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Objective:To investigate the cause of missed diagnosis by transthoracic echocardiography (TTE) based on the characteristics of partial anomalous pulmonary venous connection (PAPVC), and to improve the ultrasonic diagnostic accuracy of PAPVC in children.Methods:The TTE results of 252 children under 12 years old who were confirmed with PAPVC at Guangdong Provincial People′s Hospital from January 2011 to June 2019 were reviewed retrospectively.The types of PAPVC and the associated atrial septal defects (ASD) as well as the confirmed and missed cases by TTE were analyzed.Results:PAPVC was right-sided in 238 patients (94.4%), left-sided in 8 patients (3.2%), and bilateral in 6 patients (2.4%). There were 177 cases (70.2%) whose pulmonary veins were abnorma-lly connected to the right atrium(RA), 37 cases (14.7%) to the junction of the RA and the superior vena cava (SVC), 27 cases (10.7%) to the distal SVC, and 6 cases (2.4%) to the inferior vena cava.Besides, pulmonary veins of 5 cases (2.0%) flew back to the RA through the coronary sinus.One hundred and ninety PAPVC cases were combined with sinus venous defects (SVD) and 53 cases combined with secondum ASD.Two hundred and twenty-one cases were accurately diagnosed while 31 cases were underestimated by TTE.The omission diagnostic rates of right superior pulmonary veins connecting to the distal SVC, 1 or 2 right pulmonary veins connecting to the RA or the junction of the RA and SVC, and left-sided PAPVC were 8 out of 18 (44.4%), 22 out of 215 (10.2%) and 1 out of 8 (12.5%), respectively.Among 54 cases with right superior pulmonary veins anomalously connected to the RA or the junction of the RA and SVC, 88.9% of them (48/54 cases) were combined with superior SVD.Among 161 cases with the right inferior pulmonary veins or 2 right pulmonary veins connected to the RA, 78.2% of them (126/161 cases) were combined with inferior SVD.There were statistically significant differences in ASD types between the 2 right-sided PAPVC groups.Conclusions:SVD is often associated with 1 or 2 right pulmonary veins connected to the RA or the junction of the RA and SVC.The cases with right superior pulmonary veins connected to the distal SVC are prone to be underestimated by TTE.Whether there is abnormal blood flowing into SVC should be noticed during superior sternal fossa examinations.Each pulmonary vein should be examined in detail in the TTE test and accurate diagnosis of PAPVC can be made in most cases.
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Objective To observe the clinical characteristics and improve the diagnosis and treatment of anomalous aortic origin of a coronary artery from the opposite sinus (AAOCA)in children and adolescents. Methods Between January 2012 and December 2016,11 children/ adolescents [8 were boys,3 were girls,age was (10. 1 ± 5. 6) years old]diagnosed with AAOCA were retrospectively reviewed at Department Pediatric Cordiology,Guangdong General Hospital. The clinical features,transthoracic echocardiography (TTE),computed tomography images,electro-cardiogram (ECG),cardinc troponin I(CTnI)and creatinine kinase - MB(CK - MB)were analyzed. Results The di-agnosis of AAOCA was confirmed by TTE and computed tomography images in all 11 cases. Left coronary artery origina-ted from the right sinus in 5 patients,and right artery coronary originated from the left sinus in 6 cases. Five patients with intramural course had the history of exercise - related syncope. Of these 5 cases with exercise - related syncope,4 cases were diagnosed as left coronary artery originated from the right sinus and 1 case was diagnosed as right artery coronary originated from the left sinus. ECG,CTnI and CK - MB revealed acute myocardial ischemia in these patients with exercise - related syncope. Unroofing procedures were undergone in these 5 patients. The remaining 6 patients had no syncope and symptoms of cardia ischemia,sports activities were restricted and follow - ups were counseled in these 6 patients. There was no sudden death events later,and none of the patients demonstrated any evidence of new myocardial ischemia in the follow - up study. Conclusions AAOCA can be associated with syncope and myocardial ischemia in children and adolescents. The correct diagnosis of AAOCA requires a very high index of suspicion. Sports activities restriction and follow - up should be counseled in cases with AAOCA. Unroofing procedures should be performed in symptomatic cases with AAOCA and an intramural course.
ABSTRACT
Objective To summarize the echocardiographic features of fetal absent pulmonary valve syndrome (APVS).Methods Clinical data and echocardiograms of 12 cases of fetal APVS prenatally diagnosed in the Department of Pediatric Cardiology,Guangdong Cardiovascular Institute from July 2015 to April 2017 were reviewed retrospectively and the echocardiographic features of APVS were summarized.Results Aneurysmal dilation of the main pulmonary artery and branches arteries were found in all cases,causing tracheal and esophageal compression in one case and lung compression in two cases.Color Doppler revealed systolic and diastolic turbulent blood flow with "to-and-fro" blood flow in pulmonary arteries and pulmonary regurgitation.All fetuses were situs solitus and atrioventricular concordance.Eleven cases were also identified with malposition of the great arteries accompanied with ventricular septal defect,while the other one with normally related great arteries and intact ventricular septum was diagnosed with dysplasia of distal left pulmonary artery and pericardial effusion.Absent ductus arteriosus,increased cardiothoracic ratio and myocardial hypertrophy were found in four,two and five cases,respectively.Fetal umbilical arterial systolic/diastolic ratio and resistance index of all cases were normal.Conclusions Typical APVS is characterized by absent or rudimentary pulmonary valve accompanied with prominant dilated pulmonary arteries,pulmonary valve stenosis and severe pulmonary regurgitation.It is usually accompanied by conotruncal defects,but attention should also be paid to other atypical cardiac abnormalities during screening.