Atrioventricular Septal Defect with Common Atrioventricular Junction Guarded by a Common Valve Consisting of Left Atrioventricular Trifoliate Valve
A 32-year-old man presented to our clinic with the following history. His family history was negative for congenital heart defects and the patient had no siblings. Immediately after birth the cardiac status with a systolic murmur along left sternal border leading to a pediatric center where is diagnosed. The growth and mental development were normal, but marked cyanosis, weakness, clubbing, and intolerance of physical activity was present. At the age of nine the patient was referred to a cardio-pediatric center abroad where was diagnosed as complete atrioventricular canal defect, pulmonary atresia, and persistent ductus arteriosus. The patient condition was improved significantly and remained stable for the next eight year. At 17 years of age he was hospitalized again at the same center because of cyanosis, palpitations, and weakness with intolerance to physical activity. The patient had several documented episodes of atrial fibrillation.\nAt the age of 32, the patient presented to our clinic with severe cyanosis, he complained of shortness of breath with minimal exertion, and chest pain. The physical examination was remarkable for a 4/6 systolic murmur heard at the left sternal border, and cardiac frequency 130 beats per minute. The patient was noted to experience severe shortness of breath with even minimal exertion and was deeply cyanotic.\nThe electrocardiogram demonstrated atrial fibrillation, with ventricular frequency 100/min, and bifascicular block. Chest radiograph indicated a small pleural effusion on the right and bilateral hilopulmonary stasis. The heart was moderately enlarged with prominence of the aorta. An echocardiogram was performed (Figure , ).\nPatient was diagnosed in his early childhood with a congenital anomaly. In the very early echocardiography findings the presence of rudimentary inter ventricular and inter atrial septum leading to a common atrioventricular canal with one atrioventricular valve was described. This is also in the context of the malformations occurring during the intrauterine development of the embryo. From a perspective of adult congenital heart disease, the enlargement and the slight modifications of the heart cavities through the age, leaded to echocardiography view of hardly seen the rudimentary part of the interventricular septum, creating a perception of a single ventricular and a single atrium heart. An atrioventricular septal defect with common atrioventricular junction guarded by a common valve was seen. Both atriums were dilated, while pulmonary atresia and patent ductus arteriosus were also diagnosed in the early childhood. The main diagnoses were: atrioventricular septal defect with common atrioventricular junction guarded by a common valve, pulmonary atresia, persistent ductus arteriosus, and congestive heart failure.