• Journal of Chest Surgery
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• 제38권2호
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• pp.172-174
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• 2005

유미흉은 드물지만 심각한, 잘 알려져 있는 흘부 및 심장 수술의 합병증이다. 동맥관 개존증 결찰 및 대동맥 축착증 교정술을 시행 받은 3개월 된 남자 환아에서 수술 후 유미흉이 발생하였다. 환자는 흉강 배액과 함께 옥트레오타이드 주사요법과 중쇄지방산 분유를 이용한 보존적 방법으로 성공적으로 치료되었다. 이에 저자들은 수술 후 발생한 유미흉 환자에서 옥트레오타이드를 사용한 보존적 치료로 좋은 결과를 치험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제18권4호
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• pp.574-581
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• 1985

Surgical treatment for PDA has been pivotal in historical development of surgery for congenital heart disease. A clinical study on 36 cases of operated PDA were performed during period from Aug. 1981 to Jul. 1985 at the Department of Thoracic & Cardiovascular Surgery in Chonbuk University. The following results are obtained. 1. The 8 males and 28 females ranged in age from 2 yrs, to 24 yrs, [mean 11 yrs.] 2. Chief complaints of the patients were dyspnea on exertion in 61%, palpitation in 39%, frequent URI in 12%, and no subjective symptoms in 11%. 3. On auscultation, continuous machinery murmur heard in 94% and systolic in 14%. 4. Radiologic findings of chest P-A showed increased density of pulmonary vascularity in 94%, cardiomegaly in 69%, and within normal limits in 5% of the patients. 5. EKG findings of the patients revealed LVH in 69%, RVH in 6%, BVH in 6%, and within normal limits in 17%. 6. Of the 36 patients, cardiac catheterization was performed in 34 patients. The results showed mean Qp/Qs = 2.25, mean Pp/Ps=0.42, and mean systolic pulmonary arterial pressure=53mmHg. 7. Surgical methods were as followed: The 32 case of ductal ligation and one case of division & suture technique for PDA through the left posterolateral thoracotomy were done. And 2 cases of ductal ligation one suture closure through the pulmonary artery were performed under the cardiopulmonary bypass. 8. Intraoperative complication was ductal rupture with division 8< suture for PDA and transient hoarseness in 1, recanalization in 1, and urethral stricture in 1 case postoperatively. 9. One patient died due to ductal rupture intraoperatively and operative mortality was 2.8%.

• Journal of Genetic Medicine
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• 제11권2호
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• pp.86-90
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• 2014

Smith-Lemli-Opitz syndrome (SLOS) is a rare autosomal recessive disorder caused by 7-dehydrocholesterol reductase deficiency. The characteristic clinical features are syndactyly of the second and third toes, facial dysmorphism, multiple malformations, and intellectual disability. Few cases of SLOS have been reported in Korea. We observed a male patient with SLOS who presented with typical facial features, undescended testes, microcephaly, bilateral syndactyly of the second and third toes, and cardiac defects, including patent ductus arteriosus and atrial septal defect. Mutation analysis of the DHCR7 gene identified compound heterozygous mutations of c.907G>A (p.Gly303Arg) and c.1055G>A (p.Arg352Gln). In a review of the literature, c.1054C>T (p.Arg352Trp) was the most common mutation reported in Far East Asian countries. This report describes the clinical features, biochemical data, molecular characteristics, and clinical outcome of a Korean patient with SLOS.

• Clinical and Experimental Pediatrics
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• 제65권4호
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• pp.188-193
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• 2022

