Durability of Aortic Homografts in Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.

Objectives: It is well-known that right ventricle-to-pulmonary artery homograft conduit durability is worse for smaller conduits and smaller/younger patients. However, there is limited literature on age and conduit-size specific outcomes, or on the role of conduit oversizing. Methods: Patients diagnosed with tetralogy of Fallot and major aortopulmonary collateral arteries undergoing right ventricular outflow tract (RVOT) reconstruction with a valved aortic homograft conduit from November 2001 through March 2023, at our institution were included. Conduits were grouped and evaluated by diameter, diameter Z-score, and patient age at implant. The primary time-related outcome was freedom from RVOT reintervention. Factors associated with freedom from time-related outcomes were assessed with univariable Cox regression analysis. Results: A total of 863 RVOT conduits were implanted in 722 patients. On multivariable analysis, younger age, male sex, Alagille syndrome, smaller diameter of the conduit, and smaller Z-score were associated with shorter freedom from reintervention. Among patients with smaller diameter conduits, larger Z-scores were associated with longer freedom from conduit reintervention (P < .001). Transcatheter interventions were commonly used to extend conduit lifespan across ages and conduit sizes. Conclusions: Larger conduit diameter, older age, and higher conduit Z-score were associated with longer freedom from reintervention in patients undergoing RVOT reconstruction in this cohort. Oversizing of conduits, even beyond a Z-score of 4, is generally appropriate.

Use of temporary tracheostomy occlusion to reduce the risk of sternal wound infection after sternotomy in congenital cardiac surgery.

OBJECTIVE: To describe a method of reducing the risk of sternal wound infection after sternotomy in children with a pre-existing tracheostomy. To report our outcomes using this method from 1 January, 2013 to 31 August, 2023. METHODS: We describe a method for temporarily occluding the tracheal stoma with a removable implant with the primary goal of reducing the risk of sternotomy wound infection by preventing soilage due to tracheostomal secretions. We then performed a retrospective review of all children who underwent temporary tracheostomal occlusion between 1 January, 2013 and 31 August, 2023 at our quaternary care children's hospital. Clinical variables were extracted from the hospital medical records. The rates of antibiotic use and minor and major complications during the period when the stoma plug was in place were recorded. RESULTS: Totally, 19 patients underwent tracheal stoma plugging prior to sternotomy and were included in our analysis. There were two cases of sternal wound infection; one case occurred while the stoma plug was in place, and one developed four days following plug removal. There was one minor complication, with one patient requiring stoma revision via serial dilation at bedside at the time of recannulation. There were no deaths. CONCLUSION: Temporary occlusion of the tracheal stoma with an impermeable plug is a viable option for reducing the risk of sternal wound infection in children with a pre-existing tracheostomy who are undergoing sternotomy.

Right Ventricular and Outflow Tract Functional Characteristics After Repair of Tetralogy of Fallot with Major Aortopulmonary Collaterals.

This study describes right ventricle (RV) characteristics and right ventricle to pulmonary artery (RV-PA) conduit function pre- and post-repair in patients with tetraology of Fallot with major aortopulmonary collaterals (TOF/MAPCAs). We reviewed patients who underwent single-stage, complete unifocalization, and repair of TOF/MAPCAs between 2006 and 2019 with available pre- and early postoperative echocardiograms. For a subset of patients, 6-12 month follow-up echocardiogram was available. RV and left ventricle (LV) characteristics and RV-PA conduit function were reviewed. Wilcoxon signed rank test and McNemar's test were used. 170 patients were reviewed, 46 had follow-up echocardiograms. Tricuspid valve annular plane systolic excursion (TAPSE) Z-scores were reduced from pre- (Z-score 0.01) to post-repair (Z-score -4.5, p < 0.001), improved but remained abnormal at follow-up (Z-score -4.0, p < 0.001). RV fractional area change (FAC) and LV ejection fraction were not significantly different before and after surgery. Conduit regurgitation was moderate or greater in 11% at discharge, increased to 65% at follow-up. RV-PA conduit failure (severe pulmonary stenosis or severe pulmonary regurgitation) was noted in 61, and 63% had dilated RV (diastolic RV area Z-score > 2) at follow-up. RV dilation correlated with the severe conduit regurgitation (p = 0.018). Longitudinal RV function was reduced after complete repair of TOF/MAPCAs, with decreased TAPSE and preserved FAC and LV ejection fraction. TAPSE improved but did not normalize at follow-up. Severe RV-PA conduit dysfunction was observed prior to discharge in 11% of patients and in 61% at follow-up. RV dilation was common at follow-up, especially in the presence of severe conduit regurgitation.

