An overview of arrhythmogenic

Understanding conditions that can cause this disorder will help nurses educate and care

CNE for patients. 1.5 contact hours By Fidelindo Lim, DNP, CCRN; LEARNING O BJECTIVES Chris Noah Hsiao, BSN, RN; and Navi Johal, BSN, RN, CEN, PMD 1. Compare the causes of arrhythmogenic cardiomyopathy (ACM) as to pathophysiology, clinical course, and management. 2. Discuss nursing implications for patients with ACM.

The authors and planners of this CNE activity have disclosed no rel- evant financial relationships with any commercial companies per- taining to this activity. See the last page of the article to learn how to earn CNE credit.

Expiration: 8/1/24

6 American Nurse Journal Volume 16, Number 8 MyAmericanNurse.com ARAN*, an otherwise healthy 16-year-old Brugada brothers in Spain, is a genetic dis- South Asian male, arrives at the emergency de- ease of the cardiac ion channel function. The partment with paramedics after having a syn- syndrome causes dysfunction in the ion copal episode while watching a soccer game. channels via up or down regulations, de- The initial ECG shows polymorphic ventricular pending on the genetic mutation or variant tachycardia, which requires cardioversion in involved, which can lead to . Pa- the field. Slight ST-elevations in the right pre- tients with the disease can experience syn- cordial leads are noted but no T-wave inver- cope, ventricular tachycardia (VT), ventricu- sions. Aran denies any cardiac history, but a lar fibrillation (VF), cardiac arrest, or SCD. consultation with his parents re- The most common ECG manifestations are veals a family history of sudden cardiac death right bundle branch block (RBBB) and ST- (SCD) and that the father has had peri-synco- segment elevations in the right precordial pal and syncopal episodes. leads. BrS affects more men than women and An electrophysiologic study shows an easily especially those of South Asian or Japanese inducible Type 1 ST-elevation, and pro- descent. El Sayed and colleagues report that grammed electrical stimulation induces ven- overall prevalence is 5:10,000. The disease is tricular tachycardia. Aran is diagnosed with the leading cause of SCD in men under 40 Brugada syndrome (BrS). An implantable car- years and accounts for 12% of all SCD world- diac monitor is placed with plans for an im- wide. (Web resource: SADS.org) plantable cardioverter defibrillator (ICD), pro- phylactic quinidine, regular follow-ups, and Pathophysiology genetic testing. No clear pathophysiology for BrS exists, but genetic links are well established. When the disease was initially discovered, and up until Our understanding of the major risk factors a decade ago, most thought it was a condition of cardiovascular disease—such as diet, elevat- of an otherwise structurally normal . Re- ed blood pressure, high body mass index, in- cent advances in higher resolution–computed creased total cholesterol, high fasting glucose tomography (CT) and cardiac magnetic reso- level, smoking, and sedentary lifestyle—has nance imaging (MRI) have revealed some increased, but little was known about arrhyth- structural abnormalities. However, because no mogenic cardiomyopathy (ACM) until recently. pathognomonic pattern exists, the debate ACM is defined as an arrhythmogenic dis- lingers as to whether the abnormalities cause order of the myocardium not secondary to BrS or are a result of it. ischemic, hypertensive, or valvular heart Genetically, an SCN5A mutation affecting disease. Because of the low prevalence of the sodium channel flow is the most common conditions that lead to ACM, many health- (up to 30%) BrS variant. The mutation causes care providers may not be familiar with a decrease in the early peak current in the car- managing the disease or identifying those at diac muscle and slows or blocks the conduc- risk. In 2019, the Heart Rhythm Society tion rate in parts of the heart, allowing for (HRS) issued a comprehensive consensus wave-break, which leads to arrhythmias. Ac- report on ACM management. This article pro- cording to Brugada and colleagues, other ge- vides an overview of selected genetic (BrS, netic variants affecting the cardiac sodium, left ventricular noncompaction), systemic (sar- potassium, or calcium channel subunits ac- coidosis, cardiac ), infiltrative (car- count for 2% to 5% of inherited BrS cases. Es- diac amyloidosis), and infectious (Chagas dis- timates show that in up to 70% of patients ease) disorders that can lead to ACM. with the inherited disease, no genetic variant Although these conditions may result in a host has been identified. of systemic complications, the discussion will focus on cardiac and the role of Clinical course nurses. BrS is diagnosed through analysis of an ECG. Three ECG patterns have been identified as Brugada syndrome hallmarks of the disease, all having ST eleva- tions in the precordial leads; the most com- BrS, which is named after the cardiologist mon is type 1 coved ST elevation. Under the

