Impact of evolving strategy on clinical outcomes and central pulmonary artery growth in patients with bilateral superior vena cava undergoing a bilateral bidirectional cavopulmonary shunt

Objective

We reported a high incidence of thrombosis, central pulmonary artery hypoplasia, and mortality for bilateral bidirectional cavopulmonary shunts. We hypothesized that technical modifications in the cavopulmonary anastomosis and anticoagulation would limit thrombus and central pulmonary artery hypoplasia, and thereby improve outcomes.

Methods

Sixty-one patients (median age, 8.4 months; weight, 6.6 kg) underwent bilateral bidirectional cavopulmonary shunt from 1990 to 2007. The cohort was divided into 2 groups: 1) the conventional group (1990–1999, n = 37) and 2) the V-shaped group, with a hemi-Fontan or modification in which the cavae were anastomosed to the pulmonary artery adjacent to each other so they formed the appearance of a V (1999–2007, n = 24). Central and branch pulmonary artery growth, survival, and reinterventions were determined.

Results

The pre-Fontan study showed equivalent superior venae cavae and Nakata indices. The central pulmonary artery index and central pulmonary artery/Nakata index ratio were significantly higher in the V-shaped group (P < .05). There were no differences in freedom from death or transplant (conventional 69% vs V-shaped 75% at 3 years, P = .5), and a nonsignificant trend toward improving freedom from reinterventions (63% vs 81% at 3 years, P = .15) and thrombosis (82% vs 95% at 1 year, P = .11) was observed in the V-shaped group. Multivariate analysis showed anastomotic strategy, low saturation, and thrombosis were predictors for death. Anastomotic strategy, lack of anticoagulation, thrombosis, and small superior venae cavae were predictors for reintervention (P < .05). Predictors for thrombus included small superior venae cavae, Nakata index, and low saturation (P < .03).

Conclusions

Surgical modifications for bilateral bidirectional cavopulmonary shunts were associated with the larger central pulmonary artery size. Lack of anticoagulation and anastomotic strategy affected reintervention. Anastomotic strategy and postoperative thrombus affected mortality.

CTSNet classification: 20

Abbreviations and Acronyms: BCPS, bidirectional cavopulmonary shunt, BSA, body surface area, CVP, central venous pressure, NS, not significant, PA, pulmonary artery, Sao₂, arterial saturation, SVC, superior vena cava, TAPVD, total anomalous pulmonary venous drainage