October

Hepatic magnetic resonance T1-mapping and extracellular volume fraction compared to shear-wave elastography in pediatric Fontan-associated liver disease

Safety and efficacy of anticoagulant therapy in pediatric catheter-related venous thrombosis (EINSTEIN-Jr CVC-VTE). Thom K, Lensing AWA, Nurmeev I, Bajolle F, Bonnet D, Kenet G, Massicotte MP, Karakas Z, Palumbo JS, Saracco P, Amedro P, Chain J, Chan AK, Ikeyama T, Lam JCM, Gauger C, Pap ÁF, Majumder M, Kubitza D, Smith WT, Berkowitz SD, Prins MH, Monagle P, Young G, Male C. Blood Adv. 2020 Oct 13;4(19):4632-4639. doi: 10.1182/bloodadvances.2020002637   Take Home Points: Both rivaroxaban and standard anticoagulants (heparin and vitamin K antagonists) seem to be a safe treatment for children with central venous catheter related venous thromboembolism (CVC-VTE). Persistent need of the CVC and residual venous thromboembolism in children younger than 2 years were associated with continuation of anticoagulant therapy beyond the study period. Commentary from Dr. Inga Voges (Kiel, Germany), section editor of Pediatric/Fetal Cardiology Journal Watch: The EINSTEIN-Jr study compared rivaroxaban to standard anticoagulants in 500 children of all ages for treatment of acute venous thromboembolism (VTE) of any type. Pediatric patients were randomized in a 2:1 ratio to rivaroxaban or comparator (heparin or vitamin K antagonist). In this subanalysis of the EINSTEIN-Jr study the authors analyzed the safety of anticoagulation and the clinical risk profile and characteristics of children with central venous catheter (CVC) related thromboembolism (CVC-VTE). They also evaluated if the risk factor profile affects the duration of anticoagulant treatment. Children with CVC-VTE were included if they were treated with unfractionated heparin, low-molecular weight heparin or fondaparinux. CVC-related, non–CVC-related recurrent deep VTE or other venous thrombosis as well as major or clinically relevant non-major bleeding were documented. In patients with no symptomatic recurrent VTE repeated imaging was performed and compared to baseline to assess vein recanalization. Overall, 126 patients with CVC-VTE were included (Figure 1). 90 patients received rivaroxaban and 36 were treated with standard anticoagulants. 76 patients were symptomatic, and 50 patients had asymptomatic CVC-VTE. Clinically significant venous thrombosis occurred in 2 children and 3 children on rivaroxaban were diagnosed with clinically relevant non-major bleeding (Table 2). 103 patients received a repeat imaging test. Complete vein recanalization was found in 55%, incomplete recanalization in 37%, no change in 6,8% and asymptomatic deterioration in 1% of the patients. Residual VTE on repeat imaging (limited to children younger than 2 years) and presence of a CVC at the end of the study were associated with continued anticoagulant therapy beyond the study period. Although the sample size was small, the authors were able to demonstrate the safety of rivaroxaban and standard anticoagulant medication in patients with CVC-VTE. However, the authors also mention that larger multicenter studies are needed.

READMORE

Decreasing Interstage Mortality After the Norwood Procedure: A 30-Year Experience

