Single-Ventricle Outcomes After Neonatal Palliation of Severe Ebstein Anomaly With Modified Starnes Procedure.

BACKGROUND: We have previously shown that neonates in profound cardiogenic shock caused by a severe Ebstein anomaly can be successfully salvaged with fenestrated right ventricular exclusion and systemic to pulmonary shunt (modified Starnes procedure). The long-term outcome of single-ventricle management in these patients is not known. METHODS: We retrospectively reviewed the records of patients who underwent neonatal Starnes procedure between 1989 and 2015. Patient demographics, clinical variables, and outcome data were collected. RESULTS: Twenty-seven patients (13, 48% boys) underwent the Starnes procedure at 7 (5-9) days of life. All were intubated and on prostaglandin, 24 (89%) were inotrope dependent, and 22 (81%) had no antegrade flow from the right ventricle. Three patients underwent nonfenestrated right ventricular exclusion, 2 (67%) of whom died. Of the remaining 24, 3 (13%) died during the same hospitalization. The 22 neonatal survivors have been followed for 7 (6-8) years: 1 patient is awaiting a Glenn procedure; 1 died after undergoing a Glenn procedure; and the remaining 20 patients have successfully undergone Fontan completion. Their indexed pulmonary vascular resistance was 1.8 (1.2-2.3) W/m2, and mean pulmonary pressure was 12 (9-18) mm Hg. At last follow-up, 1 patient had died, and the remaining patients had normal left ventricular function, and all but 1 have New York Heart Association class I symptoms. Two patients have required pacemaker implantation, whereas the rest are in sinus rhythm. Survival for the entire cohort at 1, 5, and 10 years is 81±4%, 81±5%, and 76±3%, respectively, whereas for those with fenestrated right ventricular exclusion, survival at 1, 5, and 10 years is 87±2%, 87±2%, and 81±4%, respectively. CONCLUSIONS: Long-term single-ventricle outcomes among neonatal survivors of the modified Starnes procedure are excellent. There is reliable remodeling of the excluded right ventricle and good function of the left ventricle.

A Novel Model for Xenograft Right Ventricle to Pulmonary Artery Conduit.

The last 40 years have shown dramatic improvement in outcomes for neonatal cardiac surgery for a spectrum of congenital heart disease diagnoses. With more patients surviving into adulthood, the long-term impact of initial management strategies of these patients has come into focus. This is particularly true for patients with pediatric heart valve disease. Many patients born with right ventricular to pulmonary artery (RVPA) discontinuity require placement of a valved conduit in the neonatal period. Valved conduit options are limited in this patient population due to patient size and inability to respond to somatic growth. Genetically engineered porcine (GEP) donors may offer a xenograft conduit alternative that can grow with the patient. We have developed a model utilizing GEP donor RVPA conduits placed in infantile nonhuman primate (NHP) recipients. Our recipient is maintained on single-drug immunosuppression and demonstrates no evidence of pulmonary valve insufficiency or stenosis during short-term follow-up. Further studies and long-term outcomes are necessary to determine the utility of this technology in human application.

Surgical pulmonary arterioplasty at bidirectional cavopulmonary anastomosis leads to favorable pulmonary hemodynamics at final stage palliation.

