發(fā)布時間：2018-09-04 19:25

【摘要】：目的 探討唐氏綜合征（DS）與先天性心臟病（CHD）的關(guān)系。 方法 回顧性分析1997年1月至2013年3月重慶醫(yī)科大學(xué)附屬兒童醫(yī)院收治的575例DS患者的臨床資料，分析DS合并CHD的患病率、類型、染色體核型與CHD類型之間的關(guān)系、肺動脈壓力及手術(shù)治療情況。 結(jié)果 共收治575例DS患者，，合并CHD370例（64.35%），其中間隔類缺損322例（87.03%），包括ASD57例（15.41%），VSD36例（9.72%），AVSD12例（3.24%），復(fù)合間隔類缺損217例（58.65%）。非間隔缺損類先心（包括PDA、TOF、DORV、PA等）48例（12.97%）。DS不同核型與CHD類型無明顯統(tǒng)計學(xué)差異。246例（66.49%）合并肺動脈壓力升高。70例（18.92%）患者進(jìn)行了介入或外科治療，術(shù)后均有不同程度肺動脈壓力下降。40例患者合并其他畸形，如胃腸道畸形、多指/趾、視力障礙等。 結(jié)論 DS與CHD發(fā)生密切相關(guān)。合并的CHD類型以間隔類缺損為主，ASD最為常見，其次為VSD。染色體核型與CHD類型無關(guān)。DS合并CHD容易早期出現(xiàn)肺動脈高壓，應(yīng)早期進(jìn)行評估并制定綜合治療方案。
[Abstract]:Objective to investigate the relationship between Down's syndrome (DS) and congenital heart disease (CHD). Methods the clinical data of 575 DS patients admitted to the affiliated Children's Hospital of Chongqing Medical University from January 1997 to March 2013 were analyzed retrospectively. The prevalence, type, chromosome karyotype and CHD type of DS complicated with CHD were analyzed. Pulmonary artery pressure and surgical treatment. Results 575 cases (64.35%) of DS were treated, including 322 cases (87.03%) of septal defect, including 36 cases (9.72%) of ASD57 with VSD, and 217 cases (58.65%) of complex septal defect. 48 cases (12.97%) of non-septal defect (including PDA,TOF,DORV,PA et al). There was no significant difference between different karyotype of DS and CHD type. 246 cases (66.49%) were complicated with pulmonary artery pressure elevation. 70 cases (18.92%) were treated with interventional or surgical treatment. There were 40 patients with different degree of pulmonary artery pressure drop and other deformities, such as gastrointestinal malformation, multiple fingers / toes, visual impairment and so on. Conclusion DS is closely related to CHD. Septal defect was the most common type of CHD, followed by VSD.. Chromosome karyotype is not related to CHD type. DS combined with CHD is prone to early pulmonary hypertension.
【學(xué)位授予單位】：重慶醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2014
【分類號】：R714.5

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