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ISSN: Print -2349-0977, Online - 2349-4387

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Year : 2014  |  Volume : 1  |  Issue : 3  |  Page : 195-210

Correlative imaging in congenital heart disease

Department of CT and MRI, Sir Ganga Ram Hospital, New Delhi, India

Date of Web Publication27-May-2015

Correspondence Address:
Anurag Yadav
Department of CT and MRI, Sir Ganga Ram Hospital, Old Rajinder Nagar, New Delhi - 110 060
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2349-0977.157764

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For the evaluation of congenital heart diseases (CHDs), echocardiography is the initial diagnostic modality and Catheter angiography is the gold standard for delineation of the anomalies. Though echocardiography is the initial diagnostic modality in CHDs, it has its limitations and Catheter angiography is relatively invasive and should be reserved for therapeutic procedures. Multidetector computed tomography (MDCT) has emerged as an important tool for cardiac imaging owing to constant advancements in computed tomography scanners. With the judicious and innovative use of three-dimensional reconstructions, the depiction of congenital anomalies is more accurate and it is possible to perform a virtual surgery to aid the surgeon in planning the approach to the patient as a one or two step procedure, counseling the parents about the outcome and giving a fair estimate of the cost of treatment. MDCT acts as a one stop shop for a complete evaluation of the cardiac, extra-cardiac, visceral and skeletal anomalies; their combinations and subsequent effects on each other. Excellent delineation of the vascular compressions on the tracheo-bronchial tree, degree of main and branch pulmonary artery stenosis, anomalous drainage of pulmonary and systemic veins, interruptions and narrowing of the aorta, anomalies of origin and course of coronary arteries are unique to MDCT.

Keywords: Congenital heart diseases, echocardiography, multidetector computed tomography

How to cite this article:
Yadav A, Buxi T, Reddy S, Gupta S, Rawat KS, Ghuman SS. Correlative imaging in congenital heart disease. Astrocyte 2014;1:195-210

How to cite this URL:
Yadav A, Buxi T, Reddy S, Gupta S, Rawat KS, Ghuman SS. Correlative imaging in congenital heart disease. Astrocyte [serial online] 2014 [cited 2020 Sep 27];1:195-210. Available from: http://www.astrocyte.in/text.asp?2014/1/3/195/157764

  Introduction Top

Congenital heart disease (CHD) is one of the major problems worldwide causing high morbidity and mortality with incidence ranging from 3.7 to 17.5/1000 live births in the world [1] and 8-10/1000 live births in India. They may occur in many different combinations and may be very complex, and their accurate diagnosis requires a sequential segmental approach. Several approaches to classifying CHD have been suggested.

The physiological classification systems based on clinical manifestations like cyanosis and the presence of an increase or decrease in pulmonary vascularity was proposed. [2]

  1. Acyanotic with increased pulmonary vascularity.
  2. Acyanotic with normal pulmonary vascularity (with either outflow obstruction or valvular insufficiency).
  3. Cyanotic with decreased pulmonary vascularity (with an intra-cardiac defect that shunts the blood away from the lung).
  4. Cyanotic with increased pulmonary vascularity.

A segmental analysis of the CHD was introduced in 1972 by Van Praagh [3] and this approach is now used worldwide. It is flexible and easy to understand, applicable to any imaging modality, and thus particularly useful in clinical practice.

  Materials and Methods Top

Cardiac computed tomography angiography (CTA) was performed at a single center on Philips Ingenuity 128 slice computed tomography (CT) scanner using a collimation of 64 mm × 0.625 mm, slice thickness of 0.9 mm, pitch of 0.8, rotation time of 0.5 s with a 512 matrix and iDose. The dose and rate of intravenous contrast were adjusted according to the weight of the patient. Echocardiography was done on Philips IE33 X-matrix ultrasound system using S7-2, S5-1 and S12-4 probes. The anomalies are described systematically at the levels of cardiac chambers, aorta, pulmonary arteries and pulmonary veins.

