Total anomalous pulmonary venous connection: outcome of postoperative pulmonary venous obstruction.
OBJECTIVE
Pulmonary venous obstruction (PVO) is an important cause of late mortality in total anomalous pulmonary venous connection (TAPVC). We aimed to describe current practices for the management of postoperative PVO and the efficacy of the different interventional procedures.


METHODS
We conducted a retrospective international collaborative population-based study involving 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. Patients with TAPVC born between January 1, 1998, and December 31, 2004, were identified. Patients with functionally univentricular circulation or atrial isomerism were excluded. All available data and images were reviewed.


RESULTS
Of 406 patients undergoing repair of TAPVC, 71 (17.5%) had postoperative PVO. The diagnosis was made within 6 months of surgery in 59 (83%) of the 71 patients. In 12, serial imaging documented change in appearance of the pulmonary veins. Good-sized pulmonary veins can progress to diffusely small veins and rarely atresia. Patients presenting after 6 months had less severe disease; all are alive at most recent follow-up. Fifty-six (13.8%) of 406 patients underwent intervention for postoperative PVO: 44 had surgical treatment and 12 had an initial catheter intervention. One half underwent 1 or more reinterventions. Three-year survival for patients with postoperative PVO was 58.7% (95% confidence intervals, 46.2%-69.2%) with a trend that those having a surgical strategy did better (P = .083). Risk factors for death included earlier presentation after TAPVC repair, diffusely small pulmonary veins at presentation of postoperative PVO, and an increased number of lung segments affected by obstruction.


CONCLUSIONS
Postoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur.
Royal Brompton Hospital & National Heart and Lung Institute, Imperial College, London, United Kingdom.
Anna N
AN
Seale
Hideki
H
Uemura
Steven A
SA
Webber
John
J
Partridge
Michael
M
Roughton
Siew Y
SY
Ho
Karen P
KP
McCarthy
Sheila
S
Jones
Lynda
L
Shaughnessy
Jan
J
Sunnegardh
Katarina
K
Hanseus
Hakan
H
Berggren
Sune
S
Johansson
Michael L
ML
Rigby
Barry R
BR
Keeton
Piers E F
PE
Daubeney
D006348:Cardiac Surgical Procedures / Q000009:adverse effects* / Q000401:mortality; D018450:Disease Progression; D057510:Endovascular Procedures / Q000009:adverse effects / Q000401:mortality; D005060:Europe / Q000453:epidemiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D015995:Prevalence; D016016:Proportional Hazards Models; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D012587:Scimitar Syndrome / Q000401:mortality / Q000601:surgery*; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome