Ductus Arteriosus Aneurysm

E-chocardiography Journal: Alphabetical List / Chronological List / Images / Home Page

Dr. Rishi Anand.

During fetal development, oxygen-rich blood is conveyed to the fetus through the umbilical artery. With the fetal lungs being collapsed, there is high pulmonary vasculature resistance. The ductus arteriosus is a communication which exists between the pulmonary artery and the descending thoracic aorta which bypasses this high resistance pulmonary vasculature.

With the baby’s first breath, circulating prostaglandins which maintain the patency of this ductus arteriosus are reduced. This, in combination with the reduction in the resistance of the pulmonary vasculature, leads to the closure of the ductus arteriosus. Now oxygen-poor blood is pumped from the right ventricle to the lungs where it is oxygenated and then returned to the left side of the heart to be delivered to the rest of the body.

In approximately 1 of 2000 births, the communication between the pulmonary artery and the aorta fails to close. Risk factors for failure of the ductus arteriosus to close include prematurity, hypoxia, and maternal infection with rubella.

With the persistence of the ductus arteriosus, blood, whose path follows the path of least resistance, is shunted from to the left side of the heart back to right side of the heart thereby reducing the delivery of oxygen-rich blood to the organs and tissues of the body.

Among complications such as heart failure and pulmonary hypertension is the possiblity of ductus arteriosus aneurysm.

Ductus Arteriosus Aneursyms are Associated with Connective Tissue Disorders

Je BK, Yoo SY, Lee W et al. Aneurysm of the ductus arteriosus in a patient with Larsen syndrome. Pediatr Radiol. 2006 Nov;36(11):1215-8. Epub 2006 Sep 28.
Larsen syndrome is characterized by multiple congenital joint dislocations and a characteristic flattened facial appearance. The etiology is unknown, but the syndrome is considered a probable connective tissue disorder and the cardiovascular manifestations are similar to those of Marfan syndrome. We describe an 8-year-old girl with the characteristic features of Larsen syndrome who was also affected by a saccular aneurysm of the descending aorta, just distal to the left subclavian artery.

Dyamenahalli U, Smallhorn JF, Geva T et al. Isolated ductus arteriosus aneurysm in the fetus and infant: a multi-institutional experience. J Am Coll Cardiol. 2000 Jul;36(1):262-9.
OBJECTIVES: The purpose of this study was to describe the clinical characteristics and outcome and to elucidate the pathogenesis of ductus arteriosus aneurysm (DAA). BACKGROUND: Ductus arteriosus aneurysm is a rare lesion that can be associated with severe complications including thromboembolism, rupture and death. METHOD: We reviewed the clinical records, diagnostic imaging studies and available histology of 24 cases of DAA, diagnosed postnatally (PD) in 15 and antenatally (AD) in 9 encountered in five institutions. RESULTS: Of PD cases, 13 presented at <2 months, and all AD cases were detected incidentally after 33 weeks of gestation during a late trimester fetal ultrasound study. Of the 24, only 4 had DAA-related symptoms and 6 had associated syndromes: Marfan, Smith-Lemli-Opitz, trisomies 21 and 13 and one possible Ehlers-Danlos. Three had complications related to the DAA: thrombus extension into the pulmonary artery, spontaneous rupture, and asymptomatic cerebral infarction. Six underwent uncomplicated DAA resection for ductal patency, DAA size or extension of thrombus. In the four examined, there was histologic evidence of reduced intimal cushions in two and abnormal elastin expression in two. Five of the 24 died, with only one death due to DAA. Of 19 survivors, all but one remain clinically asymptomatic at a median follow-up of 35 months; however, two have developed other cardiac lesions that suggest Marfan syndrome. A review of 200 consecutive third trimester fetal ultrasounds suggests an incidence of DAA of 1.5%. CONCLUSIONS: Ductus arteriosus aneurysm likely develops in the third trimester perhaps due to abnormal intimal cushion formation or elastin expression. Although it can be associated with syndromes and severe complications, many affected infants have a benign course. Given the potential for development of other cardiac lesions associated with connective tissue disease, follow-up is warranted.

Diagnosis of Ductus Arteriosus Aneurysms Includes Echocardiography, CT, MRI, and Angiography

Siu BL, Kovalchin JP, Kearney DL. Aneurysmal dilatation of the ductus arteriosus in a neonate. Pediatr Cardiol. 2001 Sep-Oct;22(5):403-5.
Fetal ultrasound provides the capacity for early detection of a variety of congenital heart diseases. We report a case of aneurysmal dilatation of a patent ductus arteriosus detected in utero and subsequently confirmed by transthoracic echocardiography in the neonatal period. Prompt recognition of the ductus aneurysm with resultant surgical ligation may have averted potentially serious complications from this condition.

