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Illustrated Imaging Essay On Congenital Heart Diseases Part3

The article discusses the multimodal imaging approach to diagnosing and managing cyanotic congenital heart diseases (CHD), focusing on the importance of radiographic analysis in categorizing these conditions based on pulmonary vascularity. It highlights specific diseases such as Tetralogy of Fallot and pulmonary atresia, detailing their imaging features and surgical management strategies. The need for a multidisciplinary team in the evaluation and treatment of complex CHD is emphasized, along with the roles of various imaging modalities like echocardiography, MRI, and CT.

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0% found this document useful (0 votes)
18 views11 pages

Illustrated Imaging Essay On Congenital Heart Diseases Part3

The article discusses the multimodal imaging approach to diagnosing and managing cyanotic congenital heart diseases (CHD), focusing on the importance of radiographic analysis in categorizing these conditions based on pulmonary vascularity. It highlights specific diseases such as Tetralogy of Fallot and pulmonary atresia, detailing their imaging features and surgical management strategies. The need for a multidisciplinary team in the evaluation and treatment of complex CHD is emphasized, along with the roles of various imaging modalities like echocardiography, MRI, and CT.

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Illustrated Imaging Essay on Congenital Heart Diseases: Multimodality


Approach Part III: Cyanotic Heart Diseases and Complex Congenital
Anomalies

Article in Journal of Clinical and Diagnostic Research · August 2016


DOI: 10.7860/JCDR/2016/21443.8210

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DOI: 10.7860/JCDR/2016/21443.8210
Review Article

Illustrated Imaging Essay on


Radiology Section

Congenital Heart Diseases:


Multimodality Approach Part III:
Cyanotic Heart Diseases and
Complex Congenital Anomalies
Venkatraman Bhat1, Vinay Belaval2, Karthik Gadabanahalli3, Vimal Raj4, Sejal Shah5

ABSTRACT
From the stand point of radiographic analysis most of the complex cyanotic congenital heart diseases (CHD), can be divided in to
those associated with decreased or increased pulmonary vascularity. Combination of a specific cardiac configuration and status of lung
vasculature in a clinical context allows plain film diagnosis to be predicted in some CHD. Correlation of the position of the cardiac apex
in relation to the visceral situs is an important information that can be obtained from the plain film. This information helps in gathering
information about the atrio-ventricular, ventricular arterial concordance or discordance. Categorization of the cyanotic heart disease
based on vascularity is presented below. Thorough understanding of cardiac anatomy by different imaging methods is essential in
understanding and interpreting complex cardiac disease. Basic anatomical details and background for interpretation are provided in the
previous parts of this presentation.

Keywords: Ebsteins anomaly, Echocardiography, Cardiac imaging, Pulmonary atresia,


Tetralogy, Transposition of great arteries, Truncus arteriosus

Complex Congenital Heart Disease Tetralogy of Fallot (TOF) [Table/Fig-2]: Tetralogy of Fallot (TOF)
Evaluation of complex congenital heart disease (CHD) demands [Table/Fig-3a&b,4] is the most common congenital cyanotic heart
obtaining detailed and specific information regarding cardiac disease, beyond the neonatal period, accounts for 6-10% [9].
chambers, connections, muscle contractility and quantification Predominant pathology of TOF is the outflow narrowing of the right
of the flow across valves, septal defects and collaterals. Plain ventricle and varying degree of the narrowing of pulmonary artery
radiography remains starting point and mainstay as a global and branches. Other components include ventricular septal defect
assessment tool in pre and postoperative evaluation of the patients. and overriding of aorta. Plain radiography typically shows upturned
Echocardiography initiates detailed investigative process, guided cardiac apex due to right ventricular hypertrophy. (Coeur en Sabot
by clinical questions. If additional information is needed [1-4], MRI cardiac configuration) Lungs show varying degree of oligemia
provides most precise information required for preoperative and reflecting the extent of pulmonary outflow obstruction. Prominent
post-operative evaluation for CHD. MDCT is a valid alternative with right aortic arch is noted in up to 25% of patients. Reconstitution of
some limitations (inadequate functional information, radiation dose a pulmonary vascularity is achieved by systemic collaterals and left
and contrast related complications), essentially providing structural to right shunting at the ductal level. The goal of surgical procedures
information necessary for surgical management. CT evaluation also in TOF is to increase the capacity of the right ventricular outflow by
provides details about pulmonary anomalies, structural information surgical correction and increase pulmonary flow. Palliation in the
regarding lungs, secondary complications and evaluation of form of systemic to pulmonary shunt is performed for increasing
tracheo-bronchial compression. CT also provides detailed mapping the capacity of pulmonary arteries in staged procedures.
of collateral vasculature, information about postoperative status of Imaging requirement is to demonstrate the extent and severity of
surgical procedures, grafts and bony changes in association with narrowing of pulmonary outflow and arteries, demonstration of
CHD [5-8]. Integrated multimodality approach is required for, the associated anomalies and showing extent of pulmonary atresia
management of the complex CHD, often decided in consultation or discontinuity. Measurement of right ventricular volume is very
with a multidisciplinary team consisting of paediatric cardiologist, critical for staged reconstruction of outflow. MRI is the procedure of
cardiac surgeon and an imaging specialist. choice for functional assessment although MDCT offers accurate
anatomical information in this regard [10]. Evaluation of coronary
Following commonly encountered entities are artery anomaly, exclusion of large conus branch in the region of
described in detail [Table/Fig-1] outflow and demonstration of anomalous right coronary from LAD
1. Tetralogy of Fallot (TOF) and variants, artery can be done by CT angiography [11]. Various measurable
2. Pulmonary Artesia- (pseudo-Truncus), indices and ratio are described in the following section to provide
quantitative a measure of size of pulmonary artery and branches.
3. Double Outlet Right Ventricle (DORV),
Quantitative information regarding size of pulmonary artery will help
4. Transposition of Great Arteries (TGA), in deciding required stages of corrective procedure. Pulmonary
5. Truncus Arteriosus (TA), atresia with VSD [Table/Fig-5a&b] is a variant of TOF, due to
6. Total Anomalous Pulmonary Venous Connections (TAPVC): complete atresia the pulmonary artery. Surgical procedures are
(a) Supracardiac; (b) Intracardiac; and (c) Infracardiac varieties. more complex in patients with large atretic segment. Role of MDCT
7. Ebsteins anomaly. is well established in pre-operative assessment [12]. Unusually

