CARDIAC IMAGING 3 Flashcards
Is TGA Cyanotic/Acyanotic
Increased/normal/decreased vascularity?
- Cyanotic with increased vascularity
What types of TGA are there?
- Types
- D-TGA
- Aorta originates from RV
- PA originates from LV
- Normal position of atria and ventricles: AV concordance
- L-TGA
- Transposition of great arteries
- Inversion of ventricles: AV discordance
What must TGA have to be compatible with life and why?
- Two independent circulations exist:
- Blood returning from body → RV → blood delivered to body
- Blood returning from lung → LV → blood delivered to lung
- This circulatory pattern is incompatible with life unless there are associated anomalies that permit mixing of the two circulations (e.g., ASD, VSD, or PDA).
What are the Haemodynamics of TGA?
What is enlarged/normal re atria/ventricles/vessels
Hemodynamics
Depends on the type of mixing of the two circulations
What are the radiographic features of TGA?
- Plain radiograph
- “Egg-on-side” cardiac contour:
- narrow superior mediastinum secondary to hypoplastic thymus (unknown cause) and abnormal relationship of great vessels
- As pulmonary resistance decreases, pulmonary vascularity increases
- Right heart enlargement
- Pulmonary trunk not visible because of its posterior position
What is the treatment of TGA?
- Treatment
- PGE 1 is administered to prevent closure of PDA. Palliative measures include temporization methods before definitive repair. Corrective operation performed during first year of life:
- Correct reattachment of large vessels (Jatene arterial switch procedure)
- Creation of an atrial baffle (Mustard, Senning, or Schumaker procedure) Rashkind procedure: atrial septostomy with balloon
- Blalock–Hanlon: surgical creation of atrial defect
- PGE 1 is administered to prevent closure of PDA. Palliative measures include temporization methods before definitive repair. Corrective operation performed during first year of life:
What is the difference between L TGA and D TGA?
Which is cyanotic and which is acyanotic?
Corrected Transposition of Great Arteries:
- AKA:
- (L-TGA)
- Levo-transposition
- Large vessels and ventricles are transposed (AV discordance and ventriculoarterial discordance). Poor prognosis because of associated cardiac anomalies. If isolated, this is an acyanotic lesion.
What is L-GTA associated with?
4
Associations
- Perimembranous VSD, >50%
- Pulmonic stenosis, 50%
- Anomaly of tricuspid valve
- Dextrocardia
What are the Rad Features of L-TGA
- Plain radiograph
- Pulmonary trunk and aorta are not apparent because of their posterior position.
- LA enlargement
- Abnormal AA contour because of the leftward position of the arch
- Right pulmonary hilus elevated over left pulmonary hilus
- US
- Anatomic LV on right side
- Anatomic RV on left side
- Cardiac CT and MRI
- Problem-solving tool, defines anatomy, assess complications such as baffle leaks or thrombosis
- Case courtesy of Dr Sajoscha Sorrentino, Radiopaedia.org, rID: 14874
-
Case Discussion
- There is situs inversus with laevocardia.
- Cardiac pacemaker with unusual placement of the electrode tips is demonstrated.
- While the heart shadow may not give it away at once, note the unusual position of the pacemaker electrodes.
- Not only that the patient has situs inversus, this is also a diagnosed case of levo-transposition of the great arteries with an ostium secundum atrial septal defect, large membranous ventricular septal defect and pulmonary (valve) stenosis. He previously underwent Blalock-Taussig shunt procedure, closure of the septal defects and correction of the pulmonary stenosis.
Truncus Arteriosus (TA)
Results from failure of formation of the spiral septum within the TA. As a result, a single vessel (truncus) leaves the heart and gives rise to systemic, pulmonary, and coronary circulation. The truncus has 2 to 6 cusps and sits over a high VSD.
Case courtesy of Dr Vincent Tatco, Radiopaedia.org, rID: 43228
There is cardiomegaly.
Lungs are hyperinflated with increased pulmonary vascularity.
Right-sided aortic arch is demonstrated.
Case Discussion
This is a chest radiograph of a patient with truncus arteriosus type I. The diagnosis was confirmed on echocardiography. Truncus arteriosus is a common differential diagnosis in chest radiographs of cyanotic patients presenting with cardiomegaly, pulmonary plethora and right-sided aortic arch.
2 articles feature images from this case
What are the associations of Truncus arteriosis?
- Associations
- All patients have an associated high VSD.
- Right AA, 35%
What are the 4 types of Truncus Arteriosus?
Which is the most and least common?
- Types ( Figs. 2.49 – 2.50 )
- Type 1 (most common): Short main PA from truncus
- Type 2: Two separate PAs from truncus (posterior origin)
- Type 3 (least common): Two separate PAs from truncus (lateral origin)
- Type 4 (pseudotruncus) PA from descending aorta = pulmonary atresia with VSD; findings of a tetralogy of Fallot combined with pulmonary atresi
What are the Haemodynamics of Truncus Arteriosis?
Cyan/Acyan
Plethora/no plethora
Chamber enlargement
Arterial enlargement?
- Hemodynamics
- Admixture lesion with both L–R (truncus → PA) shunt and R–L (RV → VSD → overriding aorta) shunt.
What are the xray findings of Truncus Arteriosus?
- Radiographic Features
- Plain radiograph
- Enlargement of aortic shadow (which actually represents the truncus)
- Cardiomegaly because of increased LV volume
- Increased pulmonary vascularity
- Pulmonary edema, occasionally present
- Right AA, 35%
- US, MRI, cardiac CT, and angiography to determine type
- Plain radiograph
What is the treatment for Truncus Arteriosus?
- Treatment
- Three-step surgical procedure:
- Closure of VSD so that LV alone empties into truncus
- PAs removed from truncus and RV-PA conduit placed
- Insertion of a valve between the RV and PA
- Three-step surgical procedure:
- https://pubs.rsna.org/doi/full/10.1148/rg.2017160033
What is the key concept of TAPVC
Total Anomalous Pulmonary Venous Connection (TAPVC) ( Fig. 2.51 )
-
Pulmonary veins connect to:
- systemic veins or the
- RA
- rather than to the LA.
- TAPVC exists when all pulmonary veins connect anomalously.
- The anomalous venous return may be obstructed or nonobstructed.
What are thr 4 types of TAPVC?
What are their characteristics?
-
Supracardiac connection
- (50%)
- Supracardiac TAPVC is the most common type
- infrequently associated with obstruction.
- Left vertical vein
- SVC
- Azygos vein
- (50%)
-
Cardiac connection
- (30%)
- RA
- Coronary sinus
- Persistent sinus venosus
- (30%)
-
Infracardiac connection
- (15%)
- majority are obstructed
- PV
- Persistent ductus venosus
- IVC (caudal to hepatic veins)
- Gastric veins
- Hepatic veins
- Mixed types (5%)
What are 3 associations of TAPVR?
- Associations
- Patent foramen ovale, ASD (necessary to sustain life)
- Heterotaxy syndrome (asplenia more common)
- Cat’s eye syndrome
What are the clinical findings of TAPVR?
What do the symptoms depend on?
- Symptomatology depends on presence or absence of obstruction
- Obstructed:
- pulmonary edema within several days after birth
- Nonobstructed:
- asymptomatic at birth. CHF develops during first month.
- Obstructed:
- 80% mortality by first year
What are the haemodynamics of Unobstructed Pulmonary Vein TAPVR?
Hemodynamics
Unobstructed Pulmonary Vein
TAPVC causes a complete L-R shunt at the atrial level; therefore to sustain life, an obligatory R-L shunt must be present. Pulmonary flow is greatly increased, leading to dilatation of RA, RV, and PA.
What are the Haemodynamics of obstructed TAPVC?
- Obstructed Pulmonary Vein
- Obstruction has three consequences:
- PVH and PAH
- Pulmonary edema
- Diminished pulmonary return to the heart, which results in low cardiac output
- Obstruction has three consequences:
In cases where the pulmonary veins are obstructed,
the return of oxygenated blood to the heart is
impeded. Consequently, the proportion of oxygenated
blood in the right atrium is reduced, thus, greater
desaturation follows. Furthermore, obstruction to the
pulmonary venous return leads to back-pressure in the
pulmonary venous system, resulting in pulmonary
venous congestion, which has a classical chest X-ray
appearance. Clinically, this manifests as worsening
desaturation and an increased work of breathing as
seen in this case
What are the radiographic Features of nonobstructed TAVPC?
What sign is there for supracardiac TAVPC?
- Plain radiograph of nonobstructed TAPVC
- Snowman heart (figure-of-eight heart) in supracardiac type; the supracardiac shadow results from dilated right SVC, vertical vein, and innominate vein.
- Snowman configuration ( Fig. 2.55 ) not seen with other types
- Increased pulmonary vascularity
- Plain radiograph of obstructed TAPVC
- Pulmonary edema
- Small heart
- Case courtesy of Dr Aditya Shetty, Radiopaedia.org, rID: 27800
Plain radiograph of obstructed TAPVC
-
Plain radiograph of obstructed TAPVC
- Pulmonary edema
- Small Heart
https://www.eurorad.org/case/1687
The heart is normal in size and there is bilateral airspace opacification, consistent with pulmonary oedema.
A full-term infant with uneventful delivery presented with respiratory distress, difficulty in feeding and cyanosis. The oxygen saturation by pulse oximeter was 50% in room air. Septic screen was negative and there was no history of haemoptysis.
IMAGING FINDINGS
After an uncomplicated birth at term and discharge home well at day two of life, the patient presented with gradual shortness of breath and difficulty in feeding due to tiredness over a 10-day period. At a routine postnatal check it was noticed that he was dusky and in mild respiratory distress. On transfer to the Casualty department, the oxygen saturation was found to be 50% in room air. He was apyrexial and the white cell count was normal. An initial chest radiograph demonstrated a normal-sized heart with normal pulmonary vascularity and clear lungs.
