Multidetector computed tomography and 2- and 3-dimensional postprocessing in the evaluation of congenital thoracic vascular anomalies

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Journal of Cardiovascular Computed Tomography (2008) 2, 245–255

ictorial Essay

ultidetector computed tomography and 2- and-dimensional postprocessing in the evaluationf congenital thoracic vascular anomalies

ldo Morra, MDa*, Alberto Clemente, MDa,b, Mario Del Borrello, MDa,alvatore Berretta, MDc, Pietro Greco, MDd

Department of Radiology, Euganea Medica Diagnostic Center, Via Colombo no. 13, 35020, Albignasego (PD), Italy;Department of Radiology, University of Ferrara, Italy; cDepartment of Oncological Surgery, University of Catania, Italy;

OBJECTIVE: We report examples of congenital anomalies of the thoracic vessels studied withmultidetector computed tomography (MDCT), with special respect to bidimensional (2D) and 3-di-mensional (3D) postprocessing.

METHODS: Nonselected patients with vascular and nonvascular disorders underwent chest MDCTwith the use of a 16-row scanner. Postprocessing was done to obtain 2D and 3D postprocessing images.

CONCLUSION: MDCT with 2D and 3D postprocessing is helpful in evaluating complex vascularcongenital anomalies of the chest.© 2008 Society of Cardiovascular Computed Tomography. All rights reserved.

KEYWORDS:Congential thoracicvascular anomalies;Multidetector row CT;Postprocessing

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Congenital anomalies o f t h e thoracic vessels are causedy false embryogenesis and are relatively common. Theyay cause symptoms and be diagnosed at birth,1 or theyay be asymptomatic and discovered incidentally on

maging.Previously conventional angiography was considered the

eference standard for evaluating vascular disease. How-ver, this has largely been supplanted by noninvasive im-ging methods, particularly since the introduction of sophis-icated diagnostic tools such as magnetic resonance andultidetector computed tomography (MDCT) angiography.his latter technique is capable of studying the entire chesturing a single breathhold, and it allows clinicians and

Conflict of interest: The authors report no conflicts of interest.* Corresponding author.E-mail address: [email protected]

wSubmitted January 21, 2008. Accepted for publication May 21, 2008.

934-5925/$ -see front matter © 2008 Society of Cardiovascular Computed Tomoi:10.1016/j.jcct.2008.05.147

adiologists to identify congenital thoracic vascular anom-lies noninvasively.2

ethods

We studied 2705 outpatients, 2103 adult subjects for aonvascular disorder and 602 adult subjects who wereuspected of having vascular or coronary artery disease;ll of them were adult (mean age, 58 years), 41% wereomen and 59% were men. They underwent chestDCT angiography in the period between December

003 and December 2007. MDCT scans were performedith the use of a 16-channel MDCT scanner (LightSpeed6; General Electric Company, Milwaukee, WI). Fortandard chest CT we used a craniocaudal acquisition,ollimation of 3.75 mm, table increase of 27.50 mm, tubeoltage of 120 kV, tube current of 250 mA; the data set

ography. All rights reserved.

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246 Journal of Cardiovascular Computed Tomography, Vol 2, No 4, July/August 2008

igure 1 A 39-year-old man with history of corrected tetralogy of Fallot had from undefined precordialgia. CT electrocardiographic-gatedcan showed a right-sided aortic arch (A, axial image), the entire heart volume, and the right-sided aortic arch (B, 3D-VR). The rightoronary artery was also viewed (arrow). A 62-year-old female oncology patient. CT images showed a right-sided ascending aorta and aorticrch (C, axial), an aberrant left subclavian artery (ALSA) (arrow), arising from a Kommerell’s diverticulum (pointed arrow) (D, coronal

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247Morra et al MDCT of thoracic vascular anomalies

n the case of vascular study we used an angiographicechnique during simultaneous electrocardiographic recording,raniocaudal acquisition, collimation of 0.625–1.25 mm, tablencrease of 27.50 mm, tube voltage of 120 kV, and tube currentf 380–440 mA. In all cases, approximately 120–150 mL ofow molecular weight nonionic iodinated contrast agent (320–50 mg I/mL) was injected intravenously at 4–5 mL/s, fol-owed by a 50-mL saline chaser bolus through a catheter

igure 2 A 63-year-old female oncology patient. The examinatioxial image; B, sagittal MPR; C, 3D-VR) crossing the midline betwithout creating deviation or compression of mediastinal structuresediastinal enlargement (not shown). An aneurysmal dilatation o

mage; E, sagittal MPR; F, 3D-VR) causes extrinsic compression

laced in the cubital vein. a

The postprocessing was performed with the use of 2orkstations (General Electric Company Advantage Win-ow 4.3; TeraRecon Aquarius 3.6.2.3) to obtain 2-dimen-ional to 3-dimensional (3D) images with multiplanar re-ormatting (MPR), maximum intensity projection (MIP),nd volume-rendering (VR) techniques for the evaluation ofhe following anomalies of the thoracic vascular system:nomalies of the thoracic aorta (right-sided aortic arch,

s an aberrant right subclavian artery (arteria lusoria) (arrows) (A,e spine and the esophagus (pointed arrow) to reach the right side

2-year-old man with dysphagia who had radiographic evidence ofberrant right subclavian artery (arteria lusoria) (arrow) (D, axialesophagus (E, pointed arrow).

