Table 2 Prenatal findings and outcome.
| GA at first presentation w+d | Type of DIV | Malposition of the great arteries | Out-flow tract obstruction | Outlet chamber/position | Additional cardiac anomalies | Additional extra-cardiac anomalies | Diagnosis/obduction | Deliv-ery | GA at birth w+d | Birth weight (g) | Percen-tile weight/length | Out-come | Surgery | Age at death or last follow-up (years/months) | Long-term outcome | |
| Abbreviations: ALSA: aberrant left subclavian artery; BT shunt: Blalock-Taussig shunt; CoA: aortic coarctation; CD: caesarean delivery; DCDA twins: dichorionic diamniotic twins; DILV: double inlet left ventricle; DIRV: double inlet right ventricle; DIIV: double inlet indeterminate ventricle; DKS: Damus-Kaye-Stansel procedure; D-MGA: dextro-malposition of the great arteries; INFD: infant death; ICV: interrupted inferior caval vein; IVC: interventricular communication; L-MGA: levo-malposition of the great arteries; LPSCV: left persistent superior caval vein; LV: left ventricle; MAPCA: major aorto-pulmonary collateral artery; NND: neonatal death; Pa: pulmonary artery; PA: pulmonary atresia; PAB: pulmonary artery banding; PCPC: partial cavo-pulmonary connection; PM: pacemaker; RAA: right aortic arch; RPA: right pulmonary artery; RV: right ventricle; SUA: single umbilical artery; TCPC: total cavo-pulmonary connection; TOP: termination of pregnancy; TP: pulmonary trunk; VB: vaginal birth; valv. PS: valvular pulmonary stenosis | ||||||||||||||||
| 1 | 16+3 | DILV | D-MGA | PS | RV/right | CoA | – | DILV, D-MGA, peripheral PS, preductal CoA | VB | 18+4 | – | TOP | – | – | – | |
| 2 | 24+5 | DIRV | D-MGA | PS | DORV/right | Severe CoA | – | DIRV+DORV, D-MGA, PS, subpulmonary VSD, aortic arch atresia | VB | 26+0 | – | TOP | – | – | – | |
| 3 | 21+6 | DIRV | L-MGA | – | DORV/right | – | – | DIRV+DORV, L-MGA | VB | 23+5 | – | TOP | – | – | – | |
| 4 | 14+1 | DILV | no | – | RV/right | – | Left-sided renal agenesis | DILV – Holmes heart, left-sided renal agenesis | VB | 17+2 | – | TOP | – | – | – | |
| 5 | 20+1 | DILV | L-MGA | – | LV/left anterior | Narrow aortic arch | – | DILV, L-MGA, narrow aortic arch | VB | 20+2 | – | TOP | – | – | – | |
| 6 | 18+4 | DIRV | L-MGA | valv. PS | LV/left | LPSCV | – | DIRV, L-MGA, valv. PS, LPSCV | VB | 19+4 | – | TOP | – | – | – | |
| 7 | 18+1 | DIRV | D-MGA | – | DORV/right | – | – | DIRV+DORV, D-MGA | VB | 21+1 | – | TOP | – | – | – | |
| 8 | 26+1 | DILV | no | PA | RV/right | – | SUA | DILV – Holmes heart, PA | VB | 28+1 | – | TOP | – | – | – | |
| 9 | 29+2 | DILV | D-MGA (not side-by-side) | PS | DOLV/left | – | – | DILV+DOLV, D-MGA, PS | – | – | – | – |
Lost to follow-up |
– | – | – |
| 10 | 22+5 | DILV | D-MGA | – | RV/right | – | – | DILV, D-MGA | VB | 39+3 | 4260 | 95/64 | NND | Mod. Norwood (aortic arch reconstruction by patch-plasty, DKS, Sano-shunt) | 16 days | |
| 11 | 20+5 | DILV | L-MGA | – | RV/left anterior | Hypo-plastic aortic arch | – | DILV, L-MGA, hypo-plastic aortic arch | VB | 36+5 | 3180 | 65/38 | INFD | Mod. Norwood with patch extension of the aortic arch into the desc. Ao, BT shunt to the RPA, atrioseptectomy |
3 months | |
