Pediatric Cardiology

Inpatient Resource Utilization for Hypoplastic Left Heart Syndrome from Birth Through Fontan.  Kuntz M, Valencia E, Staffa S, Nasr V. Pediatr Cardiol. 2024 Mar;45(3):623-631. doi: 10.1007/s00246-023-03372-x. Epub 2023 Dec 30. PMID: 38159143 Take home points: Between 2016-2021, median total adjusted charges over the course of 3-stage palliation for HLHS were $1,475,800 (stage 1 $604,300, stage 2 $234,000, and stage 3 $256,260) and higher than reported on previous studies Pulmonary artery stenosis was the most common admitting diagnosis for interstage hospitalizations (3.4%) Cardiac catheterization and feeding tube placement were the most common interstage procedures Commentary from Dr. Jared Hershenson (Greater Washington DC), section editor of Pediatric Cardiology Journal Watch: 3-stage surgical palliation for HLHS requires substantial resources. There are also elective and emergent admission and procedures necessary during interstage periods. Over the past decades, survival has improved, and more patients are undergoing palliation. Resource allocation is important for an individual hospital as well as from a public health perspective, so with cost changes, inflation, etc., defining the amount currently needed/expected is necessary. This study searched the Pediatric Health Information System (PHIS) database that contains data from over 50 children’s hospitals in the US for HLHS (ICD-10 Q23.4) between 2016-2021. Admissions corresponding to all 3 stages were identified and patients were excluded if they did not complete all 3 at the same institution. Comorbid conditions, length of stay (total and ICU), duration of mechanical ventilation, ECMO, readmission, and cardiac arrests were reported. All admissions that occurred interstage were also analyzed. Charges for each stage and interstage admissions/procedures were determined. 199 patients were identified. Table 1 shows demographic data and Table 2 shows the comorbid conditions, with rhythm disturbances and NEC most common in stage 1 (or combined stage 1 and 2 in the same hospitalization), and rhythm disturbances and gastrostomy in stage 2 and 3. Table 3 shows the index hospitalization data. There were 474 interstage hospitalizations among the cohort which represents 2.4 admissions/per patient; 1/3 required a procedure/surgery which were most commonly cardiac catheterization for pulmonary artery stenosis and enteral feeding access, and 1/3 required ICU admission. See table 5. Median total charges were $1,475,800. Stage 1 had the highest charges compared to the other 2 planned stages (median charges for stage 1 $604,300, stage 2 $234,000, and stage 3 $256,260). The highest interstage charges occurred with admissions within 30 days after stage 3, median $173,100. See the article for a breakdown of charges (clinical, imaging, laboratory, pharmacy, supply, other). The authors note that this is updated data from the last decade, and costs are significantly higher for all stages than previously reported. Reasons for this were not specifically determined in this study, but there are likely more higher risk patients having surgical palliation than in prior eras. Patient specific risk characteristics associated with resource utilization could also not be determined using the PHIS database, but future studies looking at this would be useful. While charges may overestimate actual cost, they still allow for planning for future resource utilization. LOS was higher in this study than in previous reports; this may be due to better survival, but there may be other factors as well. Additionally, charges may be higher for non-surviving HLHS patients, so this study may underestimate true total charges amongst all patients that may not reach stage 3 palliation. Interstage hospitalizations at other hospitals were also not included.

