• Journal of Chest Surgery
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• v.41 no.4
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• pp.499-503
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• 2008

Berry syndrome (a distal aortopulmonary window and a right pulmonary artery originating from the ascending aorta, an intact ventricular septum, a patent ductus arteriosus and an interrupted aortic arch) is a rare complex congenital cardiac malformation. We describe a case of one stage repair with using an autologous arterial flap in a 19-day-old neonate who was suffering with this malformation, and we report on the development of postoperative right pulmonary artery stenosis and its successful management with performing multiple balloon angioplasties.

• Journal of Yeungnam Medical Science
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• v.31 no.1
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• pp.38-42
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• 2014

Cardiovascular beriberi is caused by thiamine deficiency and usually presents as high cardiac output failure associated with predominantly right-sided heart failure and rapid recovery after treatment with thiamine. Because of its rarity in developed countries, the diagnosis can often be delayed and missed. We recently experienced a case of cardiovascular beriberi with pulmonary hypertension which successfully treated with thiamine infusion. A 50-year-old man with chronic heavy alcoholics was refered to our department for dyspnea with mental change. Echocardiography showed marked right ventricular (RV) dilatation and flattening of the interventricular septum with a D-shaped deformation of the left ventricle. Moderate tricuspid valve regurgitation was found and estimated RV systolic pressure was 52 mm Hg. Because of his confused mentality and history of chronic alcohol intake, neurological disorder due to thiamine deficiency was suspected and intravenous thiamine was administered and he continuously received a daily dose of 100 mg of thiamine. Follow up echocardiography showed marked reduction of RV dilatation and improvement of a D-shaped deformation of the left ventricle. He finally diagnosed as cardiovascular beriberi on the basis of dramatic response to intravenous thiamine. Thiamine deficiency can cause reversible pulmonary hypertension, and can still be encountered in the clinical setting. Thus high index of suspicion is critically needed for diagnosis.

• The Korean Journal of Nuclear Medicine
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• v.24 no.2
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• pp.244-253
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• 1990

Sixty-four patients with paradoxical ventricular wall motion noticed both in angiocardiography or 2-dimensional echocardiography were assessed by ECG gated blood pool scan (GBPS). Endless cine loop image, phase and amplitude images and paradox image obtained by visual inspection of each cardiac beat or Fourier transformation of acquired raw data were investigated to determine the incremental value of GBPS with these processing methods for identification of paradoxical ventricular wall motion. The results were as follows: 1) Paradoxical wall motions were observed on interventricular septum in 34 cases, left ventricular free wall in 26 and right ventricular wall in 24. Underlying heart diseases were ischemic (23 cases) valvular(9), congenital heart disease (12), cardiomyopathy (5). pericardial effusion(5), post cardiac surgery(3), col pulmonale (2), endocarditis(1) and right ventricular tumor(1). 2) Left ventricular ejection fractions of patients with paradoxical left ventricular wall motion were significantly lower than those with paradoxical septal motion(p<0.005). 3) The sensitivity of each processing methods for detecting paradoxical wall motion was 76.9% by phase analysis, 74.6% by endless cine loop mapping and 68.4% by paradox image manipultion respectively. Paradoxial motions visualized only in phase, paradox or both images were appeared as hypokinesia or akinesia in cine loop image. 4) All events could be identified by at least one of above three processing methods, however only 34 cases (48.4%) showed the paradoxical molies in all of the three images. By these findings, we concluded that simultaneous inspection of all above three processing methods-endless cine loop, phase analysis and paradox image-is necessary for accurate identification and assessment of paradoxical ventricular wall motion when performing GBPS.

