[HSF] Tetralogy of Fallot

[Nasser F. Abou'Seada]

Dear Dr. Daniel 
	I would agree more with John about the strategy and technique for
this case. a case of an old TOF of this age, one should be clear in his mind
about the INCIDATIONS for "interference", AIM of any "intervention",
Strategy adopted and Technique to be utilized. 

	I do totally concur with John that the aim should be to reach a
status of "Pink Tetralogy" rather than a total correction. 

With an occluded RC, I would not think of a total correction for a
compromised RV, "options for revascularization ???" ..
Trying to radically correct such case and subject the RV to a volume
overload whereas it has been for 55 years a victim of pressure overload,
might just get the operator to cry by her side, not being able to do
anything. The anatomy and pathophysiology or her hemodynamics should be
looked at in view of her reaching such an age of 55, with such a diagnosis
of TOF, a rather totally different pathology. 

Moreover; a severely HYPERTROPHIC LV, 14 mm, with EDD 56mm, in face or a
severe AR ..!!! ... can be accounted for by the VSD-related haemodynamics,
but in no way can the EDD be in such severe AR ...!! ..... I'd be very
careful in evaluating such finding. Let alone thinking about correcting AR
...!! 

Closure of the VSD - in HER particular case- might just as well end in a
ventricular failure, Depriving such an RV from an assisting hypertrophic LV.
!! 

	I do agree with John and Prasanna that "HER" problem can be
approached via augmenting her PBF, better through the PV with a protective
infundibulum than to flood her lungs by a systemic to pulmonary shunt
"central?" ..... patient would drown in her own blood ..on table !!! .....
An MBTS, should that be ever feasible in her case, would increase work of
the heart greatly, a compromised heart already "VSD, AR, RC occlusion,
Ischaemic hypertrophic LV, ... "

I totally concur with John that the "trick" is to avoid over-resection of
the infundibulum, flooding the lung with increased PBF .... pt would drown
in her own blood ... 

Doming of the valve is a good sign in the eyes of performing a suitable
intervention - rather than interference- in her particular case. I do agree
that using BPV would ravage the valve, in a severely blind way, tearing the
valve in a non expected manner, especially in such an age. In a non
complicated case I would have thought of open trans-PA pulmonary valvotomy
where the commissures can be split under vision and accurately freed /
separated from the PA wall. still, I'd be very careful to consider an
ischaemic arrest in such a case, with prohibited total correction, and AR,
IWMI. 

I do totally concur with John about utilizing a closed heart method, like
the Brock's procedure, through the RV ... valve will not be ravaged,
protective infundibulum -in HER case- is secured, especially that it appears
that the obstruction is mainly valvular. a severe infundibular stenosis
would be incompatible with her age !! ... also a proper gradient -and blood
flow- can be worked out while on table.

Should that fail, there is always an option of an on pump open release of
PV, and calculated resection of the obstructing infundibulum "should be
worked out from cath" 

I would think that the main aim - achieving a status of PINK FALLOT, rather
than total correction- is to determine the strategy adopted and technique
used. 

As John Hutter of Bristol Royal Infirmary 1990 once told me, it is not
courage to jeopardize a man's life, doing a rather text book procedure for a
text book indication, as I believe that it takes a surgeon 6 weeks to know
HOW to do it, 6 years to know WHEN to do it, 60 years to know WHEN NOT to do
it. 

Good luck with your case 

please keep us posted. 

Yours   

NFA

 From: jbflegejr at aol.com
 This patient sounds like a good candidate for the Brock procedure which
 probably has not been performed in the U.S. for the past 40 years and
 maybe no place else either, but that does not negate its value in
 selected cases, and where the principal obstruction is of the pulmonary
 valve which you describe, it should be considered. The goal is to
 reduce the pulmonary outflow obstruction enough to allow the pulmonary
 flow to approximate the systemic flow and produce what is sometimes
 termed "acyanotic tetralogy". In London and later in Iowa City we did
 this with Hegar dilators passed through the wall of the right ventricle
 and dilated the valve progressively until the pulmonary artery
 pressures were around 30 to 40 mmHg and the arterial oxygen saturations
 were above 90%. A left anterior thoracotomy or a median sternotomy
 provides exposure but the sternotomy would be best as more options
 would be available to you. It is important not to overdo it and flood
 the lungs. Infundibular obstruction can be relieved using a Brock punch
 through the right ventricule but this may be difficult in the older
 adult and probably should not be done by someone without a lot of
 experience. From your description, it sounds predominately valvular.
 One might be tempted to try a PCI with a dilating balloon which might
 be disastrous since the secret to success is to do just enough and not
 too much. A systemic pulmonary shunt might be considered but cannot
 achieve nearly as high arterial oxygen saturation as the Brock
 operation and increases the cardiac work more. Brock and others wrote
 about this in 50's and 60's. John Flege

From: biga at multitel.com.uy
Dear members:
I would like to know your opinions and treatment about this case.
55 year old woman with an unoperated Tetralogy of Fallot. 
This year she has been Hospitalized because of HF.
She  refuses the definit surgery but does not a palliative procedure of
lower risk.
The following describes her clinical situation and the last echocardiogram :
Age : 55. White race. 
Works in an office. She describes excercice disphnea, functional class II. 
She has had hospitalized 3 times this year because HF (not acute lung
edema).
She has elements of systemic venous hypertension as low limbs edema,
yugular and high limbs venous ingurgitation. 
She has universal cyanosis and elements of cronic hypoxemia.
 
Last transthoracic echocardiography  showed : 
50% overriding of the aorta, 
wide ventricular septal defect with few bidireccional flow (probably because
of equal pressures), 
left ventricle hypertrophy 14 mm, diastolic diameter of 56 mm, 
right ventricle hipertrophy, 
bi-atrial dilation, 
ejection fraction of 40%, 
pulmonic valve with domo movement, maximum gradient of 100 mmHg at pulmonary
valve, 
hypertrophy of infundibular area, 
severe aortic regurgitation, 
dilation of inferior vena cava. 
There is no angiography of pulmonar vessels.


She also has comorbidities : 
grade I esophagus varices, 
inferior myocardial infarction in 2002 by occlusion of the right coronary.

Laboratory shows an hemoglobin of 22 g/dl, hematocrit of 64%, 123000
platelets,
creatinin of 1.3 g/dl.
Arterial gasometry PaO2 of 40 mmHg while compensated.

Best regards
Dr. Daniel Bigalli
Montevideo-Uruguay