Occurrence and significance of progressive left ventricular wall thinning and relative cavity dilatation in hypertrophic cardiomyopathy

Paolo Spirito*, Barry J. Maron, Robert O. Bonow, Stephen E. Epstein

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

256 Scopus citations

Abstract

This investigation was undertaken to assess the prevalence of systolic dysfunction, left ventricular (LV) wall thinning and cavity enlargement in a large population of patients with hypertrophic cardiomyopathy (HC), and to determine the role of these conditions in the natural-history of this disease. Of 217 consecutively studied patients with HC, most of whom were severely symptomatic, 197 (91 %) had an LV ejection fraction of 50% or more and 20 (9%) had an ejection fraction of less than 50% as assessed with radionuclide anglography. Changes in LV wall thickness and cavity dimension were evaluated using serial M-mode and 2-dimensional echocardiography over an average follow-up of 3.6 years in 67 of the 217 patients (54 with ejection fraction of ≥ 50 % and 13 with ejection fraction <50%). A substantial decrease (at least 5 mm) in LV wall thickness was seen in 8 of the 13 patients (62%) with an ejection fraction {succeeds above single-line equals sign} 50%, but in only 2 of the 54 patients (4%) with an ejection fraction ≥ 50 % (p <0.001). LV cavity dimension increased significantly over the period of follow-up in the 13 patients with depressed ejection fraction (from 44 ± 5 to 49 ± 7 mm, p <0.005); however, absolute cavity size remained normal (≤52 mm or less) in 10 of these 13 patients. The clinical course was poor in each of the 8 patients who showed LV systolic dysfunction, wall thinning and an enlarging cavity; all had severe symptoms, including 4 who died of congestive cardiac failure, and 1 who underwent cardiac transplantation. It is concluded that LV systolic impairment, associated with progressive LV wall thinning and relative cavity enlargement, are present in almost 10 % of patients with HC and severe symptoms. These functional and morphologic changes are usually associated with refractory cardiac failure and poor prognosis, and appear to identify a subset of patients with HC who have a distinctive and well defined natural history.

Original languageEnglish (US)
Pages (from-to)123-129
Number of pages7
JournalThe American journal of cardiology
Volume60
Issue number1
DOIs
StatePublished - Jul 1 1987

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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