The application value of Juxtaposition of the great vessels in diagnosis of right atrial isomerisim / 中华胸心血管外科杂志

Objective To assess the application value of juxtaposition of the great vessels in diagnosis of right atrial isom-erism( RAI) by echocardiography.Methods We retrospectively analyzed 73 patients with right atrial isomerism in Guangzhou Women and Children's Medical Center from 2010-2017.All these patients were performed by echocardiography and compu-ted tomography(CT).We used the results of CT as the golden standard.Results 73 cases of RAI were diagnosed by CT.60 cases of RAI were diagnosed by echocardiography.Juxtaposition of the descending aorta(DAO) and inferior vena(IVC) was demonstrated in all these 60 patients.29 of 60 were on the left side of the spine(39.73％).31 of 60 were on the right side of spine(42.47％).In the other 13 patients of echocardiography missed diagnosis, the arrangement of the great vessels was nor-mal in 10 cases, and inverse in 3 cases.Conclusion Juxtaposition of the great vessels is one of the most important findings in patients with RAI.It is quick and simple and has a higher application value in diagnosis of RAI.

Comparison of the prenatal ultrasound,autopsy and vascular cast in diagnosis of right atrial isomerism / 中华超声影像学杂志

Objective To explore the value of prenatal ultrasound,autopsy and vascular cast in diagnosis of right atrial isomerism(RAI).Methods Forty cases diagnosed as RAI by prenatal ultrasound from May 2012 to May 2017 were analyzed.And the results of autopsy and vascular cast after odinopoeia were compared,then up the characteristics of RAI were summed.The feature of venous drainage was the point.Results For all the 40 cases,the relative position of magenblase,the cross section of aortaventralis, the cross section of inferior vena cava and umbilical vein were lost"C"shape view.In intracardiac anomalies magenblase and cardiac apex on the different sides accounted for 77.5%,the atrioventricular septal defect accounted for 67.5%,the right ventricle double outlet accounted for 65.0%,and pulmonary artery stenosis accounted for 52.5%.The major finding of venous drainage were:bilateral superior vena cava accounted for 52.5%,left inferior vena cava accounted for 60.0%,abnormal hepatic venous drainage accounted for 20.0%,pulmonary vein co-antrum accounted for 45.0%.Three cases of cardiac malformation and 4 cases of abnormal pulmonary venous drainage were misdiagnosed,and 4 cases of abnormal hepatic venous drainage were missed by prenatal ultrasound.Conclusions RAI always combines distinctive cardiac malformations and abnormal venous drainage.Prenatal ultrasound has significant diagnostic value.It's important to have further understanding about RAI through the feedback of autopsy and vascular cast.

Isolated and Complex Scimitar Vein Anomalies and Their Differentiation from the Meandering Right Pulmonary Vein

PURPOSE: Four pediatric patients with isolated (the adult form) and complex (the infantile form) scimitar vein anomalies were reviewed and compared with patients with meandering right pulmonary veins. MATERIALS AND METHODS: From January 1990 to December 2006, 4 female patients, aged 2 days to 3.5 years, with isolated and complex scimitar vein anomalies were retrospectively studied. The clinical features, chest radiographs, echocardiography, magnetic resonance imaging, magnetic resonance angiography, computer tomography, bronchography, cardiac catheterization with angiography, surgery, and autopsy were reviewed to substantiate the diagnosis of isolated and complex scimitar vein anomalies. RESULTS: Clinical manifestations were lung infections with radiographic scimitar signs in 4 patients (3 right; 1 left), respiratory distress, congestive heart failure, pulmonary hypertension and/or cyanosis in 3 patients, dextrocardia, heterotaxy, and/or right atrial isomerism in 2 patients, as well as dextroversion, right bronchial isomerism, bronchial stenosis, and/or sepsis in 1 patient. Two patients with right atrial isomerism expired. CONCLUSION: The clinical discrimination between scimitar vein anomaly with and without cardiovascular and bronchopulmonary malformations is crucial for the outcomes vary.

Dextroisomerismo atrial en adultos: Estudio clínico-ecocardiográfico / Right atrial isomerism in adults: Clinical and echocardiographic study