MyAmericanNurse.com August 2021 American Nurse Journal 7 HRS guidelines, BrS is confirmed when a type myocardium gradually compacts from the epi- 1 ST elevation is observed either spontaneous- cardium inward. When this fails, LVNC can re- ly or after I.V. administration of a sodium sult. Mutations in MYH7 and MYBPC3 genes channel blocker in at least one right precor- have been found in 30% of patients with dial lead (V1 and V2) combined with at least LVNC. These genes play a role in the function one of the following: documented VF or poly- of sarcomeres, the structure within muscle morphic VT, inducibility of ventricular ar- fibers that causes contraction. rhythmias with programmed electrical stimula- tion, family history of SCD before age 45, Clinical course history of non-vasovagal syncope, or noctur- LVNC diagnosis requires echocardiography or nal agonal breathing. cardiovascular MRI to measure the ratio of Patients with BrS can remain asymptomatic thickness, mass, or volume of the noncom- for life, but those who have symptoms, which pacted layer of the left ventricle to the com- Left can vary from syncope to SCD, frequently pacted layers. If the ratio of X (distance from ventricular start to experience them close to the mean epicardial surface to trough of trabecular re- age of 40 years. Certain factors, such as med- cess) to Y (distance from epicardial surface to noncompaction ication or fever, can induce arrhythmias asso- peak of trabeculation) is <0.5, the patient (LVNC), also ciated with BrS. meets diagnostic criteria. Although some pa- tients are asymptomatic, others may experi- known as Management ence SCD, blood clots, frequent premature honeycomb Treatment options for patients with BrS are ventricular complexes, ventricular tachycar- limited and guided by risk stratification. The dia, palpitation, and exercise intolerance be- myocardium, recommended pharmacologic intervention is cause of the ventricle’s inability to contract is a heart quinidine, which suppresses arrhythmias. De- and relax normally. According to Towbin and finitive treatment is an ICD. New research and Jefferies, two-thirds of individuals with LVNC muscle current medical investigations are showing develop (HF). Except for dilated disorder in promising results in using ablation to stop fre- and hypertrophic cardiomyopathy, LVNC fre- quent arrhythmic storms. quently presents with features similar to those which the left found in pulmonary atresia and intramyocar- ventricle Left ventricular noncompaction dial hematoma. becomes thick Left ventricular noncompaction (LVNC), also Management and spongy, known as honeycomb myocardium, is a heart For the most part, LVNC treatment is symptom- muscle disorder in which the left ventricle be- specific, and HF is the most common reason leaving the comes thick and spongy, leaving the heart for hospitalization. Treatment guidelines for heart weak weak and unable to contract or relax proper- HF should be applied on the basis of the pa- ly. The National Institute of Health estimates tient’s clinical scenario; treatment may include and unable to that LVNC affects eight to 12 patients per 1 beta-blockers, ACE inhibitors, angiotensin-II contract or million annually; however, because of the receptor blockers, diuretics, and aldosterone sometimes-hidden clinical manifestations, its antagonists. Antidysrhythmic , such as relax properly. true prevalence may be underreported. Epi- ICD implantation and ablation, is recommend- demiology is also hampered by the lack of ed for patients with life-threatening dysrhyth- consensus on a diagnostic standard. The con- mia, especially those with nonsustained VT. In dition usually affects the left ventricle, but in patients with atrial fibrillation or presence of 22% to 38% of patients, the pathology is atrial or ventricular clots, oral anticoagulation found in both ventricles. (Web resource: is recommended. The risks and benefits of cardiomyopathy.org/left-ventricular-non heart transplant also should be explored. compaction/intro) Sarcoidosis Pathophysiology Although the exact cause of LVNC is un- Sarcoidosis is a multisystem, inflammatory dis- known, it’s thought to result from a disruption ease that primarily targets the lymph nodes of the compaction process during left ventri- and lungs. The hallmark manifestation is the cle myocardium development. Normally, the accumulation of noncaseating granulomas

