Shone complex

Shone complex

Shone complex is a series of obstructive birth defects of the left side of the heart. There is ring above the mitral valve, causing obstruction, parachute like mitral valve with obstruction, narrowing below the aortic valve and coarctation of aorta. It was described by Shone and colleagues in 1963 [1]. It was a report of 8 cases of which only 2 had all the four anomalies. Remaining 6 cases were forme fruste with only 2 or 3 of the described anomalies. There could be associated defects like ventricular septal defect, which alter the clinical picture.

Stented bovine jugular vein graft (Melody) has been implanted in Shone complex patient requiring mitral valve replacement. Patient had undergone Ross-Konno operation initially and needed mitral valve replacement at 6.6 months for severe mitral regurgitation [2]. The patient underwent successful heart transplantation at 8.5 months for left ventricular diastolic dysfunction.

Related article: Shone complex – surgical results

References

1. Shone JD, Sellers RD, Anderson RC, Adams P Jr, Lilliehei CW, Edwards JE. The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 1963;11:714–25.
2. Dranseika V, Pretre R, Kretschmar O, Dave H. Melody valve to replace the mitral valve in small children: Lessons learned. Ann Pediatr Cardiol. 2021 Jan-Mar;14(1):35-41.