Peivandi AA, von Bulow S, Schmid FX. Aneurysm of the ductus arteriosus Botalli. Herz. 2000 Nov;25(7):703-6.
A congenital aneurysm of the ductus arteriosus Botalli was detected by chest X-ray as an intrathoracic mass in a 7-day-old infant. Following confirmation of the diagnosis by echocardiography and MRI the aneurysm was successfully resected via left lateral thoracotomy without cardiopulmonary bypass. The postoperative course was uneventful. Six years after operation the patient is asymptomatic and growing normally. An intrathoracic mass may be considered in the differential diagnosis especially in infants and children. Aneurysms of ductus arteriosus potentially are associated with serious complications. Timely diagnosis and early surgical intervention are decisive for prevention of serious complication and death.

Acherman RJ, Siassi B, Wells W et al. Aneurysm of the ductus arteriosus: a congenital lesion. Am J Perinatol. 1998;15(12):653-9.
The objective of this study was to evaluate the clinical, radiological, and echocardiographic findings in 11 neonates with aneurysm of ductus arteriosus presented in our institutions between 1993 and 1996, and to postulate a new theory for the pathogenesis of this lesion. Medical records, radiographic studies, and echocardiograms were reviewed. All infants underwent follow-up echocardiograms every 2 to 3 days until the aneurysm spontaneously resolved or surgery was performed. The infants were predominantly term males; six had evidence of fetal distress, two were diagnosed prenatally by fetal echocardiogram, chest X ray evidenced mediastinal mass in six patients. The first echocardiogram showed structurally normal heart with an aneurysmal patent ductus arteriosus. In eight patients the aneurysm completely resolved by 5 to 10 days. One infant underwent surgical resection of the aneurysm after observation for 11 days with no change in size. Thrombosis of the aneurysm was noted in two patients; both underwent surgery. Increasing reports of ductal aneurysms in infants may reflect the availability of high-resolution echocardiography and more frequent use of echocardiography in the neonatal intensive care unit. Spontaneous resolution occurred in the majority of cases as in previous reports. We postulate that, at least in some cases, aneurysm of the ductus arteriosus is a congenital lesion that may represent poststenotic dilation of the ductus due to turbulent flow through a stenotic segment at its pulmonary artery end during fetal life. The presence of aneurysm of the ductus arteriosus should be excluded in selected cases of fetal distress, by fetal echocardiography.

Laurin S, Sandstrom S, Ivancev K et al. Ductus arteriosus aneurysm imaging using modern diagnostic methods. Acta Radiol. 1992 Jul;33(4):285-91.
An aneurysm of the ductus arteriosus carries a high risk of rupture. The radiologic findings are described, based on a review of the literature, and on our experience of 5 patients, successfully diagnosed and treated. A left-sided mediastinal mass is always present, obscured only if the aneurysm has already ruptured. Although the lesion seems to be congenital, conventional radiology sometimes demonstrates wall calcifications. Ultrasonography is well suited to demonstrate the vascular nature of the mass, but interpretation may be difficult because of intervening air-containing lung parenchyma. CT, especially using dynamic scanning with contrast enhancement, is an excellent noninvasive diagnostic method. Angiography is frequently necessary to demonstrate patency or occlusion of the aortic and pulmonary ends of the ductus. The differential diagnosis must include a large number of mediastinal tumors. A temporary widening of the ductus in the newborn is common and must be differentiated from a true aneurysm. Surgical treatment today is usually successful.

Lee WJ, Chen SJ, Wu MH et al. Regression of ductus arteriosus aneurysm in a neonate demonstrated by three-dimensional computed tomography. Int J Cardiol.1999 Feb 28;68(2):231-4.
We present a case of ductus arteriosus aneurysm in a neonate in whom the diagnosis was made by echocardiography, three-dimensional surface rendering computed tomography (CT) and was subsequently confirmed by angiography. Regression of aneurysm after indomethacin treatment was clearly demonstrated in a follow-up CT scan. To our knowledge, this is the first report of ductus arteriosus aneurysm and its regression detected by 3D surface rendering CT.