8 American Nurse Journal Volume 16, Number 8 MyAmericanNurse.com (nonsolidifying areas of inflammation that AV blocks, QRS complex fragmentation, ST consist of macrophages, epithelioid cells, and T wave changes, pathological Q waves, and mononuclear cells, and CD4+ T cells with a sometimes epsilon waves (a small positive de- few peripheral CD8+ T cells) in these organs. flection “blip” buried at the end of the QRS com- The Mayo Clinic reported a sarcoidosis preva- plex). Echocardiography may reveal myocardial lence of approximately 4.7 to 64 per 100,000 wall thickness and left ventricular hypertrophy, with the highest rates among northern Euro- which resembles hypertrophic cardiomyopathy. peans and Blacks. Heart also are used to determine the According to Birnie and colleagues, the spread of noncaseating granuloma. prevalence of sarcoidosis is notably higher in females (the female-to-male prevalence ratio Management of almost 2:1) and 70% of those affected are Limited randomized controlled trials on the between the ages of 25 to 60 years old. Al- management and treatment of CS exist. Birnie though the etiology of sarcoidosis is unknown, and colleagues developed a helpful treatment studies suggest that genetic predisposition and algorithm. Corticosteroids will help slow in- environmental factors play a role in its develop- flammation and fibrosis progression. Anecdot- ment. (Web resource: stopsarcoidosis.org/) al reports of antimalarial, methotrexate, and azathioprine use for patients who don’t re- Pathophysiology spond to corticosteroids or as steroid-sparing In cardiac sarcoidosis (CS), heart tissue be- alternatives have been documented in the lit- comes infiltrated with sarcoid granulomas; the erature. Permanent pacemaker and ICD also myocardium is the most affected. This condi- are considered for patients with CS. tion leads to three stages of cardiac pathologic features—edema, granulomatous inflamma- tion, and fibrosis—which results in post-in- flammatory scarring. Noncaseating granulomas Cardiac amyloidosis is a form of restrictive car- form in the heart, primarily in the left ventricle, diomyopathy caused by extracellular deposi- making the left ventricular ejection fraction the tion of proteins in the myocardium. The ven- most important mortality indicator. Hulten and tricles become stiff and inflexible, leading to peers estimated that 20% to 25% of those with reductions in filling and, ultimately, cardiac pulmonary/systemic sarcoidosis have asymp- output. Two types of cardiac amyloidosis exist: tomatic cardiac involvement. light-chain amyloidosis (AL) and CS is the leading cause of death (up to 85%) amyloidosis (ATTR), also known as trans - in Japanese patients with sarcoidosis. Gene thyretin cardiomyopathy (ATTR-CM). HLA DQB1*0601 and tumor necrosis factor ATTR is an inherited autosomal dominant trait allele TNFA2 found in Japanese patients are (needs only one mutated gene to be affected shown to predispose them to CS. According to by the condition) caused by pathogenic vari- Birnie and colleagues, angiotensin-converting ants in the transthyretin gene TTR (ATTRv). Ac- enzymes are elevated in 60% of those with sar- cording to Siddiqi and Ruberg, the incidence of coidosis; however, this biomarker lacks sensitiv- AL is 1 per 100,000, resulting in 2,500 to 5,000 ity and specificity for diagnosing the condition. new cases annually in the United States. ATTR prevalence increases with age: 25% of those Clinical course over age 80 have some type of amyloid depo- Sarcoidosis with cardiac involvement has a sition related to ATTR. The condition is rare, poor prognosis (it’s life-threatening in 5% of but according to Gilstrap and colleagues, from patients). CS manifestations largely depend on 2000 to 2012, a notable increase in prevalence noncaseating granuloma location and severity, and incidence rates occurred in the United but some of the primary features include con- States, particularly among older Black men. duction abnormalities and arrhythmias (such (Web resource: bit.ly/35mQGjh) as AV block), HF, and SCD. No diagnostic gold standard for CS exists, Pathophysiology but fluorodeoxyglucose positron emission to- Unstable proteins are deposited in the my- mography of the heart may reveal inflammatory ocardium, where they misfold and aggregate lesions in the myocardium. ECG may show to become amyloid fibrils. In cardiac amyloi-