Objective: Pulmonary arterioplasty (PA plasty) at bidirectional cavopulmonary anastomosis (BDCA) is associated with increased morbidity, but outcomes to final stage palliation are unknown. We sought to determine the influence of PA plasty on pulmonary artery growth and hemodyamics at Fontan. Methods: We retrospectively reviewed clinical data and outcomes for BDCA patients from 2006 to 2018. PA plasty was categorized by extent (type 1-4), as previously described. Outcomes included pulmonary artery reintervention and mortality before final palliation. Results: Five hundred eighty-eight patients underwent BDCA. One hundred seventy-nine patients (30.0%) underwent concomitant PA plasty. Five hundred seventy (97%) patients (169 [94%] PA plasty) survived to BDCA discharge. One hundred forty out of 570 survivors (25%) required PA/Glenn reintervention before final stage palliation (59 out of 169 [35%]) PA plasty; 81 out of 401 (20%) non-PA plasty; P < .001). Twelve-, 24-, and 36-month freedom from reintervention after BDCA was 80% (95% CI, 74-86%), 75% (95% CI, 69-82%), and 64% (95% CI, 57-73%) for PA plasty, and 95% (95% CI, 93-97%), 91% (95% CI, 88-94%), and 81% (95% CI, 76-85%) for non-PA plasty (P < .001). Prefinal stage mortality was 37 (6.3%) (14 out of 169 PA plasty; 23 out of 401 non-PA plasty; P = .4). Five hundred four (144 PA plasty and 360 non-PA plasty) patients reached final stage palliation (471 Fontan, 26 1.5-ventricle, and 7 2-ventricular repair). Pre-Fontan PA pressure and pulmonary vascular resistance were 10 mm Hg (range, 9-12 mm Hg) and 1.6 mm Hg (range, 1.3-1.9 mm Hg) in PA plasty and 10 mm Hg (range, 8-12 mm Hg) and 1.5 mm Hg (range, 1.3-1.9 mm Hg) in non-PA plasty patients, respectively (P = .29, .6). Fontan hospital mortality, length of stay, and morbidity were similar. Conclusions: PA plasty at BDCA does not confer additional mortality risk leading to final palliation. Despite increased pulmonary artery reintervention, there was reliable pulmonary artery growth and favorable pulmonary hemodynamics at final stage palliation.

Ross procedure in neonates and infants: A valuable operation with defined limits.

OBJECTIVE: The Ross procedure is an important tool that offers autologous tissue repair for severe left ventricular outflow tract (LVOT) pathology. Previous reports show that risk of mortality is highest among neonates and infants. We analyzed our institutional experience within this patient cohort to identify factors that most affect clinical outcome. METHODS: A retrospective chart review identified all Ross operations in neonates and infants at our institution over 27 years. The entire study population was analyzed to determine risk factors for mortality and define outcomes for survival and reintervention. RESULTS: Fifty-eight patients underwent a Ross operation at a median age of 63 (range, 9-156) days. Eighteen (31%) were neonates. Eleven (19%) patients died before hospital discharge. Multiple regression analysis of the entire cohort identified young age (hazard ratio [HR], 1.037; P = .0045), Shone complex (HR, 17.637; P = .009), and interrupted aortic arch with ventricular septal defect (HR, 16.01; P = .031) as independent predictors of in-hospital mortality. Receiver operating characteristic analysis (area under the curve, 0.752) indicated age younger than 84 days to be the inflection point at which mortality risk increases. Of the 47 survivors, there were 2 late deaths with a mean follow-up of 6.7 (range, 2.1-13.1) years. Three patients (6%) required LVOT reintervention at 3, 8, and 17.5 years, respectively, and 26 (55%) underwent right ventricular outflow tract reintervention at a median of 6 (range, 2.5-10.3) years. CONCLUSIONS: Ross procedure is effective in children less than one year of age with left sided obstructive disease isolated to the aortic valve and/or aortic arch. Patients less than 3 months of age with Shone or IAA/VSD are at higher risk for morbidity and mortality. Survivors experience excellent intermediate-term freedom from LVOT reintervention.

Recommendations for centers performing pediatric heart surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Recommendations for Centers Performing Pediatric Heart Surgery in the United States.

Care and outcomes for the more than 40,000 patients undergoing pediatric and congenital heart surgery in the United States annually are known to vary widely. While consensus recommendations have been published across numerous fields as one mechanism to promote a high level of care delivery across centers, it has been more than two decades since the last pediatric heart surgery recommendations were published in the United States. More recent guidance is lacking, and collaborative efforts involving the many disciplines engaged in caring for these children have not been undertaken to date. The present initiative brings together professional societies spanning numerous care domains and congenital cardiac surgeons, pediatric cardiologists, nursing, and other healthcare professionals from diverse programs around the country to develop consensus recommendations for United States centers. The focus of this initial work is on pediatric heart surgery, and it is recommended that future efforts focus in detail on the adult congenital population. We describe the background, rationale, and methodology related to this collaborative effort, and recommendations put forth for Essential Care Centers (essential services necessary for any program), and Comprehensive Care Centers (services to optimize comprehensive and high-complexity care), encompassing structure, process, and outcome metrics across 14 domains.

