• Clinical and Experimental Pediatrics
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• 제48권11호
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• pp.1256-1256
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• 2005

심실중격결손을 동반하지 않은 폐동맥 폐쇄(pulmonary atresia with intact ventricular septum)는 전체 선천적 심장병의 1%를 차지하는 드문 질환으로 형태학적 특성에 따라 다양한 치료적 접근이 필요하다. 이에 저자들은 정상 크기발달의 우심실을 가진 심실중격결손을 동반하지 않은 폐동맥 폐쇄가 있는 신생아에서 풍선판막성형술 후 지속적인 저산소증을 보여 동맥관 스텐트를 이용하여 거의 정상적인 심장구조와 기능으로 호전된 1례를 경험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제31권8호
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• pp.816-819
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• 1998

점액종은 심장 종양의 가장 흔한 형태이며 주로 좌심방에서 발견된다. Carney 등에 의하면가 족성을 가지 며 점액종(심장, 피부, 유방), 반점상의 피부 색소침착와 내분비성 질환이 동반된 증후군을 점액종 증후군 이라 명명하였다. 저자들은 얼굴과 좌완에 피부병변이 있고 좌심방과 우측 유두에서 점액종이 발견되고 가 족력이 있는 19세 여자를 Carney 증후군을 가진 가족성 심방 점액종으로 진단하여 좌심방 중격과 승모판에 부착된 점액종을 성공적으로 절제하였다. 심장 점액종의 가족성이 있는 동일 가족 구성원은 증상이 발현되 지 않더라도 심장 점액종을 발견하기 위해 선별적 심방초음파 검사가 주기적으로 실시되어야하며 재발과 다 발성병변이므로 수술시 종양의 완전 절제와 수술후 추적검사가 필요하다.

• Journal of Chest Surgery
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• 제27권4호
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• pp.259-265
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• 1994

Cor triatriatum is a relatively rare cardiac anomaly, whose major feature is a fenestrated membrane separating an upper common pulmonary venous chamber from a lower true left atrial cavity. Interatrial communications may be present between the right atrium and the common pulmonary venous chamber or the true left atrium. From April 1981 to April 1992, 24 patients with cor triatriatum were treated at Seoul National University Hospital. Ages ranged from 1 month to 24 years with mean of 7.4 years. Twenty patients had interatrial communications through a patent foramen ovale, primum or secundum defect of the atrial septum. Four had no interatrial communications. Fourteen patients had associated anomalies; partial anomalous pulmonary venous connection in 3, total anomalous pulmonary venous connection in 2, persistent left superior vena cava in 3, and other anomalies in 6 patients. Surgical corrections were performed through right atriotomy in 18 patients, left atriotomy in 4, and both atriotomy in 2. Three patients [12.5%] died early after operation; two of them were associated with single ventricle. Six out of 21 survivors [28.6%] experienced complications; recurrent pneumonia, pulmonary embolism, ischemic encephalopathy, diaphragmatic palsy and tachyarrhythmias. At the time of follow up, all survivors, except one, were in functional class I. Surgical correction of cor triatriatum restored normal hemodynamic status with relatively low operative mortality, especially in patients not complicated with severe anomalies. This report summarizes the clinical diagnosis, associated anomalies, interatrial communications, surgical approach and late result of 24 patients underwent surgical corrections in our hospital.

• Korean Circulation Journal
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• 제52권7호
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• pp.513-526
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• 2022

Background and Objectives: Atrial tachycardias (ATs) from noncoronary aortic cusp (NCC) uncovered after radiofrequency ablation for atrial fibrillation (AF) are rarely reported. This study was conducted to investigate the prevalence and clinical characteristics of NCC ATs detected during AF ablation and compare their characteristics with de novo NCC ATs without AF. Methods: Consecutive patients who underwent radiofrequency catheter ablation for AF were reviewed from the multicenter AF ablation registry of 11 tertiary hospitals. The clinical and electrophysiological characteristics of NCC AT newly detected during AF ablation were compared with its comparators (de novo NCC AT ablation cases without AF). Results: Among 10,178 AF cases, including 1,301 redo ablation cases, 8 (0.08%) NCC AT cases were discovered after pulmonary vein isolation (PVI; 0.07% in first ablation and 0.15% in redo ablation cases). All ATs were reproducibly inducible spontaneously or with programmed atrial stimulation without isoproterenol infusion. The P-wave morphological features of tachycardia were variable depending on the case, and most cases exhibited 1:1 atrioventricular conduction. AF recurrence rate after PVI and NCC AT successful ablation was 12.5% (1 of 8). Tachycardia cycle length was shorter than that of 17 de novo ATs from NCC (303 versus 378, p=0.012). No AV block occurred during and after successful AT ablation. Conclusions: Uncommon NCC ATs (0.08% in AF ablation cases) uncovered after PVI, showing different characteristics compared to de-novo NCC ATs, should be suspected irrespective of P-wave morphologies when AT shows broad propagation from the anterior interatrial septum.

