Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus.

OBJECTIVE: To test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were as follows: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. RESULTS: The study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI: 1.07,5.27; P = .034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). CONCLUSIONS: A minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.

Right atrial thrombus removal with use of the ONO retrieval device.

Intracardiac masses and specifically right atrial thrombi can be difficult to manage and carry a high mortality rate. Typically, surgical removal or mechanical thrombectomy can be performed though may not be suitable for all patients. We present a unique case of a sickle cell patient with a large pedunculated right atrial thrombus that was successfully extracted using the novel ONO Retrieval Device.

The Need for Surgery After Vascular or Cardiac Trauma, or Technical Adverse Events in the Congenital Cardiac Catheterization Laboratory.

Data on the frequency and outcome of surgical interventions as a result of adverse events (AE) encountered in the pediatric and congenital cardiac catheterization laboratory are limited. This study analyzes the outcomes of specific types of AE that are most likely to require immediate surgical intervention. Data from the C3PO registry were analyzed to identify specific types of significant vascular/cardiac trauma or technical adverse events (stent/device/coil embolization/migration). The relationship between these AE and an "adverse outcome" (defined as either surgery, ECMO, or death) were analyzed. Between 01/2014 and 12/2017, 25,731 cases were entered into the C3PO registry. Vascular or cardiac trauma were observed in 92 cases (0.36% cases in C3PO), and technical adverse events were observed in 176 cases (0.68% cases in C3PO). The two highest procedure type risk categories (PREDIC3T) accounted for 61% of the cases in the cardiac/vascular trauma cohort, and 34% in the technical AE cohort. For vascular/cardiac trauma, 24 (26%) had an adverse outcome, with ECMO in 8 (9%), surgery in 19 (20%), and death in 9 (10%). For technical AE 25 (14%) had an adverse outcome, with ECMO in 3 (2%), surgery in 23 (13%), and death in 3 (2%). Survival after cardiac surgery secondary to an AE was 68% for cardiac/vascular trauma, and 96% for technical adverse events. RF perforation of the pulmonary valve was the procedure most likely to result in cardiac/vascular trauma (10%), with 57% of those having an adverse outcome. Atrial septal interventions accounted for 29% of all adverse outcomes in the cardiac/vascular trauma cohort. Non-elective or emergent cases were associated with a significantly higher incidence of an adverse outcome for both, cardiac/vascular trauma (OR 7.1) and technical adverse events (OR 2.7). Surgery within the last 30 days was associated with a significantly higher incidence of an adverse outcome for cardiac/vascular trauma only (OR 4.2). Significant cardiac/vascular trauma or stent/device/coil embolization/migration are rare, but high consequence AE. With appropriate surgical and ECMO backup, a high survival can be achieved. The potential need for and impact of immediate surgical backup seems to be higher for cardiac/vascular trauma (in particular after specific case types), than for device/coil migration/embolization, and as such case specific backup arrangements are required.

Spontaneous neonatal pulmonary arterial thrombosis - cases, mechanisms, and literature review.

Spontaneous pulmonary artery thrombosis in neonates is rare and can be life-threatening. Clinical presentation may mimic pulmonary hypertension or CHD. Further, not all children present with identifiable risk factors. We report the case of two infants with pulmonary artery thromboses who underwent rapid diagnosis and therapy, one with percutaneous intervention and the other with anticoagulation. We also conducted a literature review to highlight the importance of early identification and referral to a centre capable of performing appropriate medical and interventional therapies.

Neurocognitive function and health-related quality of life in adolescents and young adults with CHD with pulmonary valve dysfunction.

BACKGROUND: Neurocognitive impairment and quality of life are two important long-term challenges for patients with complex CHD. The impact of re-interventions during adolescence and young adulthood on neurocognition and quality of life is not well understood. METHODS: In this prospective longitudinal multi-institutional study, patients 13-30 years old with severe CHD referred for surgical or transcatheter pulmonary valve replacement were enrolled. Clinical characteristics were collected, and executive function and quality of life were assessed prior to the planned pulmonary re-intervention. These results were compared to normative data and were compared between treatment strategies. RESULTS: Among 68 patients enrolled from 2016 to 2020, a nearly equal proportion were referred for surgical and transcatheter pulmonary valve replacement (53% versus 47%). Tetralogy of Fallot was the most common diagnosis (59%) and pulmonary re-intervention indications included stenosis (25%), insufficiency (40%), and mixed disease (35%). There were no substantial differences between patients referred for surgical and transcatheter therapy. Executive functioning deficits were evident in 19-31% of patients and quality of life was universally lower compared to normative sample data. However, measures of executive function and quality of life did not differ between the surgical and transcatheter patients. CONCLUSION: In this patient group, impairments in neurocognitive function and quality of life are common and can be significant. Given similar baseline characteristics, comparing changes in neurocognitive outcomes and quality of life after surgical versus transcatheter pulmonary valve replacement will offer unique insights into how treatment approaches impact these important long-term patient outcomes.