In segmental approach, the cardiac anatomy is assessed first by dividing the heart into three distinct segments, which are based on 10 embryonic regions: These segments are:

The visceroatrial situs

It refers to the position of the atria in relation to the nearby anatomical structure (including the stomach, liver, spleen and bronchi).

The ventricular loop orientation

The positions of the ventricles are identified on the basis of their internal morphological features. The ventricular loop may tend rightward (D-loop) or leftward (L-loop).

The origin and position of the great vessels

It is determined by identification of the great vessels and their connection (atrio ventricular and ventriculo arterial connection).

Other abnormalities of individual segments of the cardiovascular anatomy are also assessed in a segmental fashion based on the visualization of blood flow into, through and out of the heart that is, the anomalies of cardiac chambers, septa, valves, outflow tracts and other associated anomalies, including the lung parenchyma.

  Situs Top

The abdominal and thoracic viscera are asymmetrical, and for this reason normal situs can be recognized by position of the atria and viscera relative to the midline. The very high association with the inferior vena cava draining into the right atrium [4] has led to the development of the term visceroatrial situs. The presence of asymmetry in the bronchial branching pattern results in the term bronchial situs. The right main bronchus is shorter, wider and more vertically oriented than the left main bronchus. The right lung is trilobed when compared to bilobed left lung. The bronchial situs [5] nearly always corresponds to the visceroatrial situs.

The cardiac situs is assessed by locating and identifying the left and right atria. Anatomically, the differentiation of atrial chambers is based on the morphological aspects of the atrial appendages. In general, the right atrial appendage is broad and triangular shaped, whereas the left atrial appendage is narrow, pointed, and tubular. Most of the time, the appendages are not reliably identifiable at CT imaging, and the localization of non-cardiac organs such as the bronchial anatomy is a reliable indicator of the atrial arrangement.

Situs solitus

It is the usual arrangement of organs and vessels within the body. The systemic atrium or morphological right atrium is on the right with tri-lobed lung, liver, gall bladder, and inferior vena cava on the right side. The morphological left atrium is on the left with bi-lobed lung, stomach, single spleen, and aorta on the left side. The incidence of CHD in patients with situs solitus and levo cardia is only 0.6-0.8% [6] [Figure 1]a.
Figure 1: The anatomic relationships that characterize: (a) Situs solitus and (b) situs inversus. left atrium (LA), left lung (LL), right atrium (RA), right lung (RL).

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Situs inversus

It refers to an anatomic arrangement that is the mirror image of situs solitus. The systemic atrium is on the left with tri-lobed lung, liver, gall bladder, and inferior vena cava on the left side. The pulmonary atrium is on the right with bi-lobed lung, stomach, single spleen, and aorta on the right side. Situs inversus is seen in 0.01% of the population, and the incidence of the CHD in patients with situs inversus is 3-5% [6] [Figure 1]b.

Situs ambiguous (heterotaxy syndrome)

It refers to visceral malposition and dysmorphism associated with indeterminate atrial arrangement. [7] The complexity of this syndrome is reflected in the various terms used to sub classify it, including asplenia syndrome, double right sidedness, right isomerism, or Ivemark syndrome and polysplenia syndrome, double left sidedness, or left isomerism. The incidence of CHD in patients with heterotaxy is very high, ranging from 50% to 100%. Two subsets of situs ambiguous are well-recognized : Right isomerism and left isomerism.

Heterotaxy syndrome with right isomerism

The classic right isomerism implies bilateral trilobed lungs with bilobed minor fissures and eparterial bronchi. The main bronchus is located superior to the ipsilateral main pulmonary artery (MPA) on each side. A midline liver is running across the upper abdomen, absent spleen and stomach present in an indeterminate position. Both atrial chambers have right sided characteristics. The abdominal aorta and inferior vena cava would classically be located on the same side of the spine [Figure 2]a.
Figure 2: The Abnormal Anatomic Features seen in situs ambiguus with (a) right isomerism (asplenia) and (b) left isomerism (polysplenia). Left lung (LL), right lung (RL).

