Results of palliation with an initial modified Blalock-Taussig shunt in neonates with single ventricle anomalies associated with restrictive pulmonary blood flow.

We report our current-era results after placement of a modified Blalock-Taussig shunt (mBTS) as the first surgery in the multistage palliation strategy of neonates born with single ventricle (SV) anomalies associated with restrictive pulmonary blood flow. Between 2002 and 2012, 174 neonates with SV underwent mBTS. Competing risks analysis modeled events after mBTS (death or transplantation, transition to Glenn) and subsequently after Glenn (death or transplantation, transition to Fontan) and examined risk factors affecting outcomes. Competing risks analysis showed that 2 years after mBTS 27% of patients had died or received transplantation and 73% had undergone Glenn. Five years after Glenn, 6% had died, 73% had undergone Fontan, and 21% were alive awaiting Fontan. Overall survival 8 years after mBTS was 68%. On multivariable analysis, risk factors for mortality were increased shunt size to weight ratio (hazard ratio [HR]:1.2 per 0.1 ratio increase [1.0 to 1.4], p = 0.015), postoperative extracorporeal membrane oxygenation (HR: 4.0 [2.2 to 7.4], p < 0.001), chromosomal and extracardiac malformations (HR: 2.5 [1.3 to 5.0], p = 0.008), cardiopulmonary bypass use (HR: 2.5 [1.3 to 4.5], p = 0.004), and underlying pulmonary atresia with intact ventricular septum (PAIVS) or atrial isomerism (HR: 2.1 [1.1 to 4.2], p = 0.035). Palliation outcomes with mBTS in SV patients trail behind the generally improved results of congenital heart surgery. Several anatomic and patient-related factors continue to affect survival. Mortality is high in low weight neonates in whom a balance between overcirculation due to large shunt and low pulmonary blood flow and risk of occlusion due to small shunt is difficult to attain. Patients with chromosomal and extracardiac malformations, atrial isomerism, and PAIVS continue to be the most challenging and are associated with higher hospital and interstage mortality.

Alsoufi B ，Gillespie S ，Kogon B ，Schlosser B ，Sachdeva R ，Kim D ，Clabby M ，Kanter K ... -  《-》

Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome.

Management of infants with heterotaxy syndrome and functional single ventricle is complicated due to associated cardiac and extracardiac anomalies. We report current era palliation results. Between 2002 and 2012, 67 infants with heterotaxy syndrome underwent multistage palliation. Competing risks analyses modeled events after surgery (death vs Glenn procedure) and examined factors associated with survival. In addition, early and late outcomes following first-stage palliation surgery were compared with a matched contemporaneous control group of patients with nonheterotaxy single ventricle anomalies. Fifty-eight patients (87%) required neonatal palliation, including a modified Blalock-Taussig shunt (n = 34; 51%), Norwood operation (n = 12; 18%) or pulmonary artery band (n = 12; 18%), whereas 9 patients (13%) underwent a primary Glenn procedure. Competing risks analysis showed that at 1 year after first-stage palliation surgery, 29% of the patients had died or undergone transplantation and 63% had undergone a Glenn procedure. By 5 years after the Glenn procedure, 64% of patients had undergone a Fontan procedure. The overall 8-year survival rate was 66%. On multivariable analysis, factors associated with mortality were unplanned reoperation (hazard ratio [HR], 2.9; 95% confidence interval [CI], 1.1-7.3; P = .005) and total anomalous pulmonary venous connection repair (HR, 2.3; 95% CI, 1.0-5.6; P = .056). Comparison with the contemporaneous matched patients with nonheterotaxy single ventricle anomalies showed that first-stage palliation in the patients with heterotaxy was associated with a higher rate of in-hospital death (27% vs 10%; P = .022), and significantly longer durations of ventilation and intensive care unit stay. Interstage mortality, survival after the Glenn procedure, and progression to the Fontan procedure were comparable in the 2 groups. The management of infants with heterotaxy and a functional single ventricle remains challenging. First-stage palliation is associated with high operative mortality and increased resource utilization owing to surgical morbidity. Nonetheless, outcomes beyond hospital discharge are comparable to those for patients with other single ventricle anomalies. Efforts to improve survival in those patients should focus on perioperative care.

