Characterizing the anatomic spectrum, surgical treatment, and long-term clinical outcomes for patients with Shone's syndrome

Surgical Considerations in Shone Complex

“…All patients who underwent univentricular palliation were excluded. More recently, Lee et al 12 reported 121 cases, from a 65-year period, with a 92% survival rate over 20 years (Figure 8). 12 In this instance, any patient with at least 2 levels of left-sided heart or great vessel obstruction was included, regardless of whether the mitral valve was involved.…”

“…Reprinted from The Journal of Thoracic and Cardiovascular Surgery, Online ahead of print, Leah J. Lee, Dominique L. Tucker, Sohini Gupta, Naseeb Shaheen, Jeevanantham Rajeswaran, Tara Karamlou, Moses Anabila, Yezan Salam, Munir Ahmad, Robert Stewart, Gosta Pettersson, Hani K. Najm, Characterizing the anatomic spectrum, surgical treatment, and long-term clinical outcomes for patients with Shone's syndrome, Copyright 2022, with permission from Elsevier. Reference: 12 .…”

“…Even the largest case series published to date are single center reports featuring an average of 2 to 4 cases per year. 6,12 Considerable variation in precise definitions applied to the term adds to the challenge.…”

“…4,6,8,14 Aortic coarctation is most often the first lesion repaired, with an average age at the time of first operation in large series between 6.5 days to 2 weeks. 6,11,12 While valvar aortic stenosis requiring operative intervention is less common in Shone complex, when present in the neonate it can present a significant therapeutic challenge. Depending on the severity of valvar stenosis and the anatomy of the left ventricular outflow tract and aortic valve, a variety of interventions ranging from transcatheter balloon valvuloplasty to pulmonary autograft implantation or prosthetic valve replacement may be undertaken at some point in the patient's course.…”

Commentary: Is there a role for hybrid in Shone's when Norwood is indicated?

“…encompasses a broader spectrum of a rare congenital heart disease in which 2 hemodynamically restrictive left heart lesions are involved in the pathophysiology, irrespective of their simultaneity. 1 As said by Lee and colleagues, 2 the heterogeneity in the definition convolutes the categorization of these patients as well as our understanding of optimal treatment and outcome. Fifteen-year mortality for patients with multiple left-sided obstructive lesions is high, at 10% to 30%.…”