| 12 | 28+0 | DIIV | L-MGA | – | Left | – | – | DIIV with L-MGA, mild subaortic obstruction | CD | 38+0 | 2900 | 26/27 | Alive | BT shunt, atrioseptectomy, ligation of DA, DDD-PM | 0 3/12 | Postoperative AV block III; next check-up in 2 weeks |
| 13 | 22+1 | DIRV | no | – | LV/left | – | – | DIRV with normally related great arteries | CD | 35+4 | 3900 | >97/93 | Alive | BT shunt | 0 4/12 | 70cm, 7,4kg; PCPC planned |
| 14 | 27+4 | DILV | no | – | RV/right | LPSCV | Gallbladder duplication | DILV – Holmes heart – with narrow (antegrade) TP from the rud. outlet chamber | CD | 39+3 | 3534 | 50/79 | Alive | Bilateral PAB, epimyocardial PM | 0 4/12 | 63cm, 5,6kg; post-interventional sinus-bradycardia; next check-up in 4 weeks |
| 15 | 23+5 | DILV | MGA | PA | Left | Dextro-cardia with mirror imaging | DCDA twins, SUA | Mirror image dextrocardia; DILV; outflow tract lying on left via rud. outlet chamber into aorta; PA with hypopl. TP right parallel to aorta; native Pa´s and MAPCAs | VB | 38+4 | 2315 | 7/22 | Alive | None so far | 0 11/12 | 52cm, 3kg; central pulmonary arteries too small for Fontan circulation, so compassionate care requested by parents |
| 16 | 30+5 | DILV | D-MGA | PS | Right | – | SUA | DILV, D-MGA, PS, right AV insufficiency | CD | 38+1 | 3130 | 32/62 | Alive | AV valve reconstruction, PAB, PCPC | 1 1/12 | 82cm, 11,4kg, BMI 17, normal development, no signs of cardiac insufficiency |
| 17 | 25+6 | DILV | L-MGA | – | RV/left anterior | – | – | DILV- L-MGA | CD | 38+5 | 3100 | 32/52 | Alive | Norwood-Sano, DKS, PCPC | 1 3/12 | 74cm, 11kg, BMI 20 |
| 18 | 28+1 | DILV | L-MGA | – | RV/left anterior | – | – | DILV- L-MGA | VB | 39+3 | 3500 | 46/64 | Alive | Norwood-Sano, DKS; atrioseptectomy, PCPC | 1 7/12 | Lean nutritional condition, no signs of cardiac insufficiency |
| 19 | 25+6 | DIIV | L-MGA | PA | Left | – | Azygos contin-uation, interrupted ICV | DIIV, L-MGA, PA, Azygos continuation, interrupted ICV | CD | 39+3 | 3050 | 20/60 | Alive | BT shunt, Kawashima | 1 10/12 | 85cm, 10,8kg, BMI 15, good development. Heterotaxy excluded. She is thriving, runs freely, and speaks well |
| 20 | 24+2 | DILV | D-MGA | – | RV/right anterior | Severe CoA, AV block III | – | DILV, D-MGA, severe CoA, AV block III | CD | 38+0 | 3150 | 36/18 | Alive | Norwood-Sano, PAB; atrioseptectomy, PM implantation | 1 11/12 | 84cm, 11kg, BMI 15,6, normal development, no signs of cardiac insufficiency |
| 21 | 25+4 | DILV | D-MGA | – | RV/right | CoA | – | DILV, D-MGA, CoA | VB | 40+0 | 3910 | 74/86 | Alive | Norwood-Sano with aortic arch reconstruction and atrioseptectomy, PCPC | 4 8/12 | 104cm (44. Perc), 16kg, BMI 14.8; age-appropriate resilience; poor tooth status; Fontan planned, after dental restoration |
| 22 | 21+6 | DILV | D-MGA | – | RV/right anterior | Hypoplastic aortic arch, LPSCV | – | DILV, D-MGA, hypoplastic aortic arch, LPSCV | VB | 37+2 | 3650 | 91/40 | Alive | Norwood-Sano, atrioseptectomy, PCPC + ligation of LPSCV, TCPC | 5 0/12 | 109cm (31. Perc), 18.6kg; BMI 15.7; slight physical limitations, speech development delay |
