Pediatric Cardiology

Declining Incidence of Postoperative Neonatal Brain Injury in Congenital Heart Disease. Peyvandi S, Xu D, Barkovich AJ, Gano D, Chau V, Reddy VM, Selvanathan T, Guo T, Gaynor JW, Seed M, Miller SP, McQuillen P.J Am Coll Cardiol. 2023 Jan 24;81(3):253-266. doi: 10.1016/j.jacc.2022.10.029.PMID: 36653093 Take home points: Over a 20-year period, patients with complex congenital heart disease (CHD), the prevalence of preoperative white matter injury (WMI) remained stable, but postoperative WMI declined Improved postoperative systolic, mean, and diastolic blood pressure in the first 24 hours postoperatively was the most important clinical risk factor in reducing postoperative WMI Longitudinal studies will be necessary to determine if this will lead to improved neurodevelopmental outcomes Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: As surgical mortality has declined and patients are living longer with even the most complex CHD, the focus has shifted to other morbidities, in particular, neurodevelopmental issues. This includes attention, processing speed, memory, impulsivity, executive function, and decision-making problems amongst others. This has personal and communal impact as there is an association with lower educational attainment and less employment opportunities. Preoperative brain injury (primarily WMI) is likely due to prenatal factors leading to decreased cerebral oxygen delivery, genetic factors, and possibly the time between birth and initial surgery. It has been reported in 10-35% of patients with neonatal complex/cyanotic CHD. Postoperative WMI may be more modifiable. Prevalence of postoperative WMI has ranged from 33-75% of patients. There have been many changes over the years to try to decrease risks, including improved bypass techniques and support times, improved hematocrit, and likely better post-operative ICU care. This study aimed to describe the temporal trends of neonatal brain injury over a 20-year period between 2001-2021 using pre- and post-operative brain MRI, hypothesizing that improved clinical care may decrease the rate of brain injury. Most patients enrolled were diagnosed with d-TGA or single ventricle physiology (SVP) and expected to require neonatal surgery. Pre-operative MRI was performed as soon as a patient was stable enough to be transported to the MRI scanner. Postoperative MRI were done prior to discharge, with an average of 15 days between studies. 270 patients were enrolled prospectively. 246 patients had a preoperative MRI and 220 had a postoperative MRI. The 20-year period was divided into four 5-year epochs. Primary outcome was presence of WMI, and secondary outcomes included other forms of brain injury (eg. Stroke). The absolute frequency of preoperative WMI or stroke did not change over the study period. However, postoperative WMI decreased and was significantly lower in epoch 4 compared to epoch 1 with an overall decline of ~ 18.7% and a prevalence of ~11% in the most recent epoch. See central illustration. Patients with d-TGA and SVP were stratified and risk factors for WMI studied separately. In both groups, there was no difference in timing of surgery, but both groups had longer bypass and cross-clamp times over the study period. See Tables 4 and 5. In the SVP group, systolic, mean, and diastolic blood pressures were significantly higher in Epoch 4; in the d-TGA group, mean and diastolic BPs were higher. See Figure 2. Some changes in inotropic support were noted, primarily increased use of epinephrine and less milrinone. Since the study was observational, causal inference is limited; however, the rates of early postoperative hypotension were significantly lower by epoch 4, suggesting that changes in postoperative care and better cerebral perfusion may have an important impact on preventing WMI. However, this is in contrast to previous data suggesting elevated SVR and low cardiac output with poor outcomes. The balance between optimal cerebral perfusion and preventing low cardiac output can be tenuous in the critically ill infant and may require new strategies. Additionally, the rates of preoperative WMI did not change, so further understanding of this process to help prevent prenatal WMI is necessary. Whether the lower postoperative WMI will be associated with true improvement in neurodevelopmental testing remains to be seen. An accompanying editorial written by Dr. Jane Newburger eloquently places this study in the context of the overall canon of research on ND in CHD. Other studies have not shown improved ND outcomes even with lower postoperative WMI, with other factors (length of stay and socioeconomic status) having a greater impact. Additionally, preoperative WMI may have a greater impact on developmental scores than postoperative WMI. Further longitudinal study and multicenter analysis will be necessary in order to develop evidence-based pathways and interventions to improve ND outcomes.

