History

Fact Explanation
Cardiovascular symptoms may arise, several years following the genital infection Cardiovascular syphilis is referred to Treponema pallidum infection of the heart and vessels which is a manifestation of tertiary syphilis. Tertiary syphilis occurs after about 3 to 10 years after primary infection. Cardiovascular syphilis can lead to aortitis, aortic aneurysm, coronary stenosis, aortic insufficiency and, rarely, to myocarditis. Cardiovascular syphilis is commonly seen in males than in females, and it accounts for about 10 to 15% of the heart diseases seen in patients more than 50 years of age. [1,2,4,5]
Asymptomatic During the early stages of the disease patients can be asymptomatic. Patient may present with symptoms after about two to twenty years after the primary infection. [5]
Chest pain Coronary artery stenosis and endarteritis obliterans lead to angina. Patients complain of retrosternal chest pain on exertion which relieved with rest. [4]
Sudden death Coronary artery stenosis can result in sudden cardiac death due to diminished myocardial perfusion or due to the development of life threatening arrhythmia (ventricular tachycardia, ventricular fibrillation). Rupture of aortic aneurysm is another possible cause for sudden death. [7,9]
Symptoms of heart failure Acute heart failure can occur with acute coronary syndrome and congestive heart failure develops due to valvular damage (aortic regurgitation). Patients complain of dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea and reduced exercise tolerance. Presence of congestive heart failure affects the prognosis adversely. [6]
Symptoms of aortic aneurysm Inflammation of the aortic wall leads to aortitis which later progresses to obliterative endarteritis of the vasa vasorum. This process causes ischemic necrosis of the elastic fibres and connective tissue in the tunica media of the aorta. All these changes weaken the aortic wall leading to dilatation and aneurysm formation. [10,11] Aortic aneurysm is a late complication of the cardiovascular syphilis. The classic triad of ruptured aortic aneurysm is abdominal or back pain, hypovolaemic shock, and a pulsatile abdominal mass. Other complaints include pulsatile anterior chest wall mass, palpitations, dyspnea, chest pain which radiates to back, dysphagia, cough, and hemoptysis (due to tracheal compression). [3,6,8,9]
History of primary syphilis [1] History of primary syphilis is helpful in making the clinical suspicion of possible cardiovascular syphilis.
Symptoms of brain abscess Septic embolization is a rare but a serious complication of syphilitic heart disease. Patients may complain of early morning headache, nausea, vomiting and focal neurological symptoms. [9]
References
  1. HEGGTVEIT H. A.. Syphilitic Aortitis: A Clinicopathologic Autopsy Study of 100 Cases, 1950 to 1960. Circulation [online] 1964 March, 29(3):346-355 [viewed 14 July 2014] Available from: doi:10.1161/01.CIR.29.3.346
  2. SHANKARAPPA RK, MOORTHY N, PANNEERSELVAM A, KARUR S, DWARAKAPRASAD R, NANJAPPA MC. Bilateral Coronary Ostial Lesions in Cardiovascular Syphilis Treated by Means of Percutaneous Coronary Stenting Tex Heart Inst J [online] 2013, 40(5):630-632 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3853807
  3. GHAZY T., MATSCHKE K., KAPPERT U.. A monstrous aneurysm of the descending aorta as a sole manifestation of tertiary syphilis treated endovascularly. European Heart Journal [online] December, 32(19):2411-2411 [viewed 14 July 2014] Available from: doi:10.1093/eurheartj/ehr095
  4. MACHADO MAURICIO DE NASSAU, TRINDADE PERCIVAL F., MIRANDA RAFAEL CARLOS, MAIA LILIA NIGRO. Lesão bilateral dos óstios coronários na sífilis cardiovascular: relato de caso. Rev Bras Cir Cardiovasc [online] 2008 March, 23(1):129-131 [viewed 14 July 2014] Available from: doi:10.1590/S0102-76382008000100022
  5. BLOMQUIST OA. THE TREATMENT OF CARDIOVASCULAR SYPHILIS Calif Med [online] 1948 Aug, 69(2):111-113 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1643457
  6. WEBSTER B. Present Status of the Management of Cardiovascular Syphilis Br J Vener Dis [online] 1960 Mar, 36(1):14-17 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1047308
  7. O'BRIEN JF, SMITH CA, FISHERKELLER MA. Cardiovascular Syphilis: A CLINICAL STUDY OF 578 CASES Br J Vener Dis [online] 1955 Jun, 31(2):74-85 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1054009
  8. O'GARA P. T.. Aortic Aneurysm. [online] 2003 February, 107(6):43e-45 [viewed 14 July 2014] Available from: doi:10.1161/01.CIR.0000054210.62588.ED
  9. MADKE BS, AGRAWAL NB, VAIDEESWAR P, PRADHAN M, ROJEKAR AV, KHOPKAR US. Luetic aortopathy: Revisited Indian J Sex Transm Dis [online] 2010, 31(2):118-121 [viewed 14 July 2014] Available from: doi:10.4103/0253-7184.75011
  10. PAULO N., CASCAREJO J., VOUGA L.. Syphilitic aneurysm of the ascending aorta. Interactive CardioVascular and Thoracic Surgery [online] December, 14(2):223-225 [viewed 14 July 2014] Available from: doi:10.1093/icvts/ivr067
  11. SARAIVA ROBERTO SANTOS, CéSAR CLAUDIO ALBERNAZ, MELLO MARCO ANTONIO ARAúJO DE. Aortite sifilítica: diagnóstico e tratamento. Relato de caso. Rev Bras Cir Cardiovasc [online] 2010 September, 25(3):415-418 [viewed 14 July 2014] Available from: doi:10.1590/S0102-76382010000300021

