• Clinical and Experimental Pediatrics
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• v.58 no.7
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• pp.256-262
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• 2015

Purpose: Kabuki syndrome is a multiple congenital malformation syndrome, with characteristic facial features, mental retardation, and skeletal and congenital heart anomalies. However, the cardiac anomalies are not well described in the Korean population. We analyzed the cardiac anomalies and clinical features of Kabuki syndrome in a single tertiary center. Methods: A retrospective analysis was conducted for a total of 13 patients with Kabuki syndrome. Results: The median age at diagnosis of was 5.9 years (range, 9 days to 11 years and 8 months). All patients showed the characteristic facial dysmorphisms and congenital anomalies in multiple organs, and the diagnosis was delayed by 5.9 years (range, 9 days to 11 years and 5 months) after the first visit. Noncardiac anomalies were found in 84% of patients, and congenital heart diseases were found in 9 patients (69%). All 9 patients exhibited left-side heart anomalies, including hypoplastic left heart syndrome in 3, coarctation of the aorta in 4, aortic valve stenosis in 1, and mitral valve stenosis in 1. None had right-side heart disease or isolated septal defects. Genetic testing in 10 patients revealed 9 novel MLL2 mutations. All 11 patients who were available for follow-up exhibited developmental delays during the median 4 years (range, 9 days to 11 years 11 months) of follow-up. The leading cause of death was hypoplastic left heart syndrome. Conclusion: Pediatric cardiologist should recognize Kabuki syndrome and the high prevalence of left heart anomalies with Kabuki syndrome. Genetic testing can be helpful for early diagnosis and counseling.

• Journal of Chest Surgery
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• v.12 no.3
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• pp.281-288
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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 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 Chest Surgery
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• v.35 no.2
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• pp.113-117
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• 2002

With an increasing awareness of the limitations of both mechanical prostheses and bioprostheses, aortic valvuloplasty has gained attention as an alternative procedure for aortic valve disease. Material and Method: Eight consecutive patients underwent aortic valvuloplasty caused by leaflet prolapse between June 1799 to June 2000. Mean age of the patients was 18.4$\pm$12.6 year. Four paitents(50%) were male. Six patients had tricuspid valves and ventricular septal defect and two patients had bicuspid valves. The extent of aortic insufficiency was 3.5$\pm$0.5 by preoperative Doppler echocardiography. The technique involved triangular resection of the free edge of the prolapsed leaflet, annular plication at the commissure, and resection of a raphe when present in bicuspid valves. Result: There was no in-hospital mortality or morbidity. Mean follow-up was complete at 11.973.6months. There was no late mortality or morbidity. The amount of the severity of aortic insufficiency, as assessed by echocardiography preoperatively, postoperatively and at late follow-up was 3.5$\pm$0.5, 0.6$\pm$0.5 and 0.8$\pm$0.6, respectively(p value : 0.01). There was one patient with grade 2/4 aortic insufficiency and in the other patients, grade 1/2 or trivial aortic insufficiency were detected with late echocardiograms. Conclusion: Triangular resection in the patients with aortic leaflet prolapse offers a good early clinical result, but long-term follow-up is necessary.

• Journal of Chest Surgery
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• v.48 no.3
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• pp.164-173
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• 2015

Background: Hypertrophied myocardium is especially vulnerable to ischemic injury. This study aimed to compare the early and late clinical outcomes of three different methods of myocardial protection in patients with aortic stenosis. Methods: This retrospective study included 225 consecutive patients (mean age, 65{\pm}10 years; 123 males) with severe aortic stenosis who underwent aortic valve replacement. Patients were excluded if they had coronary artery disease, an ejection fraction <50%, more than mild aortic regurgitation, or endocarditis. The patients were divided into three groups: group A, which was treated with antegrade and retrograde cold blood cardioplegia; group B, which was treated with antegrade crystalloid cardioplegia using histidine-tryptophan-ketoglutarate (HTK) solution; and group C, treated with retrograde cold blood cardioplegia. Results: Group A contained 70 patients (31.1%), group B contained 74 patients (32.9%), and group C contained 81 patients (36%). The three groups showed significant differences with regard to the proportion of patients with a New York Heart Association functional classification ${\geq}III$ (p=0.035), N-terminal pro-brain natriuretic peptide levels (p=0.042), ejection fraction (p=0.035), left ventricular dimensions (p<0.001), left ventricular mass index (p<0.001), and right ventricular systolic pressure (p <0.001). Differences in cardiopulmonary bypass time (p=0.532) and aortic cross-clamp time (p=0.48) among the three groups were not statistically significant. During postoperative recovery, no significant differences were found regarding the use of inotropes (p=0.328), mechanical support (n=0), arrhythmias (atrial fibrillation, p=0.347; non-sustained ventricular tachycardia, p=0.1), and ventilator support time (p=0.162). No operative mortality occurred. Similarly, no significant differences were found in long-term outcomes. Conclusion: Although the three groups showed some significant differences with regard to patient characteristics, both antegrade crystalloid cardioplegia with HTK solution and retrograde cold blood cardioplegia led to early and late clinical results similar to those achieved with combined antegrade and retrograde cold blood cardioplegia.

