• Journal of Chest Surgery
• /
• 제26권9호
• /
• pp.701-704
• /
• 1993

Four to seven percent of infants born with aortic atresia have a normal-sized left ventricle in association with a ventricular septal defect and a normal mitral valve. In contrast to the more common group of infants with aortic atresia whose left ventricle is hypoplastic, this important subgroup has potential for complete operative correction involving both the right and left ventricle. Our approach was to perform complete repair at one stage. The patient who underwent single-stage repair was discharged from the hospital in good condition. Achieving a physiologically normal circulation simplifies the postoperative management of this condition.

• Journal of Chest Surgery
• /
• 제20권2호
• /
• pp.404-410
• /
• 1987

An 18-day-old male neonate with hypoplastic left heart syndrome underwent surgical intervention by modification of the Norwood procedure on September 23, 1986. Hypoplastic left heart syndrome is a serious congenital cardiac anomaly that has a fatal outlook if left untreated. Included in this anomaly are [1] aortic valve atresia, and hypoplasia of the ascending aorta and aortic arch, [1] mitral valve atresia or hypoplasia, and [3] diminutive or absent left ventricle. Patent ductus arteriosus is essential for any survival, and there is usually a patent foramen ovale. Coarctation of the aorta is frequently associated with the lesion.z With a limited period of cardiopulmonary bypass, deep hypothermia, and circulatory arrest, the ductus arteriosus was excised. The main pulmonary artery was divided immediately below its branches, and the distal stump of the divided pulmonary artery was closed with a pericardial patch. The aortic arch was incised, and a 1 5mm tubular Dacron prosthesis was inserted between the main pulmonary artery and the aortic arch. A 4mm shunt of polytetrafluoroethylene graft was established between the new ascending aorta and the right pulmonary artery to provide controlled pulmonary blood flow. Following rewarming, the heart started to beat regularly, but the patient could not be weaned from cardiopulmonary bypass. At autopsy, the patient was found to have hypoplasia of the aortic tract complex with mitral atresia and aortic atresia. A secundum atrial septal defect was noted. Right atrial and ventricular hypertrophy was present, and the left ventricle was entirely absent. Although unsuccessful in this case report, continuing experience with hypoplastic left heart syndrome will lead to an improvement in result.

• Journal of Chest Surgery
• /
• 제37권10호
• /
• pp.856-860
• /
• 2004

심내기형을 동반한 대동맥궁 단절은 매우 중한 자연경과를 갖고 있는 희귀한 선천성 심장 기형이다. 식도 폐쇄증과 기관 식도루를 동반한 심첨부 근육성 심실 중격 결손과 대동맥궁 단절을 생후 3일된 체중 2.6 kg의 신생아에서 단계적 수술법으로 치료한 경험을 보고한다. 1차 수술로서 우측 개흉술을 통한 식도 폐쇄증의 교정과 함께 좌측 개흉술에 의한 대동맥궁 광범위 단-단 문합술 및 폐동맥 교약술을 시행하였다. 1차 수술 후 87일째 정중 흉골 절개를 통해 심첨부 근육성 심실 중격 결손을 폐쇄하였다. 심실 중격 결손 폐쇄 전 유문부 근육절개술, 대동맥 전방고정술, 대동맥 풍선확장술 등의 추가 시술이 필요하였다. 최종 수술 후 3개월째 양호한 추적 결과를 경험하였기에 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제34권11호
• /
• pp.870-874
• /
• 2001

Takayasu 동맥염은 만성 비특이성 동맥염으로 젊은 여자에 호발하며 대동맥궁의 분지 혈관이나 신동맥 등 여러 혈관들을 광범위하게 침범하여 폐쇄를 일으키는 질환으로 알려져 있다. 최근 나이가 많은 52세의 여자 환자에서 신동맥 이하의 복부 대동맥에만 국한적으로 침범하여 삭did(cord-like) 폐쇄를 일으킨 Takayasu 동맥염의 비교적 드문 예를 경험하고 18mm PTFE(polytetrafluoroethylene) 인조혈관을 이용하여 하행 흉부 대동맥과 총장골동맥 분기 직상부 복부 대동맥 사이에 측단 우회술을 시행하여 좋은 결과를 얻었기에 보고한다.