Decreasing Interstage Mortality After the Norwood Procedure: A 30-Year Experience. Kaplinski M, Ittenbach RF, Hunt ML, Stephan D, Natarajan SS, Ravishankar C, Giglia TM, Rychik J, Rome JJ, Mahle M, Kennedy AT, Steven JM, Fuller SM, Nicolson SC, Spray TL, Gaynor JW, Mascio CE. J Am Heart Assoc. 2020 Oct 20;9(19):e016889. doi: 10.1161/JAHA.120.016889. Epub 2020 Sep 23. PMID: 32964778 Free article.   Take Home Points: Risk of interstage mortality after the Norwood significantly decreased over the years. Performance of the superior cavo-pulmonary connection (SPVC) at an earlier age, increase in use of a right ventricle to pulmonary artery shunt, and an interstage monitoring program may be the factors that have contributed to this decrease. Further research will be necessary to determine the specific contribution of these interventions as well as the influence of other factors such as prenatal diagnosis, other changes in surgical practice including improved perfusion/bypass techniques, stem cell therapy, or checklists, towards decreasing interstage mortality. Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric/Fetal Cardiology Journal Watch: Since the 3-stage approach for HLHS was established, there has been a progressive decline in overall mortality and long-term survival has improved. However, there has been increased focus on the interstage period between the Norwood and SPVC due to a high risk of mortality during that time. There have been multiple studies looking at an interstage monitoring program (ISMP), with somewhat conflicting data on whether this reduces mortality. Additionally, there has been a shift in some centers to use an RV to PA shunt instead of a BTT shunt for the Norwood, as well as an earlier stage 2 operation, in order to attempt to decrease mortality during that interstage period. The objectives of this study were to examine an overall incidence of interstage mortality during a 30-year period, evaluate interstage mortality across 4 predetermined eras based on changes in operative or medical management, and to analyze the impact of patient characteristics and operative factors on interstage mortality. Adjusted and non-adjusted logistic regression models were used to identify the risk factors for mortality. This was a retrospective chart review of 1111 patients that were available for interstage analysis (after excluding those that died or had SCPC before discharge, had biventricular conversion, or heart transplantation) over a 30-year period from 1984 - 2017. 33% had HLHS (MA/AA) and 30% had an HLHS variant. 12% had a suspected or confirmed genetic abnormality. The study period was divided into 4 eras; era 1: 1988-1994 (initial use of SCPC), era 2: 1995-2001 (change in surgical team), era 3: 2002-2010 (introduction of RV-PA shunt), and era 4: 2011-2017 (introduction of ISMP). Overall interstage mortality was 10.8% (120/1111) and decreasing significantly only for the most recent era (4.6%) (see figure 2 and table 2). While age at SCPC decreased over the 4 eras, there was no statistically significant difference for age of death. The authors do note that since the shortest length of time between the Norwood and SCPC was during the same period as the introduction of the ISMP, it was not possible to determine their individual relative contributions. Birth weight, gestational age, length of stay, genetic abnormality, and race were identified as risk factors using single covariate adjusted (era) logistic regression. A multiple logistic regression model indicated that only gestational age and race were associated with interstage mortality after adjusting for era (see table 4). A secondary analysis was conducted to estimate the relationship between shunt type after adjusting for era, gestational age, use of ECMO, and race, with an improvement in survival in those with RV-PA shunts compared to BTT shunts. Notably, the RV-PA shunt was first used in era 3 accounting for 27.4% of shunt type, and this increased to 50% in era 4. The risk of mortality significantly decreased by era 4 but it is unclear how much of each factor influenced that change. Is one more important than another or is there an “exponential” influence when all factors are present? Additionally, and especially in the last 5 years, there have been other changes in surgical practice, including improved bypass/perfusion techniques, stem cell therapy, and checklists post-bypass in some centers. Possibly even more importantly, this study did not discuss prenatal diagnosis as a factor that may also improve interstage (and overall) mortality. Future NPC-QIC prospective data may also delineate other factors that may be critical in improving mortality.    

READMORE

Racial Disparities in Hospital Mortality Among Pediatric Cardiomyopathy and Myocarditis Patients

