Team dynamics after implementation of guidelines for anaesthesia for children with CHD.

Guidelines were created at our single centrer institution for which anesthesiology team should care for pediatric cardiac patients for noncardiac surgery. The goal of the survey was to assess inter-team dynamics after the implementation of guidelines and revealed that practice behaviour can quickly change but a sustained change in team dynamics and workplace culture takes time.

Surgical repair of "Swiss Cheese" ventricular septal defects with two-patch and right ventricular apex-exclusion technique: mid-term follow-up results.

BACKGROUND: "Swiss Cheese" ventricular septal defects represent a serious congenital heart disease with suboptimal clinical outcomes and a lack of consensus regarding its management. This study presents mid-term follow-up results of surgical repairs for "Swiss Cheese" ventricular septal defects, utilizing the two-patch and right ventricle apex-exclusion technique. METHODS: A retrospective review was conducted on 13 patients who underwent surgical repair utilizing the two-patch and right ventricle apex-exclusion technique at our institution between May 2014 and October 2021. The procedure involved the closure of defects in the outflow tract ventricular septal and the apex trabecular ventricular septal regions using two patches, with concurrent exclusion of the right ventricular apex from the right ventricular inflow tract. RESULTS: Median follow-up was 4.9 ± 2.1 years (range: 2-9 years). All cases were successful without mortality or major complications. Two years post-surgery, cardiac magnetic resonance revealed median values for left ventricular ejection fraction, right ventricular ejection fraction, left ventricular end-diastolic volume and right ventricular end-diastolic volume of 63.9% ± 1.8% (range: 61-67%), 49.2% ± 2.6% (range: 46-55%), 39.15 ± 2.11 ml (range: 36.2-42.7 ml), 44.55 ± 3.33 ml (range: 38.7-48.6 ml), respectively. No thrombosis occurred. The latest echocardiography results confirmed normal cardiac function in all cases. CONCLUSIONS: The surgical repair of "Swiss Cheese" ventricular septal defects utilizing the two-patch and right ventricle apex-exclusion technique is a viable approach with favorable mid-term outcomes. More cases and long-term follow-up results are needed to validate the feasibility and safety of this technique.

Heart transplantation for pediatric patients with malignant arrhythmias: Indications and Outcomes.

BACKGROUND: Arrhythmias can lead to cardiac arrest (CA) and heart failure. When intractable, heart transplant (HTX) can become the only viable treatment. This rare, high-risk cohort has not been reported as a distinct group. OBJECTIVE: Characterize the outcomes of pediatric patients listed for HTX with the primary indication being malignant arrhythmia (MA). METHODS: Using the Pediatric Heart Transplant Society prospective registry, we identified all patients <18 years-old listed between 2014-2022. MA as the listing indication was categorized into primary tachy-arrhythmia (PT), inherited arrhythmia (IA), congenital heart disease (CHD) and cardiomyopathy (CM) with secondary arrhythmia. Demographic, listing and transplant data were analyzed. RESULTS: Among 4630 patients listed and 3317 transplanted, MA was the indication in 63 (1.4%) and 49 (1.5%), respectively. MA patients were categorized as PT in 11, IA in 4, CHD in 6 and CM in 42. When compared to the non-MA cohort, patients listed for MA were older (mean (SD) age 10.6 (6.2) vs. 6.1 (6.2) years, p<0.01), more likely to present with a CA (43% vs. 11%, p<0.01), and less likely to be in the intensive care unit (40% vs. 58%, p<0.01) or on inotropes (30% vs. 60%, p<0.01) at listing. Outcomes including waitlist mortality, transplantation, post-transplant survival and freedom from rejection were comparable to the non-MA cohort. CONCLUSION: Patients with MA constitute a small proportion of those listed for HTX in childhood. CM was the most common category, while IA and PT were rare. Their waitlist mortality and post-transplant outcomes were comparable to the non-MA cohort.

Twenty-Seven-Year Institutional Experience With Surgery for Adults With Congenital Heart Disease.