• Clinical and Experimental Pediatrics
• /
• 제45권2호
• /
• pp.267-272
• /
• 2002

저자들은 재태 $31^{+5}$주 산전 심초음파검사에서 좌심형성부전 및 대동맥축삭으로 진단되어 추적 관찰한 태아가 출생 후 전부하의 증가에 의해 단기간 내에 좌심실 용적의 증가를 보임으로 생후 최소 며칠은 심근세포 증식의 가능성이 있음을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제34권9호
• /
• pp.720-723
• /
• 2001

우관상동맥 폐쇄증을 동반한 Marfan 증후군환자를 보고한다. 환자는 45세 여자로 약 1년 전에 Marfan 증후군으로 진단받고, 최근 흉통을 주소로 내원하였다. 환자는 손가락과 발가락이 긴 지주증과 돌출흉이 있고 몸통에 비해 사지가 긴 전형적인 외형을 갖고 있었다. 시력이 매우 약하고 수정체의 아탈구가 있었다. 심초음파상 대동맥판막 폐쇄부전증은 경도로 있었고, 좌심실의 중등도 확장과 심박출율의 중등도 저하가 있었다. 수술시에 우관상동맥의 폐쇄증을 발견하였다. 대동맥판막 폐쇄부전증과 대동맥 확장증에 대해서 SJM 27mm composite graft를 이용하여 좌관상동맥에는 Bentall 술식과 우관상동맥에는 PTFE 6mm를 이용하여 변형된 Piehler 식 방법으로 수술을 하였다. 원래의 우관상동맥구로 추정되는 부분부터 우관상동맥 원위부의 혈류가 있는 곳까지의 결손부위는 약 4cm 가량 되었다. 관상동맥 폐색증과 Marfan 증후군의 조합은 매우 드문 질환으로 양측 관상동맥의 혈류 재건을 위하여 각기 다른 술식으로 수술하였다.

• Journal of Chest Surgery
• /
• 제20권4호
• /
• pp.659-672
• /
• 1987

Nitrofen [2,4-dichlorophenyl-P-nitrophenyl ether] is a diphenyl ether herbicide used for pre and post-emergent control of broad leafed weeds. This chemical was known to induce a variety of congenital cardiovascular anomalies with diaphragmatic hernia and hydronephrosis in the rate fetuses. The present study was conducted to produce congenital cardiovascular anomalies in the rat fetuses by oral nitrofen administration at the indicated doses and days of gestation, and to find the characteristics of nitrofen-induced cardiovascular anomalies. All the observed fetuses were removed from the pregnant Sprague-Dawley rats sacrificed on the twenty-first day of gestation. They were preserved in 10 per cent formalin and dissection for examination were carried out under a dissecting microscope using forceps and scissors. Following results and conclusion were based on dissecting microscopic findings on 482 offsprings. 1. The eleventh day of gestation was the most sensitive day for nitrofen induction of congenital cardiovascular anomalies in the rat. This incidence was dose-related in rats exposed on the eleventh day of gestation. 2. Ventricular septal defect was the most common single anomaly that represented more than half of the total cardiovascular anomalies, followed by aortic arch anomalies and tetralogy of Fallot. 3. Cardiac anomalies derived from infundibular maldevelopment such as tetralogy of Fallot and pulmonary atresia with ventricular septal defect were only observed in the eleventh gestation day treated group. 4. Aortic arch anomalies were found in high frequency and the great majority were characteristically anomalous right subclavian artery with left aortic arch. Key words; nitrofen, congenital cardiovascular anomalies.

