CARDIAC IMAGING 2 Flashcards

Question

Which Chambers/arteries/vessles are enlarged/changed in Ebsteins' Anomaly?

Answer 1

CHD With PVH/CHF 1. Cor triatriatum 2. Mitral stenosis 3. Hypoplastic left heart syndrome (HLHS) 4. Coarctation of the aorta 5. Cardiomyopathy 6. Primary endocardial fibroelastosis 7. Anomalous ICA 8. Obstructed TAPVC

Answer 2

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.

Answer 3

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)

Answer 4

* 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

Answer 5

Radiographic Features * PA enlargement if associated valvular stenosis * RV hypertrophy

Answer 6

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

Answer 7

* Complete agenesis of the tricuspid valve with no direct communication of the RA and RV

Answer 8

* 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.

Answer 9

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.

Answer 10

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 Case courtesy of Senior Radiographer 2 Elton John Decena, RRT., Radiopaedia.org, rID: 85039

Answer 11

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.)

Answer 12

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

Answer 13

* Cardiomegaly * Ebsteins * Pulomary atresia * Tricuspid * No Cardiomegaly * Tetralogy of Fallot

Answer 14

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%

Answer 15

* Associations * ASD, 15% * (especially the **sinus venosus defect**)

Answer 16

Pulmonary arterial hypertension (PAH) * Large main PA * Usually normal hilar arteries

Answer 17

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)

Answer 18

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

Answer 19

Increased incidence in males: 1. AS 2. coarctation 3. pulmonary atresia 4. tricuspid atresia 5. hypoplastic left heart 6. TGA

Answer 20

Tetralolgy of Fallot

Answer 21

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.

Answer 22

Pink Tetralogy VSD with mild pulmonic stenosis

Answer 23

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

Answer 24

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

Answer 25

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: 1. ventricular septal defect (VSD) 2. right ventricular outlfow tract narrowing or complete obstruction 3. right ventricular hypertrophy 4. overriding aorta 5. atrial septal defect (ASD) or patent ductus arteriosus (PDA) Case courtesy of Dr Praveen Jha, Radiopaedia.org, rID: 19288

Answer 26

* Associations * Patent foramen ovale or ASD is always present. * D-TGA, 35% * VSD common * Pulmonary atresia * Hypoplastic right heart * Extracardiac anomalies (GI, bone)

Answer 27

1. Ventricular septal defect 30% 2. Atrial septal defect 10% 3. Tetralogy of Fallot 10% 4. Patent ductus arteriosus 10% 5. Coarctation of aorta 7% 6. Transposition of great arteries 5%

Answer 28

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

Answer 29

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

Answer 30

* Coarcation * Interupted aortic arch (IAA) * Aortic Stenosis * Pulmonary Stenosis

Answer 31

Coarctation of Aorta Types ( Fig. 2.34 ) * Infantile type (diffuse type, preductal): tubular hypoplasia * Adult type (localized type, postductal, periductal): short segment; common

Answer 32

Associations ( Fig. 2.24 ) * Holt–Oram syndrome: ostium secundum defect * Lutembacher syndrome: ASD and mitral stenosis * Down syndrome: ostium primum defect

Answer 33

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

Answer 34

Normal vascularity Right main PA = same size as trachea at level of AA Peripheral arteries (seen on end) = same size as adjacent bronchus

Answer 35

Eisenmenger pulmonary vasculature 1. Combination of shunt and PAH 2. Aneurysmal hilar arteries 3. 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

Answer 36

Associations 1. Coarctation syndrome: * triad of * coarctation, * PDA, * VSD 2. In Turner syndrome, **most common cardiac abnormality** 3. Bicuspid aortic valve, 50% 4. Hypoplasia of the aortic isthmus (small arch) 5. Circle of Willis aneurysms 6. PDA aneurysm 7. Intracardiac defects; occur in 50% with infantile type 8. **Marfan** syndrome

Answer 37

Clinical Findings * Most are asymptomatic. * Large defects lead to CHF at 2–3 months of age.

Answer 38

Types * Membranous, 80% * Muscular, 10% * AV, 5% (T21) * Conal, 5% (mostly supracristal)

Answer 39

Maternal Lithium intake

Answer 40

* 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 ).

Answer 41

Tetralogy of Fallot ( Fig. 2.36 ) Most common cyanotic CHD of childhood Tetrad 1. Obstructed RV outflow tract 2. Right ventricular hypertrophy (RVH) 3. VSD 4. Aorta overriding the IVS

Answer 42

* 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

Answer 43

Treatment * Most stenoses are amenable to balloon valvuloplasty. * Surgical reconstruction may be necessary for dysplastic types.

Answer 44

**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.

Answer 45

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.

Answer 46

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

Answer 47

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

Answer 48

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).

Answer 49

* 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

Answer 50

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

Answer 51

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

Answer 52

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)

Answer 53

* Enlarged left atrium * PDA * VSD * Normal Left Atrium * ASD * Endocardial Cushion defect

Answer 54

* 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/

Answer 55

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

Answer 56

Hemodynamics There is an L–R shunt because the pressure in the aorta is higher than in the pulmonary circulation.

Answer 57

Transposition of the great arteries (TGA) occurs in approximately 30% of patients with tricuspid atresia.

Answer 58

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

Answer 59

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

Answer 60

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

Answer 61

* 5 T's * TGA * TA * Tingle Ventricle * TAPVR * Tricuspid atresia

Answer 62

Hemodynamics All blood crosses through a large ASD into the LA.

Answer 63

* 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.

Answer 64

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

Answer 65

* Highest incidence of PDA occurs in premature infants (especially if hyaline membrane disease is present) and in maternal rubella; females \> males

Answer 66

* Plain radiograph * Poststenotic dilatation of main and/or left PA (jet through stenosed valve dilates PA) * Right PA of normal size * RV (hypertrophy)

Answer 67

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

Answer 68

* 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

Answer 69

* 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

Answer 70

Ebsteins Anomaly Box shaped heart. Case courtesy of Dr Jeremy Jones, Radiopaedia.org, rID: 24348

Answer 71

* Blood flows from LA to RA

Answer 72

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