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.

A Case of Residual Atrial Septal Defect with Left Atrial Isomerism Requiring Reoperation 37 Years after the First Operation / 日本心臓血管外科学会雑誌

A 47-year-old female was admitted to our hospital for management of dyspnea. She had undergone surgery for an atrial septal defect (ASD) at the age of 17. Computed tomography revealed left isomerism, inferior vena cava interruption with azygos continuation and a residual ASD. Intra-operative findings showed that the residual ASD was positioned across the orifice of the hepatic vein. The previous suture line could be identified in the partially-closed atrial septum above the residual defect. Re-closure was performed without difficulties, and the patient's condition was good at discharge. Closure of ASD is a simple and basic procedure in cardiac surgery but care must be taken not to leave a shunt at the lower part of the defect, especially in cases with ASD defects in the lower margin.

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.

Clinical Characteristics and Independent Factors Related to Long-Term Outcomes in Patients with Left Isomerism

BACKGROUND AND OBJECTIVES: We studied the results of patient management for left isomerism (LI) and sought to determine factors that may influence survival and prognosis. SUBJECTS AND METHODS: We reviewed the medical records of 76 patients who were compatible with LI criteria between 1982 and 2014. RESULTS: Of the total study population, 29 patients (38.1%) had functional univentricular heart disease, 43 patients (56.5%) had cardiac anomalies suitable for biventricular hearts, and four patients (5.2%) had normal heart structure. Extracardiac anomalies were noted in 38.1% of the study population, including biliary atresia in 7.8% of all patients. Of the 25 patients who underwent Kawashima procedures, 24.0% developed pulmonary arteriovenous fistulas (PAVFs). During the median follow-up period of 11.4 years (range: 1 day to 32 years), 14 patients died. The 10-year, 20-year, and 30-year survival rates were 87%, 84%, and 76%, respectively. Preoperative dysrhythmia and uncorrected atrioventricular valve regurgitation were significantly associated with late death. There was no significant difference in the number of surgical procedures and in survival expectancy between patients in the functional single-ventricle group and in the biventricular group. However, late mortality was higher in functional single-ventricle patients after 18 years of age. CONCLUSION: Patients with LI need to be carefully followed, not only for late cardiovascular problems such as dysrhythmia, valve regurgitation, and the development of PAVFs, but also for noncardiac systemic manifestations.

Results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly / 中华胸心血管外科杂志

Objective To evaluate the results of surgical treatment for patients with heterotaxy syndrome and cardiac anomaly.Methods From September 2008 to October 2013,34 patients with heterotaxy syndrome underwent 46 cardiac operations at Guangzhou Women and Children Medical Center.22 were male,and 12 female.Mean age at operation was 22 months (4 months to 14 year).There were right atrial isomerism 24 cases,left atrial isomerism 10 cases.Only one had a single VSD,and others were all with complex cardiac anomaly,including complete atrioventricular canal defect 26 cases,tricuspid atresia 4 cases,mitral atresia 1 case,anomalous pulmonary venous connection 18 cases,pulmonary venous stenosis 4 cases,double superior vena cava 17 cases,and interrupted hepatic portion of the inferior vena cava 5 cases.Fontan procedure was performed in 5 patients,bidirectional Glenn in 13,Kawashima in 3,central shunt in 1,atrioventricular valve replacement in 5,and pulmonary artery banding in 5.Results There were 4 died during hospitalization.The follow-up duration was 1 month to 5 years.There were 2 died.The early to middle term mortality was 17.6％ (6/34).In the same period the mortality for 103 patients without heterotaxy syndrome underwent univentricular repair was 4.8％ (5/103).There was significant statistical difference (P ＜ 0.05).Conclusion Right heart bypass operation remains the preferred palliative procedure for patients with heterotaxy syndrome,and the eraly and middle term results were satisfied.

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.

