New Insights into Sinus Venosus Defects from Cross-Sectional Imaging

Sinus venosus defects include two varieties,superior and inferior sinus venosus defects.The superior sinus venosus defect is characterized by abnormal communication between two closely related venoatrial structures:1)the normally positioned superior vena cava-right atrium complex and 2)the right pulmonary vein-left atrium complex that is displaced leftward,forward and upward.Inferior sinus venosus defects primarily involve the inferior vena cava-right atrial junction while the right pulmonary vein-left atrial junction can also be affected.Because of the rarity and wide variation of the defects,the morphological characterization of sinus venosus defects is inconsistent among investigators and often inaccurate.Modern imaging technologies with high spatial and temporal resolutions have allowed accurate and detailed assessment of the pathological anatomy in larger numbers of cases.In this pictorial essay,we revisit the established and controversial features of the sinus venous defects using twodimensional(2D)and three-dimensional(3D)images obtained by magnetic resonance(MR)or computed tomography(CT)with brief discussion on imaging and treatment strategies.

Correlation between ductus venosus spectrum and right ventricular diastolic function in isolated single-umbilical-artery foetus and normal foetus in third trimester

BACKGROUND Single umbilical artery(SUA)is the most common umbilical cord malformation in prenatal diagnosis.The presence of an SUA can cause blood circulation disorder in the foetus and functional changes of the foetal heart,affecting foetal circulation.The right ventricular diastolic functions in foetuses with isolated SUA and in normal foetuses in the third trimester were evaluated using the spectral Doppler of blood flow in the foetal ductus venosus(DV).AIM To evaluate the right ventricular diastolic functions in foetuses with isolated SUA and in normal foetuses in the third trimester.METHODS Colour Doppler was used to measure the spectrum of foetal DV and tricuspid orifice in 34 foetuses with isolated SUA aged 28-39 wk and in age-matched healthy controls.The DV flow velocities and velocity ratios were measured.The early passive/late active(E/A)ratio at the tricuspid orifice and tissue Doppler Tei index of the foetal right ventricular in the two groups were also measured.RESULTS During the third trimester,the isolated SUA group showed a lower‘a’-wave peak velocity in the DV than the control group(P<0.05).The correlations between the velocity ratios and E/A ratio at the tricuspid orifice in the two groups were analysed,and the correlation between the ventricular late diastolic velocity/ventricular diastolic peak flow velocity and E/A ratios was the best(R^2of the isolated SUA group:0.520;R2 of the control group:0.358).The correlations between the velocity ratios and tissue Doppler Tei index of foetal right ventricular in the two groups were analysed,and the correlation between the pulsatility index for veins(PIV)and tissue Doppler Tei index ratios was the best(R2 of the isolated SUA group:0.865;R2 of the control group:0.627).CONCLUSION In the isolated SUA group,the atrial systolic peak velocity‘a’decreased,and this finding might be related to the changes in foetal cardiac functions.The ratio of ventricular late diastolic velocity to ventricular diastolic peak flow velocity was closely related to the E/A ratio at the tricuspid valve and can be used to identify changes in the right ventricular diastolic functions of isolated SUA and healthy foetuses.PIV was closely related to the tissue Doppler Tei index of the foetal right ventricular and can be used to identify the right ventricular overall functions of isolated SUA and healthy foetuses.

Superior sinus venosus atrial septal defect

Atrial septal defect (ASD)is the third commonest congenital heart disease in adults.The most common type of ASD is the ostium secundum accounting for 75%-80%of all ASDs.Less common are the ostium primum (accounting for 15%of all ASDs),the sinus venosus defect (5%-10%) and the coronary sinus defect (less than 1%).[1,2]

Partial Anomalous Pulmonary Venous Connection and the Nature of Associated Sinus Venosus Defect

Background:Partial anomalous pulmonary venous connection(PAPVC)is frequently associated with atrial septal defect(ASD),especially sinus venosus defect(SVD).Although Waggstaffe described the pathology of SVDs in 1868,the exact anatomic features and the nature of SVD remains controversial.SVDs with no posterior atrial rim were observed in recent years.However,no studies suggested that absence of the residual posterior atrial septal tissue might be the key feature of SVD.The aims of this study were to investigate if absence of posterior rim of atrial septum played a crucial role in patients with SVD.Methods:From January 2011 to December 2019,256 children with PAPVC combined ASD and 878 children with isolated ASD who underwent corrective cardiac surgery were consecutively enrolled.Comprehensive review of preoperative transthoracic echocardiography,computed-tomography images and surgical findings were performed by experienced pediatric cardiologists.The subtypes of PAPVC,locations and types of ASD,and presence of posterior atrial rim of associated ASD were investigated.Results:PAPVC was right-sided in 244 children,left-sided in 6 children,and bilateral in 6 children.In PAPVC cases,ASD without posterior atrial rim existed in 226 SVD cases.ASD without posterior atrial septum only existed in cases with one or more right pulmonary veins returning to right atrium(RA)or to RA-superior vena cava junction.In cases with isolated ASD,there were 3 SVD,and the other 875 cases were secundum ASD.Conclusions:ASD without posterior atrial rims was associated with one or more right pulmonary veins returning to RA or RA-superior venous cava(SVC)junction.For SVD,the key feature is that the defect is in the posterior of the interatrial septum with no posterior septal rim,rather than adjacent to the SVC or to the inferior vena cava.