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Increased Pulmonary Vascularity Decreased Pulmonary Vascularity McGoon Ratio = diameters of distal RPA+ distal LPA (immediately
pre-branching portions)/ aortic diameter just above diaphragm
Total anomalous pulmonary venous Tetralogy of Fallot
connection (TAPVC) {normal ratio: 2.0-2.5}.
Truncus arteriosus Tricuspid atresia Nakata Index = cross sectional areas of RPA and LPA (in mm2)/
Transposition of great arteries Pulmonary atresia BSA {normal= 330 mm2/BSA}.
Single ventricle Pulmonary stenosis and atrial septic defect Details regarding variants of TOF, namely Pulmonary Atresia:
Double outlet right ventricle Ebstein anomaly
[Table/Fig-8a&b] and absent pulmonary valve are provided in
tables [Table/Fig-9].
[Table/Fig-1]: Cyanotic congenital heart disease [9].

TOF
Components VSD (usually perimembranous) RVOT obstruction (at infundibular, RVH Overriding of aorta
pulmonary valvular or combination)
Incidence [13] 1-3 cases per 1000 live births. Most common cyanotic CHD.
Arises from single gene defect involving TBX1 gene (TBX1
gene encodes for a transcriptional factor integral to
development of cardiac outflow tracts)
Association [13] Right aortic arch (25%). Abnormal coronary
arteries (5%) LAD arising from RCA with
prepulmonic course being most common.
Complete AVSD (2%) Persistent left SVC
Syndromes CHARGE syndrome Di George syndrome
Shprintzen (velo-cardio-facial) syndrome
Imaging features Plain Boot shaped heart (cor en Sabot)= concave Pulmonary oligemia Thymic atrophy Right aortic arch
MPA segment with an upturned apex
Imaging features specific Echo defines all components of TOF
MRI useful in post-surgical follow-up for RV volume assessment,
pulmonary regurgitation quantification and to rule out residual VSD
CT evaluation of pulmonary arteries, collaterals and coronary arteries
* McGoon Ratio and Nakata Index are used for quantification
of degree of PA hypoplasia
Management [13] Palliative procedures:
• Modified Blalock-Taussig shunt most preferred; placement of Gore-Tex interposition graft between subclavian artery and ipsilateral pulmonary
artery Definitive surgery: Total ICR (intracardiac repair) VSD patch closure + widening of RVOT (infundibular tissue resection) + pulmonary valvotomy
Note: Presence of coronary anomalies (esp. LAD from RCA with RVOT crossing) is a contraindication for primary repair.
[Table/Fig-2]: Tetralogy of Fallot.

[Table/Fig-3b]: A three-month-old female presented with cyanosis on feeding,


diagnosed as Tetralogy on echocardiography, confirmed on MDCT examination: (e)
[Table/Fig-3a]: TOF Severe outflow obstruction. axial CT images shows a large subaortic ventricular septal defect( arrow) and RVH; (f)
A three-month-old female presented with cyanosis on feeding, diagnosed as Tetralogy on Sagittal CT demonstrates large aorta(triangle) with normal branching pattern; (g) axial
echocardiography, confirmed on MDCT examination; (a) Diagrammatic representation of cardiac CT image at a slightly higher level shows severe narrowing of pulmonary infundibulum
anatomy in TOF; (b) Plain radiograph shows moderate cardiac enlargement with upturned apex
(RV configuration) (triangle) and decreased pulmonary arterial markings; (c) Echocardiography
and proximal pulmonary artery (arrow). Right and left pulmonary arteries are relatively
long axis view demonstrates a large malaligned perimembraneous VSD (arrow); (d) Colour Doppler small. Also, there are enlarged collateral arteries in chest wall (open arrow) (h) 3D
examination of RV outflow demonstrates narrowing of pulmonary artery with turbulent flow. reconstruction of aorta shows enlarged major aorto-pulmonary collateral arteries
(MAPCA) (arrows).
large systemic collateral arteries may be noted, occasionally
with bronchial impression [Table/Fig-6a-c]. In a patient with TOF Pseudotruncus: Pseudotruncus refers to severe form of TOF in
with absent pulmonary valves, there is associated pulmonary which pulmonary artery and their proximal branches are completely
regurgitation and severe dilatation of the branches of pulmonary atretic, disconnecting right ventricles from pulmonary artery [Table/
arteries. Dilated arteries can cause pressure effect on adjacent Fig-10]. Large collateral arteries from the aorta provide blood-
airways and cause secondary respiratory symptoms [Table/Fig-7]. flow to pulmonary circulation by reconstituting the more distal
Phase contrast MR techniques allow measurement of regurgitant pulmonary branches.
flow; constitute part of pre and postoperative assessment.
2 Journal of Clinical and Diagnostic Research. 2016 Jul, Vol-10(7): TE01-TE10
www.jcdr.net Venkatraman Bhat et al., Imaging Essay of Congenital Heart Disease

[Table/Fig-6a]: Pulmonary atresia with bronchial compression by collateral artery.


