• Journal of Chest Surgery
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• 제34권7호
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• pp.547-551
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• 2001

대동맥궁 재건술시 시행하는 완전순환정지는 술후 신경학적 손상과 관련된다. 저자들은 국소 순환으로 뇌와 심근혈류를 유지하면서 완전순환정지를 시키지 않고 시행한 변형 Norwood술식을 2명에서 시행하였기에 보고한다. 한 명은 체중이 3.1kg인 생후 13일된 여아로서 좌심형성부전증후군의 이형 환자였고 다른 한 명은 생후 38일된 체중 3.4kg의 남아로서 심한 대동맥 발육부전 및 축착증과 좌심실유출로 협착을 동반한 Taussig-Bing 기형이었다. 두 환아 모두 무명동맥에 직접 동맥캐뉼라를 삽관한 다음 저체온 상태에서 무명동맥과 관상동맥 혈류를 유지하면서 Norwood술식을 시행하였으며 두 명 모두 술후 신경학적, 심장 혹은 신기능 합병증은 없었다. 이 방법은 좌심형성부전증후군이나 대동맥 축착증 혹은 단절증과 같은 대동맥궁 기형 환자의 수술시 완전순환정지로 인해 발생될 수 있는 허혈성 손상으로부터 뇌와 심장을 보호할 수 있는 한 방법으로 생각된다.

• 한국정밀공학회지
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• 제24권10호
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• pp.123-130
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• 2007

Hypoplastic left heart syndrome is currently the most lethal cardiac malformation of the newborn infant. Survival following a Norwood operation depends on the balance between systemic and pulmonary blood flow, which is highly dependent on the fluid dynamics through the interposition shunt between the two circulations. The purpose of this study is an optimization of the systemic-to-pulmonary artery shunt. In this study, We used computational fluid dynamic(CFD) models to determine the velocity profile in a systemic-to-pulmonary artery shunt and suggested a simplified method of calculating the blood flow in the shunt based on Ultrasound systems. We analyzed the flow characteristic variations and oscillatory shear index(OSI) due to the anastomosis angle and shunt diameter changing. Four different CFD models were constructed with the shunt sizes ranging from 3 to 3.5mm. The angle between the brachiocephalic trunk(BCT) and the shunt were $30^{\circ}$ and $45^{\circ}$, respectively. When the diameter is 3.0 mm, the oscillatory shear index decreased by 1.2% at $30^{\circ}$ as opposed to at $45^{\circ}$. When the diameter is 3.5 mm, it increased by 18% more at $30^{\circ}$ as opposed to at $45^{\circ}$. When the joint angle is $30^{\circ}$ and the diameter is 3.0 mm, the oscillatory shear index decreased by 4.1% in comparison with the 3.5 mm diameter. When the angle is $45^{\circ}$ and the diameter is 3.0 mm, the index increased by 14.6% in comparison with the 3.5 mm diameter.

• Journal of Chest Surgery
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• 제52권4호
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• pp.232-235
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• 2019

A 2.5-kg neonate with coarctation of the aorta and a small left ventricle experienced a severe pulmonary hypertensive crisis. An emergency pulmonary artery-to-systemic artery shunt was placed to break the positive feedback loop caused by pulmonary hypertension and functional mitral stenosis. This shunt provided immediate relief of suprasystemic pulmonary hypertension and the resultant low cardiac output.

• Clinical and Experimental Pediatrics
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• 제49권9호
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• pp.917-929
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• 2006

Over the last several decades there has been a remarkable change in the therapeutic strategy of congenital heart disease. Development of new tools and devices, accumulations of experience, technical refinement have positively affected the outcome of interventional treatment. Many procedures including atrial septostomy, balloon valvuloplasty, balloon dilation of stenotic vessel with or without stent implantation, transcatheter occlusion of abnormal vascular structure, transcatheter closure of patent arterial duct and atrial septal defect, are now performed as routine interventional procedures in many institutes. In diverse conditions, transcatheter techniques also provide complementary and additive role in combination with surgery. Intraoperative stent implantation on stenotic vessels, perventricular device insertion, and hybrid stage 1 palliative procedure for hypoplastic left heart syndrome have been employed in high risk patients for cardiac surgery with encouraging results. Transcatheter closure of ventricular septal defect has been performed safely showing comparable result with surgery. Investigational procedures such as percutaneous valve insertion and valve repair are expected to replace the role of surgery in certain group of patients in the near future. Continuous evolvement in this field will contribute to reduce the risk and suffering from congenital heart disease, while surgery will be still remained as a gold standard for significant portion of congenital heart disease.

