Real-time three-dimensional echocardiography for evaluation on biventricular volume and function for infants with total anomalous pulmonary venous drainage / 中国医学影像技术

Objective: To explore the feasibility of real-time three-dimensional echocardiography (RT-3DE) for evaluation on biventricular volume and function for children with total anomalous pulmonary venous drainage (TAPVD). Methods: Totally 16 children with simple TAPVD combined with atrial septal defect or patent foramen ovale (TAPVD group) and 19 normal infants( as control group) were recruited. The following indexes, including left ventricular anteroposterior dimension (LVD), right ventricular transverse dimension (RVD), left ventricular ejection fraction by M-mode (LVEF-M), tricuspid annular plane systolic excursion (TAPSE) and peak velocity of tricuspid annulus (TAV) were measured with two-dimensional echocardiography. Then left/right ventricular ejection fraction (LVEF-3DE, RVEF-3DE) obtained by RT-3DE and left/right ventricular end diastolic volume corrected by body surface area (BSA) (LVEDV/BSA, RVEDV/BSA) were measured. The above parameters were compared between 2 groups. Results: RVD, LVEF-M and RVEDV/BSA in TAPVD group were significantly higher than those in control group, whereas LVD, TAPSE, TAV, RVEF-3DE and LVEDV/BSA were lower than in control group (all P0.05). Conclusion: RT-3DE can be used to evaluate biventricular volume and function in children with TAPVD.

The limitation of transthoracic echocardiography in diagnosing partial anomalous pulmonary venous drainage / 中华儿科杂志

Objective@#To improve the diagnostic accuracy of transthoracic echocardiography (TTE) by analyzing its limitations in diagnosing partial anomalous pulmonary venous drainage (PAPVD).@*Method@#This was a retrospective analysis of PAPVD patients seen at the Children′s Hospital of Fudan University from October 1 2006 to October 1 2016. The echocardiographic data were compared to findings on multi-slice spiral CT (MSCT), cardiac catheterization or surgery. The echocardiography machines used were Philip IE33, GE Vivid 7 and Vivid i with frequency ranging from 5.0 MHz to 7.5 MHz. The cardiac structure was analyzed according to Van Praagh segments.@*Result@#A total of 43 cases of PAPVD were enrolled, male∶ female ratio 20∶23 with average age (27.9±21.4) months. Among them, 3 cases were simple PAPVD and 40 cases had other associated congenital heart diseases. TTE was successful in diagnosing 29 cases (67%) while 14 cases were missed. The diagnostic rate for right pulmonary vein drainage into superior vena cava, right atrium, inferior vena cava were 5/10, 17/20, and 3/5 respectively while left pulmonary vein drainage into left innominate vein was only 1/4. Added TTE images to re-exam the 9 of the 14 missed cases, 5 cases of abnormal drainage from right superior pulmonary vein were diagnosed, while 4 cases of drainage from right lower or left pulmonary vein were only picked up by indirect signs.@*Conclusion@#The distance of the pulmonary veins from the routine ultrasound view and the possibility of branch number variation may limit the accuracy of TTE in diagnosing PAPVD, especially for drainage from right lower and left pulmonary vein. But TTE is still the preferred diagnostic method. The diagnostic rate could be increased by paying special attention to non-routine views including the suprasternal fossa, the right parasternal and subcostal area.

Scimitar syndrome: a rare congenital venolobar anamoly.

Scimitar syndrome is a rare congenital anomaly presenting with partial anomalous pulmonary venous drainage from right lung to inferior vena cava. This is the rarest anomaly seen in 1 to 3 in 100000 births. Embryologically it is thought to be a primary developmental anomaly of lung with secondary anomalous venous drainage. This is seen commonly in right lung and is common in females. Most of the cases are asymptomatic or minimally symptomatised. If symptoms are present patient usually presents with pulmonary hypertension and symptoms of left to right shunt. Clinically this syndrome is diagnosed by chest x- ray by the presence of “Scimitar sign”. Surgical treatment effective in this syndrome.

Síndrome da cimitarra: A propósito de três casos singulares / Scimitar syndrome: the purpose of three individual cases

Os autores relatam três casos de síndrome da cimitarra (SC), que em sua forma habitual cursa com drenagem venosa anômala no pulmão direito, de aspecto radiográfico característico, hipoplasia no referido pulmão e dextroposição cardíaca. Enfatizam sobre a radiografia simples do tórax, como principal exame na investigação diagnóstica, e o valor do estudo angiográfico, na elucidação de anomalias vasculares quando houver indicação cirúrgica. Outros exames complementares, como a tomografia computadorizada, são avaliados para a perfeita caracterização dessa síndrome e de suas variantes...

The authors write about three cases of scimitar syndrome which, commonly, releases as an atypical venous drainage of the right lung, with characteristic image, as well as hypoplasia of the same lung and dextropositioned heart. They emphasize the thoracic radiographic image as the most important complementary method of diagnosis and the importance of the angiographic study for the diagnose of vascular abnormalities with surgical indication. Other exams such as computerized tomography are of value in characterization of syndrome and its variants...

Síndrome de cimitarra asociado a atresia pulmonar con comunicación interventricular: Primer caso reportado / Scimitar syndrome associated to pulmonary atresia with interventricular communication: First reported case

Se presenta el caso de un masculino de 15 meses de edad con síndrome de cimitarra y atresia pulmonar con comunicación interventricular. El diagnóstico se hizo mediante cateterismo cardíaco y angiocardiografía y confirmado por el estudio de autopsia. Se hacen las consideraciones clínicas y quirúrgicas de esta excepcional asociación que a la fecha es, hasta donde sabemos la primera reportada en la literatura.

We present the case of a 15 months-old male with Scimitar Syndrome associated with ventricular septal defect and pulmonary atresia. The diagnosis was made by cardiac catheterization and angiography and was confirmed by the necropsy. Clinical and surgical considerations of this exceptional association were made. To the best of our knowledge this is the first case reported in the relevant literature.