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The Korean Journal of Pathology 1988;22(3): 295-300.
Mitral Atresia Associated with Corrected Transposition of Great Arteries.
Dong Heon Yoon, Jeong Wook Seo, Je Geun Chi, Yong Soo Yoon, Jeong Yeon Choi, Yong Jin Kim, In Won Kim
1Department of Pathology, College of Medicine, Seoul National University, Seoul, Korea.
2Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea.
3Department of Thoracic and Cardiovascular Surger, College of Medicine, Seoul National University, Seoul, Korea.
4Department of Diagnostic Radiology, College of Medicine, Seoul National University, Seoul, Korea.
A case of congenital absence of right atriovntricular (A-V) connection of heart is reported. Patient was 81 day old male infant with chief complaints of dyspnea and irritability. Clinical evaluation revealed increased pulmonary vascularity on chest x-ray, absence of right A-V connection, regurgitation of left A-V valve, right-sided ventricular hypoplasia and L-TGA. Clinical assessment of the case was tricupsid atresia with LTGA and mitral regurgitation. Pulmonary artery banlding and artial septectomy was performed. Parenteral alimentation through the femoral veins resulted in obstruction of IVC and subsequent multiorgan failure. The autopsy findings of the heart were corrected transposition of great arteries with associated anomallies of right-sided mitral atresia, Ebstein's malformation of left-sided tricuspid valve, ventricular septal defect, muscular hypertrophy of right-sided left ventricle with luminal obliteration. Long segment of IVC was completely occluded due to occlusive thrombi with fungal infection. Bilateral kidneys were infarcted and azygos vein was markedly engorged. Microscopic examination revealed candidal colonization in the inferior vena cava and septic embolzation in brain.
Key Words: Mitral Atresa; Corrected TGA
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