Katz Michael G, Ohad Dan G, Putter Philip, Shtraizent Nataly, Shahar Ehud, Tal Smadar, Eliyahu Efrat
Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, United States.
Department of Pediatric Cardiac Surgery, Icahn School of Medicine at Mount Sinai, New York, NY, United States.
Front Vet Sci. 2024 Sep 23;11:1415030. doi: 10.3389/fvets.2024.1415030. eCollection 2024.
Myxomatous mitral valve disease (MMVD) stands out as the most prevalent acquired canine heart disease. Its occurrence can reach up to 40% in small breed dogs and escalates in geriatric canine populations. MMVD leads to thickening and incomplete coaptation of valve leaflets during systole, resulting in secondary mitral valve regurgitation. Serious complications may arise concurrently with the worsening of mitral valve regurgitation, including left-and right-sided congestive heart failure, and pulmonary hypertension (PH). Ultimately, the PH progression might contribute to the patient's demise or to the owner's decision of euthanasia. Most currently available FDA-approved therapies for PH are costly and aim to address the imbalance between vasoconstriction and vasodilation to restore endothelial cell function. However, none of these medications impact the molecular dysfunction of cells or impede the advancement of pulmonary vascular and right ventricular remodeling. Recent evidence has showcased successful gene therapy approaches in laboratory animal models of PH. In this manuscript, we summarize the latest advancements in gene therapy for the treatment of PH in animals. The manuscript incorporates original data showcasing sample presentations, along with non-invasive hemodynamic assessments. Our findings demonstrate that the use of metabolic gene therapy, combining synthetic adeno-associated virus with acid ceramidase, has the potential to significantly reduce the need for drug treatment and improve spontaneously occurring PH in dogs.
黏液瘤性二尖瓣疾病(MMVD)是最常见的获得性犬类心脏病。在小型犬中,其发病率可达40%,在老年犬群体中发病率更高。MMVD会导致瓣膜小叶在收缩期增厚且不能完全对合,从而引起继发性二尖瓣反流。二尖瓣反流加重时可能会同时出现严重并发症,包括左、右心充血性心力衰竭和肺动脉高压(PH)。最终,PH的进展可能导致病犬死亡或促使主人决定实施安乐死。目前美国食品药品监督管理局(FDA)批准的大多数用于治疗PH的疗法成本高昂,旨在解决血管收缩与舒张之间的失衡以恢复内皮细胞功能。然而,这些药物均无法影响细胞的分子功能障碍,也无法阻止肺血管和右心室重塑的进展。最近的证据表明,在PH的实验动物模型中基因治疗方法取得了成功。在本手稿中,我们总结了动物PH治疗中基因治疗的最新进展。该手稿纳入了展示样本呈现的原始数据以及非侵入性血流动力学评估。我们的研究结果表明,将合成腺相关病毒与酸性神经酰胺酶相结合的代谢基因治疗,有可能显著减少药物治疗的需求,并改善犬类自发性PH。