Background: Respiratory distress syndrome (RDS) is a common cause of hospitalization and death in preterm infants who require surfactant treatment and respiratory support. Purpose: This study aimed to compare the clinical outcomes of minimally invasive surfactant therapy (MIST) and the INtubation, SURfactant administration, and Extubation (INSURE) technique in preterm infants with RDS. Methods: In this clinical trial, 112 preterm infants born at 28-36 weeks of gestation and diagnosed with RDS randomly received 200-mg/kg surfactant by MIST or the INSURE method. In the MIST group, surfactant was administered using a thin catheter (5F feeding tube); in the INSURE group, surfactant was administered after intubation using a feeding tube and the tracheal tube was removed after positive pressure ventilation was started. Nasal continuous positive airway pressure was applied in both groups for respiratory support and the postprocedure clinical outcomes were compared. Results: The mean hospitalization time was shorter for infants in the MIST group than for those in the INSURE group (9.19±1.72 days vs. 10.21±2.15 days, P=0.006). Patent ductus arteriosus was less frequent in the MIST group (14.3% vs. 30.4%, P=0.041). Desaturation during surfactant administration occurred less commonly in the MIST group (19.6% vs. 39.3%, P=0.023). There were no significant intergroup differences in other early or late complications. Conclusion: These results suggest that surfactant administration using MIST could be a good replacement for INSURE in preterm infants with RDS since its use reduced the hospitalization time and the number of side effects.

• Journal of Genetic Medicine
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• 제18권1호
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• pp.48-54
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• 2021

Genetic imbalances are a major cause of congenital and developmental abnormalities. We report the first case of a 3p26 microdeletion and 5q35.2q35.3 microduplication in a newborn with multiple congenital anomalies evaluated using chromosomal microarray analysis (CMA) and fluorescence in situ hybridization (FISH). The patient was born at 30 weeks and 2 days of gestation with a body weight of 890 g. He had symmetric intrauterine growth restriction, microcephaly, facial dysmorphism (hypertelorism, blepharophimosis, mild low-set ears, high-arched palate, and micrognathia), and right thumb polydactyly. Echocardiography revealed an atrial septal defect and patent ductus arteriosus. Furthermore, CMA revealed a concurrent microdeletion in 3p26 and a microduplication in 5q35.2q35.3. FISH analysis showed that these genetic changes resulted from a translocation mutation between chromosomes 3 and 5. The patient's mother had mild intellectual disability, short stature, and facial dysmorphism, while his father had a normal phenotype. However, parental FISH analysis revealed that the asymptomatic father carried a balanced translocation of chromosomes 3p26 and 5q35. CMA and FISH tests are useful for diagnosing neonates with multiple congenital abnormalities. Further parental genetic investigation and proper genetic counseling are necessary in cases of chromosomal abnormalities inherited from parental balanced translocations.

• Clinical and Experimental Pediatrics
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• 제49권9호
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• pp.959-965
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• 2006

목 적 : 신생아 중환자실에서 환자 치료의 질적, 양적 성장으로 인해 초극소 저출생체중아의 생존률은 많은 향상을 보였음에도 불구하고 동맥관개존증 및 뇌실내출혈과 연관된 이환율 및 사망률은 높은 실정이다. 이에 저자들은 초극소 저출생체중아에서 동맥관개존증과 뇌실내출혈을 예방하기 위한 시도된 약물 중 indomethacin의 예방적 투여에 대한 효과와 부작용을 분석하고자 하였다. 방 법 : 2004년 6월부터 2006년 4월까지 삼성서울병원 신생아 집중치료실에 입원한 재태연령 29주, 출생체중 1,000 g 미만의 초극소 저출생체중아 84명을 대상으로 후향적으로 의무기록을 조사하였다. Indomethacin을 생후 6시간 이내에 24시간 간격으로 0.1 mg/kg를 투여받은 예방군 28명과 대조군 56명으로 분류하여 두 군의 인구학적 및 주산기 인자, 동맥관개존증과 뇌실내출혈의 발생율 및 합병증과 사망률에 대해서 비교하였다. 결 과 : 인구학적 및 주산기 인자로서 재태연령과 출생체중은 각각 예방군에서 $25.1{\pm}1.3$주, $746{\pm}132g$이었으며 대조군에서는 $25.2{\pm}1.6$주, $738{\pm}142g$으로 두 군간에 차이가 없었다. 그 외에 성 비, 부당경량아, 쌍생아의 비율, 1분 및 5분 아프가 점수, 분만 방법, 임신성 고혈압, 임신성 당뇨, 산전 스테로이드 투여, 호흡곤란증후군의 유무, 융모양막염의 여부에서도 두 군간에 차이가 없었다. Indomethacin의 예방효과가 있을 것이라 기대했던 동맥관개존증과 3도 이상의 고도의 뇌실내출혈의 발생 비율에서는 두 군간에 의미 있는 차이는 없었다. 그러나 첫 번째 치료적 indomethacin이 투여된 빈도는 예방군에서 더 많았고 indomethacin 투여된 시간은 예방군에서 유의하게 늦었다. 사망률은 두 군 사이에 차이가 없었고 합병증과 관련된 인자로 폐출혈, 기흉, 기관지폐이형성증, 미숙아 망막증, 괴사성 장염의 발생에서는 유의한 차이는 없었다. 그러나 자발성 회장 천공의 발생은 대조군에서는 없었던 반면에 예방군에서는 10.7%로 나타났고, 괴사성 장염에서 천공된 경우를 합한 경우에도 예방군에서 의미있게 증가하였다. 또한 완전장관영양에 도달한 시기는 예방군에서 $31.5{\pm}17.1$일이었고, 대조군에서는 $22.1{\pm}7.6$일로 예방군에서 유의하게 늦었다. 태변이 배출되는 데까지 걸린 기간과, 위장관 출혈의 발생빈도, 신 기능의 손상 정도의 평가에서는 두 군간의 의미있는 차이는 없었다. 결 론 : 초극소 저출생체중아에서 출생 후 저용량 indomethacin을 예방적으로 사용하여 뇌실내출혈이나 동맥관개존증의 비율을 감소시키진 못하였고, 오히려 장관 천공의 비율을 증가시키고 장관영양의 진행이 늦어지는 부작용을 초래할 수 있다.