A three-year-old female presenting with exertional dyspnoea, diagnosed with a pulmonary atresia
on echocardiography: (a) Diagrammatic representation of vascular anatomy in Pulmonary atresia;
(b) Plain radiograph shows mild cardiac enlargement with upturned apex(arrow) (RV configuration)
and decreased pulmonary arterial markings.

[Table/Fig-4]: TOF Moderate outflow obstruction.


A 21-year-old female presented with palpitations, right ventricular outflow obstruction on
echocardiography diagnosed with PS; (a) Diagrammatic representation of cardiac anatomy in TOF
(b) Plain radiograph shows normal cardiac size and configuration. Pulmonary arterial markings are
decreased; (c) RVOT reconstructed CT; and (d) axial CT image at a slightly higher level shows
severe narrowing of pulmonary infundibulum, pulmonary valve and proximal PA (arrows). RPA and
LPA are relatively small; (e) Reconstructed CT view shows a large sub-aortic VSD (arrow) and large
overriding aorta. There is biventricular enlargement and hypertrophy.

[Table/Fig-6b]: A three-year-old female presenting with exertional dyspnoea,


diagnosed with a pulmonary atresia on echocardiography: (a) axial CT images shows a atretic main
pulmonary artery (star) and small right and left pulmonary arteries; (b,c) Reconstructed outflow CT
views shows overriding of aorta and large sub-aortic VSD(circles).

[Table/Fig-5a]: Pulmonary Atresia.


An eight-month-old male with cyanosis at birth, feeding difficulty; failure to demonstrate pulmonary
artery on echocardiography diagnosed with pulmonary atresia: (a) Diagrammatic representation of
cardio-vascular anatomy in pulmonary atresia; (b) Plain radiograph shows mild cardiac enlargement
with upturned apex (RV configuration) and decreased pulmonary arterial markings. Collateral vessel
around right hilar region are prominent (open arrow); (c) axial CT images shows a large sub-aortic
ventricular septal defect (arrow) and RVH; (d) Reconstructed outflow CT view shows overriding of [Table/Fig-6c]: A three-year-old female presenting with exertional dyspnoea, diagnosed with a
aorta and large sub-aortic VSD (arrow). pulmonary atresia on echocardiography: (a) 3D reconstruction of aorta and airways (red) shows
anatomy of pulmonary arteries, enlarged collateral arteries (MAPCA) (open arrows) impressing on
the airways (triangle); (b).

[Table/Fig-7]: TOF absent Pulmonary valves: A One-day-old female child presenting with
[Table/Fig-5b]: An eight-month-old male with cyanosis at birth, feeding difficulty respiratory distress, cyanosis, diagnosed with TOF with absent pulmonary valve on echo and
and failure to demonstrate pulmonary artery on echocardiography: Diagnosed CT: (a) Diagrammatic representation of vascular anatomy in TOF with absent pulmonary valve: (b)
Plain radiograph shows hyperinflated left lung (arrow) due to partial left bronchial compression; (c)
with pulmonary atresia: (e) Axial CT slightly at a lower level shows blind ending Coronal 3-D airway reconstruction demonstrates left bronchial compression(triangle); (d) Axial MIP
pulmonary infundibulum (angled arrow). Main pulmonary artery is absent. Right and CT image shows gross dilatation of pulmonary arteries with relative narrowing at valve level (arrow);
left pulmonary arteries reconstituted by collaterals (triangles); (f) 3D reconstruction of (e) CT coronal MIP view shows severe dilatation of RPA and LPA (triangles); (f) Axial CT images
aorta shows enlarged collateral arteries (arrow) (MAPCA). reveals subaortic VSD (circle).

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Subtypes PA-IVS (Pulmonary atresia with intact ventricular septum) PA-VSD (Pulmonary atresia with VSD)
PA-IVS (Hypoplastic right heart syndrome) Characterized by pulmonary atresia, hypoplastic RV and hypoplasia of tricuspid valve annulus
Incidence [13] 7.1-8.1 per 100,000 live births
Association Coronary artery anomalies e.g. RV dependent coronary circulation (coronary circulation perfused entirely by desaturated RV
blood with proximal coronary arteries), coronary ostial atresia ASD/PFO PDA.
Syndromes Di George syndrome
Imaging features Plain Normal or mild cardiomegaly Pulmonary oligemia Hyper translucent lung fields
Imaging features specific Echo thick, immobile atretic pulmonary valve; smallish hypertrophied RV; small tricuspid valve; ASD and PDA
Catheter angiography for coronary anomalies and hemodynamic assessment
MRI depicts all findings including tricuspid valve and RV size
CT preferred for coronary artery evaluation
Imaging is useful in classifying pulmonary atresia based on presence or absence of 3 portions (inlet/trabecular/infundibular) of RV
into tripartite, bipartite and monopartite types [13].
Management [13] Medical: Start PGE1 to maintain ductal patency
Surgical: depends on RV size and coronary artery anomalies
• Two-ventricular repair adequate RV size and RVOT necessary
• One and one-half ventricular repair in borderline RV size
• One ventricular repair (Fontan operation) in monopartite RV and/or RV dependent coronary circulation.
[Table/Fig-8a]: Pulmonary atresia PA-IVS.

Frequency [13] 2.5-3.4% of all CHD. M>F.


