Pulmoner arterden köken alan sol koroner arter anomalisinin (ALCAPA) geç ortaya çıkışı: klinisyenler nasıl tanı koyabilir?

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Introduction
Congenital coronary anomalies are seen in less than
1-2% of infants and approximately 1% of adults undergoing coronary angiography. Anomalous origin of
the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is an extremely rare anomaly and survival to adulthood is exceptional (1-3). We herein report a case of a young adult with ALCAPA syndrome.
Case Report
A 21-year-old male was admitted with the complaints of fatigue and exertional dyspnea to our hospital.
His previous cardiac history was unrevealing. Physical
examination revealed a grade 2/6 apical systolic murmur. ECG depicted nonspecific T wave changes in anterior derivations. Cardiothoracic index was found to
be slightly increased in chest X-ray. Transthoracic echocardiography (TTE) revealed mild mitral regurgitation with an ejection fraction of 60%. Doppler investigation showed turbulent diastolic flow within the
interventricular septum and inferior wall (Figure 1).
In addition, 2D and Doppler investigations demonstrated that the left coronary artery (LCA) was originating from the main pulmonary artery (Figure 2).
Cardiac catheterization was performed. The LCA
was not visualized at the left sinus valsalva. The right
coronary artery (RCA) was dilated and tortuous. LCA,
which was filling retrogradely via collateral vessels
from the RCA appeared during right coronary injection. It was originating from the main pulmonary artery, which also visualized during the RCA angiography (Figure 3). Surgical correction of the coronary
anatomy was planned.
Discussion
Anomalous origin of the LCA from the pulmonary
artery is a rare anomaly, accounting for 0.24% of all
congenital heart anomalies. This anomaly, which is
also called “Bland-White-Garland Syndrome” is seen
*Department of Cardiology, Gulhane Military Medical Faculty
Reprint request: Dr. Cem Barçın, Department of Cardiology, Gulhane
Military Medical Faculty, Etlik-06018, Ankara
E-mail: cembarcin@hotmail.com
Date submitted: February 10, 2010 • Date accepted: August 10, 2010
SUMMARY
Anomalous origin of the left coronary artery from the pulmonary artery
(ALCAPA) is a rare disease characterized by myocardial ischemia which
becomes symptomatic shortly after birth leading to left heart failure and
death. Survival to adulthood have been reported in some cases. Recognition and diagnosis of ALCAPA syndrome is important due to its potentially
life-threatening complications. This case report emphasizes the importance
of diagnosing late presentation of the ALCAPA syndrome.
Key words: Coronary artery anomaly, late presentation, pulmonary artery
ÖZET
Pulmoner arterden köken alan sol koroner arter anomalisinin (ALCAPA)
geç ortaya çıkışı: klinisyenler nasıl tanı koyabilir?
Pulmoner arterden köken alan sol koroner arter anomalisi (ALCAPA), do-
ğumdan kısa süre sonra semptomatik olan, sol kalp yetmezliği ve ölüme yol
açan miyokardiyal iskemi ile karakterize nadir görülen bir hastalıktır. Erişkinliğe kadar sağkalım birkaç vakada bildirilmiştir. ALCAPA sendromunun fark
edilmesi ve tanı konulması, olası hayatı tehdit eden komplikasyonları nedeniyle önemlidir. Bu olgu sunumu, geç ortaya çıkan ALCAPA sendromunun
tanısının önemini vurgulamaktadır.
Anahtar kelimeler: Koroner arter anomalisi, geç ortaya çıkış, pulmoner
arterVolume 52 • Issue 4 ALCAPA • 281
in approximately 18% of the children with congestive heart failure under 2 years old (2,4). The left anterior descending artery, left circumflex coronary artery and RCA may have individual origins from the
pulmonary artery, with similar pathophysiologic and
clinical picture. Individual origins of the coronary arteries have been thought to be benign (2). On the
other hand, when the LCA arises from the pulmonary artery, this anomaly can cause myocardial ischemia that may be progressive and lead to left ventricular dysfunction, mitral insufficiency, congestive heart failure and sudden cardiac death. Asymptomatic
presentation after infancy and survival into adulthood is extremely rare (3,4). The clinical presentation
is related with the coronary artery involved and its
myocardial distribution, the pulmonary vascular resistance, and the number and size of collateral vessels. Collateral circulation between RCA and LCA is
the major determiner for the extent of the myocardial ischemia. When the collateral circulation is sufficient, symptoms may be absent or unremarkable and
these patients are classified as adult type. Without
significant collateral circulation, severe myocardial
ischemia deteriorates myocardial function with the
associated signs and symptoms of heart failure. These
patients are classified as the infantile type (2-5).
In adults with ALCAPA RCA is dilated and tortuous
because of the collateral vessels that this vessel gives
to LCA, as in our case (Figure 1).
Myocardium can tolerate low oxygen concentration as in cyanotic heart disease but it is vulnerable
in low perfusion pressure. After the birth, as the pulmonary artery pressure falls, left ventricular function begins to disturb because of both low perfusion
and low oxygen pressure in patients with ALCAPA.
Fortunately, this fall in pulmonary artery pressure is usually gradual as left ventricular ischemia and
dysfunction tend to increase the left ventricular enddiastolic and left atrial pressure which in turn retard the fall in pulmonary artery pressure. This process may slow the deterioration of the left ventricular function. But, pulmonary artery pressure and left
Figure 1. Doppler imaging showing turbulent diastolic flow within the interventricular septum (A) and inferior (B) wall
Figure 2. Two-dimensional (A) and Doppler (B) investigation showing that the left coronary artery (LCA) was originating from the main
pulmonary artery (PA)282 • December 2010 • Gulhane Med J Barçın et al.
ventricular perfusion pressure decrease to critical levels between the 6th and 8th weeks of life and patients may be symptomatic in this period. On the other hand, in some patients with ALCAPA, a rich network of collateral vessels from the RCA may improve and increase the perfusion and O2
pressure in the
LCA. In this group, LCA drains into relatively lowpressured pulmonary artery. Although this may cause a “pulmonary steal phenomenon”, these patients
survive to adulthood and constitute the adult type of
ALCAPA syndrome.
In patients with ALCAPA, ischemic injury usually
causes left ventricular dilatation and mitral regurgitation due to papillary muscle dysfunction. In the present case, left ventricular function was in normal limits with only a mild dilatation, and only a mild mitral regurgitation was observed. The main echocardiographic finding related to ALCAPA was diastolic turbulent flow in the left ventricle due to collateral flow
from the giant RCA.
The prognosis in patients with adult type ALCAPA is
poor. Sudden cardiac death is common and mortality
until the age of 35 may be as high as 90% (2). Therefore
surgical correction of the anomaly is recommended in
all patients as we planned in the present case.
In conclusion, although extremely rare, late presentation of the ALCAPA syndrome is possible and may
be suspected from the echocardiographic findings.
Regardless of the presence of the symptoms, surgery
is recommended as soon as possible due to the risk of
sudden cardiac death