大動脈轉位兒童的生長發展趨勢－病例對照研究

Abstract

研究背景及目的：隨著醫療科技的進步，護理品質的提升，先天性心臟病兒童死亡率逐年的下降，面對存活率的提高，兒童的生長及發展可能受到疾病以及環境等相關因素影響。「生長」及「發展」為兒童成長的兩大面向，其生長特性及發展任務隨著各個階段而有所不同，持續性的改變影響兒童成長至成人的發展。大動脈轉位為發紺型先天性心臟疾病，目前鮮少其生長與發展的相關研究，故本研究目的為評估大動脈轉位兒童之生長及發展，並分析大動脈轉位兒童與一般兒童生長及發展之差異性，以及探討影響大動脈轉位兒童生長及發展的相關因子。 研究方法：本研究以兩種研究方法探討大動脈轉位兒童之生長趨勢及發展特性，首先以回溯性研究收集2004年1月至2015年9月出生診斷為大動脈轉位兒童的基本屬性、家庭環境特性、疾病治療特性以及生長幅度資料，共127位。再以病例對照研究評估2011年9月至2015年9月出生診斷為大動脈轉位兒童之發展特性，採用病例對照法，共28位為病例組；依性別以及年齡配對選取健康兒童28位為對照組，進行病例回顧、問卷調查以及實際發展評估收集資料。分析方法使用統計軟體SPSS 20.0，類別變項運用皮爾森卡方檢測(Pearson chi-square)以及費雪精確性檢定(Fisher’s exact test)；連續性變項運用T檢定以及無母數二獨立樣本檢定(Mann-Whitney U)，並利用ROC曲線(Receiver operating characteristics curves)分析影響發展之加護病房住院天數的敏感度及特異度。 研究結果：分為兩大面向─生長及發展，大動脈轉位兒童生長幅度較緩慢，但體重於六個月後逐漸正常化，身高於一歲後逐漸正常化，其中一歲以下體重小於3百分位與加護病房住院超過28.5天有顯著性關係( p = 0.015)，兩歲及三歲體重小於3百分位與大動脈轉位類型有顯著關係(p = 0.047)(p = 0.018)，以 TGA with VSD、Taussig-Bing syndrome以及Single ventricle診斷需特別注意其生長趨勢。0-4歲大動脈轉位兒童在整體發展評估(p = 0.001)較一般兒童緩慢，特別是粗動作發展(p = 0.01)以及語言發展(p = 0.01)。影響大動脈轉位兒童粗動作發展遲緩之可能危險因子為住院期間需要葉克膜治療(p = 0.011)；影響大動脈轉位兒童語言發展遲緩之可能危險因子為加護病房住院天數(p = 0.004)、加護病房總住院天數(p = 0.012)、出院前鼻胃管存留(p = 0.039)、手術矯正年齡(p = 0.042)以及家庭結構(p = 0.042)；由ROC曲線分析，加護病房住院天數超過23.5天，可預測大動脈轉位兒童需要物理治療介入 (sensitivity = 90.3%, specificity = 67.1%, AUC = 0.835)，加護病房住院天數超過28.5天，可預測需要語言治療(sensitivity = 88.9%, specificity = 71.6%, AUC = 0.845)及職能治療介入 (sensitivity = 100%, specificity = 67.3%, AUC = 0.857)。 討論及結論：大動脈轉位兒童生長趨勢不穩定且發展遲緩比例較高，應提高醫護人員及家屬對於先天性心臟病兒童生長及發展的重視，於手術後恢復期，提早照會營養師及復健師進行評估，以預防及減少生長及發展的問題，於門診定期追蹤時，醫護人員應特別注意高危險性生長發展遲緩的兒童，適時轉介早期療育並長期追蹤。積極鼓勵家屬儘早學習居家照護，以增加病童於住院期間的語言及動作刺激並減少住院天數，並衛教兒童生長及發展的相關知識及定期返診追蹤的重要性。建議未來探討大動脈轉位兒童於學齡期至成人期的生長發展變化，以及早期療育對於大動脈轉位兒童未來發展的重要性。

Background/Objectives: With advanced surgical procedures and nursing care quality, the mortality rate of children with congenital heart disease decreased in recent years. Transposition of the great artery (TGA) is one of the most common cyanotic heart diseases, and most affected the prenatal, neonatal and infant growth and development. With limited knowledge related to this issue, this study is aimed to evaluate the growth and neurodevelopment of children with TGA. Methods: Two research methods were conducted to explore the growth and development in TGA children. First, a retrospective chart review was conducted to evaluate the growth curve of 127 TGA children born from January 2004 to September 2015 in a university children hospital in Taiwan. Second, a case-control study was adopted to compare the neurodevelopmental outcome between control (n=28) and TGA (n=28) children born between September 2011 and September 2015. Data of clinical characteristics, family resources, disease status, and growth curves were collected to analyze predictors. Categorical data was analyzed by chi-square or Fisher’s exact test and continuous variables were analyzed by t test or Mann-Whitney U test. Receiver operating characteristic (ROC) curves were analyzed to identify a cutoff length of intensive care unit (ICU) stay affecting development. Results: The observed body weight slowly increased from the 15th to the 25th percentile in 6-month-old and from the 25th to the 50th percentile in 1-year-old TGA children. Body weight lower than the third percentile was significantly related to the type of TGA at 2 (p = 0.047) and 3 (p = 0.018) years of age. TGA children with ICU stays of >28.5 days had body weights lower than the third percentile at 1 year of age (p = 0.015).The neurodevelopment delay are significant in TGA children compared with control group (p = 0.001), especially in gross motor (p = 0.01) and language development (p = 0.01). Associated risk factors with gross motor delay is ECMO treatment (p = 0.01). Factors with language delay are length of ICU stays (p = 0.004), remain of nasogastric tube feeding before discharge (p = 0.039), the age of surgical correction (p = 0.042) and family structure (p = 0.042). Most importantly, length of ICU stays more than 32.5days was a predictor for TGA children with language delay [sensitivity = 77.8%, specificity = 84.2%, area under the curve (AUC) = 0.827]. Length of ICU stays more than 28.5days was a predictor for TGA children need to language theraphy (sensitivity = 88.9%, specificity = 71.6%, AUC = 0.845). Length of ICU stays more than 23.5days was a predictor for TGA children need to physical therapy or occupational therapy (sensitivity = 90.9%, specificity = 68.7%, AUC = 0.846). Result and Discussion: TGA children are at a higher risk of developmental impairment and unstable growth trends. We should stress more emphasis on the importance of growth and developmental follow-up in this population. We should consulted nutritionist and rehabilitation specialist for early assessment in post-operative periods to avoid long term problem. It is necessary to encourage family to learn home care education during hospitalization and increased language and motor stimulation to decreased hospital stays. Early evaluation and rehabilitation programs are crucial for these children and their families.