Association ASD/PFO Origin of aorta from RV
Syndromes Velo-cardio-facial syndrome DiGeorge syndrome
Imaging features Plain Normal or mild cardiomegaly (Boot shaped heart) Pulmonary oligemia Hyper translucent lung fields
Imaging features specific Echo mal-aligned VSD with overriding of aorta, pulmonary atresia MRI can depict all findings CT preferred for coronary artery anomalies and
collaterals
Management Medical: Start PGE1 to maintain ductal patency
Surgical:
• Central shunt surgery: direct connection of ascending aorta and hypoplastic MPA
• RV-to-PA connection
[Table/Fig-8b]: PA-VSD.

Components [13] TOF components + rudimentary nubbins of tissue or complete absence of pulmonary valvular tissue
Absent pulmonary valves, severe PR aneurismal dilatation of MPA and branch pulmonary arteries airway compression. Rabinovitch et al described
abnormal tufts of smaller pulmonary arteries that compress the intrapulmonary bronchi with reduction of the number of alveoli.
Prevalence Seen in ~3% of patients with TOF.
Association Frequent absence of ductus arteriosus, Increased nuchal fold thickness (NFT) in I trimester [14]
Syndromes Chromosomal abnormalities of chromosomes 6 and 7; deletion of chromosome 22q11 and DiGeorge syndrome
Imaging features Plain Cardiomegaly (moderate to Grossly dilated central PAs Decreased peripheral Tracheal compression Air trapping in lungs
marked) pulmonary vasculature
Imaging features specific Echo TOF findings+ dysplastic pulmonary annulus+ massive PR on doppler
Fetal MRI PA size+ symmetry of aeration of lungs secondary to airway compression and over-inflation + lung volume estimation [15,16]
Post-natal MRI PR quantification CT better evaluation of airway compression and secondary lung changes.
Management [7] Complete primary repair= VSD closure + pulmonary homograft placement to replace dysplastic pulmonary valve and dilated PAs
[Table/Fig-9]: TOF with absent pulmonary valve.

Double Outlet Right Ventricle (DORV) [Table/Fig-11]: DORV is


a cono-truncal defect characterized by origin of both great arteries
predominantly from RV [Table/Fig-12]. A ventricular septal defect
is invariably present; its location in relation to the semilunar valves
may be subaortic (50%), subpulmonary (30%), uncommitted, or
remote [17]. A large majority (66%) of patients with DORV have
some degree of pulmonary stenosis or atresia. DORV has a
complex spectrum of physiology based on the location of VSD
and flow of blood from ventricle to great arteries. Surgical planning
depends upon complexity of physiology, associated anomalies
[Table/Fig-13] and whether or not there is pulmonary arterial
hypertension [18]. CT imaging with 3D reconstruction of the
septum and ventricular outflow provides accurate characterization
of types of VSD with good correlation with surgical observation
and autopsy [19].
Transposition of Great arteries (TGA) [Table/Fig-14a&b]:
Transposition of great artery is the most common cyanotic heart
disease in the neonatal group. Depending on chamber-vessel
connection there are two entities; D-TGA and LTGA. In D TGA, [Table/Fig-10]: Pseudotruncus. A 10-year-male patient presenting with exertional dyspnoea,
investigated for cardiac disease diagnosed with severe pulmonary atresia: (a) Diagrammatic
there is ventricular-arterial discordance with anterior location representation patent of vascular anatomy in severe pulmonary atresia (pseudo-truncus); (b) axial
of the aorta, arising from the right ventricle [Table/Fig-15a&b]. CT images shows a large ascending aorta (arrow) and absent pulmonary artery. Large collateral
artery is seen arising from descending aorta (open arrow); (c) Reconstructed coronal CT view shows
Coronary arteries arise from aorta, from posterior cusps. Due to collateral arteries reconstituting pulmonary arteries.(triangles); (d) 3D reconstruction of aorta shows
atrio-ventricular concordance and ventriculo-arterial discordance, enlarged collateral arteries (open arrow) (MAPCA).

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www.jcdr.net Venkatraman Bhat et al., Imaging Essay of Congenital Heart Disease

DORV [13] Characterized by origin of both great arteries predominantly from RV, bilateral muscular infundibula and absence of AV-semilunar
valve continuity.
4 Subtypes (based on location DORV with subaortic VSD blood from LV flows via VSD to aorta and blood from RV flows mainly to PA= physiology similar to VSD
of VSD in relation to great arteries) DORV with subpulmonic VSD (Taussig-Bing syndrome) blood from LV flows via VSD to PA and blood from RV flows mainly
to aorta= physiology similar to TGA
DORV with doubly committed VSD absent infundibular septum
DORV with non-committed VSD, VSD remote from aortic and pulmonary valves
Incidence 1-3% of all CHD. M:F ratio=2:1
Association Coarctation of aorta
Interrupted aortic arch
Pulmonary obstruction
Syndromes Trisomy 13, 18
Deletion 22q11
Imaging features Plain Without pulmonary obstruction Moderate cardiomegaly (RV type), convex MPA, pulmonary plethora,
thymic atrophy and hyperinflated lungs
With pulmonary obstruction Mild cardiomegaly (RV type), concave MPA segment, pulmonary
oligemia and left arch
Imaging features specific Echo side by side orientation of great vessels with aorta anterior and to the right ; overriding of aorta; absent fibrous continuity of semilunar
and AV valves MRI very helpful for localization and relation of VSD to great arteries CT.
Management [13] Palliative:
• Pulmonary artery banding for uncontrollable CHF in infancy
• Balloon atrial septostomy for Taussig-Bing anomaly
• Systemic-to-PA shunting- in pts with associated PS
Definitive repair: Total correction= VSD closure with placement of internal and external conduits to establish physiological blood flow
between LV/aorta and RV/PA.
[Table/Fig-11]: DORV