Fate of the Right Ventricular Outflow Tract Following Valve-Sparing Repair of Tetralogy of Fallot.

Valve-sparing repair (VSR) of tetralogy of Fallot (TOF) tends to result in higher residual right ventricular outflow tract (RVOT) gradients. We evaluated the progression and clinical implications of RVOT gradients following VSR of TOF. Demographic, clinical, and operative data were retrospectively collected from consecutive TOF patients who underwent VSR at our institution between 01/2010 and 06/2021. RVOT gradient, pulmonary valve annulus (PVA) diameter and Boston Z-scores were recorded from serial echocardiograms. Data are presented as median and interquartile range or number and percentage. A total of 156 children (boys 92, 59%) underwent VSR at 6.5 (4.9-8.4) months of age and 6.6 kg (5.6- 7.7) weight. There was 1 (0.6%) operative mortality. The remaining 155 patients were followed for 69.4 months (4-106.2). RVOT gradient was 2.4m/s (1.7-2.9) at discharge. It transiently increased, then declined and stabilized during follow-up. PVA Z-score was -1.7 (-3.1 to 0.5) at discharge and 'grew' to -0.8 (-1.7 to 0.4) at last follow-up. Freedom from RVOT re-intervention was 97%, 94% and 91% at 1, 5 and 10-year follow-up. Among 67 (43%) patients with PVA Z-score < -2, a similar RVOT gradient pattern was observed and freedom from RVOT re-intervention was 97%, 95% and 95% at 1, 5 and 8-year follow-up. Following VSR of TOF, RVOT gradients transiently increase and then fall as PVA growth catches up, resulting in durable intermediate outcomes. Patients with PVA Z-score < -2 demonstrated a similar pattern of hemodynamics in the RVOT and excellent freedom from reintervention.

Resident education in congenital heart surgery does not compromise outcomes.

OBJECTIVE: Most of all congenital cardiac surgical programs participate in public outcomes reporting. The primary end point is transparency. In this era, academic programs with surgical residents face the challenge of producing outstanding results while allowing residents to learn by doing. We sought to understand the effect of education on our surgical outcomes. METHODS: We collected data for all American Board of Thoracic Surgery index cases done at our institution over a 10-year period. We identified 3406 cases and categorized them into 2 groups according to primary surgeon: attending (2269) versus resident (1137). In a multivariable logistic regression model we examined the effect of operating surgeon on in-hospital mortality, major morbidity, and length of stay. We used propensity score matching subsequently to balance differences between cohorts, and multivariable logistic regression was repeated. RESULTS: Using the entire cohort, multivariable logistic regression model adjusted for age, sex, weight, lack of preoperative comorbidity, presence of preoperative respiratory failure, The Society of Thoracic Surgeons--European Association for Cardio-Thoracic Surgery category, and need for deep hypothermic circulatory arrest, showed a higher odds of survival in the resident cohort (odds ratio, 1.484; 95% confidence interval, 0.998-2.206; P = .05). Propensity score matching identified 1137 pairs of attending and resident cases with well-balanced preoperative variables. Logistic regression modeling using the matched cohort showed equivalent 30-day mortality, 30-day major morbidity, and length of stay. CONCLUSIONS: There was no difference in mortality, major morbidity, or length of stay when similar cases were compared that were operated on by attendings versus those by a resident. Effectively educating congenital heart surgeons without compromising an operation's quality requires thoughtful approach, including case selection and graded responsibility.

Biventricular Repair in Interrupted Aortic Arch and Ventricular Septal Defect With a Small Left Ventricular Outflow Tract.

