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. 2022 Nov 28;7(1):ytac452. doi: 10.1093/ehjcr/ytac452
Patient Date Description
Twin A Index date Patient seen for the first time after referral by patient’s primary cardiologist. Patient diagnosed with basal septal hypertrophic cardiomyopathy
Resting left ventricular outflow tract (LVOT) gradient 83 mmHg
Metoprolol succinate 50 mg twice daily, verapamil 120 mg daily, and disopyramide phosphate 100 mg twice daily started over a period of 1 month
1–3 months Metoprolol dose decreased and verapamil discontinued due to sinus bradycardia
Disopyramide phosphate dose increased
8 months No resting LVOT obstruction
Valsalva-induced gradient 60 mmHg
No further dosing adjustments
24 months Valsalva-induced gradient 76 mmHg
Disopyramide phosphate increased to 600 mg in three divided doses
32 months Severe resting LVOT gradient of 108 mmHg
Alcohol septal ablation recommended
33 months Patient had alcohol septal ablation
No more resting or provocable LVOT obstruction
40 months Cardiac magnetic resonance imaging showed patchy delayed gadolinium enhancement of the basal septum
96 months No resting or Valsalva-induced LVOT obstruction
Twin B Index date After her twin sister’s diagnosis, patient seen for the first time. Patient diagnosed with basal septal variant of hypertrophic cardiomyopathy
Resting LVOT gradient of 146 mmHg
Metoprolol succinate 50 mg daily started
1 month Resting LVOT gradient improved to 100 mmHg. Metoprolol dose increased.
2 months Resting LVOT gradient 90 mmHg
Disopyramide phosphate 100 mg twice daily added
4 months Resting LVOT gradient stable at 90 mmHg.
Disopyramide phosphate dose increased; verapamil 180 mg daily added.
6 months No more resting or provocable LVOT obstruction.
20 months Cardiac magnetic resonance imaging shows patchy delayed gadolinium enhancement of the basal septum.
84 months No resting or Valsalva-induced LVOT obstruction.