[Table/Fig-12]: Double outlet right ventricle (DORV) with aberrant right subclavian [Table/Fig-13]: DORV, anomalons conus branch.
artery. One-year-old male presented with the cyanosis on feeding and feeding difficulty, suspected as TOF
One-year-old male referred to exclude CHD, diagnosed with a DORV in association with ARSA; on echocardiography , diagnosed with DORV: (a) Diagrammatic representation patient of vascular
(a) Diagrammatic representation of vascular anatomy in DORV, both great arteries arising from RV; anatomy in DORV; (b) Plain radiograph shows right ventricular cardiac configuration, oligemic
(b) Plain radiograph reveal cardiomegaly with oligemic lungs; (c) Sagittal oblique MIP views shows lungs and right aortic arch (arrow); (c) Outflow view shows both aorta and PA arising from RV, LV
dilated aortic arch with normal branching. There is a small PDA(triangle); (d)Axial MIP images shows communicating with a large VSD(arrow); (d) Axial CTA reveals normal size pulmonary arteries and
infundibular and valvular stenosis(arrows); (e) Outflow view shows both aorta and PA arising from right aortic arch (arrow) (e) Axial CT at aortic root show conus branch crossing RTOT(open arrow);
RV, LV communicating with a large VSD(star); (f) CT Coronal MIP view shows ARSA( open arrow) (f) Coronal CT reconstruction shows tight infundibular stenosis (open arrow).
and narrowing of origin of LPA (arrow).

Imaging by MDCT and /or MRI is helpful in the demonstrating


systemic venous blood passes through the right heart to the aorta a post-procedural complications like stenosis at various sites
and pulmonary venous blood passes through the left heart to the of anastomosis [4,9]. In L-TGA there is a discordant atrio-
lungs. Survival is dependent on the existence of a communication ventricular and ventricular arterial connection. Some patients may
between pulmonary and systemic circulations (patent ductus be asymptomatic or present late. Treatment involves the double
arteriosus, ventricular septal defect, or atrial septal defect) [17]. switch procedure (Senning of mustard) or Rastelli's repair [9].
Plain radiography in appropriate clinical setting, often provide clue Truncus arteriosus (TA) [Table/Fig-16]: Truncus arteriosus is an
to the diagnosis. Mild biventricular cardiomegaly (egg on side) uncommon cono-truncal anomaly characterized by a single arterial
with a narrow cardiac pedicle in a cyanotic neonate suggests the vessel that originates from the heart, overrides the ventricular
diagnosis of transposition of great arteries. Lungs in most case septum, and supplies the all the three, systemic, pulmonary,
show increased arterial flow pattern. The immediate postnatal and coronary circulation. There is well known association with
palliative procedure required in patients with D-TGA is to create DiGeorge syndrome and chromosome 22q11 deletion [20]. The
an atrial septal defect. Total repair of D-TGA involves performing classification of truncus arteriosus is made on branching pattern of
the rerouting the systemic return to left atrium through a channel the pulmonary artery, described originally by Collett and Edwards
(Senning procedure) and directing pulmonary return to the right [21] modified subsequently by Van Praagh [22]. Echocardiography
atrium. In Rastelli procedure transventricular rerouting of the is adequate for diagnosis and surgical planning in most of patients.
left ventricular outflow is performed. Pulmonary trunk is divided MRI or MDCT may be needed for delineation of branching pattern
and a conduit is placed within right ventricle and the pulmonary of pulmonary arteries, demonstration of aortopulmonary collateral
trunk. Jantane procedure is a 3-stage arterial switch procedure. vessels, pulmonary venous abnormalities and configuration of
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Subtypes Complete TGA (D-TGA) Congenitally corrected TGA (L-TGA)


D-TGA (Dextro-TGA)= aorta anterior and to the right of pulmonary artery, Complete separation of systemic and pulmonary circulations AV concordance and VA discordance
(Aorta arising from RV and PA arising from LV)
Incidence [13] 1 in 4000 live births. M: F=2:1 to 3:1. Increased risk in infants of diabetic mothers.
Association VSD/ASD/PFO= necessary for survival LVOT obstruction (dynamic or fixed) coexisting with VSD Pulmonary valve stenosis.
Syndromes Laurence-Moon-Biedl syndrome.
Imaging features Plain “Egg on a string” sign combination of abnormal great vessel anatomy + apparent narrowing of superior mediastinum by hyperinflated
lungs and stress-induced thymic atrophy
Imaging features specific Echo Direct demonstration MRI useful for post-surgical follow-up for assessment of degree of systemic RV failure and tricuspid
regurgitation, to look for intraatrial baffle leak or obstruction and to rule out obstruction of systemic/pulmonary venous pathways
CT
Management [13] Palliative:
1. Rashkind procedure: balloon atrial septostomy
Definitive repair:
• Atrial baffle operations: Mustard and Senning
• Arterial switch surgery (Jatene)- procedure of choice
• Rastelli operation: Intraventricular baffle to close VSD and redirect L outflow tract to aorta (useful in presence of both VSD
and pulmonary stenosis)
[Table/Fig-14a]: D-TGA.