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Heterotaxy syndrome with left isomerism

The classic left isomerism implies bilateral bilobed lungs, hyparterial bronchi and the main bronchus passes inferior to the ipsilateral MPA on each side. A centrally located liver (often smaller), stomach in an indeterminate position, multiple spleens and bilateral left atrial morphology are classically seen. There is, by definition, abnormal systemic venous drainage. Interruption of the inferior vena cava with azygos or hemiazygos continuation is the most consistent finding seen [Figure 2]b.

Determining the ventricular loop

During embryonic development, the heart is in the form of a linear tube that contains several segments that eventually give rise to the cardiac components. [8] Proceeding caudocephalad along this tube these segments include primitive atria, the left ventricular structure, the bulbus cordis which will eventually develop into the right ventricle and the truncus arteriosus. During the development, the tube bends over on itself [9],[10] and normally folds to the right, forming a dextro-loop (D-loop), with resultant positioning of the bulbus cordis to the right of the left ventricle. [11] The ventricular loop may tend left ward forming levo-loop (L-loop) [Figure 3].
Figure 3: The formation of a d-loop (left) and an l-loop (right) from the embryonic cardiac tube (center). Atria (A), aorta (Ao), bulbus cordis (BC), left ventricle (LV), pulmonary artery (PA), right ventricle (RV), truncus arteriosus (TA), embryonic left ventricle (V).

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Identification of right and left ventricles

The cardiac structures are identified on the basis of their morphological features. [12] In the right ventricle, the trabeculae are coarse, and the presence of an apical moderator band is characteristic. The trabeculae of the left ventricle are thin and delicate, and the septal surface is smooth. In addition, the papillary muscles of the right ventricle are attached to both the interventricular septum and the free wall, whereas the two papillary muscles of the left ventricle are attached only to the free wall.

In complex cases, it may be difficult to determine which ventricle is the morphologic right ventricle and which is the morphologic left ventricle. In such cases, the identification may be based on the assumption that in the presence of a right-sided aortic valve, the right ventricle is located to the right of the left ventricle (D-loop), and in the presence of a left-sided aortic valve, the right ventricle is located to the left of the left ventricle (L-loop). This is known as the loop rule. [6] L-looping is most frequently seen in association with transposition of the great arteries in the condition anatomically corrected transposition.

Cardiac connections

Once the cardiac chambers have been identified in morphological terms, it should be possible to state that vessel or chamber is connected to each other. Atrioventricular connections [13],[14] can be of five types : Concordant, discordant, ambiguous, double inlet, and absent right or left connection. The position of the atrioventricular valves is correlated with the orientation of the ventricular loop. [15] Mitral valve is associated with the morphologic left ventricle, and the tricuspid valve is associated with the morphologic right ventricle. [16],[17] Besides permanent truncus arteriosus, four types of ventriculoarterial connection may develop : Concordant connection (the pulmonary artery arises from the right ventricle, and the aorta arises from the left ventricle); discordant connection, which is synonymous with transposition of the great vessels (the pulmonary artery arises from the left ventricle, and the aorta arises from the right ventricle); double outlet right ventricle (the great vessels arise from the right ventricle); and double outlet left ventricle (the great vessels arise from the left ventricle). Several variants may be observed with regard to the positions of the great vessels. The vessels may be in normal position (solitus), inverted position (inversus), D-transposition or L-transposition, D-malposition or L-malposition [Figure 4].
Figure 4: The possible configurations of the great vessels such as situs solitus, situs inversus, d-transposition (solid arrow), l-transposition (arrowhead), double outlet right ventricle (dashed arrow). Anterior descending coronary artery (AD), anterior (Ant), aorta (Ao), aortic valve (AoV), bulbus cordis (BC), left ventricle (LV), malposition of the great arteries (MGA), pulmonary artery (PA), posterior (Post), pulmonary valve (PV), right (Rt), right ventricle (RV), transposition of the great vessels (TGA), embryonic left ventricle (V).

