Midterm outcomes of right ventricular outflow tract reconstruction using the Freestyle xenograft.

Kuo JA, Hamby T, Munawar MN, Erez E, Tam VKH. Congenit Heart Dis. 2019 Mar 12. doi: 10.1111/chd.12765. [Epub ahead of print] PMID: 30861318 Similar articles Select item 30915321     Take-Home Points:   This is study is a midterm analysis of Freestyle porcine aortic root conduit for RVOT reconstruction in pediatric and adolescent patients. Overall, the Freestyle conduit demonstrated excellent durability up to 10-years, especially in patients >10 years. Risk factors for earlier reintervention included age <10 years, weight <39 kg, smaller body surface area, and valve size 25 mm or less. Orthotopic versus heterotopic position was not a significant factor. Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  This report from Cook Children’s Healthcare System in Ft. Worth, Texas, analyzes outcomes of the Freestyle porcine aortic root (Medtronic, Inc., Minneapolis, MN)  for right ventricular outflow tract (RVOT) reconstruction. The authors retrospectively evaluated 163 patients who underwent the procedure between 2002 – 2015 at their institution. Primary outcomes included survival and reintervention rates (surgery, transcatheter valve implantation, balloon valvuloplasty, or bare metal stent placement). The most common primary diagnoses included tetralogy of Fallot (57.1%), pulmonary atresia with ventricular septal defect (14.7%), and truncus arteriosus (10.4%). The median age was 12.2 years (IQR 6.4-16.4) and 40% of patients were <10 years old. The median weight was 39.0 kg (IQR 19.9-59.3). The median follow-up of 5.4 years (IQR 2.9-8), and there were 3 non-cardiac deaths during follow-up.   Thirty-eight (23%) patients required reintervention, and 84% of these were for conduit stenosis. Freedom from reintervention was 93.2% at 5 years and 48.4% at 10 years for the entire group, though these trends were likely heavily weighted by age group. For patients <10 years, the 10-year freedom from reintervention was only 18.0% compared to 82.6% for patients >10 years at time of conduit placement. Risk factors for earlier reintervention included age <10 years, weight <39 kg, smaller body surface area, and valve size 25 mm or less. Orthotopic versus heterotopic position was not a significant factor. No incidences of endocarditis were reported.   As with most reports regarding RVOT reconstruction options, this study faces limitations of being limited to only one institution and without including comparisons with other conduit options.   However, this report is one of the largest experiences with the Freestyle conduit to-date and offers meaningful mid-term follow-up of this conduit. The results of the present study suggest the Freestyle conduit is a durable option for RVOT reconstruction, especially in older pediatric patients.   The Freestyle conduit offers several advantages for RVOT reconstruction including stentless construction, ease of handling, and “off the shelf” availability of a wide range of sizes. In addition, its rigid structure may provide a suitable landing zone for future transcatheter pulmonary valve replacement if adequately sized. Oversizing the conduit is also helpful for accommodating any late anastomotic narrowing. While the technique of implantation (orthotopic versus heterotopic) did not significantly influence rate of reintervention, maintaining laminar flow through the RVOT may be important for long-term durability of any RVOT conduit. Determining which conduits fare better in different age groups remains a key question in congenital cardiac surgery, however.


Adult Congenital Heart Disease: Current Early Expectations after Cardiac Transplantation.

