5 key points:
- Reduction in peak systolic strain of basalpostero-lateral segments (preference location) is the a powerful predictor to distinguish between patients with and without late-enhancement on CMR
- Global systolic strain correlates with the amount of late-enhancement on CMR
- Longitudinal function (basal posterolateral) is already impaired in patients without hypertrophy and no late-enhancement on CMR (especially in women)
- Peak systolic strain rate increases significantly in the posterior wall in response to treatment (enzyme replacement therapy)
- No specific cut-off values for regional or GLS are published regarding diagnostic accuracy, prognosis, or response to therapy.
- Weidemann F, Breunig F, Beer M, Sandstede J, Turschner O, Voelker W, Ertl G, Knoll A, Wanner C, Strotmann JM. Improvement of cardiac function during enzyme replacement therapy in patients with Fabry disease: a prospective strain rate imaging study. Circulation. 2003 Sep 16;108(11):1299-301. Epub 2003 Sep 2.
- Weidemann F, Breunig F, Beer M, Sandstede J, Störk S, Voelker W, Ertl G, Knoll A, Wanner C, Strotmann JM. The variation of morphological and functional cardiac manifestation in Fabrydisease: potential implications for the time course of the disease. Eur Heart J. 2005 Jun;26(12):1221-7.
- Weidemann F, Niemann M, Breunig F, Herrmann S, Beer M, Störk S, Voelker W,Ertl G, Wanner C, Strotmann J. Long-term effects of enzyme replacement therapy on fabry cardiomyopathy: evidencefor a better outcome with early treatment. Circulation. 2009 Feb 3;119(4):524-9.
- Niemann M, Herrmann S, Hu K, Breunig F, Strotmann J, Beer M, Machann W,Voelker W, Ertl G, Wanner C, Weidemann F. Differences in Fabry cardiomyopathy between female and male patients:consequences for diagnostic assessment. JACC Cardiovasc Imaging. 2011 Jun;4(6):592-601.
- Krämer J, Niemann M, Liu D, Hu K, Machann W, Beer M, Wanner C, Ertl G,Weidemann F. Two-dimensional speckle tracking as a non-invasive tool for identification ofmyocardial fibrosis in Fabry disease. Eur Heart J. 2013 Jun;34(21):1587-96.
- Saccheri MC, Cianciulli TF, Lax JA, Gagliardi JA, Cáceres GL, Quarin AE,Kisinovsky I, Rozenfeld PA, Reisin RC. Two-dimensional speckle tracking echocardiography for early detection ofmyocardial damage in young patients with Fabry disease. Echocardiography. 2013 Oct;30(9):1069-77.
- Shanks M, Thompson RB, Paterson ID, Putko B, Khan A, Chan A, Becher H, OuditGY. Systolic and diastolic function assessment in fabry disease patients using speckle-tracking imaging and comparison with conventional echocardiographic measurements. J Am Soc Echocardiogr. 2013 Dec;26(12):1407-14.
- Morris DA, Blaschke D, Canaan-Kühl S, Krebs A, Knobloch G, Walter TC,Haverkamp W. Global cardiac alterations detected by speckle-tracking echocardiography in Fabrydisease: left ventricular, right ventricular, and left atrial dysfunction arecommon and linked to worse symptomatic status. Int J Cardiovasc Imaging. 2015 Feb;31(2):301-13.
- Labombarda F, Saloux E, Milesi G, Bienvenu B. Loss of base-to-apex circumferential strain gradient: A specific pattern of Fabrycardiomyopathy? Echocardiography. 2017 Apr;34(4):504-510.
- Moon JC, Sachdev B, Elkington AG, McKenna WJ, Mehta A, Pennell DJ, Leed PJ, Elliott PM. Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease. Evidence for a disease specific abnormality of the myocardial interstitium. Eur. Heart J. 24, 2151–2155 (2003).