The occurrence of pulmonary artery obstruction in an 8 day old infant as a complication of an aneurysm of a nonpatent ductus arteriosus is reported, together with the echocardiographic and angiographic findings. To relieve the obstruction, the aneurysm and an intrapulmonary thrombus were successfully removed with the use of cardiopulmonary bypass when the infant was 3 months old

Boal, Danielle K.

Fripp, Raymond R.

Waldhausen, John A.

Whitman, Victor

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Ductus arteriosus aneurysm presenting as pulmonary artery obstruction: Diagnosis and management 

234 JACC Vol. 6, No. I 
July 1985:234-6 
Ductus Arteriosus Aneurysm Presenting as Pulmonary Artery 
Obstruction: Diagnosis and Management 
RAYMOND R, FRIPP, MD, FACC, VICTOR WHITMAN, MD, JOHN A. WALDHAUSEN, MD, FACC, 
DANIELLE K. BOAL, MD 
Hershey, Pennsylvania 
The occurrence of pulmonary artery obstruction in an 
8 day old infant as a complication of an aneurysm of a 
nonpatent ductus arteriosus is reported, together with 
the echocardiographic and angiographic findings. To re-
lieve the obstruction, the aneurysm and an intrapul-
An aneurysm of a non patent ductus arteriosus is an uncom-
mon cardiovascular anomaly. Sequelae that have been re-
ported (1,2) include rupture, embolism, infection and 
thrombosis of the aortic arch. Obstruction of the pulmonary 
artery tree has not been previously described in association 
with this abnormality. This report describes the physical 
findings, diagnosis and management of a 3 month old infant 
with obstruction to the main and left pulmonary arteries 
secondary to an aneurysm of the non patent ductus arteriosus 
and a thrombus extending into the pulmonary artery. 
Case Report 
A male infant weighing 3.3 kg was born at term with I 
and 5 minute Apgar scores of 9 and to, respectively. Vom-
iting associated with feeding occurred from the first day of 
life, but the infant was otherwise well and was discharged 
at 3 days. At age 8 days, he was readmitted to the hospital 
with lethargy, poor feeding and vomiting. Shortly after ad-
mission, "cardiovascular collapse" occurred. The infant 
was resuscitated and transported to the medical center. On 
arrival, peripheral perfusion was good and a grade 3/6 ejec-
tion systolic murmur was audible in the pulmonary area 
with radiation into the left lung field. Marked hepatomegaly 
From the Departments of Pediatrics, Surgery and Radiology, The Penn-
sylvania State University College of Medicine, The Milton S. Hershey 
Medical Center, Hershey, Pennsylvania. Manuscript received November 
6, 1984; revised manuscript received January 14, 1985, accepted February 
I, 1985. 
Address for reprints: Raymond R. Fripp, MD, Department of Pedi-
atrics, The Milton S. Hershey Medical Center, P.O. Box 850, Hershey, 
Pennsylvania 17033. 
© 1985 by the American College of Cardiology 
monary thrombus were successfully removed with the 
use of cardiopulmonary bypass when the infant was 3 
months old. 
(J Am Coli Cardiol 1985;6:234-6) 
was present in addition to hyperbilirubinemia and prolonged 
prothrombin and partial thromboplastin times. Renal failure 
was indicated by the presence of elevated blood urea nitro-
gen and serum creatinine levels and decreased urinary out-
put. An electrocardiogram showed right ventricular hyper-
trophy and right axis deviation. A chest X-ray film revealed 
a normal-sized heart with a round density in the left upper 
mediastinum and decreased pulmonary flow to the left lung. 
Pulmonary flow to the right lung was normal (Fig. I). 
A two-dimensional echocardiogram demonstrated a solid 
mobile mass within the main pulmonary artery (Fig. 2). 
This mass appeared to partially occlude the left pulmonary 
artery but the right pulmonary artery was patent. The aortic 
arch was normal, but the ductus arteriosus could not be 
visualized from the suprasternal position. 
Catheterization findings. After an exchange transfu-
sion, cardiac catheterization was performed. The right ven-
tricular pressure was suprasystemic at 95/4 mm Hg and the 
left ventricular pressure was 75/4 mm Hg. The left pul-
monary artery pressure distal to the mass was 18/8 mm Hg. 
A right ventriculogram (Fig. 3A) showed a nonopacified 
smooth mass within the main pulmonary artery that partially 
ocduded the left pulmonary artery. A left ventriculogram 
(Fig. 3B) demonstrated patency of the aortic end of the 
ductus arteriosus and a large aneurysm of the ductus. No 
left to right shunting into the pulmonary artery was seen. 
An aneurysm of a nonpatent ductus arteriosus with obstruc-
tion to the main and left pulmonary arteries due to throm-
bosis was thought to be the most likely diagnosis, and sur-
gical relief of the obstruction was recommended. Because 
of persistent hepatic and renal failure and, subsequently, 
the development of non-A, non-B hepatitis. surgery was 
0735·1097/85/$3.30 
JACC Vol. 6. No. I 
July 1985:234-6 
Figure I. Anteroposterior chest X-ray film showing the soft tissue 
density in the left upper mediastinum. 
delayed until the patient was 3 months of age. During this 
time, the infant ate and gained weight in a normal manner. 
Surgical procedure and findings. The operation was 
