Cardiac Involvement in Werdnig-Hoffmann's Spinal Muscular Atrophy

Cardiology. 1999;92(3):178-82. doi: 10.1159/000006968.


Despite the increasing interest in cardiac involvement (CI) of neuromuscular disorders, only few data are available on CI in spinal muscular atrophy (SMA). We tried to determine the cardiac manifestations of SMA, their incidence rates and the necessity of cardiac therapy in patients with SMA and CI. Eight patients with SMA, aged 10-79 years, underwent clinical cardiologic examination, ECG, echocardiography and ambulatory ECG. The most frequent findings were angina pectoris, palpitations, exertional dyspnea, ST abnormalities, couplets, thickened myocardium and diastolic dysfunction. In conclusion, cardiac manifestations of SMA mainly comprise ECG abnormalities and thickened myocardium. CI in SMA frequently necessitates cardiac therapy.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Child
  • Echocardiography
  • Electrocardiography, Ambulatory
  • Electromyography
  • Female
  • Heart Diseases / diagnosis
  • Heart Diseases / epidemiology
  • Heart Diseases / etiology*
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Prognosis
  • Prospective Studies
  • Severity of Illness Index
  • Spinal Muscular Atrophies of Childhood / complications*
  • Spinal Muscular Atrophies of Childhood / physiopathology