Gradiente elevado no trato de saída do ventrículo esquerdo: estenose aórtica, miocardiopatia hipertrófica obstrutiva ou ambas?

The dilemma of the patient with both AS and LVOTO is now commonly encountered in clinical practice; indeed, physicians must be aware of the complex interaction and coexistent nature of both diseases, especially as both HOCM and TAVR have increased in awareness and prevalence. Importantly, the clinician must be aware of the complex interplay hemodynamically, with the two diseases confusing the TTE imaging and potentially affecting each other anatomically and clinically. There is no set guideline on how to approach this from a surgical or percutaneous approach, but we have outlined a set of recommendations which should serve the clinician and patient well. The three cases that are presented illustrate that methodical diagnosis in addition to the order of treatment do indeed matter. In the first case, there was AS and an underestimated LVOT gradient that was also present. Once the AS was corrected, the true LVOT gradient potential was evidenced and she decompensated, likely because there was a rapid decrease in afterload. Patients with concomitant LVOTO are not able to adjust quickly to the hemodynamic changes created by the rapid decline in afterload, as, for example, in HOCM patients who receive nitroglycerin. The second case demonstrated that when the LVOTO was severe and the AS nonsignificant (mild or moderate), the patient was able to live without symptoms for several years after successful alcohol septal ablation (ASA). She eventually needed an aortic valve and mitral valve replacement but that was postponed for several years until the AS became more significant, and the surgical risk was lowered by the elimination of the need for concomitant myectomy. In the last case, the patient was able to have both an ASA and TAVR within 3 months of each other without hemodynamic compromise. Indeed, this latter therapy sequence may be the best way to treat patients with both diseases in the future, as both ASA and TAVR continue to evolve into intermediate and lower-risk patient populations and the safety of ASA continues to be evident.

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Evaluation and Management of Concomitant Hypertrophic Obstructive Cardiomyopathy and Valvular Aortic Stenosis

Shenouda

Silber

Subramaniam

et al. 2016

Curr Treat Options Cardio Med

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Discrete Subaortic Stenosis: Perspective Roadmap to a Complex Disease

Massé

Shar

Brown

et al. 2018

Front. Cardiovasc. Med.

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Discrete subaortic stenosis (DSS) is a congenital heart disease that results in the formation of a fibro-membranous tissue, causing an increased pressure gradient in the left ventricular outflow tract (LVOT). While surgical resection of the membrane has shown some success in eliminating the obstruction, it poses significant risks associated with anesthesia, sternotomy, and heart bypass, and it remains associated with a high rate of recurrence. Although a genetic etiology had been initially proposed, the association between DSS and left ventricle (LV) geometrical abnormalities has provided more support to a hemodynamic etiology by which congenital or post-surgical LVOT geometric derangements could generate abnormal shear forces on the septal wall, triggering in turn a fibrotic response. Validating this hypothetical etiology and understanding the mechanobiological processes by which altered shear forces induce fibrosis in the LVOT are major knowledge gaps. This perspective paper describes the current state of knowledge of DSS, articulates the research needs to yield mechanistic insights into a significant pathologic process that is poorly understood, and proposes several strategies aimed at elucidating the potential mechanobiological synergies responsible for DSS pathogenesis. The proposed roadmap has the potential to improve DSS management by identifying early targets for prevention of the fibrotic lesion, and may also prove beneficial in other fibrotic cardiovascular diseases associated with altered flow.

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Gradiente elevado no trato de saída do ventrículo esquerdo: estenose aórtica, miocardiopatia hipertrófica obstrutiva ou ambas?

Cited by 2 publications

References 19 publications

Evaluation and Management of Concomitant Hypertrophic Obstructive Cardiomyopathy and Valvular Aortic Stenosis

Evaluation and Management of Concomitant Hypertrophic Obstructive Cardiomyopathy and Valvular Aortic Stenosis

Discrete Subaortic Stenosis: Perspective Roadmap to a Complex Disease

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