Abstract
Lyme carditis most commonly manifests as cardiac conduction abnormalities, specifically high-degree atrioventricular block, which may require temporary pacing in conjunction with early intravenous antibiotic therapy. The Suspicious Index in Lyme Carditis (SILC) score has been developed to assess the likelihood that a patient with atrioventricular block has Lyme carditis. Other manifestations of Lyme disease include endocarditis, myocarditis, pericarditis, myopericarditis, pancarditis, and dilated cardiomyopathy. Antibiotics are the mainstay of treatment for Lyme carditis, but cardiovascular dysfunction should be treated according to guidelines for non-lyme disease patients.
Access provided by Autonomous University of Puebla. Download chapter PDF
Similar content being viewed by others
Keywords
1 Introduction
Lyme disease (LD) is a tick-borne illness caused by pathogenic species of the Borrelia genus, most commonly Borrelia burgdorferi (B. burgdorferi). In Europe, other genospecies such as B. afzelli and B. garinii cause significant human disease and are associated with chronic skin infection and neurologic disease, respectively [1]. For further details on the epidemiology of LD, refer to Chap. 2.
The systemic manifestations of LD vary widely and include cardiovascular, neurological, and joint involvement [2]. Lyme carditis (LC) refers to LD manifestations involving the heart. LC most commonly presents as high-degree atrioventricular block (AVB) in 90% of cases [3, 4]. However, endocarditis, myocarditis, pericarditis, myopericarditis, pancarditis, and dilated cardiomyopathy (DCM) have also been reported [5, 6]. Thus, practitioners in regions of high prevalence must be able to detect and treat LC. An overview of the diagnosis and management of LC is presented in this chapter (Table 1). The presentation of LC in the pediatric population is discussed in Chap. 11.
2 Signs and Symptoms
Patients with LC and conduction abnormalities may present with cardiac symptoms including pre-syncope, syncope, and palpitations, but also non-specific findings such as erythema migrans (50%), fatigue (40%), and/or fever (28%). Other patients may be completely asymptomatic. [3, 4] The non-specific nature of these symptoms contributes to the uncertainty when detecting LC (Table 2).
Similarly, patients with myocarditis, pericarditis, myopericarditis, and pancarditis may be asymptomatic or report chest pain, syncope, dyspnea, and other symptoms of cardiac dysfunction [7, 8]. Notably, myopericarditis related to LD in particular can present with symptoms mimicking acute coronary syndrome (ACS) [10].
Lyme endocarditis should be considered for cases of endocarditis in endemic regions with no other identifiable cause, as well as endocarditis in the context of a recently reported tick bite. Signs and symptoms of LD endocarditis are non-specific, and patients may present with increasing fatigue, palpitations, dyspnea, intermittent fevers, anorexia, unproductive cough, or be asymptomatic [6, 9,10,11].
Emerging literature suggests that DCM may be a rare manifestation of LC, occurring in the late disseminated stage of LD months to years after initial infection. Significantly, severe DCM can progress to heart failure and subsequent transplantation. Patients present with typical symptoms of DCM, such as reduced exercise capacity, dyspnea, and chest pain, although some may be asymptomatic [12].
3 Diagnosis
3.1 Conduction Abnormalities
The most common conduction abnormality is AVB, which typically occurs in the first two months after LD exposure but may be seen as early as the first week after infection [2]. AVB can develop transiently but when present, can progress rapidly to life-threatening rhythm disturbances. Accordingly, patients are at risk of developing a fatal third-degree block without early antibiotic intervention [13, 14]. The Suspicious Index in Lyme Carditis (SILC) score has been developed to assess the likelihood that a patient presenting with AVB has LC (Chap. 7). Risk factors used in this scoring tool can be remembered with the mnemonic CO-STAR: Constitutional symptoms, Outdoor activity/endemic area, Sex (LC more likely in males), Tick bite, Age, and Rash. Patients are classified as low risk (0–2), intermediate risk (3–6), or high risk (7–12), as demonstrated in Table 3 (sensitivity 93.2%)[3, 4].
Other conduction abnormalities seen in LC include intra-atrial block, supraventricular tachycardia, sinus node disease and dysfunction, ventricular and atrial fibrillation, bundle branch block, and ventricular tachycardia. An electrocardiogram (ECG) should be considered in all patients with LD.