Riggs KW, Zafar F, Radzi Y, Yu PJ, Bryant R 3rd, Morales DL. Ann Thorac Surg. 2019 Aug 10. pii: S0003-4975(19)31167-1. doi: 10.1016/j.athoracsur.2019.06.067. [Epub ahead of print] PMID: 31408642 Similar articles Select item 31493728     Take-Home Points: This large study of the UNOS database compared early outcomes after cardiac transplantation for patients with and without adult congenital heart disease. Post-transplant outcomes for ACHD patients have improved over the past decade despite ACHD patients having relatively more risk factors than non-ACHD patients. ACHD patients with few risk factors had comparable survival up to 5 years as non-ACHD patients. Further long-term follow-up may determine whether post-transplant survival in ACHD patients surpasses that of nACHD patients given the relatively younger age of ACHD at the time of transplantation. Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  The authors sampled the United Network of Organ Sharing (UNOS) database for patients >17 years old with or without adult congenital heart disease (ACHD) who underwent cardiac transplantation between 2000-2018 and primarily evaluated 1-year post-transplant survival.  A possible era effect was analyzed by grouping patients from 2000-2008 (early) and 2009-2018 (late) then comparing the late era with the non-adult congenital heart disease (nACHD) group.   Over 35,000 nACHD patients and over 1,100 ACHD patients were included in the analyses. It is unclear whether ACHD patients who underwent heart-liver transplants were included, but this is typically a very small proportion of heart transplant patients. Late era patients were slightly older (32 versus 37 years), slightly heavier (67.1 versus 71.3 kg), more often sensitized (23% versus 38%), and had longer waitlist times (95 versus 149 days). However, the late era group exhibited better 1-year and overall survival. Compared to nACHD patients, ACHD patients were younger (37 versus 57 years), more likely to be female (38% versus 26%), had fewer ventricular assist devices (14% versus 46%), greater incidence of sensitization 38% versus 29%), and longer waitlist times (149 versus 104 days). Multivariable analysis revealed risk factors for 1-year mortality in ACHD patients included decreased renal function, body mass index >25 kg/m2, total bilirubin >1.2 mg/dL, and graft ischemic time.   When comparing ACHD patients with nACHD patients, ACHD patients who had less than two risk factors had similar 1-year survival as nACHD patients. Moreover, ACHD patients with preserved renal and liver function had similar overall survival as all nACHD patients and possibly trending superior survival beyond 5 years post-transplant (Figure).   These findings demonstrate improved ACHD transplant outcomes over the past decade, which is even more remarkable especially considering the greater association of risk factors during that interval. Unfortunately, granular details such as inotrope use and aspects of other medical management are lacking in this large study. However, it is also interesting that ACHD patients (who are relatively younger) may have better long-term outcomes compared to the older nACHD patients. Hopefully, this finding may help instruct future revisions of UNOS listing criteria for ACHD patients. Finally, the authors postulate that wider utilitization of VADs in ACHD patients may help to mitigate preoperative risk factors and improve post-transplant outcomes.    


Long-Term Outcomes of Coarctation Repair Through Left Thoracotomy.

Gropler MRF, Marino BS, Carr MR, Russell WW, Gu H, Eltayeb OM, Mongé MC, Backer CL. Ann Thorac Surg. 2019 Jan;107(1):157-164. doi: 10.1016/j.athoracsur.2018.07.027. Epub 2018 Sep 8.   Take Home Points:   Discrete coarctation of the aorta can often be repaired via left thoracotomy and extended end-to-end repair. This retrospective, single-center study evaluated their experience with extended end-to-end repair and showed long-term results with low mortality, low reintervention rates and low incidence of late hypertension. Commentary from Dr. Timothy Pirolli (Dallas), section editor of Congenital Heart Surgery Journal Watch:  Coarctation of the aorta is a relatively common congenital anomaly that, in isolation, can often be repaired with extended end-to-end repair via left thoracotomy and without the need for cardiopulmonary bypass. However, residual coarctation can lead to need for re-intervention and possibly hypertension. The extended end-to-end repair has become the standard surgical preference at the majority of centers, but questions about long-term results remain. Also, there remains controversy over the size cutoff points for when a coarctation can be best managed with an extended end-to-end repair vs. a more invasive arch repair on cardiopulmonary bypass via sternotomy.  The authors of this study sought to evaluate the long-term results of their coarctation repair experience.   The study reviewed patients with isolated coarctation or coarctation with a VSD who underwent surgery between 2000 and 2015. Patients with more complex congenital cardiac malformations were excluded from the study. A total of 251 patients were identified. Of those 186 patients were identified with follow-up (mean 6.1 ± 4.2 years). Table 1 details the preoperative characteristics of the cohort. The primary goals were to evaluate mortality, re-intervention rates and incidence of hypertension. The authors also sought to evaluate the cut-off Z scores for the proximal and descending arch for repair via left thoracotomy.   The surgical technique of repair via left thoracotomy was standard for the institution and was performed in 91% of the 186 patients.  The remaining 9% underwent arch repair via sternotomy. Intraoperative details are listed in Table 2. Interestingly 11% of the coarctation repairs via left thoracotomy were performed with partial cardiopulmonary bypass in this series. The authors describe the “older patients deemed to have collateral circulation (femoral arterial pressure lower than 45 mmHg during test-clamping of the transverse aortic arch)” as needing partial CPB, but it would’ve been helpful to know the ages and weights of these patients to help understand which patients fit in this category.   There were 3 deaths in the follow-up period. Two were non-cardiac in origin and one was a fatal myocardial infarction in a syndromic patient 6 months after repair. The Kaplan-Meier curve for re-intervention is found in Figure 1.  A total of 18 patients had a gradient between upper and lower extremities of > 20 mmHg, but only 4 of them required late re-intervention for recurrent coarctation. All four had transverse aortic arch hypoplasia (Z score < 2) at the time of initial repair. The follow-up for hypertension was the weakest point in the conclusions as it was based on only 18% of the patients in the cohort. So, seemingly, no meaningful conclusions can be made about presence of hypertension long-term.   The authors also evaluated the predictors of surgical approach (sternotomy vs. thoracotomy). Not surprisingly, the presence of a VSD favored sternotomy. Transverse aortic arch hypoplasia did not necessitate repair by sternotomy, however mean proximal arch measurements were smaller in the sternotomy group than in the thoracotomy group (0.35 ± 0.6 cm vs. 0.64 ± 0.3 cm). When the z-scores of proximal transverse arch were examined, the results showed a z-score of -4.98 ± 0.82 for sternotomy group and -2.69 ± 1.84 for the thoracotomy group. The authors performed a logistic regression model for continuous variables to determine cut-points for deciding upon sternotomy vs. thoracotomy. This is illustrated in table 5 and the proximal transverse arch z-score cut-point was -4.1 and for the distal transverse arch it was -2.8. This may be the single-most important findings from this study and compares similarly for cut-off points from other studies. This could help guide surgeons on how small is “too small” for a coarctation repair via thoracotomy.   The limitations of the study were discussed by the authors and included the short “long-term” follow-up period, the incompleteness of follow-up in the cohort, variability of echo findings and the nonrandomized, retrospective nature of the study at a single center. As extended end-to-end repair has become the standard at most institutions, a prospective study of repair via thoracotomy vs. sternotomy is likely impossible. This report adds further evidence that extended end-to-end repair of coarctation is an excellent technique with excellent long term outcomes. The finding of such a low Z-score as a cut-off for the proximal transverse arch should reassure surgeons that performing a repair via thoracotomy is not only feasible, but also has good long term outcomes.


