• Journal of Chest Surgery
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• 제29권11호
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• pp.1191-1196
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• 1996

최근에는 폐동맥고혈압이 있는 심실중격결손증에서는 환아의 체중이 10Kg이하이거나 1세 이하라도 조기에 수술을 하는 것이 추천되고 있으며 본원에서도 같은 방침으로 개심술을 시행하여 왔다. 본원에서는 1986년 10월부터 1995년 12월까지 체중이 10Kg이하인 심실중격결손증환자 88명을 개심술로 수술하여 그 사망률을 환아의 연령, 체중 그리고 폐동맥고혈압의 정도에 따라 비교하였다. 총 사망자 수는 10명이었고(11.4%), 6개월 이하의 환아 수술에서는 14명중 5명이 사망하였고(35.8%), 6개월에서 12개월 사이에서는 43명중 4명이 사망하였고(9.3%), 1년 이상된 환아의 수술에서는 31명중 1명이 사망하였다(3.2%). 그러나 폐동맥고혈압의 정도에 의해 사망률을 비교한 결과는 큰 차이를 보이지 않았다. 그리고 6개월 이하의 수술례에서는 폐동맥고혈압의 정도에 관계없이 사망율이 높았다. 이상의 결과로 10Kg 이하의 저체중 심실중격결손증의 수술은 극심한 심부전이나, 성장부전이 있거나 폐동맥고혈압이 비가역적인 폐혈관 질환을 야기할 정도가 아니면, 적어도 6개월 이상 지나서 시행하는 것이 좋을 것으로 사료된다.

• Journal of Chest Surgery
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• 제46권2호
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• pp.98-103
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• 2013

Background: Atrial fibrillation (AF) is a common complication in elderly patients with atrial septal defect (ASD). The purpose of this study was to examine the efficacy of the maze procedure in these patients. Materials and Methods: Between February 2000 and May 2011, 46 patients underwent the maze procedure as a concomitant operation with ASD closure. Three patients who underwent a right-sided maze were excluded, and one patient was lost to follow-up. The mean follow-up duration was $3.2{\pm}2.5$ years. Electrocardiography was performed 1 month, 3 months, 6 months, and 1 year after surgery, and checked annually after that. Results: AF persisted in 4 patients after surgery. One year after surgery, among 38 patients, 55.3% remained in sinus rhythm without antiarrhythmic drugs. However, when including the patients who took antiarrhythmic drugs, 92.1% were in sinus rhythm. Freedom from AF recurrence at 3 months, 6 months, 1 year, 2 years, 3 years, and 5 years after surgery were $97.4{\pm}2.6$, $94.4{\pm}3.8$, $91.2{\pm}4.9$, $87.8{\pm}5.8$, $79.5{\pm}7.6$, and $68.2{\pm}12.4$, respectively. There was no early mortality after operation. Conclusion: Concomitant treatment with the maze procedure and ASD closure is safe and effective for restoring the sinus rhythm.

• Journal of Chest Surgery
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• 제43권1호
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• pp.58-62
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• 2010

심실 중격 결손과 폐동맥 협착을 가지고 있는 대혈관 전위 환자의 수술적 치료방법으로 Rastelli 수술법이 보편화되어 왔으나, 추적 관찰 중에 심장외 도관의 폐쇄, 좌심실 유출로의 폐쇄, 부정맥이 높은 확률로 발생하는 등의 장기 성적이 만족스럽지 못하였다. 이와 같은 문제점을 해결하기 위해 본원에서 2003년과 2006년에 심실 중격 결손과 폐동맥 협착을 가지고 있는 대혈관 전위 환자 2명을 반회전 동맥간 회전술 및 Lecompte 술식을 이용하여 수술하였고, 추적 관찰을 통해 양심실 유출로의 중기 변화에 대한 경험을 보고한다.

• Journal of Chest Surgery
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• 제25권1호
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• pp.32-41
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• 1992