The patient deteriorated over the next few hours, requiring intubation and ventilation. There was no history of haemoptysis or of visualised blood during intubation. A repeat chest radiograph was obtained (Fig. 1). It demonstrated a normal-sized heart and diffuse bilateral airspace opacification consistent with pulmonary oedema. Pleural effusions were not present. The upper mediastinum was not widened. In view of the history of cyanosis and respiratory distress with an oxygen saturation of 50% and the rapid onset of airspace opacification with a normal heart size a provisional diagnosis of obstructed total anomalous pulmonary venous return was made. An echocardiogram was performed and confirmed the presence of a common pulmonary venous chamber, behind the left atrium, which accepted all four pulmonary veins and which connected to the infradiaphragmatic IVC by an obstructed vertical vein. A small 6mm restrictive ASD shunting from right to left was also found. The ventricular septum was normal; mild tricuspid regurgitation was noted. The main pulmonary artery was enlarged, a PDA was also present, again with right to left shunting. The aortic arch was left sided and normal. The left ventricular outflow tract was normal. The IVC and SVC were connected to a morphologic right atrium.
Supportive medical therapy was instigated and the patient underwent cardiac surgery within 24 hours. At surgery the findings on echo were confirmed and repaired. The common chamber receiving all four pulmonary veins was anastomosed to the left atrium and the ASD was sutured closed. The patient was discharged home well on full feeds on day 20 post-op.
what are the radiographic features of TAPVC?
Obstructed and Non-obstructed?
what is the treatment of TAPVC?
- Treatment
- Consists of a three-step procedure:
- Creation of an opening between the confluence of pulmonary veins and the LA
- Closure of the ASD
- Ligation of veins connecting to the systemic venous system
What is a single ventricle?
What morphology does it have
what is the relationship to the atria/Vessels?
Morbidity?
Single Ventricle
- Most commonly, the single ventricle has LV morphology and there is a rudimentary RV.
- The great arteries may originate both from the dominant ventricle or one may originate from the small ventricle.
- Rare anomaly with high morbidity.
- Common ventricle: absence of the IVS.
Re Single Ventricle, what other malformation is usually present?
• Malposition of the great vessels is usually present.
What is a Double outlet right ventricle?
What other malformation is always present?
Double-Outlet Right Ventricle (DORV)
The great vessels originate from the RV.
A VSD is always present; other malformations are common.
Rare anomaly.
Radiographic features are similar to those of other admixture lesions and depend on concomitant anomalies.
Cardiac CT and MRI can be used to assess anatomy, function, and visualized collaterals.
Both CT and MRI can be used for diagnosis of baffle leaks and thrombosis and to assess venovenous collaterals.
What are the Rad Features of Single ventricle?
Radiographic Features
Variable appearance: depends on associated lesions
Pulmonary circulation may be normal depending on the degree of associated pulmonic stenosis.
https://radiologykey.com/hearts-with-single-ventricle-physiology/
What is this condition?
What are the symptoms?
What are the associations?
What are the rad findings?
- Aorta Pseudocoarctation
- Asymptomatic variant of coarctation
- no pressure gradient across lesion (aortic kinking)
- Associations
- Bicuspid aortic valve (common)
- Many other CHDs
- Radiographic Features
- Figure-3 sign
- No rib notching
- Usually worked up because of superior mediastinal widening (especially on left) on CXR
http://learningradiology.com/archives2010/COW%20417-Pseudocoarctation/pseudocoarctcorrect.htm
What is this?
What are the different types?
- Interruption of Aortic Arch (IAA)
- Types
- Type A: Occluded after LSA, similar to coarctation
- Type B: Occluded between LCA and LSA
- Type C: Occluded between brachiocephalic artery and LCA
- Types
https://www.researchgate.net/figure/CT-angiography-showing-type-A-interrupted-aortic-arch-distal-to-the-left-subclavian_fig1_248385305
What is this condition associated with?
Interupted Aortic Arch
Associations
- Usually associated with VSD and PDA
- DORV and subpulmonic VSD (Taussig–Bing malformation)
- Subaortic stenosis
https://www.researchgate.net/figure/Three-dimensional-reconstruction-image-showing-type-A-interrupted-aortic-arch-with_fig2_248385305
What are the rad findings of Interupted Aortic Arch on Xray
- Neonatal pulmonary edema
- No aortic knob
- Large PA
Discussion
Interrupted aortic arch (IAA), or completeinterruption of the aorta, describes congenital absence of the luminal continuity between the ascending and descending portions of the aorts. IAA was first described in 1778, and as a fairly rare occurrence, IAA comprises less than 1.5% of instances of congenital heart disease. In many cases, IAA is associated with intracardiac malformations. The cause of IAA is unknown but may be due to abnormal fetal blood flow patterns. IAA is associated with decreases in ascending aortic flow and increases in ductal flow.
Classification of IAA is based on which segment of the aortic arch is absent(percentages are from 1997):
Type A (13%): Interruption occurs between the left subclavian artery and descending aorta.
Type B (84%): Interruption occurs between the left subclavian artery and the left common carotid artery.
