Congenital Heart Surgery

Outcomes of single ventricle palliation in infants with heterotaxy syndrome.

Outcomes of single ventricle palliation in infants with heterotaxy syndrome. Vodiskar J, Kido T, Strbad M, Cleuziou J, Hager A, Ewert P, Hörer J, Ono M. Eur J Cardiothorac Surg. 2021 Mar 30:ezab141. doi: 10.1093/ejcts/ezab141.   Take Home Messages: Single ventricle physiology with heterotaxy pertains a high mortality risk. Restricted pulmonary blood flow and atrioventricular valve regurgitation are The two most important risk factors for mortality. Providing unrestricted pulmonary blood flow and repairing total anomalous pulmonary venous connection offers survival advantages.   Commentary by Dr. Frederic Jacques (Québec city, Canada), chief section editor of Congenital Heart Surgery Journal Watch:  Heterotaxy syndrome is a recognized risk factor for overall poor outcome with single ventricle patients. This syndrome that mainly defines abnormal abdominal visceral configuration is associated with atrial appendages isomerism. Vodiskar et al. (Munich, Germany), reported their experience with 53 patients operated from 2001 to 2019. Their cohort comprises 66% right dominant ventricle, 49% total anomalous pulmonary venous connection, and 80% pulmonary stenosis/atresia. Thirteen patients were born with moderate to severe atrioventricular regurgitation. Consequently, these patients were treated with a non-heterogenous surgical protocol. In fact, 87% required some sort of neonatal palliation of which roughly one quarter died. A group of 41 patients underwent a bilateral cavopulmonary connection (17% as an initial surgery). About one tenth died. Of the 58% that reached the total cavopulmonary connection, none died. Unplanned reintervention occurred in 60% after Stage-1, mainly to better adjust pulmonary inflow, but also to address atrioventricular valve regurgitation. Through multivariate analyses, the authors identified restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02–10.2; P = 0.05) and moderate to severe atrioventricular regurgitation (HR 3.57; 95% CI 1.27–10.0; P = 0.02) as independent risk factors for mortality. Providing a sustainable pulmonary inflow and correcting the pulmonary venous connection were two ways identified to come up with better survival.   Comment:   This paper reports on a large cohort of patients with heterotaxy in addition to single ventricle physiology. Not surprisingly, this is a motley group of patients comprising various combinations of systemic and pulmonary venous connections, various types and degrees of outflow obstruction, as well as various atrioventricular valve configurations and degree of regurgitation. Nevertheless, the authors did an honest effort in trying to pinpoint risk factors for mortality and potential factors to tackle down in order to devise better outcomes. The findings echo intuitions, as with one functioning ventricle, one could expect that any inflow or outflow anomaly would lead to worse outcomes, and that correcting those would potentially reverse this burden, at least partially. The paper, just like similar previous papers from other authors, also highlight how limited this kind of single centre analysis is for such a rare disease. Even large cohorts from large institutions have somewhat been limited in their capacity to derive meaningful messages that go further what can be expected form the insight of common sense. In order to change in a more radical fashion the outcomes with this very difficult group of patients to deal with, multicentre strategies are likely mandated. An international registry comprising all the anatomical variables faced with the spectrum within the heterotaxy syndrome is needed in order to get more granular in the analysis of these patients.

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Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot.

Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot. Goldstein BH, Petit CJ, Qureshi AM, McCracken CE, Kelleman MS, Nicholson GT, Law MA, Meadows JJ, Zampi JD, Shahanavaz S, Mascio CE, Chai PJ, Romano JC, Batlivala SP, Maskatia SA, Asztalos IB, Kamsheh AM, Healan SJ, Smith JD, Ligon RA, Pettus JA, Juma S, Raulston JEB, Hock KM, Pajk AL, Eilers LF, Khan HQ, Merritt TC, Canter M, Juergensen S, Rinderknecht FA, Bauser-Heaton H, Glatz AC.J Am Coll Cardiol. 2021 Mar 2;77(8):1093-1106. doi: 10.1016/j.jacc.2020.12.048.   Editorial:  Primary Versus Staged Neonatal Repair of Symptomatic Tetralogy of Fallot: Time to Randomize? Hornik CP.J Am Coll Cardiol. 2021 Mar 2;77(8):1107-1109. doi: 10.1016/j.jacc.2020.12.043.     Take Home Points   Neonates with symptomatic Tetralogy of Fallot may undergo early primary repair or staged repair consisting of initial palliation to augment pulmonary blood flow followed by complete repair. A retrospective, multicenter study found a similar observed risk of death at 4 years with early primary repair compared to a stager approach.   Commentary by Dr. Luis Quinonez (Boston USA), section editor of Congenital Heart Surgery Journal Watch:   Summary   Question:  What is the best surgical strategy for the newborn with symptomatic TOF, complete or staged surgical repair?   Design: Retrospective, multicenter (Congenital Cardiac Research Collaborative)   Population:  Symptomatic neonates with TOF with PS or PA undergoing early primary repair (n=230) and staged repair (n=342)   Analysis:  Risk stratified; Propensity score adjustment   Outcomes:  Death, cumulative morbidity, reinterventions   Results:   Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the staged group.   Reintervention risk was higher in the SR group.   Less neonatal morbidity in the staged group, whereas overall cumulative morbidity burden favoured the primary group.                     Commentary   This is a very important paper that should be read.   It addresses an ongoing dilemma in our specialty:  Staged vs. primary repair of symptomatic neonatal TOF with PS or PA.  The study is retrospective but multicenter.  There is a selection bias to stage the sickest patients.  The study attempts to risk adjust and uses propensity methodology.  The main finding of the paper is that overall mortality is similar between staged and primary repair, despite early mortality risk in the primary repair group.  The burden of reintervention occurs in the staged group.  Cumulative morbidity favoured the primary repair group.   A few things are worth pointing out:  The increased popularity of stent-based palliation may affect the outcomes of patients with the staged approach, in a positive or negative direction.  It is unclear whether PDA stenting is superior to a Blalock-Taussig shunt and will likely be the subject of a randomized trial in the near future.  Valve-sparing interventions were not different between the two groups, even when looking at the patients with PS alone.  The argument that sparing the pulmonary valve is more likely in an older patient may not hold.  The rate of pulmonary arterioplasty was similar in both groups, suggesting no advantaged for either treatment strategy.  Because the median follow-up is about 4 years, we cannot know if either strategy will have any long-term advantage.   I am concerned that with the current public reporting systems that emphasize early mortality (30-day or operative), there may risk aversion against primary repair and in favour of a staged approach.  This problem may be averted by public reporting of longer-term outcomes (even up to a year).   This paper provides equipoise to justify a randomized trial.  The accompanying editorial focuses on the challenges of doing surgical randomized trials, suggesting randomization methodology to account for surgeon or institution expertise.

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Outcomes of single ventricle palliation in infants with heterotaxy syndrome

Outcomes of single ventricle palliation in infants with heterotaxy syndrome Janez Vodiskar 1 2, Takashi Kido 1 2, Martina Strbad 1 2, Julie Cleuziou 1 2 3, Alfred Hager 4, Peter Ewert 4, Jürgen Hörer 1 2, Masamichi Ono 1 2 Eur J Cardiothorac Surg. 2021 Mar 30;ezab141. doi: 10.1093/ejcts/ezab141. PMID: 33783481;  DOI: 10.1093/ejcts/ezab141     Take Home Points:   Patients who need an intervention to manage the pulmonary circulation at neonatal period carry the risk for death. TAPVC is no longer a risk for mortality in this study when it is addressed at the first palliation.   Commentary by Dr. Yasuhiro Kotani (Okayama, Japan), section editor of Congenital Heart Surgery Journal Watch:   Summary:   A total of 53 patients with heterotaxy who underwent staged single ventricle palliation were included in this study. Thirty-five (66%) patients had a right ventricular dominance, common atrioventricular septal defect was present in 37 (70%) patients. Anomalous pulmonary venous drainage was present in 29 (55%) patients. Forty-six (86%) patients underwent first-stage palliation and, of these patients, there were 12 deaths (26%). A total of 41 patients, including those who did not have first palliation, received a bidirectional cavopulmonary connection (BCPC). There were 4 deaths after BCPC and 31 (58%) patients received total cavopulmonary connection (TCPC). Overall survival rate was 92% at 1 month, 74% at 1 year and 68% at 10 years. No patient died after TCPC. Independent risk factors for mortality in the multivariate Cox regression were a presence of restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02–10.2; P = 0.05) and greater than mild atrioventricular valve regurgitation (HR 3.57; 95% CI 1.27–10.0; P = 0.02). Total anomalous pulmonary venous connection was not identified as a risk factor in this study. Mortality in patients with single ventricle and heterotaxy are high and this was often seen in the first 6 months of life.   Significance:   Although previous studies showed TAPVC as a risk factor for death, this study demonstrated that TAPVC is no longer a risk when it is intervened from the first palliation which makes us think of alternating the timing of TAPVC repair. Most of deaths and reoperations occurred after first palliation but in other words, the patients do well once they overcome all adverse events during the first 6 months of life.   Comment:   We all know that patients with heterotaxy do badly compared to other form of single ventricle. The study from Munich, Germany showed the consistent findings with high mortality and morbidity. In total of 53 patients, 46 patients had a first palliation, mainly to control the pulmonary blood flow. There were 7 early 5 late deaths, i.e., 26% of mortality in patients who had a first palliation. They stated the cause of death was cardiac in all patients. Although no details were shown, one can speculate that low systemic perfusion due to the imbalanced pulmonary blood flow was a main reason as they demonstrated restrictive pulmonary blood flow which need to be addressed as a risk factor for mortality.  Seven patients had a balanced pulmonary circulation which did not require intervention at neonates and this is sub-group that does well regardless of interventions. They intervene TAPVC at the first palliation and only 2 of 14 (14%) had reintervention for PVO.   This excellent outcome of TAPVC contributed the finding that TAPVC was not identified as a risk for death. As the authors mentioned, it is quite difficult to manage the balance between pulmonary and systemic circulation at neonatal period, especially after surgical/catheter intervention. Although many factors, such as pulmonary blood flow and ventricular function/atrioventricular valve regurgitation are interactively affected to the pulmonary/systemic circulation, this study did not investigate the relationship. Therefore, the study focusing on this is necessary.

