Stage II palliation of hypoplastic left heart syndrome without cardiopulmonary bypass
ABSTRACT Bidirectional cavopulmonary anastomosis has been performed without cardiopulmonary bypass for some single-ventricle heart defects. Limited data are available for the outcomes of off-pump bidirectional cavopulmonary anastomosis in infants with hypoplastic left heart syndrome. The purpose of this study is to determine the early outcomes for stage II palliation of hypoplastic left heart syndrome without cardiopulmonary bypass.
This is a retrospective review of infants having surgical palliation of hypoplastic left heart syndrome from April 2003 to March 2010 at a single institution.
Seventy-five infants had a modified Norwood procedure, 65 with a right ventricle-pulmonary artery conduit, 10 with an aortopulmonary shunt, 2 with atrioventricular valve repair, and 3 with extracorporeal life support. Sixty-eight patients had hypoplastic left heart syndrome or one of its variants, and 7 had other single-ventricle lesions. There were 2 stage I deaths. Stage I survival was 97% (95% confidence interval, 88%-99%). Another 5 infants succumbed in the interstage period. Of the 68 stage I and interstage survivors, 61 had bidirectional cavopulmonary anastomoses, 20 without cardiopulmonary bypass. Median age was 6 months (range, 4-13 months), and median weight was 6.1 kg (range, 5.2-9.0 kg). There were no conversions to cardiopulmonary bypass when off-pump bidirectional cavopulmonary anastomosis was attempted. There were no hospital deaths. Median ventilation duration was 10 hours (range, 6-18 hours), and length of stay was 5 days (range, 4-9 days). Follow-up was available on all infants at a median duration of 17 months (range, 3-43 months), with no unplanned reinterventions.
Bidirectional cavopulmonary anastomosis without the use of cardiopulmonary bypass can be performed safely and with low mortality for selected infants with hypoplastic left heart syndrome. Midterm to long-term outcomes remain to be determined.
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ABSTRACT: The optimal shunt size for patients who have the Norwood operation with a right ventricle-to-pulmonary artery conduit is controversial. The goal of this study is to compare outcomes of 2 shunt sizes in this population. Between 2002 and 2010, 75 consecutive patients diagnosed with hypoplastic left heart syndrome and its variants underwent the Norwood procedure with a right ventricle-to-pulmonary artery conduit. The outcomes of 20 neonates weighing <3 kg were analyzed. The cumulative 30-day stage 1 mortality was 97% (95% confidence interval, 88%-99%) for all patients (73/75), 20 of whom weighed <3 kg. Nine patients had a 6-mm (group 1) and 11 patients had a 5-mm (group 2) right ventricle-to-pulmonary artery conduit. Thirty-day stage 1 survival was 88% (8/9) in group 1 and 90% (10/11) in group 2 (P = .88). The central pulmonary artery confluence size at prebidirectional cavopulmonary shunt catheterization was 4.5 ± 1.2 mm in group 1 and 2.5 ± 1.0 mm in group 2 (P = .009). The mean transpulmonary gradient was higher in group 2 (7.8 ± 3.1 mm Hg vs 4.2 ± 1.9 mm Hg; P = .036). The incidence of pulmonary artery intervention was 16% (1/7) in group 1 and 75% (6/8) in group 2 (P = .030). Survival rate at 6 months was 66% (6/9) in group 1 and 70% (7/10) in group 2 (P = .89). Use of a 6-mm right ventricle-to-pulmonary artery conduit showed better central pulmonary artery growth and less need for pulmonary artery intervention in the authors' experience.The Journal of thoracic and cardiovascular surgery 08/2012; 144(5):1091-4. DOI:10.1016/j.jtcvs.2012.07.061 · 3.99 Impact Factor
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ABSTRACT: The single-ventricle reconstruction trial randomized patients with single right ventricle lesions to a modified Blalock-Taussig or right ventricle-to-pulmonary artery shunt at the Norwood. This analysis describes outcomes at the stage 2 procedure and factors associated with a longer hospital length of stay (LOS). We examined the association of shunt type with stage 2 hospital outcomes. Cox regression and bootstrapping were used to evaluate risk factors for longer LOS. We also examined characteristics associated with in-hospital death. There were 393 subjects in the analytic cohort. Median stage 2 procedure hospital LOS (8 days; interquartile range [IQR], 6-14 days), hospital mortality (4.3%), transplantation (0.8%), median ventilator time (2 days; IQR, 1-3 days), median intensive care unit LOS (4 days; IQR, 3-7 days), number of additional cardiac procedures or complications, and serious adverse events did not differ by shunt type. Longer LOS was associated (R(2) = 0.26) with center, longer post-Norwood LOS (hazard ratio [HR], 1.93 per log day; P < .001), nonelective timing of the stage 2 procedure (HR, 1.78; P < .001), and pulmonary artery (PA) stenosis (HR, 1.56; P < .001). By univariate analysis, nonelective stage 2 (65% vs 32%; P = .009), moderate or greater atrioventricular valve (AVV) regurgitation (75% vs 24%; P < .001), and AVV repair (53% vs 9%; P < .001) were among the risk factors associated with in-hospital death. Norwood LOS, PA stenoses, and nonelective stage 2 procedure, but not shunt type, are independently associated with longer LOS. Nonelective stage 2 procedure, moderate or greater AVV regurgitation, and need for AVV repair are among the risk factors for death.The Journal of thoracic and cardiovascular surgery 09/2013; 147(6). DOI:10.1016/j.jtcvs.2013.07.063 · 3.99 Impact Factor
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ABSTRACT: Background-Our purpose was to test the following hypotheses: (1) patients with hypoplastic left heart syndrome who develop significant tricuspid regurgitation (TR) or require tricuspid valve (TV) surgery in the medium term have detectable TV abnormalities by 3-dimensional echocardiography (3DE) prestage 1 palliation and (2) TR is associated with reduced survival and increased TV intervention. Methods and Results-Infants were prospectively studied with 3DE and 2DE prestage 1 and followed up for the end points of TR, TV surgery, transplantation, or death. From prestage 1 3DE, spatial coordinates of TV annulus and leaflets were extracted; annulus size, leaflet area, prolapse volume, tethering volume, bending angle, and papillary muscle angle were measured. TR was assessed prestage 1 and at latest follow-up. Of 70 patients, 62 (88.6%) had mild or less TR and 8 (11.4%) had moderate or greater TR prestage 1. Prestage 1 tethering volume correlated to leaflet area (r=0.736; P<0.001), annulus area (r=0.651; P<0.001), right ventricular end-diastolic area (r=0.347; P=0.003), fractional area change (r=-0.387; P<0.001), and TR grade (r=0.447; P<0.001). At follow-up, 46 (65.7%) had mild or less TR (group A) and 24 (34.3%) had moderate or greater TR (group B). Prestage 1 3DE showed greater TV tethering volume and flatter annulus in group B. Survival was better in group A. Conclusions-Increased TV tethering volume and flatter bending angle prestage 1 palliation is associated with TV failure at medium-term follow-up. Increased prestage 1 tethering is related to having larger TV annulus, larger leaflet area, larger right ventricular size, and reduced systolic function. TR progression results in increased TV intervention and decreased survival.Circulation Cardiovascular Imaging 07/2014; 7(5). DOI:10.1161/CIRCIMAGING.113.001161 · 6.75 Impact Factor