Table 2.
Clinical information of the reported cases
| patient # | Gender | Ethnicity | Birth weight (kg) | Mother’s age, pregnancy | Gestation (weeks) | Apgar 1 & 5 min | Delivery | Life span (days) | Diagnosis | Medications | ECMO | Associated anomalies |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | Caucasian | 2.06 | 28y, G2P2 | 37 & 3/7 | 8, 9 | Vaginal | 29 | Autopsy | Sildenafil | No | Incomplete rotation of bowel, high arched palate, hypocalvaria, incomplete lung lobation, incomplete rotation of bowel |
| 2 | F | Hispanic | 2.8 | 26y, G3P2 | 39 & 5/7 | 8, 9 | Vaginal | 18 | Autopsy | Nitric Oxide, Milrinone, Dopamine, Fentanyl | Yes | Malrotation of intestine, imperforate anus, butterfly vertebra |
| 3 | M | Caucasian | 3.3 | 22y, G4P2 | 36 | 9, 9 | Vaginal | 35 | Autopsy | N/A | Yes | Aortic coarctation, Meckel’s diverticulum |
| 4 | F | Hispanic | 3.6 | 28y, G3P2Ab1 | 39 | 9, 9 | Elective c-section | 16 | Autopsy | N/A | Yes | Bilateral incomplete lobation of lungs, lymphangiectasis, limited autopsy |
| 5 | M | Caucasian | 3.0 | 38 & 2/7 | 9, 9 | Elective c-section | 5 | Autopsy | Nitric oxide, Milrinone,, Sildenafil, Epinephrine, Dobutamine, Neuromuscular blockers | Yes | Abnormal lung lobation, malrotation of the intestine, duodenal atresia | |
| 6 | F | Caucasian | 3.2 | 28y, G2P1 | 39 | 8, 9 | Vaginal | 28 | Biopsy | Nitric oxide, Milrinone, Epoprostenol | Yes | No autopsy |
| 7 | M | Hispanic | 2.7 | 25y, G1P0→1 | 36 & 4/7 | 8, 9 | Vaginal | 10 | Biopsy | Nitric oxide, Dopamine, Milrinone, Sildenafil, Epinephrine, hydrocortisone | Yes | None identified |
| 8 | F | Caucasian | 3.3 | 28y, G3P2 | 39 | 6, 9 | Vaginal | 120 | Autopsy | Sildenafil | No | Bilobed right lung |
| 9 | F | Caucasian | 4.02 | 38y, G2P1 | 38 | 8, 9 | C-section for polyhydramnios | 10 | Autopsy | Nitric oxide, morphine, dopamine | Yes | None identified |
| 10 | F | African American | 3.1 | 25y, G2P2 | 40 & 4/7 | 8, 9 | Vaginal | 14 | Autopsy | Nitric oxide, epinephrine, dopamine, epoprostenol, Sildenafil | Yes | None identified, Limited autopsy |
| 11 | F | Caucasian | 3.7naline | 38y, G2P1→2 | 40 | 8, 9 | Vaginal | 95 | Nitric Oxide, Sildenafil, Epoprostenol, Milrinone, Hydrocortisone, Morphine, | Yes | Atrial septal defect | |
| 12 | F | Caucasian | 4.1 | N/A, G1P1 | 42 | N/A | Vaginal | 111 | Biopsy | Nitric Oxide, Sildenafil | Yes | N/A |
| 13 | F | Caucasian | 3.2 | 39y, G1P1 | 39 | 10, 10 | Vaginal | 42 | Biopsy | Sildenafil, Epoprostenol, Milrinone, Dopamine, Epinephnire, Norepinephrine | Yes | No lung lobation |
| 14 | M | Caucasian | 3.3 | 26y, G1p1 | 41 & 2/7 | 9, 10 | Vaginal | 15 | Autopsy | Nitric oxide, epoprostenol, surfactant and catecholeamines | No | None identified |
| 15 | M | Caucasian | 3.0 | 36y, G2P1 | 39 | 8, 9 | C-section (h/o prior c-section) | 7 | Biopsy | Nitric oxide, Milrinone, dopamine, epoprostenol | No | Malrotation of the intestine, atrial septal defect, thickened distended bladder with muscular hypertrophy |
| 16 | M | Caucasian | 2.1 | 31y, G3P2 | 32 | 4, 8 | Vaginal | 0.75 | Autopsy | Dopamine, epinephrine, fentanyl | No | Hyaline membrane disease, patent ductus arteriosus, multiple intestinal atresias, imperforate anus, undescended testis, Hirschprung disease |
| 17 | F | Caucasian | 1.4 | 30y, G1P1 | 33 & 3/7 | 10, 10 | Vaginal | 7 | Autopsy | Epoprostenol | No | Duodenal atresia, polysplenia, annular pancreas and left hydronephrosis |
| 18 | M | Caucasian | 2.8 | 42y, G3P3 | 38 | 9, 10 | Vaginal | 19 | Biopsy | Nitric Oxide, Milrinone, Dopamine, Ambrisentan | Yes | Hydronephrosis, partial stenosis at transition to colon transversum |
| 20 | M | Caucasian | 26y, G1P0→1 | 40 | 8, 8 | Vaginal | 51 | Autopsy | Nitric oxide, dopamine, epinephrine, Fentanyl, | Yes | Malrotation of the intestine, hydronephrosis, bilateral hydroureter, enlarged and thick bladder | |