L-TGA (Levo-TGA)= aorta anterior and to the left of pulmonary artery AV and VA discordance (Aorta arising from right sided LV and PA arising from left sided RV)
Incidence [13] 1 in 13,000 live births. M>F.
Association VSD, pulmonary stenosis (valvular or subvalvular), abnormalities of systemic AV (tricuspid) valve and conduction system anomalies
Syndromes Laurence-Moon-Biedl syndrome
Imaging features Plain Cardiomegaly with straight Pulmonary Plethora (in TGA + VSD) LA enlargement and pulmonary venous
left upper heart border congestion (TGA + severe left AV valve
regurgitation)
Imaging features specific Echo Direct demonstration MRI and CT for demonstration of cardiac/extracardiac anomalies
Management [12] Palliative:
• Modified BT shunt (in pts with severe PS)
• Pulmonary artery banding for uncontrollable CHF in infancy
Definitive repair:
• Classic repair: leaves the anatomical RV as systemic ventricle
• Anatomic repair: makes the anatomical LV as systemic ventricle; less risk of post-op TR and RV failure
[Table/Fig-14b]: L-TGA.

[Table/Fig-15b]: A two-month-old male cyanotic child, suspected on echocardiography to have


TGA, large VSD PS, diagnosed with transposition of great arteries, VSD and PS; (e) Lateral MIP CT
[Table/Fig-15a]: TGA. A two-month-old male cyanotic child, suspected on echocardiography image demonstrates anterior location of aorta (arrow). There is a narrowing of the pulmonary arterial
to have TGA, large VSD PS, diagnosed with transposition of great arteries, VSD and PS: (a) origin (open arrow) (f). Modified oblique view shows large ventricular septal defect, anteriorly located
diagrammatic illustration of TGA with narrowing of the pulmonary outflow; (b) Plain radiography aorta (arrow) and posterior pulmonary artery with narrowing at the origin. (triangle).
demonstrate mild biventricular cardiac enlargement. Lung fields are unremarkable; (c) Axial MIP
image showing anteriorly located large aorta (arrow) and posteriorly located pulmonary artery (and
open arrows); (d) CT axial image demonstrates biventricular enlargement with large ventricular characteristically shows signs of severe pulmonary oedema or
septal defect (circle).
congestion with normal cardiac size in obstructed TAPVC [23]. In
aortic arch [Table/Fig-17a&b]. MR imaging is most optimal in supracardiac TAPVC shows cardiomegaly with wide mediastinum
evaluating in the assessment of postoperative complications [17]. and plethoric lungs. (Snow man or Figure of ‘8’ heart) Imaging
by CT or MRI plays a greater role in imaging these anomalies
Total anomalous pulmonary venous connection [Table/Fig-21a&b]. Even non-gated MDCT examinations provide
(TAPVC): accurate delineation of Anatomy, much superior to trans-
Supracardiac [Table/Fig-18] Intracardiac [Table/Fig-19] and infra­ esophageal echography [24] Prompt surgical relief of obstruction
cardaic types [Table/Fig-20]. These anomalies involve drainage is required on an urgent basis [9]. Corrective procedure involves
of the pulmonary veins to the systemic circulation. Intracardiac the reconnection of the pulmonary vein to the left ventricle.
TAPVC is associated with atrial isomerism (polysplenia or asplenia). Prognosis is unfavorable for infra diaphragmatic TAPVC. Examples
Anomalous pulmonary venous drainage can be associated with of intracardiac [Table/Fig-22a&b] and Infracardiac TAPVC [Table/
obstruction, especially infracardiac variety. Plain radiography Fig-23a,b] are illustrated in the images.

6 Journal of Clinical and Diagnostic Research. 2016 Jul, Vol-10(7): TE01-TE10


www.jcdr.net Venkatraman Bhat et al., Imaging Essay of Congenital Heart Disease

Subtypes: Collett and


Edwards classification [18]

Type I = pulmonary trunk arises from proximal truncus


Type II and III= no pulmonary trunk; branch PA arise from posterior and lateral mid-segments of truncus
Type IV=pulmonary circulation dependent on MAPCAs.
Incidence 1% of CHD. 1 in 11,000 live births(10--5)
Association [13] Right aortic arch, interrupted aortic arch, coarctation, PDA Coronary artery anomalies(stenotic coronary ostia, abnormal
branching and course) Unilateral absence of pulmonary artery
Syndromes DiGeorge syndrome Pierre Robin syndrome
Imaging features Plain Moderate cardiomegaly High cephalic origin Right aortic arch Depressed diaphragm Thymic atrophy
with narrow base of PA
Imaging features specific Echo large truncus, large VSD and a large echogenic truncal valve with fibrous continuity with anterior mitral leaflet
MRI -similar findings
Management Primary repair: done in first week of life; consists of VSD closure and placement of valve conduit between RV and PA
(Rastelli procedure)
[Table/Fig-16]: Truncus arteriosus.

