Bob Hart is a 56-year-old divorced man with a history of acute on chronic systolic heart failure (HF) secondary to nonischemic cardiomyopathy, type 2 diabetes, and obesity. He was admitted to the cardiac service with multiple HF-related symptoms including shortness of breath, cough, weight gain, lightheadedness, generalized weakness, and fatigue. His HF was first diagnosed 9 years ago and a recent echo revealed a left ventricular ejection fraction (EF) of <20%. Bob underwent right heart catheterization and was found to have a low cardiac index. During the admission his condition worsened, which prompted the team to start a dobutamine infusion and begin workup for possible advanced HF therapies. In addition to HF symptoms, Bob suffers from bilateral leg pain secondary to diabetic neuropathy. Psychosocially, he is currently undergoing a divorce and lives alone, but he has an adult daughter who is very involved in his care.

Bob was evaluated by a multidisciplinary team that included a cardiologist, cardiothoracic surgeon, social worker, and palliative care team. His treatment options included a left ventricular assist device (LVAD) implant as destination therapy or continuation of inotropes with focus on comfort care. He was not a transplant candidate due to morbid obesity and complications related to diabetes. After extensive discussion with the interdisciplinary team, Bob assessed his risks and benefits and determined that home-based management without surgery was congruent with his goals of care. While in the hospital his medical therapy was optimized and he was started on low-dose oxycodone to help manage refractory dyspnea and neuropathic pain not controlled with gabapentin.

Bob was discharged home with an inotrope infusion and initiation of home hospice. The hospice team provided frequent palliative domain-focused support to Bob and his daughter. He died at home 2 months following this hospitalization, surrounded by his family with minimal symptom burden. Bereavement services were provided by hospice to the family following the patient’s death.

Heart disease is the number one cause of death in the United States, accounting for almost a quarter of all deaths in 2013.¹ HF is a chronic, terminal illness mentioned in over 11% of all death certificates in the United States.² Palliative care is an integral component of chronic HF care that can assist with treatment of refractory symptoms, clarify goals of care in the face of prognostic uncertainty, help with determining the appropriate location of care, and provide psychosocial support for the patient and family. This care is provided with close collaboration between a palliative care consultant and the primary cardiology team. Palliative care should be introduced early in the disease process with increasing focus as HF progresses (Figure 44-1). Early integration of palliative medicine and institutional funding for palliative care is now a recommendation by the American Heart Association.³

The word palliate is defined as “to alleviate (a disease or its symptoms) without effecting a cure; to relieve or ease (physical or emotional suffering) temporarily or superficially.”⁴ The scope of palliative care often extends beyond this, as management of disease often palliates symptoms. Palliative care utilizes a multidisciplinary team approach that often includes physicians who specialize in palliative medicine, nurse practitioners, nurses, psychologists, social workers, pharmacists, chaplains, occupational therapists, physical therapists, speech therapists, care coordinators, and nursing assistants to alleviate multiple facets of suffering and promote quality of life. It is often provided along with other disease-focused medical treatments.⁵

Symptom burden associated with HF adversely affects quality of life for patients. The differential of all symptoms evaluated in a palliative care visit is beyond the scope of this chapter, but it is important to explore reversible causes of symptoms in all patients. Symptoms are often a result of worsening HF, and optimizing HF therapy is the primary symptom management strategy. Unfortunately, despite treatment of reversible causes, there are many cases where optimal management of disease fails to alleviate symptoms.

Patients with end-stage HF experience a large symptom burden, with nearly half suffering from nausea, constipation, and anxiety. Symptoms such as pain, fatigue, and breathlessness are even more common at 77%, 82%, and 88%, respectively.⁶ Tools, such as the Edmonton Symptom Assessment Scale (ESAS), assist palliative care clinicians in identifying these symptoms common in advanced illnesses including HF. Symptoms addressed include pain, tiredness (a measure of generalized weakness), nausea, depression, anxiety, drowsiness (a measure of insomnia), appetite, wellbeing, shortness of breath, and a category titled “other.”⁷ The Memorial Symptom Assessment Scale (MSAS) is another common symptom assessment scale that includes further detail including difficulty concentrating, cough, dry mouth, numbness/tingling in hands/feet, feeling bloated, problems with urination, diarrhea, sweats, problems with sexual interest or activity, itching, dizziness, and difficulty swallowing.⁸ There are many other symptom assessment scales, some broad similar to the ESAS and MSAS, and some that deal only with a specific symptom. Systematic assessment using these tools aids the clinician in the diagnosis of symptoms along with evaluation of severity and response to therapy. In addition to clinical utility, these scales aid in research and quality improvement in palliative care.

In HF, similar to other serious potentially life-limiting illnesses, suffering and distress may occur in various domains that reach beyond physical symptoms. Thus the palliative care interdisciplinary team assesses 8 different domains to provide a whole-person assessment.⁹

1. 
   

   The structure and process of care domain includes assessing patient and family understanding of disease, prognosis, and patient preferences for type and site of care. This would also include discussion of the role of advanced HF therapies. A diagram provided to the patients can be helpful in explaining HF trajectory and prognosis (Figure 44-2).