Examination

Fact Explanation
Pulse Tachycardia and arrhythmia can be detected. Corrigan’s pulse is positive if significant aortic regurgitation is present. [1]
Cardiac auscultation Tambour-like second heart sound is the most significant sign of aortic involvement. Aortic flow murmur can be heard as a rough systolic murmur over the aortic area. [5]
Cardiomegaly Cardiomegaly is detected by the presence of laterally and inferiorly displaced cardiac apex. [3]
Left ventricular hypertrophy The left ventricle is hypertrophied due to aortic regurgitation. [3,5]
Signs of heart failure Peripheral edema, pulmonary rales are detected due to heart failure. [2]
Pulsation over the suprasternal notch Visible and palpable pulsations over the suprasternal notch can be detected in the presence of aortic aneurysm. [5]
Blood pressure Diastolic hypertension can be detected in some patients. The pulse pressure is increased in patients with aortic regurgitation. [4]
Clinical signs of aortic regurgitation Quincke’s pulse (nail bed pulsations), Quincke’s pulse, wide pulse pressure, Duroziez’s murmur over the femoral artery, Traube’s sign (pistol shots femorals) and de Musset’s sign (head-nodding with each cardiac pulsations) [1]
Tracheal tug [1] Tracheal tug is positive due to aortic root dilatation.
References
  1. MORPHET J. Cardiovascular Syphilis Can J Cardiol [online] 2008 Dec, 24(12):886-887 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2643226
  2. MACHADO MAURICIO DE NASSAU, TRINDADE PERCIVAL F., MIRANDA RAFAEL CARLOS, MAIA LILIA NIGRO. Lesão bilateral dos óstios coronários na sífilis cardiovascular: relato de caso. Rev Bras Cir Cardiovasc [online] 2008 March, 23(1):129-131 [viewed 14 July 2014] Available from: doi:10.1590/S0102-76382008000100022
  3. MASTERTON G. Cardiovascular syphilis with amyloidosis and periods of alternating heart block. Br J Vener Dis [online] 1965 Sep, 41(3):181-185 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1047729
  4. WEBSTER B. Present Status of the Management of Cardiovascular Syphilis Br J Vener Dis [online] 1960 Mar, 36(1):14-17 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1047308
  5. MADKE BS, AGRAWAL NB, VAIDEESWAR P, PRADHAN M, ROJEKAR AV, KHOPKAR US. Luetic aortopathy: Revisited Indian J Sex Transm Dis [online] 2010, 31(2):118-121 [viewed 14 July 2014] Available from: doi:10.4103/0253-7184.75011

