Mehmood Muddassir, Agarwal Richa, Raina Amresh, Correa-Jaque Priscilla, Benza Raymond L
Cardiovascular Institute, Allegheny General Hospital, Pittsburgh, Pennsylvania, USA.
Pulm Circ. 2016 Dec;6(4):616-618. doi: 10.1086/688670.
Despite new therapeutic options, pulmonary arterial hypertension (PAH) remains a progressive disease associated with substantial morbidity and mortality. As such, additional strategies for monitoring and adjunctive management of this disease are important. A 59-year-old woman with scleroderma-associated PAH received an implantable hemodynamic monitor (IHM) as part of a research protocol at our institution. Pulmonary artery pressures, heart rate, and cardiac output (sensor-based algorithm) were measured on a daily basis, and parameters of right ventricular (RV) performance and afterload were calculated. At the time of IHM implant, the patient had functional class III symptoms, was receiving triple-drug therapy, and had normal hemoglobin levels. Four months after implant, and with further optimization of prostacyclin therapy, she had improvement in her symptoms. However, shortly thereafter, while the patient was receiving stable drug therapy, her case regressed with worsening symptoms, and the patient received a new diagnosis of iron deficiency anemia. Oral iron supplementation resulted in normalization of hemoglobin levels and improvement in the patient's iron profile. A gradual and sustained reduction in pulmonary pressures was noted after initiation of oral iron accompanied by increased RV performance and favorable reduction in RV afterload. The patient had significant symptomatic improvement. Iron deficiency is an underappreciated yet easily treatable risk factor in PAH. Use of IHM in this case longitudinally illustrates the optimization of pulmonary hemodynamics and RV afterload in tandem with clinical improvement achieved by a simple therapy.
尽管有了新的治疗选择,但肺动脉高压(PAH)仍然是一种进展性疾病,伴有大量的发病率和死亡率。因此,针对这种疾病的监测和辅助管理的额外策略很重要。一名患有硬皮病相关PAH的59岁女性作为我们机构一项研究方案的一部分接受了植入式血流动力学监测器(IHM)。每天测量肺动脉压力、心率和心输出量(基于传感器的算法),并计算右心室(RV)功能和后负荷的参数。在植入IHM时,患者有Ⅲ级功能症状,正在接受三联药物治疗,血红蛋白水平正常。植入四个月后,随着前列环素治疗的进一步优化,她的症状有所改善。然而,此后不久,在患者接受稳定药物治疗时,她的病情出现倒退,症状恶化,患者被新诊断为缺铁性贫血。口服铁剂补充使血红蛋白水平恢复正常,患者的铁指标得到改善。开始口服铁剂后,肺动脉压力逐渐持续下降,同时右心室功能增强,右心室后负荷得到有利降低。患者的症状有显著改善。缺铁是PAH中一个未得到充分认识但易于治疗的危险因素。在这个病例中使用IHM纵向说明了肺血流动力学和右心室后负荷的优化与通过简单治疗实现的临床改善是同步的。