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Extracardiac anomalies(ECA) refer to a series of abnormalities in the musculoskeletal system or thoracoabdominal organs. We present a case of complex congenital heart disease(CHD) combined with asplenia and review the management strategies for this disease.
A 2-year-old female patient was admitted to our hospital with symptoms of progressive cyanosis and shortness of breath after crying or activity for 2 years. Her blood pressure was 89/68mm/Hg and heart rate was 110bpm. A physical examination revealed a grade III/VI systolic murmur over 3/4th intercostal space on left sternal border and a grade II-III/VI systolic murmur over 2/3th intercostal space on right sternal border. Ultrasonography suggested congenital absence of spleen. Echocardiography showed functional single ventricle, transposition of the great arteries, severe stenosis of pulmonary valve and right ventricular outflow tract, atrial septal defect, abnormal hepatic venous return, and normal cardiac function. Computed tomography(CT) revealed complex congenital cardiac malformations: single ventricle, transposition of the great arteries, pulmonary artery stenosis, and only one coronary artery was seen originating from the aortic root and dividing into left and right coronary artery 12mm after the origination. Laboratory data showed elevation of hemoglobin (190g/l) and pro-brain natriuretic peptide (493pg/ml).
The patient underwent latero-terminal anastomosis of the superior vena cava and the right pulmonary artery (bidirectional Glenn procedure). The pulmonary artery pressure in operation after anastomosis was 20mmHg, and SpO2 was about 80%. The patient was followed up in surgery intensive care unit due to severe pulmonary infection postoperative. Tracheal intubation was performed two times and followed by a tracheotomy. Vancomycin and Meropenem were used for antibacterial treatment. The patient's condition was gradually improved 2months later, and she was discharged with continued oral anti heart failure and antiplatelet drugs. Echocardiography rechecked 6months postoperative revealed the bloodstream in superior vena cava to right pulmonary artery was unobstructed. CT showed the proximal superior vena cava was closed and the distal segment was anastomosed to the pulmonary artery, azygos vein and its branches dilatation, collateral circulation formation in the mediastinum, and the diameter of pulmonary artery was wider than before. Considering the complexity of the patient's heart malformation, total superior vena cava and pulmonary artery anastomosis in 2 years was recommended.
ECA occur in more than 25% of significant CHD, often manifest as multiple malformations and sometimes as isolated anomaly. The presence of ECA markly increases the mortality in patients with CHD. Asplenia is a common type of abnormality among them, and sometimes is considered as an indicator of complex congenital heart disease. Which may induced a high risk of life-threatening invasive infections, especially due to encapsulated bacteria, so prompt diagnosis and appropriate treatment of asplenia are crucial for favorable prognosis. Treatments such as antibiotic prophylaxis, and immunization for encapsulated bacteria are recommended during perioperative period to decrease the risk of morbidity and mortality. In conclusion, a comprehensive and systematic examination for patients to exclude ECA is very important in the risk assessment and treatment planning for patients with complex CHD.