Racial Disparities in Hospital Mortality Among Pediatric Cardiomyopathy and Myocarditis Patients. Olsen J, Tjoeng YL, Friedland-Little J, Chan T.Pediatr Cardiol. 2020 Oct 6. doi: 10.1007/s00246-020-02454-4. Online ahead of print.PMID: 33025028   Take Home Points Racial differences in health care outcomes exist for multiple illnesses in the adult population, this appears to also be true in the pediatric population with the diagnosis of myocarditis and cardiomyopathy. African American race and Hispanic ethnicity were independent risk factors for mortality. African American race was also associated with use of ECMO, mortality while on ECMO, and cardiac arrest. However, when adjusting the model for ECMO and arrest reduced the impact of African American race. Hispanic ethnicity was still associated with mortality even after controlling for variables. Commentary from Dr. Clifford Cua (Columbus Ohio USA), section editor of Pediatric/Fetal Cardiology Journal Watch: This was a retrospective cross-sectional study using the Kids’ Inpatient Database (KID). This is a national administrative database produced every 3 years with a random sampling of pediatric patients that have been discharged from a hospital. The goal of this study was to determine if any associations existed between race/ethnicity and hospital outcomes of pediatric patients diagnosed with cardiomyopathy or myocarditis. Patients < 18 years of with an ICD-9 code of cardiomyopathy or myocarditis with no other cardiac diagnosis were identified. Data from 2003, 2006, 2009, and 2009 were used. Race/ethnicity (non-Hispanic white, non-Hispanic African American, Hispanic, and other), demographic data, hospital procedures, and hospital mortality were collected. Associations between ethnicity/race and mortality were evaluated and subsequent models incorporated cardiac arrest, ECMO, VAD, or transplant in the analysis. Total of 34,617 patients were evaluated (white = 38.6%, African American = 20.4%, Hispanic = 15.4%, and other = 7.9%). Patients were mostly > 1 year of age (88.7%), male, 59.9%), had cardiomyopathy (70.8%), and were treated at non-pediatric hospitals (73.7%). Non-white patients were more likely to have the diagnosis of cardiomyopathy, use government insurance, live in lower income neighborhoods, and undergo fewer transplants. Rate of cardiac arrest, ECMO, and VAD did not differ between ethnicities. Overall mortality was 4.6% with white patients having significantly lower rates (4.1%) than African Americans (4.7%), Hispanic (5.4%), and other (5.8%). Other significant variables associated with mortality are presented in Table 3. Initial statistical multivariate model showed increased odds for mortality for African American (OR 1.25 [1.01 – 1.53]) and Hispanic (OR 1.29 [1.03 – 1.60]) patients. When ECMO, VAD, or transplant was included in the model, the mortality associations were no longer present for African Americans, but persisted for Hispanic patients. African American race was associated with use of ECMO (OR 1.46 [1.04 – 2.05]) and cardiac arrest (OR 1.23 [1.02 – 1.48]). African American and “other” race had higher mortality when ECMO was utilized. In patients that did not undergo ECMO, Hispanic race was associated with mortality. Adult studies have shown differences in health care outcomes based on race/ethnicity. This paper also documents decreased overall survival in African American and Hispanic patients compared to white patients with the diagnosis of cardiomyopathy or myocarditis. The reasons for these differences are multifactorial. By controlling for certain variables, the authors suggest that at least hospital selection and referral patterns are not the main reasons for these differences. They suggest pre-hospital and in-hospital factors or possibly intrinsic factors may be the key drivers. Limitations of the study include its retrospective nature. The lack of granular data and incomplete or incorrect data entry in the database are also shortcomings. Despite this, the large descriptive “n” allows for starting point to further evaluate why these differences exist.  

