• Journal of Chest Surgery
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• v.12 no.3
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• pp.289-296
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• 1979

For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

• Journal of the korean academy of Pediatric Dentistry
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• v.30 no.3
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• pp.510-514
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• 2003

The Axenfeld-Rieger syndrome is a rare autosomal dominant disorder characterized by dental and ocular abnormalities. The essential ocular features include partial or complete bilateral hypoplasia of the iris stroma, abnormalities of the angle structures with congenital iris adhesions, and anterior displacement of Schwalbe's corpuscles. Common oral findings are hypodontia(especially in anterior maxillary segment), microdontia, misshaped teeth, delayed eruption of the teeth. Additionally, other systemic symptoms can be seen and early detection by the pedodontist through dental diagnosis should prevent visual impairment.

• Journal of Chest Surgery
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• v.21 no.6
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• pp.1040-1051
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• 1988

From 1959 through Jun. 1988, 2094 cases of various Cardiovascular diseases were operated, which were consisted of 1215 open heart surgery under extra-corporeal circulation or hypothermia and 879 conventional Cardiovascular Surgery at Department of Thoracic & Cardiovascular Surgery in National Medical Center. There were 1111 congenital heart anomalies and 983 acquired cardiovascular diseases. Among 1111 congenital cases, acyanotic cases were 748 and cyanotic cases were 363. In acquired heart cases, valvular heart diseases [702 cases] were the most frequent and a small No. of cardiac tumor and coronary artery diseases were included. Overall operative mortality was 9.3%, consisting of 4.6% for acyanotic anomaly, 20.6% for cyanotic anomaly and 8.7% for acquired heart diseases. In open heart surgery, overall mortality was 11.6% and 6% in conventional cardiovascular surgery.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.1
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• pp.28-33
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• 2013

Purpose: Anorectal malformations are often associated with other anomalies, reporting frequency with 40-70%. Gastrointestinal anomalies have been known to be relatively less common than associated anomalies of other organ system. This study was performed to assess a distinctive feature of cases associated with esophageal atresia. Methods: Clinical data (from January 2000 through December 2011) on the 196 subjects with anorectal malformations, managed in our Hospital, were reviewed. Total 14 neonates were identified with accompanying esophageal atresia and retrospective analysis was conducted. Results: The incidence was 7.1% and there were 8 male and 6 female subjects. Only 2 cases were associated with esophageal atresia without tracheoesophageal fistula. Although variable cases of anorectal malformation in female subjects, almost cases were anorectal malformations with rectourethral fistula in male. Other associated anomalies were identified in all cases, with more than 3 anomalies in 10 cases. There were 4 VACTERL (Vertebral abnormalities, Anal atresia, Cardiac anomalies, Tracheoesophageal fistula, Esophageal atresia, Renal and Limb anomalies) associations accounting for 28.6%, but could not identify chromosomal anomaly. Most cases were managed with staged procedure, usually primary repair of esophageal atresia and diverting colostomy. Overall mortality rate was 21.4%, mainly caused by heart problems. Conclusion: This study shows that early diagnosis and rational surgical approach with multidisciplinary plan are mandatory in managing anorectal malformations with esophageal atresia, when considering a high frequency of associated anomaly and a relative high mortality.

• Journal of Chest Surgery
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• v.14 no.4
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• pp.369-380
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• 1981

In this department 504 cases of valve replacement were done since 1968 to the end of October 1981. Since October 31, 1978 to the end of October 1981 ,333 Ionescu-Shiley bovine pericardial xenograft bioprosthetic cardiac valves were replaced in 265 patients. There were 149 males and 116 female. Ages ranged from 2 to 63 years with 25 cases under 15 years of age. Among 265 cases of Ionescu valve replacement there were 157 MVR, 36 VAR, 6 TVR, 45 MVR+ AVR, 16 MVR+TVR and 5 MVR+AVR+TVR cases with mortality of 5.7%, 8.3%, 16.7%, 8.9%, 18.8% and 20% for each group respectively. Over all mortality rate in 265 Ionescu valve replacement cases was 7.9% with 21 total deaths. Main causes of operative deaths were due to LCOS in 7, bleeding in 5, arrhythmia in 3, air embolism in 2,and heart block in 2 cases. There were 12 late complications with 6 deaths. Over all long-term survival rate was 89.8%. MVR showed the highest long-term survival rate with 92.4%, and MVR+AVR+TVR the lower with 80% lower with 80%.Average follow-up period was 14 month duration. Twenty five congenital anomaly cases were operated with Ionescu-valve replacement that consisted of 7 VSD+AI, and 5 Ebstein anomaly cases with over all operative mortality of 16% and late mortality of 14.3% among 21 operative survivors. There were 25 Ionescu valve replacement cases in pediatric patients under the age of 15 years, with 4 operative deaths. Fourteen MVR, 7 AVR, and 3 TVR cases were found. Even though long-term follow-up study was short in postoperative period with total of 33~.0 months among 244 operative survivors ranging one to 36 months, the late survivors showed beneficial long-term results two thromboembolic episodes in 244 patients were found. More cases and longer term follow-up study are warranted for valve replacement in pediatric and TVR cases with Ionescu-valves which have advantageous hemodynamic structures compared with other bio-prosthetic heart valves.