From August 1982 to December 1991, 58 consecutive infants with tetralogy of Fallot underwent primary repair. Age ranged from 22 days to twelve months [n=58, 8.7$\pm$2.7 months] and body weight from 3.1 to 13 kilograms [n=58, 7.8$\pm$1.7 kilograms]. Qne infant had absence of the pulmonary valve; one had Ebstein`s anomaly and one had supramitral ring. Thirty-two patients [56%] experienced anoxic spell. Preoperative pulmonary artery indices were measured in 38 cases, ranging 126-552mm2/M2BSA[n=38, 251$\pm$79mm2/M2BSA]. All infants required a right ventricular outflow tract patch; in 41, the patch extended across the pulmonary valve annulus, in 13 of them, monocusps were constructed. All had patch closure of ventricular septal defect. Two infants had REV operation for avoiding injury to the canal branch of the right coronary artery which cross the right ventricular out flow tract. Post repair PRV/LV were measured at operating room in 40 cases, which revealed mean value of 0.49$\pm$0.12 [range: 0.25-0.74]. The hospital mortality was 10.3% [6 patients], and causes of deaths were right heart failure due to sustained right ventricular hypertension[4] and right ventricular outflow tract obstruction, intractablesuraventricular tachyarrhythmia[1], hypoxia[1] due to residual right to left shunt across the atrial septal defect in patient associated with Ebstein`s anomaly. All infants were doing well at follow-up from 1 to 101 months[20.6 months /patient, 1, 072 patient-month] Serial postoperative echocardiograms revealed no residual ventricular septal defects and estimated RVOT gradients between 0 and 40 mmHg except 3 cases [50, 50, 60 mmHg]. There were no late deaths and late ventricular arrhythmias or congestive heart failure. Redo operations were done in 2 cases because of residual right ventricular outflow tract obstruction. This experience with infants with tetralogy of Fallot suggests that, if mortality is tolerable, eletive repair of tetralogy of Fallot could be reasonably undertaken during the first year of life, and even better results could be anticipated along with improvement of methods of myocardial protection and postoperative care.

• Journal of Chest Surgery
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• 제19권4호
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• pp.627-632
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• 1986

This report describes our 17-years experience with intracardiac repair in 123 patients older than 15 years with tetralogy of Fallot. Major clinical manifestation was cyanosis and clubbing [102 Pts], but other minor associated manifestation were infective endocarditis, pulmonary tuberculosis, brain abscess, congestive heart failure, nephrotic syndrome, and tuberculous spondylitis. Prior palliative shunts had been performed in 10 patients. Preoperative hemoglobin ranged from 9.7 gm/dl to 25 gm/dl [mean 19 gm/dl]. The type of ventricular septal defect were typical perimembranous type, and total canal defect [13%]. The right ventricular outflow tract obstruction was due to combined [58.5%], infundibular [35%], and valvular stenosis [6.5%]. Transannular patch was used in 17% of patients. Hospital mortality was 9.8% in overall, but decreased to 1.7% since 1982. There was two late death [12 year actuarial survival [97%] due to fulminant hepatitis, residual abnormalities [PS, VSD]. Ninety two percent of survivors at follow-up are asymptomatic and leading an active normal life. Residual ventricular septal defect was detected with radionuclide single pass study in 15.3% of patients but almost cases were Qp/Qs less than 1.5, and only two patients had been candidates for reoperation.

• Journal of Chest Surgery
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• 제23권3호
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• pp.430-437
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• 1990

We clinically evaluated 121 cases of ventricular septal defect which we operated from April, 1986 to December, 1989 at Inha General Hospital, Seong-Nam, Department of Thoracic and Cardiovascular Surgery, College of Medicine, Inha University. These patients were occupied 54.8% of all congenital heart diseases operated on its same period. Of the 121 patients, 63 patients were male[52.1%] and 58 patients were female[47.9i]. The two most common symptoms were frequent upper respiratory infection and dyspnea on exertion. By Kirklin s anatomical classification, type I constituted 34.7%, type II 61.98%, type III 0.03% and type IV not occupied. Associated cardiac anomalies were found in 34 cases, and PDA was most common associated anomaly, occupied in 22 cases. On the cardiac catheterization data, there were statistically significant correlation between VSD size[cm2 /BSAm2] and systolic pulmonary arterial pressure[sPAP], pulmonary to systemic flow ratio[Qp/Qs] & pulmonary to systemic pressure ratio[Pp/Ps] respectively, Type II [r=0.53, p<0.01] was more correlated than type I [r=0.49, p<0.05] between VSD size and Qp /Qs. We could not found the correlationship between age and Qp/Qs [Type I; r=0.16, Type II; r=-0.15] All cases were operated under cardiopulmonary bypass and 58 cases[46.3%] were operated through the right atrial approach, and 34 cases[28.1%] through the pulmonary arterial approach. Operative mortality rate was 4.13%[5 cases].

• Journal of Genetic Medicine
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• 제18권2호
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• pp.147-151
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• 2021

The Say-Barber-Biesecker-Young-Simpson variant of Ohdo syndrome (SBBYSS) (Online Mendelian Inheritance in Man #603736) is a rare autosomal dominant disorder and clinically features blepharophimosis with ptosis, a mask-like facial appearance, cryptorchidism, congenital heart defect, long thumbs/great toes, and thyroid dysfunction. The etiology of SBBYSS has been shown to be due to heterozygous KAT6B gene mutation. Here we report a case of a neonate with SBBYSS identified a novel mutation in KAT6B gene. The patient showed typical dysmorphic facies, cryptorchidism with micropenis, overriding fingers, and long thumbs and toes at birth. He had also hypothyroidism, large atrial septal defect, and sensorineural hearing loss. The next generation sequencing identified a heterozygous novel variant, c.5206C>T (p.Gln1736Ter) in KAT6B gene. At the 9 months of age, he underwent patch closure for atrial septal defect. Until the 12-month follow-up, he was under-developed.

