CARDIAC IMAGING 2 Flashcards
Are ASD’s more common in males or females?
Female preponderance
What is the Trilogy of Fallot?
- PA stenosis
- Right ventricular hypertrophy
- patent foramen ovale
The “trilogy,” consisting of pulmonary stenosis with intact ventricular septum, right ventricular hypertrophy, and a defect in the atrial septum, was first described by Fallot.1 In his series of seven patients, the stenosis was valvular in six and infundibular in one. All seven had a patent foramen ovale, and in one a second defect of the atrial septum was present.
Although the trilogy of Fallot is considered to be an infrequent lesion, growing experience with surgery for congenital heart disease has shown it to be commoner than previously though
In Tricuspid atresia, how does the size of the associated ASD impact flow dynamics and xray findings?
- tricuspid atresia with a large atrial septal defect
- little or no cardiomegaly
- tricuspid atresia with a restrictive (ie small) atrial septal defect
- substantial right atrial enlargement
- which results in cardiomegaly.
- Tricuspid atresia can be associated with increase in pulmonary blood flow,
- secondary to
- large left-to-right shunt at the ventricular septal level or
- the concurrence of transposition of the great vessels.
- secondary to
Aims of CT when ix Coarctation
- Allows precise size and length estimation,
- detection of collaterals,
- postoperative complications and
- stent graft patency postintervention
What is the treatment of Ebsteins anomaly?
Treatment
- ECMO for temporization
- Tricuspid valve reconstruction
- Cardiac pacemaker for arrhythmias
- Bidirectional Glenn shunt (SVC–PA to increase pulmonary blood flow)
what is the treatment of Endocardial cushion defects?
Treatment
•
Primary surgical repair before age 2
What are the clinical findings of TOF?
Clinical Findings
- Squatting when fatigued (to increase pulmonary flow and thus O 2 saturation)
- Episodic loss of consciousness
- Cyanosis by 3–4 months; time of presentation depends on degree of RV outflow obstruction.
What are the Radiographic features of VSDs?
Small?
Significant shunt?
Eisenmegner?
Radiographic Features
Because of the variation in size of VSD, the radiographic features are variable and may range from normal cardiac size and pulmonary vessels to large RV, LV, and LA.
CXR findings
- Small VSD:
- normal CXR
- Significant shunt (Q pulm /Q aorta >2):
- enlargement of heart, PAs, and LA
- Eisenmenger physiology
- Enlarged PAs
- Cardiac and LA enlargement may decrease RVH
- Peripheral PAs become constricted (“pruning”)
- Calcified PA (rare)
Plain radiograph
The chest radiograph can be normal with a small VSD. Larger VSDs may show cardiomegaly (particularly left atrial enlargement although the right and left ventricle can also be enlarged). A large VSD may also show features of pulmonary arterial hypertension, pulmonary oedema, pleural effusion, and increased pulmonary vascular markings.
what type of CHD is Valvular Pulmonary Stenosis?
re vascularity and cyanosis
Acyanotic CHD With Normal Pulmonary Vascularity
*
What 3 other defects are AP window defects associated with?
Associations
- PDA, 10%–15%
- VSD
- Co-Arctation
What is the rule of thumb to predict cyanosis by xray?
Cyanosis: there are no definitive signs to predict cyanosis by plain radiograph however, as a rule of thumb, cyanotic patients (right-to-left shunt) have small PAs, and the main PA segment may not be visible or is concave.
What is the underlying mechanism of
Acyanotic CHD With Normal Pulmonary Vascularity
Acyanotic CHD With Normal Pulmonary Vascularity
- Normal pulmonary vascularity is associated with either:
- outflow obstruction
- valvular insufficiency before onset of congestive heart failure (CHF):
-
Outflow obstruction
- Coarctation of aorta
- Interruption of aortic arch (IAA)
- AS
- Pulmonic stenosis
- Valvular insufficiency (rarely congenital)
- Corrected transposition of great arteries (L-TGA) (isolated)
Top 6 CONGENITAL HEART DISEASE conditions presenting in the
First Month of Life
(Serious Clinical Problems, High Mortality)
First Month of Life (Serious Clinical Problems, High Mortality)
- Hypoplastic left heart 35%
- Transposition of great arteries 25%
- Coarctation 20%
- Multiple serious defects 15%
- Pulmonary atresia/stenosis 10%
- Severe tetralogy of Fallot 10%
What is the underlying mechanism of Cyanotic CHD With Decreased Pulmonary Vascularity?
