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Background and Objectives@#This study presents an update of the surgical outcomes ofcongenital heart disease (CHD) according to Korea Heart Foundation (KHF) data. @*Methods@#We investigated the data of the 7,305 patients who were economically supportedby KHF in 2000–2014. Of them, we analyzed surgical outcomes of the 6,599 patients whounderwent CHD surgery. @*Results@#The median patient age was 1.9 years (range, 0–71.5 years). Of the 6,599 patients,5,616 (85.1%) underwent biventricular repair and 983 (14.9%) underwent palliativeprocedures. The mean Basic Aristotle Score was 6.6±2.2. A complex procedure (definedas Basic Aristotle Score above 6) was performed in 3,368 patients (51.0%). The earlymortality rate was 3.8%, while the late mortality rate was 1.8%. Previous reports of the KHF(1984–1999) showed that the early surgical and late mortality rates were 8.6%, and 5.3%,respectively. There were 491 neonates (7.4%); among them, the early mortality rate was 12.2%and late mortality rate was 3.7%. There were 2,617 infants (40.0%); among them, the earlymortality rate was 6.0% and the late mortality rate was 2.3%. A total of 591 patients from 30countries were helped by the KHF. @*Conclusions@#More neonatal surgeries (491 vs. 74 patients) were performed than those inthe past (1984–1999). The surgical outcomes were much better than before. Our surgicaloutcomes revealed that the Republic of Korea has been transformed from a country receivinghelp to a country that helps other low socioeconomic status countries.

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No abstract available.

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BACKGROUND: The bidirectional cavopulmonary shunt (BCPS) is one of the primary palliative procedures for complex congenital heart disease. It has many advantages, but it is known to have high risks in young infants. MATERIAL AND METHOD: From 1995 to 2003, 48 infants under the age of one year underwent BCPS. All the patients were Fontan candidates due to functional univentricular heart physiology. There were no significant differences in preoperative variables, except in mean age (67.58+/-3.78 vs. 212.91+/-13.44 days), and mean body weight (4.51+/-0.29 vs. 6.62+/-0.27 kg), between group A ( or=3 months, n=36). RESULT: In group A, the arterial oxygen saturations serially measured were significantly lower. Hospital mortality was 25%, and 19%, respectively. During follow up, there were 2 late mortalities in group A, and 5 in group B. CONCLUSION: This study showed that operative risk in young infants was comparable to that of older patients, and BCPS could be a good option as a primary palliative procedure, and may eliminate other repeated palliative procedures which could be the risk factors for Fontan candidates. However, in high-risk patients accompanying pulmonary hypertension, or heterotaxia syndrome, other palliative procedures should be considered.

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No abstract available.

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BACKGROUND: Coronary artery fistula is rare congenital anomaly, which account for 0.27~0.40% of all congenital heart diseases. We report the clinical observations of 45 patients with coronary artery fistula. MATERIAL AND METHOD: We reviewed all patients presented with or without symptoms of coronary artery fistula between 1987 and 2004. Age ranged from 1 to 83 years. Twenty-six patients were female. The patients were divided into 2 groups according to the presenting symptoms. Twelve patients were in group A (asymptomatic) and 33 patients in group B (symptomatic). The most common clinical presentation in group B was angina (18) followed by dyspnea (7), atypical chest pain (5), syncope (1), fatigue (1), and palpitation (1). Twenty-five patients were associated with other cardiac diseases, which were atrial septal defect (4), coronary artery occlusive disease (6), hypertension (12), and valvular heart disease (2). RESULT: Patients were followed-up for a mean period of 64.8+/-62.7 months. There was no complication related to coronary artery fistula during the follow-up period in both group. There was no mortality related to coronary artery fistula. CONCLUSION: In symptomatic patients, early surgical treatment is recommended considering the low perioperative morbidity. In asymptomatic patients receiving medical treatment, close follow up may be necessary.