• Journal of Chest Surgery
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• 제42권4호
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• pp.434-440
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• 2009

배경: 미숙아에 있어서 동맥관의 외과적 폐쇄술은 인도메타신 투여 후 치료 실패나 적응증이 되지 않는 경우 일반적으로 시행하고 있다. 그러나 초극소 저출생 체중아(ELBW, <1,000그램)에서는 일반적인 개흉술이나 흉관 삽입 자체 만으로도 사망률이나 이환율에 많은 영향을 미치게 된다. 이에 본 연구에서는 후방 소개흉술(dosal rninithoracotomy)을 통해 동맥관을 페쇄하고, 흉관을 삽입하지 않은 수술 방법의 단기성적을 조사하였다. 대상 및 방법: 2006년 3월부터 2008년 11월까지 동맥관의 의과적 폐쇄술을 시행한 재태기간 30주 이하의 미숙아 24명을 대상으로 하였다. 수술은 모두 신생아 중환자실에서 시행하였고 환아를 엎드려 누윈 상태에서 좌흉부를 30도 올려주는 체위를 취하고 견갑골 직하부위에 2 cm크기의 피부절개를 통해 제 3늑간으로 접근하였다. 면봉을 이용하여 벽측늑막 밖에서 조심스럽게 후종격동으로 박리를 시행하여 동맥관 주변조직을 박리 후 클립(Hemoclip)을 이중으로 사용하여 폐쇄 하였다. 늑막층의 손상이 없거나 미세한 경우는 흉관을 삽입치 않고 창상 봉합을 하여 수술을 종료하였다. 결과: 환아의 출생 시 재태기간은 23주에서 30주 사이로 평균 26.5$\pm$2.1주였으며, 수술 시의 평균 연령은 11$\pm$11일(0$\sim$33일)이었고 수술 시 평균 체중은 933$\pm$271그램(570$\sim$1,700그램) 이었다. 18명의 환아가 인도메타신 치료에 실패하고, 6명의 환아는 약물치료의 적응이 되지 않아 수술적 치료를 시행하였다. 모든 환아에서 수술 직후 확인한 단순 흉부 방사선상 의미있는 기흉은 관찰되지 않았으며, 술후 시행한 심초음파에서 2예를 제외하고는 완전한 폐쇄를 확인하였다. 잔존 단락이 있던 예들은 추적 관찰한 심초음파 검사에서 각각 수술 후 5일과 59일에 폐쇄가 확인되었으며 건강하게 퇴원하였다. 8명의 환아가 사망하였으나 수술과 직접적인 관계의 사인은 없었다. 결론: 미숙아의 동맥관 개존증의 수술요법으로 후방 소개흉술은 만족스러운 수술시야를 확보해 동맥관 폐쇄에 어려움이 없으며, 술후 흉관의 삽입을 줄임으로써 안전하고 효과적인 방법으로 여겨진다.