• Journal of Chest Surgery
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• v.21 no.4
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• pp.649-655
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• 1988

One hundred and one patients with tetralogy of Fallot who were older than 16 years of age underwent a total correction of the anomaly between May, 1964 and July, 1987. This group comprised 14.9% of the 679 consecutive patients who had repair of the tetralogy at our institution during the same period. Of the 101 patients, 8 had a previous shunt procedure for palliation. The preoperative mean hemoglobin value was 16.9*1.0% and the mean systemic oxygen saturation, 84.4*0.9%. In 76 patients[75.2%], a type II ventricular septal defect was seen whereas in 14 patients[13.9%], the defect was type I. In 72 patients[71.3%], other cardiac anomalies were present which included patent foramen ovale in 37.6%, atrial septal defect in 8.99b, vegetations in 6.9%, right sided aortic arch in 5.9% and coronary artery anomaly in 5.0%. The right ventricular outflow obstruction was caused most commonly by combination of infundibular and valvular stenosis[74.3%], followed by isolated infundibular stenosis[19.8%] and valvular stenosis [5.9%] alone in order. The preoperative mean diameter of the pulmonary valve ring size was 10.2*0.5 mm in diameter. A transannular patch enlargement of the right ventricular outflow tract was performed in 28 patients and, in 12 a pericardial monocusp was utilized. Major anomalous aorto-pulmonary vessels were encountered in 5 patients which were detected before or during the operation. In 3 patients, they were ligated beforehand to control the flooding of the operative field. Postoperatively, the mean systolic pressure gradient between the right ventricle and the main pulmonary artery was 16.2*2.3 mmHg and the mean systolic pressure- ratio between the right and the left ventricle was 45.3*2.0%. Perioperative complications including bleeding in 8.9%, pleural effusion in 7.9%, dysrrhythmia in 4.9%, and residual VSD in 4.0%. Operative mortality was 8.9%. There has been no operative death in the recent 65 cases since 1981. There were 2 late deaths, 68 and 113 months after surgery. There were 2 late detachment of the VSD patch during the follow-up period. Of the 6 patients with patch detachment found during the postoperative period, 3 had subacute bacterial endocarditis before or after the operation indicating The serious nature of this complication. Two of these patients subsequently underwent a successful reoperation.

• Journal of Chest Surgery
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• v.14 no.1
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• pp.17-25
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• 1981

In 1980, 416 cases of open heart surgery were done in this Department with over all operative mortality of 12.3%. 1. There were 288 congenital anomalies consisting of 174 acyanotic and 114 cyanotic varieties, which showed operative mortality of 6.9% and 25.4% respectively. 2. There were 128 cases of acquired lesions, 124 valvular disease and 3 myxoma being the main lesions. 3. There were 128 cases of valve replacement with operative mortality of 7.8%. 4. The most frequently operated anomaly was VSD, 90 pure VSD and 21 cases were associated with one or 2 cardiac anomalies. Over all operative mortality in 111 VSD cases was 8.1% but in 90 pure VSD cases it was 6.7%. 5. Tetralogy of Fallot showed the highest incidence in cyanotic group with 88 cases, consisting of 68 pure and 20 with other cardiac anomalies. Over all mortality in 88 cases was 19.3% but in pure form 16.2%. 6. In 128 valve replacement cases over all mortality was 9.4%. There were 85 mitral, 11 aortic, 2 tricuspid, 21 mitral with aortic, 6 mitral with tricuspid, 3 mitral, aortic, and tricuspid valve replacement cases. For mitral valve replacement operative mortality was 5.9%. 7. Twenty-one cases of babies under 10kg body weight were operated on with over all operative mortality of 28.6%. Sixteen cases of VSD were found with operative mortality of 25%. 8. Among 128 cases of valve replacement 7 were under the age of 15 years and 12 were between 15 and 20 years old. Five pediatric cases underwent mitral valve replacement without mortality, 9 year old boy was the youngest among them. In this Department open heart surgery for infancy and complex anomalies showed still hip operative risk which should be improved in the coming years. For open heart surgery Shiley oxygenators and 2 sets of A-O de-lux 5 head roller pump were utilized exclusively. For valve replacement Ionescu-Shiley bovine pericardial xenografts were mainly used. In pediatric and rural patients Persantin with aspirin regimen was satisfactorily administered for anticoagulation after valve replacement. Routinely Coumadin was administered for one year after valve replacement* In patients who had thrombus on valve sites, chronic atrial fibrillation, and giant left atrium Persantin-Aspirin regimen was used when one year coumadin administration was discontinued.

