Approach to advanced heart failure at the end of life

Heart failure prevalence.

Approximately 500 000 Canadians have congestive HF. More than 50 000 new patients are diagnosed each year and more than half of new cases occur in people older than age 80.¹ Approximately 50% of patients with HF die within 5 years of diagnosis.² The condition is responsible for a high burden of hospitalization for symptoms that are currently difficult to manage in the ambulatory setting.^3–5

Definition of advanced HF.

The Heart Failure Society of America defines advanced HF as “those patients who have advanced, persistent HF with symptoms at rest despite repeated attempts to optimize pharmacologic and nonpharmacologic therapy,” as shown by 1 or more of the following factors: frequent hospitalizations (≥ 3 per year), chronic poor quality of life with inability to accomplish activities of daily living, need for intermittent or continuous intravenous support, or consideration of assistive devices as destination therapy.⁶

Pathophysiology and symptom burden.

Damage to ventricular function results from numerous causes, including myocardial ischemia or infarction. Decreased left ventricular function (LVF) leads to symptoms of forward HF (low output), which causes confusion, fatigue, anorexia, and renal dysfunction. Decreased LVF also leads to backward pressure (high filling pressure) on the cardiopulmonary system resulting in dyspnea, ascites, liver congestion, and peripheral edema. Additionally, decreased LVF causes a cascade of neurohormonal mediators such as the renin-angiotensin-aldosterone system, the sympathetic nervous system, and tumour necrosis factor. These neurohormonal mediators sustain and increase damage to the ventricles, which sets up a vicious cycle whereby decreased LVF causes neurohormonal changes that worsen LVF. This explains why HF medications (β-blockers, angiotensin-converting enzyme inhibitors [ACEIs], angiotensin II receptor blockers) are effective. They work by decreasing the effect of the neurohormonal cascade, limiting the effect on ventricular damage and improving symptoms. These not only improve symptoms but also decrease mortality.⁷ In addition to symptoms of dyspnea, fatigue, and confusion, patients with advanced HF experience pain, anorexia, depression, and anxiety. Numerous studies show that patients with end-stage HF experience symptoms similar to patients with cancer at the end of life.⁷

Challenges in palliating end-stage HF.

There are several additional challenges in caring for those with non-malignant illnesses such as HF compared with caring for patients with an oncologic illness. Models of palliative care, developed primarily for those with oncologic illness, need to be adapted to acknowledge these challenges, as an increasing number of patients being referred for palliative care have non-malignant illness such as HF, chronic obstructive pulmonary disease, and dementia.

One of these challenges includes the trajectory of HF being much more variable and unpredictable than the trajectory for most oncologic illnesses. There are few reliable prognostic indicators that predict when patients are entering the end phase of their illness. Figure 1^8,9 presents a trajectory comparison between a patient with end-stage HF and that of a patient with cancer. For patients with HF, the trajectory is often stable for a period of time, and as their disease progresses, they experience repeated decompensations with return to near baseline function and health.^8–11 This is in contrast to the somewhat more predictable trajectory for patients with cancer at the end of life, where there is generally a steady decline in function in the last 3 months of life.¹² In the absence of prognostic indicators and with the variability in function, health care practitioners should focus on the needs and goals of this patient population rather than solely on prognosis.

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Figure 1.

Illness trajectories: A) Cancer trajectory vs B) end-stage heart or lung failure trajectory.

Reproduced with permission from Pallium Canada.⁸ Adapted with permission from Lunney et al.⁹

A second challenge in caring for patients with end-stage HF is that patients and caregivers often lack understanding of the terminal nature of HF.^10,11,13 While the reasons for this are unclear, there are several possibilities. First, there are no obvious time points or events that clearly herald the transition from active care to palliative care. Unlike chemotherapy for the patient with cancer, therapy for HF does not lose efficacy or stop. Medications used to treat chronic HF are the same as medications used to palliate end-stage HF.¹² Additionally, there are fewer diagnostic approaches that definitively illustrate disease progression (compared with computed tomography and magnetic resonance imaging in oncology). For patients with cancer, these transition points can provide an opportunity for health care providers to discuss disease progression and for patients to gain understanding of their illness.

