Greenberg Cardiology Division, Department of Medicine, Weill Cornell Medical College, New York, New York.
Richard T. Silver Myeloproliferative Neoplasm Center, Hematology and Oncology, Department of Medicine, Weill Cornell Medical College, New York, New York.
J Am Soc Echocardiogr. 2019 Dec;32(12):1574-1585. doi: 10.1016/j.echo.2019.07.010. Epub 2019 Oct 3.
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.
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.
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.
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.
基于小型观察性研究，骨髓增殖性肿瘤（MPN）与肺动脉高压（PH）有关，但 MPN 中 PH 的机制和临床意义尚不清楚。本研究的目的是通过研究与 PH 相关的症状、死亡率以及 PH 对心脏重塑的影响，使用定量超声心动图方法来扩展对 MPN 患者中 PH 的认识。
该人群包括接受经胸超声心动图检查的 MPN 患者的回顾性队列研究：多普勒衍生的肺动脉收缩压应用既定的 PH （≥35mmHg）和晚期 PH （≥50mmHg）截断值；通过常规指标（三尖瓣环平面收缩期位移[TAPSE]、S'）和整体纵向应变评估右心室（RV）功能。通过标准化回顾评估症状和死亡率。
研究了 301 例患者；56%的患者经超声心动图证实存在 PH（20%为晚期），这与通过侵入性定量肺动脉收缩压检测到的 PH 发生率（67%）相似。PH 与不良的左心室（LV）重塑指标相关，包括心肌质量增加和舒张功能障碍（所有 P 值均<.001）：LV 质量和充盈压（P<.01）与 PH 相关，与 LV 射血分数无关。应变和 TAPSE 以及 S'的 RV 功能障碍随着 PH 的增加而增加（P≤.001），并且在晚期 PH 患者中比无 PH 患者大约高三倍。有 RV 功能障碍的患者更有可能报告呼吸困难，晚期 PH 患者也是如此（P<.05）。在中位 2.2 年的随访期间，全因死亡率为 27%。PH 分级（危险比，1.9；95%置信区间，1.1-3.0；P=.012）和 TAPSE 和 S' 显示的 RV 功能障碍（危险比，3.3；95%置信区间，1.3-8.2；P=.01）与死亡率独立相关；用整体纵向应变替代 TAPSE 和 S'，RV 功能障碍与死亡的相关性相似（危险比，3.2；95%置信区间，1.5-6.7；P=.003），与 PH 无关。
PH 在 MPN 患者中非常普遍，与 LV 舒张功能障碍有关；超声心动图定量 RV 功能障碍增加了死亡率的风险，与 PH 无关。