Differential Diagnoses

Fact Explanation
Rheumatic heart disease Rheumatic heart disease can also cause aortic regurgitation. The diagnosis is made by modified Jones criteria. [1,2]
Infective endocarditis Infective endocarditis is another cause for the development of aortic regurgitation. Infective endocarditis can also occur on top of syphilitic valvular disease as well. [1]
Calcifying disease [1] Valve calcification is commonly seen in elderly patients (more than 60 years). Aortic calcification can lead to congestive heart failure. [3]
Takayasu's arteritis Inflammation of the large vessels (aorta and its main branches) leads to wall thickening, fibrosis, stenosis and thrombus formation. [4]
Giant cell arteritis Giant cell arteritis causes a vasculitis reaction in the large vessels typically affecting the branches of the external carotid artery. Giant cell arteritis is commonly seen in elderly patients. [5]
References
  1. WEBSTER B. Present Status of the Management of Cardiovascular Syphilis Br J Vener Dis [online] 1960 Mar, 36(1):14-17 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1047308
  2. NARULA J., CHANDRASEKHAR Y., RAHIMTOOLA S.. Diagnosis of Active Rheumatic Carditis : The Echoes of Change. Circulation [online] 1999 October, 100(14):1576-1581 [viewed 14 July 2014] Available from: doi:10.1161/01.CIR.100.14.1576
  3. DEMER L. L., TINTUT Y.. Vascular Calcification: Pathobiology of a Multifaceted Disease. Circulation [online] 2008 May, 117(22):2938-2948 [viewed 14 July 2014] Available from: doi:10.1161/CIRCULATIONAHA.107.743161
  4. JOHNSTON SL, LOCK RJ, GOMPELS MM. Takayasu arteritis: a review J Clin Pathol [online] 2002 Jul, 55(7):481-486 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1769710
  5. BARRACLOUGH K, MALLEN CD, HELLIWELL T, HIDER SL, DASGUPTA B. Diagnosis and management of giant cell arteritis Br J Gen Pract [online] 2012 Jun, 62(599):329-330 [viewed 14 July 2014] Available from: doi:10.3399/bjgp12X649313

