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Background: We hypothesized that reconstructed pulmonary artery (PA) size and postrepair PA pressure are associated with airway compression (AC) after complete unifocalization for pulmonary atresia, ventricular septal defects, and major aortopulmonary collateral arteries., Methods: Complete unifocalization was performed in 48 consecutive patients between 2000 and 2016. Clinical course and outcome were reviewed, predictors for AC were identified by logistic regression, and the freedom from death was analyzed using Kaplan-Meier method., Results: Postoperative respiratory distress occurred in 23 patients (48%), and AC occurred in 14 (29%). The median duration of follow-up was 3.7 years. AC was caused by central PA and aorta in 7, conduit in 3, and branch PA in 4. Surgical treatment was required in 5 patients (conduit downsizing, suspension of branch PA, conduit + aorta, branch PA + aorta, and aorta + trachea in 1 patient each). Three patients (21%) subsequently required airway stenting. Most (85.7%) of the AC occurred in patients with high right ventricular systolic pressure/left ventricular systolic pressure (>65%), large Nakata index (>200 mm 2 /m 2 ), and large conduit index (>35 mm/m 2 ). Patients with AC had significantly worse 3-year survival (no AC, 91.2%; AC, 64.2%; p = 0.01). Multivariate analysis identified higher right ventricular systolic pressure/left ventricular systolic pressure (p = 0.04), larger conduit index (p = 0.03), and Nakata index (p = 0.004) as predictors for AC., Conclusions: AC is a common cause of postoperative respiratory distress and tends to be associated with higher postrepair PA pressure, more frequent right ventricular dysfunction, and worse medium-term survival. The study underscores the importance of incorporating all available lung segments to achieve a low PA pressure, potentially preventing pathologic dilatation of the reconstructed PA. Management of patients with poor major aortopulmonary collateral arteries anatomy and physiology remains a challenge., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objectives: Mortality associated with the modified Blalock-Taussig shunt (MBTS) remains high despite advanced perioperative management. This study was formulated to provide data on (1) current indications, (2) outcomes, and (3) factors affecting mortality and morbidity., Design: A retrospective single center chart review identified 95 children (excluding hypoplastic left heart lesions) requiring a MBTS. Mortality and major morbidity were analyzed using the Kaplan Meier method and risk factor analysis using Cox's proportional hazard regression., Results: Median age was 8 (0-126) days, weight 3.1(1.7-5.4) kg. Seventy-three percent were neonates, 58% duct dependent and 73% had single ventricle physiology. Ninety-seven percent had a sternotomy approach for shunt placement with 70% receiving a 3.5 mm graft. Mean graft index (shunt cross sectional area [mm 2 ]/BSA [m 2 ]) was 44.39 ± 8.04 and shunt size (mm) to body weight (kg) ratio 1.1 ± 0.2. Hospital mortality was 12%, with an interval mortality of 6%. Shunt thrombosis/stenosis occurred in 23% and pulmonary over circulation in 30%, while shunt reoperation was required in 12% and catheter intervention in 8% of the cohort. At 1-year, survival was 82.0% (95% CI [72.7%, 88.4%]), and survival free of major morbidity 61.4% (95% CI [50.7%, 70.5%]). Duct dependency predisposed to mortality (P = .01, HR 6.74 [1.54, 29.53]) and composite outcome (mortality and major morbidity) (P = .04, HR 2.15, CI [1.036, 4.466]) and higher graft index to mortality (P = .005, HR 1.07 [1.02, 1.12])., Conclusions: The commonest indication for a MBTS in the current era was single ventricle palliation. Morbidity and mortality was considerable, partly explained by the higher at risk population. Alternative methods to maintain pulmonary blood flow in place of a MBTS requires further investigation., (© 2017 Wiley Periodicals, Inc.)

