Challenges in pulmonary hypertension: managing the unexpected

Abstract

The diverse challenges associated with diagnosis and management of patients with pulmonary hypertension are illustrated in this case-based review. Case 1 describes a patient diagnosed with pulmonary arterial hypertension (PAH) with right heart failure and active systemic lupus erythematosus who was effectively treated with an up-front triple combination of PAH therapies and immunosuppressive therapy. In case 2, a diagnosis of pulmonary veno-occlusive disease was reached after a combined approach of clinical suspicion, physical examination, and invasive and noninvasive tests. Cautious PAH therapy and high-dose diuretics provided clinical benefit in this patient and served as a bridge to lung transplantation. These cases highlight the need for ongoing follow-up of patients with PAH, comprising frequent assessment of treatment success and continued diagnostic evaluation.

FIGURE 1

a–c) Chest computed tomography excluding acute lung embolism and parenchymal lung disease and d–g) echocardiography performed during diagnostic work-up in a patient with pulmonary arterial hypertension with right heart failure and active systemic lupus erythematosus (case study 1). d) Elevated tricuspid regurgitant jet velocity; e) reduced TAPSE; f) dilated VCI. TAPSE: tricuspid annular plane systolic excursion; VCI: vena cava inferior.

FIGURE 2

High-resolution computed tomography scans during diagnostic evaluation in a patient with pulmonary veno-occlusive disease, which had been previously diagnosed as idiopathic pulmonary arterial hypertension, showing no evidence of parenchymal disease in a, b) two different slices and c, d) illustrating diffuse ground-glass opacities with thickening interlobular septa (case study 2).