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Case report
Secondary syphilis presenting with aortitis and coronary ostial occlusion
  1. John A Chadwick1,
  2. Anita MacNab1,
  3. Jaydeep Sarma1,
  4. Simon Ray1,
  5. Isaac Kadir2,
  6. Eavan G Muldoon3,4
  1. 1Department of Cardiology, University Hospital of South Manchester, UK
  2. 2Department of Cardiothoracic Surgery, University Hospital of South Manchester, UK
  3. 3Department of Infectious Diseases, University Hospital of South Manchester, UK
  4. 4The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK
  1. Correspondence to Dr Eavan G Muldoon, University Hospital of South Manchester, Southmoor Road, Manchester M23 9LT, UK; eavan.muldoon{at}uhsm.nhs.uk

Abstract

Aortitis is an established manifestation of tertiary syphilis. We report a rare case of aortitis with ostial occlusion and left ventricular failure in secondary syphilis. Her management required a true multidisciplinary approach from multiple specialities due to complications of concomitant psychosis and a history of anaphylaxis to penicillin. This case illustrates the complexities of diagnosing and managing a rare presentation of this increasingly prevalent infection.

  • SYPHILIS
  • CLINICAL CARE (GENERAL)
  • PENICILLIN

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Case history

A 35-year-old woman was admitted with acute confusion. On arrival she had features of psychosis. She had a medical history of alcohol excess and substance abuse but no intravenous drug use. She denied any high-risk sexual behaviours. Physical examination revealed a grade 2–3 early diastolic murmur with no clinical evidence of cardiac failure. She was normotensive at 137/77 with a heart rate of 72 and oxygen saturations of 98% on room air. She was febrile at 38.5°C on presentation. There was no rash evident on examination. ECG showed lateral ST segment depression and T-wave inversion. A bedside transthoracic echocardiogram demonstrated a dilated left ventricle with severe aortic regurgitation and a normal aortic root. The mid-distal left anterior descending (LAD) coronary artery territory showed severe dysfunction. A CT thorax demonstrated no aortic dissection, however, did comment upon a mildly prominent ascending aorta.

A diagnosis of endocarditis was postulated. A transoesophageal echocardiogram (TOE) demonstrated grade 3–4 aortic regurgitation with central cusp failure and secondary altered geometry of the aortic valve. This was secondary to generalised thickening and oedema of the aortic root, which was not dilated. No evidence of endocarditis was seen. The TOE confirmed moderate left ventricular dysfunction and mild dilatation, with clear evidence of LAD infarction.

A diagnosis of aortitis was made. Three separate sets of blood cultures were taken and were negative. Syphilis serology was positive with Treponema pallidum enzyme immunoassay (EIA) positive, rapid plasma reagin (RPR) 1:256 and Treponema pallidum particle agglutination (TPPA) >1:5120. T. pallidum IgM antibodies were positive. The remainder of her infective and autoimmune screen (including HIV testing) was negative. Cerebrospinal fluid examination for white cell count, protein, glucose, venereal disease reference laboratory (VDRL) and syphilis PCR was normal.

Given the patient's history of anaphylaxis with penicillin, she was commenced on doxycycline (200 mg once daily) with prednisolone 60 mg for the first 3 days. Her mental state deteriorated, becoming increasingly difficult to manage, necessitating section under the Mental Health Act 2007 (UK) and transfer to psychiatric intensive care. Two weeks later, her physical condition deteriorated and she was transferred to coronary care in pulmonary oedema. High-dose methylprednisolone was commenced as empirical management of aortitis. In spite of this, she deteriorated further requiring intubation.

An angiogram demonstrated severe bilateral ostial disease with left main stem and right coronary artery stenosis (figure 1).

Figure 1

Coronary angiogram showing a tight ostial left main stem stenosis despite no evidence of atherosclerotic disease downstream.

She was reviewed by infectious diseases and following penicillin desensitisation was started on intravenous benzylpenicillin at 2.4 g six times a day for 17 days.

The timing of surgery was carefully considered, and the decision made to delay surgery if possible to allow for treatment of her infection, and associated decrease in local inflammation. However, due to her clinical condition, surgical valve replacement and coronary artery bypass grafting was performed 8 days later. Intraoperatively, the aorta and one cusp of the aortic valve were thickened and inflamed. Due to poor left ventricle (LV) performance, an intra-aortic balloon pump was placed and the patient required extracorporeal membrane oxygenation (ECMO). Her postop course was complicated by Escherichia coli and Candida albicans blood stream infection and critical illness myopathy.

Histology from the excised aortic valve showed myxoid degenerative changes with no histopathological evidence of endocarditis, in support of the echocardiogram findings. T. pallidum PCR testing of the valve tissue was negative. Follow-up RPR fell to 1:128 at 1 month then 1:64 at 6 months.

Discussion

Since the late 1990s, the incidence of syphilis has increased in UK with outbreaks in major cities including Manchester.1 ,2 The majority of the cases seen are either primary or secondary syphilis.

Cardiovascular Syphilis is a recognised manifestation of tertiary syphilis, occurring 10–30 years after the initial infection.3 Aortitis in secondary syphilis has only been reported in a handful of cases.4–6

Aortic involvement in syphilis leads to a range of pathological findings. Aortic insufficiency occurs in 15%–29% from clinicopathological studies.3 ,7 Surgical findings in patients undergoing valvular replacement report minimal valvular inflammation.8 Ostial lesions without ventricular failure occur in 3%–14%, and coronary artery bypass grafting has been successful in management of this condition.8 A 1952 series reported left ventricular failure in 10%.9 The authors are not aware of any other reports of coronary ostial involvement in secondary syphilis.

CT, MRI and echocardiography are valid diagnostic imaging methods in aortitis. The specificity of CT scanning for syphilitic aortitis is not defined; early aortitis may not be visualised.10 In this case, CT findings were subtle and TOE in the hands of a skilled operator was key to the diagnosis.

Penicillin is the established first-line antibiotic in the management of syphilis, and in cases of penicillin allergy desensitisation is recommended.11 The evidence for the use of non-penicillin regimens in cardiovascular syphilis is weak. Steroids are also recommended prior to antibiotics to mitigate the risk of aortic rupture. This was particularly problematic in our case due to the patient's psychosis.

This patient's presentation emphasises the importance of considering syphilis in the differential diagnosis outside high-risk populations. Syphilis is renowned for its protean manifestations, and delays in diagnosis are not uncommon.12 The complexity of this case required a broad multidisciplinary approach to ensure the appropriate management for this patient.

Key messages

  • It is vital to consider syphilis in the differential outside of high-risk populations.

  • Antibiotic management in those with anaphylaxis to penicillin is not well established and desensitisation is recommended after specific allergy testing.

  • Complex cases like this require a multidisciplinary approach with the expertise of several specialities.

References

Footnotes

  • Handling editor Jackie A Cassell

  • Contributors JAC, JS and EGM were responsible for conception and planning the report. AM was responsible for acquisition and interpretation of cardiac imaging. JAC and EGM were responsible for acquisition of clinical and laboratory data and analysis and interpretation of data. JAC was responsible for drafting the article. IK, SR, JS and EGM contributed to critical revisions of the article for important intellectual content.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.