• Journal of Chest Surgery
• /
• 제25권4호
• /
• pp.406-411
• /
• 1992

Thirty-four patients were received bidirectional cavopulmonary shunt[BCPS] from Aug. 1989 to Apr. 1991 at Sejong General Hospital, Puchon, Korea. Their ages were from 43 days to 21 years old with 19 cases of infant, 10 from 1 to 5 years old and 5 cases above 6 years old. Their diagnoses were as follows: 13 cases with uni-ventricular heart, 9 tricuspid atresia, 6 double outlet of right ventricle, 4 pulmonary atresia with intact ventricular septum, and 2 transposition of great arteries with pulmonary stenosis. Among them, 10 patients had received other palliative operations before. The BCPS operations were performed under the cardiopulmonary bypass and 10 patients who had bilateral superior vena cava received bilateral BCPS. Other associated procedures were 9 cases of takedown of Blalock-Taussig shunt, 3 pulmonary artery angioplasty, 1 unifocalization, 1 repair of total anomalous pulmonary venous return, 1 Damus procedure, 1 relief of sub-aortic stenosis, 1 right ventricular outflow tract reconstruction and one case of tricuspid valve obliteration. There were 3 operative deaths[8.8%] and two late deaths. The remainders show good postoperative state and their oxygen saturation was increased significantly. Conclusively, the bidirectional cavopulmonary shunt is very effective and safe palliative or pre-Fontan stage operation for the many complex congenital anomalies with low pulmonary blood flow especially for the patients who have the risk of Fontan repair.

• Journal of Chest Surgery
• /
• 제26권3호
• /
• pp.180-185
• /
• 1993

From February 1982 to December 1991, 49 neonates and 105 infants in less than 3 months of age underwent open heart surgery in Seoul National University Hospital. There were 98 males and 56 females, and their mean ages were 16 days in neonatal group and 67 days in early infant group. Their body weight and height were less than 3 percentile of normal developmental pattern. In order of decreasing incidence, the corrected conditions included Transposition of great arteries with or without ventricular septal defect [43], isolated ventricular septal defect [34], Total anomalous pulmonary venous return [21], Pulmonary atresia with intact ventricular septum [9] and others [47]. Various corrective or palliative procedures were performed on these patients; Arterial switch operation [36], patch closure for ventricular septal defect [34], Repair of total anomalous pulmonary venous return [21], RVOT reconstruction for congenital anomalies with compromised right ventricular outflow tract [17]. Profound hypothermia and circulatory arrest were used in 94 patients [ 61% ]: 42 patients [ 85.7% ] for neonatal group and 52 patients [ 49.5% ] for early infant group. The durations of circulatory interruption were within the safe margin according to the corresponding body temperature in most cases [ 84% ]. The hospital mortality was 36.4% ; 44.9% in neonatal group and 32.4% in infant group 1 to 3 months of age. The mortality was higher in cyanotic patients [ 46.6% ], in those who underwent palliative procedures [ 57.8% ], in patients whose circulatory arrest time was longer than safe periods [ 60% 0] and in patients who had long periods of cardiopulmonary bypss and aortic crossclamping. In conclusion, there has been increasing incidence of open heart surgery in neonates and early infants in recent years and the technique of deep hypothermia and circulatory arrest was applied in most of these patients, and the mortality was higher in cyanotic neonates who underwent palliative procedures and who had long cardiopulmonary bypass , aortic cross-clamping and circulatory arrest.

• Journal of Chest Surgery
• /
• 제44권2호
• /
• pp.108-114
• /
• 2011

Background: Homograft cardiac valves and valved-conduits have been available in our institute since 1992. We sought to determine the long-term outcome after right ventricular outflow tract (RVOT) reconstruction using homografts, and risk factors for reoperation were analyzed. Materials and Methods: We retrospectively reviewed 112 patients who had undergone repair using 116 homografts between 1992 and 2008. Median age and body weight at operation were 31.2 months and 12.2 kg, respectively. The diagnoses were pulmonary atresia or stenosis with ventricular septal defect (n=93), congenital aortic valve diseases (n=15), and truncus arteriosus (N=8). Mean follow-up duration was $79.2{\pm}14.8$ months. Results: There were 10 early and 4 late deaths. Overall survival rate was 89.6%, 88.7%, 86.1% at postoperative 1 year, 5 years and 10 years, respectively. Body weight at operation, cardiopulmonary bypass (CPB) time and aortic cross-clamping (ACC) time were identified as risk factors for death. Forty-three reoperations were performed in thirty-nine patients. Freedom from reoperation was 97.0%, 77.8%, 35.0% at postoperative 1 year, 5 years and 10 years respectively. Small-sized graft was identified as a risk factor for reoperation. Conclusion: Although long-term survival after RVOT reconstruction with homografts was excellent, freedom from reoperation was unsatisfactory, especially in patients who had small grafts upon initial repair. Thus, alternative surgical strategies not using small grafts may need to be considered in this subset.