• Neonatal Medicine
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• v.18 no.2
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• pp.404-408
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• 2011

Scimitar syndrome (SS) is a rare congenital anomaly characterized by hypoplasia of the right lung and partially anomalous pulmonary venous drainage to the inferior vena cava. The term scimitar derives from the shadow created by the anomalous pulmonary vein on chest X-ray that closely resembles that of a curved Turkish sword. It rarely presents as an isolated abnormality. Various cardiac and non-cardiac anomalies have been association with SS, such as right lung hypoplasia, dextroposition of the heart, hypoplasia of the right pulmonary artery, systemic arterial blood supply to the right lower lung from the infra-diaphragmatic aorta, and a secundum type of atrial septal defect. However, an imperforate anus has not been reported previously in association with SS. We describe the first case of infantile scimitar syndrome accompanied with an imperforate anus in a newborn who presented with tachypnea and right pulmonary atelectasis.

• Clinical and Experimental Pediatrics
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• v.58 no.1
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• pp.37-40
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• 2015

The presence of a single coronary artery is a rare congenital anomaly; such patients often present with severe myocardial ischemia. We experienced the case of a 13-year-old girl with the right coronary artery originating from the left circumflex artery. She visited our Emergency Department owing to severe chest pain; her cardiac enzyme levels were elevated, but her initial electrocardiogram (ECG) was normal. Echocardiography showed normal anatomy and normal regional wall motion. When she presented with recurrent chest pain on admission, the ECG showed significant ST-segment elevation in the left precordial leads and inferior leads with ST-segment depression in aVR lead, suggesting myocardial ischemia, and her cardiac enzyme levels were also elevated. We performed coronary angiography that showed a single right coronary artery originating from the left circumflex artery without stenosis. We confirmed the presence of a single coronary artery using coronary computed tomography. In addition, the treadmill test that was performed showed normal results. She was discharged from the hospital without any medications but with a recommendation of a regular followup.

• Journal of Chest Surgery
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• v.17 no.3
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• pp.371-380
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• 1984

Corrected transposition of the great arteries [C-TGA] is one of the rare congenital heart disease in which there is both a discordant atrioventricular relationship and transposition of the great vessels. With this arrangement, systemic venous blood passes through the right atrium into the morphologic left ventricle and out the pulmonary artery. Pulmonary venous blood returns to the left atrium, flows into the morphologic right ventricle and out the aorta. Thus, in the rare case when no additional cardiac anomaly is present, a hemodynamically normal heart exists. But more often they are symptomatic as a result of one or several of the commonly associated defects. This paper describes 13 patients who underwent repair of one or more cardiac anomalies associated with corrected transposition at SN UH, from June 1976 through June 1984. 1.8 were males and 5 females, with ages ranging from 3 years to 27 years. 2. Segmental anatomy was {S,L,L} in 12, or {I,D,D} in 1. 3.Associated anomalies were ventricular septal defect in 10, pulmonary outflow tract obstruction in 6, tricuspid insufficiency in. 4, atrial septal defect in 3, subaortic stenosis in 1, mitral insufficiency in 1, and patent ductus arteriosus in 1. 4.None had complete heart block preoperatively, and 3 developed complete heart block intraoperatively. But one of them recovered sinus rhythm on the postoperative 7th day spontaneously. 5.There were 3 cases of hospital morality. But there was no morality since Dec. 1980. 6.Patients with single ventricle, hypoplastic ventricle or those who had palliative surgery alone are not included in this review.