Myocarditis , Myopericarditis, Pericarditis, and Pancarditis
ECG changes in patients with myocarditis, pericarditis, myopericarditis, and pancarditis may include abnormal left ventricular depolarization and repolarization, manifesting as ST segment and T wave abnormalities. Comparison with a prior baseline ECG is helpful to detect new conduction abnormalities [7, 8]. In up to 60% of LC patients, ST changes and T wave inversion are seen primarily in the inferolateral leads, with rare elevation of serum cardiac biomarkers [7, 8, 15]. Patients with myopericarditis related to LD may have ECG changes mimicking an acute myocardial infarction and elevated cardiac troponin levels [10]. In these patients, a troponin is indicated to assess for myocardial injury. It is also essential that ACS be ruled out with coronary angiography.
When myocarditis and/or pericarditis is suspected, appropriate cardiac imaging should be pursued. A chest x-ray should be used to look for alternative diagnoses and to assess for cardiomegaly. All patients should have an echocardiogram to assess cardiac function parameters such as ejection fraction, pericardial fluid accumulation, and wall motion abnormalities. Diffuse ventricular hypokinesis, associated with decreased ejection fraction, may be seen with myocarditis rather than the regional wall motion abnormalities which are expected in ACS. [5] Cardiac magnetic resonance imaging may help to characterize ventricular functional parameters, wall edema secondary to inflammation (i.e. late gadolinium enhancement), and signs of pericarditis (i.e. pericardial thickening and effusion)[4]. Endomyocardial biopsy (EMB) is the gold-standard for diagnosing myocarditis; however, due to the inherent risks of tissue sampling, EMB is only currently recommended for cases which do not respond to antibiotic management to clarify diagnosis and degree of tissue damage [16].
3.2 Endocarditis
Classic findings of endocarditis include new heart murmur, new valvulopathy on echocardiography, and positive blood cultures [17, 18]. Comorbidities, such as immunosuppression, diabetes and indwelling catheters may increase the risk of contracting endocarditis during LD. [9] Lyme endocarditis has previously been detected in patients with existing structural abnormalities including mitral valve prolapse and regurgitation, bicuspid aortic valve, and tricuspid valve regurgitation [6, 10, 11].
Lyme endocarditis may also occur with accompanying ECG changes, such as AV block [19]. If surgical intervention is required, tissue DNA taken from the valve of interest should be tested with real-time polymerase chain reaction (PCR), as blood and tissue culture may be negative. Limitations of tissue and serological evaluation include the low sensitivity of ELISA in the early stages of LD, persistence of LD seropositivity in resolved infections, susceptibility to cross-reaction with non-LD antibodies, and subjective nature of Western immunoblot assay interpretation. Thus, diagnosis initial should be confirmed with a second test (16S rRNA PCR and sequencing, ELISA, and/or Western blot) [10, 11, 18, 19].
3.3 Dilated Cardiomyopathy
Echocardiography is the mainstay investigation to diagnose DCM once LD has been confirmed. Findings include ventricular dilation and systolic dysfunction characterized by an ejection fraction below 40%. Abnormalities may also be seen on ECG, including indicators of hypertrophy, bundle branch block, left axis deviation, and ventricular dysrhythmias [20]. LC-DCM is further discussed in Chap. 14.
4 Management
Due to a paucity of literature on LC, there are no validated guidelines for management. Typically, temporary pacing and supportive therapy are the mainstays of treatment of patients with symptomatic high-degree AVB. In LC, AVB can spontaneously reverse and treatment predominantly involves the early administration of appropriate antibiotic therapy [4]. Permanent pacemakers are rarely required as most cases of LC resolve with appropriate antibiotic therapy, underscoring the critical importance of early detection and management. Pacemaker insertion has inherent risks, requires lifelong follow-up, and introduces unnecessary cost to the healthcare system [4, 21]. Given the aforementioned, we present a previously published algorithm for the diagnosis and management of LC (Fig. 1) [4].
4.1 SILC Score and Antibiotic Therapy
Patients with a low SILC score should receive antibiotic therapy upon confirmation of Lyme serology. In cases of intermediate to high SILC score, antibiotic therapy should be initiated immediately, while awaiting Lyme serology results [4]. Most commonly, an intravenous course of ceftriaxone is required until the resolution of acute symptoms and conduction abnormalities (typically 10–14 days), followed by an oral antibiotic, such as doxycycline, on discharge for a total duration of 14–21 days [11, 17, 18]. The use of intravenous amoxicillin and gentamicin for two weeks, followed by a month-long course of oral amoxicillin, has also been effective in a minority of cases [10, 22]. The majority of LC cases will resolve with antibiotic treatment of LD. Antibiotic regimens are summarized in Table 4. For patients with high-degree AVB, within the first 14 days of antibiotic therapy, they typically progress to Wenckebach second-degree block, then to first-degree block, decreasing PR interval, and finally to a normal rhythm (Fig. 2). [4, 5, 23, 24].
