• Journal of Chest Surgery
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• v.42 no.5
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• pp.639-644
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• 2009

Characterized by unique phenotypic features such as aortic aneurysm/dissection, hypertelorism, bifid uvula/cleft palate and generalized tortuosity in the arterial system, Loeys-Dietz syndrome is a newly described aggressive connective tissue disorder associated with mutation in the gene encoding transforming growth factor-$\beta$ receptor type I or type II. Some phenotypic manifestations of Loeys-Dietz syndrome overlap with those of Marfan syndrome or Ehlers-Danlos syndrome type IV. However, due to its more malignant pathophysiologic nature, physicians should be alert to Loeys-Dietz syndrome. High suspicion, early diagnosis, preventive surgery and serial imaging assessments are warranted for optimal management of Loeys-Dietz syndrome. We present here a case of a young patient with Loeys-Dietz syndrome who had aortic rupture, bifid uvula and hypertelorism. We also present a review of the medical literature.

• Proceedings of the Korean Nutrition Society Conference
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• 2003.10a
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• pp.91-91
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• 2003

• Journal of Chest Surgery
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• v.35 no.6
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• pp.439-448
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• 2002

Pulmonary artery banding (PAB) in the functional univentricular heart (UVH) is a palliative procedure for staging toward the Fontan procedure; however, it is known to be a risk factor. Material and method： The records of all 37 patients with functional UVHs who underwent surgical palliation using PAB between September 1989 and August 1999 were reviewed retrospectively. We investigated the aortic arch obstruction, the development and progression of subaortic stenosis after PAB, and risk factor of mortality according to surgical method. Result： In 37 neonates and infants with single ventricular physiology, aortic arch obstruction was combined in 7. There were 6 early deaths (16.2%) after PAB and 3 late deaths (8.1%) after Fontan operation. The actuarial overall survival including early mortality at 3 and 5 years were 80.7$\pm$6.6%, 72.2$\pm$8.2% respectively. Among 31 patients who survived PAB, 27 patients (87.1 %) could become candidates for Fontan operation； 22 patients (71.0%) completed Fontan operation with 3 deaths and 5 were waiting bidirectional cavopulmonary shunt(BCPS) or Fontan operation (follow-up mean 4.5 year, minimal 2 year). Subaortic stenosis developed in 8 patients after PAB (8/29, 27.6%)； 3 cases in the patients without arch anomaly (3/22, 13.6%) and 5 in those with arch anomaly (5/7, 71.4%). The subaortic stenosis was managed with Damus-Kaye-Stansel procedure (DKS) in 6 patients without operative mortality and conal septum resection in 2 without long-term survivor. Analysis of risk factors established that aortic arch obstruction was strongly associated with subaortic stenosis (p＜0.001). The only risk factor of late mortality was Fontan procedure without staged palliation by BCPS (p=0.001). Conclusion： PAB is effective as an initial palliative step in functional UVH. And the high risk group of patients with aortic obstruction can undergo effective short-term PAB as an initial palliative step, with subsequent DKS for subaortic stenosis. This strategy, initial PAB and careful surveillance, and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later Fontan procedures.

• Journal of Chest Surgery
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• v.40 no.1 s.270
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• pp.17-24
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• 2007

Background: The aim of this study is to assess the clinical results of various procedures in congenital aortic stenosis. Material and Method: From August 1987 to June 2004, 53 patients of congenital aortic stenosis underwent procedures such as percutaneous balloon valvuloplasty, aortic valvuloplasty, Ross procedure, and aortic valve replacement. The mean age of initial procedures was $8.2{\pm}6.0$ years. Percutaneous balloon valvuloplasty was peformed in sixteen patients, aortic valvulopiasty in thirty two patients, Ross procedure in nineteen patients, and aortic valve replacement in fourteen patients. The mean follow duration was $80.6{\pm}60\;(0{\sim}207)$ months. Result: There was 15.1% (8/53) of early mortality and no late mortality. The six patients with critical aortic stenosis were died of left ventricular dysfunction in early series (before 1 year; 4 cases) and two patients died after the Ross procedure and aortic valve replacement respectively. After percutaneous balloon valvuloplasty, most patients needed reoperations (14/16). Thirteen patients needed reoperation, after aortic valvuloplasty. After Ross procedure, two patients needed reoperation due to aortic regurgitation caused by progressive aortic root dilatation. The actuarial survival rate after Ross procedure at 7 years was 90.5%. Conclusion: In young children before the age of one, percutaneous balloon valvuloplasty was considered as :he safe initial palliative procedure. But children over one year-old, aortic valvuloplasty were the effective procedure. Ross procedure can be preformed safely with good results.

