• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.2
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• pp.156-162
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• 2014

A 25-month-old boy was referred to the hospital due to large head detected on routine physical examination. At visit, dysmorphic facial appearances, including broad nose, prominent forehead, and coarse face, were noted. Nasal obstruction with nasal voice, prominent adenoids, and bilateral middle ear effusions were detected. His abdomen was distended, and liver and spleen were palpated about 3 finger and 2 finger breadths, respectively. He was operated for bilateral inguinal hernias. The motion of both elbow joints was mildly limited on supination and pronation. Urinary level of glycosaminoglycan was elevated and the enzyme activity of iduronate sulfatase in leukocytes was decreased. The mutational analysis of the gene iduronate 2-sulfatase (IDS) revealed c.263G>A (p.Arg88His) mutation. His developmental scale showed delayed development and there was cardiac valvular involvement (tricuspid regurgitation and mitral valve prolapse). After the diagnosis of Hunter syndrome, enzyme replacement therapy started on a weekly basis without progression of any clinical features. Here we report a case of early diagnosed Hunter syndrome detected by large head on routine examination. Thus, it is important to associate Hunter syndrome in the patient with large head especially, if there is the history of bilateral inguinal hernia and prominent adenoids to increase the possibility of early diagnosis and treatment.

• Journal of Chest Surgery
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• v.30 no.7
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• pp.663-669
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• 1997

This is a report on the clinical outcome and hemodynamic profile of the ATSwcardiac valve prosthesis, which is a recently introduced pyrolytic carbon bileaflet prosthesis. We retrospectively reviewed the early outcome of 100 consecutive patients who underwent isolated cardiac valve replacement with the ATS(w prosthesis from October 1994 through June 1996 at our hospital. All patients were evaluated with Doppler echocardiography before discharge from the hospital. The mean age of the patients was 48.6 years(range: 2 to 74). A tota of 124 prosthesis were implanted; 71 mitral, 46 aortic, and 7 tricuspid. The two most frequently used sizes were 27 mm(40.8%) and 29 mm(35.2%) in the mitral position, and 23 mm(30.4%) and 21 mm(28.3%) in the aortic position. There was no early or late death. The total follow-up period was 950 patient-months with 99% follow-up rate. Serious late morbidity occurred in three patients; reoperation in two patients for late rupture of Sinus of Valsava in one and for endocarditis with prosthetic dehiscence in the other, and intracranial hemorrhage due to hypertension in one patient. There has been no thromboembolic complication or structural valval deterioration. In the mitral position, the average values of peak and mean transprosthetic pressure gradients and valve area calculated from pressure half time were 6.9$\pm$2.8 mmHg, 2.6$\pm$ 1.5 mmHg, and 2.7 $\pm$0.8 cm2 respectively. In the aortic position, the peak and mean pressure gradients'were 26.4 $\pm$ 15.9 mmHg and 14.2 $\pm$ 7.9 mmHg. For the mitra prostheses larger than 25-mm size, there was no significant difference among prosthetic sizes in terms of transprosthetic gradients, whereas there was a significant negative correlation between the prosthesis size and the transprosthetic gradients for the aortic valves. The peak and mean Pressere pradients were 52.2 $\pm$ 17.6 tmHg and 26.9$\pm$ 7.4 mmHg across the 19-mm aortic Prostheses, and 27.1 $\pm$ 11.9 mmHg and 13.3$\pm$6.6 mmHg across the 21-mm size. Above results can lead to the conclusion that the early clinical outcome of the ATS valve prosthesis is quite satisfactory, And the hemodynamic characteristics are comparable, if not better, with other bileaflet prostheses.

• Journal of Chest Surgery
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• v.37 no.9
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• pp.768-773
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• 2004

