Hirakawa Kyoko, Aoki Tatsuo, Tsuji Akihiro, Ogo Takeshi
Division of Pulmonary Circulation, Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Centre, 6-1, Kishibe-Shimmachi, Suita, Osaka 564-8565, Japan.
Department of Cardiovascular Medicine, Kumamoto University, Kumamoto 860-8556, Japan.
Eur Heart J Case Rep. 2022 Aug 22;6(9):ytac351. doi: 10.1093/ehjcr/ytac351. eCollection 2022 Sep.
Calcium channel blockers (CCB), the first accepted treatment, is effective only in a small number of idiopathic pulmonary arterial hypertension (I-PAH) patients with vasoreactivity [these patients are identified by a positive acute pulmonary vasoreactive test (AVT) response]. While the majority of I-PAH patients is non-vasoreactive and CCB non-responders, modern advanced pulmonary hypertension (PH)-specific therapies, which act on one of the three different mechanistic pathways-endothelin, nitric oxide (NO), and prostacyclin pathways, are effective. Treatment response to advanced PH-specific vasodilators in PAH patients with vasoreactivity is unknown.
A 30-year-old woman with I-PAH was referred to our centre with worsening symptoms and deteriorating PH. She was being administered oral triple combination of advanced PH-specific treatment including a phosphodiesterase-5 inhibitor, an endothelin receptor antagonist, and a long-acting prostacyclin analogue. The patient showed positive AVT with NO inhalation while on these advanced PH-specific drugs. We added high-dose CCB, which dramatically normalized her pulmonary blood pressure without further symptoms, and she has remained stable for 5 years.
Our case describes a PAH patient with vasoreactivity, who was resistant to three different types of advanced PH-specific vasodilators but was exclusively sensitive to CCB treatment. Some CCB responders may have a specific CCB-sensitive PAH phenotype refractory to other pulmonary vasodilators. This case highlights the role of identifying CCB responders in this era of use of modern, advanced PH-specific vasodilators. The investigation of the mechanisms underlying CCB sensitivity in PAH is necessary.
钙通道阻滞剂（CCB）作为首个被认可的治疗药物，仅对少数具有血管反应性的特发性肺动脉高压（I-PAH）患者有效[这些患者通过急性肺血管反应性试验（AVT）阳性反应得以识别]。虽然大多数I-PAH患者无血管反应性且对CCB无反应，但作用于三种不同机制途径之一——内皮素、一氧化氮（NO）和前列环素途径的现代先进肺动脉高压（PH）特异性疗法是有效的。PAH血管反应性患者对先进的PH特异性血管扩张剂的治疗反应尚不清楚。
一名30岁的I-PAH女性患者因症状加重和PH恶化被转诊至我们中心。她正在接受包括磷酸二酯酶-5抑制剂、内皮素受体拮抗剂和长效前列环素类似物在内的先进PH特异性三联口服联合治疗。在使用这些先进的PH特异性药物期间，该患者吸入NO时AVT呈阳性。我们加用了高剂量CCB，这使她的肺动脉压显著恢复正常且无进一步症状，并且她已保持稳定5年。
我们的病例描述了一名具有血管反应性的PAH患者，该患者对三种不同类型的先进PH特异性血管扩张剂耐药，但仅对CCB治疗敏感。一些CCB反应者可能具有对其他肺血管扩张剂难治的特定CCB敏感PAH表型。该病例凸显了在现代先进的PH特异性血管扩张剂使用时代识别CCB反应者的作用。有必要对PAH中CCB敏感性的潜在机制进行研究。
