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CASE PRESENTATIONCASE PRESENTATION

A YOUNG LADY WITH A YOUNG LADY WITH HEADACHEHEADACHE

PRESENTER:PRESENTER: Dr. Syed Mohammad Ali Dr. Syed Mohammad Ali

RomelRomel MD ( Cardiology) 3MD ( Cardiology) 3rdrd part part

MODERATOR:MODERATOR: Dr. Mohammad UllahDr. Mohammad Ullah Asstt. ProfessorAsstt. Professor Dept. of Cardilogy,NICVD.Dept. of Cardilogy,NICVD.

PARTICULARS OF THE PARTICULARS OF THE PATIENTPATIENT

Name : Mrs. X. Name : Mrs. X. Age : 29 years.Age : 29 years. Sex : Female.Sex : Female. Religion : Islam.Religion : Islam. Marital Status : Married.Marital Status : Married. Occpation : Service holder.Occpation : Service holder. Address : Gazipur, Dhaka.Address : Gazipur, Dhaka. Date of examination : 20 March, 2011.Date of examination : 20 March, 2011.

THE PRESENTING THE PRESENTING COMPLAINTCOMPLAINT

Headache for 3 years.Headache for 3 years.

HISTORY OF PRESENT ILLNESSHISTORY OF PRESENT ILLNESS

According to the statement of the patient According to the statement of the patient she was alright 3 years back. Then she she was alright 3 years back. Then she has developed episodic, global headache . has developed episodic, global headache . It is associated with neck pain , dizziness It is associated with neck pain , dizziness and malaise. It was not associated with and malaise. It was not associated with history of fall, loss of consciousness, history of fall, loss of consciousness, blurring of vision, aura, nausea, blurring of vision, aura, nausea, vomiting. Her headache has no diurnal or vomiting. Her headache has no diurnal or perimenstrual variation, no trigerring perimenstrual variation, no trigerring factor.factor.

She has got no history of joint She has got no history of joint pain, pain in the limbs on pain, pain in the limbs on walking or at rest, skin rash. walking or at rest, skin rash. Colour change of palm and sole Colour change of palm and sole on exposure to cold.on exposure to cold.

She has no significant weight She has no significant weight loss. Her appetite is good.loss. Her appetite is good.

She has got no H/O palpitation, She has got no H/O palpitation, chest pain or breathlessness.chest pain or breathlessness.

Past History: Past History: She had TB Lymphedinitis 2 years She had TB Lymphedinitis 2 years

back and treated for 1 year.back and treated for 1 year. No history of migraine ,DM, HTN, No history of migraine ,DM, HTN,

Bronchial asthma.Bronchial asthma.

Family history: Family history: Nothing Nothing contributory.contributory.

Personal history : Personal history : Nothing Nothing contributory.contributory.

Menstrual history Menstrual history :: Normal, she takes OCP intermittentlyNormal, she takes OCP intermittently.. Obstetric history Obstetric history :: Married for 5 years. Nulliparus, no Married for 5 years. Nulliparus, no

history of abortion. history of abortion. Treatment historyTreatment history: : Amlodipin, Bromazepum.Amlodipin, Bromazepum. Socioeconomic history Socioeconomic history :: Middle classMiddle class

GENERAL EXAMINATIONGENERAL EXAMINATION

Appearance : GoodAppearance : Good Biuld : AverageBiuld : Average Nutrition : AverageNutrition : Average Anaemia : Anaemia : Jaundice : AbsentJaundice : Absent Cyanosis :Cyanosis :

GENERAL EXAMINATION…….GENERAL EXAMINATION……. Clubbing :Clubbing : Koilonychia : AbsentKoilonychia : Absent Leoconychia :Leoconychia :

Oedema : AbsentOedema : Absent Dehydration : AbsentDehydration : Absent Skin condition : Normal, no xenthelesma, Skin condition : Normal, no xenthelesma,

xenthomata.xenthomata. Neck vein : Not raised with normal Neck vein : Not raised with normal

wave patternwave pattern

Respiratory rate : 15 breaths / minRespiratory rate : 15 breaths / min

Joints : NormalJoints : Normal

SYSTEMIC EXAMINATION : CVSSYSTEMIC EXAMINATION : CVS

Pulse : 84 / min, regular and Pulse : 84 / min, regular and normal in character, normal in character,

BB Right hand – normal volumeRight hand – normal volume left hand -- Small volumeleft hand -- Small volume Right Carotid – normal volume, Right Carotid – normal volume, NN bruit present bruit present

RB RB Left Carotid -- Small volume, no bruit. Left Carotid -- Small volume, no bruit.

