• 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.

• Journal of Chest Surgery
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• v.10 no.1
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• pp.113-117
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• 1977

The tetralogy of Fallot is one of the most frequent and serious congenital cardiac malformation accompanied cyanosis. For relief of cyanosis, the Waterston operation is a successful, palliative procedure in infant & young child under age of five with obstructive lesions of the right: side of the heart who require a systemic-pulmonary arterial shunt for survival. A patient, aged 3 and weighing 13 kg., who had been cyanotic since one month after birth,. was admitted to the University of Severance Hospital under a diagnosis of tetralogy of Fallot, The side to side anastomosis between the right pulmonary artery & the ascending aorta was performed in March 1976. The anastomotic channel was made only 4 ram. in diameter, thereafter massive unilateral pulmonary congestion on the side of the anastomosis developed shortly after operation. And the. patient died of congestive heart failure within a hour. And so the purpose of this report is describe the immediate & late effect of systemic-pulmonary shunt for T.O.F. with review of literatures.

• Journal of Veterinary Clinics
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• v.18 no.4
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• pp.452-454
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• 2001

A two-month-old female Yorkshire terrier was referred to the Veterinary Teaching Hospital, College of Veterinary Medicine, Kyungpook national University. The patient was presented with a history of dyspnea, cough, exercise intolerance and abdominal distension, but she was appetence. In physical examination the puppy was coughed on slight tracheal compression. Rectal temperature, pulse and respiratory rate were normal, and grade 3/6 systolic murmur heard at the left heart base. The murmur was crescendo-decrescendo. Electrocardiography showed sinus arrhythmia, right-ventricular hypertrophy pattern, and right axis deviation. Thoracic radiography revealed cardiomegaly, bulging of the main pulmonary artery, and enlarged left side heart. Abdominal radiography revealed abdkominal distention. Echocardiography showed hypertrophy of right ventricle and turbulence in the pulmonary artery in parasternal oblique view. Subvalvular pulmonic stenosis was diagnosis based upon the clinical signs, physical examination, electrocardiography, radiography and echocardiography. We treated the patient with furosemide, enalapril and $\beta$-blocker. After the clinical signs of cough, abdominal distension and dyspnea were disappeared, she was on just $\beta$-blocker for prevention of occurrence of congestive heart failure. Now she was recovered her health, and she is not on any medication.

• Journal of Chest Surgery
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• v.26 no.11
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• pp.886-889
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• 1993

The DiGeorge syndrome is a rare congenital anomaly of absent or hypoplastic thymus and parathyroid glands. Authors experienced a case of DiGeorge syndrome with complex cardiac anomaly. The complex cardiac anomaly was tetralogy of Fallot with origin of the right pulmonaly artery from the posterolateral ascending aorta.His face showed hypertelorism,short philtrum,"fish-like"mouth and micrognathia. This patient underwent total correction of tetralogy of Fallot and end-to-side anastomosis between right pulmonaly artery and side of main pulmonaly artery. He expired on postoperative second day due to right heart failure and hypoxia.d hypoxia.

• Journal of Chest Surgery
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• v.43 no.2
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• pp.176-179
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• 2010

Cardiac papillary fibroelastomas are the second most common primary tumor of the heart and they most commonly affect the left cardiac valves. However, occurrence of this tumor on the right side of the heart has been rarely reported, with only a few cases having been documented on the pulmonary valve. We present here a rare case of a papillary fibroelastoma that occurred on the pulmonary valve and this was successfully managed by replacing the pulmonary valve in a patient with congestive heart failure.

• Journal of Chest Surgery
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• v.11 no.2
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• pp.194-198
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• 1978

Congenital aortic stenosis, a relatively uncommon congenital heart disease, may cause heart failure and may be fatal. In recent years, increased clinical awareness and improved diagnostic and operative technique has made accurate diagnosis and successful treatment possible. Recently we experienced 2 cases of congenital aortic stenosis, and which was corrected surgically. The first case was 9 years old boy, and second case was 16 years male. Preoperative diagnosis was entertained by angiography and cardiac catheterization in both cases. In each case, aortic valve opening was widened by incision along the fused commissure between the combined left and right coronary cusp on one side, and the noncoronary cusp on the other side. Post-op. pressure gradient between the aorta and left ventricle markedly reduced, in the first case, 50mmHg, and in the 2nd case, 55mmHg.Both patients discharged with good results 2 weeks after open heart surgery.

