左向右分流型先天性心脏病合并肺动脉高压119例临床分析

发布时间：2018-09-03 17:26

【摘要】：目的：为了提高左向右分流型先天性心脏病合并肺动脉高压的治疗效果,分析总结119例患儿的诊疗经过及随访情况。方法：回顾性分析我科自2005年1月-2010年12月收治的119例左向右分流型先天性心脏病合并肺动脉高压患儿的临床资料,患儿年龄1月-15岁,平均42.4±2.8月,其中男74例,女45例。通过超声心动图等辅助检查,部分患儿行心导管检查,明确肺动脉高压的程度。所有患儿接受内科药物治疗,部分患儿行介入治疗,部分患儿行外科手术治疗,出院后定期门诊随访。结果：119例患儿中左向右分流型先天性心脏病的类型包括单纯室间隔缺损42例,室间隔缺损合并房间隔缺损8例,室间隔缺损合并动脉导管未闭7例；单纯房间隔缺损者29例,房间隔缺损合并动脉导管未闭5例；单纯动脉导管未闭者28例。全组患儿的临床症状和体征中以频繁上呼吸道感染、气促和心脏杂音、P2亢进最为常见。辅助检查中,胸片显示心胸比率0.48-0.72；大多数患儿心电图提示右心室或双心室肥厚。所有患儿行超声心动图检查,治疗前测得全组患儿肺动脉压力28～102(66.52±11.33)mmHg。所有患儿均接受内科药物治疗。全组病人中有66例患儿接受介入治疗,其中1例术后1天发生溶血,最终全部治愈出院。有53例患儿接受外科手术治疗,49例经积极治疗后好转出院；外科治疗者术后发生切口感染1例,上呼吸道感染8例,并发肺炎1例,心律失常8例,住院死亡4例,分别死于低心排综合征和肺动脉高压危象。治疗后全组患儿肺动脉压力8-90(20.23±7.13)mmHg,p0.01,其中肺动脉压力下降40mmHg以上者79例(66.4%)。全组病人中有17例系合并重度肺动脉高压者,经术前药物控制后行外科手术,术后14例(82%)肺动脉压力较术前下降20mmHg,2例出现低心排综合征,1例出现肺动脉高压危象。术后3个月、6个月、1年复查超声心动图显示术后早期肺动脉压力变化不显著(81.3±13.2mmHg)。出院后对所有患儿进行长期随访,有98例(82.4%)患儿获得随访。随访时间1个月-5年,患儿心胸比、心腔大小、左心功能均较治疗前有所改善。结论：(1)对左向右分流型先天性心脏病合并肺动脉高压患者应加强早期诊断,及时选择介入或者手术治疗时机,可改善心脏的结构和功能。(2)对于合并中重度肺动脉高压的先天性心脏病患儿,适时把握手术指征,可一定程度地提高治愈率。(3)超声心动图在诊断先天性心脏病及评估肺动脉压力中具有重要价值。
[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 卤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 卤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 卤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 卤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.
【学位授予单位】：广西医科大学
【学位级别】：硕士
【学位授予年份】：2012
【分类号】：R725.4

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