Repair of total anomalous pulmonary venous connection: risk factors for postoperative obstruction.

White BR, Ho DY, Faerber JA, Katcoff H, Glatz AC, Mascio CE, Stephens P Jr, Cohen MS. Ann Thorac Surg. 2019 Mar 15. pii: S0003-4975(19)30354-6. doi: 10.1016/j.athoracsur.2019.02.017. [Epub ahead of print] PMID: 30885849 Similar articles Select item 30661465   Take Home Points:   Patients born with total anomalous pulmonary venous return have a high risk of recurrent obstruction post-repair though there is still a question of which patients are at highest risk. This retrospective, single-center study evaluated their experience with TAPVR repair and sought to identify risk factors for postoperative pulmonary venous obstruction as well as to try to standardize (and risk stratify) the preoperative echo findings for postoperative obstruction. Commentary from Dr. Timothy Pirolli (Dallas), section editor of Congenital Heart Surgery Journal Watch:  For patients born with total anomalous pulmonary venous return (TAPVR), surgical repair involving creating an unobstructed connection between the venous confluence and the left atrium is the only viable option. Despite an optimal repair, some patients still develop postoperative venous obstruction, which may be untreatable and lead to death. There is no definitive consensus on how to define either pre- or post-operative pulmonary venous obstruction and the risk factors for post-operative obstruction are unclear. The authors of this study sought to better delineate the preoperative and operative risk factors for postoperative obstruction by reviewing a large cohort of TAPVR repairs at their center (Children’s Hospital of Philadelphia). The authors also sought to examine what degree of preoperative obstruction might be predictive of outcome and to create an echo grading system for obstruction.   They looked at all babies undergoing TAPVR repair between January 2006 and October 2017 and found 119 patients. This cohort included patients who had single ventricles and also patients undergoing concurrent cardiac surgical repairs. Notable findings included a whopping 39.5% of patients had single ventricle and 53.8% of all patients had some degree of obstruction. The authors developed definitions for obstruction grade.  “Trivial” obstruction was defined as a mention of obstruction in the echo report but with a Doppler velocity of  < 1.2 m/s. Mild obstruction was defined as a Doppler velocity of ≥ 1.2 m/s but < 2 m/s. Moderate obstruction was defined as Doppler velocity of ≥ 2 m/s, a cath gradient of ≥ 4 mmHg or clinically-noted respiratory distress requiring urgent surgery. Severe obstruction was defined as need for immediate postnatal surgical intervention or stenosis of the vertical vein requiring stenting in cath lab. See Table 1 below for the baseline characteristics of all patients.   The primary endpoint was postoperative obstruction analyzed in a survival framework. Postoperative obstruction was defined as a Doppler velocity of ≥ 1.2 m/s in either the confluence or an individual pulmonary vein or a cath gradient of ≥ 4 mmHg from a pulmonary vein to the atrium. The patients were followed and a flowchart of their results is found in Figure 1. Overall only 21% patients met the criteria for postoperative obstruction, with most of these occurring within the first 6 months after the repair (Figure 2). Interestingly, no postoperative obstruction was identified later than 2.1 years after surgery. The authors also examined if the type of TAPVR affected likelihood of survival without postoperative obstruction and showed the “mixed” type was the highest risk (Figure 3). They also found that heterotaxy syndrome and single ventricles were at higher risk for postoperative obstruction (Figure 4). Finally, in Figure 5, using their preoperative definitions of grades of obstruction, they found using cut-offs of trivial or mild obstruction increased the risk of postoperative obstruction significantly. Other factors such as gestational age, genetic syndrome, type of surgical repair (direct vs. “sutureless”), and whether or not the vertical vein was ligated did not show any statistically significant differences in postoperative obstruction.   This is a thorough review of the recent TAPVR experience at CHOP and contributes very useful information into how to counsel families and manage this challenging anomaly. The development of a preoperative (and postoperative) grading system for pulmonary venous obstruction is certainly helpful and seems to correlate with their outcomes nicely. The findings of association of single ventricle, heterotaxy and mixed-type TAPVR are not newsworthy, but the lack of associations of early age at surgery, genetic syndrome, and surgical technique (among other factors), is helpful. The study suffers from the same limitations as the majority of single-center, retrospective reports; however this was more than just your standard “show-and-tell” retrospective study.  The relatively common (and often lethal) morbidity of postoperative pulmonary vein stenosis has persisted despite advances in diagnosis, surgical technique and post-operative management. This report should help cardiologists and surgeons standardize the risk stratification for patients cursed with this high-risk anatomical arrangement.