4.2 Pacing for High Degree AVB
Temporary pacing is only indicated for patients with symptomatic or high-risk bradycardia. In these cases, standard transvenous temporary or modified temporary-permanent transvenous pacing can be utilized. In order to encourage early ambulation for hospitalized patients with LC, the modified temporary-permanent transvenous pacing device uses an active fixation lead, attached to a re-sterilized permanent pacemaker generator taped to the skin as an external device [4, 5, 25, 26]. Once AV conduction has been restored, an exercise stress test should be conducted to ensure 1:1 AV conduction at a heart rate of > 120 bpm. Permanent pacemaker is only recommended if 1:1 AV conduction is not restored at 14 days after admission. Patients with a point of Wenckebach at > 90 bpm should receive a follow-up ECG four to six weeks post-discharge. If the point of Wenckebach occurs at a heart rate of < 90 beats per minute, permanent pacemaker should be considered. [4] Temporary-permanent pacing in Lyme carditis is discussed further in Chap. 9. If patients undergo implantation of a permanent pacemaker, follow-up with a cardiac device clinic is necessary to screen for restoration of normal AV conduction and consideration of device explanation, which is detailed in Chap. 13.
4.3 Additional Considerations
Generally, the management of patients with complicated LC should follow guidelines for cardiac patients without LD. For example, in case of pericarditis progressing to cardiac tamponade, pericardiocentesis should be performed. Patients with pericarditis should also be treated with anti-inflammatory medications including colchicine and non-steroidal anti-inflammatories drugs (NSAIDs) with careful consideration of additional gastric protection medications. In severe cases of LD endocarditis, surgical valve replacement may be required [11, 19, 27]. Indications for surgical valve replacement are clearly outlined in societal valve guidelines [28, 29]. All patients with LD endocarditis require early involvement of a multi-disciplinary team which includes cardiac surgery, infectious disease specialists and allied health professionals. For severe or refractory post-surgical cases of endocarditis, ongoing outpatient intravenous ceftriaxone should be considered and re-operation may be required [6, 11, 30]. Patients with ventricular dysfunction related to myocarditis or dilated cardiomyopathy should be seen by a heart failure specialist for education, optimization of guideline-directed heart failure therapy and monitoring of volume status.
5 Conclusion
Patients living in LD endemic regions are at risk for LC-related morbidity and mortality. The primary presentation of LC is AVB, but several other LC manifestations may impact LD patients, including myocarditis, pericarditis, and endocarditis. LC may also play a role in the development of DCM. Practitioners treating patients from endemic regions with symptomatic LD or cardiovascular symptoms without identifiable etiology should apply the SILC score. Antibiotics are the mainstay of treatment, but cardiovascular dysfunction should be treated according to guidelines for non-LD patients. Early detection and intervention are crucial to prevent long-term cardiovascular damage.
References
Kugeler KJ, et al. Estimating the Frequency of lyme disease diagnoses, United States, 2010–2018. Emerg Infect Dis. 2021;27(2):616–9.
Shapiro ED, Wormser GP. Lyme disease in 2018: what is new (and what is not). JAMA. 2018;320(7):635–6.
Besant G et al. Suspicious index in Lyme Carditis (SILC): systematic review and proposed new risk score. Clin Cardiol. 2018.
Yeung C, Baranchuk A. Diagnosis and treatment of Lyme carditis: JACC review topic of the week. J Am Coll Cardiol. 2019;73(6):717–26.
McAlister HF, et al. Lyme carditis: an important cause of reversible heart block. Ann Intern Med. 1989;110(5):339–45.
Haddad O, et al. Mitral Valve endocarditis: a rare manifestation of Lyme disease. Ann Thorac Surg. 2019;108(2):e85–6.
Munk PS, Orn S, Larsen AI. Lyme carditis: persistent local delayed enhancement by cardiac magnetic resonance imaging. Int J Cardiol. 2007;115(3):e108–10.
Cox J, Krajden M. Cardiovascular manifestations of Lyme disease. Am Heart J. 1991;122(5):1449–55.