Cyanotic CHD With Decreased Pulmonary Vascularity
- Decreased pulmonary vascularity because of obstruction of pulmonary flow.
- In addition, there is a R-L shunt because of an intracardiac defect.
- Normal heart size
- Tetralogy of Fallot
- Fallot variants
- Tricuspid atresia
- Increased heart size
- Ebstein anomaly
- Pulmonary stenosis with ASD
- Pulmonary Atresia
- Normal heart size
What are the clinical findings of pulmonary stenosis?
whate is an associoated syndrome?
3 characteristics of this syndrome
Clinical Findings
Most patients are asymptomatic.
Dysplastic type may show familial inheritance; also associated with Noonan syndrome (short stature, webbed neck, hypogonadism).
What are the characteristics of Ebstein Anomaly?
Ebstein Anomaly ( Fig. 2.40 )
Deformity of the tricuspid valve with distal displacement of the tricuspid leaflets into the RV inflow tract; results in atrialization of superior RV.
Patent foramen ovale or ASD nearly always present, 80%
Aim of CXR assessment in Congenital heart disease
Use of Imaging Modalities for Evaluation of CHD in Pediatric and Adult Populations
Chest Radiograph
- Determine to which of the four categories a CHD belongs (based on pulmonary vascularity and cardiac contour abnormalities).
- Abdomen: determine situs
- Bones: certain CHDs are associated with osseous abnormalities such as 11 ribs or hypersegmented sternum (Down syndrome)
What differentiates an AP window septal defect from Truncus Arteriosus?
- Differentiation from TA:
- Two semilunar valves are present.
- No VSD
What are treatment options for Tricuspid atresia?
- Treatment
- Palliative
- Maintain patent PDA with prostaglandins.
- Blalock–Taussig
- Glenn anastomosis (SVC → PA)
- Definitive (older patients)
- Fontan procedure: RA is connected to main PA ( Fig. 2.44 ).
- Palliative
when does the PDA close?
PDA closes functionally 48 hours after delivery.
PDA closes anatomically after 4 weeks.
3 x ddx of Congenital heart disease with Increased incidence in females?
Increased incidence in females:
- ASD
- PDA
- Ebstein anomaly
what are the haemodynamics of Valvular Pulmonary Stenosis
Hemodynamics
• Obstruction of RV outflow
What are the clinical findings of Ebsteins Anomaly?
What is the 1st year mortality rate?
Clinical Findings
- Tricuspid regurgitation and/or obstruction
- Arrhythmias (RBBB, WPW)
- 50% mortality in first year
What are the haemodynamics of pre and post ductal coarctation?
- Preductal type has concomitant R–L shunting via PDA or VSD.
- Postductal coarctation has L–R flow through PDA.
- Collaterals to descending aorta
- Internal mammary to intercostals
- Periscapular arteries to intercostals
Which Chambers/arteries/vessles are enlarged/changed in Ebsteins’ Anomaly?
8 Ddx of CHD With Pulmonary venous hypertension/Congestive Heart Failure
CHD With PVH/CHF
- Cor triatriatum
- Mitral stenosis
- Hypoplastic left heart syndrome (HLHS)
- Coarctation of the aorta
- Cardiomyopathy
- Primary endocardial fibroelastosis
- Anomalous ICA
- Obstructed TAPVC
What is the incidence of Congenital heart disease?
Overall incidence of all anomalies is approximately 1% of newborns.
The most common structural defects are bicuspid aortic valve and mitral valve prolapse (MVP), most of which are asymptomatic.
what are the different types of ASD?
which is most common?
which is part of ECDs
which is a/w PAPVC?
Types ( Fig. 2.23 C )
- Ostium secundum type:
- most common, 60%
- Ostium primum ASD:
- part of the ECD syndromes, 35%
- Sinus venosus defect (at entrance of SVC):
- always associated with PAPVC anomalous venous return, 5%
- Unroofed coronary sinus
- (rare)
Whate are the Radiographic Features of congenital Aortic Stenosis?
- Radiographic Features
- Cardiomegaly: LV hypertrophy
- Poststenotic dilatation of aorta is present only in valvular AS.
