case_images: PMC10073726_01
case_images: Chest radiography (A) and cardiac computered tomograhy angiography (B) preoperatively indicated that the lungs were clearly textured, the heart shadow was significantly enlarged (A); the left and right pulmonary veins were not connected to the left atrium but merged into a vertical vein and drained upward into the left brachiocephalic vein, innominate vein and then the superior vena cava. (B) LA, left atrium; PA, pulmonary artery; PV, pumonary vein; RA, right atrium; RV, right ventricle; SVC, superior vena cava; VV, vertical vein.
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case_images: PMC10073726_01_fcvm-10-1121037-g001.jpg
case_images: F1
case_images: Furthermore, the ECG indicated a significant atrial flutter and a right bundle branch block (Supplementary Figure S1A). Preoperative chest radiography indicated that the lungs were textured with a widened mediastinum and a significantly enlarged cardiothoracic ratio of 0.91 (Figure 1A). The three-dimensional reconstruction figure showed that the entire left and right pulmonary veins were not directly drained into the LA but merged into a vertical vein (with a diameter of 44 mm) and flowed upward into the left brachiocephalic vein, IV and then into the SVC (Figure 1B, Supplementary Video S1). The ECG at discharge indicated a significant atrial flutter and a right bundle branch block same as pre-operatively (Supplementary Figure S1B).
case_images: Transthoracic echocardiography preoperatively revealed that a right to left atrial septal shunt was detected with the diameter of around 40 mm (A,B); the ratio of ascending aorta to the pulmonary trunk was 24:32 (C); the left and right pulmonary veins returned to a vertical vein then innominate vein and the superior vena cava. (D) AAo, ascending aorta; ASD, atrial septal defect; LA, left atrium; LV, left ventricle; PV, pumonary vein; RA, right atrium; RV, right ventricle; RVOT, right ventriclar outflowtract; SVC, superior vena cava; VV, vertical vein.
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case_images: PMC10073726_01_fcvm-10-1121037-g002.jpg
case_images: F2
case_images: Transthoracic echocardiography revealed an atrial septal defect at the lower part of the interatrial septum with right to left shunt and ~40 mm in diameter (Figures 2A,B). The diameter of ascending aorta and the pulmonary trunk were 24 mm and 32 mm, respectively (Figure 2C). The entire four pulmonary veins returned to a vertical vein (around 23 mm), the IV, and the SVC (Figure 2D).
case_images: Intraoperative view of repair procedure: during the operation, the right atrium, right ventricle, pulmonary artery and superior vena cava were found significantly enlarged (A); A double-chamber incision was made and the posterior wall of the left atrium was opened (B, arrow); the common pulmonary veins were anastomosed with the posterior wall of the left atrium. A proper size of the bovine pericardium patch (C, arrow) was used to close the ASD and enlarged the left atrium. A proper size of autologous pericardium was used to close the right atrial incision (D, arrow). AAo, ascending aorta; PA, pumonary artery; RA, right atrium; SVC, superior vena cava.
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case_images: PMC10073726_01_fcvm-10-1121037-g003.jpg
case_images: F3
case_images: TAPVC surgical repair was scheduled and performed (Figure 3). The diameter ratio of the ascending aorta to the pulmonary trunk was 1 : 2.5 (Figure 3A). A double-chamber incision was made including the incision from the LA posterior wall (Figure 3B, arrow). ASD and enlarge the LA (Figure 3C). A 28 mm band (SOVERING TRICUSPID BANDTM, Sorin Group Italia Srl, Italy) was selected for the tricuspid repair. The autologous pericardium was adequately sized to close the right atrial incision (Figure 3D).
case_images: Postoperative transthoracic echocardiography (A,B) and cardiac CTA with three dimensional reconstruction figure (C,D) showing that the deformity correction was satisfactory without residual shunt (A) and pulmonary veinous obstruction (B) but with left and right pulmonary veins connected to the left atrium directly (C,D). LA, left atrium; LAA, left atrium; LPV, left pulmonary vein; LV, left atrial appendage; PA, pumonary artery; PV, pumonary vein; RA, right atrium; RPV, right pulmonary vein; RV, right ventricle.
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case_images: PMC10073726_01_fcvm-10-1121037-g004.jpg
case_images: F4
case_images: Postoperative transthoracic echocardiography (Figures 4A,B), cardiac CTA, and a three-dimensional reconstructed figure (Figures 4C,D) showed that the surgical correction was satisfactory with entire pulmonary veins directly connected to the LA.