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At the cardiac level

Atrial diverticulae

Atrial diverticulae are thin outpouchings via a defect in the free wall of atria or in their appendages and do not contain all the layers of the atrial wall. They are frequently asymptomatic but can be associated with atrial tachyarrhythmias. Giant diverticula may cause thoracic pain and compressive symptoms. Rarely, progressive atrial dilation, thrombus formation and rupture of the diverticulae can also occur [18] [Figure 5].
Figure 5: (a) Computed tomography angiography and (b) volume rendered images showing enlarged right atrium with multiple septae (arrows) suggestive of atrial diverticulae. (c) Transesophageal echocardiography of the same patient showing membranous structures in right atrium. (d) Catheter angiography showing multiple sacculations and diverticulae in the right atrium. (e) Surgical specimen demonstrating excised diverticulae filled with saline. (f) Multiple diverticulae seen in the right atrium intraoperatively.

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Cor triatrium

Cor triatrium is a rare congenital abnormality seen in 0.4% of patients with CHD. [19] It is a condition in which the left (cor triatrium sinistrum) or the right atrium (cor triatrium dextrum) is divided into two compartments by a fold of tissue, a membrane or a fibromuscular band. The proximal portion of the corresponding atrium receives venous blood, and the distal portion is in contact with the atrioventricular valve. The clinical presentation depends on the size of the communicating orifice [Figure 6].
Figure 6: (a) Computed tomography angiography demonstrating drainage of pulmonary veins into separate partition made by a membrane in the left atrium. (b) Transesophageal echocardiography showing membrane in left atrium partitioning it to create accessory chamber for opening of pulmonary veins.

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Atrial septal defect

Atrial septal defects represent 10% of all CHD. Imaging features include enlargement of the right atrium and ventricle, dilatation of the pulmonary artery and its branches and an atrial septal defect depending upon the location. [20] There are four types of ASD : Ostium secundum, ostium primum, sinus venosus in the upper aspect of the atrial septum and coronary sinus type. Ostium secundum atrial septal defects are the most common type of inter atrial communication located within the oval fossa. Ostium primum septal defects are the next most common type. The least common type of interracial communication is a sinus venosus defect [Figure 7].
Figure 7: Computed tomography angiography (a) axial and (b) coronal reformatted images showing abnormal communication between the atria. The right atrium is enlarged and left atrium is small in size.

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Ventricular septal defect

Ventricular septal defects (VSDs) account for 20% of all CHD and 50% of VSD are associated with other congenital malformations. VSD is described as a lacuna of variable size in the interventricular septum. [21] VSD can be classified into four main categories according to their location and margin : Outlet, membranous, trabecular, and inlet. VSD are classified in terms of their appearance from the lumen of the right ventricle as perimembranous, muscular and doubly committed juxta arterial defects.

Tetralogy of Fallot (TOF) is the most common cause of cyanotic CHD. It occurs in 10% of cases of CHD. Imaging features includes infundibular stenosis, VSD, overriding of the aorta, right ventricular hypertrophy. [22] Antero-superior deviation of the infundibular septum is considered the developmental cause for subpulmonary infundibular stenosis and VSD in TOF. Major aortopulmonary collateral arteries (MAPCAs) are frequently the main sources of pulmonary flow in patients with TOF with pulmonary atresia. These may be seen in cases of pulmonary hypoplasia or stenosis. The vessels may originate from the ascending aorta, arch of the aorta or descending aorta and may course to the right or left to reinforce the narrowed pulmonary artery [Figure 8].
Figure 8: Computed tomography angiography (a) and (b) axial, (c) coronal and (d) oblique sagittal images showing absence of pulmonary trunk, ventricular septal defect, overriding of aorta. (e) coronal maximum intensity projection and (f) volume rendered images showing multiple large collaterals arising from the aortic arch and descending thoracic aorta on either side giving rise to the pulmonary vasculature. The main pulmonary artery, right and left pulmonary arteries are not visualised.