MyAmericanNurse.com August 2021 American Nurse Journal 9 dosis, the pathology is mainly misfolded mon- sis of TTR, reducing TTR protein levels. oclonal immunoglobulin light chains (ALs) Experts suggest that cardiac amyloidosis from an abnormal clonal proliferation of plas- should be considered in the differential diag- ma cells or transthyretin (TTR), a liver-synthe- nosis of patients ≥65 years with new onset HF. sized protein involved in the transportation Consultation with a neurologist should be of thyroxine and retinol-binding protein. Left considered if neurologic involvement is evi- ventricular hypertrophy is a key feature of car- dent or suspected. diac amyloidosis, so left ventricular wall thick- ness ≥14 mm usually warrants evaluation for Chagas disease the condition. Named after the Brazilian Carlos Clinical course Chagas, this zoonotic disease is caused by Echocardiogram, cardiac MRI, and bone the protozoan parasite Trypanosoma cruzi, scintigraphy are used to diagnose ATTR-CM; which is transmitted to humans by blood- Named after Kittleson and colleagues developed a testing sucking triatomine bugs. Less common algorithm. Most patients have signs and symp- modes of transmission are via blood product the Brazilian toms of HF, such as dyspnea, , and pe- transfusion, , consump- physician ripheral edema. In patients with HF, clinical tion of food or beverages contaminated by features that warrant evaluation for ATTR-CM the vector or vector feces, and from mother Carlos include intolerance to antihypertensive or HF to fetus. Chagas disease isn’t endemic in the Chagas, this medication, persistent low-level elevation in United States. According to Montgomery and serum troponin, discordance between QRS colleagues, the approximately 300,000 chron- zoonotic voltage on an ECG, wall thickness on imag- ic Chagas disease cases in the United States disease is ing, unexplained AV block or left ventricular are among people originally from Latin wall thickening, and family history of car- American countries (Brazil, Bolivia, Argenti- caused by the diomyopathy. On the other hand, AL tends to na) where the disease is endemic. Between protozoan be more symptomatic before left ventricular 1955 and 2015, only 28 confirmed cases of wall thickening. In addition, heart murmurs local transmission within the United States parasite might be detected because ATTR myocardial were documented. (Web resource: who.int/ Trypanosoma deposition has been noted in about a quarter health-topics/chagas-disease#tab=tab_1) of patients with degenerative aortic stenosis. cruzi, which is Although imaging may create suspicion for Pathophysiology transmitted to amyloidosis, a cardiac is still the gold How Chagas disease causes cardiomyopathy standard for diagnosis. isn’t well understood. Various explanations in- humans by clude antigens that persist in the heart, parasite- blood-sucking Management mediated myocytolysis, damage to cardiac mi- Cardiac amyloidosis treatment focuses on crovasculature, -mediated cytotoxicity, triatomine managing HF and dysrhythmias and initiating and nonspecific damage caused by eosinophils bugs. disease-modifying agents. In symptomatic and and neutrophils. A combination of these process- asymptomatic individuals with cardiac amyloi- es may lead to cardiomegaly, chronic myocardi- dosis and second-degree AV block type II, tis, and conduction system damage. high-grade AV block or third-degree AV block, Notable rhythm abnormalities are RBBB a permanent pacemaker is recommended. In and left anterior fascicular block. Years to patients with cardiac amyloidosis and sympto- decades after the acute infection, the patient matic atrial arrhythmias, sotalol or amiodarone might experience ventricular dysrhythmias, si- may be considered. Cardiac ablation is an op- nus node dysfunction and bradycardia, per- tion for those with cardiac amyloidosis and sistent or intermittent complete heart block, symptomatic atrial arrhythmias. an apical aneurysm (usually in the left ventri- Disease-modifying agents are used as cle), thromboembolic events, and progressive TTR stabilizers and silencers. Stabilizers are dilated cardiomyopathy. non steroidal anti-inflammatory agents (such as tafamidis) that help prevent misfolding Clinical course and deposition of amyloid fibrils. Silencers Although acute illness can last a few weeks or (such as patisiran) target the hepatic synthe- months, Chagas disease typically is undiag-