Background: We hypothesized that mean pulmonary artery pressure (PAP) detected on a pulmonary flow study may predict medium-term survival and right ventricular systolic pressure (RVSP) in patients with pulmonary atresia (PA), ventricular septal defect (VSD), and major aortopulmonary collateral arteries (MAPCAs)., Methods: Fifty patients with PA/VSD/MAPCAs underwent unifocalization between 2000 and 2013, and 40 of these patients had a pulmonary flow study since 2003. Predictability of the mean PAP on VSD status, medium-term survival, reintervention, and RVSP were analyzed., Results: Forty-seven of the 50 patients (94%) had complete unifocalization at a median age of 11 months (range, 1-194 months), and 37 patients (74%) achieved VSD closure. Among the 40 patients who underwent a pulmonary flow study, the VSD was closed in 34 (85%), with salvage VSD fenestration in 4 (10%), and was intentionally left open in 6 (15%). Survival was 85.5% at 1 year and 78.5% at 5 years. A mean PAP ≥25 mm Hg was associated with worse survival (P = .011). Cox regression analysis identified a mean PAP ≥25 mm Hg as the sole predictor for death (P = .037). Patients with an open VSD had an increased risk of reoperation (P = .001) and pulmonary artery reintervention (P = .010), and had a trend toward increased risk of death (P = .059), compared with those with a closed VSD., Conclusions: PAP obtained from the intraoperative pulmonary flow study is associated with medium-term survival and late RVSP in patients with PA/VSD/MAPCAs. VSD closure for patients with a mean PAP ≥25 mm Hg on a flow study is considered high risk, and sensible judgment and a low threshold for VSD fenestration are required., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: The purpose of this study was to evaluate the clinical utility of intraoperative aortic blood flow measurements on clinical outcome in patients undergoing pulmonary artery banding (PAB)., Methods: We reviewed 12 patients who underwent a PAB between September 2008 and March 2013 who also had intraoperative aortic blood flow measurements. Diagnosis included biventricular physiology in 6, single-ventricle physiology in 4, and inadequate systemic ventricle in 2 patients. Aortic blood flow was measured at the time of surgery by Transonic flow probe (Transonic Systems Inc, Ithaca, NY). Aortic flow, intraoperative hemodynamics, and clinical outcomes were analyzed to determine the potential predictive utility of intraoperative variables on postoperative outcomes., Results: The aortic flow increased after PAB from 1.56 ± 0.73 to 2.20 ± 1.10 L · min(-1) · m(-2) (41.0% increase; p = 0.001). The efficacy of the PAB procedure was found to be directly related to the percentage increase in aortic blood flow measured intraoperatively. Three patients with less than 20% increase in aortic blood flow died, required re-PAB, or developed ventricular dysfunction, while patients with successful PAB had more than 40% increase in aortic blood flow. The percentage increase in aortic blood flow was not predictable based on pre-band or post-band absolute aortic blood flow measurements. The percentage increase in aortic blood flow was inversely correlated to the tightness of the PAB as defined relative to that predicted by the Trusler formula (r = 0.67; p = 0.01)., Conclusions: This study identifies the change in the aortic blood flow as a new, physiologically based parameter to help predict PAB outcome., (Copyright © 2015 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: Pulmonary artery growth is an important determinant of outcome in single-ventricle strategies. Higher rates of pulmonary artery intervention have been reported with hybrid-based palliation when compared with Norwood palliation., Methods: We performed a retrospective review of pulmonary artery growth and clinical outcomes in patients undergoing hybrid-based single-ventricle palliation., Results: The stage I hybrid procedure was performed in 72 patients between 2004 and 2012, of whom 54 were on a Fontan palliative pathway. Thirty-four infants completed stage II, and 20 infants underwent the Fontan operation. The mean diameters of the right pulmonary artery (5.6 ± 1.9 mm) and left pulmonary artery (5.6 ± 2.1 mm) were similar before stage II. After stage II, the right and left pulmonary artery diameters were 8.5 ± 2.1 mm and 5.8 ± 1.3 mm, respectively (P < .001), and after the Fontan operation, these were 8.8 ± 2.0 mm and 6.4 ± 1.1 mm, respectively (P = .002). The mean right pulmonary artery z score was normal throughout, but the left pulmonary artery did not maintain a normal size. The cumulative pulmonary artery intervention rate was 50% at any time after stage II. Fifteen interventions (88%) were performed after stage II (35% during the same hospitalization, 71% <60 days). The most intervened site was the midsection of the left pulmonary artery (41%). Initial pulmonary artery intervention was balloon dilation in 59% of patients and stent implantation in 41% of patients. Half of patients with initial balloon dilation required reintervention., Conclusions: There is significant risk of left pulmonary artery compromise after the second stage of hybrid palliation associated with a high intervention rate., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: