TABLE 1.
Barth syndrome patient (Case 1) imaging data
| Date | Modality | SF% | EF% | LVEDD mm (Z) | LV hypertrophy | LV function | RV function |
|---|---|---|---|---|---|---|---|
| 11/12/20 | TTE | 13 | 26–39 | 19 (−0.1) | Moderate‐severe | Normal | |
| 11/13/20 | TTE | 21 | 44–48 | 19 (0) | Mild, dyskinetic | Low‐normal to mild dysfxn | |
| 11/14/20 | TTE | 26 | Severe | Mild, dyskinetic | Mild | ||
| 11/15/20 | TTE | 19 | 45 | 20 (+0.8) | Mild, dyskinetic | Mild | |
| 11/19/20 | TTE | 10 | 40–50 | 21 (+1.1) | Moderate, dyskinetic | Mild | |
| 11/24/20 | TTE | 17 | 25–28 | 23 (+2.6) | Moderate | Severe, dyskinetic | Normal |
| 11/25/20 | TTE | 11 | 35 | 21 (+1.2) | Moderate | Severe, dyskinetic | Normal |
| 11/30/20 | TTE | 13 | 26–32 | 22 (+1.8) | Moderate | Severe, dyskinetic | Normal |
| 12/02/20 Initiation of sacubitril‐valsartan | |||||||
| 12/4/20 | TTE | 18 | 37–38 | 20 (+0.7) | Moderate | Moderate, dyskinetic | Low‐normal |
| 12/05/20 Initiation of elamipretide at 0.25 mg/kg | |||||||
| 12/7/20 | TTE | 15 | 39–66 | 20 (−0.3) | Mild | Low‐normal | Normal |
| 12/10/20 Off milrinone last | |||||||
| 12/12/20 Elamipretide to 0.5 mg/kg | |||||||
| 12/12/20 | TTE | 22 | 41–53 | 18 (−1.0) | Mild | Mild‐moderate, dyskinetic | Normal |
| 12/16/20 | TTE | 41–54 | 20 (+0.2) | Mild | Mild, dyskinetic | Normal | |
| 12/22/20 | TTE | 29 | 48 | 21 (+0.3) | Mild | Mild, dyskinetic | Normal |
| 12/30/20 | TTE | 23 | 51–54 | 20 (−0.3) | Mild, LVNC | Mild, dyskinetic | Normal |
| 1/15/21 | TTE | 21 | 44–46 | 22 (−0.1) | Mild, LVNC | Mild, dyskinetic | Normal |
| 2/19/21 | TTE | 22 | 58 | 19 (−2.4) | Mild, LVNC | Mild, dyskinetic | Normal |
| 4/15/2021 | TTE | 30 | 50–57 | 23 (−0.8) | Mild, LVNC | Low‐normal, dyskinesic | Normal |
Abbreviations: EF, ejection fraction; LVEDD, left ventricle end‐diastolic dimension; LVNC, left ventricular noncompaction; RV, right ventricle; SF, shortening fraction; TTE, transthoracic echocardiogram.