Department of Cardiology and Vascular Medicine, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada-Dr. Sardjito Hospital, Yogyakarta, ID.
Department Cardiology and Vascular Medicine, Faculty of Medicine, Universitas Indonesia, National Cardiovascular Center Harapan Kita Jakarta, ID.
Glob Heart. 2021 Apr 20;16(1):23. doi: 10.5334/gh.944.
Recent advances in the diagnosis and management of pulmonary arterial hypertension (PAH) have led to a significant improvement in the outcomes for patients with PAH. However, prompt and accurate diagnosis of PAH remains an unmet challenge due to lack of awareness and lack of meticulous data to profile the etiology and pathophysiology of this rare progressive disease, especially in low- and middle-income country. In Indonesia, the true prevalence and incidence of different subtypes of PAH in general population is still unknown. The Congenital HeARt Disease in adult and Pulmonary Hypertension (COHARD-PH) registry was the first single-center prospective registry in Indonesia, which indicated that almost 80% of adult patients with congenital heart disease (CHD) had experienced PAH and even Eisenmenger syndrome due to delayed diagnosis. Screening for early detection of asymptomatic CHD in children is yet to be systematically established in Indonesia, leading to undiagnosed and uncorrected CHD in adulthood. There are no specific national guidelines focusing on diagnostic workup and treatment of PAH in Indonesia. Furthermore, the lack of adequate diagnostic facilities, limited treatment availability, and limited drug coverage under the National Health Insurance Scheme are key issues that remain unaddressed. This review focuses on the diagnosis, treatment, and management of PAH associated with CHD in Indonesia as per international guidelines. We have proposed recommendations to effectively control and prevent PAH associated with CHD in Indonesia. The paper should be of interest to readers in the area of medical management and policy makers especially in low- and middle-income countries.
Pulmonary arterial hypertension (PAH) is a rare progressive subtype of pulmonary hypertension with poor overall prognosis and outcomes.Prompt and accurate diagnosis of PAH remains an unmet challenge in low- and middle-income countries due to poor knowledge about the etiology and pathophysiology of this syndrome. Also, the symptoms and signs of early-stage PAH are usually nonspecific or undetectable in newborn and infants, thus presenting a challenge for physicians to establish early diagnoses of PAH.The challenging factors in low- and middle-income countries, especially Indonesia archipelago are limitations of healthcare infrastructure, limited expertise, lack of awareness, lack of timely PAH screening strategies, poor antenatal care and unpredictable availability of PAH medications.There are no specific national guidelines focusing on diagnostic workup and treatment of PAH in Indonesia. Under-utilization of treatment guidelines and lack of adequate diagnostic treatment facilities have resulted in sub-optimal management of PAH patients in Indonesia.Adherence to international guidelines is an important aspect of PAH management in Indonesia. Updated disease and functional classifications of PAH as per international guidelines along with new research findings on prognostic factors can help in making better management decisions for PAH patients at different stages of the disease.
肺动脉高压（PAH）的诊断和治疗的最新进展显著改善了 PAH 患者的预后。然而，由于缺乏认识和缺乏详细的数据来描绘这种罕见的进行性疾病的病因和病理生理学，PAH 的及时准确诊断仍然是一个未满足的挑战，尤其是在中低收入国家。在印度尼西亚，一般人群中不同类型 PAH 的真实患病率和发病率仍然未知。先天性心脏病成人和肺动脉高压（COHARD-PH）登记处是印度尼西亚第一个单中心前瞻性登记处，该登记处表明，由于诊断延误，近 80%的先天性心脏病（CHD）成年患者已经经历了 PAH 甚至艾森曼格综合征。在印度尼西亚，还没有系统地建立筛查儿童无症状 CHD 的方案，导致成年时未诊断和未经纠正的 CHD。印度尼西亚没有专门针对 PAH 的诊断和治疗的国家指南。此外，缺乏足够的诊断设施、有限的治疗选择以及国家健康保险计划下的有限药物覆盖范围，这些都是尚未解决的关键问题。本综述根据国际指南重点介绍了印度尼西亚与 CHD 相关的 PAH 的诊断、治疗和管理。我们提出了建议，以有效控制和预防印度尼西亚与 CHD 相关的 PAH。本文应引起医疗管理和决策者，特别是中低收入国家决策者的关注。
肺动脉高压（PAH）是一种罕见的进行性肺动脉高压亚型，整体预后和结局较差。由于对这种综合征的病因和病理生理学缺乏了解，中低收入国家对 PAH 的及时准确诊断仍然是一个未满足的挑战。此外，PAH 的早期症状和体征在新生儿和婴儿中通常不明显或无法检测，因此医生难以早期诊断 PAH。中低收入国家，特别是印度尼西亚群岛的挑战因素包括医疗保健基础设施的限制、专业知识的缺乏、意识的缺乏、缺乏及时的 PAH 筛查策略、产前护理不佳以及 PAH 药物的不可预测可用性。印度尼西亚没有专门针对 PAH 的诊断和治疗的国家指南。治疗指南的利用不足和缺乏足够的诊断治疗设施导致印度尼西亚 PAH 患者的管理不佳。遵守国际指南是印度尼西亚 PAH 管理的一个重要方面。根据国际指南更新 PAH 的疾病和功能分类以及关于预后因素的新研究结果，可以帮助对不同疾病阶段的 PAH 患者做出更好的管理决策。