Extracorporeal membrane oxygenation use in the first 24 hours following pediatric heart transplantation: Incidence, risk factors, and outcomes.

Godown J, Bearl DW, Thurm C, Hall M, Feingold B, Soslow JH, Mettler BA, Smith AH, Profita EL, Singh TP, Dodd DA. Pediatr Transplant. 2019 Apr 11:e13414. doi: 10.1111/petr.13414. [Epub ahead of print] PMID: 30973190 Similar articles Select item 30973305   Take Home Points:   Primary graft dysfunction (requiring ECMO) after pediatric heart transplant is not uncommon and can lead to significant morbidity or mortality. This retrospective study was designed using large administrative databases to find the incidence of, risk factors for, and outcomes of primary graft dysfunction and ECMO utilization after heart transplant. The results indicate that primary dysfunction is not rare (7.9% of transplants) and that outcomes are strongly associated with the duration of ECMO.  Commentary from Dr. Timothy Pirolli (Dallas), section editor of Congenital Heart Surgery Journal Watch:  Primary graft dysfunction after heart transplant is often described as a “black box” of unknown etiology that requires extracorporeal membrane oxygenation (ECMO) support to allow the heart to recover, which may not occur. It constitutes 40% of mortality after transplant and often a single factor is not identified as the precipitating cause. The authors of this study sought to examine the incidence of, factors for and outcomes from the use of ECMO within 24 hours after heart transplant in children. They sought to examine two large administrative databases to utilize a unique data linkage to examine their questions.   The SPTR database includes information from every organ transplant since 1987. The PHIS database is an administrative/billing database that captures clinical and resource utilization data, including ICD-9 and -10 codes, from over 50 children’s hospitals. The researchers queried all heart transplants in children under 18 years old between 2002 and 2016 from the linked database and found 2820 patients. Primary graft dysfunction was then examined by using the PHIS to obtain billing codes for ECMO initiation within 24 hours of heart transplant. This was also the method to delineate the duration of ECMO therapy for each of these patients. Baseline demographics and data regarding the heart transplant were obtained. Analyses of this data using multivariate logistic regression models were used to assess risk factors for primary graft dysfunction. The authors also looked at each major transplant group (cardiomyopathy vs. congenital heart disease) to evaluate if there are separate risk factors for each. Outcomes evaluated included ECMO decannulation, re-transplantation and death.   The first key finding from this report is that 7.9% (n=224) of heart transplant recipients required ECMO within the first 24 hours after transplant. The median time on ECMO was 2 days with a maximum duration of 50 days. An evaluation of the multivariable logistic regression of patient demographics and primary graft dysfunction is summarized in table 2. It is not surprising that the authors found that younger patients and patients already on ECMO at time of transplant were independently associated with primary graft dysfunction. The risk factors for ECMO are also evaluated when the transplant recipients are separated into cardiomyopathy vs. congenital heart disease subgroups (Table 3.) Interestingly, only 14.3% (n=32) of the patients placed on ECMO died while on ECMO as seen in Figure 1. Of the 191 patients who were decannulated, 167 (87.4%) survived until discharge. The study also highlights that the survival to discharge decreases as the duration of the ECMO course increases, from 89% survival for 1-3 day ECMO runs to 18.8% survival for more than 10 days. This data is also presented graphically in Figure 2. The risks factors for in-hospital mortality for patients requiring post-transplant ECMO are presented in Table 5. Unsurprisingly, long ischemic times, length of ECMO and post-transplant dialysis were associated with in-hospital mortality. The need for post-transplant ECMO was associated with inferior in-house patient survival compared to those who did not need ECMO. However, for those who survived to discharge after ECMO, there was no long-term difference in survival when compared to those who did not require ECMO (Figures 3 and 4).   This unique method of assessing ECMO utilization in the first 24 hours after transplant by using large administrative databases gives a more thorough understanding of which patients are at risk for the need for post-transplant ECMO and which patients who do need ECMO will survive to discharge. The cynic in me points to the fact that hospitals are not going to forget to bill for ECMO and thus this data should be more complete than other voluntary collaborative databases. Still this data comes from just 28 hospitals and there is obviously much variability among transplant programs’ practices. The findings here will allow transplant programs to help guide their patients’ families into the short- and long-term outcomes from a post-transplant ECMO run. The results seem to be more favorable than expected. It is clear that this study does not allow for a better understanding of what the indication for ECMO was for these patients. And there is obviously the risks of errors and missing information in this data, but the summary of findings should give both practitioners and families hope that the need for post-transplant ECMO support is not a death sentence. Tables and Figures:


