36. Congenital heart disease without shunts. Flashcards
what are the congenital heart diseases without shunts ?
pulmonary valve stenosis
aortic valve stenosis
coarctation of the aorta
hypoplastic left heart syndrome
aetiology of pulmonary valve stenosis ?
associated with tetralogy of Fallot and Noonan syndrome
Maternal rubella
clinical features of pulmonary valve stenosis ?
Depending on the grade of stenosis, symptoms of heart failure may occur
dyspnea and easy fatigability;
severe stenosis may be more
symptomatic and even cyanotic because of right-to-left shunting at the atrial level
most of theme they are asymptomatic
physical examination of pulmonary valve stenosis ?
Systolic murmur heard best over the second left ICS at the sternal border which radiates to the back
S2 wide splitting
systolic ejection click - which decreases in inspiration
diagnosis of pulmonic valve stenosis?
echocardiography - doppler
ecg
right axis deviation
prominent R waves in V1 and R waves in AVR
typically not required
Cardiac catheterization
pulmonary angiography
what is the treatment for pulmonic valve stenosis ?
Asymptomatic patients with peak doppler gradient >60 mm Hg are recommended for balloon valvotomy.
trans catheter balloon pulmonar valvuloplasty
surgery - commissurotomy is balloon dilation is not possible
what is the pathophysiology of aortic valve stenosis ?
bicuspid aortic valve - which occurs in utero fusion of two or thee aortic valves
predisposes patents to dystrophic calcification and degeneration
clinical manifestation of aortic valve stenosis
critical stenosis
symptoms of congestive heart failure, including poor feeding, rapid breathing, poor urine output, and fussiness, as the ductus arteriosus closes within the first few days of life and systemic blood flow decreases
appear pale
mottled skin
hypotensive
Older children are usually asymptomatic
- dyspnea
angina
syncope
what re the physical examination findings of aortic valve stenosis ?
decrease amplitude of blood pressure
decreased pulse pressure
pluses parvus et tarsus - weak and delayed
systolic thrill over carotid
auscultation harsh crescendo decrescendo late systolic ejection murmur - best heard in 2nd right ICS which radiates to the carotidd early systolic ejection click soft S2 S4 HEARD IN APEX
diagnosis of aortic valve stenosis?
ECHOCARDIOGRPAHY
ecg - sokolow yin index for left ventricular hypertrophy
chest x ray - pulmonary edema
cardiac catheterisation / coronary angiography
what is the management of aortic valve stenosis ?
Neonates who are critical, aggressive treatment with prostaglandin E1 infusion, inotropic support, diuretics,
correction of metabolic acidosis and mechanical ventilation to improve the systemic perfusion
significant aortic valve insufficiency in combination with mild to moderate stenosis may be carefully treated with afterload reduction =diuretic therapy although with caution it can cause hypotension
vasodilators such as nitroprsside
Inotropic drugs, such as dopamine, dobutamine, and epinephrine, may be indicated in cases of reduced cardiac output in the presence of decreased left ventricular systolic function
Patients with increased work of breathing and pulmonary edema benefit from intubation, positive pressure ventilation, and diuretic therapy.
for those symptomatic with angina, syncope or dyspnea on exertion
asymptomatic but developed ST/T-wave changes on ECG at rest or with exercise
asymptomatic but have a catheterization peak LV-to-peak aortic gradient > 60 mm Hg
percutaneous balloon aortic valvoplasty,
surgical aortic valvotomy
and valve replacement, all of which are intended for biventricular repair
what causes congenital coarctation of aorta?
Associated with Turner syndrome
accompanied by a bicuspid aortic valve , VSD, and/or PDA
Congenital coarctation of aorta is often accompanied by what?
Bicuspid aortic valve
VSD
PDA
What are the charachteristics of coarctation of aorta
Increased flow proximal to the narrowing and decreased flow distal to it
In preductal (infantile) type which is the most common the narrowing is proximal to the ligamentum arteriosum or ductus arteriousus
Post ductal rule narrowing is distal to it
Symptoms of coarctation of aorta?
As long as there is patent ductus arteriosus and the aortic narrowing is not that constrictive = which is true in most cases- it symptomatic
Otherwise there is differential cyanosis especially pertaining to the lower extremities
Cold feet
Lower extremity claudification
There is brachial femoral delay
Blood pressure in the upper extremities are higher than in the lower extremities
If narrowing is distal to subclavian artery
If origin of subclavian artery is the point it narrows them bp in right arm higher than that of left arm and cyanosis in the left arm
Brachiocephalic hypertension : headache, epixstasis, tinnitus
What are the physical findings in coarctation of arota?
Systolic ejection murmur of the left posterior hemithorax
Or continuous murmur below the clavicle between shoulder blade.
Blood pressure brachial femoral delay
Pulse oximetry is low
Diagnosis for coarctation of aorta ?
Echo- Doppler
X ray - cardiomegaly , figure of three sign
Rib notching - collateral blood flow to intercostal vessels and interthoracic vessels
Enlarged vessels compress the rib
Affects the inferior border of the 3rd and 8th rib
Genetic testing for Turner syndrome
MRI only in complicated cases
Treatment for coarctation of aorta ?
Initial management is the infusion with the prostaglandin E1
Surgical correction or balloon angioplasty for below 5 years
Older transcatheter stent placement
What are the complication of coarctation of aorta ?
Secondary hypertension
Aortic dissection and rupture
Berry aneurysm
What is the etiology of hypoplastic left heart syndrome
Trisomy of 13 - patau syndrome
18 - edwards syndrome
Turner syndrome
What is the symptom for hypoplastic left heart syndrome
Asymptomatic but when patent ductus arteriosus closes aggregation Cyanosis Tachypnea Hypotension Cool extremeties
What are the physical findings in hypoplastic left heart syndrome
Just a loud S2
Treatment for hypoplastic left heart disease
Continuous prostaglandin infusion
Surgical repair Which is three step staged Norwood procedure - neonate Glenn procedure - 3-6 months Fontan procedure - 2-3 years
Even with the procedure the five year survival is low
