Ventricular Septal Myectomy for the Treatment of Left Ventricular Outflow Tract Obstruction Due to Fabry Disease

Raju, Bharath; Roberts, Charles S.; Sathyamoorthy, Mohanakrishnan; Schiffman, Raphael; Swift, Caren; McCullough, Peter A.

doi:10.1016/j.amjcard.2020.07.020

The American Journal of Cardiology

2020

DOI: 10.1016/j.amjcard.2020.07.020

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Ventricular Septal Myectomy for the Treatment of Left Ventricular Outflow Tract Obstruction Due to Fabry Disease

Bharath Raju

Charles S. Roberts

Mohanakrishnan Sathyamoorthy

et al.

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“…The literature describes only a small group of patients who have undergone surgical myectomy, 4,5 or single myectomy case including discussion of our previous abstract data. 6 However, only a single case report of a successful alcohol septal ablation (ASA) was published previously. 7 Our aim was to assess the feasibility and effectiveness of an ASA in Fabry patients with symptomatic LVOTO.…”

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confidence: 99%

Usefulness of Alcohol Septal Ablation in the Left Ventricular Outflow Tract Obstruction in Fabry Disease Cardiomyopathy

Zemánek

Marek

Dostálová

et al. 2021

The American Journal of Cardiology

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Fabry disease (FD) is an X-linked linked genetic disorder caused by a-galactosidase A deficiency. The typical clinical manifestation is left ventricular hypertrophy, often mimicking hypertrophic cardiomyopathy (HC). In contrast to sarcomeric HC, left ventricular outflow tract obstruction (LVOTO) is less frequent. We describe 6 male patients with genetically confirmed FD and symptomatic LVOTO. All of them underwent a transcatheter alcohol septal ablation with an immediate effect on the obstruction in all cases and without any serious complications. The median LVOT maximal pressure gradient was 85 (60 to 170) mm Hg. The hemodynamic effect persisted during subsequent follow-up (ranging from 6 months to 16 years). Five patients reported substantial symptomatic improvement. Four patients were receiving specific FD therapy before the interventional procedure. In conclusion, alcohol septal ablation appears to be effective in the treatment of LVOTO in patients with FD and appears to be comparable to the limited published experience with surgical septal myectomy. Despite some important differences between FD HC and sarcomeric HC, the recommendation for treating LVOTO should be similar.

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confidence: 99%

Usefulness of Alcohol Septal Ablation in the Left Ventricular Outflow Tract Obstruction in Fabry Disease Cardiomyopathy

Zemánek

Marek

Dostálová

et al. 2021

The American Journal of Cardiology

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Analysis of Echocardiographic and Mri Anatomical Characteristics and Hemodynamic Characteristics of Left Ventricular Outflow Tract Stenosis

Wang

Song

et al. 2021

J. Mech. Med. Biol.

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Objective: To analyze the anatomical morphological and hemodynamic characteristics of left ventricular outflow tract stenosis (LVOTS) by echocardiography and MRI. Methods: The Echocardiography data of 112 patients with LVOTS were retrospectively analyzed by measuring the basal interventricular septal thickness (IVST-b), the left ventricle posterior wall thickness (LVPWT), and the peak pressure gradient of LVOTS, as well as by observing the characteristics of spectral pattern and the presence of systolic anterior motion of mitral valve leaflets. A Siemens 3.0T MRI scanner was used to scan the contrast-enhanced left ventricular (LV) volume of all cases. The obtained end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV) and ejection fraction (EF) of LV were compared with the Echocardiography results. Results: The 112 patients were divided into four groups: hypertrophic obstructive cardiomyopathy (Group I, 42 cases), hypertensive left ventricular hypertrophy (Group II, 40 cases), basal septal hypertrophy in the elderly (Group III, 21 cases), and the subaortic membrane (Group IV, 9 cases). The continuous wave (CW) Doppler characteristic of Groups I, II, and III was broadsword-shaped jet, and that of Group IV was equilateral triangle-like spectrum. The IVST-b, IVST-b/LVPWT ratio and peak pressure gradient of LVOTS of Group I was significantly higher than those in Groups II and III ([Formula: see text]). The LVPWT of the first three groups was slightly correlated with the LVOTS peak pressure gradient ([Formula: see text], respectively, [Formula: see text]). There were no statistically significant differences between Echocardiography and MRI results regarding the LV EDV, ESV, SV, and EF ([Formula: see text]), and no statistically significant differences were found between Echocardiography and MRI results regarding the myocardial thicknesses of septal, anterior, lateral, and inferior walls ([Formula: see text]). The Pearson’s correlation analysis demonstrated correlations between MRI and Echocardiography results for LV EDV, ESV, SV, and EF ([Formula: see text], 0.002, 0.009, and [Formula: see text], respectively). The MRI enhancement was shown as delayed enhancement in 52 cases, localized enhancement in 8 cases, diffuse enhancement in 6 cases, and transmural enhancement in 3 cases, with abnormal enhancement lesions distributed in the area of ventricular septum free wall junctions and ventricular septum. Conclusion: Using MRI to evaluate LV function of hypertrophic cardiomyopathy is reliable and accurate. MRI enhancement can be used for the quantitative measurement of myocardial fibrosis. Echocardiography can distinguish the stenosis types of LVOTS. The IVST-b and existence of SAM may be important anatomical characteristics determining the degree of dynamic stenosis, and MRI combined with Echocardiography can provide important detailed information.

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Anderson-Fabry disease cardiomyopathy: an update on epidemiology, diagnostic approach, management and monitoring strategies

Averbuch

White

Fine

2023

Front. Cardiovasc. Med.

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Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by deficient activity of the enzyme alpha-galactosidase. While AFD is recognized as a progressive multi-system disorder, infiltrative cardiomyopathy causing a number of cardiovascular manifestations is recognized as an important complication of this disease. AFD affects both men and women, although the clinical presentation typically varies by sex, with men presenting at a younger age with more neurologic and renal phenotype and women developing a later onset variant with more cardiovascular manifestations. AFD is an important cause of increased myocardial wall thickness, and advances in imaging, in particular cardiac magnetic resonance imaging and T1 mapping techniques, have improved the ability to identify this disease non-invasively. Diagnosis is confirmed by the presence of low alpha-galactosidase activity and identification of a mutation in the GLA gene. Enzyme replacement therapy remains the mainstay of disease modifying therapy, with two formulations currently approved. In addition, newer treatments such as oral chaperone therapy are now available for select patients, with a number of other investigational therapies in development. The availability of these therapies has significantly improved outcomes for AFD patients. Improved survival and the availability of multiple agents has presented new clinical dilemmas regarding disease monitoring and surveillance using clinical, imaging and laboratory biomarkers, in addition to improved approaches to managing cardiovascular risk factors and AFD complications. This review will provide an update on clinical recognition and diagnostic approaches including differentiation from other causes of increased ventricular wall thickness, in addition to modern strategies for management and follow-up.

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Ventricular Septal Myectomy for the Treatment of Left Ventricular Outflow Tract Obstruction Due to Fabry Disease

Cited by 3 publications

References 12 publications

Usefulness of Alcohol Septal Ablation in the Left Ventricular Outflow Tract Obstruction in Fabry Disease Cardiomyopathy

Usefulness of Alcohol Septal Ablation in the Left Ventricular Outflow Tract Obstruction in Fabry Disease Cardiomyopathy

Analysis of Echocardiographic and Mri Anatomical Characteristics and Hemodynamic Characteristics of Left Ventricular Outflow Tract Stenosis

Anderson-Fabry disease cardiomyopathy: an update on epidemiology, diagnostic approach, management and monitoring strategies

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