Double drainage of total anomalous pulmonary venous connection revealed after surgical repair of a supracardiac total anomalous pulmonary venous connection
Abstract:This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
“…8 Drainage has also been reported to other sites like left brachiocephalic vein and right superior caval vein; portal vein and right superior caval vein and via two orifices into left superior caval vein. 4,7,9 Our patient also had a co-existent significant right superior caval vein stenosis close to its entry into the right atrium, thereby leading to obstruction to drainage at supra-cardiac level.…”
Double drainage of the confluence of all four pulmonary veins is extremely rare. We present the image findings in a child with double drainage of the pulmonary venous confluence into the coronary sinus and left superior caval vein with co-existent right superior caval venous stenosis.
“…8 Drainage has also been reported to other sites like left brachiocephalic vein and right superior caval vein; portal vein and right superior caval vein and via two orifices into left superior caval vein. 4,7,9 Our patient also had a co-existent significant right superior caval vein stenosis close to its entry into the right atrium, thereby leading to obstruction to drainage at supra-cardiac level.…”
Double drainage of the confluence of all four pulmonary veins is extremely rare. We present the image findings in a child with double drainage of the pulmonary venous confluence into the coronary sinus and left superior caval vein with co-existent right superior caval venous stenosis.
“…25 Sometimes, the vertical vein was deliberately not operated on in order to prevent a PH crisis, with embolization with a coil or plug to be performed if the right heart volume load increased. [26][27][28] Extracorporeal membrane oxygenation (ECMO) is another intervention used to temporize neonates until the diagnosis is confirmed or when medical management is unable to correct severe hypoxemia, acidosis, and hemodynamic instability; and interventions or surgical repair are arranged. 22,29,30 ECMO has allowed surgical correction even in the most severely ill patients.…”
Section: Catheter-based Interventionsmentioning
confidence: 99%
“…25 Sometimes, the vertical vein was deliberately not operated on in order to prevent a PH crisis, with embolization with a coil or plug to be performed if the right heart volume load increased. 26–28 Figure 2.Percutaneous intervention using coronary stent via a guiding catheter demonstrating successful dilation of the obstructed vertical vein in supracardiac TAPVC. (from: John Wiley and Sons, Catheterization and Cardiovascular Interventions, Stenting of the vertical vein in obstructed total anomalous pulmonary venous return as rescue procedure in a neonate, Lo-A-Njoe et al, 2006; 67(5): 668-670, https://doi.org/10.1002/ccd.20715).…”
Background Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic abnormality that accounts for about 1%–3% of congenital heart disease malformations. TAPVC is a condition in which there is no direct connection between all four pulmonary veins and the left atrium but makes abnormal connections to the right atrium or systemic venous system. TAPVC caused a high mortality rate of 80% in the first year of life, and 50% of them die within 3 months after birth without intervention. Objectives The aim of this review is to elucidate the various treatment considerations of TAPVC. Methods A literature search was conducted on PubMed, ScienceDirect and Google Scholar using various combinations of keywords related to treatment of TAPVC. The citations from all selected articles were reviewed for additional studies. Results and Conclusion TAPVC intervention, including medical and surgical, is tailored to each type of TAPVC. Catheter-based interventions are frequently used to temporize neonates and provide time to optimize patients medically prior to definite repair. Corrective surgery is required for all patients with this condition. Several latest catheter-based or surgical intervention technique modifications have also been reported.
“…(2) There is an abnormal vein adjacent to the descending aorta or ascending aorta that ows up into the LIV, SVC or AV. (3) The SVC could have a widened inner diameter and an increased ow velocity. (4) The size or ratio of the atria and ventricles are generally normal or abnormal.…”
Section: Tte In the Diagnosis Of Sapvcmentioning
confidence: 99%
“…Patients with SAPVC have complex drainage sites and diverse drainage branches. If they are not actively treated, the opportunity for surgery will be missed, and may cause serious consequences, which brings challenges to clinical diagnosis and surgical decision-making [2][3]. Multilayer spiral computed tomography (MSCT) is the gold standard for the diagnosis of SAPVC [4], but it has the disadvantages of radiation and a high price, which makes it unsuitable for repeated examinations, especially for infants and young children.…”
Objective To explore the clinical value of transthoracic echocardiography (TTE) in the differentiation of Supracardiac Anomalous Pulmonary Venous Connection (SAPVC) in children.Materials and methods A total of 118 children with concurrent TTE and CT databases of cases diagnosed with SAPVCs were included. We analyzed the consistency between the two for the ability to diagnose the classi cation of SAPVC, drainage sites, ectopic pulmonary veins and the segments of superior vena cava (SVC).
ResultsThe consistency between TTE and CT in diagnosing the existence of SAPVC and the classi cation were 88.1% (95% CI: 80.9%-93.4%) and 91.0% (95% CI: 84.1%-95.6%), respectively. The error rate of partial type diagnosed by TTE was signi cantly higher than that of total and mixed type (20.5% vs. 2.8%, P=0.003). The consistency between TTE and CT to determine drainage sites was 91.9% (95% CI: 85.2%-96.2%). TTE had a signi cantly higher error rate in determining pulmonary vein drainage to the SVC than in those draining into the left innominate vein (17.5 vs. 2.5%, P=0.007). The consistency of TTE and CT in judging the number of veins was 87.4% (95% CI: 79.7%-92.9%). The error rate in determining the presence of 2 and 5 ectopic pulmonary veins was signi cantly higher than those of 1 and 4 veins (P<0.05).
ConclusionTTE for diagnosing partial SAPVC and identifying the drainage site of SVC has a high error rate of misdiagnosis and missed diagnosis. The extra attention should be given to these factors in clinical practice to improve the accuracy of TTE in diagnosing SAPVC.
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