Case 2

Rationale for Septal Defect Closure

in the Elderly
Ju-Le Tan, Wei Li, and Michael J. Mullen

Age: 88 years
Gender: Female
Personal information: Grandmother
Working diagnosis: Secundum atrial septal defect

HISTORY She does not experience exertional chest pain or other cardiac
symptoms.
The patient was healthy throughout her childhood and adult
years. She had two uneventful pregnancies, and has two healthy NYHA class: III
sons and numerous grandchildren.
Mild hypertension was diagnosed 15 years ago, and the CURRENT MEDICATIONS
patient was treated with medication. Around that same time
she developed atrial fibrillation, which was treated with digoxin Digoxin 125 µg daily
and aspirin and has been permanent for the last decade.
Bendrofluazide 2.5 mg daily (a diuretic used for blood pressure
After the death of her husband several years ago, she managed
control)
to live independently. However, over the last few years she
noted decreasing effort tolerance and frequent chest infections. Perindopril 2 mg daily (for blood pressure control)
She found it difficult to manage housework, shopping, and
gardening while living on her own, and therefore moved to a Aspirin 75 mg daily (presumably for her permanent atrial
larger city to be nearer her children. On arrival she established fibrillation)
medical care with a new general practitioner.
She does not smoke or drink alcohol. There was no family C o m m e n t s : Given her atrial fibrillation, hypertension,
history of congenital or ischemic heart disease. and age, the role of anticoagulation should be discussed with
the patient. She is at risk of stroke (even after ASD repair) and
should be advised to take warfarin unless there is a contraindi­
C o m m e n t s : Although she is now 88 years old the patient cation.2
has until recently led an active and independent life. Obviously,
elderly patients with exertional breathlessness will have is­­
chemic heart disease much more commonly than an ASD, PHYSICAL EXAMINATION
but it is not uncommon for a secundum ASD to present for the
first time in an elderly patient. BP 155/86 mm Hg, HR 70 bpm, oxygen saturation 92% on room
Although the particular details are unknown, it is surprising air, near sea level
that atrial fibrillation did not prompt a more thorough workup Height 158 cm, weight 53 kg, BSA 1.53 m2
including echocardiography, which should have demonstrated
her large ASD. Surgical scars: None
Worsening symptoms in this patient with an ASD are prob­ Neck veins: 5 cm above sternal angle, normal waveform
ably due to substantial left-to-right shunting and RV volume
overloading (both tend to increase with age because of an Lungs/chest: Clear
increase in left ventricular end-diastolic pressures), and the Heart: There was an irregular rhythm, with a right parasternal
development of PAH or LV diastolic dysfunction from systolic heave. There was a normal first heart sound and wide splitting
hypertension. of the second sound with a loud pulmonary component (P2).
ASDs can be familial (autosomal dominant); at least There was also a 3/6 ejection systolic murmur in the third left
three genes have been recently identified in families with intercostal space.
ASD.1
Abdomen: The abdominal examination was unremarkable.

CURRENT SYMPTOMS Extremities: Extremities were well perfused without edema.

The patient becomes breathless after walking less than a quarter C o m m e n t s : Hypertension may need better control.
mile (380 m) on flat ground. She cannot climb one flight of stairs Her resting ventricular rate response to atrial fibrillation
without stopping with dyspnea. was well controlled. Oxygen saturation was mildly reduced,

Cases in Adult Congenital Heart Disease  7

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 prompting one to consider why she might have a right-to-left CHEST X-RAY
shunt.
Shunts / Atrial Septal Defects/Abnormal Pulmonary Venous Return

The right parasternal heave is indicative of significant RV

volume and/or pressure overloading.
The loud P2 indicates elevated pulmonary artery (PA) pres­
sures. The ejection systolic murmur is secondary to increased
flow through the RVOT and pulmonary valve. Fixed splitting
of the second heart sound would be expected in a patient with
a secundum ASD but can be difficult to hear in some patients.
There were no clinical signs of right or left heart failure.