READMORE

Early school-age cognitive performance post-pediatric heart transplantation

[et_pb_section fb_built="1" admin_label="section" _builder_version="3.22" custom_padding="||188px|||"][et_pb_row admin_label="row" _builder_version="3.25" background_size="initial" background_position="top_left" background_repeat="repeat" custom_margin="-101px|auto|-208px|auto||"][et_pb_column type="4_4" _builder_version="3.25" custom_padding="|||" custom_padding__hover="|||"][et_pb_text admin_label="Text" _builder_version="3.27.4" background_size="initial" background_position="top_left" background_repeat="repeat" min_height="1797px"]Early school-age cognitive performance post-pediatric heart transplantation Gold A, Bondi BC, Ashkanase J, Dipchand AI.Pediatr Transplant. 2020 Dec;24(8):e13832. doi: 10.1111/petr.13832. Epub 2020 Oct 26.PMID: 33105067   Take Home Points In this small (n= 25), but well controlled study of post-transplant pediatric patients, intellectual, academic, and perceptual-motor domain scores were low average while memory abilities were average. Clinical DSM-5 psychological diagnosis was present in >50% of the patients studied. Presence of a congenital heart disease and/or psychological diagnosis predicted worse neuropsychological domain scores. Close neuropsychological monitoring of these patients are needed to maximize long-term outcomes. Commentary from Dr. Clifford Cua (Columbus Ohio USA), section editor of Pediatric/Fetal Cardiology Journal Watch: Previous literature, using heterogeneous methodologies, has documented increased developmental delay, neuropsychological deficits in cognition, and decreased academic achievement in pediatric heart transplant patients with an incidence ranging from 10 – 50%. The goal of this study was to evaluate a non-biased patient sample under uniform conditions and time intervals to better understand the neuropsychological outcomes in these patients. Patients who underwent cardiac transplantation < 2 years of age were recruited. Multiple neuropsychological assessment tools (Table 1) were performed till entry into grade school. Scores were designated average (90 - 109), low average (80 – 89), well below average (70 – 79), and significantly poorer than age expectations (< 70). The study was performed from January 2014 to October 2018. Patients were excluded if they had been assessed before or could not follow up for testing. Baseline demographics as well as clinical data were recorded. Thirty-one patients underwent transplantation during the study period and 25 qualified for the study (18 females, 7 males). Median age at transplantation was 0.71 years and age at testing was 6.7 years. Fourteen patients had CHD and 11 had cardiomyopathy. 72% had neurological issues and 68% had sensory issues pre- or post-transplant. General intellect, academic, and perceptual motor indices were in the low normal range, whereas memory indices were in the normal range (Figure 1 – 3). Fourteen (56%) patients met DSM-5 criteria for a clinical diagnosis (intellectual disability mild 20%, learning disability 20%, language disorder 8%, and ADHD 12%). Another 8 (32%) patients were deemed at risk for developing issues. There was a negative correlation between prior neurological issues (stroke, seizure, microcephaly, or other) and full-scale intelligent quotient, verbal comprehension, working memory, visual learning, verbal story learning, and short delay verbal story memory. There was a negative correlation with CHD and full-scale intelligent quotient, working memory, and fluid reasoning. This study reinforces the need to closely follow up these patients from a neuropsychological standpoint so that early intervention can be performed to maximize long-term outcomes. Though not surprising, it also reinforces that patients with a congenital heart disease diagnosis or prior neurological issues are at increased risk for poorer neuropsychological outcomes. Though the study was small, from a single institution, and had a limited follow up time period, it has the benefits of being complete in its assessment of the patient population.           [/et_pb_text][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default" custom_margin="83px|auto||auto||"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture1.png" title_text="Picture1" _builder_version="4.7.4" _module_preset="default" min_height="729px"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture2.png" title_text="Picture2" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture3.png" title_text="Picture3" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture4.png" title_text="Picture4" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][et_pb_row _builder_version="4.7.4" _module_preset="default"][et_pb_column type="4_4" _builder_version="4.7.4" _module_preset="default"][et_pb_image src="https://uv0pl4on9b-flywheel.netdna-ssl.com/wp-content/uploads/2021/01/Picture5.png" title_text="Picture5" _builder_version="4.7.4" _module_preset="default"][/et_pb_image][/et_pb_column][/et_pb_row][/et_pb_section]

READMORE

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.   Dorobantu DM, Sharabiani MTA, Taliotis D, Parry AJ, Tulloh RMR,...

read more

Impact of Anesthetic and Ventilation Strategies on Invasive Hemodynamic Measurements in Pediatric Heart Transplant Recipients

Impact of Anesthetic and Ventilation Strategies on Invasive Hemodynamic Measurements in Pediatric Heart Transplant Recipients Sheldon Stohl 1 2, Margaret J Klein 3, Patrick A Ross 3 4, Sabine vonBusse 3 5, JonDavid Menteer 4 6 Pediatr Cardiol. 2020 Jun;41(5):962-971....

read more