• Journal of Chest Surgery
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• 제39권12호
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• pp.906-912
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• 2006

배경: 급성 심근경색 후 발생하는 심실중격결손은 드문 치명적인 합병증이다. 수술만이 유일한 치료법이나, 그 수술사망률은 아직도 높다. 저자들은 수술 환자들의 수술 결과와 조기사망의 원인을 알아보았다. 대상 및 방법: 1993년 8월부터 2006년 2월까지 급성 심근경색 후 발생한 심실중격결손 환자 8예(남 2예, 여 6예)를 수술하였다. 그중 7예가 좌전하행지의 단혈관질환을 가지고 있었고, 6예는 전중격뿐만이 아니라 후중격까지 넓은 경색을 가지고 있었다. 1예에서 경색과 무관한 관상동맥우회술을 시행하였으며 2예에서 삼첨판 성형술을, 1예에서 승모판 성형술을 같이 시행하였다. 결과: 수술 사망은 3예(37.5%)였다. 사망 환자들은 모두 경색의 범위가 심실의 전중격뿐만이 아니라 후중격까지 넓게 퍼져 있었고 초음파 M-mode 검사에서 좌심실 기저부의 박출률이 생존자들보다 상대적으로 낮았다($34.9{\pm}4.2\;vs.\;54.8{\pm}12.3$; p=0.036). 결론: 심근경색증에 의한 급성 심실중격결손은 대부분 단혈관질환에서 발생하였다. 심장초음파 검사상 좌심실 기저부의 박출률이 낮고 심실중격의 경색 범위가 넓어 후중격까지 진행된 환자에서 조기 사망률이 높았다.

• Journal of Chest Surgery
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• 제37권6호
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• pp.536-538
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• 2004

선천성 심기형과 흉벽기형이 동반되어 있는 경우 이 두 질환을 동시에 수술하는 경우에 변형된 늑연골의 광범위한 절제에 따른 합병증이 단점으로 지적되고 있다. 본원 흉부외과에서는 부분 방실중격 결손증과 오목가슴이 동반된 8세 환아에서 개심술 시에 Nuss 술식을 이용하여 오목가슴을 교정함으로써 광범위한 늑골절제에 따른 수술시간의 지연이나 출혈, 흉벽의 불안정성 등의 단점없이 좋은 결과를 얻었기에 이를 보고하는 바이다.

• Journal of Chest Surgery
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• 제22권4호
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• pp.620-629
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• 1989

A clinical analysis of 138 cases of ventricular septal defect operated on from 1983 to June 1988 at the department of Thoracic and Cardiovascular Surgery, School of Medicine, Chung-nam National University was done. The following results were obtained. 1. The VSD were 27.6 % and 41.0 % respectively of 500 open heart surgery cases and 334 congenital heart disease. 2. The range of age when the VSD was repaired was 3 months to 45 years, the mean age was 10.4 years and the 53.6 % of patients were under 7 years. The sex ratio was 6:4 in males favor. 3. The two common symptoms were frequent upper respiratory infection and dyspnea whose frequency was 62.5 % and 58.7% respectively. 4. 52% of the patients was below 25 percentile of the standard body weight. 5. The most common chest PA findings were cardiomegaly and increased pulmonary vascularity. 6. On anatomical classification, perimembranous type was most common [65.5%], subpulmonary arterial type was 27.6 %, perimembranous plus subpulmonary arterial type was 3.4 % multiple VSD was 0.8 % and LV-RA defect was 2.6 %. 7. Associated cardiac anomalies were founded in 39 cases [35.5 %] and PDA and aortic valve prolapse were most common associated anomalies and extracardiac anomalies were founded in 6.7 % of patients. 8. Mean extracorporeal circulation time was 68 min. and mean aortic cross-clamping time was 42 min. 9. The postoperative complications developed in 26 % of patients and the most common one was minor wound infection. 10. Right bundle branch block developed in 54.8% of the patients who had right ventriculotomy, 40.0% of the patients who had right atriotomy and 10.5% of the patients who had pulmonary arteriotomy. 11. The overall operative mortality was 5.1 % and the operative mortality of the patients who body weigh under 10 Kg was 23.8%.