BACKGROUND: Given improved contemporary survival of adults with congenital heart disease (ACHD), we aimed to evaluate trends in ACHD surgery and outcomes at a single center over a 27-year period. METHODS: Surgical databases were retrospectively queried for patients >18 years old who underwent ACHD surgery between January 1, 1994, and December 31, 2020. A total of 2,195 included patients underwent 2,425 cardiac surgical procedures within the specified time frame. Patients were grouped by era: I, 1994-2000; 2, 2001-2010; and 3, 2011-2020. Trends in primary cardiac diagnosis and surgical management were evaluated. RESULTS: The median age increased across the eras. The most common primary cardiac diagnoses (n = 2,425) overall were left ventricular outflow tract anomalies (n = 2,019, 83%), atrial septal defect (n = 407, 17%), right ventricular outflow tract anomalies (n = 360, 15%), and ventricular septal defect (n = 110, 4.5%). The most commonly observed procedures overall were operations on the left ventricular outflow tract (n = 1,633, 67%), aorta (n = 675, 28%), coronary arteries (n = 449, 19%), right ventricular outflow tract (n = 323, 13%), and atrial septal defect (n = 264, 11%). Major complications occurred in 10% of cases, and 58 patients died within 30 days of their operation yielding an operative mortality of 2.4%. CONCLUSION: To our knowledge, this is the largest single center report on surgery for adults with congenital heart disease. Surgery for ACHD has been performed at our center with relatively low morbidity and mortality over the last few decades.

Sanguineous cardiopulmonary bypass prime accelerates the inflammatory response during pediatric cardiac surgery.

BACKGROUND: The inflammatory response to cardiopulmonary bypass (CPB) in pediatric patients remains an unresolved challenge. Sanguineous CPB prime, composed of allogenic blood products, is one potentially important stimulus. This study aims to identify specific inflammatory mediators active in sanguineous CPB prime and their impact on the inflammatory response at CPB initiation. METHODS: In a post-hoc analysis of a prospective observational cohort study (NCT05154864), where pediatric patients undergoing cardiac surgery with CPB were enrolled after informed consent, patients were grouped by CPB prime type (sanguineous vs crystalloid). Arterial samples were collected post-sternotomy as a baseline and again at CPB initiation from all patients. In the sanguineous group, CPB prime samples were also collected after buffered ultrafiltration but before CPB initiation. Luminex® measured concentrations of 24 inflammatory mediators for comparison between groups. Statistical analyses were by Mann-Whitney test and Wilcoxon signed-rank test. Data are presented as median [IQR]. RESULTS: Forty consecutive pediatric patients participated. The sanguineous group (n = 26) was younger (4.0 [0.2 - 6.0] vs 48.5 [39.0 - 69.5] months; p = 2.6 × 10-7) and smaller (4.9 [34 - 6.6] vs 17.2 [14.9 - 19.6] kg; p = 2.6 × 10-7) than the crystalloid group (n = 14). Despite this, baseline concentrations of 20 complement and cytokine concentrations were comparable between groups (p > 0.05) while four showed differences between groups (p < 0.05). The sanguineous prime contained supraphysiologic concentrations of complement mediators: C2, C3, C3a, C3b, and C5a. Correspondingly, upon CPB initiation, patients receiving sanguineous prime exhibited a significantly larger burden of C2, C3, C3b, C5, and C5a (p < 0.001) relative to the crystalloid group. Cytokine and chemokine mediators were present at trace levels in the sanguineous prime. CONCLUSIONS: Sanguineous prime contains activated complement that accelerates the inflammatory response at CPB initiation in neonates and infants. Immunomodulatory interventions targeting complement during CPB prime preparation could offer substantial benefits for these vulnerable patients.

Efficacy and Safety of Esmolol in Neonatal Cardiac Surgery with Cardiopulmonary Bypass (CPB) for d-Transposition of the Great Arteries (d-TGA).

OBJECTIVE: D-Transposition of the great arteries (d-TGA) is the most common congenital heart disease requiring surgical correction within the neonatal period. Sinus tachycardia often persists postoperatively, potentially affecting cardiac function. This study aimed to investigate the efficacy and safety of the short-acting beta-1-selective beta-blocker esmolol in controlling heart rate in neonatal cardiac surgery with cardiopulmonary bypass (CPB). METHODS: A retrospective cohort study was conducted on neonates undergoing surgery for d-TGA. The study cohort included 112 patients, divided into an esmolol intervention group (n = 57) and a control group (n = 55). Baseline characteristics, hemodynamic parameters and outcome measures were assessed. RESULTS: In the esmolol group, median heart rate at ICU admission was significantly higher compared to the control group (155 vs. 147 bpm, p = 0.018). After a median time of 11 h, heart rate was lower among the esmolol patients (135 vs. 144 bpm, p < 0.001). There were no differences in other hemodynamic parameters between the two groups. Patients treated with esmolol required longer catecholamine support while no difference regarding survival, duration of invasive ventilation and ICU stay were noticed. CONCLUSION: No relevant hemodynamic difference was seen between neonates treated with perioperative esmolol and the control group and outcome did not differ. This indicates non-inferiority of perioperative betablocker therapy in young age. Prospective and placebo-controlled assessment of perioperative esmolol therapy in neonates is needed.

