TABLE II.
New Patients With Isolated Bochdalek Congenital Diaphragmatic Hernia Associated With CVMs from the Brigham-Women’s Hospital (BWH), Boston, 1972–2002. CVM Frequency Compared to Population-Based Studies
| Type of CVMa | Study |
|||
|---|---|---|---|---|
| BWH |
BWIS, Ferencz et al., 1997 |
Calzolari et al., 2003 |
Comparison of BWH and Calzolari et al. |
|
| Hospital-based surveillance | Population-based surveillance, USA includes syndromes | Population-based surveillance, Italy excluding chromosome and “recognized” syndromes | Chi square analysis, P-value | |
| No. (%) | No. (%) | No. (%) | ||
| Total CDH | 203 | Not applicable | Not applicable | |
| Total CVMs | 23 (11) | 4304 | 1328 | |
| “Early” CVMs (developmental error <4 weeks gestational age) | ||||
| Single ventricle | 1 (4) | 18 (0.4) | 43 (3) DILV | ND |
| Conotruncal CVMs | 5 (22) all types | 670 (16) all types | 205 (15) all types | NS, ≤1 |
| 1TOF | ||||
| 4 DORV | ||||
| 2 with HLHS/MA | ||||
| 1 with AVC | ||||
| Atrioventricular canal defects | 1 canal-type VSD (4) | 320 (7) all types | 24 (2) all types | ND |
| “Later” CVMs (developmental error >4 weeks gestational age) | ||||
| LVOTO CVMs | 3 (13) | 587 (14) all types | 120 (9) all types | NS, ≤1 |
| 2 coarctation | ||||
| 1 possible HLHS | ||||
| RVOTO CVMs | 0 | 427 (10) all types | 97 (7) all types | ND |
| ASD, VSD, ASD+/− VSD | 13 (56) | 1186 (28) | 634 (48) | NS, ≤1 |
| TAPVR/PAPVR | 0 | 68 (2) | 11 (1) TAPVR | ND |
Cardiovascular malformation excludes isolated patent ductus arteriosus, dextrocardia/dextroposition, patent foramen ovale, functional anomalies (valve regurgitation, ventricular hypertrophy); cardiomyopathy, ectopia cordis/Pentalogy of Cantrell, inferior vena cava/pulmonary artery compression, pericardial defect.
ASD, atrial septal defect; AVC, atrioventricular canal; BWIS, Baltimore-Washington Infant Study; CDH, congenital diaphragmatic hernia; CHD, congenital heart defect; DILV, double inlet left ventricle; HLHS, hypoplastic left heart syndrome; LVOTO; left ventricular outflow tract obstruction; MA, mitral atresia; ND, not done if number of cases too small; NS, not significant; RVOTO, right ventricular outflow tract obstruction; TAVPR/PAPVR, total/partial anomalous pulmonary venous return; TOF, tetralogy of Fallot; VSD, ventricular septal defect.