The management of patients with tetralogy of Fallot (ToF) and pulmonary atresia (PA) with major aortopulmonary collateral arteries (MAPCAs) is challenging. Frequently it is difficult to predict whether complete repair with closure of the ventricular septal defect (VSD) will be tolerated. The aim of this study was to investigate whether measurements of preoperative pulmonary blood flow are associated with early postoperative outcome after VSD closure., Methods: In this retrospective study, the data from 10 patients who had undergone a cardiac magnetic resonance imaging study with flow measurements before attempted surgical complete repair were collected. Systemic blood flow (Qs) was calculated as the sum of descending aortic blood flow distal to the MAPCA origins and superior vena cava flow. Pulmonary blood flow (Qp) was measured either from the sum of the pulmonary flow (n = 7) or calculated as the difference between ascending aortic flow and Qs., Results: Preoperative Qp/Qs averaged 1.71 ± 0.68 and correlated inversely with right ventricular systolic pressure (relative to systolic blood pressure, r = -0.75, P = .03) and positively with the total neopulmonary artery index (TNPAI, r = 0.66, P = .04), as measured by fluoroscopic angiography. Two children did not tolerate VSD closure. This was likely related to bronchial compression in 1 patient while the other had the lowest TNPAI and the lowest Qp/Qs of all patients., Conclusions: CMR provides not only anatomic but also functional information for surgical decision making in patients with ToF and PA with MAPCAs. Preoperative Qp/Qs is associated with postoperative right ventricular pressure and may be a marker of readiness for VSD closure., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: Retrograde aortic arch malperfusion after ductal stenting can be life-threatening after univentricular hybrid palliation. Arch perfusion can be maintained with a main pulmonary artery to innominate artery shunt placed during the stage I procedure: a "reverse Blalock-Taussig shunt.", Methods: A retrospective review of 37 infants who underwent hybrid palliation from January 2004 to March 2010 was performed. The infants were divided into 2 groups, those with (group I, n = 16) and those without (group II, n = 21) a reverse Blalock-Taussig shunt., Results: At the initial palliation, no differences were found in the demographics, systolic or diastolic pressures, or ventricular or atrioventricular valve function between the 2 groups. Group I had more infants with aortic atresia (P < .01) and smaller ascending aortas (P < .01). Before stage II, the retrograde aortic Doppler flow velocity increased in group I (P < .01) and was unchanged in group II. The reintervention rates before stage II were similar between the 2 groups. Before stage II, the ventricular end-diastolic pressure, left and right pulmonary artery pressures and diameters, and mixed venous and arterial saturations were similar between the 2 groups. The complication rates between the 2 groups were not significantly different, although a nonsignificant trend toward more neurologic complications was noted in group I. The Kaplan-Meier survival estimate at 1 year was similar between the 2 groups (63% for group I vs 71% for group II)., Conclusions: The presence of a reverse Blalock-Taussig shunt was not associated with more adverse events than those without. Gradual retrograde arch obstruction occurs commonly in palliated infants with aortic atresia. A reverse Blalock-Taussig shunt might play an important role to address the potential of retrograde obstruction, augmenting arch blood flow., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective: We sought to evaluate the efficacy of balloon angioplasty (BA) for severely desaturated patients due to a stenotic right ventricle (RV) to pulmonary artery (PA) shunt following modified Norwood procedure., Methods: Of 87 patients who underwent a Norwood procedure with the RV-PA shunt between February 1998 through March 2010, 22 (25%) patients underwent BA. The efficacy of BA was assessed by angiographic measurement of the changes in the internal diameters of the stenotic portions of the shunt, changes in arterial saturation and clinical outcomes., Results: BA was performed for stenotic RV-PA shunts following stage I palliation (n = 17, 77%), or those placed as an additional blood source (n = 5, 23%, 3 patients awaiting biventricular repair, 2 patients following stage II palliation). The location of the BA was at the distal anastomosis in 12 (54.5%), proximal anastomosis in 21 (95.4%) and in the mid-portion of the shunt in 11 (50%) cases. The diameters of these three shunt portions were measured from the anterior-posterior and lateral angiographic images, increasing significantly after BA (p < 0.0001) in all. Arterial saturation significantly improved after BA in all cases (66.5 ± 4.3% to 79.4 ± 3.4%, p < 0.0001). Freedom from reintervention was 100%. All patients underwent subsequent elective planned surgery at an appropriate age with no mortality., Conclusions: A BA-alone strategy for a stenotic RV-PA shunt was effective for all three shunt portions, minimizing shunt-related premature surgical intervention., (Copyright © 2012 Wiley Periodicals, Inc.)

