• Clinical and Experimental Pediatrics
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• v.56 no.3
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• pp.101-106
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• 2013

Despite developments in surgical techniques and other interventions, right ventricular (RV) failure remains an important clinical problem in several congenital heart diseases (CHD). RV function is one of the most important predictors of mortality and morbidity in patients with CHD. RV failure is a progressive disorder that begins with myocardial injury or stress, neurohormonal activation, cytokine activation, altered gene expression, and ventricular remodeling. Pressure-overload RV failure caused by RV outflow tract obstruction after total correction of tetralogy of Fallot, pulmonary stenosis, atrial switch operation for transposition of the great arteries, congenitally corrected transposition of the great arteries, and systemic RV failure after the Fontan operation. Volume-overload RV failure may be caused by atrial septal defect, pulmonary regurgitation, or tricuspid regurgitation. Although the measurement of RV function is difficult because of many reasons, the right ventricle can be evaluated using both imaging and functional modalities. In clinical practice, echocardiography is the primary mode for the evaluation of RV structure and function. Cardiac magnetic resonance imaging is increasingly used for evaluating RV structure and function. A comprehensive evaluation of RV function may lead to early and optimal management of RV failure in patients with CHD.

• Proceedings of the Korean Society of Veterinary Clinics Conference
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• 2009.04a
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• pp.127-133
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• 2009

Cardiomyopathies were previously defined as "an idiopathic myocardial disease that is not secondary to any other type of congenital/acquired heart disease or systemic diseases." With increasing understanding of etiology and pathogenesis in human medicine, the difference between cardiomyopathy and specific heart muscle disease has become indistinct. Cardiomyopathies are now classified by the dominant pathophysiology or, if possible, by etiological/pathogenetic factors. The American Heart Association recently advocated the following new definition of cardiomyopathy: Cardiomyopathies are a heterogeneous group of diseases of the myocardium associated with mechanical and/or electrical dysfunction that usually (but not invariably) exhibit inappropriate ventricular hypertrophy or dilatation and are due to a variety of causes that frequently are genetic. Cardiomyopathies either are confined to the heart or are part of generalized systemic disorders, often leading to cardiovascular death or progressive heart failure-related disability. Because the understanding of etiology or pathogenesis of cardiomyopathy has been limited in veterinary medicine, the previous classification is generally used. It is considered a dilated, hypertrophic and restrictive group on the basis of the predominant morphological and functional abnormalities. In addition, arrhythmogenic right ventricular cardiomyopathy and unclassified cardiomyopathy were also recognized in dogs and/or cats.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.937-945
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• 2006

The population of adult patients with congenital heart disease(CHD) has increased dramatically because of improved survival of infants and children with CHD. There are still a substantial number of adults with unrepaired CHD. Many adult patients do not know the possible problems of their disease such as ventricular dysfunction, arrhythmia, and what they should pay attention to. They often consider themselves "cured" or "healthy" if only they don't have overt symptoms, and visit hospitals only after deterioration of their problems. Some patients are neglected because they cannot be corrected surgically although careful medical support can improve their survival and quality of life. It is, therefore, essential that the patients, their families, and physicians understand the potential problems of CHD and need for regular follow-up. In this paper we will take a brief review for common medical problems of repaired or unrepaired CHD and also mention a few specific diseases which are common in adults.

• Journal of Chest Surgery
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• v.27 no.10
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• pp.815-823
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• 1994

This study was undertaken to hemodynamically determine the differences of myocardial protective effect between crystalloid and blood cardioplegic solution. Twenty nine children undergoing cardiac operations due to cyanotic congenital heart diseases were randomized into two groups receiving crystalloid or blood cardioplegia. Cardiac indices and other hemodynamic datum were examined postoperatively. Although there was no statistical differences between groups, postoperative stroke volume indices and left ventricular stroke work indices were slightly better with blood cardioplegia. We also found that postoperative left atrial pressures[p=0.0003], central venous pressures[p=0.004], and heart rates[p=0.014] were significantly lower with blood cardioplegia. The fact that relatively lower ventricular preloads [left atrial pressure and central venous pressure] were required to provide adequate cardiac output in blood cardioplegia group suggested superior myocardial protective effect of blood cardioplegic solution.

