Table 2.
Treatment course and myeloma disease activity for each patient
| Patient, event | Disease course and symptoms | Treatment course | IgA (units) | FLC ratio |
|---|---|---|---|---|
| Patient 1 | ||||
| 1 | MM diagnosed | Multiple cycles of lenalidomide-dexamethasone; remission achieved; received HSCT 1 year after remission; continued maintenance lenalidomide | … | … |
| 2 | PH diagnosed 3 years after HSCT; FC III symptoms; RHC (RA = 4 mmHg; PAP = 96/32 [53] mmHg; LVEDP = 7 mmHg; CO = 4.96 L/min) | Sildenafil initiated; lenalidomide continued; ambrisentan added 3 months later for persistent FC III symptoms | … | … |
| 3 | FC improves; repeat RHC (RA = 9 mmHg; PAP = 81/22 [42] mmHg; PAWP = 13 mmHg; CO = 5.69 L/min) | TAVR performed given severe AS | … | … |
| 4 | Remains stable at FC II; repeat RHC (RA = 9 mmHg; PAP = 70/23 [39] mmHg; PAWP = 18 mmHg; CO = 5.53 L/min) | Continued sildenafil and ambrisentan; continued maintainance lenalidomide | … | … |
| Patient 2 | ||||
| 1 | MM and AL amyloidosis diagnosed | Received HSCT the same year; does not receive subsequent chemotherapy | … | … |
| 2 | Cardiac amyloidosis diagnosed 5 years later | … | … | … |
| 3 | PH diagnosed 3 years after amyloidosis diagnosis; FC III symptoms; RHC (RA = 8 mmHg; PAP = 71/26 [41] mmHg; PAWP = 17 mmHg; CO = 2.0 L/min) | Sildenafil initiated | … | … |
| 4 | FC improves to class II; repeat TTE with lower measured PASP | Continued to receive sildenafil | … | … |
| Patient 3 | ||||
| 1 | MM diagnosed | Received thalidomide-cyclophosphamide-dexamethasone; received bortezomib-dexamethasone; remission achieved; received HSCT | … | … |
| 2 | Relapse of myeloma 7 months after HSCT | Received multiple cycles of lenalidomide-dexamethasone for 1 year with POD; received carfilzomib-lenalidomide-dexamethasone for 2 years with POD | … | … |
| 3 | PH first diagnosed; FC II symptoms: RHC (RA = 6 mmHg; PAP = 51/21 [31] mmHg; PCWP = 7 mmHg; CO = 3.3 L/min) | Amlodipine started; discontinued because of lower extremity edema | 406 | 1.7 |
| 4 | Persistent MM | Received bendamustine-bortezomib-dexamethasone for 1 year with POD; received clarithromycin-pomalidomide-dexamethasone for 1 year with POD; received carfilzomib-pomalidomide-dexamethasone for 1 year with POD | … | … |
| 5 | PH worsens; FC III symptoms: RHC (RA = 9 mmHg; PAP = 61/28 [39] mmHg; PCWP = 12 mmHg; CO = 2.42 L/min) | Sildenafil initiated 4 years after initial diagnosis | 3,360 | 29.9 |
| 6 | Persistent MM | Received clarithromycin-bortezomib-lenalidomide-dexamethasone for 1 cycle with POD | … | … |
| 7 | FC improves to class II; repeat TTE with improved RV function and lower measured PASP of 42 mmHg | … | 3,760 | 17 |
| 8 | Persistent MM | Received doxorubicin-bortezomib-dexamethasone for 6 months with POD; initiated monoclonal antibody elutuzumab and pomalidomide | … | … |
| 9 | PH symptoms worsen 7 months after last TTE; repeat TTE with more RV dilation; PASP is 74 mmHg | Ambrisentan added | 4,800 | 128 |
| 10 | FC improves to class II; repeat TTE with improved RV size and lower measured PASP of 38 mmHg | Continued sildenafil and ambrisentan; continued monoclonal antibody elutuzumab and pomalidomide | 4,740 | 130 |
Note
AL: amyloid light chain; AS: aortic stenosis; CO: cardiac output; FC: World Health Organization functional class; FLC: free light chain; HSCT: hematopoietic autologous stem cell transplant; IgA: immunoglobulin A; LVEDP: left ventricular end-diastolic pressure; MM: multiple myeloma; PAP: pulmonary artery pressure, expressed as systolic/diastolic pressure (mean pressure); PASP: pulmonary artery systolic pressure; PAWP: pulmonary arterial wedge pressure; PH: pulmonary hypertension; POD: progression of disease; RA: right atrium; RHC: right heart catheterization; TAVR: transcatheter aortic valve replacement; TTE: transthoracic echocardiography.