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Tricuspid atresia

Tricuspid Atresia has a prevalence of 0.3-3.7% of all congenital cardiac diseases. It represents the third most common form of cyanotic CHD. It results from the failure of formation of the tricuspid valve with direct communication between the right atrium and right ventricle. [23] Imaging features include fat deposition in the right atrioventricular groove, small right ventricle, large right atrium, and supracristal VSD [Figure 9].
Figure 9: Computed tomography angiography (a) and (b) axial and (c and d) coronal multiplanar reformatted images showing absence of pulmonary trunk and Blalock Taussig shunt between the right pulmonary artery and right carotid artery. The tricuspid valves are not visualized and the right ventricle is hypoplastic.

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Ebstein anomaly

Ebstein anomaly constitutes >1% of CHD and occurs in 1 in 210,000 live births. [24] It is defined as displacement of the attachment of the tricuspid valve leaflets from the atrioventricular junction to the right ventricular cavity with atrialization of the inlet of the right ventricle. The displacement mostly involves only the septal and posterior leaflets and is maximal at the commissure between these two leaflets. The non-displaced anterior leaflet is usually large and redundant, with variable mobility depending on the degree of tethering to the right ventricular wall [25] [Figure 10].
Figure 10: Computed tomography angiography (a) oblique sagittal and (b) axial images showing displacement of septal and posterior leaflets of tricuspid valve from the atrioventricular junction. (c) Volume rendered image showing dilated right atrium.

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At the level of great vessels

Truncus arteriosus

Truncus arteriosus is a CHD in which a single great artery leaves the base of the heart and gives rise to the coronary, pulmonary and systemic arteries. It is classified into four types.  [26] In type1, a short pulmonary trunk arises from the truncus arteriosus and gives rise to both pulmonary arteries. In type 2, each pulmonary artery arises separately from the posterior aspect of the truncus. In type 3, each pulmonary artery arises from the lateral aspect of the truncus. Type IV or pseudotruncus is a severe form of TOF rather than truncus arteriosus in which pulmonary atresia with VSD is seen [Figure 11].
Figure 11: Computed tomography angiography (a) coronal and (b, c and d) volume rendered images showing common origin of aorta and main pulmonary artery (MPA) from left ventricle. MPA continues as left pulmonary artery and right pulmonary artery arises from the PDA. Also noted is aberrant retro-esophageal right subclavian artery.

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Aortopulmonary window

Aortopulmonary window is a rare anomaly with a prevalence of about 0.1-0.2% among patients with CHD. It is caused by the failure of fusion of conotruncal ridges that are responsible for separating the truncus arteriosus into the aorta and pulmonary artery. The defect may be present anywhere from just above the semilunar valves to the more distal ascending aorta and MPA. [27] It is classified into three types : Type 1 or proximal, type 2 or distal and type 3 or total. When associated with interrupted aortic arch, the aortopulmonary windows are usually larger with greater distal extension [28] [Figure 12].
Figure 12: Computed tomography angiography (CTA) (a) coronal, (b) axial, (c and d) volume rendered images showing common confluent origin of aorta and left pulmonary artery. The arch is left sided and shows interruption after the origin of left subclavian artery (Type A). Post repair CTA (e) axial, (f) sagittal, (g and h) volume rendered images showing separate origin of aorta and main pulmonary artery. The continuation of arch with descending thoracic aorta is restored.