Serial Follow-Up of Two Surgical Strategies for the Repair of Tetralogy of Fallot.

Simon BV, Subramanian S, Swartz MF, Wang H, Atallah-Yunes N, Alfieris GM. Semin Thorac Cardiovasc Surg. 2019 Jan 5. pii: S1043-0679(18)30418-0. doi: 10.1053/j.semtcvs.2019.01.006. [Epub ahead of print] PMID: 30616004 Similar articles Select item 30704940   Take-Home Points:   A limited right ventriculotomy approach during tetralogy of Fallot repair was associated with favorable 10-year event rate, greater exercise capacity at 20 years, and less RV dilation at 30 years compared to a more extensive approach. Overall long-term survival was excellent in both groups, and there was no difference in arrhythmias over time between groups. Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  The fate of the right ventricle after tetralogy of Fallot (TOF) repair remains a key question for the management of these patients. Evidence supports utilizing a valve-preserving repair whenever possible, but frequently a hypoplastic pulmonary valve and right ventricular (RV) outflow tract necessitate a transannular repair with patch enlargement.  The group from University of Rochester Medical Center provides further information about the long-term consequences relating to the degree of the transannular incision during TOF repair.   The authors compared outcomes of children who underwent TOF repair with either an extended or limited right ventriculotomy between January 1976 and December 1985. The extended approach was primarily performed between 1976-1981 while the limited approach was primarily utilized between 1982-1985. The extended right ventriculotomy approach included incising the RV infundibulum to the RV mid-body followed by muscle bundle resection and VSD closure. In general, a transannular patch was used if the predicted postoperative RV pressure was greater than 3/4 of the systemic pressure based on catheter or intraoperative assessment. For the limited approach, the VSD was closed transatrially and a 2 cm or less incision made in the RV at the point of maximal obstruction as seen on preoperative catheterization. This opening was then closed with a patch. They excluded patients who underwent pulmonary valve replacement at the initial repair and those who were lost to follow-up after less than 5 years. Primary outcomes included reoperation, arrhythmia, or death. Secondary outcomes included arrhythmias, exercise capacity, and echocardiographic parameters   There were 21 and 17 patients in the extended and limited right ventriculotomy groups, respectively. The median follow-up for the entire group was 30.9 years. Patients in the limited ventriculotomy group were younger (2.7 versus 3.8 years, respectively) and were less likely to undergo transannular patch placement (58.8% versus 85.0%, respectively). The overall survival at 30 years was excellent at 93.6% and was similar between groups. Freedom from reoperation at 10 years was higher for the limited ventriculotomy group (94.1% versus 57.1%). There were no differences in QRS duration or incidence of arrhythmias between groups over time. In the Cox proportional hazards model for 10-year cumulative events, only the limited ventriculotomy approach was associated with a lower event rate.   The authors conclude that the limited ventriculotomy technique was associated with a lower event rate at 10 years, greater, exercise capacity in the second decade (but not the third), and smaller RV end-diastolic diameter Z score at 30 years. The study limitations include its single center nature and the small patient groups. However, this is a truly long-term follow-up period and one of the longest published. In essence, the authors evaluated a generation of patients, many of whom have proceeded with at least one pulmonary valve replacement. It is possible that the surgical approach and patient selection changed over time, and it is unclear Questions as to what is an acceptable residual RVOT gradient at the time of repair. Most TOF patients do not need routine preoperative catheterization, and using the authors’ method of comparing PA pressure to systemic pressure may not be practical in all cases. However, it is reasonable to conclude that limiting the transannular incision may be beneficial long-term.


