【摘要】：目的：為了提高左向右分流型先天性心臟病合并肺動脈高壓的治療效果,分析總結119例患兒的診療經過及隨訪情況。 方法：回顧性分析我科自2005年1月-2010年12月收治的119例左向右分流型先天性心臟病合并肺動脈高壓患兒的臨床資料,患兒年齡1月-15歲,平均42.4±2.8月,其中男74例,女45例。通過超聲心動圖等輔助檢查,部分患兒行心導管檢查,明確肺動脈高壓的程度。所有患兒接受內科藥物治療,部分患兒行介入治療,部分患兒行外科手術治療,出院后定期門診隨訪。 結果：119例患兒中左向右分流型先天性心臟病的類型包括單純室間隔缺損42例,室間隔缺損合并房間隔缺損8例,室間隔缺損合并動脈導管未閉7例；單純房間隔缺損者29例,房間隔缺損合并動脈導管未閉5例；單純動脈導管未閉者28例。全組患兒的臨床癥狀和體征中以頻繁上呼吸道感染、氣促和心臟雜音、P2亢進最為常見。輔助檢查中,胸片顯示心胸比率0.48-0.72；大多數患兒心電圖提示右心室或雙心室肥厚。所有患兒行超聲心動圖檢查,治療前測得全組患兒肺動脈壓力28～102(66.52±11.33)mmHg。所有患兒均接受內科藥物治療。全組病人中有66例患兒接受介入治療,其中1例術后1天發(fā)生溶血,最終全部治愈出院。有53例患兒接受外科手術治療,49例經積極治療后好轉出院；外科治療者術后發(fā)生切口感染1例,上呼吸道感染8例,并發(fā)肺炎1例,心律失常8例,住院死亡4例,分別死于低心排綜合征和肺動脈高壓危象。治療后全組患兒肺動脈壓力8-90(20.23±7.13)mmHg,p0.01,其中肺動脈壓力下降40mmHg以上者79例(66.4%)。全組病人中有17例系合并重度肺動脈高壓者,經術前藥物控制后行外科手術,術后14例(82%)肺動脈壓力較術前下降20mmHg,2例出現低心排綜合征,1例出現肺動脈高壓危象。術后3個月、6個月、1年復查超聲心動圖顯示術后早期肺動脈壓力變化不顯著(81.3±13.2mmHg)。出院后對所有患兒進行長期隨訪,有98例(82.4%)患兒獲得隨訪。隨訪時間1個月-5年,患兒心胸比、心腔大小、左心功能均較治療前有所改善。 結論：(1)對左向右分流型先天性心臟病合并肺動脈高壓患者應加強早期診斷,及時選擇介入或者手術治療時機,可改善心臟的結構和功能。(2)對于合并中重度肺動脈高壓的先天性心臟病患兒,適時把握手術指征,可一定程度地提高治愈率。(3)超聲心動圖在診斷先天性心臟病及評估肺動脈壓力中具有重要價值。
[Abstract]:Objective: to improve the effect of left to right shunt congenital heart disease with pulmonary hypertension. Methods: the clinical data of 119 patients with left to right shunt congenital heart disease complicated with pulmonary hypertension from January 2005 to December 2010 were retrospectively analyzed. The mean age of the patients was 42.4 鹵2.8 months, including 74 males and 45 females. By echocardiography and other auxiliary examinations, some children underwent cardiac catheterization to determine the degree of pulmonary hypertension. All the children received medical treatment, some received interventional therapy, some received surgical treatment, and followed up regularly after discharge. Results the types of congenital heart disease of left to right shunt type included 42 cases of pure ventricular septal defect, 8 cases of ventricular septal defect with atrial septal defect, 7 cases of ventricular septal defect with patent ductus arteriosus, 29 cases of simple atrial septal defect. There were 5 cases of atrial septal defect complicated with patent ductus arteriosus and 28 cases of pure patent ductus arteriosus. Frequent upper respiratory tract infection, shortness of breath and cardiac murmur were the most common clinical symptoms and signs. Chest radiographs showed cardiothoracic ratio of 0.48-0.72.The ECG of most children showed right ventricular or biventricular hypertrophy. Echocardiography was performed in all children. The pulmonary artery pressure of all children was measured before treatment. The pulmonary artery pressure was (66.52 鹵11.33) mmHg.. All the children received medical treatment. Among the 66 cases received interventional therapy, 1 case developed hemolysis 1 day after operation and was cured and discharged from hospital. There were 53 cases of surgical treatment and 49 cases of improvement and discharge after active treatment, 1 case of incision infection, 8 cases of upper respiratory tract infection, 1 case of complicated pneumonia, 8 cases of arrhythmia, 4 cases of death in hospital. Died of low cardiac output syndrome and pulmonary hypertension crisis, respectively. After treatment, pulmonary artery pressure was 8-90 (20.23 鹵7.13) mmHg,p0.01, of which 79 cases (66.4%) had decreased pulmonary artery pressure above 40mmHg. Among the 17 patients with severe pulmonary hypertension, 14 patients (82%) had pulmonary artery pressure decreased 20 mm after operation, 2 cases had low cardiac output syndrome and 1 case had pulmonary hypertension crisis. After 3 months, 6 months and 1 year, echocardiography showed no significant change of pulmonary artery pressure (81.3 鹵13.2mmHg). 98 cases (82.4%) were followed up after discharge. The follow-up time was 1 month-5 years. The heart-chest ratio, heart cavity size and left ventricular function were improved. Conclusion: (1) early diagnosis should be strengthened in patients with left to right shunt congenital heart disease complicated with pulmonary hypertension. It can improve the structure and function of the heart. (2) for the children with congenital heart disease complicated with moderate and severe pulmonary hypertension, the operative indications should be grasped at the right time. (3) Echocardiography has important value in diagnosing congenital heart disease and evaluating pulmonary artery pressure.
【學位授予單位】：廣西醫(yī)科大學
【學位級別】：碩士
【學位授予年份】：2012
【分類號】：R725.4

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