An 82-year-old woman was recently diagnosed with biopsy proven light chain (AL) cardiac amyloid.  She has class III symptoms of heart failure despite having a preserved ejection fraction. On exam, she is normotensive (124/80) with evidence of volume overload (jugular venous pressure [JVP], hepatomegaly and lower extremity edema). Her cardiac biomarkers demonstrate a troponin of 0.45 ng/ml (normal <0.02 ng/ml) and a B-type natriuretic peptide (BNP) level of 871 pg/ml (normal <100 pg/ml). She has started chemotherapy and comes for follow-up visit.  Her son and daughter accompany her to the appointment and have asked for a frank discussion of what lies ahead. 

Initiating such discussion is often difficult for many clinicians. Data suggest that communication regarding these issues is often not undertaken. Specifically, bereaved family members of heart failure patients who did not die suddenly reported that only 37% were made aware of a poor prognosis, only 8% of patients and 44% of loved ones were told by a provider that time was short, and 36% of these patients died alone. Effective communication usually begins with an assessment, including asking the patient what is her/his understanding of the condition. This is followed by providing prognostic information (i.e., realizing that while there is no prognostic certainty, the absence of definitive data is not an excuse for failing to communicate about the implications of a terminal condition).

With regards to AL cardiac amyloid, the Mayo staging system is quite helpful is estimating survival in patients suffering from this condition.  In this staging system, patients are assigned a score of 1 for each of free light chain difference of  ≥18 mg/dl, cardiac troponin T (cTnT) ≥0.025 ng/ml, and N-terminal proBNP (NT-ProBNP) ≥1,800 pg/ml, creating stages I to IV with scores of 0 to 3 points, respectively. The median overall survival from diagnosis was 94.1, 40.3, 14, and 5.8 months, respectively (P <0.001). Thus, our patient has a BNP of >800 corresponding to an NT-proBNP of easily >1,800, as well as a positive troponin. However,her free light chain difference was only 16.4, thus her score is 2, and her overall median survival is 14 months.

It is vital to prepare the patient and her family for what to expect and provide time estimates. Data from large populations of treated patients (albeit rarely including individuals >80 years of age) suggest that four levels of response can be obtained from serial measurement of light chains including: 1) amyloid complete response (normal FLC ratio and negative serum and urine immunofixation), 2) very good partial response (difference between involved and uninvolved FLCs [dFLC] <40 mg/L), 3) partial response (dFLC decrease >50%), and 4) no response. Since cardiac involvement is the major determinant of survival, it is not surprising that changes in cardiac function after therapy can be reliably assessed using the cardiac biomarker NT-proBNP. Changes in FLC and NT-proBNP can predict survival as early as three months after treatment initiation.  Thus, it would be appropriate to inform the patient that the short-term goal of chemotherapy is to lower the light chains and possibly even the natriuretic peptide. If this is achieved after three months of therapy, her prognosis may improve, but, if not, reconsideration of ongoing chemotherapy would be appropriate.

While receiving a trial of chemotherapy for her AL amyloid, it is important to discuss the patient’s preferences regarding her health care proxy (in this case, son, daughter, or both); goals if the patient is permanently brain injured; desires for cardiopulmonary resuscitation and ventilators; and preferred location for care to be delivered. In the case of AL amyloid, most patients do not die from a malignant ventricular arrhythmia, and, thus, the role of prophylactic implantable cardioverter-defibrillators (ICDs) is not clear; they are usually not recommended. After discussion of these facts, she decides not to have an ICD. She requests that she not be resuscitated in large part because this is not concordant with her wishes and she understands that resuscitation efforts in AL amyloid are not likely to be effective.

Finally, after three months of therapy, her light chains do not decline, and she begins to plan for the worst with her health care provider’s assistance.  She requests additional help at home to assist with activities of daily living and has a live-in companion 24 hours a day, seven days a week. She becomes progressively weaker, more fatigued, short of breath, and bedbound.  Hospice services are mobilized, and she expires at home eight months after her initial diagnosis.

References:

1. Mccarthy M, Hall JA, Ley M. Communication and choice in dying from heart disease. J R Soc Med 1997;90:128 –31.
2. Adler ED, Goldfinger JZ, Kalman J, Park ME, Meier DE. Palliative care in the treatment of advanced heart failure. Circulation 2009;120:2597-606.
3. Kumar S, Dispenzieri A, Lacy MQ, et al. Revised prognostic staging system for light chain amyloidosis incorporating cardiac biomarkers and serum free light chain measurements. J Clin Oncol 201220;30:989-95.
4. Palladini G, Dispenzieri A, Gertz MA, et al. New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes. J Clin Oncol 2012;30:4541-9.

Keywords: Activities of Daily Living, Arrhythmias, Cardiac, Biomarkers, Biopsy, Brain, Cardiopulmonary Resuscitation, Defibrillators, Implantable, Edema, Female, Follow-Up Studies, Goals, Health Personnel, Heart Failure, Hepatomegaly, Hospices, Humans, Lower Extremity, Natriuretic Peptides, Nuclear Family, Patient Preference, Peptide Fragments, Prognosis, Troponin, Troponin T, Venous Pressure

< Back to Listings