- CHF
- Figure 4. Supravalvar aortic stenosis. A, An anterior–posterior projection angiogram demonstrates a severe, discrete stenosis of the aorta at the level of the sinotubular junction. The ascending aorta above the sinotubular junction is normal in caliber, consistent with the classic hourglass form of supravalvar aortic stenosis. Reprinted from Stone et al32 with permission from the publisher. Copyright © 2012, Elsevier B, A lateral projection angiogram demonstrates moderate supravalvar aortic stenosis with diffuse, long-segment involvement of the entire thoracic aorta. Stenosis of the aortic arch vessels is presen
What are the rad features of
Congenital Peripheral PA Stenosis
Radiographic Features
- PA enlargement if associated valvular stenosis
- RV hypertrophy
Radiographic Features of Coaractation of the aorta
Radiographic Features
- Aortic figure-3 configuration 50%:
- Prestenotic dilatation of aorta proximal to coarctation
- Indentation of aorta caused by the coarctation
- Poststenotic dilatation
- Inferior rib notching:
- Secondary to dilated intercostal arteries
- Only ribs 3–8 involved
- Only in children >8 years
- Reverse-3 sign of barium-filled esophagus
- Prominent left cardiac border from left ventricular hypertrophy (LVH)
- Normal pulmonary vascularity
What is Tricuspid atresia?
- Complete agenesis of the tricuspid valve with no direct communication of the RA and RV
What are the clinical findings of Endocardial Cushion defects?
- Clinical Findings ( Fig. 2.27 )
- Partial canals may be asymptomatic; both the degree of mitral insufficiency and the shunt through the ASD determine the clinical picture.
- Complete AV canal consists of large L–R shunts (ASD, VSD), mitral insufficiency, and CHF.
Differentiating an ASD from a VSD using a chest X-ray is by detection of?
A. Enlargement of pulmonary artery
B. Enlarged left atrium
C. Dilated aorta
D. Pulmonary plethora
Correct answer : B. Enlarged left atrium
- Chest X-ray of a patient with atrial septal defect showing an enlarged right pulmonary artery (RPA), main pulmonary artery (MPA) and right atrium (RA). End-on view of an enlarged pulmonary vessel is also seen.
- DIFFERENTIATING AN ASD FROM A VSD USING A CHEST X-RAY
- Left atrium is not dilated in a case of atrial septal defect (ASD).
- It is dilated in patients with ventricular septal defect. (VSD).
- Pulmonary plethora is present in both ASD and VSD.
- Pulmonary artery is dilated in both.
- Size of the aorta is normal in both.
What are 2 causes of Congenital Peripheral PA Stenosis?
What syndrome is pictured?
Congenital Peripheral PA Stenosis
Causes
- Maternal rubella (common) (Image 2 wikipedia)
- Williams syndrome
- Image 1:
- Williams syndrome (WS), sometimes called Williams-Beuren syndrome, is characterised by some or all of the following features:
- craniofacial dysmorphism (e.g. elfin facies)
- oral abnormalities
- short stature (50% of cases)
- mild to moderate intellectual disability
- supravalvular aortic stenosis 2
- pulmonary artery stenosis 3
- renal insufficiency
- hypercalcaemia
- renal artery stenosis
- Williams syndrome (WS), sometimes called Williams-Beuren syndrome, is characterised by some or all of the following features:
Case courtesy of Senior Radiographer 2 Elton John Decena, RRT., Radiopaedia.org, rID: 85039
What is the underlying mechanism of Cyanotic CHD With Increased Pulmonary Vascularity (Admixture Lesions)
Cyanotic CHD With Increased Pulmonary Vascularity (Admixture Lesions)
- Common denominator of these lesions is that there is an “admixture” of systemic and pulmonary venous blood (bidirectional shunting).
- The mixing of venous and systemic blood may occur at the level of:
- Large veins
- Total anomalous pulmonary venous connection (TAPVC) (ASD is also present.)
- Large arteries
- Truncus arteriosus (TA) (VSD is also present.)
- Multiple
- Transposition of great arteries (TGA) (VSD, ASD, or PDA is also present.)
- Ventricle
- Single ventricle (VSD is present.)
- Double-outlet right ventricle (DORV) (VSD is present.)
- Large veins
What are the typical radiographic findings of Ebsteins anomaly?