Type C (3%): Interruption occurs between the left common carotid artery and the innominate artery.
In the newborn, its clinical presentation involves severe congestive heart failure. IAA was previously fatal, but currently PGE1 (prostaglandin E1) therapy is used to maintain ductal patency and allow time for preoperativehemodynamic optimization and reestablishment of blood flow to the lowerbody. Without surgical correction, the mean age at death is 4-10 days with 90% of patients with IAA dying within the first year of life. With surgical repair, the 10-year survival rate is about 47%.
What are the three top Aortic arch anomalies?
Which are symptomatic?
A large number of AA anomalies exist; however, only three are common:
- Left arch with an aberrant RSA (asymptomatic)
- Right arch with an aberrant retroesophageal LSA (asymptomatic)
- Double arch (symptomatic)
What excludes significant arch anomalies and sling?
Normal lateral esophagram excludes significant arch anomalies and sling.
What is the Diagnosis?
Double aortic Arch:
-
Double Aortic Arch.
- Frontal chest shows impression on right-side of barium-filled esophagus from higher right-sided arch and below it an impression on the left-side of the esophagus from left-sided arch.
- Lateral film shows anterior displacement of both trachea and esophagus.
- AP esophagram distinguishes double arch (bilateral esophageal indentations) from right arch.
- Double aortic arch is most common vascular ring
- Caused by persistence of Right and Left IV branchial arches
- Rarely associated with Congenital Heart Disease
- Symptoms (of tracheal compression or difficulty swallowing) may begin at birth
- Right arch is higher, left arch is lower producing reverse S on esophagram in AP
- Right arch supplies Right common carotid and Right subclavian arteries
- Left arch supplies Left common carotid and Left subclavian arteries
- On lateral, arches are posterior to esophagus and anterior to trachea
https://learningradiology.com/notes/cardiacnotes/pulmslingpage.htm
What is the Diagnosis?
Pulmonary sling:
The key view is the lateral where red arrow points to aberrant
left pulmonary artery interposed between the esophagus and trachea.
Pulmonary sling occurs because of failure of formation of Left 6th aortic arch so there is absence of Left pulmonary artery
The blood to the Left lung comes from an aberrant Left pulmonary artery which arises from Right pulmonary artery and crosses between esophagus and trachea
Bronchial cyst may produce same finding on esophagus/trachea
https://learningradiology.com/notes/cardiacnotes/pulmslingpage.htm
What is the diagnosis?
What are the rad findings?
What are the symptoms?
What are the associations?
Left AA With Aberrant RSA
- Most common congenital AA anomaly
- Asymptomatic, not a vascular ring.
- Radiographic Features
- Left arch
- Abnormal course of RSA
- Behind esophagus, 80% = retroesophageal indentation
- Between esophagus and trachea, 15%
- Anterior to trachea, 5%
- Associations
- Absent recurrent right laryngeal nerve
Case courtesy of Dr Andrew Dixon, Radiopaedia.org, rID: 9689
What condition is shown here?
What is the anatomy?
what is the diverticulum?
- Right AA With Aberrant LSA
- Interruption #3 on Fig. 2.59 .
- Only 5% have symptoms secondary to airway or esophageal compression.
- Radiographic Features
- Right arch
- Retroesophageal indentation
- Diverticulum of Kommerell: aortic diverticulum at origin of aberrant SA
- Associations
- CHD in 10%
- Tetralogy of Fallot, 70%
- ASD, VSD
- Coarctation
What is this condition?
What are the symptoms?
What are the associations?
- Right AA With Mirror-Image Branching ( Fig. 2.60 )
- Interruption #2
- No vascular ring symptoms.
- Radiographic Features
- Right AA
- No posterior indentation of esophagus
- Associations
- Cyanotic heart disease in 98%
- Tetralogy of Fallot, 90%
- TA, 30%
- Multiple defects
- Right Aortic Arch
- Types
- At least five different types
- Only two of importance
- Mirror Image Type — Type I
- Aberrant left subclavian — Type II
- General considerations
- Recognized by leftward displacement of barium-filled esophagus
- Of air-filled trachea
- Aortic knob is absent from left side
- Aorta descends on right
- Para-aortic stripe returns to left side of spine just above diaphragm
-
Mirror-image type almost always has associated congenital heart disease (CHD)
- Usually Tetralogy of Fallot
- Aberrant Left Subclavian type rarely has associated CHD
- Most common variety of right arch
- Types
-
Type 1—Mirror Image Type
- Secondary to interruption of left arch just distal to ductus arteriosis
- Associated with congenital heart disease 98% of time
- Imaging Findings
- No posterior impression on trachea or barium-filled esophagus
- Heart is usually abnormal in size or shape
- Aorta descends on right
- If there is a mirror-image right aortic arch, then
- 90% will have Tetralogy of Fallot
- 6% with Truncus Arteriosis
- 5% with Tricuspid Atresia
-
Type ll—Aberrant Left Subclavian
- Secondary to interruption of left aortic arch between LCC and LSC arteries
- Associated with cardiac defects 5-10% of the time
- Tetralogy of Fallot most often (71%)
- ASD or VSD next most often (21%)
- Coarctation of aorta rarely (7%)
- Anomalous left subclavian artery (retroesophageal and retrotracheal)
- Aorta descends on right
- Imaging Findings – Right Aortic Arch with Aberrant LSCA
- Posterior impression on trachea and barium-filled esophagus
- Heart is usually normal in size and shape
- Aorta descends on right
- Origin of RSCA may be dilated
- Diverticulum of Kommerell technically was defined with a right aortic arch and anomalous left subclavian artery (LSCA)
http://learningradiology.com/archives06/COW%20212-Mirror%20Image%20Right%20Ao%20Arch/mirrorarchcorrect.htm
What Arch abnromality causes a congenital subclavian steal?