The cumulative impact of clinical risk on brain networks and associations with executive function impairments in adolescents with congenital heart disease.

Patients with congenital heart disease (CHD) demonstrate altered structural brain network connectivity. However, there is large variability between reported results and little information is available to identify those patients at highest risk for brain alterations. Thus, we aimed to investigate if network connectivity measures were associated with the individual patient's cumulative load of clinical risk factors and with family-environmental factors in a cohort of adolescents with CHD. Further, we investigated associations with executive function impairments. In 53 adolescents with CHD who underwent open-heart surgery during infancy, and 75 healthy controls, diffusion magnetic resonance imaging and neuropsychological assessment was conducted at a mean age of 13.2 ± 1.3 years. Structural connectomes were constructed using constrained spherical deconvolution tractography. Graph theory and network-based statistics were applied to investigate network connectivity measures. A cumulative clinical risk (CCR) score was built by summing up binary risk factors (neonatal, cardiac, neurologic) based on clinically relevant thresholds. The role of family-environmental factors (parental education, parental mental health, and family function) was investigated. An age-adjusted executive function summary score was built from nine neuropsychological tests. While network integration and segregation were preserved in adolescents with CHD, they showed lower edge strength in a dense subnetwork. A higher CCR score was associated with lower network segregation, edge strength, and executive function performance. Edge strength was particularly reduced in a subnetwork including inter-frontal and fronto-parietal-thalamic connections. There was no association with family-environmental factors. Poorer executive functioning was associated with lower network integration and segregation. We demonstrated evidence for alterations of network connectivity strength in adolescents with CHD - particularly in those patients who face a cumulative exposure to multiple clinical risk factors over time. Quantifying the cumulative load of risk early in life may help to better predict trajectories of brain development in order to identify and support the most vulnerable patients as early as possible.

Trends in Gaps of Care for Patients With Congenital Heart Disease: Implications for Social Determinants of Health and Child Opportunity Index.

BACKGROUND: Lifelong continuity of care is essential for patients with congenital heart disease (CHD) to maximize health outcomes; unfortunately, gaps in care (GIC) are common. Trends in GIC and of social determinants of health factors contributing to GIC are poorly understood. METHODS AND RESULTS: This retrospective cohort study included patients with CHD, aged 0 to 34 years, who underwent surgery between January 2003 and May 2020, followed up at a pediatric subspeciality hospital. Patients were categorized as having simple, moderate, and complex CHD based on 2018 American Heart Association and American College of Cardiology guidelines. Social determinants of health, such as race, ethnicity, language, insurance status, and Child Opportunity Index, based on home address zip code, were analyzed. Of 2012 patients with CHD, a GIC of ≥3 years was identified in 56% (n=1119). The proportion of patients with GIC per year increased by 0.51% (P<0.001). Multivariable longitudinal models showed that the odds of GIC were higher for patients who were ≥10.5 years old, had simple CHD, lived out of state, lived farther from care site, received public insurance, had less protection with additional insurance plans, and with low Child Opportunity Index. A separate model for patients with only moderate/complex CHD showed similar findings. Race and ethnicity were not associated with the odds of experiencing GIC over time. CONCLUSIONS: GIC have increased over time for patients with CHD. Social determinants of health, like insurance, access, and neighborhood opportunity, are key risk factors for increasing GIC. Efforts to reduce GIC in patients with CHD should focus on addressing the impact of specific social determinants of health.

Outcomes of the Modified Warden Procedure for Partial Anomalous Pulmonary Venous Connection Drainage to a High Location in the Superior Vena Cava.