10 American Nurse Journal Volume 16, Number 8 MyAmericanNurse.com nosed because symptoms are absent or mild and nonspecific (for example, fever and anorexia) even with a high degree of para- sitemia. Diagnosis is confirmed during the acute phase via visualization of the parasite in a blood smear and during the chronic phase via immunoglobulin G serology. In most cases, a prolonged chronic phase, in which the patient is asymptomatic and the parasitemia eventually resolves, follows the acute phase. About 20% to 30% of patients might develop life-threatening medical condi- tions, such as cardiomyopathy.

Management Chagas disease can result in dilated cardiomy- opathy, which ultimately can lead to HF. Treatment guidelines for HF are used for Nurses’ role in testing symptom management. Routine laboratory tests and cardiac biomarkers, ECG, and chest Nurses can help explain testing, interpret results, and provide support to anxious patients and families. x-rays are integral to patient care. During the acute phase, the patient should be monitored Standard lab testing. Complete blood count, metabolic panel, liver for meningitis and encephalitis. function tests, lipid profile, and diabetes and heart failure markers (for Heart transplant is an option for some pa- example, brain natriuretic peptide) establish a baseline and indicate tients with end-stage cardiomyopathy, al- treatment effectiveness trends. Nurses can explain the various tests though the patient should be monitored for and put results into context. signs of reactivation of the disease (such as Genetic testing. Nurses can help patients understand procedures and acute myocarditis) in the transplanted heart. results and explain how testing can be used for risk stratification or Reactivation of Chagas disease also is a risk management. among patients who are immunosuppressed (for example, those receiving ) Cardiac studies. Diagnosis by exclusion might require the patient to with the potential for cardiomyopathy and undergo several procedures—from noninvasive (ECG, echocardiogram, meningoencephalitis. and cardiac imaging) to invasive (electrophysiology studies and en- efforts related to Chagas dis- domyocardial biopsy). Nurses can explain pre- and post-testing expec- ease prevention focus on vector eradication in tations to patients and help allay their anxiety. With 12-lead ECGs and countries where the disease is endemic. Since Holter monitoring, nurses can ensure correct lead placement to mini- mize artifacts and variability. In addition, nurses should have advanced 2007, blood donor screening for T. cruzi has ECG interpretation skills. become routine.

Nursing implications RNs and nurse practitioners are integral mem- agnosing ACM. Obtain a detailed three-gener- bers of interdisciplinary teams that care for ation medical history looking for patterns of patients with ACM. They’re involved in com- genetic and nongenetic causes (such as SCD) prehensive patient management using evi- of ACM. Don’t rush the history and use a med- dence-based interventions and protocols across ical interpreter as needed. A diagnostic algo- all levels of care and transitions. In addition, rithm (jamanetwork.com/journals/jama/full they should be able to answer patient ques- article/209410) can help exclude ischemic tions about exercise, exercise-induced ar- heart disease, hypertensive heart disease, and rhythmias, dietary modification (as they relate valvular heart disease as causes of cardiomy- to overall health and medication interaction), opathy. Some , such as those for car- sexual activity, pregnancy, and travel. diac amyloidosis, are most effective when administered before significant cardiac dys- Patient history function occurs, highlighting the importance A patient-centered interview is critical for di- of early identification.