- Right Arch With Isolated LSA
- LSA attached to left PA via ductus arteriosus.
- The LSA is isolated from aorta and obtains blood supply from LVA; produces congenital subclavian steal. Interruptions near #2 and at #3 (see Fig. 2.59 ).
- Radiographic Features
- Right arch
- No posterior esophageal indentation
- Associations
- Almost all are associated with tetralogy of Fallot.
What is the Dx?
Is this condition symptomatic?
what are the rad findings?
- Double AA
- Persistence of both fetal arches
- Concomitant CHD is rare.
- Most common type of vascular ring.
- Most symptomatic of vascular rings.
- Radiographic Features
- Right arch is higher and larger than the left arch.
- Widening of the superior mediastinum
- Posterior indentation of the esophagus on lateral view
- Bilateral indentations of the esophagus on AP view
Double aortic arch or aortic ring is a congenital abnormality. In most cases, this abnormality is diagnosed in childhood because of symptoms related to esophageal or tracheal obstruction, and surgical correction usually yields a good prognosis. Sporadic cases have been reported in adult patients, who are usually diagnosed after complaining of asthma-like symptoms or swallowing difficulties because of the compression of the trachea or esophagus by the abnormal aortic arches. Case reports of elderly patients are rare, especially symptom-free cases. The patient’s double aortic arch was defined accidentally when he began suffering from cerebral bleeding and his coronary heart disease became symptomatic. In this particular case, the trachea and esophagus were placed in the middle of the aortic ring without compression, because the right and left aortic arches were almost the same size and the inner space of the ring was large enough to fit them both inside.
https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.106.617274
What is a Pulmonary Sling?
What is the anatomy?
What are the complications?
Pulmonary Sling ( Fig. 2.61 )
Aberrant left PA arises from the right PA and passes between the trachea (T) and esophagus (E). Compresses both trachea and esophagus. Tracheobronchiomalacia and/or stenosis occurs in 50%.
What are the 7 types of vascular slings?
3 symptomatic needing surgery
4 assymptomatic
- Types
- Symptomatic (require surgery)
- Double AA
- Right arch + aberrant LSA + PDA (common)
- Pulmonary sling
- Asymptomatic
- Anomalous innominate artery
- Anomalous left common carotid
- Left arch + aberrant RSA
- Right arch + aberrant LSA (mirror image to above)
- Symptomatic (require surgery)
- Figure 3(A) Esophagogram and (B) computed tomographic scan show extrinsic compression of the esophagus by the right arch and aberrant left subclavian artery (yellow arrow) with persistent ligamentum.
- Figure 1
- (A) Right arch (R-Arch) with aberrant left subclavian artery (SA) off the Kommerell diverticulum (KD) with persistent ligamentum (PL),
- (B) left arch with aberrant right SA off the KD,
- (C) double aortic arch,
- (D) R-Arch with mirror imaging and PL off the KD,
- (E) R-Arch with aberrant left innominate artery and PL off the KD, and
- (F) pulmonary artery sling.
Label this diagram with 3 conditions:
Symptomatic (require surgery)
- Double AA
- Right arch + aberrant LSA + PDA (common)
- Pulmonary sling
Label this diagram with four conditions.
- Asymptomatic
- Anomalous innominate artery
- Anomalous left common carotid
- Left arch + aberrant RSA
- Right arch + aberrant LSA (mirror image to above)
What is this Syndrome?
Left isomerism
what is the % of CHD in the following?
