Pulmonary Hypertension: Expert Didactics and Discussion (Master Class)-Case Studies

Video Transcription

Video Summary

This presentation focused on evaluating and managing pulmonary arterial hypertension (PAH) and its mimics through case discussions, highlighting diagnostic challenges and treatment considerations. Key points include recognizing that an unmeasurable tricuspid regurgitant jet does not rule out significant pulmonary hypertension, emphasizing the fallibility of wedge pressure measurements in right heart catheterization, and the value of closely inspecting raw data (echocardiograms, hemodynamic tracings) beyond summary reports.<br /><br />A 26-year-old woman with exercise intolerance and RV dilation but normal tricuspid regurgitation velocity illustrated the importance of detailed echocardiographic assessment and repeat invasive testing to confirm diagnosis. Genetic testing revealed a BMPR2 mutation. Her management evolved from oral drugs to intravenous prostacyclin due to disease progression, illustrating challenges in timing and patient acceptance of aggressive therapy.<br /><br />An 84-year-old woman initially misdiagnosed with PAH actually had Group 2 pulmonary hypertension from left heart disease, underscoring the "zone of uncertainty" in wedge pressure interpretation, the usefulness of volume challenges during catheterization, and integrating echo findings for accurate classification.<br /><br />A 77-year-old woman with chronic thromboembolic pulmonary hypertension (CTEPH) benefitted from balloon pulmonary angioplasty after declining surgery, demonstrating advances in revascularization options and the importance of specialized evaluation to select optimal intervention.<br /><br />A 30-year-old pregnant woman with lupus and abrupt severe symptoms was found to have large-vessel pulmonary vasculitis rather than typical PAH, highlighting the need to consider alternative diagnoses and the utility of PET imaging and immunosuppressive therapy.<br /><br />Discussion covered the complexity of risk assessment, monitoring, and treatment escalation in PAH, including bridging strategies using prostacyclins and mechanical support. ICU management considerations emphasized cautious patient selection for intubation and preferred induction agents (e.g., ketamine) due to hemodynamic fragility.<br /><br />Overall, the cases reinforced that pulmonary hypertension diagnosis requires careful synthesis of clinical, imaging, and invasive data, with thoughtful individualized management tailored to evolving patient status and underlying etiology.

Keywords

pulmonary arterial hypertension

PAH mimics

tricuspid regurgitant jet

wedge pressure measurement

right heart catheterization

echocardiographic assessment

BMPR2 mutation

intravenous prostacyclin therapy

Group 2 pulmonary hypertension

volume challenge

chronic thromboembolic pulmonary hypertension

balloon pulmonary angioplasty

pulmonary vasculitis