Pulmonary Arterial Hypertension in Indonesia: Current Status and Local Application of International Guidelines

Abstract

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.

Key highlights: 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.

Keywords: Congenital heart disease (CHD); Indonesia; Pulmonary arterial hypertension (PAH); etiology; international guideline; lower middle-income region.

Figure 1

Diagnostic algorithm and different tests recommended for pulmonary arterial hypertension according to 2015 ESC/ERS guidelines and updated clinical classification of pulmonary hypertension. Adapted from: Simonneau G et al. [8], Galiè N et al. [12], and Desai AA et al. [13]. PH: Pulmonary hypertension; PAH: Pulmonary arterial hypertension; mPAP: Mean pulmonary arterial pressure; mPAWP: Pulmonary artery wedge pressure; PVR: Pulmonary vascular resistance; WU: Woods unit; IPAH: Idiopathic pulmonary arterial hypertension; HPAH: Heritable pulmonary arterial hypertension; ECG: Electrocardiogram; TTE: Transthoracic echocardiography; TOE: Transesophageal echocardiography; RHC: Right heart catheterization; PF: Pulmonary function; BGA: Blood gas analysis.

Figure 2

Results from COHARD-PH registry (2012–2019) in Indonesia in all registered patients (n = 1012) [7]. a) Proportion of probability of PH by echocardiography; b) Distribution by gender; c) Clinical symptoms; d) Congenital anomalies in CHD-associated PAH. CHD: Congenital heart disease; PAH: Pulmonary arterial hypertension; COHARD-PH: Congenital heart disease in adult and pulmonary hypertension; ASD: Atrial septal defect; VSD: Ventricle septal defect; PDA: Patent ductus arteriosus.

Figure 3

Treatment algorithm for pulmonary arterial hypertension according to 2015 ESC/ERS guidelines. Adapted from: Galiè N, et al. [12], McLaughlin VV et al. [21], and Taichman DB et al. [23] PAH: Pulmonary arterial hypertension; CCB: Calcium-channel blocker; IPAH: Idiopathic pulmonary arterial hypertension; HPAH: Heritable pulmonary arterial hypertension; DPAH: Drug-induced pulmonary arterial hypertension; ET-RA: Endothelin receptor antagonist; PDE-5i: Phosphodiesterase type 5 inhibitor; PCA: Prostacyclin analog; OC: Oral combination.