ALU 301, Ch. 13 (CONGENITAL HEART DISEASE) Complete
QUESTIONS WITH 100% CORRECT ANSWERS
With diagnostic and surgical advances
at least 90% of infants with serious congenital heart problems are expected to reach adult years, and
this growing adult population can actively seek insurance coverage
at least 90% of infants with serious congenital heart problems are expected to reach adult years, and
this growing adult population can actively seek insurance coverage
The cause is usually uncertain. Many defects likely have a strong genetic basis
There are many varieties of structural defects and combinations. The following eight conditions
account for 80% of cases of congenital heart disease:
1. three obstructions - pulmonary stenosis (PS), aortic stenosis (AS), and coarctation of the aorta
(COA)
2. three left-to-right shunts - atrial septal defect (ASD), ventricular septal defect (VSD), and patent
ductus arteriosus (PDA) 3. two complex cyanotic conditions - tetralogy of Fallot (TF) and transposition
of the great arteries (TGA).
Occasionally, congenital heart defects remain undetected until adult years.
Examples include atrial septal defect (ASD) and mild aortic valve disorders.
open heart surgery to repair COA was replaced with
balloon angioplasty in the mid1990s
A common underwriting scenario is a young adult with a history of congenital heart surgery before
the age of five years.
The current doctor has no information regarding the type of lesion or where operated. The
underwriting approach with no information in a young adult with congenital heart disease can vary
depending on the size of the case. Scar location obtained by interview or paramedical examination
can direct the medical director to the associated surgery performed. Minimal evidence would include
a current EKG, chest X-ray, and report of any murmurs by the family doctor or routine examiner. A
current echocardiogram by a reliable laboratory can provide valuable information and should be
considered for larger cases.
An atrial septal defect (ASD) is
a hole in the septum between the right and left atria. Significance of the defect depends on its
location, size (which can vary greatly), and the presence or absence of other congenital heart lesions.
These common defects can occur in different areas of the interatrial septum and include secundum,
primum, and sinus venosus defects.
Secundum ASD is
the most common ASD, occurring in the center of the septum.
Some small-sized lesions with small shunts eventually are closed by surgery or catheter intervention.
Risk of complications is less than 1% with catheter technique.
If not closed, there is a small, increased risk of atrial fibrillation. With or without atrial fibrillation, the
presence of a small right-to-left shunt can increase the risk of stroke. The mortality ratio is likely to be
close to 100%.
, Figure 1. Atrial septal defect anatomy
page 3
Early surgical closure of an ASD, before age 20, is rarely associated with complications.
ASD-related right atrial, right ventricular, and pulmonary artery enlargements usually regress to
normal sizes after surgery. The occasional subject will have significant residual dilatation of the right
ventricle and right atrium. There is a slight risk of cardiac arrhythmias, mainly atrial fibrillation, in
these patients followed up to age 50 years
With closure of a large atrial defect after age 20,
individuals usually do well but have a 25% chance of developing atrial fibrillation over the next 30
years. Persistent right atrial, right ventricular, and pulmonary artery enlargements likely contribute to
the increased frequency of atrial fibrillation in this group.
Most cardiologists expect mortality after closure of ASD in childhood to be close to normal and
only mildly increased in those with successful closures up to age 50.
Primum ASD is
the second most common ASD. This defect is located in the lower part of the septum and can be
associated with a mitral valve defect
The major concern with a primum atrial septal defect is
the degree of residual mitral regurgitation and the likelihood the regurgitation might increase in the
next 30 years. With little or no mitral regurgitation, no pulmonary hypertension, no AV block and no
arrhythmia, mortality is in the 150% range or less in published series
Severe mitral regurgitation cases may eventually need
valve replacement or an attempt at repair. Others can remain stable with significant mitral
regurgitation for a number of years and not need their valve surgery until they are in their late 40s.
Mortality ratios for these cases range from 250 to over 500%
After surgery, some individuals can develop complete AV block and
are pacemaker-dependent for their remaining years - hopefully to age 80 plus. However, some
individuals develop impaired left ventricular (LV) function after long-term right ventricular pacing.
Other individuals with residual mitral regurgitation eventually develop atrial fibrillation or flutter.
Sinus Venosus Atrial Septal Defect
This is the least common ASD, located in the upper portion of the septum.
Sinus Venosus Atrial Septal Defect Repaired
can be dealt with the same as secundum ASD. The one additional concern is that surgery can interrupt
electrical transmission within the right atrium, sick sinus syndrome can develop, and occasionally
pacing may be needed
ventricular septal defect (VSD)
-is a hole in the septum between the right and left ventricles.