20-Year Experience With Repair of Pulmonary Atresia or Stenosis and Major Aortopulmonary Collateral Arteries.

BACKGROUND: We have followed a consistent, albeit evolving, strategy for the management of patients with pulmonary atresia or severe stenosis and major aortopulmonary collateral arteries (MAPCAs) that aims to achieve complete repair with low right ventricular pressure by completely incorporating blood supply and relieving stenoses to all lung segments. OBJECTIVES: The purpose of this study was to characterize our 20-year institutional experience managing patients with MAPCAs. METHODS: We reviewed all patients who underwent surgery for MAPCAs and biventricular heart disease from November 2001 through December 2021. RESULTS: During the study period, 780 unique patients underwent surgery. The number of new patients undergoing surgery annually was relatively steady during the first 15 years, then increased substantially thereafter. Surgery before referral had been performed in almost 40% of patients, more often in our recent experience than earlier. Complete repair was achieved in 704 patients (90%), 521 (67%) during the first surgery at our center, with a median right ventricular to aortic pressure ratio of 0.34 (25th, 75th percentiles: 0.28, 0.40). The cumulative incidence of mortality was 15% (95% CI: 12%-19%) at 10 years, with no difference according to era of surgery (P = 0.53). On multivariable Cox regression, Alagille syndrome (HR: 2.8; 95% CI: 1.4-5.7; P = 0.004), preoperative respiratory support (HR: 2.0; 95% CI: 1.2-3.3; P = 0.008), and palliative first surgery at our center (HR: 3.5; 95% CI: 2.3-5.4; P < 0.001) were associated with higher risk of death. CONCLUSIONS: In a growing pulmonary artery reconstruction program, with increasing volumes and an expanding population of patients who underwent prior surgery, outcomes of patients with pulmonary atresia or stenosis and MAPCAs have continued to improve.

Airway Characteristics of Patients With 22q11 Deletion Undergoing Pulmonary Artery Reconstruction Surgery: Retrospective Cohort Study.

OBJECTIVES: We have previously shown that patients with a chromosome 22q11 microdeletion are at risk for prolonged respiratory failure after pulmonary artery reconstruction surgery compared with those with normal genotype. We sought to describe preexisting airway abnormalities in this patient population and examine relationships between airway abnormalities and outcomes. DESIGN: Single-center retrospective chart review from Society of Thoracic Surgery and Pediatric Cardiac Critical Care Consortium databases and the electronic medical record. SETTING: Lucile Packard Children's Hospital at Stanford from September 2017 to February 2019. PATIENTS: All patients undergoing pulmonary artery reconstruction surgery were considered for inclusion. INTERVENTIONS: We identified 127 patients meeting study inclusion criteria. Thirty-nine patients met specific criteria and underwent screening preoperative bronchoscopy including microdirect laryngoscopy and lower airway examination. Postoperative bronchoscopy was performed at the discretion of the intensive care team. MEASUREMENTS AND MAIN RESULTS: Airway abnormalities were detected in 25/26 of children (96%) with a chromosome 22q11 deletion who underwent preoperative bronchoscopy. Upper and lower airway pathologies were found in 19/25 (73%) and 21/25 (81%) patients, respectively, and it was common for patients to have more than one abnormality. Presence of 22q11 deletion was associated with longer duration of mechanical ventilation (9.1 vs 4.3 d; p = 0.001), use of noninvasive positive pressure support (13 vs 6 d; p = 0.001), and longer hospital stays (30 vs 14 d; p = 0.002). These outcomes were worse when compared with patients with known airway abnormalities who did not have 22q11 deletion. CONCLUSIONS: Preexisting upper and lower airway pathologies are common in patients with a chromosome 22q11 deletion who undergo pulmonary artery reconstruction surgery. Despite similar postoperative hemodynamics and outcomes as their counterparts without 22q11 deletion, 22q11 deletion is associated with more postoperative respiratory complications not entirely explained by preexisting airway abnormalities.

Standardized Training for Physicians Practicing Pediatric Cardiac Critical Care.

