Partial Anomalous Pulmonary Venous Connection: Forty-Six Years of Follow-Up

Partial Anomalous Pulmonary Venous Connection: Forty-Six Years of Follow-Up.

Nielsen AKM, Hjortdal VE.

World J Pediatr Congenit Heart Surg. 2021 Jan;12(1):70-75. doi: 10.1177/2150135120960482.

PMID: 33407025


Take Home Points:

  • In PAPVC, early surgical mortality is low.
  • Late onset of brady- and tachyarrhythmias are common with half of these patients requiring permanent pacing.
  • Event-free survival at 12 years of follow-up was 81%


Commentary from Dr. Blanche Cupido (Cape Town, South Africa), chief section editor of ACHD Journal Watch: Partial anomalous pulmonary venous connections (PAPVC) is a rare congenital anomaly most frequently involving the right upper pulmonary vein. Surgical correction results in suture lines which are substrates for arrythmias. The sinus node is also located close to the usual suture lines. Surgical techniques have over the last few decades evolved to try and minimize the arrhythmic complications. Long term outcome data pertaining to PAPVC correction is limited. In this retrospective Danish cohort study, the long-term outcomes after various surgical technique corrections of PAPVC are assessed in terms of sinus node dysfunction, arrythmias, PV stenosis, SVC graft dysfunction and mortality.


Patients operated for PAPVC between 1970 and 2010 were identified from the Danish surgical databases. After excluding those with scimitar syndrome and concomitant VSD, CTEPH and atrial isomerism, 83 patients were included in the study. Follow up was from date of surgery to May 2018 –the median duration of follow-up was 14.3 years (3-46 years). A late outcome event was defined as >30 days post-surgery.


The most frequently observed anomaly was of the right upper pulmonary vein, followed by scimitar syndrome then left pulmonary vein. Right sided veins were baffled through the RA via an ASD to the LA, the left sided vein anomalies were connected directly to the LA.


Of the 83 patients, 51% were women (n=42). The mean age of surgery was 25 ±24 years (wide range – 4 months to 77 years) with just over 50% being under the age of 18 at the time of surgery.


The distribution of surgical procedures is seen in table 1:



Single patch technique was performed in 36, two patch in 34, direct anastomosis in 10 and a conduit in 1. Every decade the number of surgeries increased with 60% of all surgeries done from 2000 to 2010. Five patients had right sided cryoablation as part of a study to prevent atrial arrythmias.


In the immediate post-operative period, 2 patients developed strokes (both over age 45). Eight patients (10%) died during follow-up at a mean age of 59 years. Only half of the deaths were cardiac in origin. Stenosis of the SVC (n=3) and pulmonary veins (n=2) was seen in a total of 5 patients (6%).


Only 47 patients had a pre-operative ECG – all had evidence of RBBB, RVH or right axis deviation. In 11 patients (13%), AF or atrial flutter was seen pre-operatively.


Late sinus node dysfunction was seen in 9 patients (11%) – 4 had bradycardia and 5 had sinus arrest ± AF/Aflutter. Pacemakers were implanted in 7 patients (8%) for sinus node dysfunction(n=5), cardiac resynchronisation therapy (n=1) and third degree AV block (n=1).


Early post-operative new onset AF/atrial flutter was seen in 8 patients (10%) – in 7 of them, this settled before discharge and they left hospital in sinus rhythm. The prevalence of late atrial fibrillation / flutter was 17%


In 81% (n=67), no arrythmias or need for pacing was noted. Figure 2 shows the even-free survival.



There was no statistically significant difference pertaining to the different surgical techniques and outcomes.



Late sinus node dysfunction and the need for permanent pacing are significant complications post surgery for PAPVC. At an average of 12 years post surgery, 11% had sinus node dysfunction and more than 50% needed pacing. There was no statistically significant difference between surgical technique and arrythmia outcomes. Late stenosis of PV or SVC was seen in 6%. Definitive conclusions are limited due to the retrospective nature and the surgical technique diversity in this study.