LABORATORY DATA
Hemoglobin 13.5 g/dL (11.5–15.0)
Hematocrit/PCV 39% (36–46)
MCV 93 fL (83–99)
Platelet count 137 × 109/L (150–400)
Sodium 141 mmol/L (134–145)
Potassium 4.4 mmol/L (3.5–5.2)
Creatinine 1.0 mg/dL (0.6–1.2)
Blood urea nitrogen 7.3 mmol/L (2.5–6.5)

C o m m e n t s : It is important to know whether the renal

function is normal (systemic hypertension, on diuretics and an Figure 2-2  Posteroanterior projection.
ACE inhibitor), especially if percutaneous intervention is to be
considered.
Findings
Cardiothoracic ratio: 76%
ELECTROCARDIOGRAM
There is gross cardiomegaly, with prominent central pulmo­
I aVR C1 C4 nary arteries and a generally plethoric pulmonary vasculature.
There is RA and presumably RV dilation (which would have
been better seen on the lateral view, yet is not available).
II aVL C2 C5
C o m m e n t s : Most likely, the large cardiac silhouette is
mainly due to RA and RV enlargement. Prominent central pul­
III aVF C3 C6 monary arteries may indicate volume or pressure overload,
although the latter is unlikely given the numerous peripheral
pulmonary vessels (no “pruning”).
II
EXERCISE TESTING
Pretest HR: 52, oxygen saturation 94%
Figure 2-1  Electrocardiogram.
Posttest HR: 66, oxygen saturation 87%

Findings Distance walked: 221 m

Heart rate: 66 bpm
C o m m e n t s : In elderly patients who are not able to reli­
QRS axis: +123° ably perform maximal exercise testing, the 6MWT is a useful
submaximal alternative for functional assessment. It is safe in
QRS duration: 133 msec the older population.3 The mean walk distance in patients older
Atrial fibrillation than 68 years of age is 344 m.
Other information from the test includes the peak heart rate
Right axis deviation and the oxygen saturation after exercise, which are particularly
Right bundle branch block valuable in congenital heart disease. This test can be easily
repeated in the future to monitor for clinical deterioration or
Nonspecific ST segment depression improvement after an intervention.
In this patient, the minimal increase in heart rate is not sur­
C o m m e n t s : RBBB with right axis deviation should imme­ prising given her medically controlled atrial fibrillation. More
diately prompt consideration of right heart disease. Right axis important, there was mildly progressive oxygen desaturation
deviation is commonly associated with a secundum ASD, while after 6 minutes (94% to 87%) suggesting right-to-left shunt
RBBB with a leftward axis would be more typical for a primum during exercise, intrinsic lung disease, or congestive heart
ASD (see Case 15). failure.
The ST segments in the inferior and anterior chest leads,
especially V3–5, are downward sloping, and due to some com­
bination of digoxin effect, RBBB, and RV hypertrophy.

8  Cases in Adult Congenital Heart Disease

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ECHOCARDIOGRAM

Rationale for Septal Defect Closure in the Elderly

Overall Findings
The RV was severely dilated. The LV was small, with a left
ventricular end-diastolic dimension of 42 mm and an end-sys­
tolic dimension of 25 mm. The LA was dilated. There was mod­
erate tricuspid regurgitation; otherwise, valve function was
normal. The RV size relative to the LV can be seen, as well as
LA enlargement.

Figure 2-5  Apical four-chamber view.

Findings
There was severe dilatation of the RA and RV, with moderate
central tricuspid regurgitation. The LV was relatively small.
Systolic function of both ventricles was normal.
Figure 2-3  Parasternal long-axis view. The secundum ASD was large, at least 30 mm in diameter.

C o m m e n t s : The RV enlargement is secondary to the C o m m e n t s : The dilatation is secondary to volume over­

chronic left-to-right shunt. The LA enlargement was probably loading of the RA and RV from left-to-right shunting. Tricuspid
due to the volume overload, hypertension, and chronic atrial regurgitation is also secondary to tricuspid annular dilatation
fibrillation itself. and often improves with reverse right heart remodeling after
ASD closure.