Initial Experience with Telemedicine for Interstage Monitoring in Infants with Palliated Congenital Heart Disease. Stagg A, Giglia TM, Gardner MM, Offit BF, Fuller KM, Natarajan SS, Hehir DA, Szwast AL, Rome JJ, Ravishankar C, Laskin BL, Preminger TJ.Pediatr Cardiol. 2023 Jan;44(1):196-203. doi: 10.1007/s00246-022-02993-y. Epub 2022 Sep 1.PMID: 36050411 Take home points: The addition of telemedicine visits in lieu of entirely in-person visits for interstage monitoring of single ventricle patients is feasible, safe, prevents unplanned ER visits, and is associated with high levels of satisfaction by both families and clinicians Larger studies as well as adjunct use of remote diagnostic technologies will help determine the full capabilities of TM in this population Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: The interstage period between stage 1 and stage 2 surgical palliation for single ventricle congenital heart disease has historically been considered high risk for morbidity and mortality. The development of home monitoring and dedicated single ventricle teams have been instrumental in improving outcomes. Especially due to the COVID pandemic, telemedicine visits have been more frequently used for routine care, but its efficacy and safety in a higher risk subgroup has not been fully evaluated. This study assessed the use of TM in the interstage period along with the standard home monitoring protocols. In their program called interstage single ventricle monitoring program (ISVMP), weekly visits were performed alternating between the PCP and cardiologist. TM replaced at least one in-person PCP visit and was not used at nights or over the weekend. The TM visit tracked clinical concerns, interim ED visits or hospitalizations, visual assessment of patient color, activity, work of breathing, and respiratory rate, along with documentation of the HR and O2 sat using the home pulse oximeter. The clinician also discussed the daily weights, nutrition, and medications with the primary caregiver. The monthly frequency of ED visits per patient and estimated costs were tracked by comparing the standard ISVMP (Aug 2018-May 2019) with ISVMP + TM (Aug 2019- May 2020). Additional tracking included hospitalizations, identification of clinical concerns, whether TM prevented unnecessary ED visits or expedited in-person visits, and clinician and caregiver satisfaction. 60 TM visits were conducted for 29 patients with a median monitoring time of 199 days. The median number of TM visits/patient was 2 (range 1-5). 98% of visits had a successful audiovisual connection at the first attempt, and the average length of the visit was 20 minutes. In 6 TM visits (6 different patients), significant clinical findings (decreased O2 sats, tachypnea, poor feeding) were identified that led to therapeutic changes that avoided an ED visit (as these findings prior to TM would have been associated with an automatic referral to the ED). Expedited follow-ups were subsequently performed in 3 of the 6 patients. An additional 5 TM visits led to expedited follow-up with one of these patients being hospitalized. There were no missed events or deaths. There was a reduction in median monthly ED visits (see Table 2). 6 hospitalizations occurred in 2 patients who were unable to be seen via TM due to night/weekend events. All visits identified at least one non-urgent issue. 97% of caregivers were satisfied with TM visits, were interested in future TM visits, and only about 17% experienced some technical difficulties that were resolved quickly. Clinicians were also highly satisfied, thought the caregivers were receptive, and the visits effective for patient care. Family/patient education and troubleshooting home equipment problems were markedly improved using TM than from in-person visits. The use of TM greatly expanded during the COVID pandemic but is likely to continue to be a useful resource, even for the highest risk patients. There is a significant saving of time and money from the family’s perspective, and if there is not an increase in patient events or negative effects on outcomes, this should be considered in more centers if medical reimbursement remains. The authors noted that there are significant logistical and staffing needs when using TM, along with the need for technical support and translation services.

