Marmara Medical Journal Volume 12 No: 2 April 1999
Case Report
ECHOCARDIOGRAPHIC DIAGNOSIS OF AORTOPULMONARY
WINDOW IN A 4-DAY-OLD BABY
(R eceived 18 February, 1999)
Figen Akalin, M.D.* / Funda Oztunc, M.D.** / Serap Turan, M.D.***
' S u b -d e p a rtm e n t o f P e d ia tric C a rd io lo g y , D e p a rtm e n t o f P e d ia tric s , S c h o o l o f M e d ic in e , M a rm a ra U n iv e rs ity , Is ta n b u l Turkey.
” In s titu te o f C a rd io lo g y , Is ta n b u l U n iv e rs ity , Is ta n b u l, T urke y.
” * D e p a rtm e n t o f P e d ia tric s , S c h o o l o f M e d ic in e , M a rm a ra U n iv e rs ity , Is ta n b u l, T u rk e y .
ABSTRACT
Aortopulmonary window is a rare cardiac abnormality leading to pulm o n ary va sc u lar d is e a s e unless
d iagnosed in ea rly infancy. E ch o card io grap h ic
diagnosis m ay be difficult if it is not searched for. Aortopulmonary window must be considered in the differential diagnosis of patent ductus arteriosus and truncus arteriosus because of their similar clinical findings and natural course. Early d iagnosis is essential for prevention of complications, especially the Eisenm enger syndrome. W e herein present an aortopulmonary window type I case diagnosed by echocardiography and treated surgically in a-four day- old baby.
Key
Words
: A orto p ulm o nary w indow , Echocardiography. Congenital heart diseaseINTRODUCTION
Aortopulm onary w indow (A P W ) is a rare cardiac abnormality occurring in about 0.2 % of the patients with congenital heart disease (1,2). A P W w as first described by Elliotson in 1830 in an autopsy study (3). The septum betw een the main pulmonary artery and ascending aorta is incom plete in this lesion (1,2). Several types of A P W have been described. Typ e I defect is located at midway betw een the semilunar valves and the bifurcation of pulmonary artery. Type II defect is more distally located and the right pulmonary artery usually originates from aorta in this type. Type III defects are large lesions combining the first two types (1,2). Early diagnosis is essential for prevention of pulmonary vascular disease. Clinical findings and natural course of the patients with this abnormality may be similar to truncus arteriosus or large patent
ductus arteriosus and A P W must be considered in differential diagnosis of these pathologies (1-3). Com plete diagnosis of A P W with echocardiography without angiography is reported in recent years (4). W e herein p res en t a c a s e of A P W d ia g n o sed by echocardiography in a-four-day old baby and review the literature about this rare pathology.
CASE REPORT
A 4-day-old girl has been referred to our hospital
b e c a u s e of a m u rm ur noticed during routine
e x am in atio n . S h e w as th e first child of non- co n sa n g u in eo u s h ealth y p are n ts . S h e w as born following an uneventful pregnancy.
On physical exam ination she w as an acyanotic, active baby. S h e w as mildly tachypneic and a continuos murm ur w as audible at the third intercostal region on the left side of the sternum . S econd heart sound was normal. T h e rest of the physical exam ination was within normal limits.
Chest x-ray showed mild en largem en t of the cardiac shadow with increased pulm onary vascular markings and the electrocardiogram show ed right axis deviation and right ventricular hypertrophy which w as normal according to the ag e of the baby.
Echocardiographic exam ination w as perform ed by using an A TL Ultram ark 9 echocardiography m achine and a 5 M H z probe. T he left atrium and the left ventricle w as found to be enlarged com paring with the right side of the heart. Ventricular contractions w ere normal, mitral valve w as mildly regurgitant. P atency of foram en ovale w as detected. Precordial and subcostal short axis views dem onstrated an aortopulm onary window 5
Marmara Medical Journal Volume 12 No: 2 April 1999
F i g . 1
Parasternal short
axis view
demonstrating the
defect betw een
the ascending
ao rta and main
pulmonary artery. F i g . 2 . : S ubcostal view demonstrating the aortopulm onary window.
mm in diam eter, which w as about 1.5 cm above the pulm onary valve (Figs. 1 and 2). The shunt through the defect w as bi-directional. Pulm onary artery was not enlarged.
