PULMOEAST: A Comprehensive Analysis of Pulmonary Hypertension in Eastern India

Abstract

Background Pulmonary hypertension (PH) is a debilitating cardiovascular disorder characterized by abnormally elevated blood pressure within the lungs. The diverse range of causes and varied clinical presentations contribute to the complexity of its diagnosis and management. In eastern India and surrounding areas, awareness of PH remains limited, and resources for its management are scarce. This study aims to address this knowledge gap by investigating clinical characteristics and treatment approaches adopted for PH patients in eastern India. Methods This retrospective-prospective cohort study included patients diagnosed with PH, defined by a pulmonary artery systolic pressure (PASP) > 50 mmHg or a mean pulmonary artery pressure (mPAP) >20 mmHg, between July 2015 and October 2023. Data retrieved from hospital records formed the retrospective cohort, while the prospective cohort comprised patients directly recruited for the study. Results The PULMOEAST study enrolled 93 patients with confirmed PH, divided into prospective (59 patients) and retrospective (34 patients) cohorts. The most prevalent cause of PH was congenital heart disease (CHD), with shunt lesions (59.13%), followed by complex CHD (13.97%) and idiopathic PH (20.43%). Six additional patients presented with rare causes of PH, and three experienced transient PH following atrial septal defect device closure. Geographic distribution revealed that 72.04% of patients originated from eastern India, while 18.27% hail from other eastern states and 8.6% from neighboring countries. Patients exhibited varying functional classes (FC), with 57 classified as FC-II and 31 classified as FC-III. Treatment strategies primarily involve supportive medications and pulmonary vasodilators. Monotherapy was administered to 26 patients (27.95%), dual therapy to 50 patients (53.76%), and triple therapy to one patient. Notably, 16 patients did not receive any vasodilator therapy as they were waiting for further evaluation. Among the vasodilator regimen, two patients received Selexipag. Three patients underwent intervention for shunt lesion closure, including one patient who received a fenestrated atrial septal occluder implant. Additionally, one patient underwent clot removal for pulmonary thromboembolism. Despite the overall positive response to treatment, the study recorded eight fatalities (8.6%) during the observation period. However, most patients exhibited significant improvement, including a decrease in functional class, during a mean follow-up duration of 14.31 months. Conclusion The PULMOEAST study undertook a comprehensive exploration of PH in eastern India and surrounding regions, revealing a stark dominance of CHD as the primary culprit. The study confirmed the pivotal role of echocardiography as a readily available and effective tool for both initial and follow-up evaluations in resource-scarce settings. It painted a hopeful picture by showcasing significant clinical improvement in most treated patients, with supportive medications and pulmonary vasodilators playing a crucial role. However, the diverse etiologies, limited access to PH-specific resources, and lack of widespread awareness within the region continue to pose substantial challenges for patients. The study underscores the need for refined diagnostic approaches, cost-effective management strategies, collaborative care initiatives, and enhanced patient education to optimize PH care and improve outcomes in eastern India.

Keywords: challenges; clinical profiles; collaborative care; comprehensive evaluation; eastern india; patient outcomes; pulmonary hypertension; treatment modalities.

Figure 1. Pulmonary artery thromboembolism

A: Continuous-wave Doppler revealing severe elevation of right ventricular systolic pressure, determined by the tricuspid regurgitation jet. B: Computed tomography pulmonary angiography demonstrating complete occlusion of the right pulmonary artery (yellow arrow). C: Chest X-ray (anterior-posterior view) exhibiting signs of differential vascularity and reduced right lung blood flow. D: A large thrombus recovered after removal. E: Chest X-ray (anterior-posterior view) post-thrombus removal, showing restored right lung blood flow.

Figure 2. Pulmonary vein obstruction

A: Chest X-ray (posterior-anterior view) indicating pulmonary venous congestion. B, C: Echocardiogram in four-chamber view showcasing dilated right heart chambers and obstruction in the left pulmonary vein by external tissue (green arrow). D: Continuous wave Doppler of the tricuspid regurgitation jet demonstrates elevated right ventricular systolic pressure. E, F: Parasternal short-axis view with color Doppler displaying obstructed pulmonary veins by soft tissue (white arrow). G: Doppler shows a significantly elevated mean gradient across the pulmonary vein. H: Computed tomography angiography depictis diffuse soft tissue (red arrow) in the mediastinum encasing the pulmonary veins, causing obstructions.

Figure 3. Secundum atrial septal defect closure with a fenestrated septal occluder

A: Chest X-ray (posterior-anterior view) illustrating cardiomegaly and a dilated main pulmonary artery. Evidence of increased pulmonary blood flow is observed. B: Transesophageal echocardiogram with color Doppler revealing a large secundum atrial septal defect (ASD) predominantly shunting left to right (white arrow). C: Continuous-wave Doppler of the tricuspid regurgitation jet demonstrating elevated right ventricular systolic pressure. D, E: Hemodynamic assessment before and after ASD device deployment with a custom-made fenestration in the fluoroscopic image (green arrow). F: Echocardiogram in parasternal short-axis view with color Doppler showing the ASD device. The fenestration is seen shunting left to right (yellow arrow).

Figure 4. Atrial flow regulator implantation

A-D: Fluoroscopic images illustrating the implantation sequence of atrial flow regulation implantation. A: Anteroposterior view showing septal puncture of the interatrial septum (white arrow). B: Oblique view after AFR deployment demonstrating fenestration (red arrow). C: Oblique view showing AFR deployment. D: post-release image showing stable device position. E-G: Follow-up echocardiogram displaying significant tricuspid regurgitation and elevated right ventricular systolic pressure by TR jet. The fenestration is flowing right to left, as seen in the modified parasternal view (G, yellow arrow).

Figure 5. Secundum atrial septal defect with left ventricular diastolic dysfunction and pulmonary hypertension

A: Chest X-ray (posterior-anterior view) showcasing significant cardiomegaly and a dilated main pulmonary artery. B, C: Transesophageal echocardiogram with color Doppler revealing a large secundum atrial septal defect (ASD) with bidirectional shunt (white arrow). D: Hemodynamic tracing demonstrating elevated pulmonary artery pressure. E: Balloon occlusion of ASD (red arrow) resulting in a substantial increase in left ventricular end-diastolic pressure (F).

Figure 6. Idiopathic dilatation of the pulmonary artery

A: Chest X-ray (posterior-anterior view) showing a cardiothoracic ratio of approximately 0.5 and a dilated main pulmonary artery. B: Echocardiogram in four-chamber view illustrating dominant left heart chambers. C: Parasternal short-axis view depicting dilated main and branch pulmonary arteries (red arrow). D: Continuous-wave Doppler of the tricuspid regurgitation jet showing mild elevation of right ventricular systolic pressure. E: Aneurysmally dilated pulmonary artery in the right ventricular angiogram and in computed tomography pulmonary angiography, two-dimensional. F: and three-dimensional reconstruction. G: is marked by red arrow.