AIM: To report the safety and effectiveness of a modified Warden procedure. METHOD: Twenty-six (26) patients underwent modified Warden surgery at our centre from September 2017 to September 2021. In all patients, the superior vena cava (SVC) was reconstructed by turning the atrial wall in the main body of the right atrium upwards and applying fresh autologous pericardial patches. There were 13 male patients and 13 female patients, and the median age of the patients was 7.0 (range, 0.3-47.0) years. The median follow-up time after surgery was 47.0 (range, 32.0-80.0) months. RESULTS: The aortic cross-clamp time was 108.50±34.72 minutes, the cardiopulmonary bypass time was 154.81±41.65 minutes, the median postoperative mechanical ventilation time was 8.5 (range, 0.0-91.0) hours, the median intensive care unit stay was 43.5 (range, 15.0-352.0) hours, and the median length of postoperative hospital stay was 7.0 (range, 6.0-19.0) days. All patients were safely and successfully discharged from the hospital. No deaths or sinus node dysfunction occurred during the follow-up period. The mean gradient of the reconstructed SVC and/or the right pulmonary vein (PV) increased in eight patients and decreased to less than 1 mmHg in six patients. One (1) adult patient had already undergone two vascular interventions, and one child had persistent stenosis but did not undergo reoperation at the time of the study. In addition, two patients were subjected to pericardial windowing. CONCLUSIONS: A modified Warden technique using a right atrial flap and autologous pericardium effectively corrected the high-plane connection between the PVs and SVC. This technique effectively avoids sinus node dysfunction; however, a short-term increase in the differential pressure of the re-established SVC or PV after the procedure is acceptable, and reintervention is required only in rare cases.

Lymphatic magnetic resonance imaging abnormalities in children with repaired tetralogy of Fallot.

INTRODUCTION: Tetralogy of Fallot patients face an elevated risk of developing chylothorax and pleural effusions post-surgery. This patient group exhibits risk factors known to compromise the lymphatic system, such as elevated central venous pressure, pulmonary flow changes, and hypoxia. This study investigates the morphology and function of the lymphatic system in tetralogy of Fallot patients through lymphatic magnetic resonance imaging and near-infrared fluorescence imaging, respectively. METHODS: Post-repair tetralogy of Fallot patients aged 6-18 years were recruited, along with age and gender-matched controls. Magnetic resonance imaging was used to assess the morphology of the thoracic lymphatic vessels and the thoracic, while near-infrared fluorescence imaging was used to assess lymphatic activity utilising lymph rate, velocity, and pressure. RESULTS: Nine patients and 10 controls were included. Echocardiography revealed that 2/3 of the patients had moderate-severe pulmonary regurgitation, while none displayed signs of elevated central venous pressure. Magnetic resonance imaging identified three patients with type 3 (out of 4 types) lymphatic abnormalities, while controls had none. The thoracic ducts showed severe (one patient) and moderate (one patient) tortuosity. Mean thoracic duct diameters were 3.3 mm ±1.1 in patients and 3.0 mm ± 0.8 in controls (p-value = 0.53). Near-infrared fluorescence imaging revealed no anomalous patterns. CONCLUSION: Despite no presence of clinical lymphatic disease, 3/9 of the repaired tetralogy of Fallot patients exhibited lymphatic morphological abnormalities. The significance of these anomalies remains uncertain currently. Further research is needed to determine whether these lymphatic alterations in this patient cohort are a result of congenital malformations, haemodynamic shifts, or prenatal and early-life saturation levels.

Quantitative and functional changes in platelets and fibrinogen following cardiopulmonary by-pass in children.

Introduction: Cardiopulmonary bypass (CPB) causes coagulopathy, increasing the risk of postoperative bleeding and mortality. The underlying causes of post-CPB coagulopathy and the factors associated with its occurrence are not yet fully understood. This study assesses platelet and fibrinogen concentration and function following CPB in children with congenital heart diseases (CHD). Methods: We analyzed prospective data from 104 patients aged 0-16 years who underwent CPB surgery for CHD. Blood samples were collected before surgery and within 30 min of CPB completion. In addition to usual coagulation tests, functional analyses were performed using point of care systems with thromboelastometry and impedance aggregometry. Results: Platelet count, fibrinogen concentration, and platelet and fibrinogen activities significantly decreased after CPB. The duration of CPB was directly associated with a reduction in platelet count and fibrinogen level (r = -0.38, p < 0.001; r = -0.21, p = 0.03, respectively), but not with their measured activity. Postoperative percentages of baseline values for platelet count (58.36% [43.34-74.44] vs. 37.44% [29.81-54.17], p < 0.001) and fibrinogen concentration (73.68% [66.67-82.35] vs. 65.22% [57.89-70.83], p < 0.001) were significantly higher in patients who did not experience hypothermia during surgery. Age was inversely associated with the decrease in platelet count (r = 0.63, p < 0.001), TRAPTEM AUC (r = 0.43, p < 0.001), fibrinogen concentration (r = 0.44, p < 0.001) and FIBTEM MCF (r = 0.57, p < 0.001). Conclusion: Post-CPB coagulopathy is multifactorial and not solely attributed to hemodilution. It also involves functional changes in coagulation cascade components, which can be demonstrated by thromboelastometry and impedance aggregometry. Young children, patients requiring prolonged CPB surgery, or those experiencing hypothermia are particularly affected.

