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Abstract：
UNASSIGNED: Apical hypertrophic cardiomyopathy (ApHCM) is a subtype of hypertrophic cardiomyopathy (HCM) that affects up to 25% of Asian patients and is not as well understood in non-Asian patients. Although ApHCM has been considered a more \"benign\" variant, it is associated with increased risk of atrial and ventricular arrhythmias, apical thrombi, stroke, and progressive heart failure. The occurrence of pulmonary hypertension (PH) in ApHCM, due to elevated pressures on the left side of the heart, has been documented. However, the exact prevalence of PH in ApHCM and sex differences remain uncertain.
UNASSIGNED: We sought to evaluate the prevalence, risk associations, and sex differences in elevated pulmonary pressures in the largest cohort of patients with ApHCM at a single tertiary center. A total of 542 patients diagnosed with ApHCM were identified using ICD codes and clinical notes searches, confirmed by cross-referencing with cardiac MRI reports extracted through Natural Language Processing and through manual evaluation of patient charts and imaging records.
UNASSIGNED: In 414 patients, echocardiogram measurements of pulmonary artery systolic pressure (PASP) were obtained at the time of diagnosis. The mean age was 59.4 ± 16.6 years, with 181 (44%) being females. The mean PASP was 38 ± 12 mmHg in females vs. 33 ± 9 mmHg in males (p < 0.0001). PH as defined by a PASP value of > 36 mmHg was present in 140/414 (34%) patients, with a predominance in females [79/181 (44%)] vs. males [61/233 (26%), p < 0.0001]. Female sex, atrial fibrillation, diagnosis of congestive heart failure, and elevated filling pressures on echocardiogram remained significantly associated with PH (PASP > 36 mmHg) in multivariable modeling. PH, when present, was independently associated with mortality [hazard ratio 1.63, 95% CI (1.05-2.53), p = 0.028] and symptoms [odds ratio 2.28 (1.40, 3.71), p < 0.001].
UNASSIGNED: PH was present in 34% of patients with ApHCM at diagnosis, with female sex predominance. PH in ApHCM was associated with symptoms and increased mortality.
UNASSIGNED: We sought to evaluate the prevalence, risk associations, and sex differences in elevated pulmonary pressures in the largest cohort of patients with ApHCM at a single tertiary center. A total of 542 patients diagnosed with ApHCM were identified using ICD codes and clinical notes searches, confirmed by cross-referencing with cardiac MRI reports extracted through Natural Language Processing and through manual evaluation of patient charts and imaging records.
UNASSIGNED: In 414 patients, echocardiogram measurements of pulmonary artery systolic pressure (PASP) were obtained at the time of diagnosis. The mean age was 59.4 ± 16.6 years, with 181 (44%) being females. The mean PASP was 38 ± 12 mmHg in females vs. 33 ± 9 mmHg in males (p < 0.0001). PH as defined by a PASP value of > 36 mmHg was present in 140/414 (34%) patients, with a predominance in females [79/181 (44%)] vs. males [61/233 (26%), p < 0.0001]. Female sex, atrial fibrillation, diagnosis of congestive heart failure, and elevated filling pressures on echocardiogram remained significantly associated with PH (PASP > 36 mmHg) in multivariable modeling. PH, when present, was independently associated with mortality [hazard ratio 1.63, 95% CI (1.05-2.53), p = 0.028] and symptoms [odds ratio 2.28 (1.40, 3.71), p < 0.001].
UNASSIGNED: PH was present in 34% of patients with ApHCM at diagnosis, with female sex predominance. PH in ApHCM was associated with symptoms and increased mortality.
摘要：
■心尖肥厚型心肌病（ApHCM）是肥厚型心肌病（HCM）的一种亚型，影响多达25％的亚洲患者，在非亚洲患者中并未得到很好的理解。尽管ApHCM被认为是一种更“良性”的变体，它与心房和心室心律失常的风险增加有关，心尖血栓,中风,进行性心力衰竭.ApHCM中肺动脉高压（PH）的发生，由于心脏左侧的压力升高，已被记录在案。然而,ApHCM中PH的确切患病率和性别差异仍不确定.
■我们试图评估患病率，风险关联，单三级中心最大的ApHCM患者队列中肺压升高的性别差异。使用ICD代码和临床笔记搜索确定了总共542名诊断为ApHCM的患者。通过与通过自然语言处理提取的心脏MRI报告的交叉引用以及通过手动评估患者图表和成像记录来确认。
■在414名患者中，在诊断时获得肺动脉收缩压（PASP）的超声心动图测量值。平均年龄59.4±16.6岁,181人（44%）为女性。女性与女性的平均PASP为38±12mmHg。男性为33±9mmHg(p<0.0001)。由PASP值>36mmHg定义的PH在140/414（34％）患者中存在，以女性为主[79/181（44%）]与男性[61/233（26%），p<0.0001]。女性性别,心房颤动,充血性心力衰竭的诊断，在多变量模型中,超声心动图上的充盈压升高与PH(PASP>36mmHg)显著相关。PH,当存在时,与死亡率独立相关[风险比1.63，95%CI(1.05-2.53)，p=0.028]和症状[赔率比2.28（1.40，3.71），p<0.001]。
■34%的ApHCM患者在诊断时存在PH，女性占主导地位。ApHCM中的PH与症状和死亡率增加有关。
■我们试图评估患病率，风险关联，单三级中心最大的ApHCM患者队列中肺压升高的性别差异。使用ICD代码和临床笔记搜索确定了总共542名诊断为ApHCM的患者。通过与通过自然语言处理提取的心脏MRI报告的交叉引用以及通过手动评估患者图表和成像记录来确认。
■在414名患者中，在诊断时获得肺动脉收缩压（PASP）的超声心动图测量值。平均年龄59.4±16.6岁,181人（44%）为女性。女性与女性的平均PASP为38±12mmHg。男性为33±9mmHg(p<0.0001)。由PASP值>36mmHg定义的PH在140/414（34％）患者中存在，以女性为主[79/181（44%）]与男性[61/233（26%），p<0.0001]。女性性别,心房颤动,充血性心力衰竭的诊断，在多变量模型中,超声心动图上的充盈压升高与PH(PASP>36mmHg)显著相关。PH,当存在时,与死亡率独立相关[风险比1.63，95%CI(1.05-2.53)，p=0.028]和症状[赔率比2.28（1.40，3.71），p<0.001]。
■34%的ApHCM患者在诊断时存在PH，女性占主导地位。ApHCM中的PH与症状和死亡率增加有关。
