circuit is preferred for treatment of failing Fontan connection with atrial tachyarrhythmia. We report a case of conversion of atriopulmonary connection to extracardiac conduit Fontan and cryoablation of atrial arrhythmia circuit in a patient with tricuspid atresia, who also had ectopic atrial tachycardia, right atrial thrombi, pulmonary venous obstruction, and ventricular dysfunction. This patient and the parents were Jehovah's Witnesses; therefore, the patient underwent the procedure without blood transfusion.

Reoperations on the Aortic Root and Ascending Aorta / 대한흉부외과학회지

BACKGROUND: Reoperations on the aortic root or the ascending aorta are being performed with increasing frequency and remain a challenging problem.This study was performed to analyze the results of reoperations on the ascending aorta and aortic root. MATERIAL AND METHOD: Between May 1995 and April 2001,30 patients had reoperations on the ascending aorta and aortic root and were reviewed retrospectively.The mean interval between the previous repair and the actual reoperation was 56 months(range 3 to 142 months).Seven patients(23.3%)had two or more previous operations.The indications for reoperations were true aneurysm in 7 patients(23.3%),prosthetic valve endocarditis in 6(20%),false aneurysm in 5(16.7%),paravalvular leak associated with Behcet's disease in 4(13.3%),malfunction of prosthetic aortic valve in 4(13.3%),aortic dissection in 3(10%),and annuloaortic ectasia in 1(3.3%).The principal reoperations performed were aortic root replacement in 17 patients(56.7%),replacement of the ascending aorta in 8(26.7%),aortic and mitral valve replacement with reconstruction of fibrous trigone in 2(6.6%),patch aortoplasty in 2(6.6%), and aortic valve replacement after Bentall operation in 1(3.3%).The cardiopulmonary bypass was started before sternotomy in 7 patients and the hypothermic circulatory arrest was used in 16(53.3%).The mean time of circulatory arrest,total bypass,and aortic crossclamp were 20 +/- 12 minutes,228 +/- 56 minutes,and 143 +/- 62 minutes,respectively. RESULT: There were three early deaths(10%).The postoperative complications were reoperation for bleeding in 7 patients(23.3%),cardiac complications in 5(16.7%),transient acute renal failure in 2(6.6%), transient focal seizure in 2(6.6%),and the others in 5.The mean follow-up was 22.8 +/- 20.5 months.There were two late deaths(7.4%).The actuarial survival was 92.6 +/- 5.0%at 6 years. One patient required reoperation for complication of reoperation on the ascending aorta and aortic root(3.7%).The 1-and 6-year actuarial freedom from reoperation was 100%and 83.3 +/- 15.2%,respectively.One patient with Behcet's disease are waiting for reoperation due to false aneurysm,which developed after aortic root replacement with homograft.There were no thromboembolisms or anticoagulant related complications. CONCLUSIONS: This study suggests that reoperations on the ascending aorta and aortic root can be performed with acceptable early mortality and morbidity,and adequate surgical strategies according to the pathologic conditions are critical to the prevention of the reoperation.

Composite Graft Aortic Root Replacement with Coronary Button Reimplantation: The Early and Mid-Term Results / 대한흉부외과학회지

