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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