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 (1.3Â mm) and branch PAs, MAPCAs arising from LSCA & DAo | Balloon angioplasty of PAs, BT shunt deferred until patient is ~3Â 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 (1.7Â 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 |