Impact of Regional Cerebral Perfusion on Outcomes Among Neonates Undergoing Norwood Operation.

Migally K, Rettiganti M, Gossett JM, Reemtsen B, Gupta P. World J Pediatr Congenit Heart Surg. 2019 May;10(3):261-267. doi: 10.1177/2150135118825274. PMID: 31084315 Similar articles Select item 31084314   Take Home Points: The use of regional cerebral perfusion (RCP) during the Norwood surgery is a technique utilized by many (but not all) surgeons to improve cerebral protection to the neonatal brain during aortic arch reconstruction. This study utilized the public use data from the Pediatric Heart Network Single Ventricle Reconstruction trial to evaluate outcomes whether RCP affected outcomes such as mortality, need for heart transplant, prolonged mechanical ventilation and prolonged hospital stay … but not neurologic outcomes. And the dataset used to come to these conclusions was not designed to evaluate RCP vs. DHCA.  Commentary from Dr. Timothy Pirolli (Dallas), section editor of Congenital Heart Surgery Journal Watch:  The traditional method of protecting the brain during aortic arch surgery has been deep hypothermic circulatory arrest (DHCA). However, in recent years, an increasing number of surgeons have been using regional cerebral perfusion (or selective antegrade cerebral perfusion) during arch surgeries to maintain some perfusion to the brain for a hypothetical improvement in outcomes, especially neurologic outcomes. The Single Ventricle Trial was conducted between 2005 and 2009 at 15 North American centers and was designed as a randomized controlled trial to evaluate modified BT shunts and Sano RV-PA conduits during the Norwood procedure for hypoplastic left heart syndrome.  The dataset from this study was made available for public use in 2013.   This present study was designed by researchers at the University of Arkansas to use this public dataset to evaluate whether patients who received RCP (as opposed to DHCA) at the time of their Norwood had different outcomes with respect to mortality, need for heart transplant, prolonged mechanical ventilation and prolonged hospital stay. These endpoints were evaluated between the two study groups (RCP vs. DHCA) from the initial study (Sano vs. BT shunt). However, the study was not designed to evaluate the difference between DHCA and RCP. This is clear because the evaluation of neurologic outcomes is not one of the primary endpoints of this present study (even though it is the main reason why surgeons may employ RCP vs. DHCA). The researchers also examined a host of other factors to determine the need for heart transplant or mortality, but only a few were statistically significant and those that were significant were not surprising. (Table 1).   Of the 549 patients qualified for inclusion in this study, 252 (45.9%) received RCP during their Norwood procedure. The information in Table 2 shows the variables that were related to the use of RCP that were statistically significant. Essentially, RCP was associated with longer CPB times, increased use of ultrafiltration and open chest after Norwood. It also showed that surgeons performing ≤ 10 Norwoods/year were more likely to use RCP.  Table 3 shows that these longer surgeries and the use of RCP had no effect on the study outcomes, which is the key point of this study. Figure 2 depicts the increasing effect no mortality of increasing RCP and DHCA times.   What do we learn from this study?  Well, when it comes to evaluating the principal difference between why a surgeon uses RCP and DHCA, which is neurologic outcomes, we learn nothing.  The “secondary” outcomes that are evaluated here show there are no differences between the use of RCP and DHCA from this dataset. The authors also emphasized the finding that surgeons who do fewer Norwoods are more likely to perform RCP during their surgeries. As a surgeon, this makes perfect sense since the cases (should) take you longer if you do fewer of them. Also surgeons that are performing the bulk of the Norwood procedure at any center in this study are likely to be at a more senior level in their career, faster, and (in general) used to performing the surgery under DHCA.  So this finding is little more than an unsurprising footnote. The inherent bias of some (especially experienced) surgeons to use DHCA vs. RCP really undermines the entire point of this study. There is no good way to parse out this factor from this dataset.   Is there really a difference in the selected outcomes in this study?  No, not from what this dataset says. But the study was not randomized (or even powered) to look at RCP vs. DHCA, so it is unclear how valid the researchers’ findings are.  And, again, there is zero mention of neurologic outcomes (not even stroke) which also is a major flaw. To break the results down to their core, we learn that during that 4 year period of the SVR trial there was no major difference between the chosen outcomes between Norwoods with RCP vs DHCA at these 15 institutions. And this fact may, or may not, be applicable to present-day practices. As usual, a better trial is needed to answer this question. Tables and Figures:  


