case of dyspnea in an adolescent girl

Our last admission day after the OP was going back to ICU to see new cases admitted. I was called by PG to rush to casualty to see a bad case.

An adolescent girl,

She was in distress.
Periphery cold,peripheral pulses not palpable.
13 year old girl was under evaluation by our cardiology colleagues, after ECHO she was sent to radiology was some scanning and she was returning back to cardiology department. On the way back she suddenly became sick and she was bought to our casualty which is on the way to cardiology.
She was holding an ECHO report, which the PG read out , dilated cardiomyopathy.
She did nt have report of the procedure she underwent in radiology, But the mother said they took a scan after injecting a drug.
First possibility considered was anaphylaxis to contrast agent.
Oxygen started, IV line secured.
But

Major question to be answered here.

Drug of choice is adrenalin . But the ECHO report with us says DCM, What to do
Will it save. OR ...
Other possibilities in the context of dilated cardiomyopathylike dangerous arrhythmia,embolism,were considered.
The drug details showed she was on digoxin and diuretics.
Ausculation of chest ruled out possibility of pulmonary odema.
Anaphylaxis remained strong possibility.

Decided to manage as anaphylaxis, gave adrenalin, and other measures
She recovered.
She was kept in ICU for a few hours and shifted taken over to our side and kept in our observation.Continued the drugs.

Detaile history 

She was fourth child ,elder siblings all healthy.

She did nt have major illness till six months back.No history of recurrent respiratory infections ,feeding problems cyanosis or any other pointers suggestive of congenital heart diseases

No family hisoty suggestive of major early unexplained deaths,rheumatological disorders in family 

History of contact with a open case of pulmonary tuberculosis, in distant relative. But she did nt complain of fever , recent weight loss, cough

She started complaining of exertional dyspnea six month back which gradually worsened inspite of treatment from local hospital.

She was forced to stop during walking to take rest for a while before proceeding further even ground level.

• Occasional palpitation also
• Ocasional head ache which is bilateral. No alteration of sensorium vomiting or visual disturbances
• No history of chest pain or syncope 
• No history suggestive of paroxysmal nocturnal dyspnea 
• No history of joint pain,skin rashes of any sort.
• No urinary complaints, 

Discussion at the end of history 

Exertional dyspnea in a previously normal adolescent girl ,possibility of respiratory cause as reason are less likely. Dyspnea without cough. If at all interstitial lung disease possible, but are rare 

Possibility of a cardiac cause high ,which leading to predominant left sided congestion.

As she did nt give history suggestive of congestive failure till this age, most likely reasons are aquired heart problems leading to failure 

But remote possibility of a congential heart disease presenting late with congestive failure are not impossibile

Egs of this situations like 

1. Left sided stenotic lesion which progresses as age advance. eg aortic stenosis,coarctaion
2. Small left to right shunt which develops some complications eg VSD with endocartitis, One interesting entity is Sub aortic VSD later developing aortic regurgitation
3. Ocasionally so far asymptomatic minor lesions may express with congestive failure with arrhythmias or anemia or thyroid disorders as co morbidities. 

These are all rare possibilities and most likely we need to consider aquired cardiac problems 

1. In tropical country like ours , Rheumatic valvular heart disease top the list . 
2. Then cardiomyopathies. DCM being the commonest ,other types also rarely 
3. Possibility of pericardial disease like effusion or constrictive pericarditis less common. still to be considered
4. Anemia , whatever reason may co exist and aggravate basic heart problem or may manifest as congestive failure as such 
5. Thyroid disorders
6. Few of neutritional deficiency like thiamin ,selenium etc 
7. Rarely hypertension due to any reason may express as congestive failure.
8. Remote possibility of high output states due to reasons outside heart 

Here the analysis of complaint is very important to pinpoint the pathological process underlying which may point to the underlying hemodynamic problem 

Basic problem here is exertional dyspnea which can not be accounted by respiratory problem.So reasons for pulmonary venous congestion due to raised left atrial pressure 

Mitra valve pathology either stenosis or regurgitaion or both most common,

Aortic valve pathology whether stenosis or regurgitation usually presents with dyspnea late , only when the left ventricle fails. During this phase syncope or exertional chest pain if present may pin point to pathology of aortic valve, either steonosis or regurgitation can lead to this.