NN There was Radio femoral delay. There was Radio femoral delay.

RFDRFD

Popliteal and ADP in both sides arePopliteal and ADP in both sides are present but small volume.present but small volume. Vessel wall was normalVessel wall was normal..

Examination Of CVS…….Examination Of CVS…….

Blood pressure:Blood pressure:

Right hand – 155 / 90 mm Right hand – 155 / 90 mm HgHg

Left hand -- 90 / 60 mm Left hand -- 90 / 60 mm HgHg

Lower limb (B/L) – 100 / 70 mm Lower limb (B/L) – 100 / 70 mm HgHg

JVP : Not raised with normal wave JVP : Not raised with normal wave patternpattern

Examination Of CVS…….Examination Of CVS…….

Precordium :Precordium : Normal in shapeNormal in shape Apex beat was in left 5Apex beat was in left 5th th

intercostal space, intercostal space, just medial to the left mid just medial to the left mid

clavicular line,clavicular line, heaving in characterheaving in character There is no left parasternal heave There is no left parasternal heave PP2 2 was not palpable, was not palpable, There was no thrill.There was no thrill.

Examination Of CVS…….Examination Of CVS…….

Heart sounds : Heart sounds : 11stst and 2 and 2ndnd heart sound heart sound

were were normally audible in all fournormally audible in all four auscultatory areas.auscultatory areas. There was a systolic murmur in There was a systolic murmur in

the back best heard in around the back best heard in around 1010thth thoracic vertebra. thoracic vertebra.

Examination of abdomen :Examination of abdomen : Bilateral renal bruit was presentBilateral renal bruit was present Other findings were revealed Other findings were revealed

normal.normal.

Fundus :Fundus : Normal.Normal.

Other systems : Other systems : NormalNormal

SALIENT FEATURESALIENT FEATURE Mrs. X , 29 years ,married woman, by Mrs. X , 29 years ,married woman, by

profession a service holder, hailing from profession a service holder, hailing from gazipur, Dhaka, attended in OPD on 20 gazipur, Dhaka, attended in OPD on 20 March, 2011 with the complaint of March, 2011 with the complaint of progressive, episodic, dull aching global progressive, episodic, dull aching global headache for 3 years. headache for 3 years.

It was associated with dizziness, neck It was associated with dizziness, neck pain and malaise.pain and malaise.

It was not associated with fever, nausea, It was not associated with fever, nausea, vomiting, aura, blurring of vision, vomiting, aura, blurring of vision, syncope, convulsion, weakness of any part syncope, convulsion, weakness of any part of the body, limb claudication, Raynaud’s of the body, limb claudication, Raynaud’s phenomena, skin rash, joint pain.phenomena, skin rash, joint pain.

SALIENT FEATURE……………SALIENT FEATURE……………

Her pulse was 84 beats/min, regular, Her pulse was 84 beats/min, regular, normal character, normovolumic in right normal character, normovolumic in right upper limb but small volume in left hand upper limb but small volume in left hand and both lower limbs. There was no radio-and both lower limbs. There was no radio-radial delay but radio-femoral delay was radial delay but radio-femoral delay was there. Vessel wall was normal. Blood there. Vessel wall was normal. Blood pressure was 155 / 90 mm Hg in right pressure was 155 / 90 mm Hg in right hand . 90 / 60 mm Hg and 100 / 70 mm Hg hand . 90 / 60 mm Hg and 100 / 70 mm Hg in left hand and lower limb respectively. in left hand and lower limb respectively. There was carotid bruit in right side.There was carotid bruit in right side.

SALIENT FEATURESALIENT FEATURE…………………………

Apex beat was in left 5Apex beat was in left 5thth intercostal space 9 intercostal space 9 cm from the midline, heaving in character.cm from the midline, heaving in character.

There was no thrill, no left parasternal There was no thrill, no left parasternal heave, no palpable Pheave, no palpable P22..

11stst and 2 and 2ndnd heart sound were normal in all heart sound were normal in all areas.areas.

