Supravalvar mitral ring with a parachute mitral valve and subcoarctation of the aorta in a child with hemodynamically significant VSD. A study of the morphology, echocardiographic diagnostics and surgical therapy

Abstract

The authors present a case of echocardiographic diagnosis of supravalvar mitral ring (a fibromembranous structure that arose from the atrial surface of the mitral leaflets) in a child with a parachute mitral valve, a ventricular septal defect, and mild narrowing of the aortic isthmus. The supravalvar mitral stenosis is a typical but very infrequently detected element of the complex of anatomical abnormalities located within the left heart and the proximal aorta, called the Shone’s complex (syndrome). Diagnosing an additional, hemodynamically significant anatomic defect during echocardiography was possible thanks to the detection of marked mobility limitation of the ring-adjacent part of the mitral valve mural leaflet as well as of an atypical image of turbulence occurring during the inflow from the left atrium to the left ventricle. The early diagnosis made it possible to perform complete correction of this complex congenital defect within a single operation.

Parachute mitral valve: Morphology and surgical management

“…Accordingly, 20 patients (9.3%) were not operated due to conservative treatment/on a follow-up/waiting for surgical operation/operation refusal/sudden death, and loss of surgical opportunity, [12,13,15,21,22,25,26,29,41,42,45,[48][49][50][51][52]57,66] while 196 patients (90.7%) received a total of 198 surgical operations as follows: non-mitral valve operations (n=70, 35.4%) [3,11,14,26,30,33,36,39,46,61] and PMV: parachute mitral valve; * One of the asymptomatic patients developed dyspnea later. [41] [39,43,45,48,53,62] • Anterolateral (n=14) [39,65] • Inferolateral [56] • Left (n=1) [32] • Central [54] • Apical [63] Supravalvular mitral ring 10 [9,10,38,39] 3.5 11 [4,7,…”

Perioperative and Anesthetic Considerations in Shone’s Complex

“…The tissue may arise from and be located entirely within the atrium, or the tissue may be connected to the MV. 1,5 SMR is considered a characteristic lesion, and it represents the most common cause of mitral stenosis (MS) in Shone's complex. 6,7 Subaortic stenosis occurs in one of two forms, either the muscular or membranous type.…”

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“…Nowadays, management of hypertension remains a not-solved problem due to the multiple side effects of existing antihypertensive drugs and growing cases of uncontrolled hypertension. In recent years, interest in the cyclic vasoactive neuropeptide urotensin-2 (U-II), which is characterized by strong vasoconstrictor properties, has been increased [38][39]. Excessive secretion of U-II and up-regulation of UT-II receptors have been identified in several pathological states, especially hypertension.…”

“…The severity of mitral valve obstruction was found to be inversely correlated with long-term outcomes, and operative mortality in patients with Schon syndrome was found to ultimately negatively affect operative mortality. However, there is no significant association between progressive mitral stenosis and PMA type, dominant papillary muscle, sex, or surgical or interventional treatment [36][37][38][39]. Since MVPs are usually not isolated lesions and are characterized by a combination of pathological changes in the mitral valve leaflets, annulus, adhesions, sub valvular apparatus, and supravalvular mitral annulus, most patients require one or more surgeries and the frequency of reoperations is high.…”