Investigations - for Diagnosis

Fact Explanation
Venereal Disease Research Laboratory (VDRL) test [3] VDRL test is a simple and inexpensive test used to screen tertiary syphilis. However VDRL test is nonspecific and has many false positive results. [7,9]
Treponema pallidum haemagglutination assay (TPHA) [3] TPHA test detects structural treponemal antigen. This is also simple and cheap screening test. Although TPHA is more specific than VDRL test, it can give rise to false positive results. [9]
FTA-ABS (fluorescent treponemal antibody test) FTA-ABS test is very sensitive in confirming the diagnosis of syphilis after the screening tests become positive. [3,9]
Enzyme immunoassays (EIAs) EIAs detect the presence of Ig G and Ig M antibody subtypes indicating chronic and acute infection respectively. [3,9]
Chest X-ray Chest X-ray shows the dilated aorta. If heart failure is present alveolar edema, Kerley B lines, cardiomegaly, upper lobe diversion and pleural effusions can be seen. linear ‘eggshell’ configurations (calcium depositions) can also be seen in some patients. Wide mediastinum is seen in the presence of aortic aneurysm in tertiary syphilis. [1,3,6]
ECG ECG shows the presence of arrhythmia (atrial fibrillation), bundle branch block and atrioventricular block. Evidence of myocardial ischemia like ST segment elevation, ST segment depression and T wave inversion can also be detected. These changes occur in tertiary syphilis. [2,4,5,8]
Coronary angiogram Coronary angiogram can detect the site and the degree of the stenosis. Coronary artery stenosis is seen in about 26% of patients with syphilitic aortitis. [2,8]
Aortic root angiogram [2] This helps in the assessment of aortic regurgitation an the aortic root dilatation in aortic aneurysm.
Echocardiogram Valvular abnormalities can be visualized. Also helpful in assessing the degree of heart failure. [2]
Thoracic CT Thoracic CT can show the presence of aortic aneurysm especially when combined with angiogram. [3]
Cardiac enzymes Cardiac enzymes are elevated in the presence of acute coronary syndrome. [4]
References
  1. MORPHET J. Cardiovascular Syphilis Can J Cardiol [online] 2008 Dec, 24(12):886-887 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2643226
  2. SHANKARAPPA RK, MOORTHY N, PANNEERSELVAM A, KARUR S, DWARAKAPRASAD R, NANJAPPA MC. Bilateral Coronary Ostial Lesions in Cardiovascular Syphilis Treated by Means of Percutaneous Coronary Stenting Tex Heart Inst J [online] 2013, 40(5):630-632 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3853807
  3. GHAZY T., MATSCHKE K., KAPPERT U.. A monstrous aneurysm of the descending aorta as a sole manifestation of tertiary syphilis treated endovascularly. European Heart Journal [online] December, 32(19):2411-2411 [viewed 14 July 2014] Available from: doi:10.1093/eurheartj/ehr095
  4. MACHADO MAURICIO DE NASSAU, TRINDADE PERCIVAL F., MIRANDA RAFAEL CARLOS, MAIA LILIA NIGRO. Lesão bilateral dos óstios coronários na sífilis cardiovascular: relato de caso. Rev Bras Cir Cardiovasc [online] 2008 March, 23(1):129-131 [viewed 14 July 2014] Available from: doi:10.1590/S0102-76382008000100022
  5. MASTERTON G. Cardiovascular syphilis with amyloidosis and periods of alternating heart block. Br J Vener Dis [online] 1965 Sep, 41(3):181-185 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1047729
  6. O'BRIEN JF, SMITH CA, FISHERKELLER MA. Cardiovascular Syphilis: A CLINICAL STUDY OF 578 CASES Br J Vener Dis [online] 1955 Jun, 31(2):74-85 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1054009
  7. OSWAL S., LYONS G.. Syphilis in pregnancy. Continuing Education in Anaesthesia, Critical Care & Pain [online] 2008 December, 8(6):224-227 [viewed 14 July 2014] Available from: doi:10.1093/bjaceaccp/mkn042
  8. NAKAZONE MARCELO A., MACHADO MAURíCIO N., BARBOSA RAPHAEL B., SANTOS MáRCIO A., MAIA LILIA N.. Syphilitic Coronary Artery Ostial Stenosis Resulting in Acute Myocardial Infarction Treated by Percutaneous Coronary Intervention. Case Reports in Medicine [online] 2010 December, 2010:1-4 [viewed 14 July 2014] Available from: doi:10.1155/2010/830583
  9. EBEL A, BACHELART L, ALONSO JM. Evaluation of a New Competitive Immunoassay (BioElisa Syphilis) for Screening for Treponema pallidum Antibodies at Various Stages of Syphilis J Clin Microbiol [online] 1998 Feb, 36(2):358-361 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC104542

Investigations - Screening/Staging

Fact Explanation
Cerebrospinal fluid (CSF) analysis Cerebrospinal fluid analysis is indicated in all diagnosed patients to screen for the presence of neurosyphilis. Positive VDRL test and CSF cell count of more than 5 cells/mm3 and CSF protein or more than 40 mg/dl is diagnostic of neurosyphilis. [1,2]
References
  1. MACHADO MAURICIO DE NASSAU, TRINDADE PERCIVAL F., MIRANDA RAFAEL CARLOS, MAIA LILIA NIGRO. Lesão bilateral dos óstios coronários na sífilis cardiovascular: relato de caso. Rev Bras Cir Cardiovasc [online] 2008 March, 23(1):129-131 [viewed 14 July 2014] Available from: doi:10.1590/S0102-76382008000100022
  2. OSWAL S., LYONS G.. Syphilis in pregnancy. Continuing Education in Anaesthesia, Critical Care & Pain [online] 2008 December, 8(6):224-227 [viewed 14 July 2014] Available from: doi:10.1093/bjaceaccp/mkn042