Figure 2-6  Transesophageal echocardiogram.

Figure 2-4  Parasternal short-axis view.
Findings
Findings The large secundum ASD measured 34 mm in diameter with
Dilated main pulmonary artery. Mild to moderate pulmonary left-to-right shunting.
regurgitation.
C o m m e n t s : TEE can ensure adequate rim tissue neces­
C o m m e n t s : The main pulmonary atery has dilated sary for closure with a transcatheter device.
in response to chronic volume loading, and dilatation of At times, an atrial defect can be so large that there is not
the pulmonary annulus has led to secondary pulmonary enough remaining septal tissue on which to anchor a closure
regurgitation. device. In this patient, although the defect was large, there was
a sufficient rim for an ASD closure device to be properly seated.
Furthermore, there was a large LA; thus the device would not

Cases in Adult Congenital Heart Disease  9

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 likely obstruct flow in the pulmonary veins or mitral valve.
Therefore, there were no concerns about using a large ASD
Shunts / Atrial Septal Defects/Abnormal Pulmonary Venous Return

closure device.

MAGNETIC RESONANCE IMAGING

Figure 2-8  Oblique sagittal short axis-view at atrial level.

Figure 2-7  Oblique transaxial four-chamber view. Findings

Large secundum ASD, measuring 34 mm in diameter.
Findings
The RA and RV were dilated but with normal RV systolic C o m m e n t s : The lower portion of the image shows the
function. The LV was small in comparison. There was mild RA (lower left) and LA (lower right). The IVC can be seen drain­
tricuspid regurgitation. The main pulmonary artery was ing upward to the RA, and flow is directed by a Eustachian
enlarged. By velocity flow mapping, the pulmonary/systemic valve. The ASD is seen above this Eustachian valve.
flow ratio (Qp/Qs) was 2 : 1.

C o m m e n t s : MRI is often not necessary if a large defect CATHETERIZATION

can be seen by echo. The additional information from an MRI
includes volume and mass of the RV, and noninvasive estimates Hemodynamics
of flow, including Qp/Qs ratio, as well as additional informa­ Heart rate 70 bpm
tion on the pulmonary venous drainage, which is particularly
relevant in patients with sinus venosus ASDs. Pressure Saturation (%)
In this four-chamber view, the dilatation of the RV is clearly SVC 75
seen (bottom of the image). Interestingly, the ASD is not visible IVC 80
in this view. Instead, the faint impression of the superior rim is RA mean 11 90
visible, which deviates toward the RA when the RV opens and RV 40/8 89
atrial pressure falls. PA 46/14 mean 23 88
PV mean 14 98
LA mean 9
LV 100/10
Ventricular Volume Quantification Aorta 109/40 mean 64 95
LV (Normal range) RV (Normal range) Calculations
EDV (mL) 83 (52–141) 171 (58–154) Qp (L/min) 7.33
ESV (mL) 40 (13–51) 82 (12–68) Qs (L/min) 3.91
SV (mL) 43 (33–97) 89 (35–98)
Cardiac index (L/min/m2) 2.56
Qp/Qs 1.88
EF (%) 52 (60–78) 52 (59–83)
PVR (Wood units) 1.91
EDVi (mL/m2) 54 (50–84) 112 (45–82) SVR (Wood units) 13.55
ESVi (mL/m2) 26 (12–30) 54 (6–32)
SVi (mL/m2) 28 (34–59) 58 (14–35) C o m m e n t s : Cardiac catheterization was performed with
the patient under general anesthesia to achieve device closure
under TEE guidance. The lower heart rate and blood pressure
reflect the anesthesia. It is important to ask the anesthetist to
use room air and not supplemental oxygen so that the series of
oxygen saturations and the calculations of shunt ratio and pul­
monary vascular resistance are accurate.
The patient’s hemodynamics showed mildly increased pul­
monary artery pressures, reflecting increased pulmonary flow,
but the pulmonary vascular resistance was not elevated. The
Qp/Qs ratio calculated here agrees with that found by MRI
but is surprisingly small considering the size of her heart
on CXR.