Situs solitus + Levocardia
Situs Solitus + Dextrocardia
Situs inversus + Dextrocardia
Situs inversus + Levocardia
In left Isomerism what are the findings Re:
- Pulmonary vascularity
- Bronchi
- Minor fissure
- Heart
- Atrium
- Ventricle
- Pulmonary veins
- Great vessels
- SVC
- Bowel
- Spleen
- Abdominal Situs
- IVC
- Pulmonary vascularity
- Increased (overcirculation)
- Bronchi
- Hyparterial
- Minor fissure
- None, normal
- Heart
- Cardiomegaly/moderate CHD
- Atrium
- ASD
- Single ventricle
- DORV
- Pulmonary veins
- PAPVC
- Great vessels
- Normal
- SVC
- Bilateral 30%
- Bowel
- Malrotation
- Spleen
- Multiple
- Abdominal situs
- Ambiguous/inversus
- IVC
- Azygous continuation
What are the Findings in Right Isomerism regarding the following:
- Pulmonary vascularity
- Bronchi
- Minor fissure
- Heart
- Atrium
- Ventricle
- Pulmonary vein
- Great vessels
- SVC
- Bowel
- Spleen
- Abdominal Situs
- IVC
- Pulmonary vascularity
- Decreased (obstructed flow)
- Bronchi
- Eparterial
- Minor fissure
- Bilateral
- Heart
- Cardiomegaly/complex CHD
- Atrium
- Common Atrium
- Single ventricle
- in 50%
- Pulmonary veins
- TAPVC
- Great vessels
- TGA 70%
- SVC
- Bilateral 50%
- Bowel
- Malrotation
- Spleen
- Asplenia
- Abdominal situs
- Ambiguous/inversus
- IVC
- normal
Re Isomerism, which has milder disease/better prognosis?
Which has cyanosis?
Why is this?
Howell–Jolly bodies are found on blood smear in a patient. What does this mean?
What is the dx?
What does survival depend on?
What are the clinical findings?
What are the Rad features?
What is the treatment?
Hypoplastic Left Heart
- Spectrum of cardiac anomalies characterized by:
- underdevelopment of LA, LV, MV, aortic valve, and aorta.
- Survival requires a large ASD and PDA
- with R-L and L-R shunting.
- Clinical Findings
- Neonatal CHF within several days after birth
- Most infants die within first week as PDA closes.
- Cardiogenic shock; metabolic acidosis
- Radiographic Features
- Increased pulmonary vascularity
- Severe pulmonary edema
- Prominent right heart, especially RA
- Treatment
- Norwood procedure: PA-descending
- PA-descending aorta conduit, followed by PA banding; palliative
- Heart transplant: curative attempt
- Case courtesy of Dr Bahman Rasuli, Radiopaedia.org, rID: 67268
What is this condition?
What are the clinical findings?
Cor Triatriatum
- In the classical type, there is abnormal incorporation of pulmonary venous structures into the left atrium with an unnecessary fibromuscular membraneous subdivision through the atrial chamber.
- obstruction to pulmonary venous return; very rare.
Radiographic Features
- Mimics congenital mitral stenosis
- LA size usually normal
Associated lesions:
- Parachute MV
- Mitral web
- PVH and CHF
What is this?
What is it a/w?
Azygos Continuation of the IVC ( Fig. 2.66 )
- Developmental failure of the hepatic and/or infrahepatic IVC. Associated with polysplenia.
- Enlarged azygos vein
- Enlarged hemiazygos vein
- Absent IVC
Case courtesy of Dr Sachintha Hapugoda, Radiopaedia.org, rID: 52176
What syndrome is this?
What are 5 associated features?
AD/AR?
% with cardiac abnormalities?
Marfan Syndrome ( Fig. 2.67 )
- Marfan syndrome
- AD connective tissue disease (arachnodactyly) with cardiac abnormalities in 60%:
- Ascending aorta
- Aneurysm
- Aortic regurgitation (common)
- Dissection
- MV
- Prolapse (myxomatous degeneration)
- Mitral regurgitation
- Coarctation
- Chest deformity, kyphosis
- Arachnodactyly
- Excessive limb length
- Ascending aorta
What syndrome is this?
What are 2 associated CHD a/w this condition?
Turner Syndrome
- Coarctation, 15%
- Bicuspid aortic valve
Short fourth metacarpal bone.
Case Discussion
Hand radiograph of a 9 year old girl with Turner syndrome with marked shortening of the 4th metacarpal and a positive metacarpal sign.
In the metacarpal sign, a line drawn along the heads of the 4th and 5th metacarpals will intersect the head of the 3rd metacarpal if shortening is present. The shortened 4th metacarpal is the key to the sign.
The sign is positive in up to 9.6% of normal individuals 3. It is however seen in a variety of conditions.
For a gamut of this sign see: short 4th/5th metacarpal.
5 signs of left atrial enlargement
- LA measurement (right LA border to LMB >7 cm)
- Barium-filled esophagus is displaced posteriorly (lateral view).
- Double density along right cardiac border; a similar appearance may also be found in:
- Patients with normal-sized LA
- Confluence of pulmonary veins
- Bulging of LA appendage
- Widening of the angle of the carina (>60 degrees)
- Anterior to posterior (A–P) dimension >4 cm on three-chamber cardiac CT/MRI
Case courtesy of Dr Ahmed Abdrabou, Radiopaedia.org, rID: 23098
2 signs of Left ventricular enlargement
Left Ventricle (LV) Enlargement ( Fig. 2.69 B )
- Left downward displacement of the apex (elongation of ventricular outflow tract)
- Round left cardiac border
2 signs of Right ventricular enlargment
- Right Ventricle (RV) Enlargement ( Fig. 2.69 C )
- Rounding and elevation of cardiac apex
- Obliteration of retrosternal space on lateral view;
- normally, more than one-third of the distance from anterior costophrenic angle to the angle of Louis (manubriosternal junction).
