SCENARIOA young female of 16 years

resident of sahiwal presented in PIC outdoor

deptt with following presenting complaints

PRESENTING COMPLAINTS

• Palpitations--------- 6 months

• SOB and Chest pain------------ 1 month

HOPI• Patient was in her usual state of health 6

months back when she started feeling PALPITATIONS.It was paroxymal in the start and got worse with the passage of time.it increased with exertion.

• One month back she also developed chest pain more around the apex of the heart and it was radiating to the back and shoulder.

• Pain was associated with SOB which was increasing with exertion,Not associated e sweatsing and syncope.

HOPI continued……….

No symptoms related to muskuloskeletal system and skin allergy or photosensity

Social/personal history:Unmarried ,Living with parents

PAST HISTORY

• No significant past medical/surgical/gynecological history

DRUG HISTORY• Taking pain killers from GPs(records not

available)• No known drug allergy• No addiction

CLINICAL EXAMINATION

• General physical examination;

• Young female lying in the bed with mild discomfort with average built and height and normal body habitus.

• VITAL PARAMETERS;• BP 100/70 almost equal in upper and lower,

limbs• Pulse 90/ min regularly regular ,good

volume ,no radio radial and radio femoral delay

• Temp 98.6 F• RR. 20/min

CVS EXAM

• JVP not raised • Slightly Displaced apex beat,ill sustained • Diastolic thrill at aortic area• Early diastolic murmur at A2 area ,blowing in character,non

radiating ,exacerbating on expiration and leaning forward • No pedal or sacral edema

• RESPIRATORY EXAM

• No visible chest deformities• Chest movements and pattern normal• Normal bilateral vesicular breathing

CNS

• GCS 15/15• No neurological deficit

• GIT EXAM

• Unremarkable

INVESTIGATIONSLABS

Labs continued……………..

X RAY CHEST

ECG

ECHO

CT

CT REPORT

DIAGNOSIS

•AORTIC ANEURYSM WITH SEVERE AR(most probably due to familial aortic aneurysm syndrome)

Management

• MEDICAL

• During her stay in ward she was treated with diuretics(spiromide) and panadol

• Meanwhile patient was assesed by surgical committee and decided to shift the patient to surgical deptt for operation.

Surgical findings

• Ascending aortic aneurysm with dissecting lumen/false lumen

• Size of the aneurysm was 10x8 cm • SURGICAL TECHNIQUE• Bantall procedure performed which involves

the aortic root replacement+AVR+coronary re-implantation

1 week postoperatively

• Patient found to be healthy and free of all the previous complaints

• She is mobilized with good functional capacity.• Patient is awaiting discharge and follow up

work up.

LITERATURE REVIEW

Aortic Aneurysm • Definition • It is defined as pathological dilatation of aorta

to 1.5 times its normal diameter • Mainly classified into thoracic AA and

abdominal AA

Disease burden

5-10 per 10,0000 person-years Among thoracic aortic aneurysm,

ascending aortic aneurysm constitute 60% of all.

CAUSES

1)Genetic Marfan’s syndrome

Loeys-dietz syndrome Familial thoracic aortic aneurysm Enhlers-danlos syndrome

Bicuspid aortic value Turner syndrome

aneurysm-osteoarthritis syndrome

CUASES CONTINUED……….

• 2)Atherosclerosis More command in descending thoracic aortic aneurysm

• 3)Degenerative• 4)Cystic medial degenerative• 5)Advance age • 6)Traumatic

CAUSES CONTINUED……

7)INFLAMMATORY takayasu arteritis,giant cell arteritis ,HLA B27 associated

spondyloarthropathies8)Infection Syphilis

– 9)POST-STENOTIC etc AS,coarctation of aorta.

