Akagi Satoshi, Kasahara Shingo, Sarashina Toshihiro, Nakamura Kazufumi, Ito Hiroshi
Department of Cardiovascular Medicine, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, Japan.
Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, Okayama, Japan.
Eur Heart J Case Rep. 2018 Mar 22;2(2):yty033. doi: 10.1093/ehjcr/yty033. eCollection 2018 Jun.
Recent advances in pulmonary arterial hypertension (PAH)-specific drugs have dramatically changed the therapeutic strategy for PAH. A strategy that includes 'treatment' with PAH-specific drugs initially and then 'repair' by closure of the cardiac defect (i.e. 'treat and repair') was devised, and has been attempted, in patients with PAH associated with a cardiac defect.
We present three cases of severe PAH associated with a ventricular septal defect (VSD) in adult patients who were initially treated with PAH-specific drugs followed by VSD closure. Two of the patients were treated with a combination of an endothelin receptor antagonist (ERA), phosphodiesterase type 5 inhibitor, and intravenous prostacyclin before VSD closure. The third patient was treated with an ERA and pulmonary artery banding before VSD closure. After 12 months of anti-PAH treatment, the pulmonary vascular resistance index and the ratio of the pulmonary vascular index to the systemic vascular resistance index decreased to levels that allowed VSD closure. At the mid- and long-term follow-up measurements after surgical closure of the VSD, the mean pulmonary artery pressure had markedly decreased.
Our case series suggests that the treat-and-repair strategy is a promising approach for adult patients with severe PAH associated with VSD.
肺动脉高压（PAH）特异性药物的最新进展极大地改变了PAH的治疗策略。对于合并心脏缺损的PAH患者，已制定并尝试了一种策略，即最初使用PAH特异性药物“治疗”，然后通过闭合心脏缺损进行“修复”（即“治疗并修复”）。
我们报告了3例成年患者，他们患有与室间隔缺损（VSD）相关的严重PAH，最初接受PAH特异性药物治疗，随后进行VSD闭合术。其中2例患者在VSD闭合术前接受了内皮素受体拮抗剂（ERA）、5型磷酸二酯酶抑制剂和静脉注射前列环素联合治疗。第3例患者在VSD闭合术前接受了ERA和肺动脉环扎术治疗。经过12个月的抗PAH治疗后，肺血管阻力指数以及肺血管指数与体循环血管阻力指数之比降至允许进行VSD闭合的水平。在VSD手术闭合后的中长期随访测量中，平均肺动脉压显著降低。
我们的病例系列表明，“治疗并修复”策略对于患有与VSD相关的严重PAH的成年患者是一种有前景的方法。