Here no history of joint pain does nt totally rule out the possibility of rheumatic valvular heart disease as there may be situation where the arthritis history may not be there always. May the a situation of "lick the joints and bite the heart " silent carditis.

Other reason for this is dilated cardiomyopathy, or any of the above entities which leads on to the left ventricular failure.

In our case there was no odema , abdominal discomfort or pain.

So our first clinical possibility in this case was rheumatic valvular heart disease mitral valve involvement either stenosis or regurgitation. 

General xamination 

• Underweight girl , average height ,looks thin,mild dystess at rest. 
• Mild pallor which can not explain the failure 
• No pedal oedema, afebrile,conscious cooperative 
• No clubbing, No lymph node enlargement , No rash,Normal joints No features of micro nutrient deficiency apart from mild pallor 

After the resuscitation Findings in the CVS 

• Pulse on the right hand good volume, non collapsing 
• Interestingly we noticed her left radial pulse was lower in volume and her peripheral pulses were not felt in lower limbs including femorals 
• This was a turning point 
• BP measured in the four limbs 
• Right upper limb BP 140/70
• Left upper limb  100/60
• Both lower limbs systolic BP 80/40
• Both carotid pulse were felt normally 
• There was no bruit over carotids 
• No bruit heard in the supraclavicular area, No bruit over renal arteries.

So following possibilities were considered

As the BP on right upper limb is high and systolic diastolic difference this high there is possibility of aortic regurgitation 

As the systolic BP between upper limbs were this different and left common carotid good volume there must be an obstruction beyond left carotid but proximal to the left subclavian 

As the BP was still lower in the lower limbs compared to left upper limb there must be obstructions of higher grades below left subclavian 

• We did nt get evidence of renal artery oclusion clinically. We could nt get bruit over aorta also 
• JVP. was raised 2 cms 
• Chest , there was no precordial bulge no hyperactivity apex not visible 
• apex palpation was difficult but thought it was in the sixth space outside midclavicular line on left side No left parasternal haeve
• Ausculation first sound was normal, second sound normal, wide variably split. No third heart sound or fourth heart sounds 
• No pericardial rub or knock

Repiratory system 

air entry was equal ,basal crepitations over both base 

 GIT

Liver palpable 2cms soft 

So where are we now

Hard finding suggest a major vessel disease, aortic leval pathology 

Is it coarctation, slowly evolving or aortic vasculitis 

Here there is evidence of constriction at multiple sites, Very interesting point is major part of aorta and major branches spared but more significant block beyond left subclavian.

Is there a aortic regurgitation 

We thought of the possibility as there was a BP difference  but there is no Early diastolic murmer 

Anyway the left ventricluar dysfucntion either due to aoric leak or a failure secondary to significant outlet obstruction. later leading on to left sided symptoms.

What is the pathology likey, Vasculitis major vessel, Whether the tuberculosis , contact has something to do with it ?

we planned to work on this 

Now only we understood why she was send to the radiology department, after doing an ECHO and report saying DCM.

They noticed our findigs thought of this possibility and were send to do this investigations 

Now see the ECG , and The imaging 

I dont have the Xray whic showed cardiomegaly and venous engorgement of upper zone boths sides 

P mitrale, evidence of left atrial enlargement
Sigificant Left ventricular enlargement.
I could t make out pressure or volume overload situation of LV
There is rsr pattern but the duration is not significantly longer



See the narrowing of the aorta mainly in the descending path, there is narrowing in lower chest and abdomen , multiple sites. Some of the area too narrow

Final fate 

We were planning to send her to higher centre

But 

Yesterday she developed sudden onset of dyspnea and went in shock 

She was shifted to PICU, 

All measures failed 

We lost her 

So 

Coming back to the original episode

was the first episode anaphylaxis or a similar event from which she escaped 

What must have occured as terminal event in this case ?

1. dissection of aorta ?
2. arrhythmia ?
3. block of narrow segment of aorta ?

Dont know 

Lot of scope for different views by experts