Subtypes supracardiac cardiac infracardiac mixed


Supracardiac Pathway of pulmonary venous return:
Tapvc Common pulmonary vein left vertical vein left innominate
vein right SVC right atrium
Incidence [13] Accounts for 50% of TAPVC patients.
Association ASD/PFO Heterotaxy syndrome
Syndromes
Imaging “snowman sign or figure-of-8 configuration” Pulmonary
features = dilated vertical vein on the left, the innominate plethora
Plain vein on the top, and the superior vena cava on the
right form the head of the snowman; the body of the
snowman is formed by the enlarged right atrium.
Imaging Echo demonstrates the anomalous pulmonary
features venous drainage MRI best for hemodynamic
specific assessment CT
Management Surgical rerouting- by large side-to-side anastomosis
[8,9,13] between common pulmonary venous pathway and LA
with ligation of vertical vein and closure of ASD
[Table/Fig-18]: Supracardiac tapvc.
[Table/Fig-17a]: Truncus arteriosus. A three-month-old male with breathing difficulty, recurrent
chest infection, diagnosed with truncus arteriosus: (a) Diagrammatic representation of vascular
anatomy in truncus arteriosus; (b) Plain radiograph shows moderate cardiac enlargement with cardiac tapvc Pathway of pulmonary venous return:
increased pulmonary arterial markings, especially on right side(open arrow); (c) echocardiography • Common pulmonary venous channel coronary sinus RA.
shows a single trunk(open arrow) providing origin to pulmonary artery and aorta; (d-f) Reconstructed
• All 4 pulmonary veins drain separately into RA.
outflow CT images shows a single large ascending aorta overriding large septal defect(circle); (e)
Reconstructed axial oblique CT view shows pulmonary arteries arising from posterior aspect of Incidence [13] Accounts for 20% of TAPVC patients.
aorta (triangle).
Association ASD/PFO
Syndromes
Imaging Moderate cardiomegaly Pulmonary
features Plain (RA/RV enlargement) plethora
Imaging features Echo dilated coronary sinus- first clue to this condition
specific MRI adequately demonstrates the anomalous pathway
with functional assessment (Qp:Qs) CT
Management [13] • TAPVC to RA: excision of atrial septum and
redirection of pulmonary venous return to LA
through a patch
• TAPVC to coronary sinus: Unroofing of coronary
sinus (incision in anterior wall of coronary sinus for
communication between CS and LA) with closure
of ostium of CS and ASD
[Table/Fig-19]: Cardiac tapvc.

[Table/Fig-17b]: A three -month-old male with breathing difficulty, recurrent chest infection, 25]. Typically there is caudal displacement, dysplasia of septal and
diagnosed with truncus arteriosus; (a) Axial CT view shows configuration of aortic arch (arrow) and posterior leaflets of tricuspid valve, dilatation of the right atrium
origin of pulmonary artery(arrow); (b) 3 D reconstruction showing larger aorta (arrow) and RPA and
LPA arising posteriorly (open arrows). Also, there is an aberrant left subclavian artery (triangle). and atrialized portion of the RV, which may pulsate paradoxically
in ventricular systole [9]. Associated cardiac anomalies and
Ebstein’s Anomaly [Table/Fig-24]: On plain radiography conduction defects can be observed. MR imaging is superior in
Ebstein’s anomaly has the classic appearance of 'box shaped demonstrating morphological features, chamber contractility and
heart'. Large heart size is common in Ebstein’s anomaly due to measuring regurgitant tricuspid flow, which has prognostic value
massively enlarged right atrium, which can also cause a posterior [4] Management of Ebstein’s anomaly depends upon the age of
bulge in lateral chest radiograph [25]. Adequate evaluation can patient, severity of the malformation, degree of right ventricular
be performed by echocardiography and MR imaging [Table/Fig- outflow tract obstruction and dynamic status of pulmonary vascular

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Venkatraman Bhat et al., Imaging Essay of Congenital Heart Disease www.jcdr.net

Infracardiac Tapvc Pathway of pulmonary venous return: Common pulmonary venous channel drains into portal vein, ductus venosus, hepatic vein or IVC
by crossing the diaphragm through esophageal hiatus
Incidence [13] Accounts for 20% of TAPVC patients. Marked male preponderance with M:F ratio of 4:1. Most of infracardiac TAPVC are obstructed.
Hence it is considered as one of the paediatric cardiac emergency.
Association ASD/PFO
Syndromes
Imaging features Plain Normal or mild cardiomegaly Pulmonary oedema features (diffuse reticular pattern and Kerley B lines)
Imaging features specific Echo - dilated descending vein passing through diaphragm and draining into systemic veins. MRI best suited for post-op follow-up
CT useful in features of obstruction on ECHO with unclear site of obstruction
Management [13] Surgical: Large vertical anastomosis between common pulmonary venous channel and LA with ligation of common channel above the
diaphragm
[Table/Fig-20]: Infracardiac tapvc.

[Table/Fig-21a]: Supracardiac TAPVC. A three-month-old female presenting with cyanosis


while feeding, abnormal vessel on echography, diagnosed with TAPVC: (a) diagrammatic
representation of confluence of pulmonary veins into a vertical vein, draining subsequently in to
[Table/Fig-22a]: Intra-cardiac TAPVC. A two-month-old female presenting with cyanosis
left innominate vein; (b) Plain radiography demonstrating biventricular cardiomegaly and plethoric
while crying, shown to have a septal defect on echocardiography diagnosed with intra-cardiac
lung fields. Widening of the mediastinum is noted on left side (arrow); (c,d) Grey scale and colour
total anomalous pulmonary venous drainage: (a) Diagrammatic illustration showing a confluence
Doppler echocardiography demonstrate ascending vertical vein( triangle and arrows) Both atria
of pulmonary artery draining into the enlarged coronary sinus; (b) Echocardiography shows dilated
are enlarged; (e) MIP image in coronal plane of contrast-enhanced CT demonstrates confluence
coronary sinus draining in to enlarged right atrium (arrow); (c) Colour Doppler exam shows enhanced
of all pulmonary veins into vertical vein and innominate vein. (Triangles); (f) Coronal CT MIP image
flow in to right atrium via coronary sinus; (d) Axial CT MIP image shows confluence of pulmonary
demonstrates pulmonary plethora, dilated MPA and vena-cavae (triangles).
veins and dilated coronary sinus, draining into right atrium (open arrows).