Tseng JJ, Jan SL. Fetal echocardiographic diagnosis of isolated ductus arteriosus aneurysm: a longitudinal study from 32 weeks of gestation to term. Ultrasound Obstet Gynecol. 2005 Jul;26(1):50-6.
OBJECTIVE: To investigate the echocardiographic characteristics of isolated fetal ductus arteriosus aneurysm (DAA) and the factors influencing its development. METHODS: Fetal echocardiograms for 509 low-risk singleton pregnancies were performed longitudinally from 32 weeks of gestation. The ventricular outflow tracts and great vessels were visualized, focusing on changes in the ductus arteriosus (DA), and fetuses with DAA or ductus arteriosus dilatation (DAD) were identified. Prenatal and perinatal findings were compared between those infants diagnosed postnatally with and those without neonatal DAA. RESULTS: Forty-one of the 509 fetuses (8.1%) studied had neonatal DAA; 2.2% (11/509) had DAA and 5.9% (30/509) DAD before delivery. The mean gestational age at the time of diagnosis of fetal DAA/DAD was 36.9 +/- 1.7 weeks. The mean maximal internal diameter of DAA/DAD was 8.0 +/- 0.8 mm at initial diagnosis, increasing to 10.8 +/- 1.6 mm before delivery. All cases originated from the aortic end of the DA. Compared with fetuses without neonatal DAA, the affected cases had a markedly more curved DA prior to diagnosis, and higher peak velocities at the aortic end of the DA (P < 0.05). Newborns with fetal DAA/DAD had a larger placental weight to birth body weight (BBW) ratio and were less likely to have a BBW appropriate for gestational age (P < 0.05). All cases of fetal DAA/DAD regressed spontaneously. CONCLUSION: Isolated neonatal DAA is a continuity of fetal DAA/DAD. The development of fetal DAA/DAD is likely to be related to the higher peak velocities in the more markedly curved DA occurring in the latter part of the third trimester. Fetal DAA/DAD usually initiates at the aortic end of the DA, followed by progression towards the pulmonary end.

Jackson CM, Sandor GG, Lim K. Diagnosis of fetal ductus arteriosus aneurysm: importance of the three-vessel view. Ultrasound Obstet Gynecol. 2005 Jul;26(1):57-62.
OBJECTIVE: To present four cases of ductus arteriosus aneurysm (DAA) detected by fetal echocardiography and highlight the value of the three-vessel view in the diagnosis of DAA. METHODS: In addition to the standard fetal echocardiographic views, we examined the three-vessel view in four cases of DAA. The three-vessel view was achieved by sliding the transducer cranially from the four-chamber plane toward the fetal upper mediastinum to demonstrate cross-sections of the main pulmonary artery, the ascending aorta and superior vena cava arranged in a straight line from the left anterior to the right posterior aspect of the mediastinum. DAA was diagnosed when there was a tortuous ductus arteriosus with a dilation that protruded leftward of the aortic arch. CASE SERIES: In the first case, an insulin-dependent diabetic woman underwent fetal ultrasound examination at 36 weeks' gestation showing right-to-left cardiac disproportion and bidirectional flow in the aorta and main pulmonary artery (PA). The three-vessel view showed a dilated ductus arteriosus (DA) which was stenosed at its distal end. In the second case, a woman had fetal ultrasound scans at 38 and 39 weeks' gestation for suspected intrauterine growth restriction and oligohydramnios. The scans identified an abnormal aortic arch and the three-vessel view showed an elongated vascular structure at the distal end of the PA, which was the DAA. In the third case, a woman with a high-risk obstetric history had multiple scans showing an abnormal PA to aorta relationship, with an apparent 'kink' in the PA. The three-vessel view clarified that this was the DAA. In the fourth case, a woman was scanned because of a 3-kg weight gain in 1 week. The fetal ultrasound scan showed moderate polyhydramnios with normal fetal growth and normal intracardiac anatomy and flow, and the three-vessel view demonstrated a large DA. In each of these cases, the DAA appeared to have occurred in isolation with non-specific clinical findings. CONCLUSION: We advocate the use of the three-vessel view, in conjunction with the standard echocardiography views currently employed, to assist in the diagnosis of DAA.

Complications Associated with Ductus Arteriosus Aneursysms Include Compression of Local Structures as a Result of Mass Effect

Pastuszko P, Eisenberg JA, Diehl JT. Ductus arteriosus aneurysm in an adult patient presenting with hoarseness. J Card Surg. 2005 Jul-Aug;20(4):386-8.
Aneurysm of the ductus arteriosus is a rare diagnosis, with most cases found in the pediatric population. The unusual adult cases reported in the literature have been associated with high morbidity and a surgical repair has been recommended. We report a case of a 60-year-old man who presented with hoarseness secondary to a ductus arteriosus aneurysm and underwent a repair of this abnormality via a left posterolateral thoracotomy utilizing partial cardiopulmonary bypass