MyAmericanNurse.com August 2021 American Nurse Journal 11 Testing of sufficient health insurance coverage, and Diagnostic testing includes standard lab tests, other socioeconomic challenges can increase genetic tests, and cardiac studies. (See Nurses’ the burden of ACM. As a member of the care role in testing.) team, offer emotional support to patients and their caregivers and provide referrals to sup- Pacemaker and ICD port groups. If a patient with ACM (such as those with car- diac amyloidosis or BrS) requires a permanent Aran pacemaker or ICD, implement best practices After the initial episode that led to his diagno- across the perioperative continuum and fol- sis with BrS, Aran remains symptom-free for low up. For example, after pacemaker or ICD 8 years. At 24, he has influenza and fever, placement, resume cardiac medications as which induces a syncopal episode with poly- prescribed, warn the patient to avoid heavy morphic VT that requires cardioversion. Ge- lifting on the same side as the device, and in- netic testing confirms a TRPM4 mutation. struct the patient to notify their provider if Nocturnal bradycardia is noted during two they feel a “shock.” If you care for patients hospitalizations. After ICD placement at age with an ICD or pacemaker who have 28, Aran has a single shockable event but that requires electrocautery, remember that otherwise remains healthy with occasional the ground return pad should be placed as far asymptomatic bradycardia at night. After a from the device and as close to the surgical non-cardiac related surgery, Aran develops RNs and nurse site as possible. type 1 BrS ECG changes (coved ST elevation), most likely due to the use of propofol. He’s practitioners Symptom management and now 30 years old and remains free of any are integral medications BrS-associated complications and has regu- Most patients with ACM take multiple med- lar outpatient EP follow-up. members of ications to manage symptoms and prevent complications. Medications may include Increased understanding interdisciplinary anticoagulants, steroids, non-steroidal anti- ACMs are low-frequency, high-stakes condi- teams that inflammatory agents, chemotherapy, disease- tions that include a host of genetic, systemic, care for modifying agents, antihypertensives, diuret- infectious, and inflammatory disorders. With ics, and antidysrhythmics. Beta-blocker therapy advances in science and technology, under- patients is recommended in patients experiencing in- standing of ACM continues to increase, lead- with ACM. appropriate ICD shocks. Where applicable, the ing to the development of consensus state- 2017 guidelines for heart failure manage- ments and treatment algorithms to improve ment should be followed. outcomes. Because the conditions associated Medication reconciliation is key to prevent- with ACM are relatively rare, patients are most ing drug–drug interactions, prescription cas- likely seen in specialty cardiac service lines. cade, and polypharmacy. Teach patients and Nurses who work in acute care, coronary caregivers how to accurately monitor blood care, and primary care settings should devel- pressure and heart rate at home. op clinical skills to comprehensively manage and support these patients and their care- Referral givers. AN As a patient advocate, you can help navigate referrals to multiple providers with expertise Fidelindo Lim is a clinical associate professor at New York Universi- in genetics, cardiology, , neurol- ty Rory Meyers College of Nursing in New York City, New York. Chris ogy, and other modalities. Patients also might Noah Hsiao and Navi Johal are staff nurses at New York University require physical and occupational therapy and Langone Health in New York City. home health assistance. Close collaboration with a case manager can make this care coor- *Name is fictitious.

dination more manageable. References Arbustini E, Favalli V, Narula N, Serio A, Grasso M. Left Psychosocial support ventricular noncompaction: A distinct genetic cardiomy- Living with the prospect of SCD can be im- opathy? [published correction appears in J Am Coll Car- mensely unsettling. In addition, disability, lack diol. 2016;68(16):1821]. J Am Coll Cardiol. 2016;68(9):

12 American Nurse Journal Volume 16, Number 8 MyAmericanNurse.com 949-66. doi:10.1016/j.jacc.2016.05.096 From discovery to a worldwide health problem. Front Public Health. 2019;7:166. doi:10.3389/fpubh.2019.00166 Benjamin EJ, Muntner P, Alonso A, et al. Heart disease and stroke statistics–2019 update: A report from the Lopez-Vé leź R, Norman FF, Bern C. American trypanoso- American Heart Association [published correction ap- miasis (Chagas disease). In: Ryan ET, Hill DR, Solomon pears in Circulation. 2020;141(2):e33]. Circulation. 2019; T, Aronson N, Endy TP, eds. Hunter’s Tropical 139(10):e56-528. doi:10.1161/CIR.0000000000000659 and Emerging Infectious Diseases. 10th ed. London, UK: Bern C. Chagas’ disease. N Engl J Med. 2015;373(19): Elsevier; 2020: 762-5. 1881-2. doi:10.1056/NEJMc1510996 MedlinePlus. Left ventricular noncompaction. ghr.nlm Bern C, Messenger LA, Whitman JD, Maguire JH. 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Catheter ablation NBK519568 for atrial arrhythmias in patients with cardiac amyloido- Gilstrap LG, Dominici F, Wang Y, et al. Epidemiology of sis. J Cardiovasc Electrophysiol. 2016;27(10):1167-73. cardiac amyloidosis–associated heart failure hospitaliza- doi:10.1111/jce.13046 tions among fee-for-service Medicare beneficiaries in the Towbin JA, Jefferies JL. due to left United States. Circ Heart Fail. 2019;12(6):e005407. ventricular noncompaction, mitochondrial and storage doi:10.1161/CIRCHEARTFAILURE.118.005407 diseases, and inborn errors of metabolism. Circ Res. Global Burden of Cardiovascular Diseases Collaboration, 2017;121(7):838-54. doi:10.1161/CIRCRESAHA.117.310987 Roth GA, Johnson CO, et al. The burden of cardiovascu- lar diseases among US states, 1990-2016. JAMA Cardiol. 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MyAmericanNurse.com August 2021 American Nurse Journal 13

