• Journal of Chest Surgery
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• v.41 no.5
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• pp.573-579
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• 2008

Background: Composite valve graft replacement is currently the treatment of choice for a wide variety of the lesions of aortic root disease. The purpose of this study was to explore the results of aortic root replacement after using the Cabrol technique over a 13-year period at our institution, and we analyzed the results to help surgeons make better decisions when repairing aortic root disease. Material and Method: Between January 1994 and December 2006, twenty-five patients underwent a Cabrol technique operation at our institution. The mean patient age was $43.7{\pm}14.1$ years old (range: $6{\sim}65$ years) and the male and female ratio was 21:4 (84% : 16%). The patients' follow-up was 100% complete, and the mean follow-up period was $60.7{\pm}50.4$ (range:$1{\sim}162$) months. Annuloaortic ectasia (n=18) was the most frequent cause of aortic disease in this series, followed by aortic dissection (n=7). The mean cardiopulmonary bypass time was $177.2{\pm}44.9$ minutes and the mean aortic cross clamping time was $123.4{\pm}34.1$ minutes. Nine patients were checked with MDCT (Multidetector computed tomography) for evaluating a well functioning secondary graft and the coronary anastomosis site. Result: The early mortality rate was 4% (1 of 25 patients). A significant stenosis, kinking or occlusion of the secondary graft was detected by MDCT in 4 patients. The overall survival rate was 88%. Conclusion: The Cabrol technique demonstrated a significant incidence of long-term complications such as secondary graft stenosis or obstruction. It could be used when the modified Bentall technique is not feasible.

• Childhood Kidney Diseases
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• v.6 no.2
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• pp.227-236
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• 2002

Purpose : A study was done to assess the incidence and classification of congenital urinary tract anomalies detected by antenatal ultrasonogram. Methods : We reviewed 558 cases of urinary tract anomaly which were detected by antenatal ultrasonogram and postnatally confirmed between June 1989 and May 2002. We investigated the incidence and classified congenital urinary tract anomalies by review of medical records, antenatal and postnatal radiologic studies retrospectively. Results : In 558 cases of congenital urinary tract anomalies, 292 cases of hydronephrosis were found and the most common. Another anomalies were composed of 65 cases of multicystic dysplastic kidney, 32 cases of hydroureteronephrosis, 31 cases of duplication of kidney, 25 cases of renal agenesis, 21 cases of simple renal cyst, 20 cases of polycystic disease, 13 cases of ureterocele, 11 cases of renal hypoplasia, 10 cases of horseshoe kidney, 9 cases of vesicoureteral reflux, 8 cases of posterior urethral valve, 7 cases of bladder diverticulum, 6 casts of megaureter, 5 cases of ectopia, 2 cases of megacystis, and 1 case of medullary cystic disease. In 82 of the 558 cases, there were two or more combined urinary tract anomalies. Associated diseases other than urinary tract were observed in 13 cases, of which the congenital heart disease was the most common. Conclusion : The congenital urinary tract anomaly is frequently found and diverse during the antenatal ultrasonography. The multicenter study is needed to investigate precise incidence and distribution of each anomalies in general population.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.3
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• pp.99-106
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• 2018

Mucolipidosis type III (pseudo-Hurler polydystrophy) is a mucolipids degrading disorder caused by a mutation in the GNPTAB gene and is inherited by autosomal recessive. It is diagnosed by examining highly concentrated mucolipids in blood and the diagnosis can be confirmed by genetic testing. Mucolipidosis type III is a rare and progressive metabolic disorder. Its initial signs and symptoms usually occur around 3 years of age. Clinical manifestations of the disease include slow growth, joint stiffness, arthralgia, skeletal abnormalities, heart valve abnormalities, recurrent respiratory infection, distinctive facial features, and mild intellectual disability. Here, we are presenting two siblings of mucolipidosis type III, a 4-year-old female and a 2 years and 7 months old male with features of delayed growth and coarse face. The diagnosis was confirmed by [c.2715+1G>A(p.Glu906Leufs*4), c.2544del(p.Glu849Lysfs*22)] mutation in targeted gene panel sequencing. In this case, c.2544del is a heterozygote newly identified mutation in mucolipidosis type III and was not found in the control group including the genome aggregation database. And it is interpreted as a pathogenic variant considering the association with phenotype. Here, we report a Korean mucolipidosis type III patients with novel mutations in GNPTAB gene who have been treated since early childhood. Owing to recent development of molecular genetic techniques, it was possible to make early diagnosis and treatment with pamidronate was initiated appropriately in case 1. In addition to these supportive therapies, efforts must be made to develop fundamental treatment for patients with early diagnosis of mucolipidosis.