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berrant left subclavian artery, Kommerell’s diverticulum,

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248 Journal of Cardiovascular Computed Tomography, Vol 2, No 4, July/August 2008

berrant right subclavian artery, aortic coarctation, andatent ductus arteriosus [PDA]), anomalies of the pulmo-ary arterial system (“absence” of pulmonary artery, partialnomalous pulmonary venous connection), anomalies of theena cava (persistent left superior vena cava [SVC]), andongenital pulmonary venolobar syndrome (pulmonary se-uestration).

nomalies of the thoracic aorta

Anomalies of the thoracic aorta include a complex group

Figure

f malformations with an incidence difficult to determine, c

ecause most of them are asymptomatic and occur in.5%–3% of the population.3

ight-sided aortic arch

Right-sided aortic arch is a rare anatomic variant (0.1% ofhe population) secondary to persistence of the entire rightorsal arch and involution of the fourth segment of the left archFig. 1). There are 2 main types: right aortic arch with mirrormage branching and right aortic arch with aberrant left sub-

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lavian artery (ALSA).

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249Morra et al MDCT of thoracic vascular anomalies

berrant left subclavian artery

ALSA occurs in almost all cases of right-sided aorticrch4,5 (Fig. 1). It crosses the midline on the left behind thesophagus. The ALSA is the last of the 4 branches arisingrom the arch and occasionally from an aortic diverticulumepresenting a remnant of the distal primitive left arch.6

ommerell’s diverticulum

Kommerell’s diverticulum is the name of the broad-basedrigin of the root of the subclavian artery (Fig. 1). It can occurn a number of anomalies of the aortic arch system that can, buto not always, cause symptoms of tracheal or esophagealompression. The diverticulum is most frequently present inases of right aortic arch with an ALSA associated with theersistence of the right aortic arch.7 This diverticulum is a rarenomaly, occurring in �1% of the population; it is usuallyssociated to an aneurysm.8

berrant right subclavian artery

Aberrant right subclavian artery (arteria lusoria) (Fig. 2),lso called arteria lusoria (named after lusus naturae, freakf naturae), is an abnormal origin of the right subclavianrtery from the aortic arch. It affects approximately.4%–2% of the population.9 This anomaly is caused by theersistence of the posterior segment of the embryologicourth aortic arc. Usually, it is asymptomatic; very rarely its related to aneurysmal dilatation10,11 (Fig. 2) and causesysphagia (called dysphagia lusoria).

ortic coarctation

Aortic coarctation is a common vascular anomaly5%–8% of all cases of congenital heart disease)12 that mayccur in isolation (2%) or in combination with cardiacnomalies.13 It is secondary to a partial involution of the leftorsal aortic arch and is characterized by a discrete narrow-ng or constriction of the lumen of the aorta distal to therigin of the left subclavian artery near the insertion of theigamentous arteriosus. It is classified as preductal, ductal,nd postductal coarctation; this last form is the adult varietyas in our case) (Fig. 3).

atent ductus arteriosus

PDA is the most common type of extracardiac shunt,14

nd it is one of the most common cardiovascular anomalies10%–12% of all congenital heart abnormalities). It is thehird most common cause of a left-to-right shunt in adultsFig. 4) after atrial and ventricular septal defects and theommonest in newborns. PDA is a vessel leading from theifurcation of the pulmonary artery to the aorta just distal tohe subclavian left artery. It is normally patent in the fetusnd closes within 2 or 3 days after birth. Its persistence

uctus closure with the change from fetal to adult circula-ion. PDA can be asymptomatic (it is often small and dis-overed occasionally) or symptomatic.15

nomalies of the pulmonary system

Absence” of pulmonary artery

Absence of pulmonary artery can be unilateral or bi-

igure 3 A 50-year-old woman with dilated ascending aorta de-ected during echocardiographic evaluation (not shown). CT scanonfirmed a moderate ectasia of the ascending aorta and a stenosis athe aortic isthmus (arrow) (A, axial 3D-VR; B, parasagittal 3D-VRmages), suggestive of postductal aortic coarctation.

ateral and may be encountered either as an isolated

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250 Journal of Cardiovascular Computed Tomography, Vol 2, No 4, July/August 2008

ntity or as an associated condition in 0.39% of theopulation. The term absent pulmonary artery is mis-eading because an intrapulmonary vessel with relativelyormal distribution is present in this condition.16 Thetresia of the pulmonary artery results from involution ofhe proximal sixth aortic arch and persistence of theonnection of the intrapulmonary pulmonary artery to theistal sixth aortic arch corresponding to fetal ductusrteriosus.