OBJECTIVES: In the vast majority of Children's Hospitals, the critically ill patient can be found in one of three locations: the PICU, the neonatal ICU, and the cardiac ICU. Training, certification, and maintenance of certification for neonatology and critical care medicine are over seen by the Accreditation Council for Graduate Medical Education and American Board of Pediatrics. There is no standardization of training or oversight of certification and maintenance of certification for pediatric cardiac critical care. DATA SOURCES: The curricula from the twenty 4th year pediatric cardiac critical care training programs were collated, along with the learning objectives from the Pediatric Cardiac Intensive Care Society published "Curriculum for Pediatric Cardiac Critical Care Medicine." STUDY SELECTION: This initiative is endorsed by the Pediatric Cardiac Intensive Care Society as a first step toward Accreditation Council for Graduate Medical Education oversight of training and American Board of Pediatrics oversight of maintenance of certification. DATA EXTRACTION: A taskforce was established of cardiac intensivists, including the directors of all 4th year pediatric cardiac critical care training programs. DATA SYNTHESIS: Using modified Delphi methodology, learning objectives, rotational requirements, and institutional requirements for providing training were developed. CONCLUSIONS: In the current era of increasing specialized care in pediatric cardiac critical care, standardized training for pediatric cardiac critical care is paramount to optimizing outcomes.

Unifocalization and pulmonary artery reconstruction in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery before referral.

OBJECTIVE: The study objective was to characterize and analyze outcomes in patients with tetralogy of Fallot and major aortopulmonary collateral arteries who had undergone surgery elsewhere before referral (prereferral surgery). METHODS: Patients with tetralogy of Fallot and major aortopulmonary collateral arteries who underwent surgery between 2001 and 2019 at our center were reviewed. Prereferral surgery and unoperated patients were compared, as were subsets of prereferral surgery patients who had undergone different types of prior procedures. Primary outcomes included complete repair with survival to 6 months, death, and perioperative metrics. RESULTS: Of 576 patients studied, 200 (35%) had undergone a wide range and number of prior operations elsewhere, including 92 who had pulmonary blood supply through a shunt and 108 who had a right ventricle pulmonary artery connection. Patients who underwent prereferral surgery with an existing right ventricle pulmonary artery connection had undergone more prereferral surgery procedures than those with a shunt and were more likely to have a right ventricle outflow tract pseudoaneurysm or pulmonary artery stent (all P < .001) at the time of referral. The cumulative incidences of complete repair and death were similar regardless of prereferral surgery status, but the cumulative incidence of complete repair with 6-month survival was higher (P = .002) and of death lower (P = .18) in patients who had prior right ventricle pulmonary artery connection compared with those who had received a prior shunt only. CONCLUSIONS: Our comprehensive management strategy for tetralogy of Fallot and major aortopulmonary collateral arteries can be applied with excellent procedural results in both unoperated patients and those who have undergone multiple and varied procedures elsewhere.

Transfusion Outcomes in Patients Undergoing Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals.

BACKGROUND: Surgical repair of tetralogy of Fallot and major aortopulmonary collaterals (TOF/MAPCAs) involves unifocalization of MAPCAs and reconstruction of the pulmonary arterial circulation. Surgical and cardiopulmonary bypass (CPB) times are long and suture lines are extensive. Maintaining patency of the newly anastomosed vessels while achieving hemostasis is important, and assessment of transfusion practices is critical to successful outcomes. METHODS: Clinical, surgical, and transfusion data in patients with TOF/MAPCAs repaired at our institution (2013-2018) were reviewed. Types and volumes of blood products used in the perioperative period, in addition to the use of antifibrinolytics and/or procoagulants (factor VIII inhibitor bypassing activity [FEIBA]; anti-inhibitor coagulant complex), were assessed. Outcome measures included days on mechanical ventilation (DOMV), postoperative intensive care unit and hospital length of stay (LoS), and incidence of thrombosis. RESULTS: Perioperative transfusion data from 279 patients were analyzed. Surgical (879 ± 175 minutes vs 684 ± 257 minutes) and CPB times (376 ± 124 minutes vs 234 ± 122 minutes) were longer in patients who received FEIBA than those who did not. Although the indexed volume of packed red blood cells (128.4 ± 82.2 mL/kg) and fresh frozen plasma (64.2 ± 41.1 mL/kg) was similar in patients who did and did not receive FEIBA, the amounts of cryoprecipitate (5.5 ± 5.2 mL/kg vs 5.8 ± 4.8 mL/kg) and platelets (19.5 ± 20.7 mL/kg vs 20.8 ± 13 mL/kg) transfused were more in those who did receive FEIBA. CONCLUSION: Perioperative transfusion is an important component in the overall surgical and anesthetic management of patients with TOF/MAPCAs. The intraoperative use of FEIBA was not associated with a decrease in the amount of blood products transfused, DOMV, or LoS or with an increase in thrombotic complications.