German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease: Report 2020—Comprehensive Data from 6 Years of Experience Andreas Beckmann, S. Dittrich, C. Arenz, et al  and German Quality Assurance / Competence Network for Congenital Heart Defects Investigators Thorac Cardiovasc Surg. 2021 Dec;69(S 03):e21-e31. doi: 10.1055/s-0041-1722978. Epub 2021 Feb 26. PMID: 33638137; PMCID: PMC7920329. Take Home Message: Large registries can be successfully maintained and can provide valuable information Registries can form a basis for internal and external quality assurance for participating institutions. Mortality in CHD interventions and surgeries is low. It is highest in neonates with high risk  complex diseases, that require multiple procedures. In Germany, mortality for isolated surgical procedures is minimal and rises to about 12% in high risk, STA5 procedures. Commentary from Dr. Anna Tsirka (Hartford, CT, USA), section editor of Pediatric and Fetal Cardiology Journal Watch Introduction In Germany, the  German Society for Thoracic and Cardiovascular Surgery (DGTHG) and German Society for Pediatric Cardiology and Congenital Heart Defects (DGPK) took the initiative to create a multicenter registry study concerning interventional and surgical therapies in patients with CHD. The nationwide registry started in 2012.  This report presents data from the first 6 years of data entry. Each included patient receives a unique personal identification (PID), valid for life. Based on the PID, any invasive interventional or cardiac surgical procedure can be assigned exactly to each individual patient, even if the treatment is provided in different institutions. This enables longitudinal, procedure-related data acquisition, as well as short-, mid- and long-term evaluations. Risk adjustment models for operations and interventions were implemented in 2014. The risk group classification for operations is performed according to the society of thoracic surgeons (STAT) mortality score while risk group adjustment for interventions is based on the internationally acknowledged Bergersen's score. In addition to procedure-related data, various indicators, including the occurrence of adverse events, in-hospital mortality, and 30-day and 90-day mortality, are evaluated. Major and minor adverse events following surgical procedures are classified according to the society of thoracic surgeons (STS) morbidity classification. Risk adjustment models for operations and interventions were implemented in 2014. The risk group classification for operations is performed according to the internationally recognized society of thoracic surgeons-european association for cardio-thoracic surgery (STAT) mortality score 4 5 while risk group adjustment for interventions is based on the internationally acknowledged Bergersen's score. 6 These classifications cover five categories for surgical and four for interventional procedures. However, it has to be remembered that the two risk scores are not comparable, as they are based on different morbidity expectations and different evaluations of special features and complications. In addition to procedurerelated data, various indicators, including the occurrence of adverse events, in-hospital mortality, and 30day and 90-day mortality, are evaluated. Major and minor adverse events following surgical procedures are classified according to the society of thoracic surgeons (STS) morbidity classification. Results Data and results encompass the period between 2013 to 2018 collected from 20 to 24 departments performing surgical and from 24 to 30 departments carrying out interventional procedures depending on the year. A total number of 35,730 patients was included, leading to 39,875 cases, and 46,700 procedures. The cases could be subdivided into 17,259 interventional, 21,027 surgical cases, and 1,589 with multiple procedures ( hybrid procedures are included under multiple procedures and comprised less than 1% of all procedures). Overall, 4,708 (11.8%) of all cases were performed in neonates, 10,047 (25.2%) in infants,  19,351 (48.5%) in children/adolescents at age of 1 to 18 years, and 5,769 (14.5%) in adults. Mortality by age and type of procedure is shown below: The observed mortality rate was lowest in interventional cases and highest for cases requiring multiple procedures with a range of 6.2 to 10.4%. This can be explained by the fact that in the majority of cases, the combination of more than one surgical and interventional procedure is frequently requires in newborns and infants with complex heart malformations, representing the highest risk groups. Mortality was minimal in procedures performed for isolated defects.  It increased by STAT risk category, as expected, but even in the highest risk category STAT5) the vast majority of patients survived (87.8%). Mortality by defect and stat category is shown in the table below: Similarly, complications were the lowest in interventional cases and highest in the patients who underwent multiple procedures as shown in the figure below: Adverse events followed the same pattern. Over 90% of interventional cases were uncomplicated, surgical cases had some adverse events in about 36% of the cases, while about 56% of cases with multiple interventions had some adverse event. Of those, 8.7% were serious or “catastrophic” Over the registry period, the overall incidence of mortality or adverse events did not change. Conclusion The creation of large registries is possible and can give valuable data. Overall mortality in congenital heart disease in Germany is low. Interventional procedures have significantly lower risk than surgical procedures. It has to be emphasized, however, that interventions and operations must not be understood as competing, but as complementary therapeutic options. Even in patients with the same anatomic diagnosis, the selection criteria for interventional or surgical procedures, may differ considerably. Registry data of course have limitations, as specific patient characteristics cannot be distinguished. Nevertheless, the data obtained is valuable and can provide longitudinal trends overtime, as well important comparative feedback for specific centers.