   
   
2. 
   

   Physical aspects of care focus on ensuring safe and timely management of pain and other physical symptoms.

   
   
3. 
   

   Psychological and psychiatric aspects address depression, anxiety, delirium, cognitive impairment, assessment of capacity, grief, and bereavement.

   
   
4. 
   

   Social aspects of care examine family structure, geographic location, support, living arrangements, caregiver availability, access to transportation, access to medications, and caregiver support.

   
   
5. 
   

   Spiritual, religious, and existential aspects of care focus on life review, hopes and fears, guilt, forgiveness, life completion, and availability of leaders specific to the patient’s religious traditions.

   
   
6. 
   

   Cultural aspects of care provide a cultural needs assessment and strives for respectful communication, decision making, and communication in a language family will understand.

   
   
7. 
   

   Care of the patient at the end of life focuses on presence, patient and family education, prognosis, symptom management, and a bereavement plan.

   
   
8. 
   

   Ethical and legal aspects of care ensure patient preferences are followed via the patient himself or herself, a medical decision maker, or advance directives regarding administration of further treatments or withholding/withdrawing treatments.

For this chapter, we will focus more specifically on physical and psychological symptoms and possible management strategies. There are many symptoms encountered in HF patients (Table 44-1). A thorough assessment of symptoms for patients with HF may include the topics discussed in the following sections.

DYSPNEA

Dyspnea is reported in 60% to 88% of patients with HF.⁶ The sensation of breathlessness is a result of the human body’s perceived inability to meet one’s ventilatory demand, and it is closely tied to higher cortical processes. Subjective reporting of dyspnea is crucial as the body is extremely sensitive to ventilation inadequacies and patients may experience dyspnea in the context of normal medical test results. Laboratory studies such as blood gasses and pulse oxygenation are not reliable measures of perceived dyspnea.¹⁰

Nonpharmacologic management strategies often prove effective in decreasing the sensation of breathlessness. Supplemental oxygen has been found to be useful in managing dyspnea only in patients who are hypoxic. Movement of air through the nares has been shown to improve dyspnea independent of oxygenation.¹¹ Any method of stimulating the trigeminal nerve with cool, moving air is effective in reducing dyspnea and may be achieved with a fan blowing toward the patient’s face or forced air/oxygen via nasal cannula.¹²

Pharmacologic therapies for dyspnea should always include aggressive medical management of HF such as diuretics, vasodilators, inotropes, and ultrafiltration techniques such as aquapheresis. Angiotensin-converting enzyme (ACE) inhibitors have also been found to reduce dyspnea.¹³

Opioids such as morphine are effective at minimizing shortness of breath in patients with dyspnea refractory to disease-directed therapy. Despite a common myth, opioids do not cause significant respiratory depression or sedation at appropriate doses. Opioid receptors are located throughout the central nervous system (CNS) as well as peripheral areas responsible for monitoring respiration such as the carotid bodies.¹⁰ Even at low doses, opioids can be effective for dyspnea.¹⁴ These doses may often be lower than what would be expected for pain management. Benzodiazepines may be appropriate in HF patients with dyspnea as a second-line agent in addition to opioid medications if anxiety is a concurrent issue.

FATIGUE

Fatigue is a hallmark symptom of heart disease and affects between 69% to 82% of patients.⁶ Important factors to consider in the fatigued HF patient include deconditioning and comorbid conditions such as anemia, hypothyroidism, depression, and sleep disorders.

Regular physical exercise tailored to the patient’s ability should be encouraged. Though seemingly counterintuitive, prolonged rest is thought to provide no benefit in fatigue and promotes further deconditioning. Patients may also be advised on energy conservation and taught to keep a diary to help plan for worsening fatigue and prioritizing activities.¹⁵ Fatigue secondary to comorbid conditions should be identified and managed appropriately. If comorbidities are optimally managed, stimulants such as methylphenidate may also be helpful in reducing fatigue.¹⁶

PAIN

Pain is extremely common in HF patients, with a reported incidence of 63% to 80%.⁶ Multiple etiologies for pain exist in HF patients. Common examples are ischemic pain, neuropathic pain, pain secondary to lower extremity or abdominal edema, and arthritic pain unrelated to cardiovascular disease. Patients may also experience pain without an easily identified etiology or other pain-causing comorbidities.¹⁷ Pain levels tend to increase as the severity of HF increases. Special attention should be placed on pain in HF patients as it is often undertreated and lags significantly behind the management of pain in cancer.¹⁸

Management of pain in patients with HF presents particular challenges. Although opioid-sparing analgesics are often first-line for pain management in chronic nonmalignant pain, such medications are often restricted in this patient population. Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or naproxen should be avoided as they can further impair renal function affected by poor cardiac output and worsen fluid retention.¹⁹ They are also contraindicated in patients who are on anticoagulation medications. Acetaminophen may be considered first-line for nonneuropathic pain; however, special attention must be paid to dosing when using it in the context of liver dysfunction. Given these restrictions, opioids are often required for pain management in HF patients.