[Table/Fig-21b]: Supracardiac TAPVC. A three-month-old female presenting with cyanosis


while feeding, abnormal vessel on echography, diagnosed with TAPVC (g, h) 3-D CT reconstructed
images viewed from posterior aspect demonstrates anomalous drainage of all the pulmonary veins
into vertical vein (arrow).

resistance. The surgical approach depending on the exact context


involve ligation of a patent ductus arteriosus, placement of a [Table/Fig-22b]: A two-month-old female presenting with cyanosis while crying, shown to have
a septal defect on echocardiography diagnosed with intra-cardiac total anomalous pulmonary
systemic to pulmonary shunt, tricuspid valve repair or reducing venous drainage: (a) axial CT images the level of pulmonary artery revealed grossly dilated MPA
(open arrow); (b) axial CT image demonstrates grossly enlarged right atrium with a large secundum
flow across tricuspid valve [26]. septal defect (open arrow). There is enlargement of the both ventricular cavities; (c) Coronal CT
MIP image shows confluence of pulmonary veins (arrows); and (d) increased pulmonary arterial
There are few additional cardiac anomalies which have not been vascularity.
illustrated in the presentation. Notable entities include a tricuspid
atresia, Cor tri-atriatum, severe isolated valvular atresia and Conclusion
hypoplastic ventricles. Evaluation of these entities is done with In this concluding session on imaging of CHD, an approach to the
the same guidelines utilising combination of echocardiography, management of cyanotic and complex congenital heart disease
plain radiography, MRI, CT and cardiac catheterization. Specific is provided. Multimodality approach with use of more than one
imaging appearances are well described [9,13,28]. imaging modality, including invasive intravascular studies are often
8 Journal of Clinical and Diagnostic Research. 2016 Jul, Vol-10(7): TE01-TE10
www.jcdr.net Venkatraman Bhat et al., Imaging Essay of Congenital Heart Disease

[Table/Fig-23a]: Infra-cardiac TAPVC. 3-day-old female neonate presenting with respiratory [Table/Fig-25]: Ebsteins Anomaly. A 17-year-old male presenting with cyanosis on diagnosed
distress, diagnosed with infra-diaphragmatic TAPVC to portal vein on MDCT. (a) Diagrammatic with Ebsteins anomaly on Echocardiography and MR: (a) Diagrammatic representation of
representation of vascular anatomy in infra-cardiac TAPVC. Anomalous common pulmonary cardiac anatomy in Ebsteins anomaly; (b) Plain radiograph shows enlarged cardiac shadow, RA
vein (CPV) is shown draining to portal vein (colored red); (b) Plain radiograph shows obscured enlargement (arrow) and pulmonary oligmia; (c) Echocardiography demonstrating enlarged RA with
cardiac shadow with evidence of pulmonary oedema; (c)Coronal MIP CT reconstruction show apical displacement of septal tricuspid leaflet (arrow); (d) Axial bright blood MRI examination shows
anomalous CPV traversing the diaphragm and joining portal vein (arrow). Minimal narrowing noted dilated RA displaced dysplastic tricuspid valves (open arrow); (e) Sagittal images shows large RA
at diaphragmatic level (triangle); (d,e) Axial CT images shows confluence of pulmonary veins (star) cavity and RV outflow tract(circle); (f) Coronal images illustrates displaced tricuspid valve (arrow).
and site of union with portal vein (open arrow).

patients on palliation have triggered advanced techniques in MR


and CT imaging. This presentation highlights imaging appearances
of commonly occurring cyanotic and complex CHD like Tetralogy
and variants ,TGA and other less common complex conditions in
the form of illustrated case studies.

Acknowledgements
Authors would like to acknowledge the contribution of all radiology
colleagues for their contribution to this work. Special thanks to
Dr. Suresh P.V, Dr. Kiran V.S, Dr Arul Narayanan and Dr. Shreesha
Maiyya from the Department of Cardiology for their extensive
clinical input. Pivotal to all activity, authors would like to thank
the clinical and administrative support of Dr. Devi Prasad Shetty
and team for making this work a possibility. Additionally authors
thank Philips Inc. for the workstation, intellispace portal which was
extensively used in the processing of volumetric CT data.

[Table/Fig-23b]: A three-day-old female neonate presenting with respiratory distress,


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PARTICULARS OF CONTRIBUTORS:
1. Director and Head, Department of Radiology and Imaging Services, Narayana Health, Narayana Hrudayalaya,
Multispeciality Hospital-Shaw Mazumdar Medical Centre, Bengaluru, Karnataka, India.
2. Junior Consultant, Department of Radiology and Imaging Services, Narayana Health, Narayana Hrudayalaya,
Multispeciality Hospital-Shaw Mazumdar Medical Centre, Bengaluru, Karnataka, India.
3. Consultant, Department of Radiology and Imaging Services, Narayana Health, Narayana Hrudayalaya,
Multispeciality Hospital-Shaw Mazumdar Medical Centre, Bengaluru, Karnataka, India.
4. Consultant, Department of Radiology and Imaging Services, Narayana Health, Narayana Hrudayalaya,
Multispeciality Hospital-Shaw Mazumdar Medical Centre, Bengaluru, Karnataka, India.
5. Senior Consultant, Department of Pediatric Cardiology, Narayana Health,
Narayana Hrudayalaya, Bengaluru, Karnataka, India.

NAME, ADDRESS, E-MAIL ID OF THE CORRESPONDING AUTHOR:


Dr. Venkatraman Bhat,
309, Greenwoods Apt, Royal Gardenia, Bommasandra, Bangalore-560099, Karnataka, India. Date of Submission: Sep 12, 2015
E-mail: [email protected] Date of Peer Review: Nov 29, 2015
Date of Acceptance: Apr 02, 2016
Financial OR OTHER COMPETING INTERESTS: None. Date of Publishing: Jul 01, 2016

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