• Journal of Chest Surgery
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• v.38 no.8 s.253
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• pp.523-528
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• 2005

Background: Investigation of the change of ventricular function and mitral regurgitation after surgical repair of patient with anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is key issue for the better surgical outcome. Material and Method: From April 1986 to July 2002, 12 patients presented with ALCAPA. The median age at repair was 4 months. Surgical methods included left coronary artery transfer to the aorta (10), Takeuchi procedure (1), saphenous vein free graft bypass (1). Mitral valve was repaired in 1. Result: There were 2 hospital death $(16.7\%)$. The mean follow-up period was $7.1\pm4.1$ years (range, 7 months to 13 years). Four patients required postoperative circulatory assist for $2.2\pm1.1$ days and one needed left ventricular assist device (LVAD) for 1day. Postoperative echocardiography demonstrated significant improvements in mean fractional shortening $(33.4\pm9.1\%\;vs\;17.7\pm9.6\%,\;n=10,\;p<0.05);$ left ventricular end diastolic dimension $(33.4\pm7.3\;mm\;vs\;44.8\pm7.0\;mm,\;n=10,\;p<0.05)$ and systolic dimension $(22.2\pm7.5\;mm\;vs\;33.4\pm7.9\;mm,\;n=10,\;p<0.05)$. Severities of mitral regurgitation decreased in all survivors at 1st and 4th year follow-up echocardiography. There were 2 reoperation due to residual MR and right ventricular outflow obstruction (Takeuchi case). Conclusion: Anatomic repair of anomalous left coronary artery from the pulmonary artery offered an excellent surgical results, especially in terms of the recovery of left ventricle function and mitral regurgitation. However, preoperative indications for mitral procedure is to be evaluated.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.299-304
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• 2009

Background: Although the results of the surgical management for complete atrioventricular septal defect (c-AVSD) have improved, the optimal surgical strategy is still controversial. The aims of this study are to evaluate the outcome of c-AVSD repair and to define the risk factors related to reoperation. Material and Method: We retrospectively reviewed the medical records of 35 patients (8 males and 27 females) who underwent the total correction of c-AVSD from August 1996 to March 2008. The median age at repair was 5.2 months (range: 3 days$\sim$82 months). Sixteen patients (45.7%) were associated with Down syndrome. Prior palliative operations were performed in 4 patients. The one-patch techniques were performed in 3 patients, and the two-patch techniques were done in 32 patients. Result: There was 1 early death (2.9%). The median follow-up period was 68 months (range: $2\sim134$ months) for 34 survivors. There was no late death. Reoperations were performed in 5 patients (14.3%) for severe left atrioventricular valvular regurgitation (AVVR). Nine patients (25.7%) showed left an AVVR of more than grade III. Associated major cardiac anomalies and the use of Gore-Tex patch for ventricular septal closure were the risk factors for postoperative left atrioventricular valve failure and reoperation. Conclusion: In this study, we found that surgical repair of c-AVSD was safe and effective. However, the high reoperation rate after repair remains a problem to be solved.