Plain radiograph
- Large squared heart (box-shaped heart) caused by:
- Left side: horizontal position of RV outflow tract
- Right side: RA enlargement
- Decreased pulmonary vascularity
ddx of Cyanosis and normal vascularity
- Cardiomegaly
- Ebsteins
- Pulomary atresia
- Tricuspid
- No Cardiomegaly
- Tetralogy of Fallot
what determines which side the RA is located Re Situs?
What is the incidence of CHD in Situs inversus?
Situs
- Liver or IVC determines on which side the RA is located.
- Tracheobronchial tree:
- right mainstem bronchus (RMB) has a more vertical angle than LMB.
- Incidence of CHD in situs inversus: 5%
What are PAPVR associated with?
- Associations
- ASD, 15%
- (especially the sinus venosus defect)
What are the signs of Pulmonary arterial hypertension (PAH)?
Pulmonary arterial hypertension (PAH)
- Large main PA
- Usually normal hilar arteries
What are the different types of Endocardial Cushion defects?
Types
- Partial AV canal (ostium primum defect ± cleft in anterior mitral or septal tricuspid leaflets)
- Transitional AV canal (ostium primum defect, cleft in both AV valves, defect in superior IVS)
- Complete AV canal (ostium primum defect, cleft in both AV valves, large defect in IVS: either common AV valve or separate mitral and tricuspid valves)
what is this sign and why does it occur
FIGURE 12-21 Frontal view of a left ventriculogram of a patient with partial endocardial cushion defect showing a goose-neck deformity. The left ventricular outflow tract is elongated and narrowed. The arrows point to the mitral cleft. AO, aorta: LV, left ventricle.
https://doctorlib.info/cardiology/park-pediatric-cardiology-practitioners/13.html
6 Ddx of congenital heart disease which have an Increased incidence in males:
Increased incidence in males:
- AS
- coarctation
- pulmonary atresia
- tricuspid atresia
- hypoplastic left heart
- TGA
what is the most common Cyanotic CHD of childhood?
Tetralolgy of Fallot
What are the different types of PAPVR?
Common connections of pulmonary veins include: Supracardiac
- Right SVC (most common)
- Left SVC
- Azygos vein
- Innominate (via vertical vein)
- Cardiac
- RA
- Coronary sinus
- Infracardiac
- IVC (scimitar syndrome)
- Portal vein (PV)
- Diagram of several different types of partial anomalous pulmonary venous connection.
- A: Drainage of the right upper pulmonary vein (RUPV) to the superior vena cava (SVC).
- B: Drainage of the left upper pulmonary vein (LUPV) via a vertical vein to the innominate vein.
- C: Drainage of the right pulmonary veins (RPVs) to the inferior vena cava (IVC). This is the scimitar syndrome.
- D: Drainage of the left upper pulmonary vein and left lower pulmonary vein (LLPV) into the coronary sinus.
- Other abbreviations: IVC inferior vena cava, LA left atrium, LPV left pulmonary vein, RA right atrium, RLPV right lower pulmonary vein, RMPV right middle pulmonary vein.
What is Pink Tetralogy?
Pink Tetralogy
VSD with mild pulmonic stenosis
What are the different types of congenital peripheal PA stenosis?
Congenital Peripheral PA Stenosis
Types ( Fig. 2.32 )
- Type 1: single, main PA stenosis
- Type 2: stenosis at bifurcation of right and left PA
- Type 3: multiple peripheral stenosis
- Type 4: central and peripheral stenosis
What are the Radiographic Features Congenital Aortic Stenosis?
Cardiac CT
- Valve calcification
- Restricted opening on cine images, allows calculation of valve area
- Hypodense linear membrane in LV outflow in cases with subaortic stenosis
https://www.ahajournals.org/doi/10.1161/CIRCIMAGING.116.004271
What is the Pentalogy of Fallot?
Tetralogy ± ASD
The pentalogy of Fallot is a variant of the more common tetralogy of Fallot, comprising the classic four features with the addition of an atrial septal defect or patent ductus arteriosus:
- ventricular septal defect (VSD)
- right ventricular outlfow tract narrowing or complete obstruction
- right ventricular hypertrophy
- overriding aorta
- atrial septal defect (ASD) or patent ductus arteriosus (PDA)
Case courtesy of Dr Praveen Jha, Radiopaedia.org, rID: 19288
What Congential heart defects is Tricuspid atresia associated with?