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Management of Complex Left Ventricular Outflow Tract Obstruction: A Comparison of Konno and Modified Konno Techniques.

Management of Complex Left Ventricular Outflow Tract Obstruction: A Comparison of Konno and Modified Konno Techniques. Haider M, Carlson L, Liu H, Baird C, Mayer JE, Nathan M.Pediatr Cardiol. 2021 Mar;42(3):614-627. doi: 10.1007/s00246-020-02522-9. Epub 2021 Feb 8.PMID: 33555370 Free PMC article.   Commentary by Dr. Luis Quinonez (Boston USA), section editor of Congenital Heart Surgery Journal Watch:   Summary   Purpose:  To compare outcomes of the Konno procedure to the Modified Konno procedure   Population:  122 patients, single institution, 1990 to 2014   Intervention:  Konno (n=51) and modified Konno operations (n=71)   Design:  Retrospective review   Primary outcome:  LVOT re-intervention   Secondary outcomes:  Overall re-interventions; transplant-free survival; composite of re-interventions and transplant-free survival; hospital mortality and major adverse events; hospital length of stay.   Results:   Median age, Konno vs. modified Konno: 8.2 years vs 3.9 years (p=0.03)   Median Follow-up: 8 years   Patients with multiple left sided lesions more likely to have Konno (p=0.017)   Hospital outcomes (Konno vs. modified Konno):  No statistical difference: mortality (7.8% vs. 4.2%), major adverse events, permanent pacemaker (11.8% vs. 9.9%)   Multivariate analysis:  Konno had longer LOS. Patients with multiple left sided lesions: more complications (48%), more surgical reinterventions (22.9%); longer ICU and hospital LOS   Post-Discharge outcomes, Konno vs. modified Konno: LVOT reintervention at 10 years:  11% vs. 47% (p=0.002) Multivariate analysis:  Modified Konno risk factor for LVOT intervention Overall reintervention at 10 years:  44% vs. 60% (p=0.114) Transplant free survival at 10 years:  92% vs. 85% (p=0.188) Multivariate analysis:  Konno or modified Konno are not risk factors for survival.   Comment   This paper is a large, single institution experiences comparing outcomes of Konno against modified Konno procedures with long-term follow-up.  The results are mostly intuitive, in that Konno patients were operated at an older age; they had less LVOT interventions; and patients with multiple left sided lesions fare worse.  Despite modified Konno patients having more LVOT interventions, the overall rate on interventions was similar in both groups over time.  Both groups would have reinterventions on the RVPA conduit, but patients who had Konno procedures would have more MV interventions, as they were more likely to have multiple left sided lesions.  Being retrospective, underlying surgical selection biases are reflected:  modified Konno in younger patients to try to preserve the aortic valve; and Konno in patients with multiple left sided lesions.   The study also demonstrates a significant operative mortality for the groups, as well as need for permanent pacemaker for both procedures.  Although the overall reintervention rate and transplant free survival was not statistically different between the two procedures, the absolute numbers favour the use of the Konno, which is worth noting.   Patients with LVOT obstruction continue to remain a surgical challenge so these data are worth reviewing.  The literature review provided is helpful.   Take Home Messages   In patients who undergo Modified Konno operation for LVOT obstruction, there is a significantly higher rate of LVOT reintervention compared to Konno operation. Transplant free survival is statistically similar for Konno and modified Konno operations. The use of the Konno operation is associated with patients who have multiple left sided lesions.

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