Alsoufi B ，McCracken C ，Schlosser B ，Sachdeva R ，Well A ，Kogon B ，Border W ，Kanter K ... -  《-》

Factors affecting death and progression towards next stage following modified Blalock-Taussig shunt in neonates.

The modified Blalock-Taussig shunt (BTS) is utilized to palliate neonates born with restrictive pulmonary blood flow including those with single ventricle (SV) or biventricular (BV) cardiac anomalies. We aim in the current study to report palliation outcomes of neonates with BTS and to examine factors affecting death and progression to the subsequent stage of palliation or repair. Between 2002 and 2012, 341 patients underwent BTS including 175 with SV and 166 with BV anomalies. Competing risk analysis modelled events after BTS (death or transplantation, transition to Glenn shunt or biventricular repair) and examined risk factors affecting outcomes. SV patients had a higher incidence of extracorporeal membrane oxygenation (ECMO) support requirement (12 vs 4%, P = 0.004) and unplanned cardiac reoperation (14 vs 7%, P = 0.051) than their BV counterparts. Additionally, hospital mortality was higher in SV than in BV patients (15 vs 3%, P < 0.001). In SV patients, competing risk analysis showed that, 2 years following BTS, 27% of patients had died or received transplantation and 73% had undergone the Glenn shunt. On multivariable analysis, factors associated with time until death or transplantation prior to Glenn were cardiopulmonary bypass [hazard ratio (HR) 3.6 (2.0-6.4), P < 0.001], unplanned cardiac reoperation [HR 2.4 (1.3-4.6), P = 0.007], pulmonary atresia [HR 2.0 (1.1-3.7), P = 0.026] and the shunt size/weight ratio [HR 1.3 (1.1-1.4) per 0.1 increase, P = 0.001]. In BV patients, competing risk analysis showed that, 2 years following BTS, 13% of patients had died or received transplantation, 85% had undergone biventricular repair and 2% were alive without biventricular repair. On multivariable analysis, factors associated with time until death or transplantation prior to biventricular repair were genetic syndromes and extracardiac malformations [HR 6.1 (2.0-18.2), P = 0.001], weight ≤2.5 kg [HR 5.6 (2.0-16.0), P = 0.001] and male gender [HR 3.4 (1.1-11.0), P = 0.041]. Palliation with BTS continues to be associated with significant operative morbidity and mortality. In addition to hospital death, there is an important interstage attrition risk prior to subsequent palliation or biventricular repair. Inherent patient characteristics (i.e. genetic syndromes and low weight) and anatomical details (i.e. SV, pulmonary atresia and concomitant cardiac anomalies) are associated with worse survival.

Alsoufi B ，Gillespie S ，Mori M ，Clabby M ，Kanter K ，Kogon B ... -  《-》

Results of palliation with an initial pulmonary artery band in patients with single ventricle associated with unrestricted pulmonary blood flow.

