• Journal of Chest Surgery
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• v.49 no.4
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• pp.295-297
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• 2016

Aorto-ventricular tunnel is a rare congenital malformation, and aorto-right ventricular tunnel (ARVT) is an even less common entity. Here, we report the case of a 3-month-old female who underwent successful surgical closure of ARVT. The origin of the right coronary artery was proximal to the ostium of the tunnel.

• Clinical and Experimental Pediatrics
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• v.54 no.5
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• pp.183-191
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• 2011

Although antenatal diagnostic technique has considerably improved, precise detection and proper management of the neonate with congenital heart disease (CHD) is always a great concern to pediatricians. Congenital cardiac malformations vary from benign to serious conditions such as complete transposition of the great arteries (TGA), critical pulmonary and aortic valvular stenosis/atresia, hypoplastic left heart syndrome (HLHS), obstructed total anomalous pulmonary venous return (TAPVR), which the baby needs immediate diagnosis and management for survival. Unfortunately, these life threatening heart diseases may not have obvious evidence early after birth, most of the clinical and physical findings are nonspecific and vague, which makes the diagnosis difficult. High index of suspicion and astute acumen are essential to decision making. When patent ductus arteriosus (PDA) is opened Widely, many serious malformations may not be noticed easily in the early life, but would progress as severe acidosis/shock/cyanosis or even death as PDA constricts after few hours to days. Ductus dependent congenital cardiac lesions can be divided into the ductus dependent systemic or pulmonary disease, but physiologically quite different from each other and treatment strategy has to be tailored to the clinical status and cardiac malformations. Inevitably early presentation is often regarded as a medical emergency. Differential diagnosis with inborn error metabolic disorders, neonatal sepsis, persistent pulmonary hypertension of the newborn (PPHN) and other pulmonary conditions are necessary. Urgent identification of the newborn at such high risk requires timely referral to a pediatric cardiologist, and timely intervention is the key in reducing mortality and morbidity. This following review deals with the clinical presentations, investigative modalities and approach to management of congenital cardiac malformations presenting in the early life.

• Korean Journal of Radiology
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• v.2 no.3
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• pp.121-131
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• 2001

Echocardiography and catheterization angiography suffer certain limitations in the evaluation of congenital heart diseases in adults, though these are overcome by MRI, in which a wide field-of view, unlimited multiplanar imaging capability and three-dimensional contrast-enhanced MR angiography techniques are used. In adults, recently introduced fast imaging techniques provide cardiac MR images of sufficient quality and with less artifacts. Ventricular volume, ejection fraction, and vascular flow measurements, including pressure gradients and pulmonary-to-systemic flow ratio, can be calculated or obtained using fast cine MRI, phase-contrast MR flow-velocity mapping, and semiautomatic analysis software. MRI is superior to echocardiography in diagnosing partial anomalous pulmonary venous connection, unroofed coronary sinus, anomalies of the pulmonary arteries, aorta and systemic veins, complex heart diseases, and postsurgical sequelae. Biventricular function is reliably evaluated with cine MRI after repair of tetralogy of Fallot, and Senning's and Mustard's operations. MRI has an important and growing role in the morphologic and functional assessment of congenital heart diseases in adolescents and adults.

• Korean Journal of Radiology
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• v.21 no.6
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• pp.717-725
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• 2020

Objective: To assess left ventricular remodeling patterns using cardiac computed tomography (CT) in children with congenital heart disease and correlate these patterns with their clinical course. Materials and Methods: Left ventricular volume and myocardial mass were quantified in 17 children with congenital heart disease who underwent initial and follow-up end-systolic cardiac CT studies with a mean follow-up duration of 8.4 ± 9.7 months. Based on changes in the indexed left ventricular myocardial mass (LVMi) and left ventricular mass-volume ratio (LVMVR), left ventricular remodeling between the two serial cardiac CT examinations was categorized into one of four patterns: pattern 1, increased LVMi and increased LVMVR; pattern 2, decreased LVMi and decreased LVMVR; pattern 3, increased LVMi and decreased LVMVR; and pattern 4, decreased LVMi and increased LVMVR. Left ventricular remodeling patterns were correlated with unfavorable clinical courses. Results: Baseline LVMi and LVMVR were 65.1 ± 37.9 g/m² and 4.0 ± 3.2 g/mL, respectively. LVMi increased in 10 patients and decreased in seven patients. LVMVR increased in seven patients and decreased in 10 patients. Pattern 1 was observed in seven patients, pattern 2 in seven, and pattern 3 in three patients. Unfavorable events were observed in 29% (2/7) of patients with pattern 1 and 67% (2/3) of patients with pattern 3, but no such events occurred in pattern 2 during the follow-up period (4.4 ± 2.7 years). Conclusion: Left ventricular remodeling patterns can be characterized using cardiac CT in children with congenital heart disease and may be used to predict their clinical course.

• Journal of Chest Surgery
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• v.47 no.4
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• pp.394-397
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• 2014

Numerous technical modifications and various complications of the Senning procedure have been described in the literature. We describe the excellent clinical status and anatomic result of a 33-year-old patient who underwent a modified Senning operation using the left atrial appendage for reconstruction more than 30 years prior to presentation.