Prevalence and clinical correlates and characteristics of "Super Fontan". Ohuchi H, Mori A, Kurosaki K, Shiraishi I, Nakai M.Am Heart J. 2023 Sep;263:93-103. doi: 10.1016/j.ahj.2023.05.010. Epub 2023 May 20.PMID: 37211285  Commentary from Dr. Anna Tsirka (Hartford, CT, USA), section editor of Pediatric and Fetal Cardiology Journal Watch Summary: Super Fontan is more common in younger, slim males, however by the age of 30, no patients meet the criteria for SF any longer.  Lower pre Fontan pulmonary vascular resistance and early Fontan completion were associated with SF.  Type of Fontan, single ventricle morphology, and ventricular systolic function did not correlate with SF status.  SF patients have superior pulmonary and hepatorenal function, as well as hemostatic function.  SF patients also have  lower risk for mortality and hospitalization for heart failure but no difference in the risk for hospitalization for arrhythmias.  Higher levels of physical activity (over 2.8 hours per week) in childhood are strongly correlated with SF status.  Introduction Patients with Fontan circulation exhibit lower exercise capacity compared to they are normal healthy peers.  A subset however of patients with Fontan circulation (10-20%) have normal exercise capacity, and they are defined as being “super Fontan “ (SF). This study aimed at evaluating the prevalence of the super Fontan and assess factors associated with it, such as hemodynamics, end-organ function, activity and trajectory of exercise capacity. Methods This is a retrospective chart review of 404 Fontan patients who underwent adequate exercise testing between 2005 and 2021 at a single hospital in Osaka, Japan.  Patient's were defined as super Fontan if they had pak VO2 over 80% of predicted.  The initial diagnosis, body habitus, Fontan complications, and medications at the time of the exercise test were recorded.  Exercise testing was performed within a week of a planned cardiac catheterization.  27% of the patients completed the daily activity questionnaire, and the trajectory of exercise capacity was determined in patients who had more than 1 exercise test with an interval of more than 2 years.  Other factors evaluated were isometric strength, pulmonary function, hemodynamics, BNP, hepatorenal function and a liver ultrasound. Results: The patient characteristics are summarized in table 1.  SF patients were male, were slimmer, had early Fontan completion.  They had less incidence of Fontan complications.  Ventricular morphology and type of Fontan did not make a difference. The prevalence of SF decreased with age:  38% of less than 10 years, 32% at 10-20, 11% at 20-30 years, and 0% at over the age of 30 years. Hemodynamic factors before Fontan that were statistically significant for the presence of SF were lower pulmonary vascular resistance,  lower end-diastolic volume index, and higher saturations, although the differences were very small, therefore not clinically significant as indicated in table 4. Hemodynamic factors at evaluation at the age of a exercise test revealed higher cardiac output, lower EDP, and higher saturations in those with SF.  Interestingly, single ventricle systolic function did not correlate with presence of SF. SF patients has had lower BNP, and better pulmonary function.  SF patient has had better liver function, and better creatinine clearance.  Interestingly, they also had better hemostatic function. Exercise during childhood Physical activity time (PAT) in childhood correlated with presence of SF.  Child  Fontan patients with PAT> 2.8 hours/week  had 9.6 times higher chance of being an adult SF.  There was no difference in strength between SF and non SF patients. Unscheduled hospitalizations (USH) for heart failure were lower in the SF group, however there was no difference in USH for arrhythmia.  SF patient has had lower mortality. Conclusion SF is associated with better long-term outcomes.  Pre- Fontan hemodynamics are associated with incidence of SF, however very strong predictors of SF were physical activity over 2.8 hours in childhood and slim physiologic. This study demonstrates the importance of promoting physical activity and healthy body composition among Fontan patients from childhood.

Acute Kidney Injury and Mid-term Outcomes After Extra-Cardiac Fontan Conversion. Kunigo T, Oikawa R, Nomura M.Pediatr Cardiol. 