2 signs of right atrial enlargement
Right Atrium (RA) Enlargement ( Fig. 2.69 D )
- Difficult to assess by plain radiograph
- Increased convexity of lower right heart border on posteroanterior view
What is the surface area of a normal mitral valve?
What are the xray findings of this condition?
- Mitral stenosis
- Plain radiograph
- PVH in nearly all patients
- Normal overall heart size (pressure overload) but enlargement of LA
- Severe stenosis
- Increase in pulmonary arterial pressure leads to RVH
- Pulmonary hemosiderosis (ossified densities in lower lung fields)
- Calcification of LA wall (laminated clot)
- Cardiac CT
Thickened and calcified leaflets.
Cardiac CT can estimate annular dimension for percutaneous valve replacement.
https://medizzy.com/feed/9550558
What are the 5 causes of Mitral regurgitation?
- Mitral Regurgitation ( Fig. 2.72 )
- Causes
- Rheumatic fever
- MV prolapse (Barlow syndrome)
- Rupture of papillary muscle (secondary to MI, bacterial endocarditis)
- Marfan syndrome
- Bacterial endocarditis
Case courtesy of Dr Mark Holland, Radiopaedia.org, rID: 18796
What are the Rad Features of Mitral Regurgitation?
- Plain radiograph
- “Big heart disease” (volume overload, cardiomegaly)
- Enlarged chambers: LA + LV
- PVH (usually less severe than in mitral stenosis)
- Calcification of mitral annulus: may have J-, C-, or O-configuration
- Often coexistent with mitral stenosis
- US
- MVP
- Enlarged LA, LV
- Cardiac MRI
- Visualization and quantification of regurgitant jet
What are the 3 types and associated causes of Aortic Stenosis?
Aortic Stenosis (AS)
- Types
- Valvular: 60%–70%, most common form
- Degenerative leaflets in patients >70 years
- Bicuspid
- Rheumatic
- Subvalvular, 15%–30%
- Idiopathic hypertrophic subaortic stenosis (IHSS); 50% are AD
- Congenital (membranous, fibromuscular tunnel)
- Supravalvular (rare)
- Williams syndrome
- Rubella
- Valvular: 60%–70%, most common form
- Case courtesy of Assoc Prof Frank Gaillard, Radiopaedia.org, rID: 15391
What are the clinical findings of Aortic Stenosis?
- Clinical Findings
- Symptoms of LV failure (common)
- Angina, 50%; many patients also have underlying CAD
- Syncope (in severe stenosis)
- Sudden death in children, 5%
Case courtesy of Assoc Prof Frank Gaillard, Radiopaedia.org, rID: 15391
What is the normal size of the aortic valve?
Size when symptomatic with exercise?
Size when symptomatic at rest?
What are the rad findings of Aortic Stenosis?
- Plain radiograph
- Often difficult to detect abnormalities by plain radiograph (usually no chamber enlargement)
- Enlargement of ascending aorta (does not occur with supravalvular AS)
- Calcification of aortic valve: rare before age 40
- US
- Multiple aortic valve echoes
- Poststenotic dilatation of aorta
- Doming of the aortic valve
- LVH
- Doppler: velocity measurements
Case courtesy of Assoc Prof Frank Gaillard, Radiopaedia.org, rID: 15391
Chest x-ray shows prominent of the right mediastinal border occupied by the ascending aorta. The descending aorta is unfolded but of normal calibre. Heart size is normal. No lung or pleural abnormality.
What are the causes of Aortic Regurgitation?
4 common
4 rare
Aortic Regurgitation ( Fig. 2.77 )
- Causes
- Rheumatic fever
- Systemic HTN (may lead to dilatation of the aortic root)
- Aortic dissection
- Endocarditis
- Rare causes:
- Marfan syndrome,
- syphilitic aortitis,
- trauma,
- collagen vascular diseases
- (ankylosing spondylitis)
What are the rad findings of Aortic Regurgitation?
Radiographic Features
- Plain radiograph
- Cardiomegaly
- Dilated structures: LV, aorta
- US
- Dilation of LV and aorta
- Atypical valve leaflets
- High-frequency vibrations of the AML
- Cardiac MRI
- Visualize regurgitant jet and quantification of regurgitant volume
Case courtesy of Dr Hani Makky Al Salam, Radiopaedia.org, rID: 10685
Case Discussion
Chest radiographs demonstrate enlargement of the left ventricle with an aortic configuration, reflects adaptive dilatation seen in aortic regurgitation.
What is (DE-CMRI)?
- Delayed-Enhancement Cardiac Magnetic Resonance Imaging (DE-CMRI)
- Technique:
- DE-CMRI involves the administration of Gd
- subsequent T1-weighted gradient-recalled echo imaging 10–30 min later
- with an inversion recovery preparatory pulse
- that nulls normal myocardial signal;
- enhancement characteristic
- wall motion,
- wall thickness,
- and chamber size.
- Technique:
On (DE-CMRI)
What are the typical findings in ischaemia?