• Clinical and Experimental Pediatrics
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• 제54권5호
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• pp.183-191
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• 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

• Journal of Chest Surgery
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• 제36권8호
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• pp.602-605
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• 2003

Norwood 시술에 있어서 체순환 혈관을 넓히는 데 동종 혈관을 이용한 방법이 많이 알려져 있으나, 동종 혈관은 숫자적으로 제한적이고 구하기 어려운 점이 있다. 본원에서는 생후 23일로 여러 부위의 좌심실 유출로 협착이 있는 남아(2.2 kg)와 생후 9일로 좌심실형성부전증 남아(3.0 kg)에서 우심낭으로 체순환 유출로 모양을 재단하여 사용하였다. 이 방법은 술식이 쉽고 재현 가능성이 높으며 동종혈관처 럼 매우 효과적이다.

• Journal of Chest Surgery
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• 제28권8호
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• pp.759-765
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• 1995

We reviewed our experiences on 33 patients who underwent a bidirectional cavopulmonary shunt[BCPS from February 1992 to July 1994. There were 19 male an 14 female patients, and their weight ranged from 4.4 to 13.3 Kg[mean weight 8.4 $\pm$2.9 Kg . The age ranged from 2 to 55 months [mean age 16.7 $\pm$15.5 months . Their diagnosis included single ventricle group in 16, unbalanced ventricles in 8 whose associated anomalies were double outlet right ventricle, transposition of great arteries and total anomalous pulmonary venous return, tricuspid atresia in 7, hypoplastic left heart syndrome in 1 who underwent a Norwood procedure and double outlet right ventricle with pulmonic stenosis and tricuspid stenosis in 1 who underwent biventricular repair. Among them 10 patients had received other palliative operation before [Norwood procedure 1, pulmonary artery banding 3, modified Blalock-Taussig shunt 6 . The BCPS operations were performed under the cardiopulmonary bypass. 16 patients underwent unilateral BCPS and 17 patients who had bilateral SVC underwent bilateral BCPS. Three patients whose associated anomalies were interruption of IVC underwent total cavopulmonary shunt. There were 5 operative deaths [mortality rate 15.1 % and 2 late deaths. The risk factor for the operation was high mean pulmonary artery pressure [p value<0.05 . The survivors showed good postoperative course and their postoperative oxygen saturation was increased significantly compared to that of preoperative status[p value<0.05 .Conclusively, BCPS operation is effective and safe palliative procedure for the many cyanotic complex congenital anomalies with decreased pulmonary blood flow especialy for the patients who have the high risk factors for Fontan operations.

• Journal of Chest Surgery
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• 제55권2호
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• pp.151-157
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• 2022

Background: We investigated surgical outcomes after the surgical repair of cor triatriatum sinister (CTS). Methods: Thirty-two consecutive patients who underwent surgical repair of CTS from 1993 through 2020 were included in this study. The morphological characteristics, clinical features, and surgical outcomes were described and analyzed. Results: The median age and body weight at operation were 9 months (interquartile range [IQR], 3-238 months) and 7.5 kg (IQR, 5.8-49.6 kg), respectively. There were 16 males (50%). According to the modified Lucas classification, type IA (classical CTS) was most common (n=20, 62.5%). Atrial septal defect was associated in 22 patients (68.8%) and anomalous pulmonary venous return in 8 patients (25%). Pulmonary hypertension was preoperatively suspected with a high probability in 18 patients (56.3%). There was 1 early death (3.1%) after emergent membrane excision and hybrid palliation in a high-risk hypoplastic left heart syndrome patient. There were no late deaths. The overall survival rate was 96.9% at 15 years post-repair. No early survivors required reoperation during follow-up. Most survivors (31 of 32 patients, 96.9%) were in New York Heart Association functional class I at a median follow-up of 74 months (IQR, 39-195 months). At the latest echocardiography performed at a median of 42 months (IQR, 6-112 months) after repair, no residual lesion was observed except in 1 patient who had moderate pulmonary hypertension (mean pulmonary arterial pressure of 36 mm Hg). Conclusion: Surgical repair of cor triatriatum could be performed safely and effectively with an extremely low risk of recurrence.