POST-TEST • An overview of arrhythmogenic cardiomyopathy CNE: 1.5 contact hours CNE Earn contact hour credit online at myamericannurse.com/arrhythmogenic-cardiomyopathy

Provider accreditation Contact hours: 1.5 The American Nurses Association is accredited as a provider ANA is approved by the California Board of Registered of nursing continuing professional development by the Nursing, Provider Number CEP17219. American Nurses Credentialing Center’s Commission on Post-test passing score is 80%. Accreditation. Expiration: 8/1/24

Please mark the correct answer 5. Which cause of arrythmogenic car- 9. Nurses caring for patients with online. diomyopathy (ACM) primarily targets suspected or diagnosed ACM should except: 1. Your patient’s ECG shows right the lungs and lymph nodes? do all of the following bundle branch block and ST-segment a. Chagas disease a. obtain a two-generation medical elevations in leads V1 and V2. This b. Sarcoidosis history. could indicate c. LVNC b. explain how genetic testing can a. Chagas disease. d. BrS be used. b. sarcoidosis. c. perform medication reconcilia- c. left ventricular noncompaction 6. Which of the following would tion. (LVNC). prompt you to suggest your patient d. provide referrals to support with heart failure be evaluated for d. Brugada syndrome (BrS). groups. transthyretin cardiac amyloidosis 2. Which of the following is caused (ATTR-CM)? 10. The definitive treatment for BrS is by a protozoan parasite? a. Intolerance to antihypertensive a. a pacemaker. a. Chagas disease medications b. an aldosterone antagonist. b. Sarcoidosis b. Decreased troponin levels c. an implantable cardioverter c. LVNC c. Left anterior fascicular block on defibrillator. ECG d. BrS d. a beta-blocker. d. Family history of stroke 3. What is the most common reason 11. Mutations in MYH7 and MYBPC3 for patients with LVNC to require hos- 7. Your patient’s ECG shows epsilon genes have been found in 30% of pitalization? waves, which may indicate patients with a. Ventricular tachycardia a. Chagas disease. a. sarcoidosis. b. Atrial fibrillation b. cardiac amyloidosis. b. Chagas disease. c. Heart failure c. sarcoidosis. c. LVNC. d. Stroke d. BrS. d. BrS.

4. Which statement about the patho- 8. Which statement about the treat- physiology of cardiac amyloidosis is ment of ATTR-CM cardiac amyloidosis correct? is correct? a. Amyloid granulomas form in the a. Cardiac ablation is contraindicat- myocardium tissue, primarily in ed. the left ventricle. b. Patisiran is used to manage ven- b. Amyloid granulomas form in the tricular arrhythmias. myocardium tissue, primarily in c. Anti-inflammatory agents are the right ventricle. used to target hepatic synthesis c. Unstable carbohydrates in the of TTR. myocardium misfold and aggre- d. Treatment includes both stabiliz- gate to become amyloid fibrils. ers and silencers. d. Unstable proteins in the myo - cardium misfold and aggregate to become amyloid fibrils.

14 American Nurse Journal Volume 16, Number 8 MyAmericanNurse.com