• Journal of Chest Surgery
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• 제33권10호
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• pp.785-791
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• 2000

Left ventricular dysfuction is common in immediate postoperative periods after surgical correction of heart diseases with chronic left ventricular volume overload. We speculated postoperative changes of left ventricular volume and unction in patients with patent ductus osus(PDA) who had underwent surgical repair at ages older than 16 years. Factors influencing postoperative left ventricular volume and function were also analyzed. Material and Method: From August 1989 to August 1999 thirty-siz adult patients with PDA 28 females and 8 males. were enrolled in this study. Their age ranged from 16 years to 57 years(mean :32 years). Types of surgical repair were division with primary closure in 22, division with patch closure in 6, internal obliteration using cardiopulmonary bypass in 4 and ligation in 4. Aortic clamping was combined during surgical repair in 22(61%) and cardiopulmonary bypass was used in 8(22%) Two-dimensional echocardiography studies were performed in 34(94%) preoperatively and in 25(66%) immediate postoperatively to assess postoperative changes of left ventricular internal dimensions. left ventricular volume and ejection fraction. Duration of postoperative follow-up ranged from 1 month to 99 months (mean:22 months) and 10 patients underwent 16 echocardiographic evaluation during this period Result : Preoperative and postoperative left ventricular systolic dimensions(LVIDs) were 42$\pm$8.0mm and 42$\pm$8.3mm left ventricular diastolic dimensions(LVIDd) were 64$\pm$10.0mm and 56$\pm$7.4mm left ventricular end systolic volumes(LVESV) were 62$\pm$19cc (z=1.87$\pm$0.06) and 59$\pm$24cc(z=1.78$\pm$0.08) left ventricular end diastolic volumes(LVEDL) were 169$\pm$40cc(z-1.17$\pm$0.1) and 112$\pm$29cc(z=0.85$\pm$0.1) and ejection fractions(EF) were 66$\pm$6.7% and 48$\pm$12.6% respectively. There were statistically significant differences between preoperative and postoperative values in LVDIDd(p=0.001) LVEDV(p=0.001) and EF(p=0.0001) while no significant difference is LVIDs and LVESV. Postoperative depression of ejcection fraction was significantly related with z-score of preoperative LVESV and LVEDV by univariateanalysis while LVEDV only was significant risk factor for postoperative LV dysfunction by multiple regressioin analysis ($\Delta$LVEF=-13.3-4.62$\times$LVEDV(z), p=0.001) During the follow-up periods ejection fractions become normalized in all except one patients. Conclusion ; Left ventricular function is usually deteriorated after the surgical correction of PDA in adult age and preoperative LVEDV is a major determinant of postoperative LV function.

• Journal of Chest Surgery
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• 제44권6호
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• pp.392-398
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• 2011

Background: The tetralogy of Fallot (TOF) with pulmonary atresia (PA) and a ductus-dependent pulmonary circulation (no major aorto-pulmonary collateral arteries (MAPCAs)) has been treated with staged repair or primary repair depending on the preference of surgeons or institutions. We evaluated the 19-year outcome of staged repair for this anomaly to find out whether our surgical strategy should be changed. Materials and Methods: Forty-four patients with TOF/PA with patent ductus arteriosus (PDA) who underwent staged repair from June 1991 to October 2010 were included in this retrospective study. The patients with MAPCAs were excluded. The average age at the first palliative shunt surgery was $40.8{\pm}67.5$ days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was $3.5{\pm}1.6$ kg (range: 1.6~8.7 kg). A modified Blalock-Taussig (BT) shunt was performed in 38 patients, classic BT shunt in 4 patients, and central shunt in 2 patients. Six patients required concomitant procedures: pulmonary artery angioplasty was performed in 4 patients, pulmonary artery reconstruction in one patient, and re-implantation of the left pulmonary artery to the main pulmonary artery in one patient. Four patients required a second shunt operation before the definitive repair was performed. Thirty-three patients underwent definitive repair at $24.2{\pm}13.3$ months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was $25.4{\pm}13.5$ months (range: 7.6~68.6 months) and their average weight was $11.0{\pm}2.1$ kg. For definitive repair, 3 types of right ventricular outflow procedures were used: extra-cardiac conduit was performed in 30 patients, trans-annular patch in 2 patients, and REV operation in 1 patient. One patient was lost to follow-up after hospital discharge. The mean follow-up duration for the rest of the patients was $72{\pm}37$ months (range: 4~160 months). Results: Ten patients (10/44, 22.7%) died before the definitive repair was performed. Four of them died during hospitalization after the shunt operation. Six deaths were thought to be shunt-related. The average time of shunt-related deaths after shunt procedures was 8.7 months (range: 2 days~25.3 months). There was no operative mortality after the definitive repair, but one patient died from dilated cardiomyopathy caused by myocarditis 8 years and 3 months after the definitive repair. Five-year and 10-year survival rates after the first palliative operation were 76.8% and 69.1%, respectively. Conclusion: There was a high overall mortality rate in staged repair for the patients with TOF/PA with PDA. Majority of deaths occurred before the definitive repair was performed. Therefore, primary repair or early second stage definitive repair should be considered to enhance the survival rate for patients with TOF/PA with PDA.