• Clinical and Experimental Pediatrics
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• v.60 no.8
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• pp.237-244
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• 2017

During the last 10 years, there have been major technological achievements in pediatric interventional cardiology. In addition, there have been several advances in cardiac imaging, especially in 3-dimensional imaging of echocardiography, computed tomography, magnetic resonance imaging, and cineangiography. Therefore, more types of congenital heart diseases can be treated in the cardiac catheter laboratory today than ever before. Furthermore, lesions previously considered resistant to interventional therapies can now be managed with high success rates. The hybrid approach has enabled the overcoming of limitations inherent to percutaneous access, expanding the application of endovascular therapies as adjunct to surgical interventions to improve patient outcomes and minimize invasiveness. Percutaneous pulmonary valve implantation has become a successful alternative therapy. However, most of the current recommendations about pediatric cardiac interventions (including class I recommendations) refer to off-label use of devices, because it is difficult to study the safety and efficacy of catheterization and transcatheter therapy in pediatric cardiac patients. This difficulty arises from the challenge of identifying a control population and the relatively small number of pediatric patients with congenital heart disease. Nevertheless, the pediatric interventional cardiology community has continued to develop less invasive solutions for congenital heart defects to minimize the need for open heart surgery and optimize overall outcomes. In this review, various interventional procedures in patients with congenital heart disease are explored.

• Journal of Yeungnam Medical Science
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• v.40 no.2
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• pp.187-192
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• 2023

Background: This study aimed to present the short- and midterm outcomes after complete atrioventricular canal defect (CAVC) repair using a single-patch technique. Methods: This study included 30 children who underwent surgical correction of the CAVC using a single-patch technique. Results: The median age of the patients was 5.7 months (interquartile range [IQR], 5.0-7.5 months), and 23 patients (76.7%) had type A CAVC. Fourteen patients (46.7%) were female and 17 (56.7%) had been diagnosed with Down syndrome. The in-hospital mortality rate was 0%. No deaths were observed during a median follow-up of 4 years (IQR, 3.5-5.0 years). Patients without Down syndrome were associated with late moderate mitral regurgitation (MR) (p=0.02). Late MR less than moderate degree was observed in 96.6%, 78.5%, and 50% of patients after 2, 4, and 5 years of follow-up, respectively, while late tricuspid valve regurgitation less than moderate degree was observed in 96.7%, 85.9%, and 59.0% of patients after 2, 4, and 6 years of follow-up, respectively. After a median follow-up of 4 years, only one patient had required surgical repair of a left ventricular outflow tract obstruction, which occurred 26 months after the first operation. Multivariable logistic regression analysis adjusted for the type of CAVC, sex, Down syndrome, age, and weight revealed that the absence of Down syndrome was a risk factor for late moderate MR (MR-2) (odds ratio, 0.05; 95% confidence interval, 0.006-0.50; p=0.01). Conclusion: A single-patch technique for CAVC surgical repair is a safe method with acceptable short- and midterm results.

• Journal of Chest Surgery
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• v.23 no.1
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• pp.22-32
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• 1990

Postoperative cardiac performance of cyanotic congenital heart disease is somewhat different from that of other cardiac diseases. For the evaluation of postoperative cardiac performance in the cyanotic congenital heart disease we measured cardiac output by thermodilution technique at 1, 4, 8, 12, 16, 20, 24, 36, 48 postoperative hours in 14 patients operated from Feb. 1989 to Nov. 1989 in The Department of Thoracic and Cardiovascular Surgery, Seoul National University Children`s Hospital. At the same time, we checked left atrial pressure [LAP], central venous pressure [CUP], and mixed venous oxygen saturation [SvO2] to detect correlation between them. Immediate postoperative cardiac index was 3.585 $\pm$ 0.945 L/min/m2, and it decreased maximally to 3.322$\pm$1.007 L/min/m2 at postoperative 16 hours. After then it increased and stabilized from 36 hours after operation, and its value was 4.426$\pm$1.358 L/min/m2. There were no correlations between cardiac index and left atrial pressure or central venous pressure. Between mixed venous oxygen saturation and cardiac index, there was no correlation in the early postoperative period but after postoperative 16 hours, there was significant correlation between them and correlation coefficients were 0.573 [16hrs], 0.743 [20hrs], 0.436 [24hrs], 0.560 [36hrs], 0.636 [48hrs], respectively. From these results, we concluded that in the corrective surgery of cyanotic congenital heart disease, cardiac performance was depressed in the early postoperative period. It improved from postoperative 16 hours, and stabilized from 36 hours after operation. During early postoperative period, mixed venous oxygen saturation should not be used as a predictor of cardiac performance but it could be used as a predictor of cardiac performance from 16 hours after operation.

• Journal of Chest Surgery
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• v.22 no.1
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• pp.50-58
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• 1989

The emergence and expansion of cardiac surgery over the past decade has resulted in an increasing number of patients undergoing cardiac operations but many kinds of heart surgery was realized only palliative, resulting in increasing numbers of secondary cardiac procedures. From 1978 to 1988, 10 cases of various congenital heart diseases and 17 cases of acquired heart diseases were reoperated at Hanyang University Hospital. The leading indication of second operation was residual shunt or valvular malfunction due to technical failure in congenital heart disease and primary valve failure, endocarditis, paravalvular leakage were for acquired heart disease. The mortality of reoperation was 0% for congenital heart disease and 11.7%[2 death among the 17 patients] for acquired heart disease. The leading causes of death were myocardial failure, sepsis with endocarditis, acute renal failure and congestive heart failure.

• Journal of Chest Surgery
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• v.27 no.7
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• pp.624-627
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• 1994

Congenital coronary arteriovenous fistula is a rare cardiac defect that causes coronary arterial flow to drain into the right cardiac chambers, the pulmonary artery, the coronary sinus, or the left cardiac chambers. The most frequently involved vessel is the right coronary artery. We experienced a case that had a coronary arteriovenous fistula associated with valvular heart disease. With the cardiopulmonary bypass done under hypothermia, mitral valve replacement was accomplished and the fistulas of both proximal and distal portions of the right coronary artery were closed with 3-0 prolene. Postoperative course was uneventful.