• Journal of Chest Surgery
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• v.38 no.12 s.257
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• pp.815-820
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• 2005

Background: Pulmonary atresia with intact ventricular septum is a rare congenital cardiac disease. There have been various surgical options, but there are still controversies. Material and Method: Fourteen neonates who were operated on between 1999 and 2000 were enrolled in this study. We measured Z-value of tricuspid valve by echocardiography. We performed right ventricular outflow tract transannular patch in 9 patients. Modified Blalock-Taussig shunt was placed according to the condition of patients. Mean follow up period was 46.0 months. Result: There were 2 operative mortality and 1 late mortality. Biventricular repair was possible in 7 patients. One and a half ventricular repair were done in 3 (tricuspid valve Z-value was -2, -2.5 and -3) and single ventricular repair in 2 patients (tricuspid z-value was -4.6, -4.5) The tricuspid valve Z-value for the patients who had biventricular repair and one and a half ventricular repair were -0.8$\pm$1.50 (-3.2$\∼$1.2) and -2.5$\∼$0.5 (-3$\∼$ -2) respectively. All patients who survived had fair to good right ventricular function, good left ventricular function and good clinical states. Conclusion: Neonatal surgical management of pulmonary atresia with intact ventricular septum has a good surgical outcome, z-value of tricuspid may be helpful for the determination of surgical options.

• Journal of Chest Surgery
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• v.32 no.2
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• pp.108-118
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• 1999

Background: To evaluate the risk factor and long-term result of arterial switch operation , a retrospective study was done. Material and Method: A retrospective analysis was done to evaluate the early and long-term results on 58 patients who underwent an arterial switch operation(ASO) for transposition of the great arteries(TGA) with intact ventricular septum, between January 1988 and December 1996. Beforesurgery, 36 patients(62.1%) underwent balloon atrial septostomy, 32 patients(51.7%) received PGE1 infusion, and preparatory banding of pulmonary artery was performed on 6 patients(mean LV/RV pressure ratio 0.53$\pm$0.11). Result: The age at operation ranged from 1 to 137 days(mean 24$\pm$26 days) and the weights ranged from 1.8 to 6.8 kg (mean 3.5$\pm$0.8 kg). There were 14 early deaths(24.1%), but of the last 24 patients operated on since 1994, there were only 2 early deaths(8.3%). In the risk factor analysis, the date of operation was the only risk factor for early death(p-value < 0.01). Eight of the 14 early deaths were due to acute myocardial failure(mainly inadequate coronary blood flow). The length of follow-up ranged from 2 months to 8 years, average of 36$\pm$27 months. The follow-up included sequential noninvasive evaluations and 21 catheterizations and angiographic studies performed 5 to 32 months postoperatively with particular attention to the great vessel and coronary anastomosis, ventricular function, valvular competence, and cardiac rhythm. There were 5 late deaths(11.4%), one of thesedeaths was related to the late coronary problems, two to aspiration, one to uncontrolled chronic mediastinitis, and one to progressive aortic insufficiency and heart failure. The most frequent postoperative hemodynamic abnormality was supravalvular stenosis and the degree of pulmonary or aortic obstruction had slowly progressed in some cases, however there were no children who had to undergo a reoperation for supravalvular pulmonary or aortic stenosis. Aortic regurgitation was identified in 9 patients, which was mild in 7 and moderate in 2 and had progressed in some cases. Two patients who had an unremarkable perioperative course were identified as having coronary artery obstructions. The other late survivors were in good condition, were in sinus rhythm, and had normal LV functions. Actuarial survival rate at 8 years was 68.8%. Conclusion: We concluded that anatomic correction will be established as the optimal approach to the TGA with intact ventricular septum, though further long-term evaluations are needed.

• Journal of Chest Surgery
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• v.29 no.8
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• pp.828-835
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• 1996