• Journal of Chest Surgery
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• 제12권3호
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• pp.207-214
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• 1979

This 3-year-old girl was observed frequent exertional dyspnea and cyanosis at crying since birth. She was not premature baby and delivered at full term normally. On physical examination, she was underdeveloped-body weight 13.5 kg, height 99 cm.- and cyanotic. There was severe clubbing on fingers. There was grade II/VI ejection systolic murmur on left lateral border of the sternum. The preoperative examinations [EKG, echocardiogram, cardiac catheterization and biventriculogram] showed that complicated T.G.A. combined vena cava[S.D.D.]. Preoperatively, we decided the corrective surgery of Rastelli operation using a. pulmonary valved conduit. The operation was performed under total circulatory arrest using deep profound hypothermia combining with extracorporeal circulation. On operation, the anatomy of the heart showed that, 1. The subaortic conus was seen and subaortic muscles were hypertrophied. 2. The VSD[type II], behind the subaortic conus-about 1 cm. in diameter, was visible only through LV cavity and, 3. The pulmonary valve ring was hypoplastic and pulmonary valvular stenosis was seen also. The subpulmonic area [LV outflow tract] was obstructed with hypertrophied muscle and mitral valve. 4. Left superior vena cava was drained to RA via coronary sinus. 5. LAD coronary artery was originated from right coronary artery and ran anterior to the pulmonary artery. According to above anatomy, we performed the VSD closure with Teflon patch, and Mustard operation combined with LV-to-pulmonary artery bypass graft using the valve contained [Hancock 16 mm] conduit. Postoperatively, adequate blood pressure could be maintained under the state of using inotropic agent [epinephrine]. On the second postoperative day, the patient died of cardiac arrest due to low cardiac output syndrome, acute renal failure and pulmonary edema.

• Journal of Chest Surgery
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• 제43권4호
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• pp.364-374
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• 2010

배경: 본 연구의 목적은 지난 7년 동안의 좌심형성부전증후군 및 그 변종에 대한 변형 노우드(Norwood) 수술의 조기 및 중기 결과를 알아보고, 조기 병원 사망에 영향을 주는 인자들을 확인하는 것이다. 대상 및 방법: 2003년 5월부터 2009년 12월까지 좌심형성부전증후군 및 그 변종으로 노우드 수술을 시행 받은 23명의 환자들을 대상으로 하였다. 수술 당시 나이는 3일에서 60일이었고, 체중은 2.2 kg에서 4.8 kg의 소견을 보였다. 주폐동맥의 전벽을 보존하는 변형된 술식을 20명의 환자에 있어서 적용하였다. 폐동맥혈류 유지는 15명에서 우심실-폐동맥 도관술, 8명에서 우측변형 브라록-타우시히 단락을 이용하였다. 19명(19/20, 95%)의 환자들에 대하여 평균 $26.0{\pm}22.8$개월 동안 추적관찰을 시행 하였다. 결과: 3명(3/23, 13%)의 조기 병원 사망이 있었다. 이중 2명은 폐정맥연결이상을 동반하고 있었다. 14명의 환자가 양방향성 상대정맥 폐동맥 단락술을 시행 받았고, 7명의 환자가 Fontan 수술을 시행 받았다. 양방향성 상대정맥 폐동맥 단락술 이전에 2명의 사망이 있었고, Fontan 수술이전에 1명의 사망이 있었다. 1년과 5년 장기 생존률은 각각 78%와 69%의 소견을 나타냈다. 이상 우측 쇄골하 동맥과 동반된 전 폐정맥연결이상이 병원 사망과 관련된 위험인자들로 나타났다. 결론: 좌심형성부전증후군 및 그 변종에 대한 변형 노우드(Norwood) 수술은 낮은 수술 사망률로 시행될 수 있었다. 전 폐정맥연결이상의 동반이 노우드 수술 후의 생존에 주요한 위험인자임을 알 수 있었다. 향후 중간단계 사망에 대한 성적향상의 노력이 필요할 것으로 판단된다.