Heart Failure: “Sneaky” Triggers and Clinical Pearls

Authors: James Karz, DO (EM Resident Physician, Rutgers – RWJMS) and Josh Bucher, MD (EM Attending Physician, Rutgers – RWJMS) // Edited by: Alex Koyfman, MD (@EMHighAK) and Brit Long, MD (@long_brit)

Clinical Case #1

A 34-year-old female with a history of hypertension and CHF presents with two weeks of worsening dyspnea on exertion. Review of systems was positive for bilateral lower extremity heaviness, orthopnea, and dry cough. She denies any fevers, chest pain, or recent travel. Her last echocardiogram showed an EF of 30-35%. She is on Losartan and Lasix and reports being compliant with her medications. Two weeks ago she had pneumonia and was treated with a Z-pack. She also reports that she is two months postpartum and was diagnosed with preeclampsia during this last pregnancy.

Vital signs: T 98.2 F, P 110, RR 18, BP 240/130, Spo2 88% on RA.

On physical exam, she has JVD, bilateral lower extremity edema, and bilateral crackles.

Clinical Case #2

The patient is a 62-year-old male with a history of ischemic cardiomyopathy, CAD s/p CABG, HFrEF s/p AICD, DM, HTN, and restrictive lung disease presenting with dyspnea, cough, and lower extremity swelling for several months. His symptoms have progressively worsened to the point where he now has difficulty ambulating. He denies chest pain, fever, or history of recent travel.

Vital signs: T 97.6 F, P 105, RR 20, BP 90/50, Spo2 91% on RA.

On physical exam, the patient is tachypneic with JVD and rales from the bases to the middle lung fields bilaterally.

Background

As a diagnosis at hospital discharge, heart failure (HF) has tripled over the last 3 decades. The cost of treating HF in the U.S. is over $34 billion per year1. Although outpatient care has improved, acute HF presentations remain a diagnostic, therapeutic, and prognostic dilemma. For ED physicians, the misdiagnosis rate of acute HF syndromes is approximately 20%2. There is a wide variety of acute HF presentations encountered in the emergency department (ED), each with dramatically different underlying pathophysiology and acute hemodynamics. Thus, in order to improve patient outcomes and address the growing economic burden of HF management, we as emergency physicians need to have a better understanding of the heterogeneity of the disease and the phenomena that precipitate its acute manifestations. Some of the more common triggers include respiratory illnesses, arrhythmias, renal failure or worsening renal function, acute coronary syndromes, uncontrolled hypertension, medication non-compliance, and dietary indiscretion. The following, however, is a list of important causes of acute heart failure syndromes that may often be overlooked during the ED evaluation. For more on heart failure, see this emDocs post.

Myocarditis

Acute myocarditis commonly presents with chest pain or dyspnea. Classic teaching suggests a viral syndrome, such as a URI or GI illness, often precedes the clinical syndrome by several days to weeks. In some patients the disease can evolve into new onset, progressive, or chronic heart failure. The incidence of myocarditis was approximately 1.5 million cases in the 2013 world population3. Young individuals are most frequently affected4.

Myocarditis represents multiple heart-specific inflammatory conditions, both infectious and noninfectious. Most often, myocarditis results from common viral infections5. Important noninfectious causes include giant cell myocarditis, drug-induced hypersensitivity, and cardiac manifestations of systemic autoimmunity, such as sarcoidosis or systemic lupus erythematosus5.

In patients presenting with acute or fulminant myocarditis, an elevated cardiac troponin level can support the diagnosis, but a normal level does not exclude it. In one study of 80 patients with clinically suspected myocarditis, an elevated troponin level was only 53% sensitive6. Furthemore, ECG signs are neither specific nor sensitive4, however, ECG abnormalities are common in myocarditis. Repolarization abnormalities, such as ST elevations and T wave inversions, are prevalent and may occur in 40-50% of patients7,8. Blood tests for antiviral antibodies correlate poorly with viral genomes detected on heart biopsies; therefore, serology cannot be used to diagnose viral myocarditis. ESR and CRP levels are often elevated in myocarditis, but cannot be used to confirm the diagnosis either.

An echocardiogram should be performed in all patients with clinically suspected myocarditis; global ventricular dysfunction, regional wall motion abnormalities, and diastolic dysfunction with preserved ejection fraction may be seen4. Improvements in the diagnostic and prognostic capabilities of cardiac magnetic resonance (CMR) have made this imaging modality the primary tool for noninvasive assessment of myocardial inflammation in patients with suspected myocarditis9. However, the diagnostic accuracy of CMR has not been well defined, and data from larger multicenter trials with standardized protocols comparing CMR to biopsy-derived criteria are lacking9.

