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Functional single ventricle with extracardiac total anomalous pulmonary venous connection

^a Department of Cardiovascular Surgery, Shizuoka Children's Hospital, 860 Urushiyama Aoi-ku, Shizuoka 420-8660, Japan
^b Division of Cardiac Intensive Care, Shizuoka Children's Hospital, Shizuoka 420-8660, Japan

Received 30 August 2008; received in revised form 23 February 2009; accepted 24 February 2009.

^_* Corresponding author. Tel.: +81 54 247 6251; fax: +81 54 247 6259. (Email: sakamoto{at}jun.ncvc.go.jp).

Objective: The purpose of this study is to evaluate the surgical repair of functional single ventricle and extracardiac total anomalous pulmonary venous connection (TAPVC). Methods: Between January 1998 and December 2007, 26 consecutive patients underwent surgical repair of extracardiac TAPVC. Their characteristics were as follows: median age, 34 (range 0–744) days; median weight 3.2 (range 2.0–9.6) kg; supracardiac TAPVC, 11 patients; infracardiac, 5; mixed, 10; right atrial isomerism, 24; pulmonary atresia, 16; and obstructed TAPVC, 17. Concomitant procedures included systemic-to-pulmonary shunt in 9 patients, pulmonary artery banding in 5, ventricle-to-pulmonary artery shunt in 1, Norwood procedure in 1, bidirectional Glenn in 9, and Fontan procedure in 1. Results: The overall survival after the repair of TAPVC was 58% (95% confidence interval [CI], 39–77%) and 54% (95% CI, 34–73%) at 1 and 5 years, respectively. Of the 14 survivors (supracardiac, 9; infracardiac, 4; and mixed, 1), 12 underwent Fontan completion; 1, bidirectional Glenn; and 1 is awaiting bidirectional Glenn. Anastomotic stenosis did not occur, but recurrent pulmonary venous ostial stenosis (PVS) was observed in nine patients. Freedom from recurrent PVS was 56% (95% CI, 34–78%) at both 1 and 5 years. Reoperation for recurrent PVS was performed in six patients; of these patients, two underwent Fontan completion, but three with bilateral and multiple PVS declined. By Cox multivariate regression analysis, mixed TAPVC (p = 0.001, hazard ratio, 13.4; 95% CI, 2.8–64.4) was a risk factor for mortality, and atrioventricular valve regurgitation, which required surgical intervention at the palliative stage (p = 0.024, hazard ratio, 23.4; 95% CI, 1.5–363.4) was a risk factor for recurrent PVS. Conclusions: The mid-term results of the surgical repair of functional single ventricle with supracardiac or infracardiac TAPVC are acceptable. The surgical treatment of patients with mixed TAPVC and with severe atrioventricular valve regurgitation is not promising, but can be improved.

Key Words: Functional single ventricle • Total anomalous pulmonary venous connection • Right atrial isomerism • Recurrent pulmonary venous ostial stenosis • Fontan completion