年龄与双向腔肺吻合术后发绀改善的早期临床研究

目的　回顾评价双向腔肺吻合术 (BCPA)后早期 ,年龄因素对发绀改善情况的影响。方法　 1992年 6月至 2 0 0 0年 12月行BCPA术 6 8例。 6 3例在体外循环下手术 ,5例在常温非体外循环下手术。结果　动脉血氧饱和度 (SaO2 )大于 0 80者 47例 ,年龄 0 3～ 8岁 ,平均 ( 3 5± 2 0 )岁 ,体表面积0 2 7～ 0 86m2 ,平均 ( 0 5 6± 0 .14)m2 ;SaO2 小于 0 80者 2 1例 ,年龄 1 3～ 16岁 ,平均 ( 7 7± 3 9)岁 ,体表面积 0 41～ 1 44m2 ,平均 ( 0 82± 0 2 3)m2 。SaO2 与病儿年龄、体表面积的相关系数分别为 - 0 6 4、- 0 6 0。在年龄大于 5 95岁和 (或 )体表面积大于 0 72m2 时 ,术后SaO2 小于 0 80。 1例死亡。结论　BCPA应早实施 ,并严密随访。尽早改行全腔肺吻合术。     

The Coagulative Profile of Cyanotic Children Undergoing Cardiac Surgery: The Role of Whole Blood Preoperative Thromboelastometry on Postoperative Transfusion Requirement

The objective of this study is to evaluate the preoperative coagulation pattern and its association to postoperative blood products transfusion in children with congenital heart disease (CHD), focusing on cyanotic patients (oxygen saturation, SATO ₂ < 85%). From January to August 2014, preoperative standard coagulation tests and rotational thromboelastometry assays were performed on 81 pediatric patients (<16 years old) who underwent surgery for CHD with the aid of cardiopulmonary bypass. Sixty patients (74%) were acyanotic and 21 (26%) cyanotic. Mean age at time of surgery was 7.9 months (interquartile range 2.9–43.6 months). Cyanotic patients had a significantly higher hematocrit (P < 0.001), a reduced prothrombin activity (PT) (P = 0.01) level, and a lower platelet count (P = 0.02) than acyanotic patients. An inverse linear association was found between patient's SATO2 and clot formation time (CFT) (INTEM, P = 0.001, and EXTEM, P < 0.0001). A direct linear association was found between patient's SATO2 and maximum clot firmness (MCF) (INTEM, P = 0.04, and EXTEM, P = 0.05). Preoperative cyanosis was also associated with a lower median MCF in FIBTEM (P = 0.02). Cyanotic patients required more frequent postoperative transfusions of fibrinogen (7/21 patients, 33% vs. 4/60 patients, 6.7%, P = 0.01) and fresh frozen plasma (14/21, 67% vs. 25/60, 42%, P = 0.08). Patients with a lower presurgery PT and platelet count subsequently required more fibrinogen transfusion P = 0.02 and P = 0.003, respectively); the same goes for patients with a longer CFT (INTEM, P = 0.01 and EXTEM, P = 0.03) and a reduced MCF (INTEM, P = 0.02 and FIBTEM, P = 0.01) as well. Cyanotic patients showed significant preoperative coagulation anomalies and required a higher postoperative fibrinogen supplementation. The preoperative MCF FIBTEM has become an important factor in our postoperative thromboelastometry‐guided transfusion protocols.     

紫绀型心脏病动物模型的建立

紫绀型心脏病是先天性心脏病研究中的重点和难点，建立这类疾病的动物模型和对其深入研究有助于推动临床诊治水平的不断提高。已知的方法包括人造肺动-静脉瘘法、下腔静脉-左心房转流法、主肺动脉-左心房分流法、非手术操作的低氧吸入法等。紫绀型心脏病动物模型的建立经过了不断地改良和改进，成为研究紫绀缺氧状态下病理生理的重要工具和手段，但总体来讲仍未十分成熟和完善，需要继续探索和研究。     

Clinical characteristics on manifestation and gene mutation of a transient neonatal cyanosis: A case report