• BMB Reports
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• v.52 no.8
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• pp.526-531
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• 2019

The vertebrate body plan is accomplished by left-right asymmetric organ development and the heart is a representative asymmetric internal organ which jogs to the left-side. Kupffer's vesicle (KV) is a spherical left-right organizer during zebrafish embryogenesis and is derived from a cluster of dorsal forerunner cells (DFCs). Cadherin1 is required for collective migration of a DFC cluster and failure of DFC collective migration by Cadherin1 decrement causes KV malformation which results in defective heart laterality. Recently, loss of function mutation of A-kinase anchoring protein 12 (AKAP12) is reported as a high-risk gene in congenital heart disease patients. In this study, we demonstrated the role of $akap12{\beta}$ in asymmetric heart development. The $akap12{\beta}$, one of the akap12 isoforms, was expressed in DFCs which give rise to KV and $akap12{\beta}$-deficient zebrafish embryos showed defective heart laterality due to the fragmentation of DFC clusters which resulted in KV malformation. DFC-specific loss of $akap12{\beta}$ also led to defective heart laterality as a consequence of the failure of collective migration by cadherin1 reduction. Exogenous $akap12{\beta}$ mRNA not only restored the defective heart laterality but also increased cadherin1 expression in $akap12{\beta}$ morphant zebrafish embryos. Taken together, these findings provide the first experimental evidence that $akap12{\beta}$ regulates heart laterality via cadherin1.

• Journal of Chest Surgery
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• v.35 no.9
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• pp.680-683
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• 2002

Infective endocarditis that involves the right side of the heart has been estimately 5% of all cases of infective endocarditis. It has been shown that about 70% of right-sided heart infective endocarditis cases have preexisting congenital heart disease or acquired valvular lesion. It would occur in intravenous drug users or end-stage renal disease patients with indwelling venous dialysis catheter. Antibiotic therapy is more effective in the right and, when it fails, the consequence of valve disruption and emboli are less. Patients receiving long-term hemodialysis are a unique population with regard in the risk of bacteremia and subsequent infective endocarditis. We experienced one case of the active infective endocarditis with right atrial vegetation without tricuspid or pulmonary valve involvement in patient with end-stage renal disease receiving long-term hemodialysis, who needed surgical correction after medical treatment failure. Then we reported it with references that right-sided heart infective endocarditis is rare, but difficult to diagnose, life-threatening because of delayed medical treatment.

• Clinical and Experimental Pediatrics
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• v.55 no.8
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• pp.297-300
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• 2012

Symptomatic pulmonary arterial hypertension (PAH) in patients with isolated atrial septal defect (ASD) is rare during infancy. We report a case of isolated ASD with severe PAH in an infant who developed airway obstruction as cardiomegaly progressed. The patient presented with recurrent severe respiratory insufficiency and failure to thrive before the repair of the ASD. Echocardiography confirmed volume overload on the right side of heart and severe PAH (tricuspid regurgitation [TR] with a peak pressure gradient of 55 to 60 mmHg). The chest radiographs demonstrated severe collapse of both lung fields, and a computed tomography scan showed narrowing of the main bronchus because of an intrinsic cause, as well as a dilated pulmonary artery compressing the main bronchus on the left and the intermediate bronchus on the right. ASD patch closure was performed when the infant was 8 months old. After the repair of the ASD, echocardiography showed improvement of PAH (TR with a peak pressure gradient of 22 to 26 mmHg), and the patient has not developed recurrent respiratory infections while showing successful catch-up growth. In infants with symptomatic isolated ASD, especially in those with respiratory insufficiency associated with severe PAH, extrinsic airway compression should be considered. Correcting any congenital heart diseases in these patients may improve their symptoms.

• Tuberculosis and Respiratory Diseases
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• v.60 no.5
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• pp.540-547
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• 2006

Background : Vital stability and right side heart failure are major prognostic factors of acute pulmonary thromboembolism. While it is important to recognize right side heart failure, it is often difficult in real practice. Recently, several studies have described early diagnostic tools for detecting right side heart failure including echocardiography and biochemical markers. This study, we evaluated the prognostic role of the B-type natriuretic peptide (BNP) in an acute pulmonary thromboembolism. Methods : Thirty-four patients with a diagnosis of acute pulmonary thromboembolism were enrolled in the study. The BNP levels were measured and echocardiography was performed at the Emergency Department. Data on the prognostic factors including ventilatory support, vital stability, pulmonary artery pressure, degree of tricuspid valve regurgitation, complications and death was collected from the patients' medical records. The patients with an acute pulmonary thromboembolism were divided into two groups based on the vital stability and the BNP level and the cutoff values and prognostic factors of the two groups were compared. Results : The predictors of the vital stability that influence the prognosis of patients with acute pulmonary thromboembolism were the BNP level, ventilatory support and death. The plasma BNP levels showed a strong correlation with the vital stability, ventilatory support, thrombolytic therapy and death. When the BNP cutoff level was set to 377.5 pg/dl in a ROC curve, the sensitivity and the specificity for differentiating between the groups with stable or unstable vital signs was 100% and 90%, respectively. Conclusion : This study indicates that a measurement of the plasma BNP levels may be a useful prognostic marker in patients with an acute pulmonary thrombo-embolism.