10  Cases in Adult Congenital Heart Disease

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 She also had coronary angiograms to exclude silent coronary the patient with a very large ASD and marked right heart
artery disease. These showed mid-LAD stenosis of 50% with no dilatation).

Rationale for Septal Defect Closure in the Elderly

other significant lesions.
2. Will atrial fibrillation or stroke risk improve after closure
Despite some previous findings suggesting possible pulmo­
of the ASD?
nary vascular disease (such as the loud pulmonary component
of the second heart sound and the desaturation with exercise), Late surgical closure of an ASD does not decrease the risk of late
the pulmonary vascular resistance is low and the operator could arrhythmia development.6 In patients with significant atrial
continue confidently with ASD closure. dilatation, and in elderly patients with other risk factors for
arrhythmia (such as hypertension in this case), return to sinus
rhythm after closure would not be expected, unless an arrhyth-
mia-targeting intervention were incorporated around the time of
closure. Nevertheless, the hemodynamic response to arrhythmia
after ASD closure will be better, and the risk of paradoxical
embolus, no matter how small, would be abolished. It should
be emphasized, however, that older patients remain at risk of
stroke (there has been speculation on the potential pathogenic
role of the dilated pulmonary veins) and should be considered
for anticoagulation with warfarin for at least 6 months, while
reverse remodeling is taking place. Patients with persistent
atrial fibrillation after ASD closure should remain on indefinite
anticoagulation.
3. Could the patient’s symptoms be secondary to coronary
artery disease?
Elderly patients being considered for secundum ASD closure
should be tested to exclude coexistent coronary artery disease.
This patient had the risk factor of hypertension, and a 50% lesion
was found in her LAD. Elsewhere there was no significant coro-
Figure 2-9  Right anterior oblique cranial angiogram.
nary artery disease. She denied any angina-like symptoms and
experienced none during her 6MWT.
Findings In general, coronary artery disease should be treated concur-
There are diffuse irregularities in the LAD and its branches, rently with ASD closure, using the same approach used for a
including a 50% mid-LAD lesion. similar patient without the congenital defect, including stent
deployment before or after ASD closure. In this patient we opted
C o m m e n t s : The relevance of these lesions in relation to to first pursue medical management of her coronary disease
the patient’s symptoms needs to be considered. Often in ACHD alone. If her exertional dyspnea did not improve significantly
too much focus is on the congenital heart defect to the exclusion following ASD closure, or she developed angina-like symptoms,
of other potential comorbidities, cardiovascular or otherwise. the LAD lesion could be addressed at a future date. If doubt
remained about the cause of her symptoms, assessment of the
patient’s coronary artery disease with a myocardial perfusion
Focused Clinical Questions scan and/or a stress echo could guide further management.
and Discussion Points
1. Should an ASD in an elderly patient be closed?
FINAL DIAGNOSIS
Secundum ASD
Transcatheter closure of an ASD is a relatively safe procedure;
its advantages are obvious, especially improving exercise toler- Chronic atrial fibrillation
ance and quality of life in a patient who is limited.4 In the elderly
Coronary artery disease
population where comorbidities are common, transcatheter
closure conveys additional benefits; it can be performed without Treated systemic hypertension
the risk of heart surgery and a sometimes prolonged postoperative
course. There are limited published data on ASD closure in the
elderly population (>65 years old) who have experienced chronic PLAN OF ACTION
RV overload and reduction in LV preload for decades. However, Transcatheter closure of the ASD
there is a clear trend toward improved functional capacity
following late repair of ASDs in older patients, irrespective of Medical management of coronary artery disease
age.
A recent publication suggested that in some elderly patients, INTERVENTION
especially those with systemic hypertension, ASD closure may
unmask restrictive LV diastolic dysfunction and lead to pulmo- At the time of catheterization, TEE showed an adequate rim of
nary edema after the procedure.5 In clinical practice, for the tissue for device closure. The defect was measured with a low-
occasional patient felt to be at risk of this complication, a tem- pressure occlusive balloon. After 10 minutes of temporary
porary balloon occlusion (10 minutes) of the ASD may allow the defect closure with the balloon, there was no rise in the LV end-
operator to assess the hemodynamics, with the defect closed. If diastolic pressure and the mitral filling pattern did not change.
the LV end-diastolic pressure increases significantly or the mitral A 36-mm Amplatzer device was selected. The left atrial disc of
inflow pattern on TEE shows a predominantly restrictive filling the Amplatzer device is 16 mm larger than the waist, which
during temporary balloon occlusion, the ASD should perhaps may be the limiting factor in some patients with a large defect
not be closed or a fenestrated closure should be considered (for but small LA. This was not an issue with our patient, as her LA