Risk factors and early outcomes associated with prolonged pleural effusion/chylothorax after paediatric cardiac surgery.

OBJECTIVES: Prolonged pleural effusion/chylothorax (PPE/C) is a less investigated complication following paediatric cardiac surgery, and its true incidence, risk factors and impact on postoperative outcomes are not well described. We aim to address these gaps in knowledge using data from a prospective, multicentre study. METHODS: Data on 9 post-operative morbidities (unplanned reinterventions, extracorporeal life support, necrotising enterocolitis, PPE/C, renal replacement therapy, major adverse events, acute neurological events, feeding issues and postsurgical infection) were prospectively collected at 5 UK centres between 2015 and 2017, following paediatric cardiac surgery. Incidence of PPE/C, associations with procedure types, and risk factors were described. Mortality (30-day and 6-month) and hospital length of stay (HLoS) were compared between those with isolated PPE/C, single non-PPE/C morbidity, no morbidity, multimorbidity PPE/C and non-PPE/C multimorbidity. RESULTS: A total of 3090 procedures (2861 patients) were included (median age, 228 days). There were 202 PPE/C (incidence of 6.5%), occurring at a median of 6 days postoperatively (IQR: 3-10). PPE/C was associated with excess early mortality only when complicating scenarios where at least two other post-operative morbidities occurred. On average PPE/C is associated with 8 more HLoS days, but the relative impact is greatest when comparing isolated PPE/C with no morbidity (p < 0.001), whereas in multimorbidity scenarios, PPE/C does not significantly contribute to an increase of HLoS. CONCLUSIONS: Addition of PPE/C increases mortality but not HLoS in multimorbidity and HLoS only in single morbidity scenarios. This reinforces the important role of prevention, early detection and management of PPE/C in complex situations.

Birth in the Operating Room for Immediate Cardiac Surgery: A Rare but Effective Strategy.

Background: With significant advancements in fetal cardiac imaging, patients with complex congenital heart disease (CHD) carrying a high risk for postnatal demise are now being diagnosed earlier. We sought to assess an interdisciplinary strategy for delivering these children in an operating room (OR) adjacent to a cardiac OR for immediate surgery or stabilization. Methods: All children prenatally diagnosed with CHD at risk for immediate postnatal hemodynamic instability and cardiogenic shock who were delivered in the operating room (OR) between 2012 and 2023 in which the senior author was consulted were included. Results: Eight patients were identified. Six (75%) patients were operated on day-of-life zero, all requiring obstructed total anomalous pulmonary venous return (TAPVR) repair. Of these six patients, 2 (33%) required a simultaneous Norwood procedure, 2 (33%) required pulmonary artery unifocalization and modified Blalock-Taussig-Thomas shunt, and 2 (33%) patients had repair of obstructed mixed TAPVR. The remaining 2 patients potentially planned for immediate surgery had nonimmune hydrops fetalis and went into cardiogenic shock at 12 and 72 hours postnatally, requiring a novel Norwood procedure with left-ventricular exclusion for severe aortic/mitral valve insufficiency. The median ventilation and inpatient durations were 19 [IQR: 11-26] days and 41 [IQR: 32-128] days, respectively. Three(38%) patients required one or more in-hospital reoperations. Subsequent staged procedures included Glenn (n = 5), Fontan (n = 3), biventricular repair (n = 2), ventricular assist device placement (n = 1), and heart transplant (n = 1). Median follow-up was 5.7 [IQR:1.3-7.8] years. The five-year postoperative survival was 88% (n = 7/8). Conclusion: While children with these diagnoses have historically had poor survival, the strategy of birth in the OR adjacent to a cardiac OR where emergent surgery is planned is a potentially promising strategy with excellent clinical outcomes. However, this is a high-resource strategy whose feasibility in any program requires thoughtful assessment.

