• Clinical and Experimental Pediatrics
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• 제56권3호
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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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• 제10권1호
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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.

• 한국임상수의학회지
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• 제18권4호
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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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• 제26권11호
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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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• 제43권2호
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• pp.176-179
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• 2010

유두상 섬유탄력종은 양성 심장 종양 중 두번째로 많이 발생하며, 주로 대동맥 판막이나 승모판막에 발생한다. 그러나, 폐동맥 판막에서 발생한 경우는 아주 드물게 보고되고 있다. 이에 저자들은 심부전증 환자에서 판막 치환술로 치료된 폐동맥 판막의 유두상 섬유탄력종을 드문 증례로 보고하는 바이다.

• Journal of Chest Surgery
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• 제11권2호
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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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• 제52권8호
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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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• 제35권9호
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• pp.680-683
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• 2002

우측 심장에 발생한 감염성 심내막염은 전체 감염성 심내막염의 5%를 차지하며 이중 70%이상에서 선천성 심기형이나 후천성 판막질환 등에 동반되어 발생한다. 환자들은 약물 남용의 과거력을 갖는 경우가 많으며, 혈액투석 등 의인성 감염성 심내막염이 발생하는 경우도 있다. 우측 심장에 발생한 감염성 심내막염은 항생제 치료에 대한 반응이 우수하고, 내과적 치료에 반응하지 않더라도 판막손상이나 색전에 의한 합병증이 상대적으로 적게 발생하기 때문에 수술이 필요하지 않은 경우가 많다. 만성신부전으로 혈액투석을 받는 환자들은 관혈적 투석경로를 통해 균혈증에 노출되고 이로 인해 감염성 심내막염이 속발된다. 본 교실에서는 혈액투석을 받던 말기신부전 환자에서 정맥내 도관과 관련하여 삼첨판막이나 폐동맥판막의 침범없이 우심방내 우종을 동반한 심내막염이 발생하였으며, 항생제 치료를 시행하였으나 반응이 없어 수술을 시행하였다. 속목정맥도관과 관련된 우측 심장의 감염성 심내막염은 혈액투석환자에서 매우 드물게 발생하지만 진단이 지연되고 이로 인해 적절한 시기에 치료를 받지 못하여 병원내 사망률을 높이므로 적극적인 진단과 처치가 필요함을 확인할 수 있었기에 이를 문헌고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제55권8호
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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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• 제60권5호
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• pp.540-547
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• 2006

연구배경: 폐혈전색전증은 혈역학적 안정성과 우심부전의 정도가 중요한 예후인자이다. 우심부전을 조기에 파악하는 것이 중요하지만 실제 임상에서는 판단이 쉽지 않은 경우가 많으므로 심초음파를 포함한 다양한 진단 방법이 연구되고 있다. 저자들은 급성폐혈전색전증의 예후 인자로서의 혈중 BNP를 평가하고자 하였다. 방 법: 2002년 1월부터 2005년 5월까지 부산대학교병원 응급실을 내원하여 급성 폐혈전색전증으로 진단된 환자중 BNP 검사와 심초음파 검사를 시행하였던 34명의 환자를 대상으로 의무기록을 검토하였다. 예후 예측인자로 중환자실 입실여부, 인공 환기 치료 유무, 활력징후의 안정성여부, 합병증의 동반, 사망여부, 심초음파의 폐동맥압력 값과 삼첨판막의 역류정도를 설정하여 혈중 BNP 농도와의 연관성을 평가하였고 혈역학적 안정성을 기준으로 두 군으로 나누어 두 군을 구분하는 BNP의 cutoff 값을 구하였다. 결 과: 활력징후의 안정성 여부, 우심실 수축기압과 삼첨판막의 역류 정도, 기계환기 시행여부, 사망여부가 혈중 BNP 농도와 통계적으로 유의한 상관관계를 보였으며 활력징후 안정성에 대한 다중회귀분석을 시행한 결과 사망, BNP 농도, 기계 환기 여부가 통계적 의의를 보였다. 활력징후 안정성을 기준으로 안정군과 불안정군으로 나눌때 양군의 분류를 위한 BNP 농도의 cut-off level은 377.5 pg/dl에서 가장 민감도와 특이도가 높았다. 결 론: 혈중 BNP는 여러 예후 예측인자들과 통계적으로 유의한 상관 관계를 보였고 측정이 쉽고 빠른 시간 내 결과를 확인할 수 있으므로 폐혈전색전증 환자의 우심부전 정도를 평가하기 위한 심초음파나 폐혈관 조영술등의 검사가 어려운 경우나 이 값들을 보정하는 경우에 있어서 유용한 예후 인자로 고려 할 수 있을 것으로 생각된다.