BACKGROUND We analyzed the main features of an infant diagnosed with temporary neonatal cyanosis in order to strengthen our understanding of the disease.CASE SUMMARY We report a patient diagnosed with temporary neonatal cyanosis.The main clinical characteristics,gene mutation and treatment are discussed and a review of related literature was conducted.The neonate aged 1 d and 5 h was admitted to hospital due to cyanosis after birth.The main clinical manifestation was cyanosis,which was not improved by auxiliary ventilation and the patient showed no obvious shortness of breath or methemoglobinemia.Gene mutation analysis showed a heterozygous c.190C>T mutation in the HBG2 gene associated with transient neonatal cyanosis,which was derived from his mother.Symptomatic supportive treatment was given for 2 mo.The neonate was discharged and gradually improved with oral administration of vitamin C and vitamin B2 for 2 wk.CONCLUSION There is no special treatment for temporary neonatal cyanosis caused by heterozygous mutation of the HBG2 gene.     

Enlarged Left Vitelline Vein Remnant as a Cause of Cyanosis after the Fontan Procedure: Resolution with an Amplatzer Vascular Plug

A 6-year-old girl with heterotaxy and a functional single ventricle had persistent cyanosis 4 years after a fenestrated Fontan procedure. Cardiac catheterization revealed a large venous fistula from a left-sided hepatic vein to the coronary sinus, resulting in desaturation. The anomalous vein was occluded with an Amplatzer vascular plug.     

免疫球蛋白对紫绀型先天性心脏病婴儿术后细胞免疫功能的影响

目的探讨免疫球蛋白对紫绀型先天性心脏病婴儿术后细胞免疫功能的影响。方法回顾性分析2012年3～12月河北省儿童医院心脏外科住院接受先天性心脏病手术治疗40例患者的临床资料,分为两组,免疫球蛋白治疗组：患儿术后1 h在常规治疗的基础上静脉给予免疫球蛋白,用量为1g/（kg·d）,疗程为2 d。对照组术后只进行常规的治疗。两组患儿分别于术前、术后0.5 h、2 d,抽取静脉血5 ml。釆用双抗体夹心酶联免疫吸附测定法（ELISA）分别检测各时间段两组患儿血清干扰素γ（IFN-γ）、白细胞介素4（IL-4）的浓度,并进行比较。结果术前及术后0.5 h两组患儿同时间段血清IL-4、IFN-γ浓度差异无统计学意义（P〉0.05）。术后0.5 h两组患儿血清IL-4、IFN-γ浓度均明显高于术前,差异有统计学意义（P=0.000）。免疫球蛋白治疗组患儿术后2 d血清IL-4浓度与术前相比差异无统计学意义（P=0.362）,而对照组患儿术后2 d血清IL-4浓度较术前明显升高,两组间差异有统计学意义（P=0.006）。术后2 d免疫球蛋白治疗组患儿血清IL-4、IFN-γ浓度明显低于对照组,两组间差异有统计学意义（P=0.039,0.007）。与术后0.5 h相比,对照组患儿术后2 d血清IL-4浓度约下降20.08%（P=0.001）,血清IFN-γ浓度上升17.80%（P=0.001）,差异均有统计学意义;免疫球蛋白治疗组患儿同时间血清IL-4浓度约下降35.38%,两组间差异有统计学意义（P=0.000）,而血清IFN-γ浓度只上升7.60%（P=0.143）,两组间差异无统计学意义。结论术后静脉应用免疫球蛋白,可有效改善因手术、体外循环等因素所导致的细胞免疫功能紊乱,有助于减少术后并发症的发生。     

Digital ischemia due to essential thrombocythemia: a case report

This report describes the case of a 34-year-old patient with essential thrombocythemia who presented with Raynaud's syndrome, was refractory to medical treatment, and developed progressive digital gangrene.     