- Associations
- Patent foramen ovale or ASD is always present.
- D-TGA, 35%
- VSD common
- Pulmonary atresia
- Hypoplastic right heart
- Extracardiac anomalies (GI, bone)
Top 6 Most Common Congenital Anomalies
All Age Groups
Bicuspid Aortic Valve and MVP Excluded
- Ventricular septal defect 30%
- Atrial septal defect 10%
- Tetralogy of Fallot 10%
- Patent ductus arteriosus 10%
- Coarctation of aorta 7%
- Transposition of great arteries 5%
Treatment of VSD
Treatment
Expectant in small asymptomatic VSD because there is spontaneous closure in infancy, 30%
Surgical therapy (patch) in patients with CHF, pulmonary hypertension (HTN), or progressive worsening
What is the underlying mechanism of Acyanotic CHD with inceased Pulmonary Vascularity?
Acyanotic CHD With Increased Pulmonary Vascularity
- Mechanism:
- L–R shunt where pulmonary flow is greater than aortic flow
- the shunt can be located in:
- Atrium: ASD
- Ventricle: VSD
- Artery: PDA, AP window
- Other: PAPVR, ECD
Ddx of No cyanosis and normal vascularity
- Coarcation
- Interupted aortic arch (IAA)
- Aortic Stenosis
- Pulmonary Stenosis
What are the different types of Coaractation of the Aorta?
Coarctation of Aorta
Types ( Fig. 2.34 )
- Infantile type (diffuse type, preductal): tubular hypoplasia
- Adult type (localized type, postductal, periductal): short segment; common
What are 3 syndromes a/w ASDs?
Associations ( Fig. 2.24 )
- Holt–Oram syndrome: ostium secundum defect
- Lutembacher syndrome: ASD and mitral stenosis
- Down syndrome: ostium primum defect
Signs of RV enlargement on xray
Radiographic features
- Frontal view demonstrates:
- rounded left heart border
- uplifted cardiac apex
- Lateral view demonstrates:
- filling of the retrosternal space
- rotation of the heart posteriorly
What is considdered Normal vascularity on xray?
Normal vascularity
Right main PA = same size as trachea at level of AA
Peripheral arteries (seen on end) = same size as adjacent bronchus
What are the features of Eisenmenger pulmonary vasculature?
Eisenmenger pulmonary vasculature
- Combination of shunt and PAH
- Aneurysmal hilar arteries
- Calcified vessels (rare)
A 37-year-old man with Eisenmenger syndrome caused by a large ventricular septum defect was admitted to the hospital with small-volume hemoptysis, cough, and left-sided chest pain for several weeks. For several years he had been classified as functional-class II, his oxygen saturation was in the ≈85%, hemoglobin was 11.0 mmol/L, and echocardiography visualized a mild tricuspid regurgitation with a gradient of 103 mm Hg (maximum). He had experienced several episodes of minor hemoptysis in the past. There was no previous history of arrhythmias or thromboembolic events.
On admission, his oxygen saturation was 82% and temperature 38.5°C. Blood samples revealed a slightly elevated C-reactive protein level of 16 mg/L (reference <10 mg/L) but normal white blood cell count. D-dimer was markedly elevated at 7.3 mg/L (reference <0.5 mg/L). Platelet count was normal. Chest x-ray showed massive dilated pulmonary arteries (Figure 1) confirmed by spiral computed tomography, which in addition revealed in situ circumferential mural thrombosis with calcification (Figure 2). Furthermore, in the left lung 2 minor infarcts were detected.
Because hemoptysis may be caused by pulmonary infarction secondary to thrombosis,1 warfarin treatment was instigated with the goal of international normalized ratio 2.0 to 3.0. Within days the hemoptysis stopped, and the patient recovered to his previous functional status. Warfarin treatment was continued after discharge. However, 2 years later he died suddenly as a result of a massive hemoptysis.
Hemoptysis occurs in the majority of patients with Eisenmenger syndrome2 and has been reported as the cause of death in 11% to 29% of patients with Eisenmenger syndrome.2,3 Furthermore, pulmonary artery thrombosis is found in 21% to 29% of patients with Eisenmenger syndrome.4,5 The coexistence of hemoptysis and pulmonary thrombosis poses a difficult clinical dilemma. Although anticoagulation seems logical for both prevention and treatment of thrombosis, it may be a double-edged sword that increases the risk of fatal bleeding. Because necropsy was not performed, dissection of the pulmonary aneurysm cannot be ruled out as the cause of the fatal bleeding. However, even in pulmonary artery dissection, successful urgent heart-lung transplantation has been reported.6 Thus there is not enough data on the role of anticoagulation in the case of pulmonary artery thrombosis and hemoptysis, a topic that clearly needs to be addressed in clinical trials.
