Download TERRIBLE T`s

THE “TERRIBLE T’s”
Cyanotic Heart Disease
GOALS
• To define the physiology of the five most
common cyanotic defects
• To describe the long term sequelae of the
corrective procedure.
• To describe the PE,ECG, and CXR results
for the more common defects.
INITIAL WORKUP: THE
HYPEROXY TEST
• Give the patient as close to FiO2 of 1 for at
least 5 minutes.
• Obtain pre and postductal ABG’S
• What should your PaO2 be on 100% O2?
• Is it reasonable to expect to get 100% using
a mask or hood?
CXR
• Mainly we are looking for situs, PBF,
cardiac size and noncardiac abnormalities.
• Boot shaped, apple shaped, box shaped,
valentine shaped, egg on it’s side shaped,
ball on string shaped, boiled peanut
shaped…el toro poo poo.
ECG
• Get it! If you can’t read it fax it!
• Remember most term infants have relative
RV dominance so a big LV would be
unusual.
• Unless it’s a significant premie where there
hasn’t been time to develop the pulmonary
vascular bed. In a premie, RVE is not the
norm
WHAT ARE THEY?
•
•
•
•
•
Tetralogy of Fallot
d-Transposition of the great arteries
Truncus arteriosus
TAPVR
Atresia’s
TETRALOGY of FALLOT
• See handout for basics.
• The primary concerns after correction are:
the degree of Pulmonary insufficiency, the
presence of tachydysrhymias, and residual
VSD’s, and the presence of significant
supravalvular pulmonary stenoses.
• There seems to be a degree of mild learning
disability post op, also ADHD.
d-TGA
• The most cyanotic common defect
diagnosed in the nursery.
• The degree of saturation will depend on the
degree of mixing of the 2 “parallel”
circuits.The mixing sites are: ASD, PDA,
and VSD. The more mixing, the higher the
“effective pulmonary blood flow”
EFFECTIVE PULMONARY
FLOW
• Blood going to the lungs which has not
been to the lungs in that cardiac cycle.
• PGE acts to increase mixing at the great
vessel level.
• BAS increases mixing at the atrial level
• We try not to increase mixing at the
ventricular level.
CLINICAL Findings d-TGA
• Cyanosis
• Possibly CHF
• If pulmonary stenosis present, may be very
blue
• S2 normally split in 1/3, narrow and loud in
2/3
• ECG: RAD with BE. May be wnl in nursery
• CXR: egg on string.Narrow medias.^PBF
D-TGA cont.
• Cath findings: slides
• ECHO: classic findings are anterior great
vessel gives off CA’s, posterior vessel gives
off two branches, may look like circle,circle
d-TGA TREATMENT
• PGE: usually all that’s needed to improve
mixing and relieve the acid base issues. At
least long enough to get the patient to a
center.
• BAS may be necessary if PGE not effective
and there’s a delay in correction.
• MUSTARD: see slide
• SWITCH
MUSTARD SEQUELAE
• Sinus node dysfunction: 10 yrs out less than
30% in dominant sinus rhythm.
• Atrial tachyarrhymias.
• Ventricular arrhymias….sudden death
• Coronary perfusion abnormalities
• RV failure
ASO: follow -up
• Supravalvular obstruction: most commonly
PS with frequent LPA stenosis.
• Coronary occlusion: occurs early. Had to
correct if later
• Neo-aortic regurg: more frequently
reported. No cases requiring surgical
correction but a few have required medical
intervention
TAPVR
• Cyanosis variable and largely dependant on
degree of pulmonary venous obstruction.
• CHF found in 41%, if early probably
because of pulm vein obstruction. If later,in
those without pn obs.
• One of the few defects which can give you a
big heart and cyanosis. The other is
Ebsteins.
TAPVR cont.
•
•
•
•
ECG: RAD,RVE, reversal of r/s
progression,RAE by 3 months
Snowman on CXR
ECHO: usually a smallish LA,
abnormalities of venous return(difficult to
find any PV’s coming to LA). May find the
common vein.
TAPVR
• The patients are not infrequently our sickest
as infants. The are profoundly hypoxic and
frequently acidotic.
• The biggest post-op issue is pulmonary
vein stenosis. If that’s present, pulmonary
HTN is inevitable and extremely difficult to
tx.
TAPVR: ECMO
• Because of the extreme condition many of
these infant present in, some of them have
been felt to have PPHN and have been
submitted for ecmo. This was especially
true before the availability of color doppler.
This meant a number of these patients were
in much better condition when they went for
repair.
TRUNCUS ARTERIOSUS
• A SINGLE GREAT VESSEL ARISES
FROM THE HEART AND GIVES OFF
THE CA’S,PA’S AND AORTA.
• 19TH MOST COMMON CONGENITAL
HEART DEFECT
• ONE OF THE LESIONS ASSOCIATED
WITH RIGHT ARCH AND AORTIC
OVERRIDE.
TRUNCUS cont
• 4 basic types based on the source of PBF.
The fourth is pulmonary atresia with vsd
otherwise known as pseudotruncus.
• 11% of cases have interrupted aortic arch
• The is associated DiGeorge syndrome
• There may be associated pulmonary
stenosis
Truncus
• If there is no pulmonary obstruction, the
dominant picture is CHF with varying
degrees of cyanosis
• There may be significant insufficiency of
the truncal valves which can have 2-5
cusps.
• Bounding pulses(it’s like a big PDA), There
may be a continous murmur if the PA’s are
tight
TRUNCUS
• ECG: LVE,RVE OR BE. OCC LAE
• CXR: RIGHT ARCH 30%, ^PBF
• ECHO: USUALLY DIAGNOSTIC
TRUNCUS:Tx
• Decongestive tx pending surgery
• Surgery consists of VSD closure and a graft
to the PA’s.
• Early surgery essential.The average age of
death untreated is 5 wks.
• Sequelae:depends on degree of truncal
valve insufficiency and pulm artery
obstruction