Pulmonary artery banding is the initial palliative surgery in patients with single ventricle cardiac anomalies presenting with unrestricted pulmonary blood flow. Reported mortality in those receiving pulmonary artery banding is high, and its application in patients with single ventricle anomalies and arch obstruction is controversial. We report current-era results after pulmonary artery banding in patients with single ventricle anomalies, including those with arch obstruction. Between 2002 and 2012, 73 patients with single ventricle anomalies and unrestricted pulmonary blood flow underwent pulmonary artery banding, including 29 (40%) who received simultaneous arch repair. Competing risk analysis modeled events after pulmonary artery banding (death/transplantation, transition to the Glenn procedure) and subsequently after the Glenn procedure (death/transplantation, transition to the Fontan procedure) and examined risk factors affecting outcomes. Hospital mortality was 3 of 73 patients (4%). Before the Glenn procedure, 16 patients (22%) underwent 18 reoperations, including shunt (n = 7), Damus-Kaye-Stansel connection plus shunt (n = 5), pulmonary artery banding adjustment (n = 5), and transplantation (n = 1). On competing risk analysis, 2 years after pulmonary artery banding, 8% of patients have died or received transplantation, 88% have undergone the Glenn procedure, and 7% were alive without the Glenn procedure. Five years after the Glenn procedure, 9% have died, 71% have undergone the Fontan procedure, and 20% were alive awaiting the Fontan procedure. Overall survival 5 years after pulmonary artery banding was 86%. On multivariable analysis, risk factors for mortality were associated genetic/extracardiac anomalies (hazard ratio, 3.7; 95% confidence interval, 1.1-12.2; P = .03) and high-risk morphology (heterotaxy, unbalanced atrioventricular septal defect, and mitral atresia) (hazard ratio, 4.1; 95% confidence interval, 1.1-15.6; P = .04). Pulmonary artery banding is an acceptable initial palliative strategy of selected patients with single ventricle cardiac anomalies and unrestricted pulmonary blood flow, including those with concomitant arch obstruction. Short- and long-term outcomes are generally good, although results diverge with the worst outcomes noted in patients with heterotaxy, unbalanced atrioventricular septal defect, or associated extracardiac anomalies.

Alsoufi B ，Manlhiot C ，Ehrlich A ，Oster M ，Kogon B ，Mahle WT ，Maher K ，McCrindle BW ，Kanter K ... -  《-》

Impact of Patient Characteristics and Anatomy on Results of Norwood Operation for Hypoplastic Left Heart Syndrome.

We sought to review current-era experience with multistage palliation of neonates with hypoplastic left heart syndrome (HLHS) and to examine the patients' anatomic, and surgical risk factors influencing outcomes. A retrospective review of 219 HLHS patients who underwent the Norwood operation from 2002 to 2012 was performed. Competing risks analyses modeled events after the Norwood operation (death/transplantation, progression to Glenn operation) and after the Glenn operation (death/transplantation progression to Fontan operation), and examined the risk factors affecting outcomes. Competing risks analysis showed that 1 year after the Norwood operation, 25% of patients had died and 75% had undergone the Glenn operation. Three years after the Glenn operation, 11% of patients had died or received transplantation, 48% had undergone the Fontan procedure, and 41% were alive awaiting the Fontan. The overall 8-year survival after the Norwood operation was 66%. The risk factors for mortality on multivariable models were postoperative requirement for extracorporeal membrane oxygenation (hazard ratio [HR]: 3.1 [1.8-5.4], p < 0.001), genetic/major extracardiac anomalies (HR: 2.7 [1.4-5.2], p = 0.002), unplanned cardiac reoperation (HR: 2.5 [1.3-5.0], p = 0.007), modified Blalock-Taussig shunt use (HR: 2.4 [1.4-3.8], p < 0.001), and prematurity (HR: 1.9 [1.1-3.5], p = 0.030). Of note, anatomic factors such as HLHS subtype, ascending aorta diameter, restrictive atrial septum, and associated cardiac lesions did not affect outcomes (p > 0.10 each). This current single-institution experience demonstrates that patients' factors (ie, prematurity, low weight, genetic/extracardiac anomalies) continue to adversely affect survival. Conversely, surgical and perioperative management advances, along with the use of the right ventricle to pulmonary artery shunt, might have neutralized the effects of anatomic factors on survival. Technical imperfections requiring reoperations are associated with failure to progress through palliation stages and diminished survival.

Alsoufi B ，Mori M ，Gillespie S ，Schlosser B ，Slesnick T ，Kogon B ，Kim D ，Sachdeva R ，Kanter K ... -  《-》