• Journal of Chest Surgery
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• 제29권3호
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• pp.311-314
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• 1996

1985년 1월 부터 1994년 12월 까지 10년간 부산대학교 흉부외과에서는 18명의 심장 점액종 환자를 수 술하였다. 17례는 좌심방 점액 종이 었고 1례는 우심방 점액종이었다. 환자의 연령은 15세에서 62세 사이로 평균 42.6세 였고 여자가 13명 남자가 5명이었다. 술전 증상으로는 운동시 호흡곤란이 전례 에서 있었 고 심계항진이 9례, 흉통이 5례, 실신을 경험한 경우가 3례 있었으며 대부분이 전신적 무력감, 체중감소, 발열, 기침, 상복부 불쾌감 등의 다양한 동반증상이 있었다 진단은 1례의 우심방 점액 종을 제외한 나머지 17례의 좌심방 점액종에서는 심장초음파검사만으로 충분하였다 수술소견상 점액 종의 부착 부위는 난원공이 12례, 난원공 이외의 심발중격이 3례, 승모판막윤이 1례, 좌심방벽이 2례 였으며 점액종의 크기와 형태는 다양하였다. 점액종을 제거하기위한 접근은 8례에서는 우심방 절개를, 10례에서는 양심방 절 개를시행하였다. 수술로 인한 조기 및 만기 사망례는 없었고 외래 추적중재발례도 없었다. 심장점액 종의 외과적 치료는 거의 완치가 가능하며 예후가 좋은 것으로 여겨진다.

• Journal of Chest Surgery
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• 제13권2호
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• pp.105-109
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• 1980

Tetralogy of Fallot has shown its familial aggregation in several familial studies. This reported case is another example which occurred in a family in two brothers. They revealed no cytogenetic abnormalities, but the anatomical cardiac malformation of them showed much similarity, total conal defect in ventricular septum and .one had patent foramen ovale, the other atrial septal defect. The familial recurrence tendency of Tetralogy of Fallot as well as other congenital heart diseases could be explained on multifactorial inheritance as shown in many reports. In spite that we couldn`t find out any environmental trigger or teratogens, our case may be accepted on the base of multifactorial mechanism.

• Journal of Chest Surgery
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• 제14권1호
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• pp.49-59
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• 1981

This is one case report of surgically treated partial atrioventricular canal. The 22 year-old male patient had no definitive history of frequent respiratory infection and cyanosis in his early childhood. Since his age of 7 years, dyspnea was manifested on exertion. First appearance of congestive heart failure was at his age of 16 years old. The physical examination revealed that the neck veins were distended and heaving of precordium. A thrill was palpable on the left 3rd-4th intercostal space extending from the sternal border toward the apex and Grade IV/VI systolic ejection murmur was audible on it. Neither cyanosis nor clubbing was noted. Liver was palpable about 5 finger breadths. Chest X-ray revealed increased pulmonary vascularity and severe cardiomegaly (C-T ratio = 74%). EKG revealed LAD, clockwise rotation, LVH and trifascicular block. Echocardiogram showed paradoxical ventricular septal movement, narrowed left ventricular outflow tract and abnormal diastolic movement of the anterior leaflet of mitral valve. Right heart catheterization resulted in large left to right shunt (Qp : Qs = 5.7: 1), ASD and moderate pulfllonary hypertension. Finally, left ventriculogram revealed typical goose neck appearance of left ventrlcalar outflow tract. On Oct. 10, 1980, open heart surgery was performed. Operative findings were: 1. Large primum defect ($6{\times}5$ Cm in diameter) 2. Cleft on the anterior leaflet of mitral valve. 3. The upper portion of ventricular septum was descent but no interventricular communication. 4. Downward attachment of the atrioventricular valves on the ventricular muscular septum. 5. Medium sized secumdum defect ($2{\times}1$ Cm in diameter). The cleft was repaired with 4 interrupted sutures. The primum defect was closed with Teflon patch and the secundum defect was closed with direct suture closure. Postoperatively atrial flutter-fibrillation in EKG and Grade U/VI apical systolic murmur were found. The postoperative course was uneventful and discharged on 29th postoperative day in good general conditions.

