Sağ koroner arterin pulmoner arterden orijin aldığı ve aorto-pulmoner pencerenin eşlik ettiği bir olgu

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Introduction
Anomalous origin of the right coronary artery from
the main pulmonary artery is a rare congenital cardiac
malformation. We report a 13-day-old-patient presenting with anomalous origin of the right coronary artery
from the pulmonary artery (ARCAPA) with aortopulmonary window (APW). Surgical correction was performed and the orifice of the right coronary artery was
derivated into the aorta with autologous pericardial
patch. The patient's postoperative course was uneventful and he has been asyptomatic in 6 months after the
surgery.
Case Report
A 7-day-old male newborn was referred to the division of Pediatric Cardiology of the Baskent University
Hospital for the assessment of a cardiac murmur. The
infant was 3300 gr. The electrocardiogram showed
sinus rhythm and right ventricular hypertrophy.
Transthoracic echocardiography revealed good biventricular function, a 3.5-4 mm wide APW and anomalous origin of the right coronary artery from the pulmonary trunk. Cardiac catheterization was performed
prior to surgical intervention and demonstrated mildly
increased pulmonary pressures. Aortography revealed
anomalous origin of the dominant right coronary artery
from the pulmonary trunk and a large APW (Figure
1,2).
Surgical correction was performed with the cardiopulmonary bypass support, moderate hypothermia
and cold antegrade crystalloid cardioplegic arrest. After
longitudinal pulmonary arteriotomy, a 4-mm-wide
APW and ARCAPA were identified. The anomalous
*Department of Cardiovascular Surgery, Baskent University
Hospital, Ankara
Reprint request: Dr. Bülent Sarýtaþ, Department of Cardiovascular Surgery, Baskent University Hospital, Ankara
E-mail: bsaritas@hotmail.com
Date submitted: January 22, 2007
Accepted: June 07, 2007
OLGU SUNUMU/CASE REPORT Gülhane Týp Dergisi 2008; 50: 125-127
© Gülhane Askeri Týp Akademisi 2008
Summary
Aortopulmonary window with anomalous origin of the right
coronary artery from the pulmonary artery is a rare but
important lesion. We report a 13-day-old patient presenting
with anomalous origin of the right coronary artery from the
pulmonary artery with aortopulmonary window. We connected the aorta with the anomalous ostium, using closure
of aortopulmonary window with the help of autologous pericardial patch.
Key words: Anomalous origin of the right coronary artery
from the pulmonary artery, aortopulmonary window, pericardial patch
Özet
Sað koroner arterin pulmoner arterden orijin aldýðý ve
aorto-pulmoner pencerenin eþlik ettiði bir olgu
Sað koroner arterin ana pulmoner arterden orijin almasýyla
beraber aortopulmoner pencere bulunmasý nadir görülen,
ancak önemli bir defekttir. Bu tanýyla hastanemize baþvuran 13 günlük bir olgu sunulmuþtur. Hastada perikardiyal
yamadan faydalanýlarak aortopulmoner pencerenin kapatýlmasý ile aorta ile anormal açýklýðýn devamlýlýðý saðlanmýþtýr.
Anahtar kelimeler: Sað koroner arterin pulmoner arterden
kaynaklanan çýkýþ anomalisi, aortopulmoner pencere,
perikardiyal yama126 · Haziran 2008 · Gülhane TD Vuran et al.
coronary ostium situated below the APW was derivated
to the aorta through intrapulmonary baffle of autologous pericard. The pulmonary artery was closed primarily.
Total cardiopulmonary bypass time was 52 minutes.
Weaning from extracorporeal circulation was unproblematic. The postoperative course was uneventful. The
patient was discharged in an asymptomatic condition
and control echocardiographies showed good right and
left ventricular function, no residual shunt and no
stenosis of the ascending aorta and pulmonary artery.
The right coronary artery blood flow was in normal
range in the follow-up period of six months.
Discussion
APW with ARCAPA is a rare but important lesion
(1-4). No genetic associations or environmental risk
factors are known. The 2 competing embriyologic theories are that APW is part of a spectrum of conotruncal
abnormalities, which includes truncus arteriosus at one
end of the spectrum, and that APW is unrelated to truncus arteriosus because the lesions associated with each
defect are so dissimilar (5,6). Most patients remain
asymptomatic because of the presence of well oxygenated blood in the pulmonary trunk owing to the left to
right shunt through the APW (4,7). However, there are
some cases presenting with angina, myocardial infarction, heart failure and sudden cardiac death (2,3,8). Our
case was also asymptomatic and referred for assessment
of a cardiac murmur. Because of the simultaneous filling of both great arteries, preoperative angiographic
diagnosis could be difficult. Injection of the contrast
into both the great arteries is the best way to diagnose
APW and to determine the relationship between pulmonary and coronary arteries and the defect (4,8,9).
Echocardiography is often the only imaging
obtained prior to the repair of many cardiac lesions. In
our case initial diagnosis was also made by transthoracic
echocardiography. This demonstrates that coronary
artery evaluation should be performed at every new
echocardiographic evaluation (7).
In general, a corrective operation is recommended
in this malformation even for asymptomatic patients.
To correct the aortopulmonary defect through a ligature
in association with this coronary anomaly led to intraoperative problems that did not permit the use of this
technique. Different techniques were employed for
connection of the anomalous coronary ostium with the
aorta. One of them is reimplantation of the right coronary artery from the pulmonary artery to the aorta (4).
In our case, we connected the aorta with the anomalous
ostium, using APW with autologous pericardial patch as
reported elsewhere (10,11).
Anomalous origin of the coronary arteries may be
seen as isolated or associated with other cardiac defects,
in particulary with conotruncal abnormalities. When a
conotruncal abnormality is diagnosed, anomalous origin of the coronaries should always be considered. Exact
preoperative diagnosis is very important for surgical
correction, and it must be done early to prevent the development of irreversible pulmonary vascular disease.