Assessment of the Reconstructed Pulmonary Circulation With Lung Perfusion Scintigraphy After Unifocalization and Repair of Tetralogy of Fallot With Major Aortopulmonary Collaterals.

BACKGROUND: Pulmonary vascular supply in tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) is highly variable. Our approach to surgical management of this condition emphasizes early repair including unifocalization and reconstruction of the pulmonary circulation, incorporating all lung segments and addressing stenoses both proximal to and within the lung, in addition to ventricular septal defect closure. At our institution, we have over 15 years of experience using lung perfusion scintigraphy (LPS) to assess the distribution of pulmonary blood flow after complete unifocalization and repair. METHODS: We reviewed clinical and quantitative LPS data in 310 patients who underwent complete unifocalization and repair of TOF/MAPCAs from 2003 to 2018 at our institution. Postrepair relative lung perfusion distributions were determined from LPS initially obtained at our institution within 60 days after repair and thereafter. RESULTS: Total lung perfusion to the right and left lungs was 58.0% ± 14.2% and 42.0% ± 14.2%, respectively. Perfusion was balanced in 75% of patients and unbalanced in 25%, including 11% in whom it was extremely unbalanced. On multivariable analysis, older age at repair, surgery other than a single-stage complete unifocalization, and native anatomy consisting of unilateral pulmonary blood supply through a ductus arteriosus were associated with unbalanced perfusion. CONCLUSION: We present our experience using LPS as an outcome measure after surgical repair of TOF/MAPCAs. Balanced lung perfusion was present in the majority of patients who had complete repair of TOF/MAPCAs performed at our center.

Assessment of airway abnormalities in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals.

BACKGROUND: Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/MAPCAs) are at risk for post-operative respiratory complications after undergoing unifocalisation surgery. Thus, we assessed and further defined the incidence of airway abnormalities in our series of over 500 children with TOF/MAPCAs as determined by direct laryngoscopy, chest computed tomography (CT), and/or bronchoscopy. METHODS: The medical records of all patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery from March, 2002 to June, 2018 were reviewed. Anaesthesia records, peri-operative bronchoscopy, and/or chest CT reports were reviewed to assess for diagnoses of abnormal or difficult airway. Associations between chromosomal anomalies and airway abnormalities - difficult anaesthetic airway, bronchoscopy, and/or CT findings - were defined. RESULTS: Of the 564 patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery at our institution, 211 (37%) had a documented chromosome 22q11 microdeletion and 28 (5%) had a difficult airway/intubation reported at the time of surgery. Chest CT and/or peri-operative bronchoscopy were performed in 234 (41%) of these patients. Abnormalities related to malacia or compression were common. In total 35 patients had both CT and bronchoscopy within 3 months of each other, with concordant findings in 32 (91%) and partially concordant findings in the other 3. CONCLUSION: This is the largest series of detailed airway findings (direct laryngoscopy, CT, and bronchoscopy) in TOF/MAPCAS patients. Although these findings are specific to an at-risk population for airway abnormalities, they support the utility of CT and /or bronchoscopy in detecting airway abnormalities in patients with TOF/MAPCAs.

Outcomes After Aortopulmonary Window for Hypoplastic Pulmonary Arteries and Dual-Supply Collaterals.