NSAIDs

Patients with HF often have a high medication burden, which frequently includes over-the-counter (OTC) preparations. In a single-center study of 161 patients with HF, 88%reported using OTCs10. NSAIDs alone account for roughly 30 billion OTC medications sold annually in the United States and have been associated with HF exacerbations in multiple observational studies10. NSAIDs have the potential to trigger HF through sodium and water retention, increased SVR, and blunted response to diuretics.  A meta-analysis of data from randomized trials involving over 300,000 patients showed that the risk of developing HF in patients on NSAIDs was nearly double that of placebo11. The risk for these adverse effects is likely greatest in patients with a prior history of or at high risk for cardiovascular disease12.

Chemotherapy and Radiation

Many classes of anticancer medications are cardiotoxic and precipitate acute or delayed-onset heart failure, including: anthracyclines, alkylating agents, antimetabolites, taxanes, and monoclonal antibodies. The development of heart failure with administration of anthracyclines (e.g., doxorubicin and danorubicin) increases with greater cumulative doses and can occur more than 20 years after the completion of therapy10. The cardiotoxic effects of anthracyclines are enhanced when administered with taxanes, trastuzumab, cyclophosphamide, or other agents that cause further cardiac injury. Mediastinal radiation is also a risk factor for the development of heart failure, especially in patients who have been exposed to anthracyclines or alkylating agents such as cyclophosphamide. The effects of mediastinal radiation alone can cause restrictive cardiomyopathy and lead to signs and symptoms of heart failure13.

Pregnancy

Peripartum cardiomyopathy (PPCM) is a rare form of dilated cardiomyopathy associated with LV systolic dysfunction and heart failure. It manifests clinically in the third trimester of pregnancy or the first 5 months postpartum and requires a high index of suspicion for diagnosis14. Many of the signs and symptoms of a normal pregnancy are similar to those of HF; therefore, the diagnosis of PPCM is often missed or delayed15. The disease most frequently occurs in obese, multiparous women > 30 years of age with preeclampsia. The incidence of PPCM in the U.S. seems to be increasing and is estimated to be approximately 1 in 3,200 deliveries, with a significantly higher incidence in African American women. Complete or nearly complete recovery occurs within 6 months in approximately 50% of cases, however, progressive clinical deterioration, heart failure, death, or need for transplantation can occur14.

Alcohol Abuse

Moderate alcohol consumption (~1-2 drinks per day) has been shown to decrease cardiovascular and all-cause mortality, including risk of death from heart failure16. However, changes in cardiac structure and function have been reported in patients who consume greater than 90 grams (~ 7 drinks) per day for five or more years17. The incidence of alcoholic cardiomyopathy may represent 21-32% of all patients diagnosed with dilated cardiomyopathy17. On echocardiogram, alcoholic cardiomyopathy demonstrates 4-chamber dilation, low cardiac output, and normal or decreased LV wall thickness. Multiple studies have shown a tendency toward improvement in LV EF in patients who abstained or drastically decreased their intake of alcohol. Survival is poor in those who continue to drink heavily18.

Takotsubo Cardiomyopathy

Takotsubo cardiomyopathy (TCM), also called apical ballooning syndrome and stress cardiomyopathy, is characterized by transient systolic and diastolic LV dysfunction, with wall motion abnormalities that usually involve the LV apex19. The disease predominantly affects elderly women and is often preceded by an emotional or physical trigger.

The majority of patients with TCM have a clinical presentation that is indistinguishable from an acute coronary syndrome20. In Templin et al., a study which compared features of TCM and ACS in age- and sex-matched cohorts, over 80% of patients diagnosed with TCM had elevated troponins and ECG findings suggestive of myocardial ischemia19. Therefore, early coronary angiography remains necessary to exclude ACS. Although the absence of coronary artery disease was once described as a criterion for the diagnosis of TCM, recent studies have shown that the two disease entities frequently coexist19. Templin et al. also found that levels of brain natriuretic peptide and LV end-diastolic pressure were markedly elevated in TCM, and it should therefore be considered an acute heart failure syndrome. Other notable findings from that study include a greater prevalence of neurologic and psychiatric disorders and a substantial risk for morbidity and mortality in patients with TCM, a condition which was once thought to be benign19.

