부산대학교 도서관

A 66-year-old male with a history of carpal tunnel syndrome and hypertension presented with dyspnea (NYHA III), angina pectoris, and sinus bradycardia. On clinical examination, the patient was stable and euvolemic. A systolic murmur (III/VI) was audible in the 4th left intercostal space laterally, augmented after a Valsalva maneuver. An electrocardiogram revealed normal sinus rhythm, heart rate of 81 bpm, normal voltage, and non-specific ST-changes in I, II, III, aVF, V5, and V6. The patient underwent a transthoracic echocardiography, which revealed thickened ventricular walls (IVSd 23 mm, PWd 20 mm), hyperdynamic left ventricular ejection fraction (LVEF 75%), LVOT gradient of 150 mmHg maximum (after Valsalva maneuver), apical sparing on strain analysis, and a dilated atria. A coronary angiography ruled out coronary artery disease. A endomyocardial biopsy revealed hypertrophic cardiomyopathy combined with focal cardiac amyloidosis (Figure 1). Subtyping and genetic analysis revealed transthyretin (ATTR) wild-type amyloidosis. Accordingly, therapy with Tafamidis 61 mg daily was initiated. Given the relevant amount of hypertrophy depicted on the biopsy samples, the high LVOT gradient, and the patient’s debilitating symptoms despite maximal medical therapy, the decision was made to attempt a transcoronary alcohol ablation of the septal hypertrophy (TASH). The TASH reduced the LVOT gradient from 150 mmHg to 35 mmHg. After the procedure, the patient experienced significant symptom improvement (NYHA II) and was discharged the next day. This case illustrates the feasibility of marked LVOT gradient reduction in patients with amyloidosis and hypertrophic cardiomyopathy through a TASH procedure. Figure 1: Histology reveals hypertrophic cardiomyocytes and cardiac amyloidosis on Congo red staining.