Objective To investigate somatic growth of children undergoing stageⅡ Fontan procedure. Methods Clinical data of 152 children who underwent stageⅡ Fontan procedure in Shanghai Children’s Medical Center, Shanghai Jiaotong University School of Medicine between January 2012 and December 2015 were reviewed. There were 84 males and 68 females at age of 4-16 years. The weight and height of those children in each operative stage were assessed, and WHO Z-score was obtained to describe the pattern of growth of the children. Results Mean weight for age Z-score (WAZ) and height for age Z-score (HAZ) were –0.73 and 0.06 at birth, –1.27±1.37 and –1.27±1.72 before Glenn procedure, –0.47±1.08 and –0.69±1.17 before Fontan procedure, –0.42±1.18 and –0.39±1.48 at follow-up, respectively. A significant decline (P<0.05) in WAZ and HAZ was found before Glenn procedure. After the Glenn procedure, there was significant catch-up growth (P<0.05). The WAZ was stabilized after Fontan procedure, while the HAZ was reversed (P<0.05) continually. Conclusion The somatic growth is impaired in children with stageⅡ? Fontan procedure. There is a trough of growth before Glenn procedure. Although catch-up growth occurs after Glenn procedure, low body weight and growth retardation remain at follow-up. Effective interventions such as more intensive nutritional strategies before Glenn procedure should be targeted at those children in the future, so as to improve their growth.
Significant atrioventricular valve (AVV) insufficiency has been associated with increased mortality and morbidity in patients with single ventricle. Although techniques such as annuloplasty, chordal elongation, and the use of pericardial patches can improve AVV function in many patients, a number of patients continue to have significant AVV insufficiency and may require AVV replacement. The present review made a comprehensive literature review concerning the outcome, optimal timing, key points of technique skills and prognosis evaluation.
We reported a case of a six-year-old boy diagnosed of single ventricle, pulmonary atresia and interrupted inferior vena cava. After modified Blalock-Taussig shunt and bidirectional Glenn procedure, he received the Fontan procedure. The Fontan procedure was done through a unilateral thoracotomy, using an autologous pericardial conduit to connect hepatic vein and azygos vein. The result of short-term follow-up was satisfactory.
ObjectiveTo evaluate the mid-term results of surgical treatment for functional single ventricle associated with total anomalous pulmonary venous connection.MethodsWe reviewed the clinical data and follow-up results of 12 patients in our hospital who underwent both single ventricle series palliation and total anomalous pulmonary venous connection correction from 2008 to 2018. There were 6 males and 6 females at age of 2.3 (1-21) years. Univariable and multivariable Cox proportional hazard regression methods were performed. The Kaplan-Meier method was used to estimate the survival rate.ResultsAll patients were successfully removed from extracorporeal circulation. The cardiopulmonary bypass time was 113.8±42.5 min. The myocardial block time was 57.7±31.7 min. There were 3 in-hospital deaths and 4 late deaths. The causes of death in hospital were pulmonary hypertension, pulmonary hemorrhage, and hypoxemia. During follow-up, the cause of death was heart failure. The survival rate at 1 year and 3 years was 58.3% (95%CI 27% to 80%) and 40% (95%CI 13% to 65%), respectively. Median follow-up was 48.3 months (range: 1 to 118 months).ConclusionFunctional single ventricle combined with complete pulmonary venous drainage is a serious clinical disease, with poor therapeutic effect and high overall mortality.
目的 總結單心室瓣膜反流的外科治療經驗，觀察治療效果。 方法 回顧性分析2006年7月至2012年1月上海交通大學醫學院附屬新華醫院61例單心室患者的臨床資料，其中男36例，女25例；手術年齡2個月至 20歲；體重2～58 kg。右心室型41例，左心室型13例，未定型型7例。根據瓣膜反流程度不同分為3組，無/微量反流組：28例，瓣膜未行處理；輕/中度反流組：21例，瓣膜未行處理；重度反流組：12例，手術同期行瓣膜成形。收集所有患者住院及隨訪資料，分析輕/中度反流組、重度反流組瓣膜反流變化趨勢，以及影響瓣膜反流的因素。結果 住院死亡5例，住院死亡率8.2% （5/61）。重度反流組患者行瓣膜成形術后反流程度較術前明顯減輕（由術前4.00級下降至術后2.08級）。隨訪56例，隨訪時間6～38個月，重度反流組隨訪10例，隨訪期間死亡2例，其余8例中重度反流2例，中度反流3例，輕度反流2例，微量反流1例；瓣膜反流程度增加趨勢明顯（由術后平均2.08級增加至平均2.75級）。輕/中度反流組隨訪19例，隨訪中無死亡，其中反流程度增加至重度3例（原1例輕度反流，2例中度反流），反流程度由輕度增加至中度3例，瓣膜反流程度由術后平均2.33級增加為平均2.58級。輕/中度反流組瓣膜反流增加率與無/輕微反流組比較差異無統計學意義（瓣膜反流增加率為31.5% vs. 19.2%，χ2=0.36，P=0.55）。單因素分析結果顯示，瓣膜反流增加者在隨訪過程中心功能較瓣膜反流無變化或減輕者明顯降低（術后左心室射血分數53.11%±5.61% vs. 59.65%±3.32%，t =-5.49，P=0.00），而左心室舒張期末容積較瓣膜反流無變化或減輕者明顯增加（t =2.58，P=0.01）。 結論 單心室合并重度瓣膜反流行瓣膜成形術近期效果較好，但隨著心功能下降、心室擴張，瓣膜反流程度加重趨勢明顯；輕/中度瓣膜反流可暫不進行處理，但部分患者瓣膜反流有增加趨勢，提示應注重術后隨訪。