Severe Aortic Stenosis

Congestive heart failure and LV dysfunction in the setting of severe aortic stenosis are associated with a high mortality rate21. Surgical replacement of the aortic valve can dramatically improve cardiac function and survival. However, proceeding directly to surgery is associated with a high perioperative morality rate in patients who are unstable and have multiple comorbidities21. In critically ill, non-hypotensive patients with severe AS and severe LV systolic dysfunction, vasodilators may improve cardiac output and help to stabilize the patient until surgery21.

Acute Valvular Regurgitation

Acute severe valvular regurgitation is a surgical emergency. Acute regurgitation may occur with any valve, but the left-sided valves are more frequently affected and have greater clinical impact22. In the setting of aortic valve disease, acute regurgitation is most often caused by endocarditis. Other causes include aortic dissection and aortic valve leaflet rupture secondary to blunt trauma.

According to Stout et al., acute mitral regurgitation (MR) may result from either “organic” or “functional” causes22. Organic causes are the result of permanent structural disruption, such as leaflet perforation from endocarditis, chordal rupture in myxomatous valve disease, or papillary muscle rupture due to an MI. These require surgical repair. Functional causes are due to LV abnormalities that result from underlying diseases such as myocardial ischemia or cardiomyopathy. The abnormal LV produces valve insufficiency, which may improve by treating the underlying disease itself.

Although uncommon in industrialized nations, acute rheumatic carditis remains a significant problem in developing countries and can cause acute MR22. In patients with prosthetic valves, acute valve regurgitation is usually due to a tear of a bioprosthetic leaflet or thrombosis of a mechanical valve.

Dilated Cardiomyopathy

DCM is a common and largely irreversible form of heart muscle disease with an estimated prevalence of 1:250014. It is the most frequent cause of heart transplant and the third most common cause of heart failure14. DCM leads to progressive heart failure and a decline in LV contractile function, ventricular and supraventricular arrhythmias, conduction system abnormalities, thromboembolism, and sudden or heart failure-related death. Typical features are biventricular enlargement and global hypokinesis. Typically, the disease manifests in the third or fourth decade, but it can also be seen in young children.

DCM can be acquired or hereditary. Acquired cases may be due to infections; toxins; chronic alcohol consumption; chemotherapeutic agents; metals (i.e. cobalt, mercury, lead, and arsenic); autoimmune and systemic disorders; neuromuscular disorders (e.g., Duchene muscular dystrophy); and mitochondrial, metabolic, endocrine, and nutritional disorders. Approximately 20% to 35% of cases are familial14. Often times the etiology is unknown, but 20% of patients diagnosed with idiopathic DCM have at least one first-degree relative with a decreased ejection fraction and cardiomegaly; excess alcohol consumption has been reported in up to 40% of patients with idiopathic DCM23.

Case Conclusion #1

The patient was admitted to the medical floors for acute exacerbation of HF with reduced EF. Echocardiogram from this hospital stay showed biventricular failure with EF of 29%, moderate LA enlargement, and moderate-to-severe functional mitral regurgitation. The patient was fitted for a LifeVest and ultimately discharged to home with outpatient follow up. Per the patient’s cardiologist, the exacerbation was most likely due to her recent pneumonia or was a manifestation of peripartum cardiomyopathy.

Case Conclusion #2

The patient was admitted for acute decompensated heart failure. His inpatient echocardiogram showed biventricular HF with an EF of 20-25%. He was placed on a milrinone IV drip and multiple diuretics. Eventually the patient was adequately diuresed and discharged to home with outpatient follow up. The patient reported that he was compliant with his medications but later admitted to routinely eating Chinese takeout.

 

Key Points:

-Always address ABCs.

-Rule out more common causes of acute decompensated heart failure first (respiratory illnesses, arrhythmias, renal failure, ACS, uncontrolled HTN, medication non-compliance, and dietary indiscretions).

-Consider reversible causes of HF (e.g., NSAIDs, alcohol) and stop the offending agent.

-There may be more than one precipitant leading to acute decompensated heart failure.

-Always evaluate for ACS.

 

References/Further Reading:

1.     Gheorghiade M, Pang PS. Acute heart failure syndromes. Journal of the American College of Cardiology. 2009 Feb 17;53(7):557-73.

2.     Collins S, Storrow AB, Kirk JD, et al. Beyond pulmonary edema: diagnostic, risk stratification, and treatment challenges of acute heart failure management in the emergency department. Annals of emergency medicine. 2008 Jan 1;51(1):45-57.