2023 Jun 24. doi: 10.1007/s00246-023-03220-y PMID: 37355505 Take home points: Preoperative and 1-year postoperative BNP levels could be associated with postoperative AKI and re-admission due to heart failure. Early postoperative nitric oxide (NO) inhalation was more frequently in patients with AKI. To prevent AKI after TCPC conversion and reduce re-admission due to heart failure, the authors recommend BNP-guided heart failure treatment preoperatively and following BNP level 3 months after the incidence of AKI even if AKI have restored. Commentary from Dr. Manoj Gupta (New York City, NY, USA), chief section editor of Pediatric & Fetal Cardiology Journal Watch. Introduction The first Fontan operation was performed in 1971, and the procedure was later modified several times. Patients suffering from long-term complications after the classic Fontan procedure need total Cavo-pulmonary connection (TCPC) conversion by the extra-cardiac conduit (ECC) method. Postoperative acute kidney injury (AKI) after initial Fontan operation in pediatric patients is common and risk factor for morbidity and mortality. In this study, we investigated the incidence of postoperative AKI and unplanned re-admission rate due to heart failure at 2-year follow-up in patients who had extra-cardiac Fontan conversion. Medical records between January 2014 and December 2021 were retrospectively reviewed. Patients who had undergone TCPC conversion by the ECC method were enrolled in this study. The indications for TCPC conversion were symptomatic atrial arrhythmia, atrial enlargement, thrombus in the atrium, low cardiac output in a catheter examination, hypoxemia due to baffle leak, heart failure due to failed Fontan, need for other cardiac surgery, and desire to have children. All the patients underwent TCPC conversion by the ECC method. The primary outcome was unplanned re-admission rate due to heart failure at 2-year follow-up. Re-admission due to heart failure was defined by a cardiologist’s diagnosis and need for treatment by them. The secondary outcomes were the incidence of AKI, variables that could cause postoperative AKI, perioperative course and 1-year outcomes. Results A total of 47 patients were converted to TCPC by the ECC method and analyzed in this study. Postoperative AKI occurred in 22 patients (46.8%) and 5 patients with AKI needed renal replacement therapy (RRT) (Fig. 1). All AKI was diagnosed on creatinine criteria within 48 h after surgery. RRT was initiated in 3 cases for anuria and 2 cases for uremia. All patients recovered from AKI and did not even meet the criteria for AKI stage 1 at discharge. No patient had preoperative hemodialysis for chronic renal failure.  Although there was no significant difference between the two groups in renal function, preoperative brain natriuretic peptide (BNP) level was significantly higher in patients with AKI than those without AKI. There were 6 patients in AKI group and 3 patients in non-AKI group with preoperative BNP values over 200 (27.3% vs. 12.0%, p=0.27). There was no significant difference between the two groups in intraoperative data; however, early postoperative nitric oxide (NO) inhalation was more frequently in patients with AKI. There were no significant differences between AKI group and non-AKI group in additional procedure including valve surgery, coronary artery bypass grafting, and Maze during TCPC conversion. At 1 year after conversion, serum creatinine level and ventricular end diastolic pressure were significantly higher and BNP level tended to be higher in patients who had AKI than those who did not have AKI. Discussion In this study, early postoperative AKI after extra-cardiac Fontan conversion was associated with unplanned re-admission due to heart failure at 2-year follow-up even when renal function returned to baseline.  Prevention of AKI could be more important to improve long-term outcomes after TCPC conversion than treatment of AKI. “KDIGO bundle” consisting of optimization of volume status and hemodynamics, avoidance of nephrotoxic drugs, and preventing hyperglycemia reduced the frequency and severity of AKI after cardiac surgery.  Older age, more than 3 prior sternotomies, prior ablation procedure and higher preoperative right atrial pressure were reported as risk factors for moderate-to-severe AKI after Fontan conversion. Sammour et al. reported that higher preoperative serum creatinine levels were associated with postoperative RRT in their case series of 23 patients after Fontan conversion. Patel et al, independent risk factors for AKI≥2 included older age, ≥3 prior sternotomies, greater preoperative right atrial pressure, and prior catheter ablation procedure. Per Niaz et al, Multivariable risk factors for AKI were asplenia, elevated preoperative pulmonary artery pressure, intraoperative arrhythmias, and elevated post-bypass Fontan pressure. References: Acute kidney injury after Fontan completion: Risk factors and outcomes April 2015 Journal of Thoracic and Cardiovascular Surgery 150(1) DOI:10.1016/j.jtcvs.2015.04.011 Acute Kidney Injury Is Associated With Increased Long-Term Mortality After Cardiothoracic Surgery https://doi.org/10.1161/CIRCULATIONAHA.108.800011 Circulation. 2009;119:2444–2453 Incidence, Predictors, and Impact of Postoperative Acute Kidney Injury Following Fontan Conversion Surgery in Young Adult Fontan Survivors Sheetal R. Patel, MD, MSCI , John M. Costello, MD, MPH, Adin-Cristian Andrei, PhD, DOI:https://doi.org/10.1053/j.semtcvs.2021.02.027 Acute Kidney Injury and Renal Replacement Therapy After Fontan Operation, Talha Niaz, MBBS, Elizabeth H. Stephens, MD, PhD, Stephen J. Gleich, MD DOI:https://doi.org/10.1016/j.amjcard.2021.08.056