Objective: We reported a high incidence of thrombosis, central pulmonary artery hypoplasia, and mortality for bilateral bidirectional cavopulmonary shunts. We hypothesized that technical modifications in the cavopulmonary anastomosis and anticoagulation would limit thrombus and central pulmonary artery hypoplasia, and thereby improve outcomes., Methods: Sixty-one patients (median age, 8.4 months; weight, 6.6 kg) underwent bilateral bidirectional cavopulmonary shunt from 1990 to 2007. The cohort was divided into 2 groups: 1) the conventional group (1990-1999, n = 37) and 2) the V-shaped group, with a hemi-Fontan or modification in which the cavae were anastomosed to the pulmonary artery adjacent to each other so they formed the appearance of a V (1999-2007, n = 24). Central and branch pulmonary artery growth, survival, and reinterventions were determined., Results: The pre-Fontan study showed equivalent superior venae cavae and Nakata indices. The central pulmonary artery index and central pulmonary artery/Nakata index ratio were significantly higher in the V-shaped group (P < .05). There were no differences in freedom from death or transplant (conventional 69% vs V-shaped 75% at 3 years, P = .5), and a nonsignificant trend toward improving freedom from reinterventions (63% vs 81% at 3 years, P = .15) and thrombosis (82% vs 95% at 1 year, P = .11) was observed in the V-shaped group. Multivariate analysis showed anastomotic strategy, low saturation, and thrombosis were predictors for death. Anastomotic strategy, lack of anticoagulation, thrombosis, and small superior venae cavae were predictors for reintervention (P < .05). Predictors for thrombus included small superior venae cavae, Nakata index, and low saturation (P < .03)., Conclusions: Surgical modifications for bilateral bidirectional cavopulmonary shunts were associated with the larger central pulmonary artery size. Lack of anticoagulation and anastomotic strategy affected reintervention. Anastomotic strategy and postoperative thrombus affected mortality., (2010 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Background: The objective was to determine if intraoperative pulmonary artery (PA) flow studies after complete unifocalization correlate with postrepair hemodynamics for pulmonary atresia (PA), ventricular septal defects (VSD), and major aortopulmonary collaterals., Methods and Results: Twenty patients (median age, 8 months; weight, 7.9 kg) underwent unifocalization between 2003 and 2008. A functional PA flow study was achieved by cannulating the unifocalized central PA before intracardiac repair and increasing flow incrementally to 2.5 L/min per m(2). Mean PA pressure (mPAP) was measured. The intent was to close the VSD for a mPAP of <30 mm Hg. Right ventricular systolic pressure (RVSP) and systemic systolic pressure were recorded. Total incorporated pulmonary segments, pulmonary segment artery ratio (ratio of incorporated segments to 18), and total neopulmonary artery index (the sum of major aortopulmonary collaterals and native PA index) were calculated. The VSD was successfully closed in 18 patients (90%). One attempted closure required an intraoperative fenestration. The study mPAP correlated with RVSP (rho=0.72; P=0.0027) and RVSP/systemic systolic pressure (rho=0.67; P=0.0063). Total neopulmonary artery index had a nonsignificant negative correlation with RVSP (rho=-0.42; P=0.079). Total incorporated pulmonary segments and pulmonary segment artery ratio were not correlated. Flow study mPAP had the highest accuracy in predicting successful VSD closure: area under the receiver-operator curve (0.83) versus total neopulmonary artery index (0.42), pulmonary segments (0.35), and pulmonary segment artery ratio (0.33)., Conclusions: The intraoperative pulmonary flow study predicted postoperative physiology significantly better than did standard anatomic measures. Conventional measures should be used with caution when determining the possibility for complete repair.