La esencia del dextroisomerismo en el corazón es la presencia bilateral de atrios con orejuelas de morfología derecha. Generalmente forma parte de un grupo de cardiopatías congénitas complejas. El objetivo de nuestro estudio es hacer una descripción de los hallazgos clínicos y ecocardiográficos en los pacientes con dextroisomerismo atrial que han sobrevivido a la vida adulta. Se estudiaron 11 pacientes adultos con cardiopatías congénitas complejas y dextroisomerismo. A todos los pacientes se les realizó historia clínica completa, ecocardiografía transtorácica y transesofágica. La clase funcional según New York Heart Association al inicio del estudio fue: I en un paciente, II en 5 y III en 5. Los estudios de laboratorio mostraron poliglobulia importante. En tres casos se encontraron cuerpos de Howell Jolly El electrocardiograma de superficie mostró marcapaso migratorio en el 54.5%. Seis (54.5%) pacientes estuvieron en dextrocardia y 5 (45.5%) en levocardia. Seis pacientes (54.5%) tuvieron defectos de la tabicación atrioventricular, 4 con doble entrada (36.4%) y uno con ausencia de conexión auriculoventricular derecha (9.1%). La conexión ventriculoarterial más frecuente fue la doble salida en 8 (72.76%). Seis casos tuvieron estenosis pulmonary uno atresia pulmonar. El retorno venoso pulmonar fue al lado izquierdo del atrio común en tres casos (27.2%), al lado derecho del atrio común en cuatro (36.4%), al techo del atrio común a través de un colector en 3 casos (27.4%) y vía vena vertical en uno (9.0%). Ninguno de ellos presentó obstrucción. Se encontró insuficiencia de la válvula atrioventricular en 7 casos (63.6%). Tres pacientes (27.3%) fueron llevados a cirugía para colocación de fístula. Su clase funcional mejoró. Se trata de una serie de casos en pacientes adultos con dextroisomerismo atrial y cardiopatías congénitas complejas que a pesar de ello llegaron a la vida adulta. La valoración clínica y ecocardiográfica nos permiten en la actualidad establecer el diagnóstico, tomar decisiones terapéuticas y hacer el seguimiento. Aún falta por determinar qué diferencias tendrán con el resto de la población cuando se presenten los padecimientos crónicos degenerativos.

The most important feature of right atrial isomerism in the heart is the presence of both atria with morphologically right atrial appendages. The main aim of this study was to do a description of clinical and echocardiographic findings in adult patients with right atrial isomerism. A total of eleven consecutive patients were identified with a diagnosis of right atrial isomerism and complex congenital heart disease. A complete clinical history and transthoracic and/or transesophageal echocardiography were performed in all patients. Also a cardiac catheterization was realized in seven patients. The functional class according the New York Heart Association was I in one patient, II in 5 and III in 5. The laboratory studies showed severe polyglobulia. In 3 cases Howell-Jolly bodies were present. The electrocardiogram showed migratory pacemaker in the 54.5% of cases. The cardiac position was dextrocardia in six patients (54.5%) and levocardia in five (45.5%). Six patients (54.5%) had complete atrioventricular septal defects, 4 (36.4%) double inlet and one absence of a right atrioventricular connection (9.1%). The most frequent ventriculoarterial connection was double-inlet in 8 (72.76%) patients. In 4 cases hypopla-sia of the pulmonary artery was found. Six patients had severe pulmonary stenosis and one pulmonary atresia. The pulmonary venous drainage was into the left-sided atrium in 3 cases (27.2%), into the right-sided atrium in 4 (36.4%), into the midline of the atrium through a collector in 3 cases (27.4%) and by vertical vein in one (9.0%). Insufficiency of the atrioventricular valve was found in 7 cases (63.6%). Three patients (27.3%) went to surgery for implantation of a fistula. It is a series of adult patients with right atrial isomerism and complex congenital heart disease that survived to adult live. The clinical and echocardiographic assessment actually allows establishing the correct diagnosis, to take therapeutic decisions and to do the follow-up in all patients. Till now we could not yet determine the differences of these patients in relation to the general population when they develop chronic degenerative disease.

Follow up of Patients with Total Anomalous Pulmonary Venous Return in Right Atrial Isomerism

PURPOSE: Total anomalous venous return(TAPVR) is associated in more than 60Yo of patients with right isomerism and can significantly complicate the management of single ventricle patients at any stage of management. We studied the results of management and sought to determine factors that may influence survival in patients with TAPVR in right atrial isomerism. METHODS: Between February 1991 and July 1999, 14 patients with TAPVR in right atrial isomerism underwent operations,' we reviewed our experience after performing single ventricle palliation RESULTS: Seven patients were of the obstructive type TAPVR and seven patients were of the non-obstructive type TAPVR. The mean age at operation was 17 months and mean body weight at operation was 7.3kg. Direct surgical repair for the pulmonary vein was performed in seven patients and in the others, TAPVR could be satisfactorily managed by the use of bilateral cavopulrnonary anastomosis(BCPS) to exclude the distal superior vena cava(SVC). At a mean follow-up of 27 months, there were five deaths, arid pulrnonary vein restenosis developed in four patients in the direct surgical repair group. In the other group, there was neither mortality nor morbidity. Also, the presence of pulmonary venous obstruction was associated with high mortality. CONCLUSION: According to our study, TAPVR can be satisfactorily managed by the use of BCPS to exclude the distal SVC in the non-obstructive type. But further evaluation of surgical methods about other types of TAPVR are warranted, because TAPVR not requiring intervention includes any low supracardiac and some mixed types.