Pulmonary hypertension (PH) has been described in myelofibrosis (MF), but it is rare and typically found in advanced disease. Although the etiology of PH in MF is unclear, early predictors may be detected by echocardiogram. The goals of our study were to evaluate the prevalence of PH as determined by echocardiography in a cohort of MF patients and to identify clinical risk factors for PH. We performed a retrospective review of MF patients from October 2015 to May 2017 at MD Anderson Cancer Center in the ambulatory clinic, and those with echocardiogram were included. Clinical, echocardiographic, and laboratory data were reviewed. Patients with and without PH were compared using a chi-square or Fisher's exact test, and logistic regression was performed with an outcome variable of PH. There were 143 patients with MF who underwent echocardiogram, and 20 (14%) had echocardiographic findings consistent with PH. Older age, male gender, hypertension, hyperlipidemia, coronary artery disease, dyspnea, hematocrit, brain natriuretic peptide (BNP), and N-terminal prohormone BNP (NT-proBNP) were significantly different between those without PH and those with PH (p < 0.05). Female gender was protective (OR 0.21, 95% CI 0.049-0.90, p = 0.035), and NT-proBNP was a significant clinical predictor of PH (OR 1.07, CI 1.02 = 1.12, p = 0.006). PH in MF is lower than previously reported in our MF cohort, but many patients had cardiac comorbidities. PH due to left-sided heart disease may be underestimated in MF. Evaluation of respiratory symptoms and elevated NT-proBNP should prompt a baseline echocardiogram. Early detection of PH with a multidisciplinary approach may allow treatment of reversible etiologies.

中文翻译：

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骨髓纤维化中肺动脉高压的患病率。


肺动脉高压 (PH) 已在骨髓纤维化 (MF) 中被描述，但这种情况很少见，通常见于晚期疾病。尽管 MF 中 PH 的病因尚不清楚，但早期预测因素可通过超声心动图检测到。我们研究的目的是评估一组 MF 患者中通过超声心动图确定的 PH 患病率，并确定 PH 的临床危险因素。我们对 2015 年 10 月至 2017 年 5 月在 MD 安德森癌症中心门诊的 MF 患者进行了回顾性评估，其中包括那些接受超声心动图检查的患者。回顾了临床、超声心动图和实验室数据。使用卡方或 Fisher 精确检验对患有和不患有 PH 的患者进行比较，并使用 PH 结果变量进行逻辑回归。 143 名 MF 患者接受了超声心动图检查，其中 20 名（14%）超声心动图结果与 PH 一致。年龄较大、男性、高血压、高脂血症、冠状动脉疾病、呼吸困难、血细胞比容、脑钠肽 (BNP) 和 N 末端激素原 BNP (NT-proBNP) 在无 PH 患者和有 PH 患者之间存在显着差异 (p % 3C 0.05）。女性具有保护性（OR 0.21，95% CI 0.049-0.90，p = 0.035），NT-proBNP 是 PH 的重要临床预测因子（OR 1.07，CI 1.02 = 1.12，p = 0.006）。 MF 的 PH 低于我们 MF 队列之前报道的，但许多患者患有心脏合并症。 MF 中由于左心疾病引起的 PH 可能被低估。对呼吸道症状和 NT-proBNP 升高的评估应提示进行基线超声心动图检查。采用多学科方法早期发现 PH 可能有助于治疗可逆性病因。