Acynotic Congenital
Heart Diseases.
FFU
May 2020
Dr Hussein 04/12/2021 1
ATRIAL SEPTAL DEFECT
• Atrial septal defects (ASDs) can occur in any portion of the atrial septum
depending on which embryonic septal structure has failed to develop
normally.
• During the embryologic development of the heart, a septum grows toward
the endocardial cushions to divide the atria.
• Failure of septal growth or excessive reabsorption of tissue leads to ASDs.
• ASDs represent approximately 10% of all congenital heart defects.
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Types of ASD.
• An ostium secundum: defect in the region of the fossa ovalis is the most common
form of ASD and is associated with structurally normal atrioventricular (AV) valves.
• Mitral valve prolapse has been described in association with this defect but is rarely an
important clinical consideration.
• Secundum ASDs may be single or multiple (fenestrated atrial septum), and openings ≥2 cm
in diameter are common in symptomatic older children.
• Females outnumber males 3 : 1 in incidence.
• Partial anomalous pulmonary venous return, most commonly of the right upper pulmonary
vein, may be an associated lesion.
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PATHOPHYSIOLOGY.
• Degree of shunt depends on defect size.
• the relative compliance of the right and left ventricles, and the relative
vascular resistance in the pulmonary and systemic circulations.
• In large defects, a considerable shunt of oxygenated blood flows from the
left to the right atrium.
• This blood is added to the usual venous return to the right atrium and is
pumped by the right ventricle to the lungs.
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Continue.
• With large defects,the ratio of pulmonary to systemic blood flow (Qp : Qs) is usually
between 2 : 1 and 4 : 1.
• The paucity of symptoms in infants with ASDs is related to the structure of the right
ventricle in early life when its muscular wall is thick and less compliant, thus limiting
the left-to-right shunt.
• As the infant becomes older and pulmonary vascular resistance drops, the right
ventricular wall becomes thinner and the left-to-right shunt across the ASD increases.
• The increased blood flow through the right side of the heart results in enlargement of
the right atrium and ventricle and dilatation of the pulmonary artery.
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continue.
• The left atrium may also be enlarged, but the left ventricle and aorta are
normal in size.
• Despite the large pulmonary blood flow, pulmonary arterial pressure is
usually normal because of the absence of a high-pressure communication
between the pulmonary and systemic circulations.
• Pulmonary vascular resistance remains low throughout childhood,
although it may begin to increase in adulthood and may eventually result
in reversal of the shunt and clinical cyanosis.
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Ostium Primum .
• An ostium primum defect is situated in the lower portion of the atrial
septum and overlies the mitral and tricuspid valves.
• It located near the endocardial cushions, may be part of a complete
atrioventricular canal defect or may be present with an intact ventricular
septum.
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A sinus venosus ASD.
• A sinus venosus ASD is situated in the upper part of the atrial septum in
close relation to the entry of the superior vena cava.
• Often, one or more pulmonary veins (usually from the right lung) drain
anomalously into the superior vena cava.
• The superior vena cava sometimes straddles the defect; in this case, some
systemic venous blood enters the left atrium, but only rarely does it cause
clinically evident cyanosis.
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CLINICAL MANIFESTATIONS.
• A child with an ASD is most often asymptomatic; the lesion is often
discovered inadvertently during physical examination.
• Even an extremely large ASD rarely produces clinically evident heart
failure in childhood.
• However, on closer evaluation, in younger children, subtle failure to
thrive may be present; in older children, varying degrees of exercise
intolerance may be noted.
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CLINICAL MANIFESTATIONS.
• The physical findings of an ASD are usually characteristic but fairly subtle and
require careful examination of the heart, with special attention to the heart
sounds.
• Examination of the chest may reveal a mild left precordial bulge.
• A right ventricular systolic lift may be palpable at the left sternal border.
• Sometimes a pulmonic ejection click can be heard.
• In most patients with an ASD, the characteristic finding is that the 2nd heart
sound is widely split and fixed in its splitting during all phases of respiration.
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CLINICAL MANIFESTATIONS.
• Normally, the duration of right ventricular ejection varies with respiration,
with inspiration increasing right ventricular volume and delaying closure
of the pulmonary valve.
• With an ASD, right ventricular diastolic volume is constantly increased
and the ejection time is prolonged throughout all phases of respiration.
• A larger shunt may result in a mid-diastolic murmur at the LLSB as a
result of the increased volume passing across the tricuspid valve.
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DIAGNOSIS.
• The chest X-ray shows varying degrees of enlargement of the right ventricle and
atrium, depending on the size of the shunt.
• The pulmonary artery is enlarged, and pulmonary vascularity is increased.
• These signs vary and may not be conspicuous in mild cases.
• Cardiac enlargement is often best appreciated on the lateral view because the right
ventricle protrudes anteriorly as its volume increases.
• The electrocardiogram shows volume overload of the right ventricle.
• And Echo. Is best diagnostic tool for ASD visualization.
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Treatment.
• Medical management is rarely indicated.
• If a significant shunt is still present at around 3 years of age, closure is
usually recommended.
• Many secundum ASDs can be closed with an ASD closure device in the
catheterization laboratory.
• Primum and sinus venosus defects require surgical closure.
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Thanks for your attention.
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