• Journal of Yeungnam Medical Science
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• v.3 no.1
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• pp.215-219
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• 1986

During 1986, 111 cases of open heart surgery were performed at Yeungnam University Hospital consisting 88 cases of congenital heart disease and 23 cases of acquired heart disease. Among 88 congenital heart disease, 72 were acyanotic group and 16 were cyanotic. Common congenital heart diseases were ventricular septal defect(51%), atrial septal defect(18%) and Tetralogy of Fallot(16%). Among 23 acquired heart disease, 22 cases were valvular heart disease and one was dissecting aortic aneurysm. Three cases of the postoperative death were present resulting 2.7% of surgical mortality rate.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.289-294
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• 1983

The report is concerned to our experience of 12 cases of open heart surgery under the extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery, Chosun University Hospital during the period between Nov, 1979 and April, 1983. 1. There were 4 cases of congenital anomaly and 8 cases of acquired heart disease. 2. There were 6 male and 6 female patients with a mean age of 20 years. [range 9 to 33 years]. 3. The cases induced 2 ventricular septal defect, 2 atrial septal defects and 8 acquired valvular heart diseases. 4. The surgical managements were 2 primary repair for atrial septal defect and 2 patch closure for ventricular septal defect, 1 triple valve replacement [AVR MVR TVR], 1 aortic valve replacement, 4 double valve replacement [AVR MVR] and 2 open mitral commissurotomy for pure mitral stenosis. 5. The average cardiopulmonary bypass time was 61.5 minutes for congenital heart disease and 201.4 minutes for acquired valvular heart disease and the average aortic cross clamping time was 36.75 minutes for the former and 165.6 minutes for the latter. 6. Postoperatively, there were 1 Alopecia, 1 Electric burn and 1 wound infection as complication. 7. Overall operative mortality was 8.3%. 7. All patients received valve replacement were recommended anticoagulation with persantin.

• Journal of Chest Surgery
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• v.19 no.2
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• pp.181-187
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• 1986

The changes of serum creatine phosphokinase [CPK], serum lactic dehydrogenase [LDH], serum glutamic oxaloacetic transaminase [GOT] and serum glutamic pyruvic transaminase [GPT] were studied in a total of 82 cases who underwent open heart operation under the cardiopulmonary bypass at the Department of Thoracic and Cardiovascular Surgery, Chungnam National University Hospital. The results were as follows 1. The average values of CPK and COT after the operation were reached to the maximal values on the first postoperative day, which were returned to the normal range until the fifth postoperative day. The average values of LDH and CPT after the operation were reached to the maximal values on the first and second postoperative day respectively, which were significantly increased until the seventh postoperative day. 2. In the relationship of the serum enzymes and duration of the extracorporeal circulation, the values on the group over 90 minute of the extracorporeal circulation were more increased than on the group below 90 minute of the extracorporeal circulation. 3. In the relationship of the changes of the serum enzymes and congenital heart diseases and acquired heart diseases, there were no significantly differences in the values between the two groups.

• Clinical and Experimental Pediatrics
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• v.55 no.6
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• pp.212-214
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• 2012

Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately one-third of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep, due to hypotonia and the anatomy of the oropharynx and airway, which make these patients susceptible to obstructive sleep apnea (OSA). In our case, pulmonary hypertension was caused, successively, by congenital heart defects (a large patent ductus arteriosus and arch hypoplasia) and obstructive sleep apnea during early infancy. The congenital heart defects were surgically corrected, but persistent pulmonary hypertension was identified 2 months after the operation. This pulmonary hypertension was due to OSA, and it was relieved by nasal continuous positive airway pressure. This case is the first report of pulmonary hypertension from OSA in a young infant with RTS.

• 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.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.