Regression of Pulmonary Arteriovenous Malformations after Transcatheter Reconnection of the Pulmonary Arteries in Patients with Unidirectional Fontan

Objective. Pulmonary arteriovenous malformations (PAVM) develop in patients with a lung deprived of hepatic blood flow. Unidirectional Fontan patients have superior vena cava (SVC) blood directed to the left or right pulmonary artery (PA), and inferior vena cava (IVC) and hepatic blood baffled to the contra‐lateral PA. Cyanosis often develops due to PAVM in the lung deprived of hepatic blood. We report experiences with transcatheter PA reconnection in patients with unidirectional Fontan and PAVM. Design. Patients with unidirectional Fontan, cyanosis, and PAVM underwent transcatheter PA reconnection. Following simultaneous injection of contrast into PAs, a transseptal needle was used to puncture from one PA to the other. A balloon‐mounted, uncovered stent was positioned bridging the pulmonary arteries. The procedure provided a nonrestrictive, permanent communication between the PAs. Results were retrospectively reviewed. Catheterization data and oxygen saturations before and after procedure were analyzed. Results. Six patients with a median age of 14 years (range 13–35 years) were identified. All patients had functional single ventricle with unidirectional Fontan and PAVM in the lung supplied solely by SVC flow. The reconnection procedure was performed successfully without complications in all cases. The median follow‐up time for all 6 patients was 12.8 months (range 5.8–19.3 months). Median oxygen saturation was 0.86 (range 0.81–0.92) prior to the procedure, and 0.95 (range 0.92–0.98) on follow‐up, with a median increase of 9% (range 8–11%). Conclusion. Patients with unidirectional Fontan and PAVM demonstrate increased oxygen saturations following reconnection of PAs, suggesting regression of PAVM. This procedure can be performed safely using uncovered stents, and it is effective in improving systemic oxygen saturations.     

Does Anticoagulation in Eisenmenger Syndrome Impact Long‐term Survival?

Objective. To determine the impact of anticoagulation on survival in Eisenmenger syndrome. Background. The use of anticoagulation for primary prevention of adverse events in patients with Eisenmenger syndrome has been proposed but not studied. Strong arguments have been made both for and against anticoagulation based on the known risk of hemoptysis and pulmonary vascular thrombosis. Design and Setting. Retrospective cohort study at a tertiary referral hospital. Patients and Interventions. One hundred forty‐four patients with established Eisenmenger physiology all underwent initial laboratory, echocardiographic, and catheterization evaluation after initial referral. We retrospectively identified patients who were started on anticoagulation (AC) and compared them to patients who did not receive anticoagulation therapy (non‐AC). Baseline variables were compared between groups, as well as between survivors and nonsurvivors. Analyses of prognostic factors and survival were done using Cox and Kaplan–Meier methods. Outcome Measures. The primary outcome was death since time of baseline evaluation. Results. We identified 48 anticoagulated and 44 nonanticoagulated patients with Eisenmenger physiology (oxygen saturation 82 ± 9%, PaO₂ 48 ± 8 mm Hg, hemoglobin 18.6 ± 4 g/dL). More atrial septal defect patients were in the AC group, but there were no other baseline differences in clinical, functional, or hemodynamic data. After mean follow‐up of 7 ± 5.4 years (range 1–31), 11 patients died in the AC and 10 died in the non‐AC group. There was no survival difference between groups (log rank test = 1.78; P is not significant). For the entire cohort, mortality was significantly associated with New York Heart Association class 3–4 (hazard ratio = 4.2), evidence of right heart failure (hazard ratio = 13.6), and a mean corpuscular volume <80 fL (hazard ratio = 3.8). Use of anticoagulation did not impact survival. Bleeding complications occurred in seven (16%) of AC patients, including two fatalities. Conclusions. Anticoagulation had no impact on long‐term survival in this limited study. These data may be useful in considering future studies addressing this question.     

An Unexpected Cause of Respiratory Distress and Cyanosis: Cardiac Inflammatory Myofibroblastic Tumor

Inflammatory myofibroblastic tumor is an uncommon spindle cell tumor, occurring mainly in children and young adults. It is an extremely rare cardiac tumor especially patients under 1 year. Although it is benign, the tumor may be very aggressive locally. The diagnosis of this unusual pediatric cardiac tumor without pathologic specimens is difficult. We report a rare case of inflammatory myofibroblastic tumors of the right ventricle in a 7‐month‐old girl presenting with respiratory distress and cyanosis.