BACKGROUND: Our institutional approach to tetralogy of Fallot with major aortopulmonary collateral arteries (MAPCAs) emphasizes early unifocalization and complete repair (CR). In the small subset of patients with dual-supply MAPCAs and confluent but hypoplastic central pulmonary arteries (PAs), our surgical approach is early creation of an aortopulmonary window (APW) to promote PA growth. Factors associated with successful progression to CR and mid-term outcomes have not been assessed. METHODS: Clinical data were reviewed. PA diameters were measured offline from angiograms prior to APW and on follow-up catheterization >1 month after APW but prior to any additional surgical interventions. RESULTS: From November 2001 to March 2018, 352 patients with tetralogy of Fallot/MAPCAs underwent initial surgery at our center, 40 of whom had a simple APW with or without ligation of MAPCAs as the first procedure (median age, 1.4 months). All PA diameters increased significantly on follow-up angiography. Ultimately, 35 patients underwent CR after APW. Nine of these patients (26%) underwent intermediate palliative operation between 5 and 39 months (median, 8 months) after APW. There were no early deaths. The cumulative incidence of CR was 65% 1 year post-APW and 87% at 3 years. Repaired patients were followed for a median of 4.2 years after repair; the median PA:aortic pressure ratio was 0.39 (range, 0.22 to 0.74). CONCLUSIONS: Most patients with tetralogy of Fallot/MAPCAs and hypoplastic but normally arborizing PAs and dual-supply MAPCAs are able to undergo CR with low right ventricular pressure after APW early in life. Long-term outcomes were good, with acceptable PA pressures in most patients.

Outcomes After Initial Unifocalization to a Shunt in Complex Tetralogy of Fallot With MAPCAs.

BACKGROUND: Our approach to tetralogy of Fallot (TOF) with pulmonary atresia and major aortopulmonary collateral arteries (MAPCAs) emphasizes early single-stage unifocalization and intracardiac repair. However, a subset of patients with small native pulmonary arteries (PAs) and MAPCAs undergo unifocalization to a shunt rather than simultaneous intracardiac repair. METHODS: Patients with TOF/MAPCAs who underwent complete unifocalization to a systemic-to-PA shunt by a single surgeon were reviewed. The decision to perform simultaneous intracardiac repair was based on an intraoperative flow study or empirical assessment of PA and MAPCA size. RESULTS: From November 2001 to December 2017, 57 patients with TOF/MAPCAs underwent unifocalization to a shunt at a median age of 6.9 months. Genetic abnormalities were documented in 60% of patients, including a chromosome 22q11 deletion in 25 and Alagille syndrome in 8. Twenty patients (35%) had undergone prior surgery elsewhere (n = 16) and/or at our center (n = 7). During a median follow-up of 5.4 years, 9 patients had additional surgery to revise the PA reconstruction before complete repair, and 38 patients underwent complete repair. Five years after unifocalization, survival was 74% ± 6%. At follow-up, the median PA to aortic systolic pressure ratio was 0.36 and was greater than 0.50 in 2 patients. CONCLUSIONS: In patients with the smallest MAPCAs and PAs, single-stage unifocalization to a shunt followed by intracardiac repair yields an excellent outcome in most cases.

Tetralogy of Fallot: Everything you wanted to know but were afraid to ask.

Tetralogy of Fallot (TOF) has four anatomic features: right ventricular hypertrophy (RVH), ventriculoseptal defect (VSD), overriding aorta and right ventricular outflow tract obstruction (RVOT) with an occurrence of 3.9 /10,000 births. The pathophysiologic effects in TOF are largely determined by the degree of RVOT and not the VSD. Intra-operative anesthetic management is also dependent on the degree of RVOT obstruction and influenced by the extent of surgical RVOT repair.

Deletion of 22q11 chromosome is associated with postoperative morbidity after unifocalisation surgery.

BACKGROUND: A 22q11 chromosome deletion is common in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals. We sought to determine whether 22q11 chromosome deletion is associated with increased postoperative morbidity after unifocalisation surgery. METHODS: We included all patients with this diagnosis undergoing primary or revision unifocalisation ± ventricular septal defect closure at our institution from 2008 to 2016, and we excluded patients with unknown 22q11 status. Demographic and surgical data were collected. We compared outcomes between those with 22q11 chromosome deletion and those without using non-parametric analysis. RESULTS: We included 180 patients, 41% of whom were documented to have a chromosome 22q11 deletion. Complete unifocalisation was performed in all patients, and intracardiac repair was performed with similar frequency regardless of 22q11 chromosome status. Duration of mechanical ventilation was longer in 22q11 deletion patients. This difference remained significant after adjustment for delayed sternal closure and/or intracardiac repair. Duration of ICU stay was longer in patients with 22q11 deletion, although no longer significant when adjusted for delayed sternal closure and intracardiac repair. Finally, length of hospital stay was longer in 22q11-deleted patients, but this difference was not significant on unadjusted or adjusted analysis. CONCLUSION: Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals and 22q11 deletion are at risk for greater prolonged mechanical ventilation after unifocalisation surgery. Careful attention should be given to the co-morbidities of this population in the perioperative period to mitigate risks that may complicate the postoperative course.