| 22 | M | Asian | 3.3 | 39y, G2P0→1 | 38 & 2/7 | 8, 9 | C-section | 16 | Clinical characteristic and genetic mutation | Nitric Oxide, epinephrine, dopamine, Fentanyl, Milrinone, Sildenafil | Yes | N/A |
| 23 | F | Caucasian | 2.8 | 15y, G1P1 | 36 | 5, 9 | Vaginal | 15 | Autopsy | Nitric Oxide, dobutamine, norepinephrine, sildenafil, epoprostenol | No | Duodenal stenosis, annular pancreas, malrotation of large intestine |
| 24 | F | Caucasian | 2.9 | 24y, G1P0 | 39 & 4/7 | 4, 7 | Vaginal | 11 | Biopsy | Dobutamine, Dopamine, Epinephrine, Milrinone | Yes | Meckel’s divurticulum, moderate acute cortical involution of thymus |
| 25 | F | Caucasian | 2.7 | 33y, G4P3→4 | 37 & 5/7 | 7, 9 | Vaginal | 19 | Autopsy | Nitric oxide, Noradrenalin, Milrinone, Dobutamine | Yes | Intestinal malrotation |
| 26 | M | Caucasian | 3.5 | 38, G4P1 | 39 | 9, 9 | Vaginal | 21 | Biopsy | Nitric oxide | No | Megaduodenum, pyloric stenosis, annular pancreas |
| 27 | F | Caucasian | 2.7 | 34y, G1P1 | 37 & 2/7 | 8, 8 | Vaginal | 16 | Biopsy | Nitric oxide, Sildenafil, Bosentan | Yes | None identified |
| 28 | M | Caucasian | 2.6 | 34y, G5P1 | 39 | 9, 9 | C-section | 19 | Autopsy | Nitric oxide, Milrinone, Sildenafil, Noeepinephrine | No | Duodenal atresia |
| 29 | F | Caucasian | 2.4 | 20y, G1P1 | 34 &5/7 | 8, 9 | Vaginal | 12 | Biopsy | Nitric oxide, dopamine, dobutamine, hydrocortisone | Yes | Suspected jejuna atresia, no autopsy |
| 30 | F | Caucasian | 2.9 | 34y, G1P0→1 | 39 &6/7 | 7, 9 | Vaginal | 15 | Autopsy | Dopamine, dobutamine, hydrocortisone | Yes | Thickening of the pylorus |
| 31 | M | Asian | 3.5 | 24, G1P1 | 38 | N/A | Vaginal | N/A | Biopsy | Nitric oxide, furosemide, nifedipine | No | Posterior urethral valves |
| 32 | F | African American | 1.7 | 37, G5P3→4 | 34 | 8, 9 | Vaginal | N/A | Biopsy | Nitric oxide, sildenafil, milrinone | No | Atrial septal defects |
| 33 | F | Caucasian | 3.2 | 37, G2P2 | 37 | 7, 9 | C-section | 24 | Biopsy | Nitric oxide | No | Butterfly vertebra T5 |
| 34.1 | M | Caucasian | 0.5 | 29 | Terminated at 23 4/7 | N/A | Vaginal | N/A | Autopsy | N/A | No | Duedenal atresia, malrotation of the large intestine, anal atresia, gallbladder agenesis |
| 34.2 | M | Caucasian | 2.6 | 30 | 39 3/7 | N/A | Vaginal | 17 | Autopsy | Nitric oxide, sildenafil, bosentan, dobutamine, adrenaline, noradrenaline | Yes | None detected |
| 35 | F | Caucasian | 2.8 | 30y, G3 P1 | 38 & 5/7 | 3, 9 | Elective C-section | 50 | Clinical characteristic and genetic mutation | Dobutamine, Milrinone, Bosentan, Sildenafil | No | Atrial septal defect, polyhydramnios |
| 36 | M | Asian/Caucasian | 3.0 | 31y, G3P3 | 37 & 5/7 | 7, 8 | C-section (breech presentation) | 2 | Autopsy | Dobutamine, dopamine, adrenaline, noeepinephrine | No | Atrial septal defect, polyhydramnios |
| 37.1 | F | Caucasian | 3.4 | 25y, G2P1 | 39 | 9, 9 | Vaginal | 17 | Autopsy | Nitric oxide, dopamine, dobutamine, tolazolin | No | N/A |
| 37.2 | M | Caucasian | N/A | 25y | Terminated at 21 weeks | N/A | Vaginal | N/A | Autopsy | N/A | No | Uretral stenosis, hydronephrosis, |
| 37.3 | M | Caucasian | 3.2 | 27y, G4P2 | 37 & 1/7 | 9, 10 | Vaginal | 0.166 | Autopsy | Surfactants & Catecholamines | No | Pulmonary hypoplasia, hydrothorax, polyhydramnios |
| 37.4 | F | Caucasian | 3.2 | 31y, G5P3 | 39 | 9, 9 | Vaginal | 1 | Autopsy | Nitric oxide, Catecholamines | No | Isolated arhinencephaly |
| 37.5 | M | Caucasian | N/A | 36y | Terminated at 22 weeks | N/A | Vaginal | N/A | Autopsy | N/A | No | Obstructive uropathy |
N/A Not available. Clinical information of cases 19 and 21 are not available. The clinical information of cases 38 – 41 have been presented in Stankiewicz et al 2009.