Utilising ductus venosus Doppler waveform and four-chamber view to screen for foetal cardiac malformation in early second trimester of pregnancy

Background Foetal echocardiography has become a diagnostic method to detect foetal congenital heart disease with high probability. However, it is not only time consuming and but also difficult to visualize outflow tract of foetus early in the second trimester of pregnancy, even for an experienced obstetric uhrasonographer. Recently, many methods for screening foetal cardiac anomalies were explored, but much more work is needed to develop an effective and suitable screening method. The aim of this study was to investigate the clinical significance of utilising the ductus venosus （DV） Doppler examination and the four-chamber view of heart to screen for foetal cardiac malformation in early second trimester of pregnancy. Methods Heart and DV of 401 consecutive foetuses in early second trimester （12^＋1- 17^ ＋6 weeks） in high risk pregnancies were examined with Acuson 128 xp/10 or Sequoia 512 ultrasound diagnostic systems. Absent or reversed flow during atrial contraction （A-wave） in the DV was defined as sufficiently abnormal to screen for foetal cardiac malformations. The foetal echocardiographic diagnosis was confirmed by postnatal echocardiography （or postmortem）. The sensitivities of screening tests were compared among the three methods： DV Doppler examination, four-chamber view alone, and the combination of both techniques.Results Satisfactory examinations were obtained in 383/401 foetuses （95%）. Thirty foetuses with cardiac abnormalities were confirmed by neonatal echocardiography （ or postmortem ）. The sensitivity of DV Doppler examination or four-chamber view alone is 63 % （19/30） and 60 % （ 18/30）, respectively. The sensitivity of combining information, DV Doppler flow waveform and four-chamber view, to screen for foetal cardiac malformation is 83% （25/30） and significantly better than that of either DV Doppler flow waveform or four chamber view alone （ P 〈 0. 05 ）. Conclusion Doppler flow waveform of DV can be used to screen for foetal cardiac malformation early in the second trimester. Combining information from Doppler flow waveform of DV and four-chamber view will improve the overall sensitivity of the screening.

Diagnostic value of real-time three-dimensional transesophageal echocardiography for preoperative assessment of sinus venosus atrial septal defect

Background Sinus venosus atrial septal defect（SVASD） is a rare atrial septal defect. The right-sided pulmonary veins may drain into the right atrium directly or via vena cava. Two-dimensional transthoracic echocardiography（2D-TTE） is certainly the initial choice of evaluation of SVASD but is unable to show its spatial relation to the surrounding structures and the configuration of the veins accurately. The purpose of this study was to evaluate the diagnostic accuracy of real-time three-dimensional transesophageal echocardiography（RT3D-TEE） for detecting SVASD and partial anomalous pulmonary venous connection（PAPVC）. Methods2D-TTE and RT3D-TEE were performed in 25 patients with the SVASD before surgical repair. Records of these patients were compared with surgical assessments. Results Twenty-three of the 25 patients with SVASD were detected by 2D-TTE. All the 23 patents were associated with PAPVC. Two of the 25 patients with SVASD were misdiagnosed as the secundum atrial septal defect by 2D-TTE. SVASD was demonstrated in all the 25 cases by RT3D-TEE, and 25 patents were associated with PAPVC. Conclusions RT3D-TEE is highly accurate, scientific method for displaying the location, size, shape of the defect and its spatial relation to the surrounding structures, and provides additional value to the surgeon and physicians for better understanding of spatial intracardiac morphology and making more accurate diagnosis and treatment.

Congenital extrahepatic portosystemic shunt complicated by the development of hepatocellular carcinoma

Congenital extrahepatic portosystemic shunt,also known as Abernethy malformation, is a rare congenital malformation. It causes shunting of blood through a communication between the portal and systemic veins such as a patent ductus venous. We report 3 cases of Abernethy malformation complicated by the development of hepatocellular carcinoma. Additionally, we comprehensively reviewed all previously reported cases and highlighted common features that may help in early diagnosis and appropriate management.Patients with Abernethy malformation may have an increased propensity to develop hepatocellular carcinoma. All 5 previously reported cases, plus the three of our patients, have a type 1（complete） shunt suggesting a role for absent portal blood flow in the pathogenesis of hepatocellular carcinoma. Congenital extrahepatic portosystemic shunt should be sought for in cases with raised serum ammonia, hepatic encephalopathy or hepatocellular carcinoma in the absence of cirrhosis.

Adult Congenital Heart Disease in the Veteran Population:A Case-Based Report

Simple forms of congenital heart disease can allow patients to go undiagnosed until they reach adulthood.Furthermore,improvements in care of patients with complex congenital heart disease are now allowing most patients to reach adulthood.As some patients with adult congenital heart disease can remain asymptomatic until later in life,it is possible for them to serve in the military and eventually fall under the care of Veterans Administration(VA)providers.Therefore it is important for providers,especially cardiologists at VA centers,to have fundamental understanding of the management of adult congenital heart disease.This article provides multiple cases of adult congenital heart disease experienced at a single VA medical center and reviews the anatomy,physiology,and surgical management of each condition.