performed through a left lateral thoracotomy and revealed 
an aneurysm of the ductus arteriosus (Fig. 4). The pul-
monary end of the ductus contained a mass that could be 
palpated within the main pulmonary artery. The aortic end 
of the ductus arteriosus was divided and oversewn. Cardio-
Figure 2. Two-dimensional echocardiographic short-axis view at 
the level of the great arteries showing the mass (arrows) within 
the main pulmonary artery (MPA) with exten~ion toward the left 
pulmonary artery. Ao = aorta; LA,- left atrium. 
FRIPP ET AL. 235 
DUCTAL ANEURYSM 
Fi~ure 3. Anglocardiograms. A, Right ventriculogram showing 
a smooth nonopacified mass (arrows) within the main pulmonary 
artery. B, Left ventriculogram showing the aneurysm of the ductus 
arteriosus (arrows) with absence of opacification of the pulmonary 
arteries. 
pulmonary bypass was established by inserting a large can-
nula into the right ventricle through the proximal pulmonary 
artery while the aorta was perfused in the mid descending 
portion. The main pulmonary artery was opened after it was 
occluded just distal to the cannula by a vascular clamp. The 
mass was dissected free of the main and proximal left pul-
236 
A 
FRIPP ET AL. 
DUCTAL ANEURYSM 
Figure 4. A, Illustration of the ductal aneurysm between the aorta 
and main pulmonary artery as observed at operation. B, Illustration 
of the technique used to remove the thrombus within the aneurysm. 
The proximal main pUlmonary artery and descending aorta are 
cannulated for cardiopulmonary bypass. The aortic end of the 
ductus has been divided and oversewn. 
monary arteries and what appeared to be an organized 
thrombus was removed (Fig. 4). After closure of the pul-
monary artery, the patient was weaned from cardiopulmo-
nary bypass. His postoperative course was uneventful and 
he was discharged 7 days later with a normal cardiac ex-
amination. Follow-up study at 5 months of age revealed the 
presence of a left diaphragmatic palsy, but the patient was 
otherwise well. 
Discussion 
Incidence. The incidence of aneurysm of the nonpatent 
ductus arteriosus has been reported to be low. However, 
Heikkinen et a1. (3) retrospectively reviewed 1,138 chest 
X-ray films of newborn infants and made the diagnosis in 
approximately 1 % of the infants based on the presence of 
JACC Vol. 6, No. I 
July 1985:234-6 
a characteristic rounded mass in the left upper mediastinum. 
In two of these infants, the diagnosis was confirmed by 
surgical intervention. 
Diagnosis. Pulmonary artery obstruction as a result of 
an aneurysm of a non patent ductus arteriosus had not been 
described. Despite this, the most likely diagnosis in our 
patient on the basis of the chest roentgenogram, echocar-
diogram and angiocardiogram was an aneurysm of the duc-
tus arteriosus. The differential diagnosis consisted of a pul-
monary embolus, thrombus or a tumor within the pulmonary 
artery. The mobility of the mass as seen on the echocar-
diogram indicated it was unlikely to be an embolus, and the 
ductal aneurysm noted on angiocardiography suggested that 
the mass within the pulmonary artery was an extension of 
a thrombus within the aneurysm rather than a tumor or an 
isolated thrombus. 
Echocardiography revealed the cause of the pUlmonary 
stenosis murmur and of the differential flow to the right and 
left lungs but did not reveal the ductal aneurysm. This may 
have been due to the more anterolateral and cranial position 
of the aneurysm when compared with the normal ductus 
arteriosus. Angiography was required to make an exact an-
atomic diagnosis of the ductal aneurysm. 
Complications. A review of the world literature by Fal-
cone et a1. (I) revealed a complication rate of 43% in 61 
patients with an aneurysm of the ductus arteriosus. Death 
occurred in 31 % of these patients and rupture of the an-
eurysm, the most frequent complication, occurred in 18%. 
Embolism and infection were the other major complications 
described. McFaul et a1. (2) reported on an infant with aortic 
arch thrombosis secondary to the extension of a thrombus 
from an aneurysm of the ductus arteriosus. Hoarseness and 
left phrenic nerve palsy may also result from a ductal 
aneurysm. 
Pulmonary artery obstruction should be considered a 
complication of a ductal aneurysm due to thrombosis with 
the thrombus extending into the pulmonary artery. It can 
probably be diagnosed noninvasively by chest roentgeno-
gram and an echocardiogram. Angiocardiography is indi-
cated, however, if uncertainty remains. Surgical removal 
of the aneurysm and relief of the obstruction can be per-
formed with the use of cardiopulmonary bypass. 
References 
I. Falcone MW, Perloff 1K, Roberts We. Aneurysm of the nonpatent 
ductus arteriosus. Am 1 Cardiol 1971 ;29:422-6. 
2. McFaul RC, Keane 1F, Nowiki ER, et al. Aortic thrombosis in the 
neonate. J Thorae Cardiovasc Surg 1981 ;81 :334-7. 
3. Heikkinen ES, Simila S, Laitinen 1, et al. Infantile aneurysm of the 
ductus arteriosus. Acta Paediatr Scand 1974;63:241-8. 


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Ductus arteriosus aneurysm presenting as pulmonary artery obstruction: Diagnosis and management

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