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BACKGROUND: The results of biventricular repair for double outlet right ventricle have been improved in recent series. We studied the surgical and long term results for total correction of double outlet right ventricle by the type of ventricular septal defect. MATERIAL AND METHOD: Between November 1979 and December 2003, 126 patients had biventricular repair for double outlet right ventricle. The mean age was 1.8 years (range 1~44) and 86 patients (68.3%) were male. We classified and studied this disease by the type of VSD. RESULT: The locations of VSD were subaortic in 79 (62.7%), subpulmonary in 17 (13.5%), doubly committed in 16 (12.7%) and noncommitted in 14 (11.1%). 28 patients had palliative operation before total correction and the mean interval to total correction was 41.0+/-45.1 months. The methods of total correction were intraventricular baffling in 37 (29.4%), intraventricular baffling with patch enlargement of right ventricular outflow tract in 49 (38.9%), intraventricular baffling with Rastelli procedure in 15 (11.9%), arterial switch operation in 8 (6.3%) and REV procedure in 4 (3.2%), etc. Hospital mortality rate was 10.3% (13 patients) and 25 reoperations were performed in 24 patients (19.0%). The risk factors for hospital mortality and reoperation were cardiopulmonary bypass time (p=0.020) and previous palliative operation (p=0.013), respectively. Follow up was possible in 98 patients and mean follow up period was 118.9+/-70.7 months. The percent survival and survival for freedom from reoperation at 15 years were 82.5% and 66.7%, respectively. The survival rate was significantly lower (p=0.003) in transposition of great artery type and remote type than in simple ventricular septal defect type and tetralogy of Fallot type, but there was no statistical differences in survival rate for freedom from reoperation. CONCLUSION: It is thought to be that acceptible surgical and long term results can be obtained with application of appropriate methods of repair for double outlet right ventricle.

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PURPOSE: We will present our mid-term result of transcatheter closure of PDA with Duct-Occlud device(pfm. AG. Germany) after 12 months follow up and report the problems during the procedure. METHODS: In total 154 patients, the Duct-Occlud devices were inserted in our institute from March, 1996 to August, 2002. Three types of Duct-Occlud device, i.e standard, reinforced, reinforced reverse cone coil were used. Echocardiographic examination was performed at 1, 6, 12 months after procedure. RESULTS: The echocardiographic closure rate was 96% after 12 months. The rates of residual shunt in the standard coil, the reverse cone coil, and the reinforced reverse cone group were 8%, 4% and 3% respectively. In PDA with diameter less than 4 mm, the closure rate was up to 98% while in large PDA with more than 4 mm, it was 72% after 12 months. Embolization of the inserted coils had occurred in 5 cases with successful retrieval using snare catheter. The rupture of the core wire during the procedure and distortion of the original coil shape had occurred in 4 cases. CONCLUSION: The transcatheter occlusion with Duct Occlud is safe and effective method for small to moderate sized PDA less than 4 mm. The minimum diameter of the PDA seems to be the predictor of residual shunt. Further refinement of the device to overcome the procedure-related problems seems to be needed.

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BACKGROUND: There are only limited numbers of reports about long-term results of tricuspid valve replacement (TVR) with bioprosthetic and mechanical prostheses. We analyzed risk factors for tricuspid valve replacement and compared long-term clinical results of bioprosthetic and mechanical valves in tricuspid position. MATERIAL AND METHOD: We reviewed 77 cases of TVR, which were performed between October 1978 and December 1996. Mean age was 38.8 15.9 years. Bioprostheses were implanted in 26 cases and mechanical prostheses were implanted in 51 cases. RESULT: The operative mortality was 15.6% and late mortality was 12.3%. Survival for bioprosthetic and mechanical valve group at 5, 10 and 13 years was 81.3% vs. 100%, 66.1% vs. 100%, 60.6% vs. 100% (p= 0.0175). Free from valve related re-operation for bioprosthetic and mechanical valve group at 5, 10 and 13 years was 100 % vs. 93.9%, 100% vs. 93.9% and 58.3% vs. 93.9% (p=0.3274). Linealized incidences of valve related re-operation for bioprosthetic and mechanical valve group was 2.27 %/patient-years and 1.10 %/patient-years. Risk factor analysis showed that presence of preoperative ascites, hepatomegaly larger than 2 finger breaths, poor preoperative NYHA functional class and number of tricuspid valve replacement were risk factors for early mortality, and the use of bioprosthetic valve and number of open heart surgery were risk factors for late mortality. CONCLUSION: Long-term survival of mechanical valve was superior to bioprosthetic valve in tricuspid position. We recommend mechanical valve in tricuspid position if the patient can be closely followed up.

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Not only morphology of pulmonary artery or pulmonary artery resistance, but major aortopulmonary collateral arteries are risk factors of Fontan procedure. We report a successful Fontan procedure after rehabilitation of pulmonary arteries by unifocalization and systemic to pulmonary shunt in a high risk Fontan candidate with functional single ventricle combined with hypoplastic pulmonary arteries and major aortopulmonary collateral arteries supplying most of the bilateral lung field.