BACKGROUND: In patients with interrupted aortic arch and ventricular septal defect (VSD) with a small left ventricular outflow tract (LVOT), either aortopulmonary amalgamation or a Ross-Konno type procedure can be performed to create stable systemic outflow. We sought to analyze factors associated with these different surgical approaches. METHODS: We retrospectively identified patients who underwent surgical repair for interrupted aortic arch/VSD at our institution between 1998 and 2017. Of these, 43 patients had a small, native LVOT that was unsuitable for systemic outflow. Patient data were retrospectively collected for this cohort and analyzed. RESULTS: Aortopulmonary amalgamation was performed at 7 days (interquartile range [IQR], 5-10) in 30 patients (group I). Within group I a primary Yasui repair with ventricular septation was performed in 3 patients and a Norwood-type repair in the other 27. Of these 27, 19 underwent subsequent biventricular conversion at 9 months (IQR, 7-11). In contrast 13 patients underwent a Ross procedure at 12 days (IQR, 6-27) (group II). Compared with group I, group II patients had a smaller VSD (3.5 vs 5.1 mm, P < .001) that was more often remote from the semilunar valves (38% vs 13%, P = .02). Operative mortality occurred in 1 group I patient (4%) at the time of biventricular conversion and 2 group II patients (15%) during the Ross procedure. After a 5.2-year (IQR, 3.2-7.4) follow-up there were 2 additional mortalities in each group, all unrelated to cardiac disease. CONCLUSIONS: When native LVOT in interrupted aortic arch/VSD is unsuitable for systemic outflow, size and location of the VSD can be used to tailor the surgical approach to establish biventricular circulation with favorable intermediate-term outcomes.

Novel use of a vascular plug to anchor an azygous vein ICD lead.

We describe the case of a young patient with severe hypertrophic cardiomyopathy and marginal defibrillation thresholds (DFTs) at implant of a standard transvenous implantable cardioverter-defibrillator (ICD) system. The patient subsequently experienced multiple failed ICD shocks during a prolonged episode of spontaneous ventricular tachycardia/fibrillation. Placement of a second single-coil shocking lead in the azygous vein resulted in acceptable DFTs, but the new lead migrated superiorly within hours of the procedure. To stabilize the lead position, a vascular plug was placed in the distal azygous vein, and the shocking lead screw was actively fixated to the meshwork of the device. Subsequent testing confirmed both adequate defibrillation and stable lead position.

Reducing the incidence of necrotizing enterocolitis in neonates with hypoplastic left heart syndrome with the introduction of an enteral feed protocol.

OBJECTIVE: Neonates with hypoplastic left heart syndrome are prone to gastrointestinal complications, including necrotizing enterocolitis, during initiation or advancement of enteral feeds. A feeding protocol was developed to standardize practice across a multidisciplinary team. The purpose of this study was to examine the impact of a standardized feeding protocol on the incidence of necrotizing enterocolitis and overall postoperative gastrointestinal morbidity. DESIGN: Retrospective case-control study. SETTING: Cardiothoracic intensive care unit of a tertiary care children's hospital. PATIENTS: Ninety-eight neonates with hypoplastic left heart syndrome admitted to the cardiothoracic intensive care unit after first-stage palliation. INTERVENTION: A retrospective chart review was performed. Two groups were analyzed: the preprotocol group (n = 52) was examined from January 2000 through December 31, 2001, and the postprotocol group (n = 46) from February 2002 through December 31, 2003. MEASUREMENTS AND MAIN RESULTS: The incidence of suspected or diagnosed necrotizing enterocolitis as defined by the modified Bell staging criteria was recorded. Data were also collected regarding postoperative day of enteral feed initiation, postoperative day full feeds attained, and postoperative hospital length of stay. Necrotizing enterocolitis was detected in 14 preprotocol (27%) and three postprotocol (6.5%) patients (p < .01). Enteral feeds were initiated later in the postprotocol group (7.5 vs. 5.5 days, p < .001), and number of days to full feeds was also later in the postprotocol group (7 vs. 4 days, p = .02). Hospital length of stay tended to be shorter in the postprotocol group (21.5 vs. 28 days, p = .25). CONCLUSION: Measures directed at reducing the incidence of necrotizing enterocolitis may reduce morbidity in neonates with hypoplastic left heart syndrome and reduce cost by decreasing hospital length of stay. A standardized feeding protocol instituted to address these problems likely contributed to reducing the incidence of necrotizing enterocolitis in this high-risk population.

Impact of prior diaphragm plication on subsequent stages of single ventricle palliation.