Failure of mitral valve repair sometimes may be ascribed to severe or progressive alteration of the subvalvar apparatus. The aim of this study was to evaluate the effects of new chordae formation on mitral repair. Material and Method: From March 1997 to february 1999, 26 patients underwent mitral valve repairs with new chordae formation, we compared the symptoms and echocardiographic findings checked at preoperative state, and intraoperative period, discharge, and their last OPD visit. There were 45 male, and 11 female patients, and their mean age was 51.2$\pm$43.4 years. Etiology of the lesions was degenerative (18), rheumatic (6), infective (1) and ischemic (1). Chordal lesions were caused by rupture (18), elongation (6), and a combination of two causes (2). Associated lesions included atrial septal defect (2), tricuspid insufficiency (7), aortic insufficiency(4), and a combination of previous two factors (2). The number of mean artificial chordae was 3.6$\pm$1.6. Annuloplasty was per-formed in all cases. The CPB time was 182,1$\pm$63.7 minutes and the ACC time was 133.1$\pm$45.6 minutes. Aver-age follow up period was 49.2$\pm$7.1 months. Result: There was no early death. Early reoperation was performed in bud patients, one patient received mitral valve replacement because of an abnormality of annuloplasty and ano-ther received pericardiostomy due to postoperative pericardial effusion. During the follow up of 49.2$\pm$7.1 moths, there was no late mortality. Postoperative NYHA functional class checked at last OPD visit was class I in 22 patients (88%), class II in 2 (8%), and class III in 1 (4%). Regarding the late echocardiogram MR was absent in 20 patients (78%), 1 in 4 (15%), and II in 1 (4%). The postrepair mitral valve area was 2.2$\pm$0.35 $\textrm{cm}^2$ Conclusion: This study suggests that mitral valve repair using new chordae formation provides good early and mid term survivals and functional improvement. We think that the artificial chorda formation with polytetrafluoroethylene suture might be safe and effective technique for mitral valve repair.

• Journal of Chest Surgery
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• v.37 no.8
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• pp.652-659
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• 2004

Background: In this study, we retrospectively analyzed the outcomes of aortic valvuloplasty in pediatric age. Material and Method: Between January 1993 and March 2004, 35 patients underwent aortic valvuloplasty for aortic stenosis (AS) or aortic regurgitation (AR). The mean age was 81.1$\pm$61.5 (1∼223) months. The mean follow up was 50.8$\pm$30.2 (3∼121) months. Nine patients had AS, 21 had AR, and 6 had AS and AR. Valve morphology was tricuspid in 24 patients, bicuspid in 9, quadricuspid in 1, and unicuspid in 1. The mean peak pressure gradients of AS were 72.0$\pm$33.0 mmHg, and the mean grades of AR were 3.1$\pm$0.9. Result: There was one late mortality without early mortality. After operation, AS improved with mean peak pressure gradients of 23.5$\pm$21.0 mmHg (p < 0.05), and AR improved with mean grades of 1.9$\pm$0.8 (p < 0.05). At mean follow up of 35.0$\pm$23.0 months, AS maintained with mean peak pressure gradients of 31.5$\pm$24.0 mmHg, but AR progressed with mean grades of 2.8$\pm$1.3 (p < 0.05). Reoperation was required in 6 patients 38.3$\pm$21.8 months after the original operation. The actuarial figures for freedom from reoperation at 2, 5 and 8 years were 96.9$\pm$3.1%, 79.5$\pm$5.5%, and 56.8$\pm$11.4%, respectively. Age at operation, presence of AS, preoperative severity of AS or AR, and morphology of aortic valve were not significant risk factors for reoperation, and improvement of AS or AR. Conclusion: Aortic valvuloplasty showed good immediate postoperative valve function. Aortic valvuloplasty offers children many years with tolerable valve function and allows to postpone aortic valve replacement or Ross procedure in pediatric patients.

• Journal of Chest Surgery
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• v.32 no.3
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• pp.255-261
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• 1999