Njie AB, Mitchell M, Pukkila-Worley R. Peripherally inserted central catheter-associated nocardia nova endocarditis in a patient receiving intravenous antibiotics for chronic lyme disease. Open Forum Infect Dis. 2021;8(3):ofab041.
Hidri N, et al. Lyme endocarditis. Clin Microbiol Infect. 2012;18(12):E531–2.
Fatima B, Sohail MR, Schaff HV. Lyme Disease-an unusual cause of a mitral valve endocarditis. Mayo Clin Proc Innov Qual Outcomes. 2018;2(4):398–401.
Motamed M et al. Disseminated Lyme disease and dilated cardiomyopathy: a systematic review. Trends Cardiovasc Med. 2022.
Centers for Disease C and Prevention. Three sudden cardiac deaths associated with Lyme carditis—United States, November 2012–July 2013. MMWR Morb Mortal Wkly Rep. 2013;62(49):993–6.
Muehlenbachs A, et al. Cardiac Tropism of Borrelia burgdorferi: an autopsy study of sudden cardiac death associated with Lyme Carditis. Am J Pathol. 2016;186(5):1195–205.
Steere AC, et al. Lyme carditis: cardiac abnormalities of Lyme disease. Ann Intern Med. 1980;93(1):8–16.
Radesich C et al. Lyme carditis: from pathophysiology to clinical management. Pathogens. 2022;11(5).
Kameda G, et al. Diastolic heart murmur, nocturnal back pain, and lumbar rigidity in a 7-year girl: an unusual manifestation of lyme disease in childhood. Case Rep Pediatr. 2012;2012:976961.
Patel LD, Schachne JS. Lyme Carditis: a case involving the conduction system and mitral valve. R I Med J (2013). 2017;100(2):17–20.
Canver CC, et al. Possible relationship between degenerative cardiac valvular pathology and lyme disease. Ann Thorac Surg. 2000;70(1):283–5.
Bozkurt B, et al. Current diagnostic and treatment strategies for specific dilated cardiomyopathies: a scientific statement from the American heart association. Circulation. 2016;134(23):e579–646.
Khalil S, et al. Lyme Carditis in the fast lane: from alternating bundle branch block to Asystole in 12 hours. Conn Med. 2015;79(9):517–20.
Nikolic A, et al. Lyme Endocarditis as an emerging infectious disease: a review of the literature. Front Microbiol. 2020;11:278.
Krause PJ, Bockenstedt LK. Cardiology patient pages. Lyme disease and the heart. Circulation. 2013;127(7):e451–4.
Sangha M, Chu A. Permanent pacemaker avoided in high grade atrioventricular block (abstr). J Am Coll Cardiol. 2018;71(11 Supplement):A363.
Fuster LS, Gul EE, Baranchuk A. Electrocardiographic progression of acute Lyme disease. Am J Emerg Med. 2017;35(7):1040 e5–1040 e6.
Wang C, et al. Treating Lyme carditis high-degree AV block using a temporary-permanent pacemaker. Ann Noninvasive Electrocardiol. 2019;24(3):e12599.
Rudenko N, et al. Detection of Borrelia bissettii in cardiac valve tissue of a patient with endocarditis and aortic valve stenosis in the Czech Republic. J Clin Microbiol. 2008;46(10):3540–3.
Writing Committee M, et al. 2020 ACC/AHA Guideline for the management of patients with valvular heart disease: a report of the American college of cardiology/American heart association joint committee on clinical practice guidelines. J Am Coll Cardiol. 2021;77(4):e25–197.
Pettersson GB, Hussain ST. Current AATS guidelines on surgical treatment of infective endocarditis. Ann Cardiothorac Surg. 2019;8(6):630–44.
Paim AC, et al. Lyme endocarditis. Am J Med. 2018;131(9):1126–9.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2023 The Author(s), under exclusive license to Springer Nature Switzerland AG
About this chapter
Cite this chapter
Liblik, K., Motamed, M., Yeung, C., Wamboldt, R., Baranchuk, A. (2023). Diagnosis and Management of Lyme Carditis. In: Baranchuk, A., Wamboldt, R., Wang, C.N. (eds) Lyme Carditis. Springer, Cham. https://doi.org/10.1007/978-3-031-41169-4_8
Download citation
DOI: https://doi.org/10.1007/978-3-031-41169-4_8
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-031-41168-7
Online ISBN: 978-3-031-41169-4
eBook Packages: MedicineMedicine (R0)