10)POST SURGICAL AVR 11)CHRONIC AORTIC DISSECTION

CLINICAL PRESENTATION

• symptomsmostly diagnosis is incidental because patients are mostly asymptomatic.Patients can present with complications etc AORTIC INSUFFICIANCYWITH, LVF,ISCHEMIA DUE TO CORONARY COMPRESSION

Clinical presentation continued……. sinus of valsalva rupture

thrombus embolic phenomenon .dysphasia due to esophageal compression .superivar vane cava syndrome .hoarseness (due to recurrent laryngeal nerve compression) .dyspnea (due to tracheal compression ) .backache

ACUTE PRESENTATION

• more acute presentation is when aneurysm ruptures OR dissection occurs. It then presents with sudden sever ,sharp chest and back pain

Signs

No specific signs of thoracic aortic aneurysm

Cardiac auscultation can give FINDINGS of AI,CHF,MI

Vascular examination can show the presence of a pulsatile mass

Pulmonary examination decrease air entry on both or one side

Pathophysiology

• The most common pathophysiology is MEDIAL NECROSIS which involves loss of elastic fibers and smooth muscles,replaced with interstitial tissue.

MANAGEMENT

guideline: Asymptomatic patients should be evaluated for

surgical repair who have degenerative TAA,IMH,PAU,MYCOTIC ANEURYSM or PSEDOANEURYSM who are otherwise suitable candidates and for whom ascending aorta or diameter of aortic sinuses is 5.5cm or greater(level of evidence C )

The patients with growth rate of more than 0.5cm/year in an aorta of less than 5.5cm should be considered for operation(level of evidence C )

GUIDELINES CONTINUED…..

Patients undergoing AVR who also have aortic root diameter more than 4.5cm should

undergo concomitant repair/replacement.

Patients with genetically mediated cause of TAA should undergo elective operation at

smaller diameters(4 to 5cm) to avoid dissection/rupture.

DIAGNOSTIC INVESTIGATIONS 1)CXRWidening of mediastinum Unusual aortic contoursDisplaced trachea and bronchi2)CTABest used for serial follow-up for surgical repair or

endovascular repair.3)MRA preferably for visualisation of aortic root where necessary4)TTEFor visualisation and assesment of aortic root and proximal

aortic diameter.

5)TEE can be used for visualisation of entire aorta

and assesment of AR which might be preent.

6)MRIFor visualisation of entire

aorta,branches,aortic valve and pericardium.

7)AORTOGRAPHYTo see the involved segment

MANAGEMENT guidelines

MEDICAL Beta blockers are geneally recommended for all the

patients of TAA but particularly for MARFAN SYNDROME to reduce the risk of aortic dilatation.

In hypertensive patients blood pressure should be controlled to below 140/90

ARBs may particularly beneficial to slow the aneurysm growth

ENDOVASCULAR REPAIR

Supported by only a couple of trials

Currently EVAR is reserved for TAA patients who are

at high risk for open repair and have ideal

anatomy.

SURGICAL THERAPY• Pre-operatively patients of TAA,who also

show evidence of ischemia should undergo coronary angio and treatment(PCI or CABG)should be planned concomitantly

• Patients with MARFANS,LOEYS-DIETZ,EHLERS-DANLOS and others with dilatation of aortic root and sinuses of valsalva should undergo excision of sinuses in combination with a modified valve –sparing root implantation operation if technically feasible or if not,root replacement with valve graft conduit.

Surgical technique

Surgical treatment involves a DACRON GRAFT to inserted in place of the diseased aorta.the

main branches are re-implanted to the graft.When aortic valve is also involved with root

aneurysm a modified BENTALL procedure (composite prothetic aortic valve+dacron

graft) or aortic valve homograft is performed.

• aneurysm involving both ascending and descending aorta can be treated with two-staged approach with an ELEPHANT TRUNK procedure (ascending aorta and root are replaced initially and distal portion is suspended in proximal portion for subsequent union with a descending aorta graft placed either by open surgical procedure or percutaneusly)

PERIOPERATIVE SURVIVAL

•90- 95 % for elective repair

COMPLICATIONS OF SURGICAL REPAIR

• MI(7.2 %)• CVA (4.8 %)• RENAL FAILURE (2.4 %)• HAEMORRAGE (7.2 %)• PARAPLEGIA (6 %)

• THANKS