From January 1993 to April 1995, 27 neonates (under age of 30 days underwent open heart surgery in the Department of Thoracic and Cardiovascular Surgery, Dong-A Medical Center. Mean age and weight were 12.1 days(2days∼306ays) and 3.29 kg(2.6kg∼4.1 kg) respectively. Cardiac anomalies were simple complete transposition of great arteries(TGA) in 11 neonates, TGA with coarctation of aorta(COA) in 1 , total anomalous pulmonary venous connection(TAPVC) in 5, double inlet right ventricle with TAPVC in 1, interrupted aortic arch(IAA) with ventricular septal defect(VSD) in 3, pulmonary atresia(PA) with intact ventricular septum(IVS) in 3, pulmonary stenosis with IVS in 1, Taussig-Bing anomaly with IAA in 1, and hypoplastic left heart syndrome(HLHS) in 1 . Postoperative complications were myocardial and/or pulmonary edema which caused open sternum in 13 patients(54.2%), acute renal failure( RF) in 10(37.0%), Intractable low cardiac output syndrome (LCOS) including weaning failure from cardiopulmonary bypass in 7(25.9%), bronchopulmonary dysplasia in 1, wound infection in 1, and paroxysmal supraventricular tachycardia in 1. Nine of 13 patients with postoperative open sternum were recovered with delayed sternal closure, and seven of 10 patients survived postoperative ARF with peritoneal dialysis. There were 8 operative deaths(29.6%): 3 in the patients with simple complete TGA, 1 In TCA with COA, 1 in PA with IVS, 1 in Taussig-Bing anomaly with IAA, 1 in DIRV with TAPVC, and 1 in HLHS. One late death occurred after arterial switch operation in simple TGA. The mosts common cause of death was low cardiac output syndrome. Our initial experience of open heart surgery in neonates showed high operative mortality and morbidity, especially in complex anomalies.

• The Korean Journal of Nuclear Medicine Technology
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• v.20 no.1
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• pp.13-19
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• 2016

Purpose In order to calculate the left ventricular ejection fraction (LVEF) accurately, it is important to acquire the best septal view of left ventricle in the multi-gated cardiac blood pool scan (GBP). This study aims to acquire the best septal view by measuring angle of ventricular septal wall (${\theta}$) using enhanced CT scan and compare with conventional method using left anterior oblique (LAO) 45 view. Materials and Methods From March to July in 2015, we analyzed the 253 patients who underwent both enhanced chest CT and GBP scan in the department of nuclear medicine at National Cancer Center. Angle (${\theta}$) between ventricular septum and imaginary midline was measured in transverse image of enhanced chest CT scan, and the patients whose difference between the angle of ${\theta}$ and 45 degree was more than 10 degrees were included. GBP scan was acquired using both LAO 45 and LAO ${\theta}$ views, and LVEFs measured by automated and manual region of interest (Auto-ROI and Manual-ROI) modes respectively were analyzed. Results $Mean{\pm}SD$ of ${\theta}$ on total 253 patients was $37.0{\pm}8.5^{\circ}$. Among them, the patients whose difference between 45 and ${\theta}$ degrees were more than ${\pm}10$ degrees were 88 patients ($29.3{\pm}6.1^{\circ}$). In Auto-ROI mode, there was statistically significant difference between LAO 45 and LAO ${\theta}$ (LVEF $45=62.0{\pm}6.6%$ vs. LVEF ${\theta}=64.0{\pm}5.6%$; P = 0.001). In Manual-ROI mode, there was also statistically significant difference between LAO 45 and LAO ${\theta}$ (LVEF $45=66.7{\pm}7.2%$ vs. LVEF ${\theta}=69.0{\pm}6.4%$; P < 0.001). Intraclass correlation coefficients of both methods were more than 95%. In case of comparison between Auto-ROI and Manual ROI of each LAO 45 and LAO ${\theta}$, there was no significant difference statistically. Conclusion We could measure the angle of ventricular septal wall accurately by using transverse image of enhanced chest CT and applied to LAO acquisition in the GBP scan. It might be the alternative method to acquire the best septal view of LAO effectively. We could notify significant difference between conventional LAO 45 and LAO ${\theta}$ view.

• Clinical and Experimental Pediatrics
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• v.46 no.3
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• pp.250-258
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• 2003