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The assessment of the accuracy of Electron Beam Computed Tomography (EBCT) for the follow-up of pulmonary vascular system after the shunt operation in cyanotic congenital heart diseases was purpose of the study. The study group consists of 16 consecutive patients with cyanotic congenital heart disease who had Blalock-Taussig (BT) shunt (n=7), bi- directional cavo-pulmonary shunt (BCPS, n=7) and unifocalization (n=2). EBT images were obtained on systolic phase under EKG gating and after intravenous administration of contrast agent. We evaluated the shunt patency, anatomy of intrapericardial pulmonary artery, parenchymal pulmonary vessels and background lung attenuation for the pulmonary blood flow, and the presence of systemic arterial and venous collaterals. Angiography (n=12) and echocardiography (n=16) were used as the gold standards. EBCT was consistent with angiogram in detecting the shunt patency and in depicting the anatomy of the intrapericardial pulmonary artery. Occlusion of the BT shunts was not detected in 2 patients by echocardiography. Diffuse or focally decreased pulmonary flow on EBCT in 8 patients was consistent with the pulmonary hemodynamics pattern revealed by cardiac catheterization. Uneven attenuation between lobes was related with multifocal supply of pulmonary flow or occlusion of lobar pulmonary arteries. Systemic collateral arteries were observed in 5 at the corresponding site of the decreased pulmonary flow. Systemic venous collaterals seen in all patients following BCPS were eventually draining to the inferior vena cava in 5 and to the left atrium in 2. EBCT provided accurate information of the pulmonary vascular system after shunt and has unique advantage over echocardiography in assessing patency of BT shunt or unifocalization tubes within the pleural space, the estimation of regional difference in pulmonary hemodynamics, and the detection of systemic collateral vessels. Therefore EBCT may provide useful information about the timing of definitive correction and the need for a second shunt or an interventional procedure prior to total repair.

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PURPOSE: We have performed an analysis on patients who received Rastelli operation in our institute and reviewed their progress postoperatively. Various factors with suspected relationship to the outcome have been considered to help in future treatment and follow-up. METHODS: We analyzed retrospectively 43 patients who either received Rastelli operation in Yonsei University Cardiovascular Center from March 1995 to April 1997 or who received post-procedural cardiac catheterization and follow-up echocardiography in the out-patient department after the procedure. RESULTS: No statistically valid relationships were found between the age of the patient, their body weight, preoperative pulmonary arterial index and pressure, presence of pulmonary branchial stenosis and postoperative results. Cases with atrioventricular concordance showed lower age and body weight, and discordant cases exhibited lower ejection fraction 3 days postoperatively. Upon follow up, lower NYHA score was seen in patients with severe residual stenosis. In the group that received cardiac catheterization after the procedure, residual stenosis and right ventricular pressure measurement in echocardiography showed good correlation with the catheterization data. CONCLUSION: In cases where conduit insertions of the right ventricular outflow tract are required to achieve total correction in complex cardiac deformity, early operation does not seem to provide a clear risk to the patient. In patients with atrioventricular discordance, careful postoperative observation of the ventricular function seems to be needed. Also, echocardiography appears to be a sound method in follow-up of patients after the correctional procedure.

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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.

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Increased or decreased pulmonary blood flow (PBF) and an increased pulmonary vascular resistance (PVR), represent common and important change in pulmonary hemodynamics. In this study, we constructed 3 hemodynamic models in 5 dogs, that is, an increased and a decreased PBF model, and an increased PVR model. A CT perfusion scan was performed in each hemodynamic model. Perfusion parameters including blood flow (BF), blood volume (BV), mean transit time (MTT), and maximal slope (MS) were calculated automatically by specialized software and analyzed for changes according to hemodynamic status. In terms of the normal state, blood flow was affected by gravity and dependent area showed higher BF and BV and lower MS and MTT than the non-dependent area. The decreased PBF model showed a significant increase in BF and MS (p=0.046, 0.005) but no significant change in BV (p > 0.05), and a slight elongation of MTT (p > 0.05) versus the normal state. The increased PBF model showed a slightly increased BV and a slightly decreased MTT (p > 0.05). The increased PVR model showed significant reduction in BF, BV, and MS (p 0.05). However, it was noticeable that the distribution of MTT with respect to gravity in the normal lung was completely reversed in the increased PVR model. In conclusion, based on our understanding of perfusion characteristic in normal state, abnormal regional hemodynamic changes in the lung can be detected and evaluated. Predicting changes in pulmonary vascular resistance should be possible by a thorough analysis of CT perfusion parameters.

