Download Adult Type Anomalous Left Coronary Artery from Pulmonary Artery

Case Report
Adult Type Anomalous Left Coronary Artery from
Pulmonary Artery
GP Parale*, SS Pawar**
Abstract
Anomalous origin of Left Coronary Artery from Pulmonary Artery (ALCAPA) is a rare congenital anomaly.
Its survival into adulthood is further rare. Clinical manifestations result from evolving morphological
- functional alterations in pulmonary circulation that occur after the birth. We report a case of 43 year old
adult patient with effort angina and without any ECG or Echo abnormalities. On coronary angiography,
typical anatomy of ALCAPA was revealed. ©
At present our patient is awaiting surgery.
INTRODUCTION
A
nomalous origin of left coronary artery from
pulmonary artery (ALCAPA) is a rare congenital
defect with reported incidence of 1 in 3 lac live births
accounting for 0.25% of congenital heart diseases1 and
survival to adulthood is furthermore rare accounting
for only 10-15% of these cases.2 Here, we report a
case of adult survivor of ALCAPA diagnosed at our
institution.
CASE REPORT
A 43 year old male hypertensive since two years
and known chronic smoker was evaluated for history
of class II effort angina over previous two months.
Physical examination was within normal limits except
for blood pressure, which was 156/108 mm of Hg in
right upper arm in supine position. X-ray chest was not
remarkable. The ECG showed ST/T changes in leads I,
avL and V6 suggesting lateral ischaemia. 2-dimensional
echocardiography revealed trivial mitral regurgitation
(MR) and concentric left ventricular hypertrophy
(LVH) with preserved left ventricular systolic and
diastolic function. There was no regional wall motion
abnormality. [Echocardiography was done before as
well as after the angiography with prior knowledge
of coronary artery anatomy. But it did not given any
clue of ALCAPA, mainly because echo window did
not permit visualisation of left coronary artery]. With
this background, patient was taken for coronary
angiography which was done in multiple views. This
revealed typical anatomy of ALCAPA.
*Chief Cardiologist; **Research Assistant, Ashwini Co-operative
Hospital and Research Centre.
Received : 17.12.2004; Revised : 3.1.2006; Re-revised : 4.2.2006;
Accepted : 27.3.2006
© JAPI • VOL. 54 • MAY 2006
DISCUSSION
ALCAPA or Blannd-Garland-White syndrome is a
rare congenital anomaly with incidence of 1 in 3 lac
live births, accounting for 0.25% of congenital heart
disease. The clinical expression of syndrome results
from evolving morphological-functional alterations in
pulmonary circulation that occur after birth.1 Soon after
birth, resistance of the pulmonary circulation is so high
permitting antegrade flow from the pulmonary artery
(PA) to left coronary artery (LCA), which perfuses the
left ventricle. Therefore, occurrence of sudden death is
extremely rare in this age group. As pulmonary vascular
Fig. 1a : Right coronary angiography in RAO position showing filling of
left coronary system through collaterals.
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397
Fig. 1b : Right coronary angiography in delayed phase showing filling
of pulmonary artery from left coronary artery confirming diagnosis
ALCAPA.
resistance falls in following weeks flow from PA to LCA
stops and left ventricular perfusion totally depends
upon collaterals to LCA developed from right coronary
artery (RCA). Death ensues if collaterals are poorly
developed while on the other hand if collaterals enlarge
after an initial period of decompensation, improvement
and survival into adulthood occurs - so called adult
type of ALCAPA.
In patients who survive into adulthood, pulmonary
circulation acts as low resistance siphon and collaterals
flow uses LCA as only conduit into pulmonary circulation
thus bypassing the left ventricular myocardium. This
coronary steal may cause overt ischaemia as well as
left ventricular diastolic overload from left to right
shunting.
Our patient was nearly asymtomatic till date and
living normal life, including normal professional
life. The presenting symptoms of retrosternal pain
and fatigue on strenuous exercise probably results
from myocardial ischemia due to failure in collateral
circulation associated with concentric left ventricular
hypertrophy.