Management - General Measures

Fact Explanation
Health education Syphilis is a sexually transmitted disease. Safe sex and use of barrier method of contraception are helpful in preventing the disease transmission. Early diagnosis and treatment of primary syphilis is necessary in preventing the occurrence of tertiary syphilis and subsequent complications of the disease. All pregnant females should be screened for syphilis and treated if positive to prevent neonatal syphilis. [3,4]
Management of heart failure [4,5] Patients with heart failure are advised to have adequate rest and limit strenuous physical activities. Pharmacological management of heart failure includes beta blockers, angiotensin converting enzyme inhibitors, diuretics and digoxin. Revascularization, cardiac transplantation and left ventricular assisted devices are other treatment options.
Management of angina [4] Nitrates , beta-adrenergic blockers, and calcium channel blockers are used in the management of angina pectoris. [6]
Management of coronary artery stenosis Percutaneous transluminal coronary angioplasty (PTCA) can be done. Coronary artery bypass graft and stenting can also be done in patients. [1]
Management of aortic aneurysms Some patients with aortic aneurysm can be left untreated. Open surgery or endovascular stenting is available treatment options for the management of symptomatic aneurysms or if aneurysms are increased risk of rupture. [2]
Management of arrhythmia Antiarrhythmic drugs are indicated for the treatment of arrhythmia. [3]
References
  1. SHANKARAPPA RK, MOORTHY N, PANNEERSELVAM A, KARUR S, DWARAKAPRASAD R, NANJAPPA MC. Bilateral Coronary Ostial Lesions in Cardiovascular Syphilis Treated by Means of Percutaneous Coronary Stenting Tex Heart Inst J [online] 2013, 40(5):630-632 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3853807
  2. GHAZY T., MATSCHKE K., KAPPERT U.. A monstrous aneurysm of the descending aorta as a sole manifestation of tertiary syphilis treated endovascularly. European Heart Journal [online] December, 32(19):2411-2411 [viewed 14 July 2014] Available from: doi:10.1093/eurheartj/ehr095
  3. MACHADO MAURICIO DE NASSAU, TRINDADE PERCIVAL F., MIRANDA RAFAEL CARLOS, MAIA LILIA NIGRO. Lesão bilateral dos óstios coronários na sífilis cardiovascular: relato de caso. Rev Bras Cir Cardiovasc [online] 2008 March, 23(1):129-131 [viewed 14 July 2014] Available from: doi:10.1590/S0102-76382008000100022
  4. BLOMQUIST OA. THE TREATMENT OF CARDIOVASCULAR SYPHILIS Calif Med [online] 1948 Aug, 69(2):111-113 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1643457
  5. WEBSTER B. Present Status of the Management of Cardiovascular Syphilis Br J Vener Dis [online] 1960 Mar, 36(1):14-17 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1047308
  6. ZANGER DR, SOLOMON AJ, GERSH BJ. Contemporary management of angina: part II. Medical management of chronic stable angina. Am Fam Physician [online] 2000 Jan 1, 61(1):129-38 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/10643954

Management - Specific Treatments

Fact Explanation
Benzathine penicillin G [1] Intravenous penicillin (6 to 10 million units) should be given for 2 to 3 weeks. Patients who are sensitive to penicillin can be treated with tetracycline. Herxheimer reaction (Vasodilation and edema of the site of lesion, when occurs at the sites of coronary artery stenosis it leads to further narrowing of the coronary artery and may lead to a myocardial infarction.) is a potential complication of treatment with penicillin and arsenic. [2,3,4,5]
Arsenic Intramuscular or intravenous arsenic is used in the treatment. [3]
References
  1. MORPHET J. Cardiovascular Syphilis Can J Cardiol [online] 2008 Dec, 24(12):886-887 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2643226
  2. GHAZY T., MATSCHKE K., KAPPERT U.. A monstrous aneurysm of the descending aorta as a sole manifestation of tertiary syphilis treated endovascularly. European Heart Journal [online] December, 32(19):2411-2411 [viewed 14 July 2014] Available from: doi:10.1093/eurheartj/ehr095
  3. BLOMQUIST OA. THE TREATMENT OF CARDIOVASCULAR SYPHILIS Calif Med [online] 1948 Aug, 69(2):111-113 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1643457
  4. EDEIKEN J., FORD W. T., FALK M. S., STOKES J. H.. Penicillin Treatment of Patients with Cardiovascular Syphilis in Congestive Failure. Circulation [online] 1950 June, 1(6):1355-1361 [viewed 14 July 2014] Available from: doi:10.1161/01.CIR.1.6.1355
  5. WEBSTER B. Present Status of the Management of Cardiovascular Syphilis Br J Vener Dis [online] 1960 Mar, 36(1):14-17 [viewed 14 July 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1047308