BACKGROUND: Phrenic nerve injury is a known cause of morbidity after single ventricle palliation. Previous studies have shown that hemidiaphragm plication improves short-term outcomes. The effect of plication on the outcomes of subsequent stages of single ventricle palliation is unknown. METHODS: From 1997 to 2015, 1146 patients underwent surgical management of single ventricle physiology at our institution. We reviewed the records of 30 patients who had undergone diaphragm plication for phrenic nerve injury before Fontan completion. Each patient was compared with 2 propensity-matched controls identified from patients who underwent the Glenn or Fontan procedure during the same period without diaphragm plication. Propensity matching was achieved for each test subject using the nearest neighbor algorithm. Data are presented as the median and quartiles or numbers and percentages. RESULTS: The cohort included 18 boys (60%). Of the 30 patients, 19 (63%) had undergone plication after first-stage palliation. Of these, 13 have undergone completion Fontan, 5 were awaiting Fontan at the last follow-up, and 1 had died. An additional 11 patients had undergone plication after Glenn and proceeded to Fontan completion. Thus, 24 patients with diaphragm plication have undergone Fontan completion. No difference was found in pulmonary pressure or resistance between the plicated patients and their propensity-matched controls. Both groups had comparable chest tube output and hospital lengths of stay. Equal proportions of patients in both groups required pulmonary vasodilator therapy and/or supplemental oxygen at hospital discharge. CONCLUSIONS: Prior diaphragm plication does not adversely affect Fontan completion in children with single ventricle physiology. The hospital course during subsequent stages of palliation for plicated patients was no different than that of matched controls.

Obstruction of lateral tunnel fontan by an adjustable occluder device.

Innovative ways of adjusting Fontan fenestrations have been developed over the last two decades. Long-term complications have not been described. We successfully treated one such complication of Fontan conduit obstruction due to an adjustable occluder device.

Simultaneous Systemic to Pulmonary Shunt and Pulmonary Artery Banding is a Viable Option for Neonatal Palliation of Single Ventricle Physiology.

A subset of neonates with single ventricle (SV) physiology has antegrade pulmonary blood flow that is deemed unlikely to be reliable until Glenn. We have used systemic to pulmonary shunt (SPS) with pulmonary artery banding (PAB) to optimize pulmonary blood flow while maintaining reserve antegrade flow. We hypothesize that this is an effective strategy that can be accomplished without the routine need for cardiopulmonary bypass. We retrospectively reviewed the records of 60 neonates who underwent combined SPS + PAB between 2004 and 2015. Data are presented as median with quartiles. Children were 8 (4-19) days old at surgery and included 38 (63%) boys. Atresia or severe stenosis of the subpulmonary atrioventricular (AV) valve associated with pulmonary blood flow across a bulboventricular foramen was present in 37 (62%). In 20 (33%), heterotaxy-associated unbalanced AV canal with pulmonary stenosis with or without anomalous pulmonary venous drainage was present. First-stage palliation was accomplished without cardiopulmonary bypass in 44 patients (73%). There were 7 (12%) hospital deaths, 4 among the 20 (20%) with heterotaxy. Fifty-three children were followed for a median 5.1 (1.8-8.2) years. Three early reinterventions were required after initial palliation (1 PAB adjustment, 2 SPS balloon angioplasties). Five additional heterotaxy patients experienced late mortality during follow-up. There were no early or emergent Glenn. Thirty-nine patients have reached Fontan circulation with a median pre-Fontan PA pressure of 14 (12-18) mm Hg. One patient converted to biventricular physiology and the remaining await completion Fontan. Heterotaxy was the only independent predictor of mortality (hazard ratio 10 (2.3-44, P < 0.001). In SV patients with unreliable antegrade PA flow, SPS + PAB is an effective first-stage palliation. SV patients with heterotaxy are at increased risk for mortality.

Liberal Use of Delayed Sternal Closure in Children Is Not Associated With Increased Morbidity.