Thus, in the care of patients with HF, the unpredictable trajectories and the lack of obvious transition points in disease progression can make conversations about goals of care, values, and advance care planning with these patients quite challenging.^11,14–16

From our experience, it is useful to begin by educating patients and caregivers about the natural history of HF. By drawing the trajectory of HF (Figure 1)^8,9 for patients and caregivers, you can explain the variable nature of the illness and explain that each decompensation is a time when death is possible. You can explain that the diuresis and other management during a decompensation is an attempt to regain previous function; however, at some point that will not be possible. This can help patients and families better understand the variable trajectory and the uncertainty associated with HF. Once a patient has a better understanding of the illness and illness trajectory, you can begin to discuss his or her values and goals for the future and prepare for end-of-life decisions. Box 1 provides examples of how to begin these discussions with patients. Recognizing that these conversations need to occur repeatedly can improve patient understanding.^7,13 Also, whenever possible, include the patient’s caregivers in these conversations.

For more information about advance care planning discussions, visit the Speak Up Campaign website, www.advancecareplanning.ca, for tools and resources; this campaign is an important initiative to help physicians support their patients during this time. Articles by Ahluwalia et al¹⁷ and Sudore and Fried¹⁸ are also good resources for advance care planning discussions.

You spend time explaining to Frank the ups and downs of the HF trajectory and, in fact, when you draw the trajectory for him he realizes that this is the nature of the illness and not something that he is doing wrong. You tell him that when he gets home from the hospital it would be a good time to have a discussion about the future and how he wants his care going forward.

A few weeks later he comes to your office with his daughter for a conversation regarding his goals of care. During the discussion he tells you that he now understands that he is nearing the end of his life and he does not want to prolong this phase. He wants to stay home as long as possible and avoid hospitalizations and further investigations. He wants you to focus on his comfort. He is not afraid of dying but wants to make sure he does not suffer from pain or symptoms. Given these expressed values and goals, you recommend against cardiopulmonary resuscitation and defibrillation. You explain that his cardiac device needs to be reprogrammed to avoid shocks at the end of life. Frank thinks (as do many patients) that he needs the defibrillator all the time to live.

Implantable cardiac defibrillator (ICD) deactivation.

Many patients with HF are treated with specialized cardiac devices. These devices function as pacemakers, and the pacemaker might have additional functions such as cardiac resynchronization therapy (CRT) or a defibrillator or both. Some patients have a device that functions only as an ICD. A CRT pacemaker coordinates the contractions of both the right and the left ventricle and improves heart function, improving the quality of life and symptoms for many patients. Defibrillation is another function that is part of some, but not all, implantable pacemaker devices, which means the device is programmed to detect malignant arrhythmias and provide an electric shock to restore normal rhythm. Some devices only provide defibrillation; these are known as implantable cardiac defibrillators. A CRT pacemaker can be equipped to provide defibrillation; this device is then called a cardiac resynchronization therapy defibrillator.

It is important to know that the defibrillator function of any implantable device is independent from pacing or resynchronization therapy. The defibrillator itself can be deactivated and the patient can continue to receive the symptom improvement therapy from CRT. Defibrillator deactivation is important when patients want to focus on comfort, as the defibrillator might provide electric shocks to the patient at the end of life, which can be distressing and uncomfortable.

There are often many opportunities to discuss the need for and use of the ICD. This can be a challenging conversation because patients and caregivers might have misconceptions about the ICD. Table 1 addresses some of these patient misconceptions.

For more information on ICD deactivation, the Cardiac Care Network¹⁹ and BC’s Heart Failure Network^20,21 provide guidelines and algorithms.

Home diuresis protocol.

The protocol for home diuresis that we developed provides stepwise guidelines for assessment, management, and monitoring. It is used only when patients’ goals are for comfort-oriented care. Their goals are such that they wish to remain at home, make comfort a priority, and avoid hospitalizations. The protocol is initiated when patients experience a worsening of symptoms attributed to HF. This protocol is available at CFPlus.^*

Home-based care of patients with advanced HF that includes optimizing diuresis can lead to improved symptom management and possibly a decreased number of hospitalizations. In turn, we hope it can reduce both patient and system burden and result in greater health-related quality of life for patients with advanced HF.

With this plan you have been managing Frank at home for the past 4 months. He has had follow-up with his cardiologist for ICD deactivation. He has required 2 escalations of his furosemide dose, and in both cases doubling his oral dose for 5 days was sufficient. He has become weaker over this time and spends most of each day in bed. He is relieved to be at home. You have discussed transfer to a palliative care facility in the near future, as he does live alone and might not have sufficient supports as his illness progresses.