• Journal of Chest Surgery
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• v.34 no.3
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• pp.256-259
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• 2001

식도 결핵은 아주 드문 질환으로 연하곤란과 흉통이 가장 흔한 증상이면 다량의 토형은 드문 것으로 되어 있다. 본원에서는 다량의 토형을 동반한 식도 결핵에 의한 식도 대동맥루를 가진 환자를 지험했다. 4세 남자 환자는 다량의 토혈로 응습실을 통해 입원했다. 내원 당시 응급으로 시행한 내시경 검사상 incisor로부터 25cm 하방에 0.7 cm의 풍부한 혈관성의 육아종성 병변을 발견하고, 응급개흉술로 식도의 종양성 병변에 대해 쐐기 절제술을 시행하였다. 식도의 종양성 병변부위는 대동맥과 심게 유착되어 있었고 식도에서 대동맥쪽으로의 식도루를 이중 결찰했다. 환자는 술후 8일째 갑작스런 흉관을 통한 다량의 출혈과 구토 후 토형이 있어 응급 재 개흉술을 시행하여 대동맥파열과 식도 문합부 파열을 확인하였으나 더 이상의 교정이 불가능하여 사망하였다. 이에 문헌고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• v.39 no.4 s.261
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• pp.289-297
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• 2006

Background: Surgery for Stanford type A aortic dissection shows a high operative mortality rate and frequent postoperative brain injury. This study was designed to find out the risk factors leading to operative mortality and brain injury after surgical repair in patients with type A aortic dissection. Material and Method: One hundred and eleven patients with type A aortic dissection who underwent surgical repair between February, 1995 and January 2005 were reviewed retrospectively. There were 99 acute dissections and 12 chronic dissections. Univariate and multivariate analysis were performed to identify risk factors of operative mortality and brain injury. Resuit: Hospital mortality occurred in 6 patients (5.4%). Permanent neurologic deficit occurred in 8 patients (7.2%) and transient neurologic deficit in 4 (3.6%). Overall 1, 5, 7 year survival rate was 94.4, 86.3, and 81.5%, respectively. Univariate analysis revealed 4 risk factors to be statistically significant as predictors of mortality: previous chronic type III dissection, emergency operation, intimal tear in aortic arch, and deep hypothemic circulatory arrest (DHCA) for more than 45 minutes. Multivariate analysis revealed previous chronic type III aortic dissection (odds ratio (OR) 52.2), and DHCA for more than 45 minutes (OR 12.0) as risk factors of operative mortality. Pathological obesity (OR 12.9) and total arch replacement (OR 8.5) were statistically significant risk factors of brain injury in multivariate analysis. Conclusion: The result of surgical repair for Stanford type A aortic dissection was good when we took into account the mortality rate, the incidence of neurologic injury, and the long-term survival rate. Surgery of type A aortic dissection in patients with a history of chronic type III dissection may increase the risk of operative mortality. Special care should be taken and efforts to reduce the hypothermic circulatory arrest time should alway: be kept in mind. Surgeons who are planning to operate on patients with pathological obesity, or total arch replacement should be seriously consider for there is a higher risk of brain injury.

• Journal of Chest Surgery
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• v.35 no.4
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• pp.267-273
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• 2002