Hornung TS, Nicholson LA, Nunn GR, Hawker RE. Neonatal ductus arteriosus aneurysm causing nerve palsies and airway compression: surgical treatment by decompression without excision. Pediatr Cardiol. 1999;20:158-60.
A 4-kg male child, born at 34 weeks to a gestational diabetic mother, had a large ductus arteriosus aneurysm causing phrenic and recurrent laryngeal nerve palsies and large airway compression. The right and left atrial appendages and distal descending aorta were cannulated, allowing left heart partial or complete cardiopulmonary bypass as necessary. On bypass the ductus was ligated, decompressed, and oversewn but not excised. Examination 1 month later suggested resolution of the recurrent laryngeal palsy and echocardiography showed regression of the aneurysm. Ductus ligation and decompression was an effective surgical treatment, which is less likely to cause complications than resection.

Treatment of Ductus Arteriosus Aneurysms Includes Surgery as well as Medical Drug Therapy with Indomethacin

Lund JT, Jensen MB, Hjelms E. Aneurysm of the ductus arteriosus. A review of the literature and surgical implications. Eur J Cardiothorac Surg. 1991;5:566-70.
One hundred and forty-four cases of aneurysms of the ductus arteriosus (DAA) have been reported in the literature of which 106 appeared spontaneously and 38 followed surgical treatment of a patent ductus arteriosus (PDA). Within the last few years there has been an increasing number of reported spontaneous DAA. However, the real incidence is presumably still underestimated. Aortography is a well established diagnostic method. In neonates, transthoracic echocardiography has shown convincing potential, whereas in older children and adults, transoesophageal echocardiography has yielded very promising results. Serious complications following spontaneous DAA are rupture, erosion, infection and thromboembolism. In infants younger than 2 months of age, the complication rate is 31%, in children between 2 months and 15 years, 66%, in adults, 47%. The rate of complications following postoperative DAA is even higher: 91% of the unoperated cases died due to rupture or infection. The operative mortality in children older than 2 months and adults is low. In the neonate group, 2 of 8 died during operation. The operative mortality in patients with postoperative DAA was 26%. Based on information from the literature, we suggest prompt surgical treatment of all spontaneous DAA in patients older than 2 months of age, and in all patients with postoperative DAA. In infants, a DAA should be closely followed with echocardiography, as spontaneous regression has been reported in this age group. If no regression is seen within a few days, it should be surgically corrected.

Doege C, Linderkamp O, Gorenflo M, et al. Treatment of an aneurysmal dilatation of the ductus arteriosus with indomethacin in a premature infant. Pediatr Cardiol. 2006 Jan-Feb;27(1):166-7.
A large ductus arteriosus aneurysm can lead to potentially serious and life-threatening complications. We report a case of aneurysmal dilatation of the ductus arteriosus in a premature infant that was successfully treated with indomethacin. Thus, surgical intervention with general anesthesia could be avoided.

Ohmi M, Shibuya T, Kawamoto S, et al. Surgical treatment of aneurysm of the ductus arteriosus in the adult. Kyobu Geka. 2002 Feb;55(2):149-54.
Aneurysm of the ductus arteriosus is rare, however, reports on this lesion have increased recently with the progress of thoracic aortic surgery in Japan. We report 3 male cases aged 58, 59, 73 years. Aneurysms and total aortic arch were replaced by artificial graft through median sternotomy using selective cerebral perfusion in 2 cases. Through left posterolateral thoracotomy using deep hypothermia with circulatory arrest, proximal descending thoracic aorta including the aneurysm was replaced in 1 case. Their postoperative courses were uneventful. Surgery for this lesion is safe and various surgical approaches and circulatory supporting methods can be selected depending on the size of aneurysm and associated lesions.

Ayabe T, Nakamura K, Nakajima S. Surgical management of ductus arteriosus aneurysm in adults. Jpn J Thorac Cardiovasc Surg. 2000 May;48(5):304-6.
Aneurysm of the ductus arteriosus is rare, especially in adults, and preoperative diagnosis is very difficult, requiring intraoperative diagnosis. A 71-year-old man was preoperatively diagnosed with ductus arteriosus aneurysm by computed tomographic scanning, magnetic resonance imaging, and aortography, which demonstrated a saccular aneurysm of the distal aortic arch. Under partial extracorporeal circulation, the aneurysm was replaced by an artificial vascular graft. The location and morphology of the aneurysm confirmed the preoperative diagnosis. The postoperative course was uneventful. Surgery for this condition is safe and is recommended the same as for common thoracic aortic aneurysm.

Back to E-chocardiography Home Page.

The contents and links on this page were last verified on February 23, 2007.