A review of the perioperative management of direct oral anticoagulants for pediatric anesthesiologists.

BACKGROUND: Although direct oral anticoagulants (DOACs) have been used in the adult population for over a decade, DOACs use has begun to rise in pediatric populations since FDA approval of rivaroxaban and dabigatran, DOACs offer several advantages for pediatric patients, to other anticoagulants, including a similar safety profile, minimal lab monitoring, and ease of administration. The rise in DOAC use has led to an increasing number of pediatric patients managed on DOACs presenting for elective and urgent procedures. Perioperative management of anticoagulation is often challenging for providers due to the lack of expert consensus guidelines and the difficulty in balancing a patient's thrombotic risk with bleeding risk for a given procedure. AIMS: Using the most up to date literature, we provide a focused review on the perioperative management of DOACs in pediatric patients. CONCLUSIONS: This work presents a focused review for pediatric anesthesiologists on clinically available DOACs, perioperative monitoring and management of DOACs, as well as options and indications for reversal. While consensus expert practice guidelines are still needed, we hope this work will familiarize perioperative physicians with these agents, recommended uses, and potential perioperative management.

Surgical treatment of anomalous origin of the left pulmonary artery from the descending aorta in a teenager: a case report.

Anomalous origin of one pulmonary artery (AOPA) is a rare congenital heart disease whose symptoms often occur in infancy, and patients have little chance of surviving into adulthood without timely treatments. AOPA is more frequent in infants and toddlers rather than in adults, and it accounts for only 0.12% of all congenital heart disease cases. In all AOPA cases, the right pulmonary artery from the ascending aorta remains common. This study reported a case with anomalous origin of the left pulmonary artery (AOLPA) from the descending aorta in a teenager who underwent double-incision surgery of median sternotomy and left lateral thoracotomies with favorable outcomes.

The Performance of Critical Congenital Heart Disease Screening in Rural Versus Urban Locations in the Northwest United States.

Objectives: Pulse oximetry screening of newborns detects critical congenital heart disease (CCHD). Rural birth location is known to affect timing and management of when infants with CHD undergo surgery, but its association with CCHD screening is unknown. We assess the relationship between rural location and postnatal CCHD diagnosis and describe lesion-specific modes of diagnosis. Study design: Data were abstracted from medical records at 2 cardiac surgery centers in Washington state. Infants with CCHD, defined as CHD requiring either cardiac intervention or resulting in death at <1 month of age, born between July 2015 and June 2020, were included and classified by method of identification. Patient home ZIP codes were used to determine rural location. Results: We included 561 newborns with CCHD; 35% were diagnosed postnatally. Predominant postnatal mechanisms of identification (n = 194) included symptomatic before CCHD screening period (56%), CCHD screening (22%), and symptomatic after false-negative screen (15%). Postnatal diagnosis rate increased with degree of ruralness (48% in small rural/isolated regions vs 32% in urban regions; P = .01) and incidence of undiagnosed CCHD at birth increased with lower nursery levels (5.5/10 000 live births in nursery level 1 vs 2.1/10 000 live births in level 4). Conclusions: CCHD screening identifies 22% of postnatally diagnosed CCHD and 7% of cases overall in our region. Postnatal diagnosis is more common in rural regions. The incidence of undiagnosed CCHD at birth increases with decreasing nursery levels. This study supports the value of CCHD screening in routine newborn care, especially in rural areas and hospitals with lower nursery levels.

Cor triatriatum dexter: an uncommon cause of neonatal cyanosis.

BACKGROUND: Cor triatriatum dexter (CTD) is an extremely rare pathology, with an incidence of < 0.4%. Its main characteristic is a partitioning of the right atrium by the persistence of the embryonic valve of the right sinus venosus. CLINICAL CASE: In this report, we describe the case of a 7-day-old newborn who presented with persistent cyanosis associated with feeding and crying. The diagnosis of CTD was made after an echocardiogram and confirmed using cardiac magnetic resonance imaging. The patient underwent successful surgery on day 14 with a favorable outcome and without complications. CONCLUSION: The importance of our case lies in the identification of rare heart disease as a cause of cyanosis and desaturation in a neonatal patient in the first days of life who did not present signs of heart failure and whose condition improved with supplemental oxygen. We also demonstrate that early diagnosis with echocardiography and surgical resolution resulted in clear clinical improvement and avoided future complications.

