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Table 2 Case examples highlighting the clinical impact of FE-MUSIC CMR on the management of neonates and infants with congenital heart disease

From: 4D MUSIC CMR: value-based imaging of neonates and infants with congenital heart disease

Pt

Weight (kg)

Pre-MUSIC diagnosis

Post-MUSIC diagnosis

Management and impact on patient care

1

2.2d

TOF-PA, discontinuous PAs

TOF-PA, right-sided aortic arch with diminutive MPA (1.3 mm) and branch PAs, MAPCAs arising from LSCA & DAo

Balloon angioplasty of PAs, BT shunt deferred until patient is ~3 kg

2

2.6

TOF-PA, ?discontinuous PAs

TOF-PA with discontinuous PAs, MAPCAs supplied RPA, ductus/APCs from distal abdominal aorta supplies LPA

Unifocalization of PAs, patch angioplasty at small MPA/RPA juncture, and modified left BT shunt

3

3.3

D-TGA with VSD, double aortic arch, sub-PS/PS

D-TGA, VSD, double aortic arch with tracheo-esophageal compression, sub-PS/PS. Incomplete tracheal rings.

Underwent balloon atrial septostomy; subsequent staged surgery with modified right BT shunt, division of DAA

4

2.5ac

Hypoplastic aortic arch; VSD

Severe aortic arch hypoplasia, near IAA, VSD, normal LV volume/size

VSD closure and aortic arch augmentation

5

3.5

TOF-PS

TOF-PS, hypoplastic MPA continuing as RPA. No APCs. LPA comes from transverse aorta. LCA originates from LCC and courses between RPA and aorta without compression

Staged unifocalization of LPA aided by visualization of unconventional coronary course

6

1.5ab

PV dysplasia, moderate PR, bicuspid AV, severe RVH, ?AP window

PV dysplasia, severe PR, anomalous RCA from LCC with acute anterior angulation, severe RVH. Left-sided aortic arch. No AP window seen

Unsuccessful PDA closure. Patient expired prior to surgery. Autopsy confirmed MUSIC findings.

7

3.6

HLHS,?pulmonary vein stenosis

HLHS, large PAs, no pulmonary vein stenosis. Preserved ventricular function. Large APC from DAo

Occlusion of APC and ductal stenting prior to hybrid Norwood with bilateral banding of PAs

8

2.8d

SV/heterotaxy with PA, ?APCs, PAPVR vs TAPVR

SV/heterotaxy, TAPVR, hypoplastic PAs with MAPCAs

Ductal stenting; No surgery

9

1.5

Parachute MV, bicuspid AV with AS, aortic coarctation

Parachute MV, hypoplastic LV, bicuspid AV with severe AS, hypoplastic aortic arch

ABVP and BAS, Subsequent aortic arch repair

10

2.6

TOF-PA

TOF-PA, confluent branch pulmonary arteries. No MAPCAs.

BT shunt, ductal ligation

11

2.4d

TOF-PA, Unclear PAs anatomy

TOF-PA, absent MPA, tortuous L/RPA, MAPCAs from proximal left vertebral artery to LPA, MAPCAs from RSCA to RPA. Severe RPA hypoplasia (1.7 mm)

Left subclavian collateral stenting. Small PAs size led to stenting and deferring unifocalization

12

4.2a

VSD, aortic arch and branching not well seen

VSD, vascular ring with right aortic arch and aberrant left brachiocephalic artery coursing posteriorly, inferiorly behind esophagus and trachea. No tracheal compression.

VSD closure, division of vascular ring. Extracardiac characterization of vascular ring’s unusual course facilitated surgical planning; surgery occurred earlier because of VSD

13

3.2a

Double aortic arch, large VSD

IAA with LPA & LSCA arising from left branch of hypoplastic AA, large VSD

VSD closure, IAA repair, LPA reimplantation rather than ring division

14

2.3

PAPVR, aortic arch hypoplasia

Scimitar syndrome with right-sided pulmonary sequestration; aortic arch hypoplasia

Occlusion of APCs, Surgical aortic arch repair

15

3.1

TOF-PA, LPA not well seen

TOF-PA, confluent branch PAs with discrete LPA stenosis, no MAPCAs

Surgery rather than watchful waiting. BT shunt with plasty of PAs rather than shunt only.

16

2.6

TOF-PA, LPA not well seen, ?APCs

TOF-PA, severe LPA stenosis, no MAPCAs

BT shunt with LPA plasty rather than watchful waiting

17

3.5a

Hypoplastic aortic arch

Double aortic arch forming complete vascular ring without tracheal or esophageal compression

Division of vascular ring rather than coarctation repair

18

2.1c

IAA/VSD, large PDA, hypoplastic bicuspid AV

IAA/VSD, large PDA, hypoplastic bicuspid AV; predominant flow thru VSD determined final surgical decision

Rastelli-type VSD closure with RV- PA conduit, Damus-Kaye-Stansel arch reconstruction

19

12.7

TOF/PA s/p repair (RV-PA conduit, VSD closure), MAPCAs s/p coil occlusion, RPA stenting

TOF/PA s/p unifocalization. No significant APCs. Findings of markedly diminished perfusion and arterial vascularity in the left lung base along with diminutive and pruned PAs to the LLL as well as dynamic compression of the LIPV determined surgical course

RV to pulmonary artery conduit replacement, aortic homograft, RPA stent removal, LPA repair

20

7.7

TOF/PS, double aortic arch with vascular ring, PAPVR

TOF/PS, double aortic arch with vascular ring. No compression of airways. 3D visualization of the PAPVR (left superior vertical vein joining the LSPV to the left innominate vein/subclavian vein junction) facilitated surgical approach and planning.

TOF repair, division of vascular ring, ligation of levoatrial cardinal vein

  1. aDiscordant echo/MUSIC findings (n = 5)
  2. bDiscordant catheterization/MUSIC findings (n = 1)
  3. cChange from single ventricle to biventricular repair or vice-versa (n = 2)
  4. dPercutaneous transcatheter intervention in lieu of immediate high risk cardiothoracic surgery (n = 3)
  5. AA ascending aorta, ABVP aortic balloon valvuloplasty, ASD atrial septal defect, AV aortic valve, BAS balloon atrial septostomy, BT blalock-taussig, DAo descending aorta, IAA interrupted aortic arch, LCA left coronary artery, L (R) PA left (right) pulmonary artery, L (R) (I) (S) PV left (right) (inferior) (superior) pulmonary vein, L (R) SCA left (right) subclavian artery, LV left ventricle, MAPCAs major aortopulmonary collateral arteries, MPA main pulmonary artery, MV mitral valve, NA not applicable, PA pulmonary atresia, PAPVR partial anomalous pulmonary venous return, PA pulmonary atresia, PAs pulmonary arteries, PDA patent ductus arteriosus, PFO patent foramen ovale, PH pulmonary hypertension, PV pulmonic valve, RCA right coronary artery, RV right ventricle, SV single ventricle, TAPVR total anomalous pulmonary venous return, TGA transposition of the great arteries, TOF tetralogy of fallot, VSD ventricular septal defect