• Journal of Chest Surgery
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• 제35권6호
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• pp.430-438
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• 2002

저체중은 많은 심기형의 수술에 있어 위험인자로 알려져 있다. 이에 저체중 출생환아에 있어서의 동맥관 개존증을 제외한 여러 심기형에 대한 외과적 치료의 결과를 조사하였다. 대상 및 방법： 1994년 9월부터 2002년 2월까지 31명의 저체중 출생아를 대상으로 하였다. 심폐기를 이용한 개심술 환아(OHS군)가 12명, 비개심술환아(CHS군)가 19명이었다. 이들 환아에 대하여 술 후 중단기 성적을 알아보기 위해 환아의 병력지를 기초로 후향적 조사를 시행하였다. 태내주수는 36.9주(32.3∼42 주)였고 수술당시 평균 나이는 32.1일(0∼87 일), 출생시 체중은 1972g(1100∼2500g), 수술시 체중은 2105g(1450∼2500g)으로 OHS군과 CHS군에 차이가 없었다. 심기형은 OHS군에서는 심실중격결손증(VSD) (n=3), VSD와 대동맥궁이상(n=2), 총폐정맥환류이상(n=2), 대혈관전위증(n=2), 동맥간증(truncus arteriosus)(n=2), 삼심방증을 동반한 단심실증(n=1) 등이었고 CHS군은 대동맥축착증(n=7), 활로 4징증(n=3),활로 4징증 및 폐동맥폐쇄증(n=3), 다발성 근성 심실중격결손(n=1), 양대혈관 우심실기시증(n=1), 온전한 심실중격이 폐동맥판폐쇄증(n=2), 삼첨판폐쇄증(n=1), 대혈관전위증 및 다발성 심실중격결손(n=1) 등이었다. 13명(41.9%)의 환아에서 술 전 인공호흡이 필요하였다. 결과： 전체적으로 4례의 조기사망(30일 이내)이 발생하였다. OHS군에서 1례(8.3%), CHS군에서는 3례(15.8%)였다. 이 조기사망의 모든 예가 폐동맥교약술을 한 환아였고 완전교정이 가능하였던 환아나 폐동맥교약술을 제외한 고식적 수술에서는 수술사망이 없었다. 지연흉골봉합이 3례에서 필요하였고 술 후 7일 이상 장기간 인공호흡이 필요하였던 경우가 OHS군과 CHS 군에서 각각 7례로 58.3%, 38.8%의 빈도였다. 만기사망이 3례 발생하였는데 이 중 2례는 심장과 관련이 없는 사망이었다. OHS군 1명에서 술 후 뇌합병증이 발견되었다. 2명을 제외한 모든 생존자에서 현재 NYHA class I의 상태로 성장하고 있다. 결론： 저체중 출생 환아에서의 심기형은 완전교정술이 가능한 경우와 폐동맥교약술을 제외한 고식적 수술이 필요한 경우 낮은 수술사망률로 교정될 수 있었으며 중기성적도 양호하였다. 그러나 술 후 비교적 높은 빈도에서 장기간의 인공호흡이 필요하였다.