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Transposition of great arteries

Complete transposition of great arteries is characterized by ventriculoarterial discordance, in which left ventricle gives rise to MPA, and right ventricle gives rise to aorta.  [29] Atrioventricular discordance, when accompanied by ventriculoarterial discordance, the transposition is functionally corrected and is called congenitally corrected transposition of great arteries. It is rare and has a prevalence of 0.4-0.6% of all CHD [30] [Figure 13].
Figure 13: Computed tomography angiography (a) axial, (b) coronal and (c) volume rendered images after pericardial patch repair of pulmonary arteries and Blalock-Taussig shunt, showing communication of right atrium with left ventricle and left atrium with right ventricle. The aorta arises from the right ventricle and main pulmonary artery is atretic with focal dilatation of pulmonary arteries at the confluence.

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At the level of the aorta

Double aortic arch

Double aortic arch is caused by persistence of right and left IVth branchial arches. Anterior and posterior arches encircle the trachea and esophagus in a tight ring, joining distally to form a common descending aorta. It is the most common of the complete vascular rings, causing tracheoesophageal compression. [31] This anomaly may cause severe respiratory symptoms and dysphagia [Figure 14].
Figure 14: (a and b) Computed tomography angiography volume rendered images showing right and left aortic arches of same size forming a complete ring. (c) View from left thoracotomy showing trachea traversing between the crotch of the right and left arches.

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An aberrant right subclavian artery (SCA) originating from the normal left sided aortic arch is the most common aortic arch anomaly with an incidence of 0.5-2%. [31] This anomaly results from the interruption of the dorsal segment of the right arch between the right carotid artery and right SCA with regression of the right ductus arteriosus in the developing double aortic arch. [32] It arises as a last branch from the descending aorta and passes behind the trachea and esophagus, crossing the mediastinum from left to right [Figure 15].
Figure 15: (a and c) Computed tomography angiography volume rendered images showing aberrant origin of right subclavian artery with retro esophageal course (variant double aortic arch) and (b) dorsal scoliosis.

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Patent ductus arteriosus

Patent ductus arteriosus is defined as persistent patency of the ductus arteriosus beyond functional closure after birth. [33] It has a prevalence of 0.02% and 0.006%. Uncomplicated PDA connects the proximal descending aorta below the origin of the left subclavian artery with the roof of the MPA near the orifice of the left pulmonary artery (LPA) [Figure 16].
Figure 16: Computed tomography angiography (a) maximum intensity projection and (b) volume rendered oblique sagittal images showing patent ductus arteriosus.

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Hypoplastic ascending aorta and arch

Hypoplasia of the ascending aorta usually occurs in association with hypoplastic left heart syndrome. The proximal arch, distal arch and isthmus are said to be hypoplastic when their external diameters are <60%, 50% and 40% respectively. [34] Multidetector computed tomography (MDCT) angiography provides valuable information about the exact location, shape, and length of the hypoplastic segment, [35] as well as the course of collateral vessels and associated findings such as aortic coarctation [Figure 17].
Figure 17: Computed tomography angiography (a) and (b) axial (c) sagittal (d and e) volume rendered images and (f) view from mid sternotomy showing hypoplasia of ascending aorta and arch.

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Coarctation of the aorta

In classic coarctation, the narrowing is located just distal to the left SCA. [36] Coarctation at or immediately proximal to the left SCA is rare and compromises that vessel. An external indentation that involves all but the ventral portion of the coarctation corresponds internally to the ridge. The aorta just distal to the coarctation is typically dilated. Uniform narrowing of the aortic arch called as tubular hypoplasia is more frequently observed in neonates. A localized coarctation and tubular hypoplasia may coexist or may occur independently [Figure 18].
Figure 18: (a, b and c) Computed tomography angiography volume rendered, (d) sagittal, (e) echocardiography and (f) mid sternotomy view images showing long segment thinning of the descending aorta.

