Apical Aneurysms and Mid-Left Ventricular Obstruction in Hypertrophic Cardiomyopathy

JACC Cardiovasc Imaging. 2023 May;16(5):591-605. doi: 10.1016/j.jcmg.2022.11.013. Epub 2023 Jan 11.

Abstract

Background: Apical left ventricular (LV) aneurysms in hypertrophic cardiomyopathy (HCM) are associated with adverse outcomes. The reported frequency of mid-LV obstruction has varied from 36% to 90%.

Objectives: The authors sought to ascertain the frequency of mid-LV obstruction in HCM apical aneurysms.

Methods: The authors analyzed echocardiographic and cardiac magnetic resonance examinations of patients with aneurysms from 3 dedicated programs and compared them with 63 normal controls and 47 controls with apical-mid HCM who did not have aneurysms (22 with increased LV systolic velocities).

Results: There were 108 patients with a mean age of 57.4 ± 13.5 years; 40 (37%) were women. A total of 103 aneurysm patients (95%) had mid-LV obstruction with mid-LV complete systolic emptying. Of the patients with obstruction, 84% had a midsystolic Doppler signal void, a marker of complete flow cessation, but only 19% had Doppler systolic gradients ≥30 mm Hg. Five patients (5%) had relative hypokinesia in mid-LV without obstruction. Aneurysm size is not bimodal but appears distributed by power law, with large aneurysms decidedly less common. Comparing mid-LV obstruction aneurysm patients with all control groups, the short-axis (SAX) systolic areas were smaller (P < 0.007), the percent SAX area change was greater (P < 0.005), the papillary muscle (PM) areas were larger (P < 0.003), and the diastolic PM areas/SAX diastolic areas were greater (P < 0.005). Patients with aneurysms had 22% greater SAX PM areas compared with those with elevated LV velocities but no aneurysms (median: 3.00 cm2 [IQR: 2.38-3.70 cm2] vs 2.45 [IQR: 1.81-2.95 cm2]; P = 0.004). Complete emptying occurs circumferentially around central PMs that contribute to obstruction. Late gadolinium enhancement was always brightest and the most transmural apical of, or at the level of, complete emptying.

Conclusions: The great majority (95%) of patients in the continuum of apical aneurysms have associated mid-LV obstruction. Further research to investigate obstruction as a contributing cause to apical aneurysms is warranted.

Keywords: apical hypertrophic cardiomyopathy; apical left ventricular aneurysm; cardiac magnetic resonance; echocardiography; hypertrophic cardiomyopathy; left ventricular aneurysm; left ventricular obstruction; papillary muscles.

MeSH terms

  • Adult
  • Aged
  • Cardiomyopathy, Hypertrophic* / complications
  • Cardiomyopathy, Hypertrophic* / diagnostic imaging
  • Cardiomyopathy, Hypertrophic* / pathology
  • Contrast Media*
  • Female
  • Gadolinium
  • Heart Ventricles / diagnostic imaging
  • Humans
  • Male
  • Middle Aged
  • Predictive Value of Tests

Substances

  • Contrast Media
  • Gadolinium