Sinus Venosus Defect and Partial Anomalous Pulmonary Venous Connection: Effect of Surgical Strategies on Sinus Node Function and Potential Obstruction of the Superior Vena Cava

Objectives: Surgical repair of sinus venosus defect (SVD) with partial anomalous pulmonary venous connection (PAPVC) to the superior vena cava (SVC) or the right atrium (RA) should avoid long term complications like permanent sinus node dysfunction (SND) or obstruction of superior vena cava (SVC). We retrospectively reviewed our single center experience on patients who underwent cardio-surgical repair using single-patch, double-patch and modified Warden-technique.

Methods: Between 1964 and 2014, totally 77 patients (mean age 14.6 ± 14.2 years) underwent surgical correction by use of golden standard technique revealed at that time. 34 patients received a single-patch (SPR), 24 patients a double-patch repair (DPR), and in 19 patients we performed a modified Warden repair technique (WR) for intracardiac, but extraanatomic repair leading to a significantly higher complexicity score. Our modification contained (1) biopericardal patch augmentation of SVC-RA anastomosis to prevent RA from chronic distraction and (2) minimalized RA atriotomy to prevent from scar formation leading both to negative impact on sinus node function.

Results: Average follow-up was 20.0 ± 12.2 years, follow-up data with holter electrocardiogram (ECG) and echocardiogram was available in 85% of all patients. Preoperatively, all patients were in sinus rhythm. There were no early deaths, but two non-surgery related cardiac deaths at a follow up of 24 and 35 years, both of them in SPR group. We did not find any pulmonary vein stenosis, which needed further treatment, but chronic inflow obstructions in SPR group, and one SVC stenosis which had to be augmented by balloon dilatation and stenting. Permanent SND was found in 25% of patients in SPG and in 28% of patients in DPG (both increasing over follow-up time). In contrast, only 5% of WR patients revealed SND (p < 0.05*), decreasing over follow-up time after surgery. Hence, onset of nodal rhythm and complex SND could be found with a high incidence particularly in those patients with extended incisions across the cavoatrial junction.

Conclusions: The Warden repair should be used for patients with SVD with PAPVR to the SVC and RA as this technical approach showed the best results preserving normal sinus node function for the patient. Rarely, when PAPVR is attached to the central part of RA, the single-patch-repair is still preferred as method of choice. Because of the high incidence for SND, double-patch repair should be abandoned.