BACKGROUND Myeloproliferative neoplasm (MPN) has been associated with pulmonary hypertension (PH) on the basis of small observational studies, but the mechanism and clinical significance of PH in MPN are not well established. The aims of this study were to expand understanding of PH in a well-characterized MPN cohort via study of PH-related symptoms, mortality risk, and cardiac remodeling sequalae of PH using quantitative echocardiographic methods. METHODS The population comprised a retrospective cohort of patients with MPN who underwent transthoracic echocardiography: Doppler-derived pulmonary arterial systolic pressure applied established cutoffs for PH (≥35 mm Hg) and advanced PH (≥50 mm Hg); right ventricular (RV) performance was assessed via conventional indices (tricuspid annular plane systolic excursion [TAPSE], S') and global longitudinal strain. Symptoms and mortality were discerned via standardized review. RESULTS Three hundred one patients were studied; 56% had echocardiography-demonstrated PH (20% advanced) paralleling a high prevalence (67%) among patients with invasively quantified PASP. PH was associated with adverse left ventricular (LV) remodeling indices, including increased myocardial mass and diastolic dysfunction (P ≤ .001 for all): LV mass and filling pressure (P < .01) were associated with PH independent of LV ejection fraction. RV dysfunction by strain and TAPSE and S' increased in relation to PH (P ≤ .001) and was about threefold greater among patients with advanced PH compared with those without PH. Patients with RV dysfunction were more likely to report dyspnea, as were those with advanced PH (P < .05). During median follow-up of 2.2 years, all-cause mortality was 27%. PH grade (hazard ratio, 1.9; 95% CI, 1.1-3.0; P = .012) and TAPSE- and S'-demonstrated RV dysfunction (hazard ratio, 3.3; 95% CI, 1.3-8.2; P = .01) were independently associated with mortality; substitution of global longitudinal strain for TAPSE and S' yielded similar associations of RV dysfunction with death (hazard ratio, 3.2; 95% CI, 1.5-6.7; P = .003) independent of PH. CONCLUSIONS PH is highly prevalent in patients with MPN and is linked to LV diastolic dysfunction; echocardiography-quantified RV dysfunction augments risk for mortality independent of PH.

中文翻译：

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骨髓增生性肿瘤相关性肺动脉高压患者右心室功能障碍的递增效用。

背景技术在小规模的观察研究的基础上，骨髓增生性肿瘤（MPN）与肺动脉高压（PH）相关，但尚未明确建立MPN中PH的机制和临床意义。这项研究的目的是通过使用定量超声心动图方法研究与PH有关的症状，死亡风险和对PH的心脏重塑性异性进行研究，以扩大在特征明确的MPN队列中对PH的了解。方法该人群包括接受经胸超声心动图检查的MPN患者的回顾性队列：多普勒血流的肺动脉收缩压设定为PH（≥35mm Hg）和晚期PH（≥50 mm Hg）的临界值；通过常规指标（三尖瓣环平面收缩期偏移[TAPSE]，S' ）和整体纵向应变。症状和死亡率可通过标准化审查来识别。结果对301例患者进行了研究。在有创定量PASP患者中，有56％的患者超声心动图显示的PH（进展为20％）与高患病率（67％）平行。PH与不良的左心室（LV）重塑指数相关，包括增加的心肌质量和舒张功能障碍（所有P≤.001）：LV质量和充盈压（P <.01）与PH无关，与LV射血分数无关。与PH相比，菌株和TAPSE和S'引起的RV功能障碍增加（P≤.001），晚期PH患者的RV功能障碍比无PH的患者高大约三倍。RV功能障碍的患者和晚期PH的患者更有可能报告呼吸困难（P <.05）。在2.2年的中位随访期间，全因死亡率为27％。PH等级（危险比，1.9； 95％CI，1.1-3.0； P = 0.012）以及以TAPSE和S'表现的RV功能障碍（危险比，3.3； 95％CI，1.3-8.2； P = 0.01）与死亡率独立相关；用全球纵向应变代替TAPSE和S'可以得到与RV无关的RV功能障碍与死亡的相似关联（危险比，3.2； 95％CI，1.5-6.7； P = 0.003）。结论PH在MPN患者中高度流行，并与LV舒张功能障碍有关。超声心动图量化的RV功能障碍增加了独立于PH的死亡风险。01）与死亡率独立相关；用全球纵向应变代替TAPSE和S'可以得到与RV无关的RV功能障碍与死亡的相似关联（危险比，3.2； 95％CI，1.5-6.7； P = 0.003）。结论PH在MPN患者中高度流行，并与LV舒张功能障碍有关。超声心动图量化的RV功能障碍增加了独立于PH的死亡风险。01）与死亡率独立相关；总体纵向应变替换为TAPSE和S'导致RV功能障碍与死亡的相似关联（危险比，3.2； 95％CI，1.5-6.7； P = 0.003），与PH无关。结论PH在MPN患者中高度流行，并与LV舒张功能障碍有关。超声心动图量化的RV功能障碍增加了独立于PH的死亡风险。