Pulmonary Artery Thrombosis and Hemoptysis in Eisenmenger Syndrome
Annette Schophuus Jensen, MD , Kasper Iversen, MD , Niels G. Vejlstrup, MD, PhD , and Lars Sondergaard, MD, MDSc
https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.107.695312
What are 8 associations of Aortic Co-arcation?
Associations
- Coarctation syndrome:
- triad of
- coarctation,
- PDA,
- VSD
- triad of
- In Turner syndrome, most common cardiac abnormality
- Bicuspid aortic valve, 50%
- Hypoplasia of the aortic isthmus (small arch)
- Circle of Willis aneurysms
- PDA aneurysm
- Intracardiac defects; occur in 50% with infantile type
- Marfan syndrome
What is the mechanism of rib notching in Co-Arcation of the aorta?
which ribs and where will demonstrate Notching?
Which ribs wont demonstrate notching (typically)?
What are the clinical findings of a PDA?
When do they present?
What symptoms?
Clinical Findings
- Most are asymptomatic.
- Large defects lead to CHF at 2–3 months of age.
What are the different types of VSD
Which is most common?
Which is seen in T21?
Types
- Membranous, 80%
- Muscular, 10%
- AV, 5% (T21)
- Conal, 5% (mostly supracristal)
What is Ebsteins anomaly associated with?
Maternal Lithium intake
What is the treatment of Tetralogy of Fallot?
- Total corrective repair
- VSD closure and reconstruction of RV outflow tract
- Palliative shunts: systemic to PA shunt allows growth of pulmonary vessels.
- Blalock–Taussig shunt (SA → PA) is indicated in symptomatic patients who are poor surgical candidates ( Fig. 2.39 ).
Which condition causes cyanosis with normal/decreased vascularity?
what are the characteristics of this CHD?
Tetralogy of Fallot ( Fig. 2.36 )
Most common cyanotic CHD of childhood Tetrad
- Obstructed RV outflow tract
- Right ventricular hypertrophy (RVH)
- VSD
- Aorta overriding the IVS
What are the common associated findings RE tetralogy of Fallot?
- Pulmonary infundibular or valve stenosis in most cases
- Right AA anomalies
- aberrant vessels
- vascular sling or rings
- 25%
- Anomalies of coronary arteries
- 5%
- LAD from RCA
- single RCA
- TEF
- Rib anomalies
- scoliosis
Treatment of Pulmonary valve stenosis?
Treatment
- Most stenoses are amenable to balloon valvuloplasty.
- Surgical reconstruction may be necessary for dysplastic types.
What is an Aortopulmonary window defect?
What are the xray findings? what are they the same as?
Aortopulmonary Window ( Fig. 2.29 )
- Synonyms
- aortopulmonary septal defect
- partial TA.
- What is it?
- Defect between ascending aorta and main or right PA
- L-R shunt
- Plain radiograph findings are identical to those seen in PDA.
What is PAPVC?
Is there cyanosis?
Partial Anomalous Pulmonary Venous Connection (PAPVC)
- Some but not all pulmonary veins drain to the systemic circulation rather than into the LA.
- Anomalous pulmonary venous connection is a L–R shunt.
- Pulmonary veins connect to RA or systemic veins.
- Cyanosis occurs because of Eisenmenger syndrome.
- PAPVC is not always cyanotic.
What are the Rad Findings of PAPVR
Radiographic Features
- Supracardiac and cardiac types resemble ASD findings.
- The anomalous vein of the infracardiac type looks like a Turkish scimitar (sword): scimitar sign.
- Infracardiac PAPVC is part of hypogenetic lung syndrome.
- Cardiac CT and MRI
- Allows visualization of pulmonary veins, shunt quantification, and aids preoperative planning.
- Case courtesy of Dr Brendon Friesen, Radiopaedia.org, rID: 28724
What are the clinical findings of ASDs?
