Shone syndrome revealed by treatment-resistant hypertension

Abstract

Introduction and importance: Shone complex is a congenital heart defect consisting of four obstructive defects in the left heart: a mitral supravalvular ring, sub-aortic stenosis, parachute mitral valve, and coarctation of the aorta (CoA), which affects only a small minority of people. Case presentation We report the case of a 25-year-old woman with a past medical history of moderate mitral stenosis, since she was 10-year-old with uncontrolled high blood pressure, treated with nicardipine. admitted to our emergency department with high blood pressure: 190/80 mmhg, in whom The transthoracic echocardiography (TTE) revealed: sub-mitral membrane, with a single sub-papillary muscle, and coarctation of the aorta and the CT scan showed narrowed aortic arch and a left superior vena cava allowing to retain shone syndrome as the main diagnosis. The patient was treated with an antihypertensive treatment combining (perindopril/indapamide/amlodipine) while waiting for surgery. Clinical discussion In this mini-review, we aim to describe this rare pathological condition its pathophysiological thoughts, and the way to diagnosis this complex early. Conclusion Treatment required the coordinated efforts of a team of specialists. It could be either surgical with different method or by Trans catheter treatments.

Nrf2 Signaling Pathway as a Key to Treatment for Diabetic Dyslipidemia and Atherosclerosis

“…In the insulin-deficient environment of T1DM, a reduced insulin level decreases LPL activity, leading to impaired clearance of VLDL [ 48 , 49 ]. The correlation between insulin supply and LPL activity has been demonstrated, where LPL activity can be increased with insulin therapy in both rodents and insulin-dependent human subjects [ 50 , 51 , 52 ]. A study conducted on 16 insulin-deficient DM patients exhibited a significant negative correlation between LPL activity and untreated insulin deficiency.…”

Unrecognized incomplete Shone’s complex. Awareness is key in congenital heart disease

“…The more frequent incomplete Shone's complex warrants presence of at least two lesions [1]. Identification of one left-sided obstructive lesion should prompt a search for other anomalies [2]. Mitral valve obstruction is considered the earliest and most common pathological event in Shone's syndrome, causing underdevelopment of the left ventricle and thus leading to LVOT obstruction and aortic coarctation [1].…”

“…Mitral valve obstruction is considered the earliest and most common pathological event in Shone's syndrome, causing underdevelopment of the left ventricle and thus leading to LVOT obstruction and aortic coarctation [1]. The severity of MV obstruction is the main predictor of long-term outcomes [1,2]. Two types of mitral rings might be the cause of progressive mitral stenosis: the supramitral ring, originating just above the mitral annulus with no adherence to the leaflets and normal subvalvular apparatus, and the intra-mitral ring adherent to the leaflets with abnormal subvalvular apparatus [3].…”

“…Determination of appropriate management strategies in Shone's complex is challenging due to the variability in presentation and severity of individual lesions [2]. Repair, rather than replacement, of the mitral valve should be the first-line strategy when feasible [2].…”

“…Determination of appropriate management strategies in Shone's complex is challenging due to the variability in presentation and severity of individual lesions [2]. Repair, rather than replacement, of the mitral valve should be the first-line strategy when feasible [2]. Stenosis at the subvalvular, valvular, and supravalvular aortic level can be relieved with the anterior aortoventriculoplasty, known as the Konno procedure [5].…”