Pharmacologic considerations for opioid medications must be made when patients exhibit hepatic or renal dysfunction and clinicians must monitor for opioid toxicities regardless of which opioid is chosen. In patients with renal failure, morphine should be avoided as it has active, renally excreted metabolites that cause neurotoxicity. Codeine is a prodrug of morphine and should also be avoided in renal disease. As it relies on liver metabolism, codeine is likely to be less effective in hepatic disease.²⁰ Hydromorphone also produces renally-excreted metabolites that may cause neurotoxicity and should be used with caution in renal impairment. Oxycodone and fentanyl do not have renally-excreted active metabolites and thus are the opiates of choice in a patient with renal failure.²¹ Morphine or hydromorphone should be considered in patients with hepatic failure as they undergo glucuronidation, which is more likely to be preserved in liver disease. Hydromorphone is an acceptable option in patients with both renal and hepatic failure. Fentanyl is also commonly used in hepatorenal failure, though data supporting fentanyl use in severe hepatic failure are lacking. Methadone is also an option acceptable in both renal and hepatic failure but is challenging to dose and generally requires consultation with palliative care or pain management specialists.^22,23

Many opioid side effects such as nausea, drowsiness, and confusion are self-limited and resolve within a week.²⁴ Exploring these issues with patients is important because side effects are often mistakenly listed as allergies and are not a contraindication to another trial. Unlike other transient opioid side effects, constipation is not transient and therefore needs continual monitoring and management in patients consistently taking opioids. Management of constipation is discussed later in this chapter.

The anticonvulsants gabapentin or pregabalin should be considered in patients with neuropathic pain. This class of medications is considered first-line agents, along with tricyclic antidepressants (TCAs), for neuropathy.²⁵ TCAs should be avoided due to risk of QT prolongation and side effects potentially compounding HF symptoms such as dry mouth and orthostatic hypotension.²⁶ Serotonin–norepinephrine reuptake inhibitor (SNRI) antidepressants, tramadol, topical lidocaine, and topical capsaicin are adjuvant therapies for neuropathic pain that have proven efficacy and should be considered. Opioids are considered third-line agents in neuropathic pain, though they may be necessary in refractory cases.²⁷

DEPRESSION

It is well known that depression is associated with cardiovascular disease and HF. A meta-analysis consisting of 27 studies revealed rates of depression between 9% and 60%. As with pain, rates of depression increase with increased severity of HF. New York Heart Association (NYHA) functional class is correlated to incidence of depression, with greater than 40% prevalence in class IV.²⁸

Depression is a challenging diagnosis in terminal illnesses such as heart failure (HF). Many symptoms of depression overlap with symptoms experienced in chronic or terminal illness. Examples of overlapping symptoms include sleep disruption, decreased appetite, and increased fatigue. Symptoms exclusive to depression include suicidality, anhedonia, and loss of hope.

Management of depression in HF is similar to management in the general population. Nonpharmacologic interventions such as cognitive behavioral therapy should be incorporated into management. Social workers can explore group support options for the patient and provide support to caregivers. Depressive symptoms in caregivers have been shown to affect a patient’s likelihood to respond to depression interventions.²⁹

A selective serotonin reuptake inhibitor (SSRI) should be considered. Data are limited, but sertraline has been studied and shown to be safe in HF.³⁰ Stimulants such as methylphenidate may also be considered, as a response will typically occur within 2 days.³¹ This is helpful in situations where the 4 to 6 week onset of SSRI efficacy is unacceptable. A common example is patients with a very short life expectancy. Common risks such as arrhythmias must be taken into account when considering stimulants.³²

ANXIETY

Anxiety has been reported to affect approximately 49% of HF patients.⁶ Anxiety may arise from different etiologies including underlying psychiatric disorders, symptoms such as pain or shortness of breath, drug side effects, or as a response to the numerous concerns a patient encounters towards the end of life. Patients with implantable cardioverter defibrillators (ICDs) are more likely to experience symptoms of anxiety, especially if they have experienced a device firing.^33,34 Clinicians should remember that hyperactive delirium may be mistaken for anxiety.

Nonpharmacologic management such as supportive psychotherapy and cognitive behavior therapy are important interventions in able patients as an adjuvant to pharmacotherapy. Music therapy, art therapy, relaxation techniques, meditation, aromatherapy, and acupuncture should be considered as well.^35,36 These therapies are now available in some hospitals and are often incorporated into hospice care. A multidisciplinary approach incorporating chaplaincy and social work is also an integral component of managing a patient’s anxiety.

For acute-onset anxiety, benzodiazepines are first-line drugs to provide prompt relief of symptoms. Lorazepam is a good initial choice as is it relatively short-acting and better tolerated in patients with liver dysfunction. Benzodiazepines should be used with caution as they may cause mental status changes and worsen delirium, especially in elderly or seriously ill patients. Other anxiety-provoking conditions listed earlier should be addressed as needed. If life expectancy is thought to be longer than a few weeks and exposure to the anxiety-provoking stimulus is expected to continue, management with SSRIs should be explored.

INSOMNIA