Outcomes of Bioprosthetic Valves in the Pulmonary Position in Adults with Congenital Heart Disease.

Egbe AC, Connolly HM, Miranda WR, Dearani JA, Schaff HV. Ann Thorac Surg. 2019 Jul 16. pii: S0003-4975(19)31033-1. doi: 10.1016/j.athoracsur.2019.05.068. [Epub ahead of print] PMID: 31323213 Similar articles Select item 31313513     Take-Home Points: This large single-institution study with longer term follow-up evaluated the durability of bioprosthetic pulmonary valve replacement in adult patients as well as risk factors for prosthetic valve dysfunction. Factors associated with prosthetic valve dysfunction included prior history of atrial fibrillation and greater than moderate right ventricular dysfunction. The use of vitamin K antagonists at the time of hospital discharge was associated with lower risk of prosthetic valve dysfunction.  Commentary from Dr. Jeremy Herrmann (Indianapolis), section editor of Congenital Heart Surgery Journal Watch:  This retrospective review adult patients who underwent surgical bioprosthetic pulmonary valve repair over an 18-year period at the Mayo Clinic evaluated at prosthetic valve dysfunction not simply as occurrences of reintervention but other hemodynamic parameters to more accurately gauge prosthetic pulmonary valve longevity. The authors defined prosthetic valve dysfunction as a peak valve velocity of >4 m/sec and/or severe pulmonary regurgitation. In all, 573 patients met inclusion criteria with a mean age 32 years at the time of valve replacement. Pericardial prostheses were most commonly used, and all valve types and parameters are listed in Table 2. Patients were followed for a mean 16.8 +/- 4.2 years.   Subsequent reintervention occurred in 201 patients (35% total; 192 surgical and 9 transcatheter valve replacement). Of these, 48 (9%) required another intervention. In all, 807 bioprosthetic pulmonary valves were implanted in 573 patients. The overall freedom from reintervention at 10 and 15 years was 83% and 61%, respectively. When looking at hemodynamic parameters, the average time-to-prosthetic valve dysfunction was 12.6 years with an incidence at 10 and 15 years of 27% and 48%, respectively. In the multivariable risk factor model, factors associated with prosthetic valve dysfunction included history of atrial fibrillation and greater than moderate right ventricular dysfunction. The use of vitamin K antagonists at the time of hospital discharge was associated with lower risk of prosthetic valve dysfunction.   This study with excellent long-term follow-up offers significant insight into the current phase of care of adult congenital heart disease patients who require ongoing right ventricular outflow tract reintervention. These data show that prosthetic valve dysfunction starts to become a significant factor 10-15 years after valve replacement. Patients with more significant right heart dysfunction may be at risk for earlier development of prosthetic valve dysfunction, though the reasons for this are not clarified in this study. Interestingly, the use of vitamin K antagonists may be helpful in improving valve durability like bioprosthetic valves inserted in left heart positions. It is unclear for how long this treatment occurred and whether patients were also treated with aspirin. Importantly, no cases of endocarditis are reported in this study.   This large series spans almost 20 years, and it is possible changes in surgical techniques and prosthetic materials may have affected prosthetic valve durability over the course of the study. Additional information about concomitant cardiac procedures at the time of pulmonary valve replacement (e.g., MAZE procedure, pulmonary arterioplasty, etc.) may be helpful for better understanding this patient group and other risks for later valve dysfunction. One significant omission may be the use of stentless porcine aortic root grafts, which is the preferred prosthesis for pulmonary valve replacement in adults at our institution. This highlights one of the major limitations of a single institution study as well as the need for more collaborative research efforts.        