There was a systolic murmur in the back There was a systolic murmur in the back best heard in around 10best heard in around 10thth thoracic vertebra thoracic vertebra..

Bilateral renal bruit was present.Bilateral renal bruit was present.

PROVITIONAL DIAGNOSISPROVITIONAL DIAGNOSIS

Takayasu’s arteritisTakayasu’s arteritis

DIFFERENTIAL DIFFERENTIAL DIAGNOSESDIAGNOSES

Coarctaion of aorta.Coarctaion of aorta. Atherosclerotic Peripheral Atherosclerotic Peripheral

vascular disease.vascular disease. Cervical rib.Cervical rib. Interrupted aortic arch.Interrupted aortic arch.

D/D Points in favors Points against

Takayasu’s arteritis • Young female, < 40 yrs.• Headache, dizziness, malaise• Past history of TB Lymphadenitis.• HTN in right Upper limb.• Small volume pulse & BP in left. upper limb & lower limb.• Right sided carotid bruit.• Bilateral renal bruit.

• No history suggestive of disease activity, like- high fever, wt. loss, anorexia, skin rash, joint pain.• No limb claudication.• No history of stoke, convulsion, hemiplegia

D/D Points in favors Points against

Coarctation of aorta

• Young age.• Hypertension.• Radio-femoral delay.• Small volume pulse & BP in left. upper limb (preductal) & lower limb.• Murmur in back.

• No history of lower limb coldness, claudication, atrophy.• No visible collateral vessel in front & back of the chest.• No upper left parasternal systolic murmur.

D/D Points in favors Points againstAtherosclerotic peripheral vascular disease

• Headache, dizziness.• HTN in right Upper limb.• Small volume pulse & BP in left. upper limb & lower limb.• Right sided carotid bruit.• Bilateral renal bruit.

• Young female.• Non-diabetic.• No family history of hypertension, DM, hyperlipidaemia.• No xenthelesma, xenthomata.• Vessel wall normal

Cervical rib • Neck pain, headache • No history of Raynaud’s phenomena.• No neurological feature, like- redicular pain, pareasthesia, hand muscle wasting.

D/D Points in favors Points againstInterrupted aortic arch

• HTN in right Upper limb.• Small volume pulse & BP in left. upper limb & lower limb.• Right sided carotid bruit.

• Rare disease.• Early onset of symptom in childhood.

INVESTIGATIONSINVESTIGATIONS

CBC :CBC : Hb – 12.5 mg/dlHb – 12.5 mg/dl ESR – 32 mm in 1ESR – 32 mm in 1stst hour. hour. TC of WBC – 12000 / mmTC of WBC – 12000 / mm33

DC of WBC – N : 74%, L : 20%DC of WBC – N : 74%, L : 20% E : 03%, M : 03%E : 03%, M : 03% RBS : RBS : 5.4 mmol / L. 5.4 mmol / L. Urine R/M/E Urine R/M/E : : Alb : Nil, Sugar : NilAlb : Nil, Sugar : Nil Pus cell : 3-5 / HPFPus cell : 3-5 / HPF RBC : Nil, RBC cast : RBC : Nil, RBC cast :

AbsentAbsent

INVESTIGATIONS………..INVESTIGATIONS……….. S. Creatinine : 0.7 mg/dlS. Creatinine : 0.7 mg/dl Blood urea : 20 mg/dlBlood urea : 20 mg/dl

Fasting lipid profile :Fasting lipid profile : S. Cholesterol – 136 mg/dlS. Cholesterol – 136 mg/dl S. HDL -- 36 mg/dlS. HDL -- 36 mg/dl S. LDL -- 79 mg/dlS. LDL -- 79 mg/dl S. Trigliceride -- 90 mg/dlS. Trigliceride -- 90 mg/dl

CRP : NegativeCRP : Negative HBsAg : NegativeHBsAg : Negative VDRL : Non reactiveVDRL : Non reactive

INVESTIGATION…Chest X-Ray P/A INVESTIGATION…Chest X-Ray P/A viewview

INVESTIGATION…ECGINVESTIGATION…ECG

INVESTIGATION……..INVESTIGATION……..

Duplex studyDuplex study

INVESTIGATION……….INVESTIGATION……….

