Elsevier

The Annals of Thoracic Surgery

Volume 84, Issue 6, December 2007, Pages 2020-2026
The Annals of Thoracic Surgery

Original article
Cardiovascular
Outcomes After Surgical Treatment of Children With Partial Anomalous Pulmonary Venous Connection

Presented at the Forty-third Annual Meeting of The Society of Thoracic Surgeons, San Diego, CA, Jan 29–31, 2007.
https://doi.org/10.1016/j.athoracsur.2007.05.046Get rights and content

Background

We explore early results and time-related morbidity after surgical repair of partial anomalous pulmonary venous connection (PAPVC) at our institution.

Methods

Between 1982 and 2006, 306 consecutive patients underwent surgery for PAPVC; of these, 236 (77%) were children with a median age of 5.3 years (range, 0.47 to 18 years). Clinical and echocardiographic follow-up was obtained.

Results

PAPVC was right-sided in 214 patients (90%), left-sided in 17 (7%), and bilateral in 5 (2%). Anomalous veins were partial in 186 patients (79%) and involved the entire lung in 50 (21%). The most common type was right PAPVC into the superior vena cava in 175 (74%), with 87% associated with sinus-venosus atrial septal defect; followed by right PAPVC into the right atrium in 29 (12%), left PAPVC into the innominate vein in 22 (9%), and scimitar syndrome in 15 (6%). Repair strategy included intracardiac baffle in 203, pulmonary vein implantation in 22, and SVC division with reimplantation on the right atrial appendage in 14. There was no early or late mortality. Freedom from reoperation, vena cava obstruction, pulmonary vein obstruction, and pacemaker implantation at 15 years was 97%, 97.8%, 86%, and 99.1%, respectively. Pulmonary vein obstruction was significantly more common in patients with scimitar compared with others (22.4% versus 98.3%, p < 0.0001). Postoperative quantitative lung perfusion scans in 13 of 15 scimitar patients showed decreased right lung perfusion (mean, 22.5%).

Conclusions

Surgical treatment of PAPVC is associated with excellent outcomes and low time-related morbidity. However, management of children with scimitar syndrome is complicated by an exceptionally high incidence of postoperative pulmonary venous obstruction and abnormally diminished perfusion of the right lung.

Section snippets

Inclusion Criteria

From July 1982 to May 2006, 306 consecutive patients were operated on for PAPVC at the Toronto General Hospital and the Hospital for Sick Children in Toronto; of these, 236 (77%) were children aged younger than 18 years old. The patients were identified using the surgical database. Clinical, operative, and outcome data were abstracted from the medical records. Approval of this study was obtained from the Research Ethics Board at our institution and individual consent was waived.

Partial Anomalous Pulmonary Venous Connection Characteristics

PAPVC is the

Entire Patient Cohort

Operations for PAPVC were done in 236 children: 120 boys (51%) and 116 girls (49%). Their median age at operation was 5.3 years (range, 0.47 to 18 years), and their median weight was 17.5 kg (range, 6.3 to 60.8 kg).

PAPVC were right-sided in 214 patients (90%), left-sided in 17 (7%), and bilateral in 5 (2%). Anomalous pulmonary veins were partial in 186 patients (79%) and involved the entire lung in 50 (21%).

The most common anomaly type was right PAPVC into SVC in 175 (74%); of those, 153 (87%)

Comment

Surgical principles of PAPVC repair involve separation of the pulmonary and systemic venous returns. A special consideration during surgical repair is the intimate relationship between the orifices of the anomalous pulmonary veins and the SVC or the IVC. Therapeutic options for repair of PAPVC vary with the type of anomaly and include creation of an intracardiac baffle to redirect pulmonary venous return to the left atrium or direct connection of the anomalous pulmonary vein to the left atrium [

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