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BACKGROUND: Liquid nitrogen freezing techniques have already met with widespread success in biology and medicine as a means of long-term storage for cells and tissues. The use of cryoprotectants such as glycerol and dimethylsulphoxide to prevent ice crystal formation, with carefully controlled rates of freezing and thawing, allows both structure and viability to be retained almost indefinitely. Cryopreservation of various tissues has various controlled rates of freezing. MATERIAL AND METHOD: To find the optimal freezing curve and the chamber temperature, we approached the thermodynamic calculation of tissues in two ways. One is the direct calculation method. We should know the thermophysical characteristics of all components, latent heat of fusion, area, density and volume, etc. This kind of calculation is so sophisticated and some variables may not be determined. The other is the indirect calculation method. We performed the tissue freezing with already used freezing curve and we observed the actualfreezing curve of that tissue. And we modified the freezing curve with several steps of calculation, polynomial regression analysis, time constant calculation, thermal response calculation and inverse calculation of chamber temperature. RESULT: We applied that freezing program on mesenchymal stem cell, chondrocyte, and osteoblast. The tissue temperature decreased according to the ideal freezing curve without temperature rising. We did not find any differences in survival. The reason is postulated to be that freezing material is too small and contains cellular components. We expect the significant difference in cellular viability if the freezing curve is applied on a large scale of tissues. CONCLUSION: This program would be helpful in finding the chamber temperature for the ideal freezing curve easily.

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BACKGROUND: The purpose of this study was to evaluate the fate of left atrioventricular valve regurgitation(LAVVR) following repair of complete atrioventricular septal defects (AVSDs). MATERIAL AND METHOD: Between July 1984 and March 2002, repair of complete AV defects were performed in 77 patients. Mean age at surgery was 30.23+/-69.11 months (range 1 to 456). Echocardiograms of all survivors after isolated AVSDs correction were reviewed. LAVVR were evaluated with color doppler echocardiography in 64 survival periodically. On each study, LAVVR severity was graded on a 1 to 4 scale, based upon the size of the regurgitated jet. RESULT: Mild deterioration of LAVV function was fairly common. LAVVR severity increased by >1 grade in 19 patients (30.2%) during the course of the study. However, the deterioration in LAVVR function occurred primarily between 12 and 24 months postoperatively. After the initial 24 postoperative months, LAVVR worsened on only 8 occasions and in each instance worsened by only 1 grade. Deterioration more than 3+ LAVVR occurred in only 3 patients. And deterioration to 4+ LAVVR was not observed after the initial 24 postoperative months but one. Survival curve analysis predicted a 88.2% of ten-year freedom rate from development of 4+ LAVVR after initial operation of complete AVSDs. CONCLUSION: Postoperative LAVVR remains fairly stable following AVSDs repair. Serious deterioration is rare after 24 postoperative months, especially after the initial 48 postoperative months. But serial follow-up study with echocariogram was need till 24 postoperative months after repair of complete AVSDs.

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BACKGROUND: Arterial switch operation (ASO) has been the most effective surgical option for transposition of the great arteries. But, the inappropriate dilation of the neoaortic root has been reported and its effect on neoaortic valve function and growth of aorta has not been well documented. MATERIAL AND METHOD: Forty-eight patients who underwent cardiac catheterization during follow up after arterial switch operation were included in this study. Arterial switch operation was performed at a median age of 18 days (range 1~211 days). Preoperative cardiac catheterization was performed in 26 patients and postoperative catheterization was performed in all patients at 15.8+/-9.6 months after ASO. Postoperative ratios of the diameters of neoaortic annulus, root and aortic anastomosis against the descending aorta were compared to the size of preoperative pulmonary annular, root and sinotubular junction. Preoperative and operative parameters were analyzed for the risk factors of neoaortic insufficiency. RESULT: There were two clinically significant neoaortic insufficiencies (grade> or =II/IV) during follow up, one of which required aortic valve replacement. Another patient required reoperation due to aortic stenosis on the anastomosis site. Postoperatively, neoaortic annulus/DA ratio increased from 1.33+/-0.28 to 1.52+/-.033 (p=0.01) and neoaortic root/DA ratio increased form 2.02+/-0.40 to 2.56+/-0.38 (p30 days) (p= 0.02), preopeative native pulmonaic valve stenosis (p=0.01), and bisuspid pulmonic valve (p=0.03) were the risk factors for neoaortic insufficiency in univariate risk factor analysis. CONCLUSION: After ASO, aortic anastomosis site showed normal growth pattern proportional to the descending aorta, but neoaortic valve annulus and root were disproportionally dilated. Significant neoaortic valve insufficiency rarely developed after ASO and neoaortic annulus and root size do not correlate with the presence of postoperative neoarotic insufficiency. ASO after neonatal period, preoperative native pulmonary valve stenosis, and bicuspid native pulmonic valve are risk factors for the development of neoaortic insufficiency.

