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Objective To report a case of atrial septal defect (ASD) with congenital fibrinogen deficiency (CFD), and review the literature. Methods A case of secondary ASD with CFD treatment in Xuanwu Hospital of Capital Medical University was reported, and the defect was repaired under CPB on beating heart. The management experience of cardiac surgery under CPB in patients with CFD was summarized by means of PubMed database and literature analysis. Results This patient was female,30 years old. She was admitted to the hospital with the complaint that ASD was found for 8 months after physical examination. On admission, echocardiography showed that the atrial septum was interrupted by about 34 mm. Color Doppler flow imaging(CDFI) detection indicated systolic left to right shunt, the pressure difference of tricuspid regurgitation was 66 mmHg, systolic pulmonary artery pressure (SPAP) was 76 mmHg, ejection fraction (EF) was 64%. Coagulation function: Prothrombin time was>120 s, thrombin time >240 s, activated partial thrombin time >180 s, and fibrinogen <0.6 g/L. Preoperative continuous intravenous infusion of fibrinogen 4 g per day was to maintain the plasma fibrinogen >1.5 g. Three days later ASD repair was performed under CPB on beating heart. Infusion of fibrinogen 2 g was performed by venous injection during operation, and another fibrinogen 2 g was given after heparin was neutralized by protamine. Operation time was 3.5 hours, and CPB time was 45min. Fibrinogen 4 g per day was supplemented postoperatively for 5 days. At the 3rd day chest drainage tube was removed. The patient was discharged at the 7th day. There were no hemorrhage and thrombosis complications. After 3 months, outpatient follow-up showed the surgical incision healed well, echocardiography showed no residual shunt in the atrial septum, and EF value was 67%. A total of 8 English literatures were obtained after searching PubMed database and manually screening, including 875 patients with hypofibrinogenemia after CPB cardiac surgery. Among them, 604 were males (69.0%) and 271 were females (31.0%). There were 6 case reports, including 1 infant(5 days old, weight 2.5 kg) and 5 adult cases, and they were all male. Patients with abnormal afibrinogenemia may have both bleeding and thromboembolic symptoms. Patients with fibrinogen deficiency who undergo CPB cardiac surgery need to be supplemented with fibrinogen, cryoprecipitation or fresh frozen plasma during the perioperative period to prevent severe postoperative bleeding complications. Conclusion Patients with fibrinogen deficiency should be given a individualized perioperative management plan according to the quantitative and other coagulation indexes of fibrinogen in plasma for cardiac surgery under CPB.
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| 科 Family | 属数 Number of genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) | 属 Genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) |
|---|---|---|---|---|---|---|
| 鹅膏菌科Amanitaceae | 2 | 11 | 5.26 | 鹅膏菌属 Amanita | 10 | 4.78 |
| 小菇科 Mycenaceae | 2 | 12 | 5.74 | 丝盖伞属 Inocybe | 5 | 2.39 |
| 多孔菌科 Polyporaceae | 8 | 14 | 6.70 | 蜡蘑属 Laccaria | 5 | 2.39 |
| 红菇科 Russulaceae | 3 | 23 | 11.00 | 小皮伞属 Marasmius | 6 | 2.87 |
| 小菇属 Mycena | 11 | 5.26 | ||||
| 光柄菇属 Pluteus | 5 | 2.39 | ||||
| 红菇属 Russula | 17 | 8.13 | ||||
| 栓菌属 Trametes | 5 | 2.39 |
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