Single-drug immunosuppression is associated with noninferior medium-term survival in pediatric heart transplant recipients. Watelle L, Touré M, Lamour JM, Kemna MS, Spinner JA, Hoffman TM, Carlo WF, Ballweg JA, Greenway SC, Dallaire F. J Heart Lung Transplant. 2023 Aug;42(8):1074-1081. doi: 10.1016/j.healun.2023.02.1705. Take home points: After heart transplantation, immunosuppression with a single immunosuppressive drug after the first year post-transplant was noninferior to standard therapy (≥2 immunosuppressive drugs) Based on this retrospective analysis, monotherapy was not associated with change in the incidence of rejection, infection, and malignancy, and even the development of Coronary Artery Vasculopathy. Commentary from Dr. Manoj Gupta (New York City, NY, USA), chief section editor of Pediatric & Fetal Cardiology Journal Watch. Introduction: Heart transplantation (HT) requires lifelong immunosuppression to prevent organ rejection and enable allograft and patient survival. Traditionally, maintenance immunosuppression following HT has required at least 2 immunosuppressive drugs. However, anecdotally, many patients are successfully maintained on monotherapy (defined as taking only a single immunosuppressive drug) for varying durations. Patients may be placed on monotherapy because of immunosuppressive drugs side effects or complications (i.e., post-transplant lymphoproliferative disease, PTLD) or they are deemed to be at low immunological risk for rejection (i.e., neonatal transplantation). To test this, the authors leveraged data from the Pediatric Heart Transplant Society (PHTS) to describe a cohort of pediatric heart transplant recipients on monotherapy and to assess graft failure and complication rates in patients on monotherapy compared to those on ≥2 immunosuppressive drugs. Methods: For the current study, we included patients with their first heart transplant before 18 years of age between 1999 and 2020 and who had ≥1 year of follow-up data available.  The primary outcome was graft failure, a composite of death and retransplantation. Secondary outcomes were graft rejection, infection, malignancy, cardiac allograft vasculopathy (CAV) and need for dialysis. Results We identified 3493 heart transplant recipients from the PHTS database meeting our inclusion criteria and exclusion criteria. Patient demographics and baseline characteristics are summarized in Table 1. The median follow-up time post-transplant was 6.7 years (range: 1.3-20.5 years, IQR 4.5-10 years) for a total of 26,740 patient-years. There were 893 patients (25.6%) switched to monotherapy at least once during follow-up with the remaining 2600 patients on ≥2 ISDs for their entire follow-up. The median time on monotherapy was 2.8 years (IQR 1.1-5.9 years) for a total of 3824 patient-years on monotherapy. Patients on monotherapy were younger at the time of transplant (median 1.3 years vs 6.2 years) and were more likely to have undergone transplantation as a neonate (48/893 vs 37/2600 patients or 5.4% vs 1.4%). The most common underlying diagnoses leading to HT were cardiomyopathy (1857/3493 patients, 53.2%) and congenital heart disease (1530/3493 patients, 43.8%) without a clear difference in diagnosis between those ever on monotherapy and those never on monotherapy. At 1-year post-transplant (i.e., the start of follow-up for the current analysis), there were 260/3493 patients (7.5%) already on monotherapy. This proportion steadily increased to peak at 23.9% at 12.4 years post-HT and then slightly decreases thereafter. The agent most frequently used during monotherapy was tacrolimus (69.7%) followed by cyclosporine (26.5%). Sirolimus was rarely used as monotherapy (1.8%). Other agents (mycophenolate mofetil (MMF), steroids, azathioprine and everolimus) were each used < 1% for monotherapy. Discussion How much immunosuppression is necessary for an individual child after heart transplantation is an important question. The use of monotherapy may offer fewer episodes of infection and malignancy post-transplant once past the higher-risk period of rejection within the first year post transplant. In this study, we found that a sizeable proportion of patients are on a single immunosuppressive drug and, importantly, monotherapy appears to be noninferior to standard (≥2 immunosuppressive drugs) post-transplant immunotherapy for the primary outcome of death or retransplantation. Conclusions Our study suggests that for children switched to monotherapy after HT, immunosuppression with a single ISD after the first year post-transplant was noninferior to standard therapy (≥2 ISDs) for the outcomes of death or retransplantation in the medium-term. We also found that monotherapy was not associated with change in the incidence of rejection, infection and malignancy, and even the development of CAV. Our data suggest that the clinician’s decision to switch these patients to monotherapy was not detrimental.

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.