Outcomes of biventricular repair for shone's complex

Abstract

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.

Mitral valve replacement in infants and younger children

“…The most common cause of MVR was congenital mitral valve disease, followed by Shone's complex. The etiological diagnosis may affect the outcomes after MVR 18 ; however, we did not observe any effect in our study.…”

Evaluation of midterm outcomes after Shone's complex surgery: Analysis of reoperation and mortality risk factors

“…Elmahrouk et al reported that preoperative AV size was a risk factor for reoperation, but they did not show a correlation between LVOT stenosis and reoperation. 5 In the presented study, there was no correlation between LVOT stenosis and reoperation. In this series, six of nine reoperation patients underwent septal myectomy, modified Konno or Ross-Konno procedure.…”

“…series, 17 out of 37 patients underwent MV surgery and concomitant LVOT surgery. 5 Perrier et al 2 reported a rate of 55% for their patients with a history of LVOT intervention.…”

Dental and Periodontal Care at the Bedside Using a Portable Dental Unit in Hospitalized Special Needs Patients: The Experience of an Italian Pediatric Hospital

“…Shone complex, or Shone syndrome, is characterized by the simultaneous presence of supravalvular mitral membrane, parachute mitral valve, muscular or membranous subvalvular aortic stenosis, and coarctation of aorta [14]. This peculiar syndrome leads to a progressive lack of hemodynamic stability; in fact, a simple disequilibrium of the pressure gradient, as commonly observable in a normal subject from clinostatism to orthostatism, could represent a serious condition capable of inducing a systemic loss of perfusion.…”