BACKGROUND: Delayed sternal closure (DSC) is often employed to optimize hemodynamics following pediatric cardiac surgery. Prior reports have suggested that DSC may be associated with increased morbidity. We sought to analyze the impact of a liberal policy of DSC on surgical outcomes at our center. METHODS: We retrospectively evaluated the clinical course of 1,000 consecutive patients between July 2005 and June 2015 whose sternum was electively left open following pediatric cardiac surgery. Data are presented as mean and standard error (parametric) or median and quartiles (nonparametric). Receiver-operating characteristic curve analysis was undertaken to identify significant points of inflection. A p less than 0.05 was considered significant. RESULTS: An a priori decision to leave the sternum open is made when complex surgery, especially in neonates and usually involving circulatory arrest, is expected to result in postoperative hemodynamic instability. Age at index surgery for the 1,000 patients was 7 (interquartile range [IQR], 3 to 19) days and weight 3.3 (IQR, 2.8 to 3.7) kg. There were 816 (82%) neonates and 569 (57%) boys. Index operations included 332 (33%) Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category 5, 483 (48%) STAT category 4, and 185 (19%) STAT category 3 procedures. A total of 103 (10%) patients required postoperative extracorporeal support. Following hemodynamic recovery, DSC was undertaken 3 (IQR, 2 to 4) days postoperatively and in 98.3% patients was performed in the intensive care unit. Overall, mortality was 6.3% and major Society of Thoracic Surgeons morbidity was 21.6%. There were 42 (4.2%) positive mediastinal surveillance cultures at the time of DSC, with the most common organism being coagulase-negative staphylococcus. Fifty-nine (5.9%) clinical sternal and mediastinal wound infections and a total of 117 infectious complications were encountered in 94 patients. Using Society of Thoracic Surgeons database outcome as benchmark, mortality and length of stay in our patients were comparable when analyzed by STAT categories or for the 2 most common index procedures (eg, Norwood and arterial switch operations). Receiver-operating characteristic curve analysis showed that 5 days of open sternum had a weak, but statistically significant, correlation with incidence of infectious complications (area under the curve, 0.56; p = 0.002). The need for DSC 5 or more days after the index procedure was observed in 177 (18%) patients and was not associated with increased wound infection. It was, however, independently associated on multiple regression analysis with major morbidity (odds ratio, 1.7; 95% confidence interval, 1.2 to 2.5; p = 0.002) and, in the subset of 897 patients who did not require extracorporeal support, with increased mortality (odds ratio, 2.2; 95% confidence interval, 1.3 to 3.6; p = 0.003). CONCLUSIONS: A liberal policy of DSC does not adversely affect surgical outcomes, including infectious complications and length of stay. We submit that need for DSC should not, by itself, be considered a source of morbidity.

Arch Augmentation via Median Sternotomy for Coarctation of Aorta With Proximal Arch Hypoplasia.

BACKGROUND: Coarctation of the aorta can be associated with hypoplasia of the proximal transverse aortic arch. One approach to manage this condition is via left thoracotomy and extended end-to-end anastomosis with the expectation that the proximal arch will grow over time. Our preferred approach is to augment the aorta via midline sternotomy. We hypothesized that this approach is safe, durable, and allows reliable growth of the aorta. METHODS: We identified the records of patients with biventricular anatomy who had coarctation of the aorta, hypoplasia of the proximal transverse arch, and no other cardiac lesion that would mandate cardiopulmonary bypass use and midline sternotomy. The records of 62 such patients operated on between 2005 and 2016 were retrospectively reviewed. Patient demographics, clinical variables and outcome data were collected and analyzed using SAS 9.4. Data are presented as median (interquartile range [IQR]). RESULTS: Sixty-two patients (23 girls [37%]) underwent repair at 10 (IQR, 5 to 21) days of life. Forty-nine (79%) patients were on prostaglandin infusion to maintain ductal patency. Fifteen (24%) patients presented in shock with end organ dysfunction, 17 (27%) were on inotropes, and 26 (42%) were mechanically ventilated. The proximal transverse arch was 41% (IQR, 34% to 47%) of the size of ascending aorta as measured by echocardiography (z-score, -5 [IQR, -5.8 to -4.3]). Following median sternotomy, repair was carried out on cardiopulmonary bypass (41 [IQR, 37 to 47] minutes). The arch was reconstructed with (n = 26 [42%]) or without (n = 36 [58%]) coarctectomy usually using homograft patch aortoplasty (n = 58 [94%]). In all but 2 patients, repair was undertaken with circulatory arrest (27 [IQR, 22 to 31] minutes). Patients were extubated 4 (IQR, 3 to 5) days later and discharged home in 12 (IQR, 8 to 18) days. There was no mortality, and 8 morbidity events (3 recurrent nerve injury, 2 chylothorax, 1 phrenic nerve injury, 1 seizure, and 1 superficial wound infection) in 7 (11%) patients. All patients are alive at 41 (IQR, 11 to 64) months of follow-up. Reintervention was required in 6 (10%) patients (5 catheter based and 3 surgical) for recurrent distal coarctation. Reintervention-free survival at 1, 3, and 5 years was 87%. Only 1 child was currently on antihypertensive therapy, and all were in New York Heart Association functional class I symptoms. At last echocardiogram, the proximal transverse arch was 97% (IQR, 84% to 103%) of the diameter of the ascending aorta (z-score, 0.8 (IQR, 0.3 to 1.3]), ejection fraction was 70% (IQR, 60% to 76%), and only 2 patients had significant left ventricular hypertrophy. CONCLUSIONS: Arch augmentation via median sternotomy is a safe and effective procedure that can be accomplished with low morbidity and mortality. The reconstructed arch retains excellent growth potential resulting in a very favorable physiologic outcome.