• 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.35 no.11
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• pp.779-784
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• 2002

Recently, the number of coronary artery bypass surgery(CABG) is increasing according to the increasing incidence of coronary artery disease. However, CABG is not a definite corrective surgery; therefore, in some patients, redo-CABG may be required. We retrospectively reviewed our redo-CABG experiences to help future redo-CABG. Material and Method: From January 1991 to April 2001, 14 cases of redo-CABG were performed in Yonsei Cardiovascular Center(M:F＝12:2) and mean age was 61,7 $\pm$ 7.1(47-72) years. Mean time from 1st. CABG to redo-CABG was 121.9 $\pm$ 50.5(6.1-179.6) months. Thirteen cases were conventional on-pump CABG and one case was off-pump CABG. In two patients, mitral valve re-replacement and mitral valve repair were performed each. All redo-CABG were performed through mid-sternotomy. During redo-CABG, left internal mammary artery and saphenous vein grafts were used in 6 patients, left internal mammary artery and left radial artery grafts were used in 2 patients, left internal mammary artery and gastroepiploic artery were used in one patient and only greater saphenous veins were used in 5 cases(In one case, cephalic vein was also used). The number of mean distal anastomosis was 2.1 $\pm$ 0.9(1-4). Result: There were no operative death and no perioperative myocardial infarctions and cerebrovascular accidents or other heart related complications. Mean follow up duration was 40.1 $\pm$ 38.6(1.1-118.5) months. During follow up period, angina was re-developed in one patient 13 months after operation. Two patients died of end-stage renal failure 14.8 months and 116.3 months after redo-CABG, respectively. During follow up period, coronary angiography was performed in 3 patients, and all grafts were patent. At last follow up, mean Canadian class was 1.3. Kaplan-Meier survival at 9 years was 90.0 $\pm$ 9.5% and event free survival at 9 years was 71.4 $\pm$ 6.9%. Conclusion: After redo-CABG, all patients improved their angina symptom and daily activity. And long-term survival after redo-CABG was excellent. Therefore, if patients have indications for redo-CABG, thenredo-CABG must be strongly recommended and performed.

• Journal of Chest Surgery
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• v.41 no.4
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• pp.417-422
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• 2008

Background: The aim of this study is to evaluate the long term results of creating various right ventricle to pulmonary artery conduits for treating complex congenital heart disease. Material and Method: Between June 1986 and July 2006, we retrospectively reviewed 245 patients who underwent reconstruction of the right ventricular outflow tract with various kinds of conduits. 410 operations were done in 245 patients, the mean age at operation was $3.2{\pm}4.9$ years (range: 7 days$\sim$45 years) and the mean body weight was $12.5{\pm}8.7\;kg$ (range: $2.4\sim76.3\;kg$). Result: We used the following conduits: Polystan conduit, Shelhigh conduit, Carpenter-Edward conduit, Dacron graft with an artificial valve, valveless Gore Tex vascular graft, homograft and hand-made bovine or autologous pericardial conduit. The mean follow up duration was $6.3{\pm}5.2$ years. Redo operation for RV-PA conduit dysfunction was performed in 131 patients, a second redo was done in 31 and a third redo was done in 3. The reoperation free rates were 67.3%, 48.5% and 39.4% for 5 years, 10 years and 15 years, respectively. The homograft showed the best durability, followed by the Dacron graft with artificial valve and the Carpentier-Edward conduit. The larger sized conduit showed better durability. Conclusion: The homograft showed lowest reoperation rate and a smaller size of conduit showed the highest reoperation rate. The reoperation rate for the RV-PA conduit was about 35% at 5 years, so it is mandatory to develop the more durable conduit for RV 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 Yeungnam Medical Science
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• v.10 no.1
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• pp.237-244
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• 1993

A free-floating ball thrombus in the left atrium is a rare complication of the mitral valvular disease. A 53-year-old man was admitted for pain and paresthesia on both legs. On admission he had auscultatory sign of mitral stenosis and mitral regurgitation, and the roentgenogram of his chest revealed a slight pulmonary venous congestion, enlargement of the pulmonary conus and cardiomegaly. Laboratory findings including complete blood counts, coagulation studies and blood chemistry were normal. An echocardiographic examination revealed a mitral stenosis and a free-floating ball thrombus in the left atrium. We performed the emergent open heart surgery for removal of the ball thrombus and mitral replacement successfully with Duromedics 29 mm valve. The size of thrombus was $39{\times}32{\times}30$ mm.