CONGENITAL HEART DEFECTS II

DR. MWENDWA
 OBJECTIVES
1. To discuss clinical presentation,
diagnosis, management and
complications of the Acyanotic
Congenital Heart defects.
 ATRIAL SEPTAL DEFECTS
Definition:
• An atrial septal defect is an opening in the
atrial septum, which allows oxygen-rich
blood to pass from the left atrium,
through the opening in the septum, and
then mix with oxygen-poor blood in the
right atrium.
• They are twice as common in females as in
males.
The atrial septum
Cartoon
 Clinical presentation
• Patients with small or moderate ASDs
and with a PFO are asymptomatic unless
a complication occurs.
• With large shunts, exertional dyspnoea
or cardiac failure may develop, most
commonly in the fourth decade of life or
later.
 Cont..
• A moderately loud systolic
ejection murmur can be heard in
the second and third interspaces
parasternally as a result of
increased PA flow.
 Diagnosis
• Baselines: FBC, U/E/C, RBS
• LFTs
• Coagulation profile
• CXR
• Echocardiography demonstrates
evidence of RA and RV volume
overload.
CXR in ASD
 Management
• Small ASDs do not require
intervention.
• Larger defects require surgery
 Questions
???
 VENTRICULOSEPTAL DEFECTS
Definition:
• A VSD is a defect in the ventricular
septum, the wall dividing the left and
right ventricles of the heart.
• The ventricular septum consists of an
inferior muscular and superior
membranous portion.
cartoon
 Pathophysiology
• During ventricular systole, some of
the blood from the left ventricle
shunts into the right ventricle, passes
through the lungs and reenters the
left ventricle via the pulmonary veins
and left atrium.
• This has two significant effects:
 Pathophysiology cont..
1. Volume overload on the left ventricle.
2. Elevation of right ventricular pressure
and volume, resulting in pulmonary
hypertension. (This is because the left
ventricle normally has a much higher
systolic pressure (~120 mm Hg) than the
right ventricle (~20 mm Hg), therefore
the left to right shunt)
 Anatomical classification of VSDs
There are four types:
• Type I: para/ perimembranous - most
common
• Type II: Muscular (inlet, swiss-cheese) –
least common
• TypeIII: Outlet
(supracristal,subpulmonary,infundibular
or conoseptal)
• Type IV: Atrioventricular : Avcanal defect
 Clinical presentation
• VSDs are usually asymptomatic at birth,
usually manifesting a few weeks after birth.
• Clinical symptoms depend on the size of
the defect.
• Patients with larger defects present with
breathlessness, diaphosesis (excessive
sweating), poor feeding, frequent chest
infections and failure to thrive in infancy.
• Patients with smaller defects may be
asymptomatic.
 Cont.. Exam findings
• Heart sounds are normal.
• Pansystolic murmur (depending upon the
size of the defect) maximal at the left
sternal border. (A Small VSD gives a louder
murmur that a large VSD)
• +/- palpable thrill (palpable turbulence of
blood flow).
• Larger VSDs may cause a parasternal heave
and a displaced apex as the heart enlarges.
 Diagnosis
• A VSD can be detected by cardiac
auscultation.
• Classically, a VSD causes a
pathognomonic holo- or pansystolic
murmur. Auscultation is generally
considered sufficient for detecting a
significant VSD.
 Cont.. diagnosis
• Echocardiography to confirm
auscultatory findings.
• CXR shows:
- an enlarged heart: biventricular
hypertrophy
- Increased vascularity in the lung fields
- Or can be normal in small VSDs
 Treatment
• Treatment is either conservative or surgical.
• Smaller VSDs often close spontaneously as the
heart grows by the age of 7-8 years, and in
such cases treated is mostly conservative.
• VSDs in infants is initially treated medically
with:
-cardiac glycosides - digoxin
-loop diuretics – furosemide
-ACE inhibitors -captopril
. Surgical intervention
Questions – still awake?
 PATENT DUCTUS ARTERIOSUS
Definition:
• Patent ductus arteriosus is a
connection between the aorta and the
pulmonary artery, which allows
oxygenated blood that should go to the
body to re-circulate through the lungs
• It is usually noted in the first few weeks
or months after birth.
cartoon
Fetal circulation
 History of the Ductus Arteriosus
• The ductus arteriosus in the fetus is an
important conduit that allows
deoxygenated blood to bypass the
collapsed lungs and enter the placenta
through the descending aorta and
umbilical arteries
• The placenta acts as an oxygenator and
returns oxygen rich blood through the
umbilical vein and ductus venosus to the
fetal heart.