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Interrupted aortic arch

Interrupted aortic arch is an extremely rare condition representing <1.5% of CHD. [37] It is characterized by interruption between the ascending and descending aorta. The absence of structural connection between the ascending and descending aorta differentiates it from severe coarctation and aortic atresia. It is classified based on the site of interruption as; type A occurs distal to the left SCA, type B occurs between the left SCA and the left carotid artery [Figure 19] and type C occurs between the left carotid artery and the innominate artery [Figure 20]. Each of these three types is further subdivided as follows; subtype 1 with a normal SCA, subtype 2 with an aberrant SCA and subtype 3 with an isolated SCA that arises from the ipsilateral pulmonary artery via ductus arteriosus.
Figure 19: Computed tomography angiography. (a, c, d, e and f) Volume rendered and (b) maximum intensity projection images showing interruption of aortic arch (type B) and prominent ascending aorta between left common carotid and subclavian artery origin.

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Figure 20: (a and b) Computed tomography angiography volume rendered images showing interrupted aortic arch (type C) with hypertrophied intercostal arteries. (c) Echocardiography and (d) intraoperative images showing interruption of arch beyond the left subclavian artery.

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At the level of pulmonary artery

Pulmonary artery stenosis

Pulmonary artery stenosis refers to the constriction of MPA or of its branches. Patients with stenosis of the pulmonary arteries mostly have associated VSD, ASD, pulmonary valvular stenosis, PDA or other defects. Pulmonary artery stenosis with associated VSD is classified into three types. [38] Type A in which the native pulmonary arteries are present and are supplied by the PDA. Type B in which the pulmonary blood flow is provided by both native pulmonary arteries and by MAPCAs. Type C in which native pulmonary arteries are absent, and the blood supply is only through MAPCAs [Figure 21]a and [Figure 22]. In few cases of pulmonary atresia, there may be the origin of MPA from coronary arteries [Figure 23].
Figure 21: (a) Computed tomography angiography volume rendered and axial maximum intensity projection images showing right lung aplasia and right pulmonary artery atresia, left pulmonary artery coursing between the trachea and esophagus making a pulmonary artery sling leading to tracheal compression. (b) Intra operative image showing aplasia of right lung and inflated left lung.

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Figure 22: Computed tomography angiography (a) volume rendered, (b) axial and (c) coronal maximum intensity projection images showing stenosis of right pulmonary artery and left inferior pulmonary vein.

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Figure 23: Computed tomography angiography (a and b) volume rendered images in a patient with ventricular septal defect and pulmonary atresia showing origin of main pulmonary artery from left main coronary artery.

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Pulmonary artery sling with lung aplasia

It is a rare vascular developmental anomaly in which the LPA arises from the posterior aspect of the right pulmonary artery and passes between the trachea and esophagus to reach the left hilum forming a sling around the distal trachea and the proximal right main bronchus. [39] Those affected by pulmonary artery sling may be classified into one with a normal bronchial pattern and other with one or more malformations of the trachea-bronchial tree like stenosis of a long segment of the trachea or absence of the pars membranacea. These have high mortality and morbidity during infancy [Figure 21] and [Figure 24].
Figure 24: (a) Computed tomography angiography coronal and sagittal images postoperative patent lumen of repositioned trachea (behind the left pulmonary artery and surgical clips. (b and c) Intraoperative images showing pulmonary sling compressing trachea.

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Pulmonary arterio-venous malformation

The incidence of pulmonary arteriovenous malformations is 2-3/100,000 persons. [40] They are rare abnormalities resulting from abnormal communication between the pulmonary arteries and veins. The clinical presentation depends on the degree of right to left shunt. They are most often congenital, though they can also result from trauma. Approximately, 70% of PAVMs are associated with hereditary hemorrhagic telangiectasia [41] [Figure 25].
Figure 25: Computed tomography angiography topogram, axial, coronal and volume rendered images showing pulmonary arterio venous malformation right lung associated with abernethy type I malformation (shunt from Portal to left brachiocephalic vein).