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BACKGROUND: By improving the flow pattern in Fontan circuit, total cavopulmonary connection (TCPC) could result in a better outcome than atriopulmonary connection Fontan operation. For the patients with impaired hemodynamics after atriopulmonary Fontan connection, conversion to TCPC can be expected to bring hemodynamic and functional improvement. We studied the results of the revision of the previous Fontan connection to TCPC in patients with failed Fontan circulation. MATERIAL AND METHOD: From October 1979 to June 2002, eight patients who had failed Fontan circulation, underwent revision of previous Fontan operation to TCPC at Yonsei University Hospital. Intracardiac anomalies of the patients were tricuspid atresia (n=4) and other functional single ventricles (n=4). Mean age at TCPC conversion was 14.0+/-7.0 years (range, 4.6~26.2 years) and median interval between initial Fontan operation and TCPC was 7.5 years (range, 2.4~14.3 years). All patients had various degree of symptoms and signs of right heart failure. NYHA functional class was III or IV in six patients. Paroxysmal atrial fibrillation (n=1), cyanosis (n=2), intraatrial thrombi (n=2), and protein losing enteropathy (PLE) (n=3) were also combined. The previous Fontan operation was revised to extracardiac conduit placement (n=7) and intraatrial lateral tunnel (n=1). RESULT: There was no operative death. Major morbidities included deep sternal infection (n=1), prolonged pleural effusion over two weeks (n=1), and temporary junctional tachyarrhythmia (n=1). Postoperative central venous pressure was lower than the preoperative value (17.9+/-3.5 vs. 14.9+/-1.0, p=0.049). Follow-up was complete in all patients and extended to 50.1 months (mean, 30.3+/-12.8 months). There was no late death. All patients were in NYHA class I or II. Paroxysmal supraventricular tachycardia developed in a patient who underwent conversion to intraatrial lateral tunnel procedure. PLE was recurred in two patients among three patients who had had PLE before the convertsion. There was no newly developed PLE. CONCLUSION: Hemodynamic and functional improvement could be expected for the patients with Fontan circulatory failure after atriopulmonary connection by revision of their previous circulation to TCPC. The conversion could be performed with low risk of morbidity and mortality.

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We performed this study to assess the correlation of residual pulmonary hypertension in the immediate postoperative period with that in the late follow-up period, to assess the histologic changes of pulmonary arteries (PA) at the time of repair for patients with congenital heart disease consisting of left-to-right shunt, and to clarify the role of lung biopsy in determining the operability and reversibility of pulmonary vascular changes. Lung biopsy was performed during repair in 38 patients, with a wide range of age, who had congenital left to right shunt and pulmonary hypertension. All were Heath-Edward grade III or less. Morphometric study included measurement of medial wall thickness (MWT) and decrease rate of pulmonary arterial concentration (PAC). Mean PA pressure in the immediate postoperative period was measured in all 38 patients. Follow-up cardiac catheterization was performed in 15 patients (average 3.8 years after repair). At operation, 5 patients of this late follow-up group were under 2 years of age and the other 10 were 2 or more. During catheterization, pulmonary hemodynamic reaction was observed both under room air inhalation and after inhalation of hypoxic gas FiO2 0.15. Mean PA pressure and pulmonary vascular resistance (PVR) in the immediate postoperative period had a significant correlation with PA pressure and PVR values before the operation, but not with morphometry, Heath-Edward grade, or with pulmonary hemodynamics in late follow-up. During the late follow-up study, 5 of the 15 patients had pulmonary hypertension (defined as mean PA pressure > or = 15 mmHg) under room air inhalation, and PA hypertension was induced in 4 additional patients after hypoxic gas inhalation. There was no incidence of PA pressure or PVR values registering above the preoperative level. The degree of PA hypertension showed a correlation with the rate of PAC decrease and also with patients' age-at-operation. Multiple regression analysis showed that both the rate of PAC decrease and the age-at-operation contributed significantly to the degree of PA hypertension. Some of the patients over age 2 had a decreased rate of PAC above the regression line, which none of the patients under age 2 experienced. In patients with Heath-Edward grade III or less, residual pulmonary hypertension in the immediate postoperative period was not correlated with histology, but in late follow-up, it was with PAC and the age-at-operation. Therefore, a decrease of PAC is assumed to be a totally or partially irreversible pulmonary vascular change depending on the patient's age-at-operation, while medial hypertrophy is thought to be a reversible pulmonary vascular change. Lung biopsy could play an important role in determining the reversibility of pulmonary vascular obstruction, particularly in patients older than 2 years. Ed- re highlights above: such hyphenation is optional, but if used then it should be applied consistently throughout the paper. As 3 of the 4 entries in the abstract use it, I have maintained it consistently below.

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