QUESTIONS WITH 100% CORRECT ANSWERS
With diagnostic and surgical advances
at least 90% of infants with serious congenital heart problems are expected to reach adult years, and
this growing adult population can actively seek insurance coverage
at least 90% of infants with serious congenital heart problems are expected to reach adult years, and
this growing adult population can actively seek insurance coverage
The cause is usually uncertain. Many defects likely have a strong genetic basis
There are many varieties of structural defects and combinations. The following eight conditions
account for 80% of cases of congenital heart disease:
1. three obstructions - pulmonary stenosis (PS), aortic stenosis (AS), and coarctation of the aorta
(COA)
2. three left-to-right shunts - atrial septal defect (ASD), ventricular septal defect (VSD), and patent
ductus arteriosus (PDA) 3. two complex cyanotic conditions - tetralogy of Fallot (TF) and transposition
of the great arteries (TGA).
Occasionally, congenital heart defects remain undetected until adult years.
Examples include atrial septal defect (ASD) and mild aortic valve disorders.
open heart surgery to repair COA was replaced with
balloon angioplasty in the mid1990s
A common underwriting scenario is a young adult with a history of congenital heart surgery before
the age of five years.
The current doctor has no information regarding the type of lesion or where operated. The
underwriting approach with no information in a young adult with congenital heart disease can vary
depending on the size of the case. Scar location obtained by interview or paramedical examination
can direct the medical director to the associated surgery performed. Minimal evidence would include
a current EKG, chest X-ray, and report of any murmurs by the family doctor or routine examiner. A
current echocardiogram by a reliable laboratory can provide valuable information and should be
considered for larger cases.
An atrial septal defect (ASD) is
a hole in the septum between the right and left atria. Significance of the defect depends on its
location, size (which can vary greatly), and the presence or absence of other congenital heart lesions.
These common defects can occur in different areas of the interatrial septum and include secundum,
primum, and sinus venosus defects.
Secundum ASD is
the most common ASD, occurring in the center of the septum.
Some small-sized lesions with small shunts eventually are closed by surgery or catheter intervention.
Risk of complications is less than 1% with catheter technique.
If not closed, there is a small, increased risk of atrial fibrillation. With or without atrial fibrillation, the
presence of a small right-to-left shunt can increase the risk of stroke. The mortality ratio is likely to be
close to 100%.
, Figure 1. Atrial septal defect anatomy
page 3
Early surgical closure of an ASD, before age 20, is rarely associated with complications.
ASD-related right atrial, right ventricular, and pulmonary artery enlargements usually regress to
normal sizes after surgery. The occasional subject will have significant residual dilatation of the right
ventricle and right atrium. There is a slight risk of cardiac arrhythmias, mainly atrial fibrillation, in
these patients followed up to age 50 years
With closure of a large atrial defect after age 20,
individuals usually do well but have a 25% chance of developing atrial fibrillation over the next 30
years. Persistent right atrial, right ventricular, and pulmonary artery enlargements likely contribute to
the increased frequency of atrial fibrillation in this group.
Most cardiologists expect mortality after closure of ASD in childhood to be close to normal and
only mildly increased in those with successful closures up to age 50.
Primum ASD is
the second most common ASD. This defect is located in the lower part of the septum and can be
associated with a mitral valve defect
The major concern with a primum atrial septal defect is
the degree of residual mitral regurgitation and the likelihood the regurgitation might increase in the
next 30 years. With little or no mitral regurgitation, no pulmonary hypertension, no AV block and no
arrhythmia, mortality is in the 150% range or less in published series
Severe mitral regurgitation cases may eventually need
valve replacement or an attempt at repair. Others can remain stable with significant mitral
regurgitation for a number of years and not need their valve surgery until they are in their late 40s.
Mortality ratios for these cases range from 250 to over 500%
After surgery, some individuals can develop complete AV block and
are pacemaker-dependent for their remaining years - hopefully to age 80 plus. However, some
individuals develop impaired left ventricular (LV) function after long-term right ventricular pacing.
Other individuals with residual mitral regurgitation eventually develop atrial fibrillation or flutter.
Sinus Venosus Atrial Septal Defect
This is the least common ASD, located in the upper portion of the septum.
Sinus Venosus Atrial Septal Defect Repaired
can be dealt with the same as secundum ASD. The one additional concern is that surgery can interrupt
electrical transmission within the right atrium, sick sinus syndrome can develop, and occasionally
pacing may be needed
ventricular septal defect (VSD)
-is a hole in the septum between the right and left ventricles.