Need for Pulmonary Arterioplasty During Glenn Independently Predicts Inferior Surgical Outcome.

BACKGROUND: Bidirectional cavopulmonary anastomosis (BDCA) can be accomplished with low morbidity and mortality. The impact of concomitant pulmonary arterioplasty (PAplasty) is not known. We hypothesized that the need for and extent of PAplasty adversely affect BDCA outcomes. METHODS: Patients who underwent BDCA at our institution between 2006 and 2014 were included. Patient demographics, operative characteristics, mortality, and morbidity were analyzed. Serious physiologic adverse event following Glenn (GAE) was defined as need for extracorporeal support, BDCA takedown or percutaneous intervention during same admission, hospital length of stay 1 SD or more from mean, or need for supplemental oxygen at discharge. PAplasty was categorized according to extent. Data were analyzed using SAS 9.4 (SAS Institute, Cary, NC). RESULTS: A total of 424 patients (231 boys, 54%) underwent BDCA for single ventricle physiology at a median of 7 (5.5 to 8.9) months of age and 6.5 (5.7 to 7.7) kg weight. A total of 112 (26%) patients required PAplasty: 23 were patch closures of the divided distal PA (type 1), 45 were central PA augmentations (type 2), 23 extended to the hilum on 1-branch PA (type 3), and 21 were bilateral hilum to hilum augmentation (type 4). Patients who required PAplasty tended to be significantly younger and more likely to have single right ventricles. There was no difference in PA pressure or resistance between patients who did and did not require PAplasty. Major Society of Thoracic Surgeons morbidity (13% vs 6%; p = 0.001), GAE (45% vs 34%; p = 0.04), and in-hospital mortality (5.4% vs 1.9%; p = 0.03) were higher in patients who required PAplasty compared with those who did not. Among the operative variables evaluated, need for PAplasty (hazard ratio [HR], 1.6; p = 0.03) independently predicted hospital mortality. Need for circulatory arrest (HR, 4; p = 0.005) and PAplasty (HR, 2.4; p = 0.0006) were independent predictors of Society of Thoracic Surgeons morbidity and need for PAplasty independently predicted GAE (HR, 1.8; p = 0.03). CONCLUSIONS: The need for PAplasty at BDCA is an independent predictor of mortality and morbidity. It is important to consider this variable when developing outcome metrics for BDCA.

Mesenteric blood flow velocities in the newborn with single-ventricle physiology: modified Blalock-Taussig shunt versus right ventricle-pulmonary artery conduit.