Repair of Untreated Older Patients With Tetralogy of Fallot With Major Aortopulmonary Collaterals.

BACKGROUND: Our programmatic approach to tetralogy of Fallot with major aortopulmonary collaterals emphasizes single-stage unifocalization with complete intracardiac repair during infancy. Little is known about suitability for complete repair in patients beyond infancy. We sought to analyze outcomes of our approach in older patients with previously untreated tetralogy of Fallot with major aortopulmonary collaterals. METHODS: Any patient with this lesion not treated before 2 years of age referred to our center from 2002 to 2017 met inclusion criteria. RESULTS: Of 33 patients, 32 were out-of-state (64% international) referrals, and 33% (n = 11) were older than 9 years, had polycythemia, or at least 1 high pressure collateral (>25 mm Hg). Complete repair was achieved in 94% (n = 31) of patients, 82% (n = 27) in one stage and 12% (n = 4) after unifocalization to a central shunt. The median right ventricular-to-aortic pressure ratio was 0.31 after the operation and 0.37 at follow-up. At a median of 4.8 years after repair, 9 patients (19%) underwent reintervention, including 5 conduit and 7 branch pulmonary artery interventions. Three patients also underwent aortic valve replacement. CONCLUSIONS: In this selected cohort of older patients with previously unoperated tetralogy of Fallot with major aortopulmonary collaterals, outcomes were comparable with infants undergoing treatment according to our approach. These findings support the notion that patients who are either born in low-resource settings or present to health care providers beyond infancy should be considered candidates and evaluated for complete repair.

Postoperative feeding problems in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals undergoing unifocalisation surgery.

BACKGROUND: Patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals are at risk for prolonged hospitalisation after unifocalisation. Feeding problems after congenital heart surgery are associated with longer hospital stay. We sought to determine the impact of baseline, intra-operative, and postoperative factors on the need for feeding tube use at the time of discharge. METHODS: We included patients with the aforementioned diagnosis undergoing unifocalisation from ages 3 months to 4 years from 2010 to 2016. We excluded patients with a pre-existing feeding tube. Patients discharged with an enteric tube were included in the feeding tube group. We compared the feeding tube group with the non-feeding-tube group by univariable and multi-variable logistic regression. RESULTS: Of the 56 patients studied, 41% used tube feeding. Median age and weight z-score were similar in the two groups. A chromosome 22q11 deletion was associated with the need for a feeding tube (22q11 deletion in 39% versus 15%, p=0.05). Median cardiopulmonary bypass time in the feeding tube group was longer (335 versus 244 minutes, p=0.04). Prolonged duration of mechanical ventilation was associated with feeding tube use (48 versus 3%, p=0.001). On multi-variable analysis, prolonged mechanical ventilation was associated with feeding tube use (odds ratio 10.2, 95% confidence intervals 1.6; 63.8). CONCLUSION: Among patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals who were feeding by mouth before surgery, prolonged mechanical ventilation after unifocalisation surgery was associated with feeding tube use at discharge. Anticipation of feeding problems in this population and earlier feeding tube placement may reduce hospital length of stay.

Association of dead space ventilation and prolonged ventilation after repair of tetralogy of Fallot with pulmonary atresia.