3.     Heymans S, Eriksson U, Lehtonen J, Cooper LT. The quest for new approaches in myocarditis and inflammatory cardiomyopathy. Journal of the American College of Cardiology. 2016 Nov 29;68(21):2348-64.

4.     Caforio AL, Pankuweit S, Arbustini E, et al. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. European heart journal. 2013 Jul 3;34(33):2636-48.

5.     Cooper Jr LT. Myocarditis. New England Journal of Medicine. 2009 Apr 9;360(15):1526-38.

6.     Lauer B, Niederau C, Kühl U, et al. Cardiac troponin T in patients with clinically suspected myocarditis. Journal of the American College of Cardiology. 1997 Nov 1;30(5):1354-9.

7.     Morgera T, Di Lenarda A, Dreas L, et al. Electrocardiography of myocarditis revisited: clinical and prognostic significance of electrocardiographic changes. American heart journal. 1992 Aug 1;124(2):455-67.

8.     Ukena C, Mahfoud F, Kindermann I, et al. Prognostic electrocardiographic parameters in patients with suspected myocarditis. European journal of heart failure. 2011 Apr;13(4):398-405.

9.     Friedrich MG, Sechtem U, Schulz-Menger J, et al. Cardiovascular magnetic resonance in myocarditis: A JACC White Paper. Journal of the American College of Cardiology. 2009 Apr 28;53(17):1475-87.

10.  Page RL, O’Bryant CL, Cheng D, et al. Drugs that may cause or exacerbate heart failure: a scientific statement from the American Heart Association. Circulation.2016 Aug 9;134(6):e32-69.

11.  Bhala N, Emberson J, Merhi A, et al. Vascular and upper gastrointestinal effects of non-steroidal anti-inflammatory drugs: meta-analyses of individual participant data from randomised trials. Lancet.2013;382(9894):769-779

12.  Antman EM, Bennett JS, Daugherty A, et al. Use of nonsteroidal antiinflammatory drugs: an update for clinicians: a scientific statement from the American Heart Association. Circulation. 2007 Mar 27;115(12):1634-42.

13.  Kushwaha SS, Fallon JT, Fuster V. Restrictive cardiomyopathy. New England Journal of Medicine. 1997 Jan 23;336(4):267-76.

14.  Maron BJ, Towbin JA, Thiene G, et al. Contemporary definitions and classification of the cardiomyopathies: an American heart association scientific statement from the council on clinical cardiology, heart failure and transplantation committee; quality of care and outcomes research and functional genomics and translational biology interdisciplinary working groups; and council on epidemiology and prevention. Circulation. 2006 Apr 11;113(14):1807-16.

15.  Elkayam U. Clinical characteristics of peripartum cardiomyopathy in the United States: diagnosis, prognosis, and management. Journal of the American College of Cardiology. 2011 Aug 9;58(7):659-70

16.  Beulens JW, Algra A, Soedamah-Muthu SS, et al. Alcohol consumption and risk of recurrent cardiovascular events and mortality in patients with clinically manifest vascular disease and diabetes mellitus: the Second Manifestations of ARTerial (SMART) disease study. Atherosclerosis. 2010 Sep 1;212(1):281-6.

17.  George A, Figueredo VM. Alcoholic cardiomyopathy: a review. Journal of cardiac failure. 2011 Oct 1;17(10):844-9.

18.  Fauchier L, Babuty D, Poret P, et al. Comparison of long-term outcome of alcoholic and idiopathic dilated cardiomyopathy. European heart journal. 2000 Feb 1;21(4):306-14.

19.  Templin, Christian, et al. Clinical features and outcomes of Takotsubo (stress) cardiomyopathy. New England Journal of Medicine 373.10 (2015): 929-938.

20.  Prasad A. Apical ballooning syndrome.Circulation. 2007 Feb 6;115(5):e56-9.

21.  Khot UN, Novaro GM, Popović ZB, et al. Nitroprusside in critically ill patients with left ventricular dysfunction and aortic stenosis. New England Journal of Medicine. 2003 May 1;348(18):1756-63.

22.  Stout KK, Verrier ED. Acute valvular regurgitation. Circulation. 2009 Jun 30;119(25):3232-41.

23.  Dec GW, Fuster V. Idiopathic dilated cardiomyopathy. New England Journal of Medicine. 1994 Dec 8;331(23):1564-75.

 

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