BACKGROUND: Neonates with ductal-dependent single-ventricle congenital heart disease palliated with a modified Blalock-Taussig shunt (mBTS) commonly have retrograde diastolic flow in the aorta, which may place them at increased risk of mesenteric ischemia. Recently, palliation with a right ventricle-to-pulmonary artery conduit, known as the Sano procedure, has been shown to eliminate retrograde diastolic flow, theoretically leading to better systemic perfusion. OBJECTIVE: To compare the changes in superior mesenteric artery (SMA) and celiac artery velocities and flow after a bolus enteral feed in patients with single-ventricle congenital heart disease palliated with an mBTS vs. those palliated with the right ventricle-to-pulmonary artery conduit. DESIGN: Prospective clinical study. SETTING: Cardiothoracic intensive care unit and pediatric ward of a tertiary care children's hospital. PATIENTS: A total of 27 patients with single-ventricle congenital heart disease (15 with mBTS, 12 with Sano) after stage-1 palliation. INTERVENTION: Doppler ultrasound of the SMA and celiac artery was performed 30 mins before and after a bolus enteral feed. MEASUREMENTS AND MAIN RESULTS: SMA and celiac artery peak systolic flow velocity, mean flow velocity, and time-velocity integral were measured. After a bolus enteral feed, 8 of 15 infants palliated with an mBTS had retrograde diastolic flow through the SMA yet demonstrated significant increases in all variables of both the SMA and celiac artery flow velocities (SMA peak systolic flow velocity: 0.96 +/- 0.33 vs. 1.2 +/- 0.4 m/sec, p = .01). Those palliated with the Sano procedure did not demonstrate SMA retrograde diastolic flow but also did not have any significant changes in their mesenteric flow variables (SMA peak systolic flow velocity: 0.79 +/- 0.16 vs. 0.89 +/- 0.26 m/sec, p = .2). CONCLUSION: Postprandial retrograde diastolic flow was observed in the majority of patients palliated with an mBTS vs. none of the patients in the Sano group. However, contrary to expectations, postprandial mesenteric blood flow velocities in those palliated with an mBTS are significantly higher than in Sano patients, although the increase is not as high as that historically seen in normal neonates. This may place this population at risk for mesenteric ischemia and feeding intolerance in the postoperative period, and the risk may be even greater for those neonates palliated with a right ventricle-to-pulmonary artery conduit.

Outcomes of Reintervention on the Autograft After Ross Procedure.

BACKGROUND: After a Ross procedure, a small subset of patients requires reintervention for autograft dilatation or valve insufficiency. We sought to determine the indications, nature, and outcomes of autograft reinterventions in the left ventricular outflow tract after a Ross procedure. METHODS: We retrospectively reviewed the charts of 316 consecutive patients, age 4 days to 70 years, who underwent a Ross procedure at our institution. Of these, 47 patients (15%) required autograft reintervention during a median follow-up of 8.2 years. RESULTS: Forty-seven patients, median age 26 years (interquartile range [IQR], 14.4 to 44.8 years), 32 (68%) men, required autograft reintervention a median 5.5 years (IQR, 1.8 to 9.4 years) after a Ross procedure. In 47% (22 of 47 patients), the autograft valve could be salvaged (15 valve-sparing aortic replacements, 7 valve repairs). The remaining 53% underwent replacement of the root (10 mechanical, 5 homograft) or valve alone (7 mechanical, 3 bioprosthetic). Twenty patients presented without autograft root dilatation 1 year (IQR, 0.5 to 2.9 years) after Ross. Fifteen of them (75%) required valve replacement. The 27 patients who demonstrated root dilatation presented 6.9 years (IQR, 4.5 to 9.7 years; p < 0.01 compared with the nondilated group) after Ross, and 17 (63%) of these valves could be spared (p = 0.01). There was no surgical mortality and 5 (11%) major morbidity events. Patients were followed up for 4.9 years (IQR, 2.1 to 7 years) after left ventricular outflow tract reintervention. For patients whose autograft valve could be spared, 3-, 5-, and 8-year freedom from valve replacement was 92%, 86%, and 86%, respectively. At last follow-up, only 1 patient had greater than mild aortic insufficiency, and all but 1 had normal ejection fraction. CONCLUSIONS: Autograft reintervention after a Ross appears to follow a bimodal distribution. Patients with primary autograft leaflet problems tend to present early without root dilatation and frequently require valve replacement. The autograft valve can be salvaged in the majority of patients who present later with root dilatation. Valve-sparing procedures are durable in the intermediate term and can be accomplished with low morbidity.