Clinical Findings
- May be asymptomatic for decades because of the low atrial pressure; even large defects are much better tolerated than VSD or PDA.
- Female preponderance
- May present with pulmonary hypertension
Radiographic Features of ASD
RA, RV, and PA enlargement; no LA enlargement (different from VSD)
The AA appears small (but in reality is normal) because of the prominent pulmonary trunk and clockwise rotation of heart (RV enlargement).
Signs of LV enlargement on Xray
- LV enlargement:
- posterior displacement behind IVC
- left ventricular dilatation:
- left heart border is displaced leftward, inferiorly and posteriorly
- left ventricular hypertrophy:
- may show rounding of the cardiac apex
- Hoffman-Rigler sign
- Shmoo sign
What are the Clinical Findings of Congenital Aortic Stenosis (AS)?
What syndrome is associated with Congenital Aortic Stenosis?
Clinical Findings of Congenital Aortic Stenosis (AS)
- Most are asymptomatic.
- Severe AS leads to CHF in infancy.
- Supravalvular type associated with Williams syndrome:
- Mental retardation
- Peripheral pulmonary stenoses
- Diffuse AS
RE VSD’s
When are small defects symptomatic?
When are large defects symptomatic?
?% close by age 10?
What are 2 complications?
Clinical Findings
- Small to moderate defects are initially asymptomatic.
- Large defects lead to CHF at 2–3 months of age.
- 75% close spontaneously by age 10.
- Complications
- 3% of patients develop infundibular stenosis.
- Eisenmenger syndrome (elevated pulmonary resistance leads to increased right-sided pressures) may develop in long-standing large defects; the end result is a R-L shunt with cyanosis
What are the Grades of Pulmonary venous hypertension?
Pulmonary arterial overcirculation (shunt vascularity):
- more common in CHD,
- less common in acquired heart disease (AHD)
-
Pulmonary venous hypertension (PVH) (edema) grades:
- Grade 1: Vascular redistribution (10–17 mm Hg)
- Grade 2: Interstitial edema (18–25 mm Hg)
- Grade 3: Alveolar edema (>25 mm Hg)
ddx of No cyanosis and increased vascularity
- Enlarged left atrium
- PDA
- VSD
- Normal Left Atrium
- ASD
- Endocardial Cushion defect
What are the Rad Findings of Tricuspid Atresia?
What other abnormality may be present?
If so, what are the findings?
- Plain radiograph
- May be normal
- No TGA: similar appearance as Fallot
- If TGA is present (30%) there is:
- Increased pulmonary flow
- Cardiomegaly
- Narrow vascular pedicle
FIG. 31-12. Tricuspid atresia with large (nonrestrictive) atrial septal defect. There is decreased pulmonary vascularity and only mild cardiomegaly. Note the flattened right atrial border (arrows), which is characteristic for this lesion when there is a large nonrestrictive atrial septal defect.
The patient with tricuspid atresia with a large atrial septal defect demonstrates little or no cardiomegaly (Fig. 31-12).
On the other hand, the patient with tricuspid atresia with a restrictive atrial septal defect experiences substantial right atrial enlargement, which results in cardiomegaly. Consequently, the former patient would be classified in group II, the latter patient in group III. Tricuspid atresia can be classified in group IV when there is an associated increase in pulmonary blood flow, which is caused by either a large left-to-right shunt at the ventricular septal level or the concurrence of transposition of the great vessels. Transposition of the great arteries (TGA) occurs in approximately 30% of patients with tricuspid atresia.
https://radiologykey.com/radiography-of-congenital-heart-disease/
Clinical findings of Coarctation of the aorta?
Clinical Findings
- Blood pressure difference between arms and legs
- Neonates/Diffuse type
- blue lower limbs
- neonatal CHF.
- Adult type
- is frequently asymptomatic
- presents in young adult
- berry aneurysms
- Claudication
- Lower BP in legs
- hypertension via Renin-Angiotensin
What are the chamber/vascularity/aorta sizes in Tetralogy of Fallot?
what are the hemodynamics of a PDA?
which atria/ventricles/aorta are enlarged?
Hemodynamics
There is an L–R shunt because the pressure in the aorta is higher than in the pulmonary circulation.
What condition is Tricuspid atresia a/w?
How often?
Transposition of the great arteries (TGA) occurs in approximately 30% of patients with tricuspid atresia.
what are the haemodynamics of endocardial cushion defects?