Background: Atrial fibrillation is one of the most prevalent of all arrhythmias and in up to 79% of the patients with mitral valve disease. This study examined whether the atrial fibrillation that occur in patients with mitral valve operation could be eliminated by a concommitant maze operation without cryoablation. Material and Method: From May 1997 to April 1998, 14 patients with atrial fibrillation associated with mitral valve disease underwent Maze III operation without cryoablation. Preoperatively there were 6 men and 8 women with an average age of 46.2${\pm}$10.7 years. Eleven patients had mitral stenosis, and three had mitral insufficiency. The associated heart diseases were aortic valve disease in 4, tricuspid valve regurgitation in 1 and ASD in 2. Using transthoracic echocardiography, the mean left atrial diameters was 54.7${\pm}$5.3 mm and thrombi were found in the left atrium of 2 patients. Postoperatively the ratio between the peak speed of the early filling wave and that of the atrial contraction wave (A/E ratio) was determined from transmitral flow measurement. Operations were mitral valve replacement in 13 including 4 aortic valve replacements, 1 DeVega annuloplasty and 2 ASD closures. Maze III operation was performed in 1 patient. Result: Five patients (38%) had recurred atrial fibrillation, which was reversed with flecainide or amiodarone at the average time of postoperative 38.8${\pm}$23.5 days. Postoperative complications were postoperative transient junctional rhythm in 6, transient atrial fibrillation in 5, reoperation for bleeding in 3, postpericardiotomy syndrome(1), unilateral vocal cord palsy(1), postoperative psychosis(1), and myocardial infarction(1). Postoperatively A/E ratio was 0.43${\pm}$0.22 and A wave found in 9(64%) patients. 3 to 14 months postoperatively (average follow- up, 8.1 months), all of patients had normal sinus rhythm and 9(64%) patients had left atrial contraction and 11(79%) patients were not on a regimen of antiarrhythmic medication. Conclusion: We conclude that Maze III operation without cryoablation is an effective surgical treatment in atrial fibrillation associated with the mitral valve disease.

• Journal of Chest Surgery
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• v.30 no.6
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• pp.607-612
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• 1997

Between March 1963, and December 1994, fifty-three patients with a ruptured aneurysm of sinus of Vasalva(RSV) were operated. The RSV originated from the right coronary sinus in 43 patients(81%), from the non coronary sinus in 7 patients(13%), and from combined sinuses in 2 patients. The RSV from the right coronary sinus mainly drained to the right ventricle(81.4%), and the RSV from the non coronary sinus mainly drained to the right atrium(71.4%). Recently, we are using patch to repair the RSV through a double approach. During the operation, concomitant . procedures were performed; 32 Patch repairs of VSD, 14 aortic valvuloplasty, 10 aortic valve replacement, 2 tricuspid valvuloplasty, and one Bentall's procedure. During the follow up period between 1 and 31 years(mean 8 years, 94% follow up), there were 2 late deaths and 14 late complications. Actuarial 10-year survival rate .was 95%, and 10-year actuarial freedom from late event was 53 %. Factor analysis revealed that the combined SBE is a risk factor of late event. Actuarial freedom from failure of aortic valvuloplasty was 55% at 9 years. Although surgical repair of RSV achieved excellent long term survival, aortic regurgitation and endocarditis revealed significant risk factor in long-term results. Therefore, more attention should be required in patients of RSV associated with aortic regurgitation or endocarditis.

• Journal of Chest Surgery
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• v.36 no.7
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• pp.510-513
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• 2003

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 hapoplasia, 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.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.305-310
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• 2009

Background: The Ross procedure is known as a good surgical option for a young age group with aortic valve problems, but few reports on the Ross procedure are available in the Korean literature. This study is a review of our midterm results of 10 year experience with the pediatric Ross operation in Asan Medical Center. Material and Method: From March 1997 to October 2008, eighteen patients who were aged less than 16 years underwent the Ross procedure. There were 11 males and 7 females. The patients median age was 8.5 years (range: $0.5\sim14.0$). The aortic valve pathophysiology was 6 patients with aortic insufficiency, 4 patients with aortic stenosis, 7 patients with mixed aortic stenoinsufficiencey and 1 patient with infective endocarditis. The valve morphology was bicuspid in 11 and tricuspid in 7. All the patients were operated on with the root replacement technique. All the pumonic valves were replaced with an allograft except for one pericardial monocusp valve. The mean follow up duration was 52.8 months (range: 5.8$\sim$138.2 months). We reviewed the echocardiographic data with focusing on the, auto-graft dysfunction and reoperation. Result: There was no hospital mortality and late mortality. According to the last echocardiographic data, 2 autografts showed aortic regurgitation grade 2, 4 autografts showed aortic regurgitation grade 1 and the others were less than trivial. Reoperation of the pulmonic position conduit was performed 4 times in three patients. The rate of freedom from reoperation at 5 years was 72.2%. On the serial follow up, the Z-values of the aortic annulus/aortic sinus were changed from $1.6{\pm}1.7/0.9{\pm}1.7$ at preoperation to $1.8{\pm}1.6$(p=0.64)/$2.2{\pm}0.9$ (p=0.01) at the last follow-up. There was no significant relation between the growth of the neoaortic root and neoaortic insufficiency. Conclusion: Our midterm results of the Ross procedure in pediatric patients showed good autograft function and growth potential. Vet reoperation due to allograft dysfunction was a major concern.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.287-292
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• 1997