BACKGROUND: This study was undertaken to investigate the outcome of composite graft aortic root replacement using coronary button reimplantation technique for the treatment of aneurysms of the ascending aorta involving the aortic root. MATERIAL AND METHOD: Between April 1995 and September 2001, 54 patients having aortic root replacement with a composite valve graft using direct coronary button reimplantation were reviewed retrospectively. Left ventricular dysfunction was present in 14 patients(25.9%), aortic regurgitation in 48(89%), and Marfan's syndrome in 17(31.5%). The indications for operation were annuloaortic ectasia in 29 patients(53.7%), aortic dissection in 11(20.4%), aneurysms of the ascending aorta involving aortic root in 12(22.2%), and aortitis in 2(3.7%). Six patients(11.1%) had previous cardiac or ascending aortic operations. Concomitant procedures were arch replacement in 21 patients(38.9%), coronary artery bypass graft in 7(13%), mitral valve repair or replacement in 4(7.4%), and others in 6. The mean time of circulatory arrest, total bypass, and aortic crossclamp were 18+/-9 minutes, 177+/-42 minutes, and 127+/-31 minutes, respectively. RESULT: There was 1 early death(1.9%). Mean follow-up was 24.6+/-19.5 months. There were two late deaths(3.8%) including one death due to the traumatic cerebral hemorrhage. The Kaplan-Meier survival rate was 98.0+/-2.0% and 93.1+/-5.1% at 1 and 6 years, respectively. Two patients required reoperation owing to a false aneurysm at the root anastomosis site anda malfunction of prosthetic aortic valve(3.8%). Staged operation for dissection of the remaining thoracoabdominal aorta was performed in 1 patient. The freedom rate from reoperation was 97.8+/-2.0% and 65.3+/-26.7% at 1 and 6 years, respectively. CONCLUSION: This study suggests that composite graft aortic root replacement using open button technique is a safe and effective therapy for a variety of aneurysms of the aortic root, resulting in good early and mid-term results. Careful follow-up of all patients following composite graft root replacement is important for the good long-term results.

Usefulness and Surgical Strategies of Pulmonary Artery Banding in Functional Univentricular Heart / 대한흉부외과학회지

BACKGROUND: Pulmonary artery banding (PAB)in the functional univentricular heart (UVH)is a palliative procedure for staging toward the Fontan procedure;however,it is known to be a risk factor. MATERIALS AND METHOD: The records of all 37 patients with functional UVHs who underwent surgical palliation using PAB between September 1989 and August 1999 were reviewed retrospectively.We investigated the aortic arch obstruction,the development and progression of subaortic stenosis after PAB,and risk factor of mortality according to surgical method. RESULT: In 37 neonates and infants with single ventricular physiology,aortic arch obstruction was combined in 7.There were 6 early deaths (16.2%)after PAB and 3 late deaths (8.1%)after Fontan operation.The actuarial overall survival including early mortality at 3 and 5 years were 8 0 .7+/-6.6%,72.2 +/-8.2% respectively. Among 31 patients who survived PAB,27 patients (87.1%)could become candidates for Fontan operation;22 patients(71.0%)completed Fontan operation with 3 deaths and 5 were waiting bidirectional cavopulmonary shunt(BCPS)or Fontan operation (follow-up mean 4.5 year,minimal 2 year). Subaortic stenosis developed in 8 patients after PAB (8/29,27.6%);3 cases in the patients without arch anomaly (3/22,13.6%)and 5 in those with arch anomal y (5/7,71.4%).The subaortic stenosis was managed with Damus-Kaye-Stansel procedure (DKS)in 6 patients without operative mortality and conal septum resection in 2 without long-term survivor. Analysis of risk factors established that aortic arch obstruction was strongly associated with subaortic stenosis (p<0.001).The only risk factor of late mortality was Fontan procedure without staged palliation by BCPS (p=0.001). CONCLUSION: PAB is effective as an initial palliative step in functional UVH.And the high risk group of patients with aortic obstruction can undergo effective short-term PAB as an initial palliative step,with subsequent DKS for subaortic stenosis.This strategy,initial PAB and careful surveillance,and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later Fontan procedures.

Biventricular Repair of Critically Ill Neonate with Ebstein's Anomaly: Report of 1 case / 대한흉부외과학회지

Patients with severe Ebstein's anomaly showing in the neonatal period, represent progressive cardiac enlargement with pulmonary hypoplasia and functional pulmonary atresia with patent ductus arteriosus-dependent pulmonary circulation. Biventricular repair in these patients had been mostly unsuccessful except for Starnes' procedure that converts the anatomy to single ventricle physiology for Fontan procedure. A 4-days old male was admitted with the diagnosis of severe Ebstein's anomaly with anatomic pulmonary atresia and severe cardiac enlargement. He successfully underwent biventricular repair with vertical plication method of atrialized right ventricle, tricupid annuloplasty, transannular right ventricular outflow tract reconstruction, atrial septal defect patch closure with fenestration, and right atrial reduction angioplasty. Postoperatively, cardiothoracic ratio was significantly reduced and mild tricuspid regurgitation was remnant in echocardiography. The patient is currently 10 months old and is fully active without restrictions.