Shortness of Breath

Patient developed intermittent atrial fibrillation and other dysrhythmias and was scheduled for pulmonary vein mapping CTA for planned pulmonary vein ablation therapy.

Figure 1. Coronal CT reconstruction and 3D rendered view demonstrates an anomalous right superior pulmonary vein (yellow arrow) draining into the SVC (black arrow).

Figure 2. Coronal CT reconstruction and 3D rendered view demonstrates an anomalous right superior pulmonary vein (yellow arrow) draining into the SVC (black arrow).

Figure 3. Axial CT image demonstrating enlarged anomalous right superior pulmonary vein (long arrow) inserting into SVC (short arrow).

Figures 4A and 4B. Four-chambered views of the heart demonstrating the sinus venosus ASD (SVASD) (yellow arrows).

Figure 5. The pulmonary artery is enlarged secondary to a left to right shunt created by a sinus venosus type atrial septal defect (SVASD) and partial anomalous right superior pulmonary vein draining into SVC.

Figure 6. The right side of the heart is enlarged from a left to right shunt, resulting from the sinus venosus atrial septal defect (SVASD) and partial anomalous right superior pulmonary vein.

Diagnosis: Sinus venosus type atrial septal defect (SVASD) associated with partial anomalous pulmonary venous return (PAPVR)

If symptoms should occur, they may be manifested by dyspnea, easy fatigability, atrial flutter or fibrillation, right heart failure, or pulmonary hypertension.

Most children with sinus venosus atrial septal defects are asymptomatic but may develop symptoms as they age.

There are two types of sinus venosus atrial septal defects (SVASD) with the more common sinus venosus atrial septal defect (SVASD).

Asymptomatic children generally undergo repair when aged 3-5. Sinus venosus defects do not close spontaneously.

Transcatheter occlusion devices are currently used for closing the more common type of atrial septal defect (secundum atrial septal defects), however, such devices are not presently used for the closure of sinus venosus atrial septal defects because of the lack of surrounding tissue adequate to seat the occlusion device.

Sinus venosus atrial septal defect is an uncommon type of atrial septal defect and when found is almost always associated with anomalous pulmonary venous return.