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Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10-year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow-up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P =0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1-30.0; P =0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.

Treatment for peripheral pulmonary artery stenosis is challenging, and conventional balloon angioplasty has not proved to be universally effective. Evaluated was the efficacy of bladed balloon (BB) dilation to address vessels resistant to conventional high-pressure (10-15 atm) balloon angioplasty (BA). Thirty-one procedures were performed on 14 children with age range 1 month to 15 years. The diameter of the BB ranged from 3 to 8 mm. After BB dilation, all children had subsequent conventional BA (balloon size range, 3-10 mm). The minimal lumen diameter (MLD) before and after the procedure, whether there was a waist at initial BA, and BB diameter-to-MLD ratio before the procedure were measured. A > 50% increase in MLD was considered successful. Four children had Williams syndrome, two children Alagille syndrome, five children Fallot's tetralogy, and three miscellaneous lesions. The resistant stenosis was located in the right central pulmonary artery in 6, right branch pulmonary artery in 7, left central pulmonary artery in 6, and left branch pulmonary artery in 12 lesions. Median BB diameter was 253% (117-440%) of the MLD and increased from 2.0 +/- 0.7 to 3.2 +/- 0.8 mm (P < 0.0001), with a mean increase of 73% +/- 62%. There was an inverse relationship between the MLD before and increase after the procedure (r = 0.75; P < 0.001). The BB diameter-to-MLD ratio before procedure was significantly associated with the increase in MLD (r = 0.70; P < 0.001). After the procedure, 18 of the 31 procedures were considered successful. In all successful procedures, the BB diameter was greater than twice the MLD before the procedure. Comparing children with Williams and Alagille syndrome with the remaining eight children, there were no significant differences in the increase in MLD. A small aneurysm and thrombus were noticed in two and three children, respectively, but no fatal complications were reported. BB angioplasty is effective for resistant peripheral pulmonary artery stenosis when conventional BA fails. The diameter of the BB should be larger than twice the minimal luminal diameter of the stenotic lesion., (Copyright 2004 Wiley-Liss, Inc.)