Plain radiograph
- Cardiomegaly
- Increased pulmonary vascularity
- Screen for other trisomy 21 findings: 11 ribs, multiple manubrial ossification centers
- Angiography
- Gooseneck deformity of LVOT on RAO view
- Abnormal prolapse of anterior MV leaflet in diastole
- Cardiac CT and MRI
- Problem-solving tool that allows visualization of septum in cases wherein US is limited
- Case courtesy of Assoc Prof Frank Gaillard, Radiopaedia.org, rID: 7453
what is the treament for PDA?
Treatment
- Indomethacin (inhibits PGE 1 , which is a potent dilator of the duct); successful in 60% of infants
- Ligation of ductus through left thoracotomy
- Catheter closure with umbrella device
What are the Rad Features of PDA?
Radiographic Features
- Small PDA: normal CXR
- Increased pulmonary vascularity
- Enlargement of LA, LV
- Eisenmenger physiology may develop in long-standing, severe disease.
- All the above features are identical to those seen in VSD; specific features to suggest PDA are:
- Unequal distribution of pulmonary arterial blood flow, especially sparing of left upper lobe
- Enlargement of aorta and AA
- PDA may be seen as faint linear density through the PA (occasionally calcifies).
- Cardiac CT helps in determining precise location and size as a roadmap before percutaneous closure.
- Case courtesy of Dr Jeremy Jones, Radiopaedia.org, rID: 24125
ddx for Cyanosis and increased vascularity?
- 5 T’s
- TGA
- TA
- Tingle Ventricle
- TAPVR
- Tricuspid atresia
What are the haemodynamics of Tricuspid atresia?
Which chambers/arteries/vessels are enlarged/smaller/normal?
Hemodynamics
All blood crosses through a large ASD into the LA.
What are the radiographic features of Tetralogy of Fallot??
- Plain radiograph
- Boot-shaped heart (enlarged RV): coeur en sabot
- Right AA, 25%
- Small or concave PA
- Cardiac CT and MRI
- Used to determine extent of systemic collateral vessels coming off the aorta.
- Useful in assessment of post-repair complications like pulmonic regurgitation and flow quantification.
what are the 2 types of pulmonary valvular stenosis?
Types
- Dome-shaped type (95%): valve with a small orifice and three fused commissural raphes
- Dysplastic type (5%): thickened, redundant, immobile leaflets; commissures not fused
In which population do PDAs typically occur?
- Highest incidence of PDA occurs in premature infants (especially if hyaline membrane disease is present) and in maternal rubella; females > males
what are the Radiographic Features of pulmonary valve stenosis?
- Plain radiograph
- Poststenotic dilatation of main and/or left PA (jet through stenosed valve dilates PA)
- Right PA of normal size
- RV (hypertrophy)
signs of left artial enlargement on xray
Chamber Enlargement
- Lateral view is more helpful in young children:
- LA enlargement
- Posterior displacement of esophagus during barium swallow
- Posterior displacement of left mainstem bronchus (LMB)
- Double density sign: density of enlarged LA behind right heart
- LA enlargement
What is the treatment of Coarctation?
- Treatment
- Resection of coarctation and end-to-end anastomosis
- Patch angioplasty: longitudinal incision with placement of a synthetic patch
- Subclavian patch: longitudinal incision of coarctation; division of SA and longitudinal opening to be used as a flap
- Percutaneous balloon angioplasty
What are the three different types of
Congenital Aortic Stenosis
- Subvalvular AS
- Membranous subaortic stenosis
- Hypertrophic subaortic stenosis (fibromuscular tunnel)
- Valvular AS (most common) ( Fig. 2.33 )
- Bicuspid aortic valve (most common congenital heart anomaly)
- Unicommissural valve (single horseshoe-shaped valve)
- Supravalvular AS
- Localized
- Diffuse
What is the Dx?
Ebsteins Anomaly
Box shaped heart.
Case courtesy of Dr Jeremy Jones, Radiopaedia.org, rID: 24348
Which artia/ventricles englarge in ASDs?
Is the aorta enlarged or normal?
- Blood flows from LA to RA
What role does the PDA play in the fetus?
In the fetus, a PDA represents a normal pathway of blood flow.
Fetal circulation:
flow from PA to aorta as intrauterine bypass of nonaerated lungs