In some cases reported in literature (12 cases in total)

igure 4 An 84-year-old woman with echocardiographic evidenarrow) (A, axial image; B, 3D-VR; C, virtual endoscopy view thf the aortic arch looking toward the distal arch.

single PDA was identified, whereas in 3 cases a bilat- o

ral ductus arteriosus was present.1,16,17 In this last un-ommon anomaly the blood supply to the lung can be byypertrophic collateral arteries and less frequently fromypertrophic bronchial arteries.1 In our case there was antresia of the common trunk of the pulmonary arteryFig. 5).

artial anomalous pulmonary venous connection

Partial anomalous pulmonary venous connection of 1

small asymptomatic patent ductus arteriosus confirmed at MDCTthe aortic lumen). The virtual endoscopic view is from the inside

ce of arough

r 2 but not all of the pulmonary veins occurs in approx-

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251Morra et al MDCT of thoracic vascular anomalies

mately 0.6% of the population (Fig. 6).18 There are manyatterns of partial anomalous pulmonary venous con-ection; usually a pulmonary vein originating from theuperior or middle right lobe or from the left supe-ior lobe, draining into the SVC, creates a left-to-right

igure 5 A 45-year-old woman with history of a complex cngiography of the thorax at the level of the aortic root showed theight pulmonary arteries (A and B, axial images, arrows). 3D-igamentum-like structure (C, 3D-VR in parasagittal cut plane viewhe lobar and segmental pulmonary arteries by a dilated patent ducsecondary PDA (A, pointed arrow; D, right arrow) arising from

rtery (B–D, pointed arrow) arising from the proximal descendin

hunt.19 r

nomalies of the vena cava

ersistent left SVC

Persistent left SVC, either alone or in association with

tal cardiac anomaly had chest pain radiating to her back. CTof the common trunk of the pulmonary artery with small left and

owed the small left pulmonary artery (pointed arrow) and thew) substituting the main pulmonary artery. The blood supplied toeriosus (PDA) (D, 3D-VR coronal cut plane view, left arrow), byght side of the descending aorta and by a hypertrophic collateral).

ongeniatresia

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ight SVC, is a rare congenital vascular anomaly with an

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252 Journal of Cardiovascular Computed Tomography, Vol 2, No 4, July/August 2008

ncidence of 0.3%–0.5% in the general population20 andetween 0.3% and 11% in patients with congenital heartisease.21 This is due to the failure in development of theeft brachiocephalic vein and to the persistence of the leftnterior cardinal vein. The left SVC commonly drains intohe right atrium by way of the coronary sinus (Fig. 7), andarely a left-sided SVC may drain into the left atrium, creating

igure 6 A 50-year-old woman (same patient as in Fig.3) with dhown). CT scan confirmed moderate ectasia of the sinotubular asceA, axial MIP image; B, oblique MIP; C and D, 3D-VR). The riulmonary veins, at the level of middle mediastinum, converge inthe brachiocephalic vein.

right-to-left shunt22,23 (Fig. 7). U

ongenital pulmonary venolobar syndrome

Pulmonary sequestration, a rare form of congenital pul-onary venolobar syndrome, is characterized by nonfunc-

ioning lung tissue separated from the normal bronchopul-onary tree and vascularized by an aberrant systemic

rtery. It is classified as either intralobar or extralobar.

scending aorta detected during echocardiographic evaluation (notaorta and showed partial anomalous pulmonary venous connectionperior and middle pulmonary veins and the left superior-lingulargle vein (A and B, pointed arrow; C and D, arrow) draining into

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253Morra et al MDCT of thoracic vascular anomalies

igure 7 A 73-year-old woman during oncologic follow-up underwent CT scan, showing the presence of a persistent left superior vena cavaSVC) (A, coronal); the additional left-sided branch of the SVC drains into the right atrium by way of the coronary sinus (B, parasagittal 3D-VR,rrow). A 29-year-old man with history of congenital cardiac anomaly with suspected coronary disorder. MDCT scan showed a left SVC (C, MIPoronal view; D, sagittal 3D-VR) draining into the left atrium, creating a right-to-left shunt.

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igure 8 A 48-year-old woman with the radiologic evidence of a pulmonary mass (not shown). CT images of the lung bases confirmedhe presence of a heterogeneous consolidation of the right lung with cystlike structures within the lesion; these aspects were suggestive ofulmonary sequestration. A large vessel originating from the thoracic descending aorta (A, axial MIP; B, coronal MIP; D, 3D-VR, arrow)nd the venous drainage (A; C, coronal MIP; D; E, 3D-VR, pointed arrow) of the lesion through the hemiazygos–azygos system were also

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255Morra et al MDCT of thoracic vascular anomalies

acic aorta (Fig. 8), but in approximately 20% of cases itomes from the upper abdominal aorta, celiac artery, orplenic artery. In the majority of cases (95%) of pulmonaryequestration, drainage of intralobar sequestration occurshrough the pulmonary veins of the parent lobe into the lefttrium, whereas 5% drains into the systemic circulationhrough the hemiazygos-azygos system (Fig. 8) or intercos-als veins or SVC toward the right atrium.24

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