Keywords

Infective aortitis, bicuspid aortic valve, aortic dilatation, vegetations, pulseless, cardiac surgery, Marfan’s syndrome

Patient presented with severe aortic stenosis (AS) due to bicuspid aortic valvewith absent pulses and Marfanoid features. Since absent pulses is not a featureof AS, the cause for the absent pulses could not be made outclinically. However, on the operating table, the cause was found to be multipleaortic vegetations on a dilated aorta due to Marfan’s syndrome, blocking the flow of blood.

Case Report

Mr S, aged 20 years, was admitted with a history of low-grade fever associated with weight loss for 2 months, breathlessness on exertion for 1 month, cough with minimal sputum for 15 days, no history of chest pain, swelling of feet and oliguria. There was nohistory of palpitation or syncope.

On examination, patient was conscious, oriented, thin built, had Marfanoid features – arm span greater than height, lower segment greater than upper segment, high archedpalate, dorsal kyphoscoliosis, wrist sign positive. He was afebrile and did not have dyspnea or anemia. There was no pedal edema, pulses were absent in all the four limbs andblood pressure (BP)could not berecorded.

On cardiovascular examination, an ejection systolic murmur could be heard in aortic area. Abdomen was soft with no organomegaly. Pectus excavatum was present on examination of respiratory system with bilateral vesicular breath sounds

Investigations

Laboratory investigations revealed the following results:

Chest X-ray showed cardiomegaly. ECG revealed left ventricular hypertrophy (LVH).

Findings on 2D echocardiography were aortic valve thickened/bicuspid/moderate LV dysfunction/concentricLVH/severe AS.

Transesophageal echocardiography (TEE) showed bicuspid aortic valve/severe AS/mild-to-moderate mitral regurgitation (MR).

Computed tomographic (CT) angiography of thoracic aorta was normal.

Doppler study of arteries of both upper limbs and lower limb were normal.

Patient was diagnosed as bicuspid aortic valve with severe stenosis with Marfanoid features. He was treated for cardiac failure and referred to cardiac surgeon. On the operating table, we were in for a surprise. The ascending aorta had aneurysmal dilatation with multiple vegetations on the jet strike area ofaorta. The bicuspid valve was thickened, severely stenosed and had multiplevegetations.

The aorta was opened and the aortic wall vegetations were removed.The aortic valve was excised. Aortic valve replacement was done with 21 mm regent St. Jude’s valve. The postoperative period was uneventful. Pulses returned to all the four limbs. The BP became recordable. Repeat 2D echocardiogram showed an ejection fraction (EF) of 55%. Repeat CRP was19 mg/L.

Discussion

Aortitis is defined by inflammation of the aorta due to infective syphilitic aortitis, infective nonsyphilitic (bacterial or fungal) aortitis and noninfective aortitis due to large vessel vasculitis (Takayasu’s arteritis).

Infective agents destroy the medial layers of the aortic wall by direct invasion and subsequent inflammation. Bacterial infection may trigger a noninfectious vasculitis by generating immune complexes. By analogy with infective endocarditis, bacterial aortitis often develops following bacteremia in the place of least resistance (pre-existing aneurysm). Infective aortitis can be due to Gram-positive Staphylococcus aureus and streptococci and Gram-negative Salmonella and Proteus. Tuberculosis andfungal aortitis are rare.

Autoimmune disorders can affect the aorta. They are diverse, such as systemic lupus erythematosus (SLE), rheumatoid arthritis, Behcet’s disease, cryoglobulinemia, Wegener’s granulomatosis, etc. Aortitis can be due to Takayasu’s arteritis and temporal arteritis, which is due to chronic inflammation of unknown origin.

Histologically, aortitis is characterized by inflammatory infiltrateswithin the medial layers of the aortic wall, smooth muscle and fibroblastnecrosis and fibrosis of the vessel wall. The inflammatory reaction withinthe aortic wall may cause aortic dilatation upto aneurysm formation.

Diagnosing aortitis can be clinically challenging. Symptoms can benonspecific as malaise, joint aches and low-grade fever and raisedmarkers of inflammation.Inflammatory stenosis of major arterial branchesleads to absent peripheral pulses, ocular and neurological problems. Aortitis related aneurysm of ascending aorta may present with newmurmurs and cardiac failure.

This patient presented with an early systolic murmur (ESM) in aortic area and cardiac failure. He was diagnosed as having bicuspid aortic valve with Marfanoid features. The absent pulses in all the limbs was an unsolved enigma.When aortic valve replacement was done the puzzle was solved. The ascending aorta had aneurysmal dilatation with multiple vegetations. Once the vegetations were removed, pulses returned. It has been found that bicuspid aortic valve has a high incidence of dilatation of the aorta compared to the normal population.

Conclusion

Pulseless disease need not be only due to Takayasu’s arteritis involving the aortic valve as is commonly understood but rarely, other acute infective causes ofaortitis can make the patient to be pulseless as proved by this case.

Suggested Reading

1. Colledge NR, Walker BR, Ralston S, Davidson S. In: Davidsons Principles and Practice of Medicine. 21st Edition, Edinburgh, New York: Churchill Livingstone/Elsevier; 2010. pp. 795-834.
2. Fauci AS, Kasper DL, Longo DL, Braunwald E, Hauser SL, Jameson JL, et al. In: Harrisons Principles of Internal Medicine. Volume 2, 17th Edition, New York: McGraw-Hill; 2008.
3. Fuster V, Walsh RA, Harrington RA, et al. In: Hurst’s: The Heart. 13th Edition, Volume 1, New York: McGraw-Hill; 2011.
4. The ESC Textbook of Cardiovascular Medicine. European Society of Cardiology: Blackwell Publishing; 2007.
5. Fuster V, Walsh RA, Harrington RA, et al. In: Hurst’s: The Heart. 13th Edition, Volume 2, New York: McGraw-Hill; 2011.