INTRODUCCIÓN: El cor triatriatum dexter es una cardiopatía muy rara, caracterizada por la división parcial del atrio derecho en dos cavidades por la persistencia de una membrana que embriológicamente representa la valva derecha del seno venoso. CASO CLÍNICO: En este reporte de caso, presentamos el caso de un neonato en su día 7 de vida que acude a valoración por presentar desaturación persistente con cianosis al llanto. El diagnóstico se realizó con ecocardiograma posterior al cual se decidió la resección quirúrgica de la membrana, procedimiento que fue llevado a cabo el día 14 de vida con éxito sin complicaciones. CONCLUSIONES: La importancia de este caso clínico radica en la identificación de una cardiopatía rara como causa de cianosis y desaturación en un paciente en etapa neonatal, el cual no presentaba datos de compromiso hemodinámico. También se muestra como un diagnóstico y tratamiento quirúrgico oportuno permitieron una resolución de los síntomas sin complicaciones futuras.

Operative and nonoperative outcomes in patients with trisomy 13 and 18 with congenital heart disease.

Objective: To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease. Methods: An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Patients were divided into 2 cohorts (operated vs nonoperated) and compared. Results: Between 1985 and 2023, 62 patients (34 operated and 28 nonoperated) with trisomy 13 (n = 9) and trisomy 18 (n = 53) were identified. The operated cohort was 74% girls, underwent mainly The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures (n = 24 [71%]) at a median age of 2.5 months (interquartile range [IQR], 1.3-4.5 months). This compares with the nonoperative cohort where 64% (n = 18) would have undergone The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures if surgery would have been elected. The most common diagnosis was ventricular septal defect. Postoperative median intensive care unit stay was 6.5 days (IQR, 3.7-15 days) with a total hospital length of stay of 15 days (IQR, 11-49 days). Thirty-day postoperative survival was 94%. There were 5 in-hospital deaths in the operated and 7 in the nonoperated cohort. Median follow-up was 15.4 months (IQR, 4.3-48.7 months) for the operated and 11.2 months (IQR, 1.2-48.3 months) for the nonoperated cohorts. One-year survival was 79% operated versus 51.5% nonoperated (P < .003). Nonoperative treatment had an increased risk of mortality (hazard ratio, 3.28; 95% CI, 1.46-7.4; P = .004). Conclusions: Controversy exists regarding the role of primary cardiac repair in patients with trisomy 13/18 and congenital heart disease. Cardiac repair can be performed safely with low early mortality and operated patients had higher long-term survival compared with nonoperated in our cohort.

Cumulative Radiation Exposure and Lifetime Cancer Risk in Patients With Tetralogy of Fallot Requiring Early Intervention.

Background: Neonates with tetralogy of Fallot and symptomatic cyanosis (sTOF) require early intervention, utilizing either a staged repair (SR) or primary repair (PR) approach. They are exposed to several sources of low-dose ionizing radiation, which may contribute to increased cancer risk. Objectives: The purpose of this study was to compare cumulative radiation exposure and associated lifetime attributable risk (LAR) of cancer between treatment strategies in sTOF. Methods: Neonates with sTOF who underwent SR or PR from 2012 to 2017 were retrospectively reviewed from the Congenital Cardiac Research Collaborative. Radiation exposure from all radiologic studies prior to 18 months of age was converted to organ-equivalent doses and projected LAR of cancer incidence using the National Cancer Institute dosimetry tools. Results: There were 242 neonates from 8 centers, including patients with 146 SR and 96 PR. Cumulative total effective dose was significantly higher for SR (median 8.3 mSv, IQR: 3.0-17.4 mSv) than PR (2.1 mSv, IQR: 0.8-8.5 mSv; P < 0.001). Cumulative organ-level doses were significantly higher in SR compared to PR. Regardless of treatment strategy, LARs were higher in females compared to males. Among organs with median exposure >1 mGy in females, the LAR was highest for breast in SR (mean 1.9/1,000 patients). The highest proportion of cancers attributable to radiation exposure was projected for thyroid cancer in females undergoing SR (7.3%). Conclusions: Cumulative radiation exposure and LARs were higher among those undergoing SR compared to PR. This will be an important factor to consider in determining the preferred neonatal treatment strategy and should substantiate efforts to reduce radiation exposure in this vulnerable population.