Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study.
BACKGROUND
late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO.


RESULTS
we conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence.


CONCLUSIONS
preoperative clinical and morphological features are important risk factors for postoperative PVO and survival.
Royal Brompton Hospital, 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
D013504:Cardiovascular Surgical Procedures; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011183:Postoperative Complications / Q000453:epidemiology*; D011667:Pulmonary Veins / Q000002:abnormalities; D011668:Pulmonary Veno-Occlusive Disease / Q000453:epidemiology*; D012189:Retrospective Studies; D012307:Risk Factors; D012587:Scimitar Syndrome / Q000601:surgery; D016896:Treatment Outcome