Purpose : The actual clinical examples of co-appliance of catheter intervention with surgical procedures in the treatment of pulmonary atresia with an intact ventricular septum(PA/IVS) which we have experienced in our institution are here shown, and the anatomical and hemodynamical profiles between each method is compared. Methods : Medical records of 33 patients with PA/IVS who underwent various treatment from January, 1995 to December, 2000 were reviewed for a retrograde study. Results : In three out of 10 patients who underwent percutaneous balloon pulmonary valvotomy (PPV), residual pulmonary stenosis were observed in their out patient department(OPD) follow-ups, eventually necessitatig balloon pulmonary valvuloplasty(BPV). One out of three patients exhibited deterioration of tricuspid regurgitation after BPV, requiring surgical tricuspid annuloplasty(TAP). Two out of the seven patients who received primarily surgical right ventricle outlet tract(RVOT) repair without any systemic-pulmonary shunt or intervention needed additional intervention employing cardiac catheterization after operation. Two patients received interventional catheterization before surgical RVOT repair. In five out of 11 cases of Fontan type operation, coil embolization of collateral circulation was done before total cavo-pulmonary connection(TCPC), and in three cases, interventional catheterization was needed after TCPC. Conclusion : Both medical and surgical treatment modalities are widely used in management of PA/IVS patients, and recent results prove that medico-surgical cooperative treatment is essential.

• Journal of Chest Surgery
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• v.31 no.2
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• pp.168-172
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• 1998

Inferior sinus venosus defect is a rare lesion in which there is a large interatrial communication adjacent to the atrial connection of the inferior caval vein. The defect is located posteriorly and inferiorly, outside the confines of the true atrial septum, and partial anomalous pulmonary venous connections are the rule. We underwent surgical repair in four patients with inferior sinus venosus defect and partial anomalous pulmonary venous return. There were three males and one female with an age range from four months to 25 years. A cross- sectional echocardiogram and cardiac catheterization had been performed preoperatively in all patients, but the correct diagnosis had been made in only one case. Surgical repair was indicated due to congestive heart failure, and one patient of 4-month-old needed urgent operation. The repair was accomplished by suturing a untreated autologous pericardial patch to the right of the pulmonary veins, so that the defect was closed and all the pulmonary venous blood was directed to the left atrium. The preoperative knowledge of the unusual anatomy allows the surgeon to repair the anomaly without difficulties. For the patients in whom interatrial communication and anomalous pulmonary venous return are suggested, surgeon has to pay careful attention to the anatomical landmarks to avoid incorrect placement of the patch.

• Journal of Chest Surgery
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• v.19 no.4
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• pp.569-577
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• 1986

Between October 1979 and June 1986, modified Fontan procedures have been performed on 22 patients by the Department of Thoracic and Cardiovascular Surgery, Yonsei University College of Medicine. Twelve patients had tricuspid atresia; one, congenital tricuspid stenosis; five, single ventricle; two, I-transposition of the great vessels; one, double outlet right ventricle, and one, pulmonary atresia with an intact ventricular septum. There were 9 operative deaths [mortality; 40.9%]. The causes of death were right heart failure in six patients and pulmonary venous hypertension in one who misdiagnosed preoperatively. Another two were deceased due to sepsis and cerebrovascular accident at postoperative 35 and 34 days in each. There were 7 patients below 4 years of age at the time of operation and among them 4 patients were deceased. The operative death was not related with patients` age above and below 4[p=0.211]. The relation between operative death of tricuspid anomaly and another cardiac malformations was statistically significant [p=0.048]. The operative procedures with or without valved conduit [woven dacron] was related significantly [p=0.043] in the case of the 21 of the patients, but the modified Fontan operation with a valved conduit was performed early stage in this series. Since 1982, we operated on 4 patients, doing a right atrium-right ventricle anastomosis without a conduit. All survived and remained in functional class I [NYHA]. The right atrial pressure [RAP] was elevated significantly after operation [mean 9.9$\pm$4.8 ~16.9$\pm$3.6 mmHg, p<0.001]. The relation between the postoperative RAP of the survival group [16.5$\pm$4.3 mmHg] and the group who died [17.4$\pm$2.2 mmHg] was statistically significant [p=0.047]. There was no relation between any operative death and any previous palliation. All patients were followed for 4 months to 80 months, except one who was lost to follow up at 2 months following surgery [mean 11.4 months, 238 patient. months]. All were in functional class I with 5 on medications and 7 not. One was reoperated at 70 months following the first operation, due to conduit stenosis. She was moderately impaired in activity, with hepatomegaly after the second operation.