Challenging in pulmonary thromboembolism diagnosis in patients with disproportionate pulmonary hypertension and severe mitral stenosis: Report of two cases.

KEY CLINICAL MESSAGE: Before valvular interventions, echocardiography, especially the TEE or the ventilation/perfusion scan, should be performed to detect silent PTE and set a more accurate treatment and surgical plan.

ABSTRACT: Pulmonary hypertension (PH) is a progressive and critical disease that can be caused by mitral stenosis (MS). Some of these patients present with disproportionate PH, which is an uncommon phenomenon and is considered a challenging diagnostic and treatment process. In these patients, other causes may also play a role in developing PH. This report presented two cases with disproportionate PH and severe MS who were scheduled for percutaneous mitral valvuloplasty (PMV). The pre-procedural echocardiography revealed systolic pulmonary artery pressure (sPAP) of 90 and 120 mmHg, mitral valve area of 0.80 and 0.55 cm2 by three-dimensional (3D) planimetry, and diastolic pressure gradient (DPG) of 13 and 18.8 mmHg, respectively. Furthermore, in the first patient, 3D transesophageal echocardiography (TEE) revealed multiple saddle-type organized thrombi in the proximal parts of the right and left pulmonary arteries, extending to the distal branches. In the second patient, 3D TEE revealed a large, relatively fresh, flow-limiting thrombosis in the proximal part of the right pulmonary artery. The diagnosis of pulmonary thromboembolism (PTE) in both patients was confirmed by CT angiography. In both patients, the valves were surgically repaired, while all thrombi were removed from the cardiac chambers and pulmonary vessels during surgery. In addition, patients underwent warfarin therapy orally. They were followed up 6 months after the intervention, and their clinical symptoms had improved significantly.