Outcomes of biventricular repair for shone's complex

J Card Surg. 2021 Jan;36(1):12-20. doi: 10.1111/jocs.15090. Epub 2020 Oct 8.


Background: Shone's complex is a rare lesion affecting the mitral valve (MV) and left ventricular outflow tract (LVOT). The objective of this study is to report the outcomes after Shone's complex repair, the growth of mitral and aortic valve and LVOT, and long-term survival.

Methods: This retrospective study included all patients diagnosed with Shone's complex, who underwent biventricular repair. Data including patients' characteristics, type of the MV lesion and the associated lesions were collected. Patients were followed up regularly with echocardiography, and the changes in mitral and aortic valve z-score and LVOT z-score were recorded.

Results: Thirty-seven patients were included in the study, the median age was 3.4 months, and 11 patients (30.6%) had pulmonary hypertension. The main procedure performed during the first surgical intervention was coarctation repair in 26 patients (70%). Twelve patients had MV repair, and five had MV replacement. Operative mortality occurred in 1 patient (2.7%), median follow up was 52 (25-75th percentile: 22-84) months. Survival at 1, 5, and 10 years was 94.4%, 90%, and 76.9%, respectively. Reoperation was required in 13 patients, mainly for LVOT repair (n = 8). Reoperation was significantly associated with associated aortic valve lesion (p = .044). The growth of the MV z-score was 0.35 per year; p < .001, aortic valve z-score 0.086 per year; p = 0.422, and the LVOT z-score was 0.53 per year; p = .01.

Conclusion: Biventricular repair of Shone's complex has good outcomes. Reoperation is frequently encountered, especially with low aortic valve z-score. The MV and LVOT have significant growth following Shone's complex repair.

Keywords: biventricular repair; coarctation of the aorta; congenital aortic stenosis; left ventricular outflow tract obstruction; parachute mitral valve; shone's complex.

MeSH terms

  • Aortic Valve / diagnostic imaging
  • Aortic Valve / surgery
  • Follow-Up Studies
  • Heart Defects, Congenital* / surgery
  • Humans
  • Infant
  • Mitral Valve / diagnostic imaging
  • Mitral Valve / surgery
  • Retrospective Studies