[HSF] Tetralogy of Fallot

[Nasser F. Abou'Seada]

Dear Professor Novick 
	I quite agree with you that a shunt would not be an ideal low risk
procedure. also I do concur that BPV might not be that adjusted and can lead
to catastrophes and at least would be a blind procedure. the pulmonary valve
is NOT likely to open along the fused fibrosed commissures "55 years", would
rather tear in an UNCONTROLLED manner along the weakest direction with a
resultant UNCONTROLLED pulmonary regurgitation. I'd think of this option if
the PATIENT -rather than the PROCEDURE- is a high risk one. 

	I can not see why a palliative procedure would increase heart
failure ?? I would have thought that a release of pulmonary stenosis,
without overdoing it, would relieve the pressure overload of the RV ! and LV
!!  "non-restrictive VSD" . I'd have thought as well that increasing the L-R
shunt fraction would not affect the LV already suffering from Volume
overload-AR ! .. au contraire, it would decrease the fraction of AR ... !! 

Essentially... I would think a palliative procedure increasing PaO2 from 40s
to 90s would improve her blood oxygen content %, having beneficial effects
on the Oxygen % extraction by the ventricles !! 

	I do look at her central cyanosis, Hb 22gm%, HCt 64%, DOE class II,
and history of NON-pulmonary oedema HF, increased systemic venous
congestion, as indications for an intervention. 

I wonder whether bypassing her already occluded RCA will be of any benefit,
as the infarct is already fibrosed. I wonder about an AVR with such
longstanding hypoxic ventricles .., as I wonder whether she would really
benefit from a high risk procedure to totally correct such an anomaly in
such an age. 

Reaching an age of 55 with such an anomaly indicates that she's got
sufficient collaterals to provide her with sufficient PBF ... CERTAINLY many
MABCA's ... probably her signs and symptoms are a result of LATE increased
infundibular stenosis reducing PBF below a threshold, and an added AR.
Total Correction at this stage without looking & blocking MABCA's would have
catastrophic sequelae ... on table ... ! 

it is a dilemma, 
I've seen quite some number of these OLD Fallot's during my training .....
with worst results on INTERFERENCE with adapted haemodynamics, and
compensating PATHOPHYSIOLOGY .... 
I'm sure you have seen much more and more cases of this type. Your opinion
and your comments would be kindly appreciated. Thanks for your input indeed,
this is our chance to learn. 

Yours  
 

NFA

> From: prasannasimha
> Bill,
> the reason for the PBV was to decrease cyanosis and not for reducing RV
> pressure Her subvalvar stenosis would not allow her to "overflow"
> anyway. I meant this only as a temporizing measure so that the cyanosis
> can increase and make it more easily manageable when the full correction
> would be done. It would act as a "natural" band anyway.
> Prasanna
> 
> ICHFNO wrote:
> > Dear Daniel:
> > Interesting problem. What palliative procedure would you do, that
carries a lower
> risk than total correction? Shunt? No, not likely to be low risk with
severe aortic
> regurgitation. Pulmonary valvotomy, either Brock performed by you, or
Balloon
> Valvotomy in the cath lab by your interventionalists? Interesting idea,
and once this is
> done, how would you be sure that you/cardiologists did not relieve the PS
to much
> and provide her with excessive pulmonary blood flow and subsequent
pulmonary
> hypertension? By the way, this procedure does not result in a decrease in
her RV
> hypertension, by definition, TOF type VSD's are the size of the aortic
valve, so
> releasing the PS would not reduce her RV hypertension below her systemic
pressure,
> i.e., the VSD is non-restrictive, so her LV and RV pressures would be
equal. So any
> palliative procedure that you do will actually increase the degree of
heart failure, not
> diminish it. I do not see the TOF as the issue for this patient, yes it
complica
> >  tes the care, but what she needs is her aortic valve replaced, RCA
bypassed, and
> her TOF corrected all in one sitting, this actually is the lowest risk
procedure for this
> patient, both short term and long term. Let us know what you decide, and
what she
> accepts.
> >
> > Sincerely,
> > WNovick