| 23 | 22+6 | DILV | D-MGA | PA | RV/left anterior | RAA, ALSA | – | DILV, D-MGA, PA, MAPCAs, RAA, ALSA | VB | 37+0 | 3210 | 59/83 | Alive | Modif. BT shunt, PCPC, TCPC | 6 8/12 | 121cm (49. Perc), 21kg, BMI 15.71, no physical limitations, asymptomatic SarsCoV2 infection |
| 24 | 25+1 | DILV | D-MGA | – | RV/right anterior | Severe CoA | – | DILV, D-MGA, severe CoA | CD | 38+1 | 2820 | 12/8 | Alive | Modif. BT shunt, PCPC, TCPC | 8 1/12 | Normal development, slight physical limitations |
| 25 | 20+1 | DILV | L-MGA | – | RV/right anterior | Severe CoA | – | DILV, L-MGA, severe CoA | VB | 39+2 | 3450 | 43/39 | Alive | Modif. Norwood + BT shunt, PAB, PCPC, TCPC, DDD-PM |
8 6/12 | 122cm (7. Perc), 29kg, BMI 19,5, AV block III, likes to go to school, slight physical limitations |
| 26 | 36+4 | DIRV | MGA | – | DORV/right | Mitral dysplasia | – | DIRV+DORV, MGA | CD | 38+5 | 3400 | 60/34 | Alive | Norwood-Sano, DKS, PCPC, TCPC | 8 9/12 | 142cm (86. Perc), 35.1kg; BMI 17.4; started school 1 year later, now in 2nd grade primary school, almost normal everyday resilience, ADHD |
| 27 | 22+0 | DILV | No | – | RV/right | – | – | DILV – Holmes heart | CD | 34+0 | 1980 | 20/92 | Alive | PCPC, TCPC | 9 4/12 | 132cm (13. Perc), 28.9kg; BMI 16.6, normal intelligence, slight physical limitations |
| 28 | 22+0 | DILV | D-MGA | PS | RV/right anterior | RAA | – | DILV, D-MGA, PS, RAA | CD | 38+1 | 3080 | 39/6 | Alive | PCPC, TCPC | 9 10/12 | The basic development, including the school situation (high school), is positive; slight physical limitations |
| 29 | 23+1 | DILV | D-MGA | – | RV/right anterior | RAA, ALSA | – | DILV, L-MGA, PS | CD | 37+5 | 3420 | 65/36 | Alive | BT shunt, PAB, PCPC, TCPC | 10 1/12 | 134cm (12. Perc), 25kg; BMI 13.9, asymptomatic SarsCov2 infection, normal intelligence, slight physical limitations, delayed speech development (his parents are both deaf) |
| 30 | 20+1 | DILV | D-MGA | PA | RV/left anterior | Dextro-cardia | – | DILV, D-MGA, PA, dextrocardia in situs solitus, MAPCA | CD | 38+0 | 3220 | 14/71 | Alive | BT shunt, closure of MAPCA, PCPC, TCPC with atrial PM | 12 3/12 | 32.7kg, sinus bradycardia after PCPC; slight physical limitations, 7th grade secondary school, hereditary spherocytosis (asymptomatic) |
| 31 | 26+5 | DILV | L-MGA | valv. PS | RV/left anterior | Dextro-cardia | – | DILV with anterior outlet chamber, dextrocardia, L-MGA, valvular PS, hypoplastic IVC | VB | 38+6 | 3220 | 29/<3 | Alive | BT shunt, PAB, atrioseptectomy, PCPC, TCPC | 13 2/12 | 148cm (9. Perc.), 50.8kg, BMI 23.19, bilateral diaphragm paresis, slight physical limitations, 7th grade secondary school |
| 32 | 23+0 | DIRV | D-MGA | valv. PS | DORV/right | – | – | DIRV+DORV, D-MGA, valv. PS | VB | 40+6 | 4570 | >97/80 | Alive | PCPC, TCPC | 14 0/12 | 164cm (36. Perc), 55kg, BMI 20.4, is now in 8th grade, dyslexia, IQ129, depressive episode with school refusal during Covid pandemic, physically well |
| 33 | 31+3 | DILV | D-MGA | – | RV/left anterior | Severe CoA | – | DILV, D-MGA, severe CoA | VB | 39+2 | 3630 | 60/72 | Alive | Norwood-Sano, PCPC, TCPC | 15 8/12 | 152cm (<3. Perc), 37kg, BMI 16; behind in growth, slight physical limitations, no pubertal development so far, currently probably failing Fontan with progressive edema |