Atrial Rhythms in Patients with Left Atrial Isomerism

PURPOSE: Histologic studies have revealed ectopic location and hypoplasia of sinus nodal tissue in left atrial isomerism. The purpose of this study is to characterize the atrial rhythms corresponding to these histologic abnormalities in left atrial isomerism. Electrocardiograms in left atrial isomerism are characterized by multiple atrial rhythms with abnormal P-wave axes, but the complex nature of atrial rhythms in left atrial isomerism remains to be clarified. METHODS: Surface electrocardiograms and Holter recordings of 16 patients with left atrial isomerism were retrospectively reviewed. For comparison, atrial rhythms in 8 patients with right atrial isomerism were studied with the same study protocol. RESULTS: In left atrial isomerism, atrial rhythm with abnormal superior P-wave axis was the most frequent in 69% of patients and multiple atrial rhythms were observed in 37% in surface electrocardiograms. In Holter recordings, transient or persistent slow atrial rhythms with or without visible P waves associated with junctional escape were documented in 9 out of 16(56%) patients. In comparison, multiple atrial rhythms were as frequent as in left atrial isomerism, but slow atrial rhythm with junctional escape was not present in 8 patients with right atrial isomerism. CONCLUSION: It is concluded that transient or persistent slow atrial rhythms associated with junctional escape, suggestive of atrial nodal dysfunction, are characteristic and helpful in the diagnosis of left atrial isomerism.

Developmental Morphology of the Atrial Venous Component in the iv/iv Mouse

PURPOSE: Developmental processes of atria in embryos or fetuses of the iv/iv mouse were studied to find the differences between right and left atrial chambers and to identify the characteristics of atrial isomerism in the abnormal laterality syndrome. METHODS: Seventy-three embryos from SI/Col iv/iv mice (Jackson's laboratory, USA) or fetuses at the 10th-13th day were used. They were examined using stereomicroscope, scanning electronmicroscope, serial section, and thick slicing in the agar block. RESULTS: Thirty-three cases (45%) had usual arrangement but 40 cases (55%) had abnormal laterality, which included 29 cases of mirror-imaged appendages, seven with left isomerism, two with right isomerism and two with undeterminate appendages. Embryonic right atrium was initially composed of a large conspicuous appendage, which was a thin walled diverticulum and small venous component. As development progressed, venous valves and septum spurium became prominent. The embryonic left atrium was composed of the less conspicuous appendage but the major part of embryonic left atrium was the primitive atrium merging into the atrioventricular canal. The atrial chamber in the right isomerism had a bilateral big appendage and small venous component and the atrioventricular orifice was located in the center of the atrial outlet. Atrial chambers in the left isomerism had a bilateral small appendage and the central venous component was wide. CONCLUSION: The atrial morphology is the best marker in the determination of the atrial laterality. The appendages in hearts with isomerism had bilateral symmetrical arrangement of the appendages.

Developmental Morphology of the Atrial Venous Component in the iv/iv Mouse

PURPOSE: Developmental processes of atria in embryos or fetuses of the iv/iv mouse were studied to find the differences between right and left atrial chambers and to identify the characteristics of atrial isomerism in the abnormal laterality syndrome. METHODS: Seventy-three embryos from SI/Col iv/iv mice (Jackson's laboratory, USA) or fetuses at the 10th-13th day were used. They were examined using stereomicroscope, scanning electronmicroscope, serial section, and thick slicing in the agar block. RESULTS: Thirty-three cases (45%) had usual arrangement but 40 cases (55%) had abnormal laterality, which included 29 cases of mirror-imaged appendages, seven with left isomerism, two with right isomerism and two with undeterminate appendages. Embryonic right atrium was initially composed of a large conspicuous appendage, which was a thin walled diverticulum and small venous component. As development progressed, venous valves and septum spurium became prominent. The embryonic left atrium was composed of the less conspicuous appendage but the major part of embryonic left atrium was the primitive atrium merging into the atrioventricular canal. The atrial chamber in the right isomerism had a bilateral big appendage and small venous component and the atrioventricular orifice was located in the center of the atrial outlet. Atrial chambers in the left isomerism had a bilateral small appendage and the central venous component was wide. CONCLUSION: The atrial morphology is the best marker in the determination of the atrial laterality. The appendages in hearts with isomerism had bilateral symmetrical arrangement of the appendages.