T he patient was operated under cardiopulmonary by pass surgery and the defect betw een the ascending ao rta an d m ain p ulm onary artery w as closed successfully. T he baby is now doing well following an uneventful postoperative period.
DISCUSSION
Aortopulmonary window is a rare cardiac abnormality and it occurs in about 0 .1 -0 .2 % of the patients with congenital heart disease. A P W is reported to be present in 0 .4 % of the patients undergoing surgery for congenital heart disease (1,4). The literature includes small series and case reports of A P W and about 350 patients have been reported up to date(1).
Marmara Medical Journal Volume 12 No: 2 April 1999
T h e septum b etw ee n th e ascending ao rta and pulmonary artery is defective in this abnormality. Our patient had a proximal defect located betw een the semilunar valves and bifurcation of the pulmonary artery and it w as decided to be a type I defect (1,2). M ost of th e p atients with A P W has asso ciated abnormalities (5 0 -8 0 % of the patients). Patency of ductus arteriosus (72 % ), interrupted aortic arch type A or coarctation of aorta (1 3 -2 0 % ), interrupted aortic arch type B (8% ), tetralogy of Fallot (6% ), right aortic arch (5-2 0% ), agenesis of ductus arteriosus (20% ), ventricular septal defect (20 % ), right pulmonary artery originating from aorta (32% ), anom alous coronary
artery originating from p ulm oray artery (2 3 % ),
transposition of great arteries (10 % ) are the common associated abnormalities (3,4). Isolated defects are less common and our patient had an isolated A P W . Although association of secundum type atrial septal defects are reported as case reports; the atrial septal defect found in our patient w as a small one and it may be considered as a patent foram en ovale in neonatal period. T he mild mitral regurgitation detected by color flow Doppler w as thought to be caused by the left ventricular overload due to AP W .
Clinical findings such as d ysp n ea , tac h y ca rd ia , tachypnea, diaphoresis, congestive heart failure and failure to thrive m ay be preset in patients with A P W at presentation (1,2). O ur patient only had a precordial murmur and mild tachypnea during initial exam ination probably b e c a u s e of the early diagnosis and continuing physiologic pulmonary hypertension.
Clinical course and prognosis of A P W is similar to other cardiac abnormalities causing large left to right shunt, and E ise n m en g e r syndrom e m ay develop unless it is treated early. Pinto et al (5) have reported that about one third of the patients with A P W betw een 2 to 38 years of age had Eisenm enger syndrome.
Two dim ensional echocardiography is usually the method used for diagnosis of A P W and the defect may be visualized by using parasternal and subcostal views. Presence of “T ” artifact at the margins of the defect is helpful for differentiation from “drop out" that may be seen at the sam e region. Echocardiographic diagnosis may be difficult in some patients especially in the presence of associated abnormalities (6,7). Color flow Doppler is beneficial in these patients. O ccasio nally cath eterisatio n and an g io g rap h y is necessary before surgery. How ever; in the series of Bertolini et al (3) 8 of 10 cases had undergone cardiac
catheterisation. M agnetic resonance imaging is also useful for d em o n stra tio n of d e ta ile d an ato m ical fe a tu res (8). In our p atien t tw o d im en sion al echocardiography w as sufficient for diagnosis and color Doppler exam ination showed the turbulent flow in the pulm onary artery.
Surgery is accepted to be the choice of treatm ent b e c a u s e of the potential com plications of AP W . Mortality related with surgery is low and long term prognosis after surgery is excellent (9).
In conclusion; A P W is a rare cardiac abnormality that may lead life threatening complications. It must be considered in infants with signs and symptom s of left to right shunt and an echocardiographic exam ination must be perform ed.
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