Sudden Unexpected Cardiac Death from Lyme Disease
ABSTRACT & COMMENTARY
Many more cases of Lyme carditis may go unrecognized
By Stan Deresinski, MD, FACP, FIDSA
Clinical Professor of Medicine, Stanford University, Hospital Epidemiologist, Sequoia Hospital, Redwood City, CA, Editor of Infectious Disease Alert
Source: Centers for Disease and Control and Prevention. Three sudden cardiac deaths associated with Lyme carditis United States, November 2012-July 2013. MMWR 2013;62:993-6.
CDC has reported 3 patients with sudden unexpected death in whom myocardial infection due to Borrelia burgdorferii was first detected post-mortem.
- The first case, a Massachusetts resident, was found unresponsive in his automobile and was subsequently pronounced dead. Relatives reported that he had complained of myalgias and arthralgias in the previous 2 weeks. As a potential organ donor, his organs were sent to a tissue bank were examination of his heart revealed evidence of myocarditis. Further examination at CDC identified the presence of spirochetes by immunohistochemistry and Warthin-Starry silver stain while PCR detected B. burgdorferii. Serological studies were consistent with early Lyme disease.
- A man with a history of Wolff-Parkinson-White syndrome collapsed at his home in New York state after complaining of chest pain and attempts at resuscitation were unsuccessful. His organs were sent to the same tissue bank that received those of the first case and, once again, histological examination revealed evidence of panmyocarditis with perivascular lymphoplasmacytic infiltrates. As in the first case, spirochetes were detected by both Warthin-Starry silver stain and immunohistochemistry, with B. burgdorferii detected by PCR. There was also serological evidence of acute infection.
- After complaining of episodic shortness of breath and anxiety for 7-10 days, a Connecticut resident collapsed and died while visiting in New Hampshire. When the medical examiner found evidence of myocarditis, cardiac tissue was sent to CDC where diffuse mixed perivascular lymphoplasmacytic pancarditis, spirochetes were identified, and PCR detected nucleic acid of B. burgdorferii. Serological studies were consistent with acute infection.
Corneas from cases 2 and 3 were transplanted into 2 recipients prior to knowledge of evidence of Lyme infection of the donors. One received doxycycline and had no evidence of transmission and the second died from unrelated causes before antibiotics could be administered.
These 3 patients presumably died an arrhythmic death resulting from their Lyme myocarditis that was undiagnosed during life. Two had underlying cardiac disease — one had Wolff-Parkinson-White syndrome and two had atherosclerosis detected post-mortem. One patient had 7-10 days of respiratory symptoms and one had chest pain just before collapse. None were known to have had a tick bite and none had usual stigmata of Lyme disease such as erythema migrans.
Myocarditis is a rare complication of acute Lyme disease with objective evidence found in approximately 3% of patients with early infection. Most, but not all patients have other clinical evidence of acute Lyme disease such as erythema migrans. Its most common cardiac manifestation is blockage of atrioventricular conduction, which can be of 1st, 2nd, or 3rd degree and which can rapidly change from one degree of severity to another. Symptoms occur a median of 21 days after infection. More severe levels of block generally improves within a week, but complete resolution of the conduction disturbance may last up to 6 weeks. The usual short duration of high degrees of atrioventricular block is such that it can be managed with temporary pacing. When they occur, myocarditis and/or pericarditis is generally mild. Based on some European reports, it has been suggested that the development of a chronic cardiomyopathy may rarely occur.
While the evidence is anecdotal, it has been suggested that patients with cardiac symptoms and/or a markedly prolong PR interval, 2nd degree or 3rd degree atrioventricular block should be hospitalized and initially treated with ceftriaxone. With resolution of high degree block and significant shortening of the PR interval, antibiotic administration may be changed to the oral route with, e.g., doxycycline.
Published reports of mortality from Lyme carditis are very rare. The occurrence of sudden cardiac death in the 3 patients reported by CDC without a diagnosis of Lyme disease during life suggests the possibility that many more cases of Lyme carditis go unrecognized. While in one of the 3 cases the diagnosis resulted from a request by the medical examiner for evaluation of cardiac tissue by CDC, in the other 2, the diagnosis was made by the tissue banks to which the explanted hearts were sent for possible use in transplantation.