• Journal of Veterinary Clinics
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• v.32 no.2
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• pp.162-167
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• 2015

Tei-index has been reported as one of reliable echocardiographic factors for evaluating cardiac function in human and small animals. In this study, Tei-index were measured and evaluated with other echocardiographic parameters in normal thirteen beagle dogs and thirty-one dogs with mitral valve degeneration. Normal range of Tei index with tissue Doppler obtained at septum and left ventricular free wall was $0.58{\pm}0.07$ and $0.60{\pm}0.07$, respectively. The values between septum and left ventricular free wall did not show significant difference. Tissue Doppler derived tei index showed better reproducibility and significantly lower values than the results obtained by pulsed wave Doppler. Tei index obtained in either method increased with the progression of clinical signs. Therefore, the increase of Tei index in dogs with mitral regurgitation is thought to be an useful factor reflecting left ventricular dysfunction.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.1-11
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• 2008

Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6,. another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.

• Journal of Chest Surgery
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• v.47 no.4
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• pp.344-349
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• 2014

Background: The Damus-Kaye-Stansel (DKS) procedure is a method for mitigating the risk of systemic ventricular outflow tract obstruction (SVOTO). However, there have been few reports on which surgical technique shows a better outcome. The objective of this study was to compare the outcome of the DKS procedure according to the surgical technique used. Methods: We retrospectively reviewed 12 consecutive patients who underwent the DKS procedure from March 2004 to April 2013. When the relationship of the great arteries was anterior-posterior, the double-barrel technique (group A) was performed. If the relationship was side-by-side, the ascending aortic flap technique (group B) was performed. Results: There was no early mortality and 1 late mortality in group B. There was no statistically significant difference in the median peak pressure gradient of preoperative subaortic stenosis in both groups: 14 mmHg (range, 4 to 53 mmHg) in group A and 15 mmHg (range, 0 to 30 mmHg) in group B (p=0.526). Further, a significant postoperative pressure gradient was not observed in either group A or group B. More than moderate postoperative neoaortic regurgitation was observed in 1 patient of group B; this patient underwent neoaortic valve replacement 66 months after the DKS procedure. No one had a recurrent SVOTO during follow-up. Conclusion: The DKS procedure is an effective way to minimize the risk of SVOTO, and there is little difference in the outcomes of the DKS procedure according to the surgical technique used.

• Journal of Chest Surgery
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• v.22 no.4
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• pp.620-629
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• 1989

A clinical analysis of 138 cases of ventricular septal defect operated on from 1983 to June 1988 at the department of Thoracic and Cardiovascular Surgery, School of Medicine, Chung-nam National University was done. The following results were obtained. 1. The VSD were 27.6 % and 41.0 % respectively of 500 open heart surgery cases and 334 congenital heart disease. 2. The range of age when the VSD was repaired was 3 months to 45 years, the mean age was 10.4 years and the 53.6 % of patients were under 7 years. The sex ratio was 6:4 in males favor. 3. The two common symptoms were frequent upper respiratory infection and dyspnea whose frequency was 62.5 % and 58.7% respectively. 4. 52% of the patients was below 25 percentile of the standard body weight. 5. The most common chest PA findings were cardiomegaly and increased pulmonary vascularity. 6. On anatomical classification, perimembranous type was most common [65.5%], subpulmonary arterial type was 27.6 %, perimembranous plus subpulmonary arterial type was 3.4 % multiple VSD was 0.8 % and LV-RA defect was 2.6 %. 7. Associated cardiac anomalies were founded in 39 cases [35.5 %] and PDA and aortic valve prolapse were most common associated anomalies and extracardiac anomalies were founded in 6.7 % of patients. 8. Mean extracorporeal circulation time was 68 min. and mean aortic cross-clamping time was 42 min. 9. The postoperative complications developed in 26 % of patients and the most common one was minor wound infection. 10. Right bundle branch block developed in 54.8% of the patients who had right ventriculotomy, 40.0% of the patients who had right atriotomy and 10.5% of the patients who had pulmonary arteriotomy. 11. The overall operative mortality was 5.1 % and the operative mortality of the patients who body weigh under 10 Kg was 23.8%.

• Journal of Chest Surgery
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• v.38 no.9 s.254
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• pp.633-636
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• 2005

Sixteen-day-old baby with severe Ebstein anomaly underwent emergency operation to relieve progressive hypoxia and congestive heart failure. Operative findings showed huge right atrium and atrialized right ventricle (aRV) with very small functional RV by distal displacemcent of tricuspid valve mechanism. We elected to perform modified Starness operation because biventricular repair was deemed unattainable. After pulmonary and tricuspid valves were primarily closed, aRV was obliterated with multiple sutures from RV apex to the base. Then a PTFE (Gore-Tex, USA) vascular graft was interposed between innominate artery and main pulmonary artery for systemic to pulmonary shunt. The patient was discharged uneventfully, and received bi-directional cavopulmonary shunt 6 months later.