 Patency of the ductus
• Factors that maintain patency:
-prostaglandins produced by the placenta and
the ductus itself
-nitric oxide like vasodilators produced by the
ductus
-low oxygen tension
• Factors that promote closure:
-high oxygen tension
-indomethacin
 Closure of the ductus
• During the first 60 hours of life,
spontaneous closure of the ductus
occurs in 55% of full-term newborn
infants.
• By 2-6 months of age, closure occurs
in more than 95% of healthy infants
 Persitence patency
• Persistent patency of the ductus
arteriosus following birth is inversely
related to gestational age.
• Reopening of the ductus:
- may occur in asphyxia
- pulmonary diseases (as hypoxia and
acidosis relax ductal tissues).
 Pathophysiology
• The direction of blood flow across the PDA
depends on the balance of pulmonary
and systemic vascular resistance.
• The secondary effects of the increased
flow lead to volume overload from the left
to right.
• A large shunt leads to dilation of the left
atrium and left ventricle.
 Clinical presentation
• Frequent chest infections
• fatigue
• sweating
• rapid breathing
• Heart failure
• disinterest in feeding, or tiring while
feeding
• poor weight gain
 Physical exam
Inspection:
• Carotid pulsations
• Hyperkinetic apical impulse
Palpation:
-systolic or continous thrill at the 2nd left
interostal space
Auscultation: continous/machinery murmur
at the 2nd LICS
 CXR findings
• Cardiomegally
• LA enlargement
• LV enlargment
• Prominent aortic Knuckle
• Pulmonary plethora
Cont..
 Other diagnostic Workup
• ECG: left ventricular changes
• ECHO: mixing of the oxygenated
and deoxygenated blood
demonstrated
• Cardiac catheterization: pressures
in the chambers and pulmonary
trunk
 Management
• MEDICAL
- Indomethacin (prostaglandin E1 inhibitor)
may close a PDA if given in the first 10 days
at 0.1 mg/kg/dose, orally, bd for three doses
-Digoxin for increasing working capacity of
heart
-Diuretics (furosemide)to reduce preload on
heart
-fluid resctriction
 Cont.. SURGICAL
• After one week following indomethacin
use, surgical closure by ligation, clipping,
or division can be done.
• Out of the neonatal period, cardiac
catheterization with coil occlusion has
become the primary mode of closure.
• Newer occlusion devices similar to ones
used for ASD closure are being
developed for closure of the large PDA in
older children and adults
 Eisenmenger’s Syndrome
• A long standing L→R shunt will
eventually cause irreversible pulmonary
vascular disease
• This occurs sooner in unrepaired VSDs
and PDAs (vs an ASD) because of the high
pressure gradient
• Once the PVR gets very high the shunt
reverses (ie- now R→L) and the patient
becomes cyanotic.
 Questions
• ?????
T0TAL ANOMALOUS PULMONARY VENOUS
RETURN
Definition:
• There are typically four pulmonary veins, two
from each lung which normally connect
directly into the left atrium.
• “Anomalous” means abnormal.
• In a child born with TAPVR, the pulmonary
veins connect to other veins, and ultimately
drain their blood into the right atrium.
• Also known as total anomalous pulmonary
venous connection (TAPVC).
 CLASSIFICATION
• Supracardiac TAPVR: The pulmonary
veins drain into the right atrium
through the superior vena cava.
• Infracardiac TAPVR: The pulmonary
veins drain into the right atrium
through the hepatic veins and the
inferior vena cava.
 Cont..
• Cardiac TAPVR: There are two types: The
pulmonary veins can directly drain into
the right atrium or into the coronary
sinus, a vein which usually only carries
blood coming out of the heart muscle.
• Mixed TAPVR: The pulmonary veins split
up and drain partially to more than one
of these options
 Symptoms
• variable cyanosis of the lips, skin and
nails
• Rapid breathing
• Heart murmur
• cardiac failure
• Pulmonary congestion with frequent
chest infections
 Diagnosis and management
• Diagnosis by:
- CXR
- cardiac catheterization
- Echocardiography
- ECG
• Management:
• Operative repair in all cases
 Questions
• ????