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At the level of pulmonary veins

Congenital pulmonary vein stenosis

Congenital pulmonary vein stenosis may occur as a focal narrowing of one or more veins at the atrial junction or as a generalized narrowing of the lumen of the pulmonary veins for a considerable distance. Both pulmonary veins from one lung are mostly affected. It can result in generalized pulmonary venous hypertension. [42] The condition may deteriorate with the development of progressive pulmonary venous congestion, followed by pulmonary arterial hypertension and may eventually cause death [Figure 22].

Total anomalous pulmonary venous connection

Total anomalous pulmonary venous connection represents 1.5% of all CHD and occurs in 6.8/100,000 population.  [43] It is characterized by formation of confluence by the pulmonary veins from both lungs behind the left atrium and connection of a venous channel from this confluence to a systemic vein, the right atrium, or both. It can be described as supra-cardiac [Figure 26], cardiac [Figure 27], infra-cardiac [Figure 28] or mixed depending on the sites of connection. Supra-cardiac and cardiac types are rarely obstructive, but infra-cardiac types are almost always obstructive because of the passage of blood through the hepatic sinusoids.
Figure 26: Computed tomography angiography (a) axial maximum intensity projection and (b) volume rendered images demonstrating all the pulmonary veins forming a common channel and opening into coronary sinus/right atrium in a patient with situs inversus. (c) Echocardiography (subcostal view) showing a common chamber behind right atrium with all pulmonary veins draining into it in a patient with situs inversus. (d) Intraoperative image showing the pulmonary veins draining into the coronary sinus.

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Figure 27: Computed tomography angiography (a and b) volume rendered and (c and d) maximum intensity projection images showing common chamber formed superior to left atrium in which all pulmonary veins open. Also seen is pulmonary artery stenosis with resultant. Major aortopulmonary collateral arteries, patent ductus arteriosus and left superior venacava.

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Figure 28: Computed tomography angiography (a) coronal volume rendered and (b and c) maximum intensity projection images showing right and left pulmonary veins joining together to form a common venous trunk which courses infra diaphragmatically and drains into the main portal vein.

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Partial anomalous pulmonary venous connection

Partial anomalous pulmonary venous connection is a rare CHD with a prevalence of 0.4-0.7%. [44] It is twice as common from the right lung as from the left lung. The pulmonary veins from some portions of both lungs show an anomalous connection. The usual sites of connection are the superior venacava and the right atrium. An associated sinus venosus type of atrial septal defect may be seen in such cases. The connection of the right pulmonary vein to the inferior venacava is called "scimitar vein" because of its curved configuration [Figure 29] and [Figure 30].
Figure 29: Computed tomography angiography (a) coronal and (b) axial, (c) echocardiography and (d) intraoperative images demonstrating anomalous drainage of left superior pulmonary vein into left superior venacava.

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Figure 30: (a) Computed tomography angiography (CTA) axial image showing right inferior pulmonary vein draining into the right atrium. CTA (b) oblique coronal and (c) volume rendered images showing drainage of left superior pulmonary vein into left brachiocephalic vein.

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  Conclusion Top

This article highlights the MDCT appearance of CHD seen in regular clinical practice. MDCT is a fast, accurate and reproducible diagnostic modality for CHD, which correlates well with intra-operative findings. It is superior to echocardiography and catheter angiography in diagnosing extra cardiac, vascular anomalies and other organ system anomalies associated with the cardiac defects. The detailed anatomy sensitizes the clinician to intervene at the appropriate time and not wait for the clinical deterioration and unsalvageable damage. By providing accurate delineation and measurements of various structures, it is helpful in planning therapeutic interventions and prognostication of outcome.

  References Top

Bolisetty S, Daftary A, Ewald D, Knight B, Wheaton G. Congenital heart defects in Central Australia. Med J Aust 2004;180:614-7.  Back to cited text no. 1
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13], [Figure 14], [Figure 15], [Figure 16], [Figure 17], [Figure 18], [Figure 19], [Figure 20], [Figure 21], [Figure 22], [Figure 23], [Figure 24], [Figure 25], [Figure 26], [Figure 27], [Figure 28], [Figure 29], [Figure 30]


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