Cardiac involvement has recently been the focus of sporadic late-onset nemaline myopathy (SLONM). However, right ventricular failure and pulmonary hypertension, in addition to repetitive cardiac arrest, are noteworthy characteristics of SLONM. We herein report a 66-year-old woman with SLONM whose main symptoms were cardiac arrest, right ventricular failure, and pulmonary hypertension. Despite permanent pacemaker replacement, cardiac arrest occurred repetitively, and even with continuous positive airway pressure, right ventricular failure and pulmonary hypertension persisted. The patient was finally diagnosed with SLONM by a muscle biopsy. Our case suggests the possibility of cardiovascular involvement in SLONM, especially right ventricular failure and pulmonary hypertension.
Introduction
Beyond pulmonary vein isolation (PVI), additional therapeutic strategies for atrial fibrillation (AF) have not been established. Remodeling of the left atrium (LA) could impact AF recurrence post‐PVI. We investigated the impact of unipolar voltage (UV) criteria for the LA posterior wall (LA‐PW) on AF recurrence post‐PVI.
Methods
We reviewed the cases of 106 AF patients (mean age 63.8 years, nonparoxysmal AF: 59%) who underwent extensive encircling PVI by radiofrequency ablation guided by a 3‐dimension mapping system, investigating the impact on AF recurrence of the UV criteria of the LA.
Results
Out of all patients, 26 patients had AF recurrence during post‐PVI follow‐up [median 603 days]. They showed a higher percentage of nonparoxysmal AF (80.8 vs. 52.5%, p = .011), longer AF duration (2.9 ± 2.7 vs. 1.0 ± 1.7 years, p = .002), and larger area size of UV < 2.0 mV in LA‐PW (2.8 ± 1.8 vs. 1.0 ± 1.5 cm2, p < .001) than those without recurrence. Cox Hazard analysis for AF recurrence adjusted by age, gender, AF duration, body mass index and left atrial volume index revealed that an area size over 2.0 cm2 of UV < 2.0 mV in LA‐PW (HR 6.9 [95% CI:1.3–35.5], p = .021) posed independent risks for AF recurrence post‐PVI. The atrial arrhythmia‐free survival rate was higher in those with no area of UV < 3.0 mV in LA‐PW compared to those with a sizable area (>2.0 cm2) of UV < 3.0 mV and <2.0 mV (95.0% vs. 74.2% vs. 57.1%, Log‐Rank: p < .001). In the AF etiology of patients with AF recurrence, 9 of 14 patients who underwent the 2nd procedure had no PV reconnection, and 8 patients required the LA‐PW isolation for their non‐PV AF.
Conclusion
UV criteria of LA‐PW is a useful parameter for AF‐recurrence post‐PVI. Lower UV in LA‐PW as an indication of electrical remodeling could indicate a higher risk of AF recurrence and the need for further therapeutic strategies.
Pulmonary arterial hypertension (PAH) is a rare and fatal disease for which some causative drugs have been developed. Qing-Dai is a Chinese herbal drug that is sometimes used as a specific treatment for ulcerative colitis in Asia, including Japan. Here, we report a case of severe Qing-Dai-induced PAH. A 19-year-old woman who has been taking Qing-Dai for 8 months was admitted for exertional dyspnea. Her mean pulmonary artery pressure dramatically improved from 72 to 18 mmHg with Qing-Dai discontinuation and PAH-specific therapy. After 6 years of onset, she had not relapsed with PAH with PAH-specific therapy.
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