A Case of Adult-Onset Acute Rheumatic Fever With Long-Lasting Atrioventricular Block Requiring Permanent Pacemaker Implantation

Abstract

SummaryA 45-year-old hypertensive Japanese woman presented with epigastric pain on inspiration, fever, complete atrioventricular block and polyarthritis. Her antistreptolysin O levels were markedly elevated. A diagnosis of rheumatic fever was made according to the modified Jones criteria. She was prescribed loxoprofen sodium, which was partially effective for her extracardiac clinical symptoms. However, she had syncope due to complete atrioventricular block with asystole longer than 10 seconds. Consequently, we implanted a permanent pacemaker. Although we prescribed prednisolone, the efficacy of which was limited for the patient's conduction disturbance, the complete atrioventricular block persisted. In our systematic review of 12 similar cases, the duration of complete heart block was always transient and there was no case requiring a permanent pacemaker. We thus encountered a very rare case of adult-onset acute rheumatic fever with persistent complete atrioventricular block necessitating permanent pacemaker implantation. (Int Heart J 2015; 56: 664-667) Key words: Carditis, ASO, Complete atrioventricular block A cute rheumatic fever is an autoimmune disorder following post-suppurative streptococcal pharyngitis, which leads variably to arthritis, chorea, dermal manifestations, and most importantly, carditis. Acute rheumatic fever usually occurs in children between the ages of 5 and 15 years. In Japan and other developed countries, the incidence of acute rheumatic fever has fallen to very low levels due to improved hygienic standards and the routine use of antibiotics for acute pharyngitis. In fact, in Japan, according to a patient survey by the Ministry of Health, Labour and Welfare, it has been too rare a disease to count the total number of patients since 2005.Cases of adult-onset acute rheumatic fever with complete atrioventricular block have been reported in developed countries, though very rarely. It has been reported that patients with adult-onset acute rheumatic fever sometimes develop complete atrioventricular block due to excessive vagal tonus activation. 1)Therefore, most researchers have reported that the dysfunction of the atrioventricular node caused by adult-onset acute rheumatic fever might be transient or reversible.2-7) Against this background, we experienced a very rare case of a patient diagnosed with adult-onset acute rheumatic fever with complete atrioventricular block requiring implantation of a permanent pacemaker. We report the details of this case and discuss the relevant earlier case reports. Case ReportFive days before admission, a 45-year-old Japanese woman who had been receiving antihypertensive therapy (doxazosin 1 mg/day) for 3 years presented with epigastric pain on inspiration. She was admitted to another hospital due to fever with temperatures above 38°C and complete atrioventricular block (65 beats/minute). Neither she nor her family members had any history of upper respiratory tract infection. There was no other history of cardiorespiratory complaints. There was no familial...

The frequency of rhythm and conduction abnormalities and benefits of 24-hour Holter electrocardiogram on detecting these abnormalities in patients with acute rheumatic fever.

“…In our study, incidence of first degree AV block was lower than this previous reports in the literature, whereas the incidence of second and third degree AV block was compatible with the literature. Although complete AV block is transient and recover spontaneously or with antiinflammatory treatment (16), in life-threatening situations such as complete AV block associated syncope, rarely there may be a need for temporary (7,20) and even permanent pacemaker implantation (17). In our study, complete AV block occurred in four patients, one of whom presented syncope, but none of them required any specific medication or pacemaker implantation for this situation and spontaneously recovered with the anti-inflammatory treatment.…”

“…Antistreptolysin-O values or acute phase reactants are not correlated with incidence of arrhythmias or conduction disorders in previously reported studies (17). However, it has been shown that there is a correlation between acute phase reactants and prolonged QT interval (3).…”

Acute rheumatic fever presenting as complete heart block: report of an adolescent case and review of literature

“…Of these, CHB reversed to sinus rhythm in 18 of 21 cases during treatment of acute rheumatic fever and persisted in three cases at 3 months. Temporary pacemaker implantation was necessary in 10 cases and only one patient (an adult) required permanent pacemaker implantation 6. Initial studies have shown that complete AV block during acute rheumatic fever occurs proximal to the bundle of His, and the escape rhythm is usually narrow 1.…”

Akut Romatizmal Ateş Hastalığında Görülen Ritim ve İleti Bozuklukları

“…Akut romatizmal ateş ve tam kalp bloğu bulunan, kalıcı pacemaker takılmak zorunda kalınan 45 yaşındaki hastanın ASO düzeyinin çok yüksek olduğu görülmüş ve bunun, AV nodda ve His huzmesinde oluşan enflamatuvar aktiviteyi gösterebileceği düşünülmüştür. Bu olguyu sunan yazarlar, kız cinsiyetin ve ileri yaşın, AV tam blok gelişiminde etkili olabileceğini öne sürmüşler, ileri yaşta AV tam blokla gelen hastalarda tipik ARA kliniğinin görülmeyebileceğini, ayırıcı tanıda ARA'nın da akla gelmesi gerektiğini ve serum ASO düzeyinin bunun için yardımcı olabileceğini belirtmişlerdir 20 . İleri düzeyde kalp bloklarının standart EKG incelemesinde fark edilmesi mümkün olmayabilir.…”

“…Enfeksiyon nedeniyle geçici pacemakerı çıkarılmak zorunda kalınan hastada tam AV blok devam etmiş ve 10 sn süren ventriküler duraklama ve senkop olması üzerine tedavinin 49. gününde iki odacıklı kalıcı pacemaker takılmıştır. Bildiğimiz kadarıyla bu hasta, kalıcı kalp pili takılan tek ARA hastasıdır 20 . Jones kriterlerinden majör bulguları olan bir hastada ikinci veya üçüncü derece AV blok olduğunda, kapak tutulumu olmasa bile bunun ARA hastalığına bağlı olduğu düşünülebilir 24 .…”