Surgical Outcomes of Cor Triatriatum Sinister: A Single-Center Experience

Abstract

Background: We investigated surgical outcomes after the surgical repair of cor triatriatum sinister (CTS).

Methods: Thirty-two consecutive patients who underwent surgical repair of CTS from 1993 through 2020 were included in this study. The morphological characteristics, clinical features, and surgical outcomes were described and analyzed.

Results: The median age and body weight at operation were 9 months (interquartile range [IQR], 3-238 months) and 7.5 kg (IQR, 5.8-49.6 kg), respectively. There were 16 males (50%). According to the modified Lucas classification, type IA (classical CTS) was most common (n=20, 62.5%). Atrial septal defect was associated in 22 patients (68.8%) and anomalous pulmonary venous return in 8 patients (25%). Pulmonary hypertension was preoperatively suspected with a high probability in 18 patients (56.3%). There was 1 early death (3.1%) after emergent membrane excision and hybrid palliation in a high-risk hypoplastic left heart syndrome patient. There were no late deaths. The overall survival rate was 96.9% at 15 years post-repair. No early survivors required reoperation during follow-up. Most survivors (31 of 32 patients, 96.9%) were in New York Heart Association functional class I at a median follow-up of 74 months (IQR, 39-195 months). At the latest echocardiography performed at a median of 42 months (IQR, 6-112 months) after repair, no residual lesion was observed except in 1 patient who had moderate pulmonary hypertension (mean pulmonary arterial pressure of 36 mm Hg).

Conclusion: Surgical repair of cor triatriatum could be performed safely and effectively with an extremely low risk of recurrence.

Keywords: Cor triatriatum; Outcomes; Surgery.

Fig. 1

Schematic diagrams of cardiac anatomy in 2 patients who could not be classified by the modified Lucas classification. Red arrows indicate flow direction. (A) Diagram of patient with cor triatriatum, secundum atrial septal defect (ASD), sinus venosus ASD and partial anomalous pulmonary venous return (PAPVR) (right lower pulmonary vein [RUPV] to right atrium [RA]). The accessory left atrial chamber (AC) that received part of the pulmonary veins was connected to both the left and right atria through the ASD and membranous opening. The true chamber was connected to the right atrium through another ASD. (B) Diagram of patient with cor triatriatum, nearly common atrium, and PAPVR (RUPV and right middle pulmonary vein [RMPV] to RA). The accessory atrial chamber that received part of the pulmonary veins was connected to both left and right atrium through ASD and membranous opening. LLPV, left lower pulmonary vein; LUPV, left upper pulmonary vein; RUPV, right upper pulmonary vein; TC, true left atrial chamber.

Fig. 2

Overall survival after repair of cor triatriatum sinister. One early death occurred, and there were no late deaths during a median of 74 months of follow-up. The 15-year overall survival rate was 96.9%.