.Itrial fibrillation is one of the most common cardiac arrhythmias requiring treatment. About 60% of patients with mitral valvular disease have atrial fibrillation and one third of patients with atrial fibrillation may have the past history of thromboembolic events. Between April 1994 and June 1995, 20 patients with organic heart diseases combined with atrial fibrillation underwent open heart surgery including Cox-maze 111 procedure. There were 6 men and 14 women with an average age of 48 years (range, 31 to 66 years). Nineteen patients had valvular heart diseases and 1 ventricular septal defEct (VSD). Mean duration of atrial fibrillation was 36 months (:42 months) (range, 1 to 132 months). T e past medical history of thromboembolic events was positive in 7 patients (35%) and left atrial thrombus was detected in 9 patients (45%). The concomitant procedures were mitral valve replacement (MVR) and aortic valve replacement (AVR) in 5 patients, MVR in 4, MVd and tricuspid annuloplasty(TAP) in 4, mitral valvuloplasty(Mln) in 3, Mln and Tln in 1, MIW and coronary artery bypass surgery in 1, AVR in 1, and patch closure of VSD in 1. Mean aortic cross-clamping time was 175 minutes (range, 116 to 270 minutes). Atrial fibrillation recurred in 16 patients (80%) during the early postoperative period, but, recurrent atrial fibrillation was converted to regular rhythm at postoperative forty-first day in average. There was no early or late death in this series of 20 patients and postoperative complications were inappropriate tachycardia in 5 patients (25%), low cardiac output syndrome in 3 (15%), aggravated hemiplegic in 1, and acute renal failure in 1. Mean follow-up interval of patient was 16.5 months (range, 10.5 to 24 months) and all patients are currently in regular rhythm. Seventeen patients (85%) are in sinus rhythm and 3 (15%) in junctional rhythm. Right atrial contraction was detected in 95% of patients and left atrial contraction in 63% on postoperative transthoracic echocardiogram. The surgical treatment of atrial fibrillation concomitant with open heart surgery is warranted in the recent clinical setting of improved myocardial protection technique, considering the untoward side-effects of atrial fibrillation.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.271-276
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• 2005

We analysed differences in operative methods and postoperative outcome according to the severity of preoperative cyanosis in adult ToF (Tetralogy of Fallot) patients. Material and Method: From August 1989 to June 2001, thirty three adult patients, 18 females and 15 males, underwent total correction for ToF. Their age ranged from 15 years to 54 years (median: 34). Patients were divided into 2 groups by preoperative $SaO_2$ (arterial oxygen saturation): group I$(n=cyanotic,\;SaO_2\;\geq94\%)$ and group II $(acyanotic,\; SaO_2\geq95%)$. Preoperative median hemoglobin level was higher in group I compared to group II (17.5 g/dl vs 15 g/dl). Postoperative follow-up duration ranged from 1 to 94 months (670 patient-month, median: 14 months), and 63 two-dimensional echocardiographic examinations were done during this period. Result: There were no early or late mortality. With regard to RVOT (right ventricular outflow tract) reconstruction, trans-annular patch and RV-PA extracardiac conduit were used in 7 and 3 patients respectively, and all of them belonged to group I. In group I, cardiopulmonary bypass time, aortic cross-clamping time, ICU day, hospital day were significantly longer than in group II, and postoperative inotropic support was significantly greater than in group II. There was no ventricular arrhythmia in both groups, and one patient in group I suffered from atrial arrhythmia, which was resolved spontaneously after tricuspid and pulmonary valve replacement. During follow-up periods, functional class, residual RVOT stenosis and pulmonary regurgitation, tricuspid regurgitation, occurrence of ventricular and atrial arrhythmias were comparable between two groups. Conclusion: In adult ToF patients with severe preoperative cyanosis, more aggressive RVOT reconstruction and careful postoperative care are mandatory. However intermediate-term outcome of this group of patients is comparable to the patients with minimal or no preoperative cyanosis.