16) Congenital heart diseases – cyanotic tetralogy of Fallot. Flashcards
Most common CCM (congenital cardiac malformation) in adults:
Interatrial septum defect
interventricular septum defect
Pulmonary stenosis
Patent ductus arteriosus Botalli
what are the normal pressure in the chambers of the heart and their respective vessels
right atrium - 0-4 mmhg
right ventricle = 25/4 mmhg
pressure in pulmonary artery - 25/10
left atrium- 8-10
left ventricle - 120/10 mmhg
pressure in aorta 120/80
classification of congenital heart disease with shunt ?
WITHOUT CYANOSIS = LEFT TO RIGHT SHUNT
intratrial septum defect
intraventicular septum defect
atrioventricular canal defect
patent ductus bothali
WITH CYANOSIS - RIGHT TO LEFT SHUNT
Fallot tetralogy
Eisenmenger syndrome - ventricle septal defect
Ebstein tricuspid anomaly
transposition of great arteries
truncus communis arteriosis
hypo plastic left heart syndrome
what is the classification of congenital heart disease without shunt
WITHOUT SHUNT
aortic / pulmonary stenosis - valvular , sub , supra
coarctation of aorta
dextrocarda
dextroversion
levoverson
what are the general clinical features of CONGENITAL HEART DISEASE WITH shunt and is CYANOTIC ?
blue babies opal grey
SQUATTING FOR RELIEF DURING HYPOXEMIC EPISODES
exertion dyspnea and tachypnea
nail clubbing
failure to thrive
what is the definition of TETRALOGY OF FALLOT
1) right ventricular outflow obstruction / because of SUB VALVULAR pulmonary stenosis
2) right ventricular hypertrophy
3) Ventricular septal defect
4) Overriding aorta (above the VSD)
TETRALOGY OF FALLOT is sometimes associated with sometimes associated with what genetic defects
Down syndrome, DiGeorge syndrome
in tetralogy of fallot what determines the severity ?
The extent of right ventricular outflow tract obstruction and central pulmonary hypoplasia
vsd
mid subvalvular stenosonsi - left to right shunt no cyanosis
severe subvalvular stenosis
right to left shunt
however through time it evolves into more and more subvalvular stenosis
what are the clinical findings specific to tetralogy of fallot
Tet spells = develop deep blue skin, nails and lips
occur suddenly and are potentially lethal. They are due to an activation of the sympathetic nervous system, which triggers spastic contractions of the hypertrophic infundibular musculature (at the transition from the right ventricle to the pulmonary artery).
hypercyanotic, hypoxic episodes with a peak incidence 2–4 months after birth
psychological and physical stress (e.g., crying, feeding, defecation)
Untreated young children tend to squat.
what are the diagnostic indication for tetralogy of fallot ?
> harsh systolic murmur that is best heard over Erb’s point = HOWEVER
With the malformation progression it may weaken in intensity
Intensity increases in squatting position
Pulse oximetry: ↓ SpO2
Hyperoxia test: to distinguish cardiac from pulmonary causes of cyanosis
= PaO2 is measured during the administration of 100% oxygen.
ECG: right axis with right heart hypertrophy
P pulmonale with increasing age
P waves with an amplitude > 2.5 mm in the inferior leads II, III, and/or aVF and also V1 . It is a result of right atrial enlargement
echo - golden standard
chest x ray -small “boot-shaped” heart
Manometry
what is the treatment strategy for tetralogy of fallot ?
if severe RVOT - give IV prostaglandin until surgery
acute hypoxia - such as tea spells
- administer oxygen
knee to chest position squatting
treatment for heart failure
such as diuretics
ionotropic drugs - digoxin
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long term managmnet surgery VSD repair correct aortal positioning enlargement of the RVOT -
definitive surgical correction
there can also be palliative surgery - anastomosis between arterial vessel and pulmonary artery to give pulmonary artery
what are the specific risk factors for transposition of great vessel ?
Intrauterine risk factors
- Infants born to diabetic mothers
- Maternal alcohol
- Maternal age > 40 years
- Rubella infection or other viral illness
what are the clinical findings ?
incompatible with life unless mixing occurs such as VSD or ASD or PDA
so there is early severe postnatal cyanosis
and tachypnea
what are the diagnostic findings specific to TGV?
Auscultation
Single loud S2
If concurrent VSD is present: systolic murmur at eras point
echo
pulse oximetry
XRAY
heart with egg on string appearance
what is the treatment for TGV ?
infusion of PGE1 - prevent closure of PDA
surgical correction within first two weeks of life
balloon atrial septostomy
- if surgery cannot be performed or hypoxia is severe
the procedures right heart catheterisation with creation or enlargement of existing ASD
what is tricuspid valve atresia ?
no blood flow between the right atrium and the right ventricle
ALMOST ALWAYS accompanied with ASD AND VSD and right ventricular HYPOPLASIA
what are the auscultory findings with tricuspid valve atresia ? and what is the diagnosis ?
if VSD is present - rough holosystolic murmur of left sternal border
echo
pulse oximetry
chest x ray
ecg - left axis deviation because left ventricular hypertrophy
right atrial enlargement so p pulmonate
minimal r waves in precordial leads
what is the treatment for tricuspid valve atresia ?
infusion of PGE1
three staged surgical procedure - without surgery children die in early childhood
what is ebstein anomaly ?
malformed and displaced tricuspid valve leaflets - giving rise to tricuspid regurgitation and right atrial enlargement
usually associated with patent foramen ovale , ASD and etc
what are the causes for ebstein anomaly ?
prenantal lithium exposure
genetic defects
what is the pathophysiology of ebstein anomaly ?
displaced valve = reduction in ventricular volume
regrurgitation = atrial dilation
small RV - artrialisation (right ventricle becomes part of the right atria) of the right ventricle
functional pulmonary valve atresia - obstruction of the rv by the large sail like anterior leaflet
so blood flows through patent foramen ovale - right to left shunt - cyanosis
what re the clinical findings of ebstein anomaly ?
prenatal - asciti or hydrops fetalis
pleural effusion
pericardial effusions
postnatal
what are the diagnostics for ebstein anomaly ?
tricuspid regurgitation - wide split S2 , holosytolic murmur at left sternal border
prenantal - ultrasound
echocardiography
xray - cardiomegaly with ra enlargement
ecg - right axis deviation
what is the treatment for ebstein anomaly ?
IV PGE1
surgery
systemic pulmonary shunt
repair of tricuspid valve
reconstruction of right ventricle
what is patent truncus arteriosus?
Failed separation of aorta and pulmonary artery during development → single trunk that receives output from both ventricles
what is patent truncus arteriosus associated with ?
di george syndrome
what are the clinical features of truncus arteriosus ?
accented and bounding peripheral pulses
and symptoms of heartfailure such as dyspnea
what are the diagnostic confirming patent truncus arteriosus?
auscultation - harsh systolic murmur - lower left sternal border
loud s2
echocardiograph
pulse oximetry
chest x ray - right aortic arch but no pulmonary artery
what is the treatment for persistent truncus arteriosus ?
stablaistion of hemodynamics - diuretics , dopamine , ventilation , correction of metabolic acidosis
surgical REPAIR DURING NEONATAL PERIOD - or die within first year of life