CT AngiogramCT Angiogram

CONFIRMED DIAGNOSISCONFIRMED DIAGNOSIS

Takayasu’s Takayasu’s arteritisarteritis

MANAGEMENTMANAGEMENT

Explanation and reassurance.Explanation and reassurance. Medical management of Takayasu’s Medical management of Takayasu’s

arteritis depends on the disease arteritis depends on the disease activity and the complications.activity and the complications.

MANAGEMENT…….MANAGEMENT……. Medical management:Medical management: Treatment of hypertensionTreatment of hypertension InterventionsInterventions

Surgical managementSurgical management

General measuresGeneral measures

DietDiet modification is necessary to manage modification is necessary to manage hypertension or renal failure.hypertension or renal failure.

ActivityActivity limitations depend on the severity limitations depend on the severity of the disease and complications. of the disease and complications.

Management organ failure, stroke, Management organ failure, stroke, complications of pregnancy, seizures, and complications of pregnancy, seizures, and intracranial hemorrhage.intracranial hemorrhage.

ICU admission is indicated for patients ICU admission is indicated for patients with critical deterioration.with critical deterioration.

Fetal monitoring is indicated in patients Fetal monitoring is indicated in patients with suspected complicationswith suspected complications

PrognosisPrognosis Takayasu’s arteritis is rare, data on mortality and Takayasu’s arteritis is rare, data on mortality and

morbidity are limited. morbidity are limited.

Mortality- 35% in Japan and china but 3% mortality world Mortality- 35% in Japan and china but 3% mortality world wide.wide.

NIH study showed that 20% of patients had a monophasic NIH study showed that 20% of patients had a monophasic illness- self-limiting.illness- self-limiting.

Remaining 80% of patients pass through relapse and Remaining 80% of patients pass through relapse and remitting course.remitting course.

60%. of these, one half experienced relapse after 60%. of these, one half experienced relapse after immunosuppressive therapy was stopped. immunosuppressive therapy was stopped.

The overall morbidity depends on the severity of the The overall morbidity depends on the severity of the lesions and their consequences. lesions and their consequences.

FOLLOW UPFOLLOW UP

Disease activity assessment.Disease activity assessment.

Blood pressureBlood pressure

Early detection and management of Early detection and management of complications, like- complications, like- stroke, , intracranial hemorrhage, , seizures, graft , graft stenosis and/or occlusion, ischemia, stenosis and/or occlusion, ischemia, organ failure, complications of organ failure, complications of hypertension, and fetal injury.hypertension, and fetal injury.

Diagnostic criteria of Takayasu’s arteritisDiagnostic criteria of Takayasu’s arteritis The criteria proposed for the clinical diagnosis of Takayasu's disease (chronic The criteria proposed for the clinical diagnosis of Takayasu's disease (chronic

inflammatory arteriopathy of unknown origin) were based on clinical and inflammatory arteriopathy of unknown origin) were based on clinical and angiographic data from 108 Japanese patients: 96 with Takayasu's disease and angiographic data from 108 Japanese patients: 96 with Takayasu's disease and 12 with another disease of the aorta. 12 with another disease of the aorta.

One obligatory criterion -- Age less than or equal to 40 years.One obligatory criterion -- Age less than or equal to 40 years. Two major criteria -- Left and right mid subclavian artery lesions.Two major criteria -- Left and right mid subclavian artery lesions. Nine minor criteria –Nine minor criteria – 1. High ESR1. High ESR 2. Common carotid artery tenderness2. Common carotid artery tenderness 3. Hypertension3. Hypertension 4. Aortic regurgitation or annuloaortic ectasia 4. Aortic regurgitation or annuloaortic ectasia 5. Lesions of the pulmonary artery5. Lesions of the pulmonary artery 6. Left mid common carotid artery6. Left mid common carotid artery 7. Distal brachiocephalic trunk7. Distal brachiocephalic trunk 8. Thoracic aorta8. Thoracic aorta 9. Abdominal aorta.9. Abdominal aorta. In addition to the obligatory criterion, the presence of two major criteria or of In addition to the obligatory criterion, the presence of two major criteria or of

one major plus two or more minor criteria, or of four or more minor criteria one major plus two or more minor criteria, or of four or more minor criteria suggests a high probability of the presence of Takayasu's disease. suggests a high probability of the presence of Takayasu's disease.

The criteria had a 84% sensitivity .The criteria had a 84% sensitivity .