ABSTRACT
Background and Objective: The prevalence of hypertrophic cardiomyopathy (HCM) is estimated to be 1 in 200 to 500 individuals, with systolic anterior motion (SAM) of the mitral valve (MV) and left ventricular outflow tract (LVOT) obstruction present in 60% to 70%. In this narrative review, we aim to elucidate the pathophysiology of SAM-septal contact and LVOT obstruction in HCM by presenting a detailed review on the anatomy of the MV apparatus in HCM, examining the various existing theories pertaining to the SAM phenomenon as supported by cardiac imaging, and providing a critical assessment of management strategies for SAM in HCM. Methods: A literature review was performed using PubMed, EMBASE, Ovid, and the Cochrane Library, of all scientific articles published through December 2021. A focus was placed on descriptive studies, reports correlating echocardiographic findings with pathologic diagnosis, and outcomes studies. Key Content and Findings: The pathophysiology of SAM involves the complex interplay between HCM morphology, MV apparatus anatomic abnormalities, and labile hemodynamic derangements. Echocardiography and cardiac magnetic resonance (CMR) vector flow mapping have identified drag forces, as opposed to the "Venturi effect", as the main hydraulic forces responsible for SAM. The degree of mitral regurgitation with SAM is variable, and its severity is correlated with degree of LVOT obstruction and outcomes. First line therapy for the amelioration of SAM and LVOT obstruction is medical therapy with beta-blockers, non-dihydropyridine calcium-channel blockers, and disopyramide, in conjunction with lifestyle modifications. In refractory cases septal reduction therapy is performed, which may be combined with a 'resect-plicate-release' procedure, anterior mitral leaflet extension, surgical edge-to-edge MV repair, anterior mitral leaflet retention plasty, or secondary chordal cutting. Conclusions: Recent scientific advances in the field of HCM have allowed for a maturation of our understanding of the SAM phenomenon. Cardiac imaging plays a critical role in its diagnosis, treatment, and surveillance, and in our ability to apply the appropriate therapeutic regimens. The increasing prevalence of HCM places an emphasis on continued basic and clinical research to further improve outcomes for this challenging population.
ABSTRACT
BACKGROUND: Left ventricular non-compaction (LVNC) cardiomyopathy in adults has primarily been studied with a phenotypic expression of low ejection fraction (EF) and dilated cardiomyopathy; however, data on LVNC with preserved EF is scarce. The present study aimed to evaluate cardiac geometry and mechanics in LVNC patients with preserved EF. METHODS: A retrospective cohort study of patients diagnosed with LVNC and a preserved EF between 2008 and 2019 was performed. LVNC was defined according to the presence of established transthoracic 2D echocardiographic (TTE) criteria as follows: (1) prominent LV trabeculations with deep recesses; (2) bi-layered myocardial appearance; and, (3) systolic non-compacted:compacted ratio≥ 2. Subjects were matched 1:1 to controls without LVNC referred for routine TTE. Geometric, functional and mechanics parameters were analyzed in the two cohorts using 2D and speckle-tracking TTE. RESULTS: Seventeen patients with LVNC and preserved EF were identified. Compared with controls, patients with LVNC had similar LV systolic function and chamber dimensions, but a larger mass and relative wall thickness, and more abnormal LV geometry (76% vs. 18%, p = 0.002), LA remodeling, and pulmonary hypertension. Global longitudinal strain was significantly decreased (-15.4 ± 3.2 vs. -18.9 ± 2.8%, p = < 0.01) and the prevalence of rigid body rotation was significantly increased (57% vs. 14%, p = 0.05) in the LVNC population. The peak twist values were comparable in both cohorts. CONCLUSIONS: Impaired LV geometry and longitudinal mechanics, as well as increased myocardial stiffness as expressed by rigid body rotation, characterize LVNC with preserved EF when compared with controls.
Subject(s)
Cardiomyopathy, Dilated , Ventricular Dysfunction, Left , Adult , Echocardiography/methods , Humans , Retrospective Studies , Stroke Volume , Ventricular Dysfunction, Left/diagnostic imaging , Ventricular Function, LeftABSTRACT
Septal myectomy is indicated in patients with obstructive hypertrophic cardiomyopathy (HCM) and intractable symptoms. Concomitant mitral valve (MV) surgery is performed for abnormalities contributing to systolic anterior motion (SAM), or for SAM-mediated mitral regurgitation (MR) with or without left ventricular outflow tract (LVOT) obstruction. One MV repair technique is anterior mitral leaflet extension (AMLE) utilizing bovine pericardium, stiffening the leaflet and enhancing coaptation posteriorly. Fifteen HCM patients who underwent combined myectomy-AMLE for LVOT obstruction or moderate-to-severe MR between 2009 and 2020 were analyzed using detailed echocardiography. The mean age was 56.6 years and 67% were female. The average peak systolic LVOT gradient and MR grade measured 73.4 mmHg and 2.3, respectively. Indications for myectomy-AMLE were LVOT obstruction and moderate-to-severe MR in 67%, MR only in 20%, and LVOT obstruction only in 13%. There was no mortality observed, and median follow-up was 1.2 years. Two patients had follow-up grade 1 mitral SAM, one of whom also had mild LVOT obstruction. No recurrent MR was observed in 93%, and mild MR in 7%. Compared with preoperative measures, there was a decrease in follow-up LV ejection fraction (68.2 vs 56.3%, p = 0.02) and maximal septal wall thickness (25.5 vs 21.3 mm, p < 0.001), and an increase in the end-diastolic diameter (21.9 vs 24.8 mm/m2, p = 0.04). There was no change in global longitudinal strain (-12.1 vs -11.6%, p = 0.73) and peak LV twist (7.4 vs 7.3°, p = 0.97). In conclusion, myectomy-AMLE is a viable treatment option for carefully selected symptomatic HCM patients with LVOT obstruction or moderate-to-severe MR.
