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A CASE OF

CYANOTIC HEART DISEASELEADING TO

ISCHEMIC STROKE

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PATIENTS PROFILE

NAME. Amir

AGE. 16 Yrs

GENDER. Male RELIGION Islam

RESIDENCE. Lahore

OCCUPATION. Student

DATE OF ADMISSION. 18 Jan 2011

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PRESENTING COMPLAINTS

Right sided weakness

Altered sensorium 04 days Inability to speak

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HISTORY OF PRESENT ILLNESS

Known case of tetralogy of fal

Sudden onset right sided weakness associated withaltered sensorium and inability to speak

Associated with fecal and urinary incontinence

No history of headache, fever , fits , frothing

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History (cont..) Past history

1996 Left sided modified BT shunt (rt. Brachiocephalic to rt. Pulmonary artery) atone year of age.

2005 Revision of BT shunt Multiple hospital visits for shortness of breath and cyanotic spells

Family history 1 siblings affected by congenital heart disease died at none months of age ,

intrauterine Cousin marriage of parents

Drug/ allergy history On ascard since childhood No H/O allergy

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History (cont..) Personal history

not significant

Socio-economic history

Lower middle class

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GENERAL PHYSICAL EXAMINATION

Young boy, average built, dysphasic, anxiousand oriented in time, place and person.

VITAL SIGNS.

PULSE 84bpm, Regular

B.P 120/60 mmHg

R/R 16 per min

TEMP 98F

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GENERAL PHYSICAL

EXAMINATION

Jaundice -ve Cyanosis +ve

Clubbing +ve

Pedal edema -ve

Lymphadenopathy -ve

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SYSTEMIC EXAMINATION

Cardiovascular System Inspection

Depressed sternum (pigeons chest) Midline scar mark of previous surgeries No visible pulsations or pigmentation

Palpation Apex beat in 5thics in midclavicular line , non tapping , non heaving No parasternal heave No other sound palpable

Auscultation S1 + S2 + O

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SYSTEMIC EXAMINATION (cont..)

Central nervous system GCS : E4M6V3 13/15 Higher mental functions intact Cranial nerves intact Sensory system intact

Motor Bulk bilaterally normal and equal Tone slightly increased in both right arm and leg Reflexes : knee and ankle jerk brisk on the right side Power right left

0/5 5/5

0/5 5/5 Signs of meningeal irritation -ve Cerebellar signs -ve

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SYSTEMIC EXAMINATION (cont..) Respiratory

Inspection Pigeons chest Respiratory rate 18/min Abdomenothoracic Chest movements equal on both sides

Palpation Trachea central Apex beat paplpable in 5thICS in mid-clavicular line No tenderness No crepitus Chest expansion 5cm Vocal fremitus normal

Percussion Percussion note normal and equal on both sides Auscultation

Bilateral vesicular breathing No added sounds Vocal resonance normal

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SYSTEMIC EXAMINATION (cont..) GIT

Inspection Normal shaped abdomen with central umblicus

No visible scar marks or pulsations

Palpation No tenderness No visceromegaly palpable

Percussion Fluid thrill ve Shifting dullness -ve

Ausculatation Bowel sounds +ve

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PROVISIONAL DIAGNOSIS

1. Congenital Cyanotic Heart Disease2. Left sided ischaemic stroke .

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INVESTIGATIONS

Hb 16.2gm/dl TLC 9.9x103/l.

PLT 254x103/l.

Blood ESR 10mm fall at the end of 1st

hour. Blood Sugar Random 133mg/dl.

Blood Sugar Fasting 93mg/dl.

Serum Lipid Profile Normal

Serum Urea 18mg/dl

Creatinine 0.4mg/dl

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INVESTIGATIONS

Sodium 137 mm0l/l

Potassium 3.8mmol/l

HBV / HCV Negative

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Chest X-Ray

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CT-Scan

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Echocardiography

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FINAL DIAGNOSIS

gchd

Left sided ischaemic stroke leading to Right sidedweakness

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Outline Review blood flow through the heart

Discuss ToF anatomic abnormalities

Etiology Clinical Presentation

Labs and Exams

Two surgical interventions

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Healthy Heart

Blood Flow Deoxygenated blood from the body enters the RA

At the same time, oxygen rich blood leaves the lungs toflow into the LA

Blood in the RA enters the RV through the tricuspidvalve

At the same time, blood flows from the LA to the LV

through the mitral valve

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Blood Flow cont. Blood in the RV is pumped through the PA to the

lungs

At the same time, LV pumps blood out the aorta tosupply the body with oxygen rich blood

As seen in ToF, structural defects lead to thecirculation of oxygen-poor blood

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ToF 4 anatomic

malformations:-Right VentricularHypertrophy-Pulmonary ValveStenosis-Transposition of

the aorta-Ventricular SeptalDefect

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ToF RVH

-secondary to PA Stenosis

-Increased P on RV leads to RVH

Transposition of Aorta

-aorta is displaced

VSD

-hole in the heart

-mixing of oxygenated and unoxygenated blood

-cyanosis

PVS

-more severe, less blood transported to the lungs andmore deoxygenated blood will pass through VSD to aorta tobe circulated throughout the body

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Etiology Theory: destruction of the neuronal crest cells during

embryogenesis

In the laboratory setting, destruction of these cellsreproduced results displayed with certain cardiacmalformations.

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Clinical Presentation

Clinical presentation is directly related to the degree of

pulmonary stenosis.

Severe stenosis results in immediate cyanosisfollowing birth. Mild stenosis will not present untillater.

Growth is retarded insufficient oxygen and nutrients

SOA on exertion

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Tet Spell Tet spells at 2-3yo,

child becomes

cyanotic, mayexperience syncope

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Exams and Tests

CBC

- hematocrit

ECG

-RVH, RAD

CXR-boot shaped heart,

right sided aortic arch

Echocardiogram

-VSD

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Surgical Intervention 1 Complete intracardiac repair of VSD and PA stenosis.

Enter chest through the sternum. Connect the heartand lung machine. Heart is stopped.

Repair the VSD with a patch. Determine if PA needs to be removed or if removing

the excessive muscle tissue will help to functioncorrectly.

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Pacemaker wires are placed temporarily because of thepotential for postoperative ventricular arrhythmias.

Individual chamber pressures are then measuredbefore the chest is closed. The pressure readings helpto determine how effective the surgery was.

Complications: infective bacterial endocarditis,pulmonic regurgitation, arrhythmias, RBBB, or leftanterior hemiblock

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Surgical Intervention 2 New method for patching the VSD

Transcatheter patches were selected specifically forVSD size. Radio-opaque loop inside of each patchallowed for attachment of double nylon thread. Maderetrieval possible if necessary.

Pts were anticoagulated with heparin initially, followed

with ASA 24 hr later.

48 hrs later the apparatus was inserted into the

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48 hrs later the apparatus was inserted into thefemoral vein were it was catherized until it reachthe ascending aorta.

Echo was used to determine its location within theheart, allowing for proper placement of the patchfor the VSD.

Pts were monitored in the ICU for 24 hrs.

Only 2 of the 16 pts in this study did not benefitfrom this study. In comparison to others, their

VSDs were much larger.

Pulmonary valvuloplastics were also performed. Complications: there were no reported

complications for VSD in this study

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Why Do We Need to Know? Not every case of ToF will be discovered by cardiologist

and pediatricians.

Some symptoms will be subtle and present later in life

to PCPs. Several case studies discussed patients who chose not

to have their ToF corrected. We need to know if ToFdefects are causing their health problem or if the

problems are from other sources.