Background: Hybrid strategies for single ventricle palliation may differ from Norwood strategies in terms of anatomic and physiologic growth stimuli to the pulmonary arteries (PA), hemodynamics, resource utilization, and survival. Few studies have directly compared these strategies., Methods: In all, 58 patients underwent Norwood (Blalock-Taussig shunt; n = 39) or hybrid (n = 19) single ventricle palliation (2004 to 2007). Hemodynamics, PA morphology, hemodynamics, resource utilization, and survival were reviewed., Results: At pre-stage 2 evaluation, there were nonsignificant trends toward lower ventricular end-diastolic pressure, higher mixed venous saturation, and larger Nakata and lower lobe indices in the hybrids. Mean PA pressures were not different between groups. Four Norwood patients (10%) underwent transplantation before stage 2 palliation. Forty-two patients underwent stage 2 palliation (bidirectional cavopulmonary shunt or stage 2 hybrid (aortic arch reconstruction and bidirectional cavopulmonary shunt). Requirement for PA plasty, postoperative CVP, stage 2 survival, and 1-year survival were similar between groups. Combined (stage 1 plus stage 2) intubation time, intensive care unit time, and hospital length of stay was shorter for hybrids in comparison with Norwood survivors (p < 0.05). Comparison of resource utilization at the time of arch reconstruction (Norwood procedure or stage 2 hybrid), demonstrated a time-related trend toward improvement (weak negative correlation: intubation, rho = -0.386, p = 0.172; intensive care unit stay, rho = -0.487, p = 0.077; hospital stay, rho = -0.429, p = 0.126) in the hybrid group, but not in the Norwood group., Conclusions: Hybrid palliation does not have a significant adverse impact on PA development, with comparable PA growth and hemodynamics. The demonstration of equivalent survival, diminished hospital utilization, and trends indicating ongoing refinement of the hybrid strategy warrants a prospective randomized trial.

Myocardial recovery following left ventricular assist device (LVAD) implant is uncommon but has been reported in children with dilated cardiomyopathy (DCM). However, there is a risk for recurrence of heart failure (HF) after LVAD explant. Pulmonary artery (PA) banding has been reported as a HF treatment strategy to alter ventriculo-ventricular (VV) interaction in DCM. We report a case of PA banding at the time of LVAD explant in a child with DCM. A term infant female, presented at 3 months of age with HF due to DCM. Her echocardiogram showed severely dilated and reduced LV function with preserved right ventricular (RV) function. Genetic testing revealed a likely pathogenic mutation of GAA c.1375G>C (p. Asp459His). Due to worsening HF, she underwent 10ml Berlin Heart EXCOR LVAD implant as a bridge to heart transplantation. She continued with anti heart failure medications along with LVAD support. Over the next few months, her LV function gradually recovered, but there was persistent mid/basal septal dyskinesia. After reassuring echocardiographic wean studies and invasive hemodynamic assessment, she underwent LVAD explant after 6 months support. Due to the dyskinetic septum and pathogenic genetic mutation, a PA band was applied at time of explant to augment VV interactions and prevent progressive LV dilation and dysfunction. She was discharged from hospital and is currently asymptomatic 18 months post explant and PA banding, although septal dyskinesia persists. At last, follow up, her echocardiogram revealed good biventricular systolic function and a PA band gradient of almost 30mmHg. Myocardial function can recover following LVAD support and HF medications. In this case, the addition of PA banding was used to optimize VV interaction after LVAD explant and to prevent further LV dilation while in remission of HF. [ABSTRACT FROM AUTHOR]