Heart
Valvular heart disease
Syphilis

Author: R. Amita, M.D. (see Authors page)

Revised: 19 March 2018, last major update July 2014

Copyright: (c) 2014-2018, PathologyOutlines.com, Inc.

PubMed Search: Syphilis [title] heart

Cite this page: Amita, R. Syphilis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/heartsyphilis.html. Accessed July 21st, 2018.
Definition / general
  • Due to spirochete, Treponema pallidum
  • Rare in developed and developing countries
  • Male:female is 2:1 to 4:1
  • Affects aortic valve and ascending aorta
Pathophysiology
  • Treponema pallida reach cardiovascular system a short time after initial infection but it takes 2 - 20 years for clinical manifestations to develop
  • Manifestations are more common in patients with insufficient treatment in early stages of infection than in those who had late treatment
  • In cardiovascular syphilis, the vasa vasorum of the ascending aorta shows dense inflammation mainly confined to the adventitia and the media
  • This leads to infiltration and replacement of the elastic layer and muscle fibers by fibrous tissue, causing the aortic wall to become weak and dilatable; due to inflammation of the wall of the proximal aorta, the coronary artery orifices become narrowed or blocked and the aortic valve becomes involved, giving rise to aortic incompetence rather than aortic stenosis
  • Aortic incompetence and coronary stenosis are due to the mesaortitis of the proximal portion of the aorta; the various changes in the syphilitic heart (ischemia, fibrosis, hypertrophy, dilatation, cardiac degeneration, angina pectoris) are end results of aortitis which increases the heart's workload and diminishes its blood supply
  • The aortic arch is the most common site for aneurysmal dilatation
  • Syphilis may also involve other large arteries, cerebral vessels or myocardium
Clinical features
  • Substernal oppression or "burning", precordial anxiety, pain or distress
  • Dyspnea, paroxysmal or nocturnal
  • Mild diastolic hypertension in young
  • Palpable and visible pulsation in suprasternal notch
Diagnosis
  • Ejection murmur and aortic diastolic murmur
  • Characteristic loud "tambour like" (booming and ringing quality like a drum) second sound is the most significant sign of syphilitic aortitis
Complications
  • Aortic aneurysm, aortic regurgitation and coronary ostia stenosis
Laboratory
  • Non treponemal tests are less sensitive (71 - 73%): VDRL test and rapid plasma reagin (RPR) test
  • Treponema specific tests are more sensitive (94 - 96%): TPHA, micro hemagglutination test, flourescent treponemal antibody absorption test
Radiology description
  • Chest X ray: irregular dilatation and linear calcification of ascending aorta
  • CT angiogram is best imaging study to define the size and anatomy of the aneurysm, but in the setting of an aneurysm, the echocardiogram and coronary angiogram are mandatory to exclude aortic regurgitation and coronary flow limiting lesions
Prognostic factors
  • Prognosis depends on age, sex, occupation, stage of infection, site and extent of lesion, nature of lesion (progressive or stationary), response to treatment, cardiac efficiency, presence of complications
  • The prognosis is better in patients with uncomplicated aortitis than in those with aortic insufficiency, aneurysmal dilatation or myocardial degeneration
  • Once the aorta is damaged or it has damaged the heart, antisyphilitic treatment may at best arrest the advance of the disease, increase life expectancy and ease symptoms
Case reports
Treatment
Clinical images

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Linear calcification of aortic wall

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Dilated aortic root and ascending aorta

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Enlarged aortic root

Gross description
  • Aorta intima has irregular wrinkling, fine or coarse, with irregularities of surface produced by scattered inflammatory fibrosis, producing the characteristic tree bark appearance
Microscopic (histologic) description
  • Perivascular inflammation around vasa vasoram causing obliterative endarteritis which begins in adventitia but extends into media by formation of capillaries
  • Inflammation is mainly lymphocytes and plasma cells
  • Resultant fibrosis leads to retraction of media and wrinkling and furrowing of intima
Microscopic (histologic) images

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Various images