Surgery for anomalous aortic origin of coronary arteries: a multicentre study from the European Congenital Heart Surgeons Association†.

Padalino MA, Franchetti N, Hazekamp M, Sojak V, Carrel T, Frigiola A, Lo Rito M, Horer J, Roussin R, Cleuziou J, Meyns B, Fragata J, Telles H, Polimenakos AC, Francois K, Veshti A, Salminen J, Rocafort AG, Nosal M, Vedovelli L, Guariento A, Vida VL, Sarris GE, Boccuzzo G, Stellin G. Eur J Cardiothorac Surg. 2019 Mar 20. pii: ezz080. doi: 10.1093/ejcts/ezz080. [Epub ahead of print] PMID: 30897195 Similar articles Select item 30893567   Take-Home Points:   AAOCA can be clinically very significant, resulting in sudden cardiac death and/or acute profound ventricular dysfunction. The management of clinically significant AAOCA involves surgical correction by a number of techniques that are generally very safe, with little morbidity and mortality. In the largest surgical AAOCA series published to date, operative and long-term mortality was 1.3% and 2.2% respectively. Event-free survival was estimated to 74.6% at 20-25 year follow-up. Most patients remained in excellent functional status, and a small subset continued to have poorly-defined chest pain after surgical repair.  Commentary from Dr. Michael Ma (Stanford, CA), section editor of Congenital Heart Surgery Journal Watch:  This multi-center retrospective review comprises the largest surgical AAOCA series to date, and demonstrates immediate and long-term results after surgical management for this condition. While it does not specifically try to answer controversies around appropriate patient selection, it does conclude that surgical therapy is very safe with little long-term morbidity or mortality.   156 patients with isolated AAOCA from 1991-2016 were studied. Generally, indications for intervention included all AAOLCA (anomalous left), symptomatic AAORCA (anomalous right), and asymptomatic AAORCA with preoperative testing suggestive of inducible ischemia. 85.9% of the cohort was considered symptomatic. Coronary unroofing (56.4%), coronary reimplantation (19.2%), and coronary artery bypass grafting (CABG) (15.4%) were most commonly employed. Two (1.3%) patients died in the immediate post-operative period; both patients presented with low cardiac output prior to surgical intervention and ultimately succumbed to complications of ventricular dysfunction. 14 (9%) patients suffered post-operative morbidity, including low cardiac output syndrome in nine with six of those requiring mechanical circulatory support, early re-intervention in seven, and mild-moderate aortic valve regurgitation (after unroofing) in two (1.3%).   At late follow-up (median two years), three (2.2%) deaths occurred in septuagenarians. Long-term morbidity included three (2.3%) operative re-interventions (aortic valve replacement, followed by pacemaker in the same patient, ascending aortic replacement with myocardial bridge unroofing in a separate patient), five (3.7%) non-operative re-interventions (three coronary stents, one electrophysiologic ablation, one internal cardioverter-defibrillator). 91.2% of late survivors were in NYHA Class I or II. 14.2% of patients continued to have symptoms, primarily undefined chest pain, with only  one correlated to an ongoing positive cardiac stress test. These findings corroborate existing, primarily single-center studies that examine the overall safety of surgery for AAOCA in a population of patients that meet consensus criteria for intervention. Despite generally favorable results, immediate and long-term complications and death did occur, and were not statistically linked to one particular subset of patients or procedure choice. Importantly, these findings add yet an additional data point for consideration in the more controversial management question of what, if any, therapies to offer the asymptomatic patient who is diagnosed with AAOCA through incidental imaging.  


Modified Lung Ultrasound Examinations in Assessment and Monitoring of Positive End-Expiratory Pressure-Induced Lung Reaeration in Young Children With Congenital Heart Disease Under General Anesthesia.

Wu L, Hou Q, Bai J, Zhang J, Sun L, Tan R, Zhang M, Zheng J. Pediatr Crit Care Med. 2019 May;20(5):442-449. doi: 10.1097/PCC.0000000000001865. PMID:  31058784 Similar articles   Take Home Points: The most prevalent region of post-intubation atelectasis in...

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