Challenges and Strategies in Managing Pulmonary Arterial Hypertension Within the Indian Healthcare System: A Consensus Statement From the Pulmonary Vascular Research Institute India Taskforce

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  Figure 1. Clinical manifestations of pulmonary arterial hypertension (PAH). The range of symptoms and signs in PAH includes early symptoms like exertional dyspnea, fatigue, palpitations, and syncope. As the disease progresses, patients may show abdominal distension, fluid retention, and hemoptysis. Examination may reveal an accentuated pulmonary component of the second heart sound (P2), murmurs of tricuspid regurgitation (TR) or pulmonary regurgitation (PR), cyanosis, and indications of right ventricular (RV) failure. Certain findings can point to underlying causes such as congenital heart disease (CHD), connective tissue disease (CTD), or chronic thromboembolic pulmonary hypertension (CTEPH).

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  Figure 2. Stepwise diagnostic algorithm for suspected pulmonary PAH. Patients presenting with unexplained dyspnea, syncope, or edema should undergo a structured evaluation. This includes taking a clinical history, assessing WHO functional class (WHO‐FC), performing a physical examination, obtaining a chest X‐ray and ECG, and measuring B‐type natriuretic peptide (BNP) or N‐terminal pro‐BNP (NT‐proBNP). Further assessment involves detailed echocardiography, focusing on tricuspid regurgitation velocity (TRV) and right heart function, as well as pulmonary evaluations such as pulmonary function tests (PFT), diffusion capacity (DLCO), arterial blood gases (ABG), and high‐resolution computed tomography (HRCT) to estimate the likelihood of PAH. Patients with intermediate or high probability of PAH should then undergo right heart catheterization (RHC). If CTEPH is suspected, a ventilation–perfusion (V/Q) scan or computed tomography pulmonary angiography (CTPA) is recommended.

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  Figure 3. Chest radiograph and electrocardiographic findings in PAH. Representative posteroanterior chest radiograph (A) shows cardiomegaly with right ventricular enlargement and prominent central pulmonary arteries, and lateral chest radiograph (B) shows reduction of the retrosternal air space, a sign of right ventricular enlargement. Electrocardiogram (ECG) (C) shows p pulmonale (p > 0.25 mV in lead II, blue arrow), right axis deviation (QRS axis > 90°), features of right ventricular hypertrophy (R/S > 1, with R > 0.5 mV in V1, R in V1 + S in V5 > 1 mV, green arrows), incomplete right bundle branch block (red arrow), RV strain pattern (ST depression and T wave in version in right precordial leads V1–V4 and II, III, aVF, yellow arrows) and prolonged QT interval corrected for heart rate. These findings support the clinical suspicion of PH but lack sufficient sensitivity and specificity for diagnosis. Imaging and ECG abnormalities should be interpreted alongside echocardiographic and hemodynamic assessment. Lung perfusion scintigraphy in a patient with PAH demonstrating relatively homogeneous bilateral tracer distribution with mild diffuse heterogeneity (green arrows), without segmental or…

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  Figure 4. Risk‐stratification guided treatment algorithm for PAH in India. Management pathway for confirmed PAH based on an initial three‐strata risk assessment. Low‐ or intermediate‐risk patients receive upfront dual oral therapy with a phosphodiesterase‐5 inhibitor (PDE5i) and an endothelin receptor antagonist (ERA). High‐risk patients are considered for initial triple therapy that includes a prostacyclin pathway agent. In conditions where parenteral or inhaled prostacyclin analogues are not available, triple combination therapy including Selexipag or sotatercept could be considered. Follow‐up assessment using a four‐strata model guides escalation. Patients who do not achieve low‐risk status may require the addition of prostacyclin pathway agents or sotatercept. Refractory cases should be evaluated for lung transplantation, heart–lung transplantation, atrial septostomy, or Potts shunt.