- Ischemia
- Delayed enhancement in acute and chronic ischemia involves the subendocardium
- but may extend to involve a variable amount of thickness of the myocardium (transmural)
- Enhancement is seen within a typical territory of a coronary artery
- Enhancement affecting >50% thickness of the myocardium associated with unlikely recovery after revascularization
https://radiologyassistant.nl/cardiovascular/cardiomyopathy/ischemic-and-non-ischemic-cardiomyopathy
What are the DE MRI features in in Nonischemic cardiomyopathies?
Mid wall
Epicardial
Endocardial
- Nonischemic cardiomyopathies
- Enhancement is not restricted to coronary artery distribution
https://www.cfrjournal.com/image-gallery/7972/9089/Figure_1%3A_Late_Gadolinium_Enhancement_Patterns_in_Ischemic_and_Non-ischaemic_Cardiomyopathies
Re the following, what are the features of a true aneurysm?
Myocardial Wall
Angiography
Location
Aneurysm Neck
Cause
Complications
Re the following, what are the features of a False aneurysm?
Myocardial Wall
Angiography
Location
Aneurysm Neck
Cause
Complications
Lipomatous Hypertrophy of the Interatrial Septum
- Benign proliferation of fat in the interatrial septum sparing fossa ovalis (dumbbell shape) with thickness >2 cm.
- May be fluorodeoxyglucose (FDG)-avid on PET.
- Typically in elderly obese patients.
- May cause arrhythmia.
- The septum may show FDG avidity on PET study because of the presence of brown fat.
- Case courtesy of Dr Jeremy Frank, Radiopaedia.org, rID: 21322
- Case courtesy of Dr Yune Kwong, Radiopaedia.org, rID: 29649
Arrhythmogenic Right Ventricular Dysplasia (ARVD)
- Inherited, progressive condition characterized by fatty infiltration of the RV.
- Can cause life-threatening cardiac arrhythmias and sudden cardiac death in young people.
- Prevalence is 1 in 5000.
- Presents as symptomatic sustained ventricular tachycardia.
- ECG shows LBBB pattern.
- MRI: RV hypokinesis, RV enlargement, poor RV EF.
- Fat signal in RV myocardium can be seen but not essential for diagnosis.
- Treatment is with a defibrillator and antiarrhythmic agents.
- Case courtesy of Dr Vlad Barskiy, Radiopaedia.org, rID: 69431
There are multiple aneurysms of the right ventricle (RV) with areas of myocardial transformation.
- Case Discussion
- RV EDV/BSA 154 ml/m2
- RV EF 29%
- Non-compact left ventricular myocardium is also demonstrated.
- Cardiac MRI findings in the right ventricle are compatible with arrhythmogenic right ventricular cardiomyopathy.
https://radiologykey.com/arrhythmogenic-right-ventricular-dysplasia/
Myocardial Noncompaction
- Myocardial Noncompaction
- Congenital cardiomyopathy leading to two-layered ventricular myocardium with prominent trabeculations.
- Ratio of noncompacted to compacted LV myocardium >2.3.
- May observe delayed enhancement by DE-CMRI.
- Associated with thromboembolism, stroke, CHF, and arrhythmia.
- Case courtesy of Dr Azza Elgendy, Radiopaedia.org, rID: 38868
- Case courtesy of Dr Tim Luijkx, Radiopaedia.org, rID: 39933
Takotsubo Cardiomyopathy
- Transient LV apical ballooning syndrome and is a rare entity found more commonly in postmenopausal women after emotional stress.
- No CAD on cardiac catheterization.
- MRI may demonstrate apical ballooning with akinesis or hypokinesis and no delayed enhancement.
- Case courtesy of Dr Gregor Savli, Radiopaedia.org, rID: 25090
Anomalous origin of LCA or RCA from PA
Venous blood flows through LCA, resulting in myocardial ischemia
15% of patients survive into adulthood because of collaterals.
Coronary computed tomography angiography clearly identifies the ALCAPA with a retropulmonary ostium (asterisks, a and b). Volume-rendered image shows the course of the anomalous coronary arteries along the epicardial surface and where the LCA connects to the MPA (c, open arrow, d). Abbreviation as Figs. 1 and 2
Discussion
ALCAPA syndrome is one of the leading etiologies of myocardial infarction in children. The enlarged, tortuous RCA and its collaterals provide a retrograde course to supply the LV and then preferentially empty into the lower pressure pulmonary artery system causing a coronary steal phenomenon. The few patients who survive through adulthood without surgery must have abundant, well-formed inter-coronary collaterals with retrograde perfusion to the LV from the RCA. Some of the late-presenting patients have a narrowing of the LCA ostium, as demonstrated in our case, which served as a protective mechanism against myocardia ischemia by limiting the steal effect and increasing myocardial perfusion pressure [4]. Symptomatic adult patients with ALCAPA syndrome may present with myocardial infarction, left ventricular dysfunction or significant MR.
https://cardiothoracicsurgery.biomedcentral.com/articles/10.1186/s13019-018-0751-4
