44
Table 8. Clinical scenarios for end-of-life management in
patients with NMD
Clinical scenario
3. A 17-year-old adolescent male with DMD was admitted with heart failure,
increasing dyspnea, nausea, and peripheral edema. He had undergone primary
prevention, single-lead ICD implantation for left ventricular dysfunction and
premature ventricular contractions with a dilated left ventricle with an ejection
fraction of 22% 4 years earlier. He has been wheelchair bound for the past 8 years
for progressive muscle weakness. He has been treated with aspirin, metoprolol,
angiotensin receptor neprilysin inhibitor, and furosemide. His LVEF on this
admission is estimated to be 20%, and he has developed an intraventricular
conduction delay with a QRS duration of 130 ms. He was treated with intravenous
milrinone with modest improvement of symptoms. An upgrade of the ICD to a
CRT-D was discussed with the patient and his parents. The patient was reluctant
despite the urging of his parents. He was discharged to home with intravenous
milrinone in addition to his admission heart failure medical regimen.
Shared Decision-making and End-of-life Care
(cont'd)