• Journal of Chest Surgery
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• 제24권3호
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• pp.310-321
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• 1991

Between April 1986 and September 1990, 34 patients with a single or dominant right ventricle underwent modified Fontan procedure for definite palliation in Seoul National University Children`s Hospital. Their age at operation ranged from 8 months to 14 years [Mean 5.5 years]. The ventricular chamber was solitary and of indeterminate trabecular pattern in 6 patients. 28 patients had posteriorly located rudimentary chamber, all of which were trabecular pouches having no communication with outlet septum. The patterns of atrioventricular connection were common inlet[9], double inlet [11], left atrioventricular valve atresia [12] and right atrioventricular valve atresia with L-loop [2]. Pulmonary outflow tracts were atretic in 7 patients and stenotic in 26 patients. Major associated anomalies included anomalous systemic venous drainage [15], dextrocardia [12] and total anomalous pulmonary venous connection[3]. Shunt operations were previously performed in 13 patients and pulmonary artery banding and atrial septectomy in 1 patients. Surgery included intraatrial baffling in 26 patients, bidirectional cavopulmonary shunt in 13 patients, atrioventricular valve obliteration in 3 patients and atrioventricular valve replacement in 3 patients. Central venous pressure measured postoperatively at intensive care unit ranged from 18cm H2O to 28cm H2O [mean 23.2cm H2O]. Hospital mortality was 35.3% [12/34], all died out of low output syndrome. Suspected causes of low output syndrome include ventricular dysfunction [8], hypoplastic or tortuous pulmonary artery [2] and elevated pulmonary vascular resistance [2]. 19 patients had 31 major complications including low output syndrome [18], arrhythmia [4], acute renal failure [3] and respiratory failure [3]. Mortality rate was significantly higher in the groups receiving intraatrial baffling and AV valve replacement respectively [p<0.05]. 20 patients were followed up postoperatively with the mean follow-up period 15.0$\pm$11.6 months. There were no late death and follow-up catheterization was performed in 10 patients. Mean right atrial pressure was 15.4$\pm$6.8mmHg and ventricular contraction was reasonable in all but one case. Thus, Fontan principle can be applied successfully to all the patients with complex cardiac anomaly of single ventricle variety and better results can be anticipated with judicious selection of patient and improvement of postoperative care.

• Journal of Chest Surgery
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• 제23권5호
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• pp.844-853
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• 1990

We have performed 27 cases of orthotopic homologous cardiac transplantation using Korean mongrel dogs and one case of sham operation for the evaluation of harmful effect of cardiopulmonary bypass itself on the dog from April, 1989 to June, 1990. Our previous reports have already demonstrated basal hemodynamic and hematologic data on the canine homologous heart transplantation and the fundamental principles of transplantation of the heart. The mean body weight of recipients was 13.2$\pm$1.2kg with a rage of 11 ~ 15kg, and the hemodynamic and hematologic pictures were almost same as the result of previous reports from our hospital, except marked decrease in postoperative platelet count[from 3.18 $\pm$0.80x106/mm3 to 1.41$\pm$0 37x 106/mm3]. Mean survival time was 24.82$\pm$49.40 hours with the longest survival of 264 hours. Donor cardiectomy included coronary vasodilatation with diltiazem, potassium arrest, and the rapid cooling of the heart suspending in the specially designed ice-bath. Median sternotomy provided excellent exposure of the surgical field. 6 \ulcorner0 prolene suture was used for the anastomosis of both atrial cuffs and the great arteries, and we found the fact that stenosis, bleeding, thrombus formation around the anastomotic site could be decreased with the use of everted horizontal mattress suture techniques. Immunosuppression was done with a combination of lower dose Cyclosporin-A, Azathioprine, methyl-prednisolone, but our cases still showed too short survival to worry about graft rejection. Still poor was our quality control of experimental animal, we had much difficulties in postmortem evaluation of the dogs. Low cardiac output due to biventricular failure, intractable supraventricular or ventricular tachyarrhythmia, postoperative massive bleeding, sepsis were most frequent findings that could be thought as a cause of death. A few cases showed subendocardial patch hemorrhage in both ventricular cavity or atrial septum at autopsy, suggesting acute subendocardial infarction. Although our team overcome most of the technical problems of orthotopic heart transplantation, we should pile up further knowledges about donor heart preservation, quality control of animal, infection, rejection, the effect of the cardiopulmonary bypass to improve the results.

• Journal of Chest Surgery
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• 제29권11호
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• pp.1276-1280
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• 1996

원발성 심장종양은 희귀성 때문에 병리학자, 심장내과의, 심장외과의에게 흥미로운 질환이다. 저자들은 수술치험한 세계적으로 희귀한 원발성 심장 연골육종을 보고하는 바이다. 환자는 37세의 여자환자로 본원에서 좌심방종양진단하에 1993년 6월 3일 입원하여 개심술하에서 완전 종양절제를 받은 바 있다. 그러나 조직소견상 연골육종이 진단되어 수술후 보조요법으로 방사선조사와 화학요법을 받은후 추적관찰하던 중 1995년 5월까지 종양 재발소견은 없었다. 그러나 환자는 두통과 전신성 경련으로 본원 신경외과에 입원하여 검사상 대뇌 전두엽부위에 단일 전이성 종괴가 발견되어 개두술하 종양절제가 가능하였으며, 조직 검사상 심장종양과 일치를 보였다. 환자는 건강한 상태로 퇴원하였다.