BACKGROUND: We set out to determine whether patients with tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (TOF/PA/MAPCA) are at risk for elevated dead space ventilation fraction (VD/VT), and whether this is associated with prolonged mechanical ventilation. We hypothesized that elevated VD/VT (>20%) in the first 24 hours after unifocalization surgery is associated with increased risk for prolonged mechanical ventilation (>7 days). METHODS: All patients with TOF/PA/MAPCA undergoing unifocalization surgery between January 2003 and December 2015 were included in this study. Average VD/VT was calculated over the first 24 hours after surgery. Demographic and surgical data were collected. Outcome data included duration of mechanical ventilation. Patients were separated into 2 groups: elevated VD/VT and normal DVSF. Groups were compared using the Student t test, Wilcoxon rank-sum test, and χ2 test. Univariable and multivariable regression analyses were performed with VD/VT as a continuous variable to test for association. RESULTS: Of the 265 included patients, 127 (48%) had an elevated VD/VT. The 2 groups did not differ significantly in any demographic characteristic. Patients with an elevated VD/VT had longer cardiopulmonary bypass times (P = .03), were more likely to have delayed sternal closure, and more likely to have prolonged respiratory failure (odds ratio, 2.2; 95% confidence interval, 1.2-4.0; P = .007). The percent VD/VT was associated with duration of mechanical ventilation in univariable (P < .001) and multivariable (P < .001) regression analyses when controlled for age, weight and bypass time. CONCLUSIONS: Elevated postoperative VD/VT is associated with prolonged mechanical ventilation in patients with TOF/PA/MAPCA following unifocalization. Elevated postoperative VD/VT may be an early indicator of patients who will require prolonged duration of mechanical ventilation, allowing optimization of medical management to promote better outcomes.

Anesthetic Management During Surgery for Tetralogy of Fallot With Pulmonary Atresia and Major Aortopulmonary Collateral Arteries.

Tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals (TOF/PA/MAPCAs) is a heterogeneous disease with varying degrees of severity, requiring complex anesthetic management. Our institution has adopted the approach of early complete repair with incorporation of all lung segments, extensive lobar and branch segmental pulmonary artery reconstruction, and ventricular septal defect closure. While the surgical management of TOF/PA/MAPCAs has been extensively described and varies depending on the institution, there is a paucity of literature on the anesthetic management for such procedures. Herein, we describe our anesthetic management based on our own institution's surgical approach at Lucile Packard Children's Hospital/Stanford University.

Pulmonary reinterventions after complete unifocalization and repair in infants and young children with tetralogy of Fallot with major aortopulmonary collaterals.

BACKGROUND: Our institutional approach to tetralogy of Fallot (TOF) with major aortopulmonary collaterals (MAPCAs) emphasizes unifocalization and augmentation of the reconstructed pulmonary arterial (PA) circulation and complete intracardiac repair in infancy, usually in a single procedure. This approach yields a high rate of complete repair with excellent survival and low right ventricular (RV) pressure. However, little is known about remodeling of the unifocalized and reconstructed pulmonary circulation or about reinterventions on the reconstructed PAs or the RV outflow tract conduit. METHODS: We reviewed patients who underwent complete repair of TOF with MAPCAs at our center at <2 years of age, either as a single-stage procedure or after previous procedures. Outcomes included freedom from conduit or PA intervention after repair, which were assessed by Cox regression and Kaplan-Meier analysis. RESULTS: The study cohort included 272 patients. There were 6 early deaths and a median of follow-up of 3.6 years after complete repair. Reinterventions on the pulmonary circulation were performed in 134 patients, including conduit interventions in 101 patients, branch PA interventions in 101, and closure of residual MAPCAs in 9. The first conduit reintervention consisted of surgical conduit replacement in 77 patients, transcatheter pulmonary valve replacement with a Melody valve in 14, and angioplasty or bare metal stenting in 10. Surgical PA reinterventions were performed in 46 patients and transcatheter reinterventions in 75 (both in 20). Most PA reinterventions involved a single lung, and most transcatheter reinterventions a single vessel. Freedom from conduit replacement or transcatheter pulmonary valve replacement was 70 ± 3% at 5 years and was shorter in patients with smaller initial conduit size. Freedom from any PA reintervention was 64 ± 3% at 5 years, with the greatest rate during the first year. On multivariable analysis, factors associated with longer freedom from any PA reintervention included lower postrepair RV:aortic pressure ratio and larger original conduit size. CONCLUSIONS: We were able to obtain follow-up data for the majority of patients, which demonstrated freedom from PA reintervention for two thirds of patients. The time course of and risk factors for conduit reintervention in this cohort appeared similar to previously reported findings in patients who received RV-PA conduits in early childhood for other anomalies. Relative to the severity of baseline pulmonary vascular anatomy in TOF with MAPCAs, reinterventions on the reconstructed PAs were uncommon after repair according to our approach, and major reinterventions were rare. Nevertheless, PA reinterventions are an important aspect of the overall management strategy.