Heart management in dystrophinopathies relies mainly on cardioprotective drugs including angiotensin converting enzyme (ACE) inhibitors, beta-blockers, and aldosterone antagonists. DMD patients exhibit simultaneously a restrictive respiratory failure, requiring long-term home mechanical ventilation (HMV), which may influence cardiac functionThis study analysed the natural history of cardiac function in patients with DMD and BMD (dystrophinopathies) on HMV. The authors reviewed the chart of DMD and BMD patients to obtain ventilation setting at HMV initiation and echocardiographic data at baseline and end follow up, as well as onset cardiac events and thoracic mechanical complication. They analysed cumulative incidence of cardiac events as well as echocardiographic parameters evolution and its association with ventilation settings. All patients were on HMV and 46% ventilated using tracheostomy. After a median follow up of 6.3 years, they found a slight decrease of the left ventricular ejection fraction (LVEF) (45% at end follow up vs 50% at baseline P = .019) and a stabilization of the LV end diastolic diameter indexed (LVEDD indexed 29.4 mm/m vs 30.7 mm/m at end follow up, P = .17). Tidal volume (VT) level was inversely associated with the annual rate of the LVEF decline (r = -0.29, P = .025). Left atrium (LA) diameter decreased with mechanical ventilation (24 mm vs 20 mm, P = .039) and a reduction of systolic pulmonary pressure (35 mm Hg vs 25 mm Hg, P = .011) was observed. The cumulative incidence of cardiac events was 12.6%. Pneumothorax occurred in 4% of patients. Hypoxic arrest secondary to the presence of tracheal plugin occurred in 4% of patients with invasive ventilation. HMV is not harmful, decreases pulmonary pressure and may protect heart in dystrophinopathies, in addition with cardioprotective drugs.
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