Background: We performed a phase IV clinical trial to examine the usefulness of a continuous infusion of nicardipine hydrochloride to control hypertension in patients with acute aortic dissection. material and Method: Systolic/diastolic blood pressure, and heart rate were monitored before and after the intravenous administration of nicardipine in 31 patients with aortic diseases. The period of nicardipine administration in each patient was from 3 to 14 days. Efficacy was evaluated by determining the average amount of blood pressure reduction on the 3rd day of drug administration. The dosage of another antihypertensive agent was slowly tapered down, and ultimately replaced by the test drug. Result: 28 patients were diagnosed as acute aortic dissection, 2 patients as rupture of the aortic arch aneurysm, and 1 patient as traumatic aortic rupture. Mean age was 53.9 $\pm$ 14.9(29~89) years, and 21 patients(67.7%) were male. 14 patients(32.3%) had complications associated with underlying aortic disease: aortic insufficiency in 7, hemopericardium in 6, acute renal failure in 1, paraplegia in 1, lower extremity ischemia in 1, and hemothorax in 1. The time needed to reach the target blood pressure was within 15 minutes in 16, from 15 to 30 minutes in 10, from 30 to 45 minutes in 3 and from 45 to 60 minutes in 2, and their baseline average systolic, diastolic, and mean arterial blood pressures(mmHg) were 147$\pm$23, 82.3$\pm$ 18.6, and 104 $\pm$ 18, respectively. Average systolic, diastolic, and mean arterial blood pressures(mmHg) on the third day of nicardipine infusion were 119$\pm$ 12, 69$\pm$9, and 86$\pm$8, and they all showed statistically significant decrease(p<0.05). The average systolic, diastolic, and mean arterial blood pressure(mmHg) after the discontinuation of the nicardipine infusion were 119 $\pm$ 15, 71 $\pm$ 14, and 86$\pm$ 13, respectively. No significant difference was observed between the average pressures measured on the third day and those measured after the discontinuation of the nicardipine infusion, and no definite side effects were observed during the study period. Conclusion: Nicardipine hydrochloride was both effective and safe at controlling blood pressure in patients with acute aortic dissection.

• Journal of Chest Surgery
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• v.30 no.10
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• pp.1019-1023
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• 1997

The anomaly which the right pulmonary artery originates from the ascending aorta is a rare and usually fatal form of congenital heart disease. This lesion is often associated with a patent ductus arteriosus. Death frequently occurs in early infancy. Anomalous origin of the right pulmonary artery is much more common than anomalous origin of the left pulmonary artery. The anomalous right pulmonary artery usually arise from the posterior aspect of the ascending aorta close to the aortic valve. We report a 1 month-old infant with right pulmonary artery arising from the ascending aorta, which was corrected successfully by direct anastomosis to the main pul onary artery.

• Journal of Chest Surgery
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• v.30 no.3
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• pp.322-325
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• 1997

We experienced a case of anomalous origin of right pulmonary artery from the ascending aorta associated with pulmonary atresia, ventricular septal defect, absence of left pulmonary artery afld multiple major aortopulmonary collateral artery (MAPCA). At ten month of age, left pulmonary artery creation with unifocalization and right pulmonary artery banding were performed as the Urst stage, followed by coil embolization of right MAPCA 1 month later, and 1 year later, the total correction was done. After total repair, the patient showed good postoperative course and excellent angiographic and hemodynamic results at 1 year follow-up study.

• Journal of Chest Surgery
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• v.33 no.12
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• pp.948-953
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• 2000

일반적으로 개심술에는 많은 양의 실현이 따르게 마련이고 그 결과로 많은 수혈을 하게 되는데, 응혈 이상증, 과민반응, 급성호흡곤란증 등의 수혈부작용과 간염 및 후천성면역결핍증 등의 질병 감염의 위험성, 그리고 종교적인 이유 등으로 최근들어 동종수혈을 줄이고자 하는 노력이 계속되고 있다. 개심술시 동종수혈에 따른 부작용을 피하기 위해, 동종수혈 없이 자가수혈만으로도 성인개심술이 가능한지 연구하였다. 서울대학교 의과대학 흉부외과학 교실에서는 1996년 1월부터 12월까지 1년 간 자가수혈만으로 개심술을 시행한 실험군 126명과 동종수혈을 시행한 대조군 45명을 대상으로 양군을 비교하였다. 양군 간에 환자의 성별, 대동맥 질환을 제외한 기타 질환, 대동맥수술과정을 제외한 단순 및 복잡수술과정, 평균 대동맥 겸자시간, 수술전 및 수술후 1일째와 7일째 혈색소수치 및 적혈구용적, 수술 전후의 protein 수치와 수술후 albumin 수치, 자가수혈량과 혈장증량제 사용량 등에서는 유의한 차이가 없었으나, 연령, 대동맥질환 및 대동맥수술과정, 평균체외순환시간, 수술 후 회수된 피의 양, 수술직후의 혈색소수치와 적혈구용적, 수술 전 albumin 수치, 그리고 3일간 흉관으로 배액된 양에 있어 차이가 있었다. 본 연구를 통해 동종수혈 없이 자가수혈만으로도 큰 차이없이 여러 종류의 성인 개심술을 성공적으로 할 수 있음을 보고한다.