Cases in Adult Congenital Heart Disease  11

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 was enlarged. The device was deployed uneventfully and the
procedure completed without any complications.
Shunts / Atrial Septal Defects/Abnormal Pulmonary Venous Return

CXR and echo the following day showed good position of

the device without a pericardial effusion. She was started on
clopidogrel 75 mg daily, to continue for 6 weeks following the
procedure. Warfarin was recommended to reduce the thrombo­
embolic risk of her chronic atrial fibrillation, but the patient and
family refused. Thus she continued on aspirin indefinitely. The
patient was advised to practice antibiotic prophylaxis for 6
months after device closure.

OUTCOME
Six months after device closure, the patient reported marked
improvement in her exercise capacity to the extent that she
could walk to her local shop without stopping because of
breathlessness, and climb a flight of stairs easily. She was able
to regain her independence and to look after herself, living on
her own.
Objectively, her oxygen saturation at rest was 97% and her
6MWT had improved from 221����������������������������������
 m������������������������������
to 382 m, with no desatura­
tion. Her echo showed that her ASD device was well positioned
with no residual shunting. Her RV and RA remained moder­
ately dilated, but the estimated pulmonary artery systolic pres­
sure had decreased from 50 mm Hg to 27 mm Hg. She remained
in chronic rate-controlled atrial fibrillation. Figure 2-11  Postprocedure lateral projection.
She developed no chest pain and continued on appropriate
medical therapy for coronary artery disease.
Findings
Atrial enlargement. ASD occluder device in proper position.

C o m m e n t s : The ASD device is visible in the proper

position.

Selected References
1. Gelb BD: Genetic basis of congenital heart disease. Curr Opin
Cardiol 19:110–115, 2004.
2. Gage BF, van Walraven C, Pearce L, et al: Selecting patients with
atrial fibrillation for anticoagulation: Stroke risk stratification in
patients taking aspirin. Circulation 110:2287–2292, 2004.
3. Enright PL, McBurnie MA, Bittner V, et al: Cardiovascular Health
Study: The 6-min walk test: A quick measure of functional status
in elderly adults. Chest 123:325–327, 2003.
4. Webb GD, Gatzoulis MA: Atrial septal defects in the adult: Recent
progress and overview. Circulation 114:1645–1653, 2006.
5. Ewert P, Berger F, Nagdyman N, et al. Masked left ventricular
restriction in elderly patients with atrial septal defects: A contrain-
dication for closure? Cathet Cardiovasc Intervent 52:177–180,
2001.
6. Gatzoulis MA, Freeman MA, Siu SC, et al: Atrial arrhythmia after
surgical closure of atrial septal defects in adults. N Engl J Med
340:839–846, 1999.

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Suarez de Lezo J, Medina A, Romero M, et al: Effectiveness of percu-
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the pre­operative film, although cardiomegaly and dilation of with pulmonary hypertension. Am Heart J 144:877–880, 2002.
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12  Cases in Adult Congenital Heart Disease

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