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Pulmonary Artery Pressure Sensor-Guided Therapy for Cardiac AL Amyloidosis in a Young Multiple Myeloma Patient
Robbe Knapen1, Koen Theunissen2, Olivier Ghekiere3
1Department of Cardiology, Jessa Hospital, Hasselt, Belgium.
Background:
Volume assessment in cardiac amyloid light chain (AL) amyloidosis is challenging during high-dose chemotherapy and autologous stem cell transplantation (SCT), as clinical findings and body weight may not accurately reflect intravascular congestion.
Case Summary:
A 45-year-old man with multiple myeloma, nephrotic syndrome, and cardiac AL amyloidosis developed marked volume fluctuations during induction therapy. Before SCT, an implantable pulmonary artery pressure (PAP) sensor was placed to guide diuretics, targeting a diastolic PAP of 22 mm Hg. During the peritransplant period, remote PAP monitoring enabled individualized diuretic titration despite mucositis, gastrointestinal losses, transfusions, neutropenic fever, delirium, and weight variation. He completed SCT without heart failure decompensation and achieved complete hematologic remission.
Discussion:
This case highlights the value of PAP-guided management in complex cardio-oncology patients with dynamic hemodynamics.
Take-Home Messages:
In cardiac AL amyloidosis, invasive hemodynamic monitoring may outperform clinical assessment. PAP-guided management may be useful during SCT when risks of congestion and hypovolemia coexist.
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