The prenatal diagnosis classification and prognostic evaluation in fetal pulmonary atresia with ventricular septal defect
	    		
		   		
		   			
		   		
	    	
    	 
    	10.3760/cma.j.issn.1004‐4477.2019.06.006
   		
        
        	
        		- VernacularTitle:肺动脉闭锁合并室间隔缺损的产前超声诊断分型及预后评估
 
        	
        	
        	
        		- Author:
	        		
		        		
		        		
			        		In LIU
			        		
			        		
			        		
			        			1
			        			
			        		
			        		
			        		
			        		
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			        		Hongdan WANG
			        		
			        		;
		        		
		        		
		        		
			        		Cunying CUI
			        		
			        		;
		        		
		        		
		        		
			        		Yanan LI
			        		
			        		;
		        		
		        		
		        		
			        		Yuanyuan LIU
			        		
			        		;
		        		
		        		
		        		
			        		Ying WANG
			        		
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			        		Taibing FAN
			        		
			        		;
		        		
		        		
		        		
			        		Bangtian PENG
			        		
			        		
		        		
		        		
		        		
		        		
		        			
			        		
			        		Author Information
			        		
		        		
		        		
			        		
			        		
			        			1. 河南省人民医院心脏中心 阜外华中心血管病医院 郑州大学人民医院心脏中心超声科450003
			        		
		        		
	        		
        		 
        	
        	
        	
        	
        		- Keywords:
        			
	        			
	        				
	        				
			        		
				        		Echocardiography,prenatal;
			        		
			        		
			        		
				        		Pulmonary atresia with ventricular septal defect;
			        		
			        		
			        		
				        		Prognosis;
			        		
			        		
			        		
				        		McGoon index
			        		
			        		
	        			
        			
        		
 
        	
            
            
            	- From:
	            		
	            			Chinese Journal of Ultrasonography
	            		
	            		 2019;28(6):493-499
	            	
            	
 
            
            
            	- CountryChina
 
            
            
            	- Language:Chinese
 
            
            
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		        	Abstract:
			       	
			       		
				        
				        	To explore the prenatal diagnosis classification and prognostic evaluation of fetal pulmonary atresia with ventricular septal defect ( PA/VSD ) . Methods T hirty‐one fetal pulmonary atresia with ventricular septal defect were classified Ⅰ - Ⅳ type by Boston classification ,and the McGoon indexes were calculated ,w hether associated with malformation and chromosomal abnormalities ,and follow‐up . Results T hirteen fetuses were diagnosed type Ⅰ PA/VSD , 6 fetuses were associated with malformation ,2 fetuses were chromosomal abnormalities , 7 fetuses′ McGoon index > 1 .20 ,6 fetuses′McGoon index<1 .20 ,8 cases had operation ( 6 cases had radical operation and had a good follow up ,2 cases had palliative operation and were waiting for radical operation) , 5 cases received termination of pregnancy . Six fetuses were diagnosed as type Ⅱ PA/VSD ,5 fetuses were associated with malformation ,1 fetus was chromosomal abnormalities ,1 fetus′s McGoon index> 1 .20 ,5 fetuses′ McGoon index< 1 .20 ,2 cases had operation ( 1 case had radical operation and had a good follow up ,1 case had palliative operation and was waiting for radical operation) ,4 fetuses received termination of pregnancy . Four fetuses were diagnosed as type Ⅲ PA/VSD ,3 fetuses were associated with malformation ,no fetus was chromosomal abnormalities ,4 fetuses′McGoon index<1 .20 ,1 case had palliative operation and was waiting for radical operation , 3 cases received termination of pregnancy . Eight fetuses were diagnosed as type Ⅳ PA/VSD ,3 fetuses were associated with malformation , 3 fetuses were chromosomal abnormalities , 1 case had unifocalization operation ,but died after operation in one day ,7 cases received termination of pregnancy . T he area under the ROC curve of McGoon index in hinting PA/VSD postnatal radical operation was 1 .000 ( P = 0 .002 ,95%CI :1 .0000 - 1 .000 ) , the border value was 1 .255 , the sensitivity and specificity were 100% ,85 .7% , respectively . Conclusions Echocardiography can diagnose the classification of fetal PA/VSD . The radical operation for cases of McGoon index >1 .255 is feasible ,the cases of type Ⅳ PA/VSD and PA/VSD with associated malformation and chromosomal abnormalities have a poor follow up .