On electrocardiography signs of infarction are absent
in adults,3 which is consistent with our finding as
electrocardiograph showed only ST/T wave changes
in leads I, avL and V6.
Two dimensional (2-D) echocardiography is
considered major support examination for diagnosing
ALCAPA. This examination however has its limitations.4
Diagnosis can sometimes be achieved on the basis of
echocardiography alone if it reveals a grossly enlarged
RCA and a dilated left ventricle with global hypokinesia.
Pulsed and color-flow Doppler can demonstrate reversal
398
of flow from the ALCAPA into the PA, which constitutes
a left-to-right shunt. In older patients, abundant highvelocity flow may be seen in the interventricular septum,
as a result of intercoronary collaterals. Two-dimensional
echocardigraphy can equally visualize the actual
anatomical origin of the ALCAPA and assess the degree
of mitral insufficiency.5 In the present case, 2-D echo
examination revealed trivial mitral regurgitation with
left ventricular hypertrophy with preserved systolic
and diastolic function and there was no regional wall
abnormality. Coronary arteries could not be visualised
properly.
Coronary angiography which is considered as the
most accurate diagnostic technique was performed since
echocardiography could not reveal the abnormality.
Three angiographic criteria are established for diagnosing
ALCAPA.4
They are as follows:
1. Retrograde filling of LCA.
2. Connection of LCA with PA.
3. Absence of LCA originating from aorta.
Since all the three criteria are fulfilled, diagnosis of
ALCAPA is confirmed.
The adult form of ALCAPA may occur in children
and necessarily occurs in adults in approximately
10-15% of cases. It is characterised by exuberance in
collateral coronary circulation, which allows survival
until adulthood, with case being reported at the age of
72 years.6
Wesselhoeft et al.2 classified the clinical spectrum of
ALCAPA as follows:
1. Infantile Syndrome : This is the most common form.
Patient develops acute episode of respiratory
insufficiency, cyanosis, irritability and profuse
sweating. Most of them die within two years.
2. Mitral Regurgitation : It is characterised by mitral
regurgitation murmur, congestive heart failure,
cardiomegaly and atrial arrythmias in children,
adolescent and adults.
3. Syndrome of Continuous Murmur : This occurs in
asymptomatic patients with angina pectoris. A
continuous murmur results from great volume of
blood flowing through collateral branches between
right and left coronary arteries.
4. Sudden Death in Adolescents or Adults : Most of
the patients are asymptomatic, but some may
experience angina on exertion, cardiac arrhythmias
and sudden death.
Once the diagnosis of ALCAPA is established, early
surgical repair for correction of defect and prevention of
complications and sequelae inherent in natural history
of disease is mandatory.
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© JAPI • VOL. 54 • MAY 2006
TA, eds. Heart diseases in Infants, Children and Adolescents,
4th ed. Baltimore : Williams and Wilkins, 1989;627-35.
REFERENCES
1.
Celso K Takimura, Allyson Nakamoto, Viviane Hotta.
Anomalous origin of left coronary artery from pulmonary
artery. Report of an adult case. Arq Bras Cardiol 2002;78:
312-14.
2.
Wesselhoft H, Fawcet JS, Johnson AL. Anomalous origin of left
coronary artery from pulmonary trunk: its clinical spectrum, its
pathology and pathophysiology based on a review of 140 cases
with seven further cases. Circulation 1968;38:403-25.
3.
Takashi M, Lurie PR. Abnormalities and diseases of coronary
vessels. In: Adams FH, Emmanouilides GC, Reimenschneider
4.
Culham JAG. Abnormalities of coronary arteries. In: Freedom
RM, Mawson JB, Yoo SJ, Benson LN, eds. Congenital Heart
Disease. Textbook of Angiocardiography. Armonk, NY : Futura
Publishing Company Inc., 1997;862-66.
5.
Jureidini SB, Nouri S, Pennington DG. Anomalous origin of
the left coronary artery from the pulmonary trunk: repair
after diagnostic cross-sectional echocardiography. Br Heart J
1987;58:173-75.
6.
Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Management of
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