Impact of Management Strategy on Feeding and Somatic Growth in Neonates with Symptomatic Tetralogy of Fallot: Results from the Congenital Cardiac Research Collaborative.

OBJECTIVE: To evaluate early growth following primary or staged repair of neonatal symptomatic tetralogy of Fallot (sTOF). STUDY DESIGN: We performed a retrospective, multicenter cohort study of consecutive infants with sTOF who underwent initial intervention at age ≤30 days, from 2005 to 2017. Management strategies were either primary repair or staged repair (ie, initial palliation followed by complete repair). The primary outcome was change in weight-for-age z-score (ΔWAZ) from the initial intervention to age 6 ± 2 months. Secondary outcomes included method and mode of feeding, feeding-related medications, and feeding-related readmissions. Propensity score adjustment was used to account for baseline differences between groups. A secondary analysis was performed comparing patients stratified by the presence of adequate growth (6-month ΔWAZ > -0.5) or inadequate growth (6-month ΔWAZ ≤ -0.5), independent of treatment strategy. RESULTS: The study cohort included 143 primary repair subjects and 240 staged repair subjects. Prematurity was more common in the staged repair group. After adjustment, median ΔWAZ did not differ between treatment groups over the first 6 months of life (primary: -0.43 [IQR, -1.17 to 0.50]; staged: -0.31 [IQR, -1.31 to 0.71]; P = .55). For the entire cohort, ΔWAZ was negative (-0.36; IQR, -1.21 to 0.63). There were no between-group differences in the secondary outcomes. Secondary analysis revealed that the subjects with adequate growth were more likely to be orally fed at initial hospital discharge (P = .04). CONCLUSIONS: In neonates with sTOF, growth trajectory over the first 6 months of life was substandard, irrespective of treatment strategy. Those patients with adequate growth were more likely to be discharged from the index procedure on oral feeds.

Transcatheter pulmonary valve-in-valve implantation within the expandable Inspiris Resilia® bioprosthetic valve.

Transcatheter pulmonary valve implantation (TPVI) is a common intervention for patients with repaired congenital heart disease. A key issue relates to the presence of an appropriately sized implantation zone for the transcatheter valve. We report the first case, to our knowledge, of TPVI within the newly engineered Inspiris Resilia® bioprosthetic valve, intentionally designed with a balloon-expandable valve ring (a.k.a. VFit zone) for prospective valve implantation.

An unexpected circular shunt: Novel method to treat semilunar valve insufficiency in a single ventricle patient on mechanical circulatory support.

Severe semilunar valve insufficiency in single ventricle patients supported with mechanical circulatory support while awaiting transplant remains a complex clinical scenario with few favorable options for management. We present the first case, to our knowledge, of transcatheter closure of the pulmonic valve in a patient palliated with a hybrid stage 1 procedure for hypoplastic left heart syndrome.

Short- and medium-term outcomes for patent ductus arteriosus stenting in neonates ≤2.5 kg with duct-dependent pulmonary circulation.

BACKGROUND: Morbidity with surgical systemic-to-pulmonary artery shunting (SPS) in infants ≤2.5 kg has remained high. Patent ductus arteriosus (PDA) stenting may be a valid alternative. The objective of this study is to evaluate outcomes following PDA stenting in patients ≤2.5 kg from four large tertiary centers. METHODS: Retrospective review of all neonates ≤2.5 kg with duct-dependent pulmonary circulation who underwent PDA stenting. Procedural details, pulmonary arterial growth, reinterventions, surgery type, and outcomes were assessed. RESULTS: PDA stents were implanted in 37 of 38 patients attempted (18 female) at a median procedural weight of 2.2 kg (interquartile range [IQR], 2-2.4 kg). Seven patients (18%) had a genetic abnormality and 16 (42%) had associated comorbidities. The median intensive care unit stay was 4 days (IQR, 2-6.75 days), and the median hospital stay was 20 days (IQR, 16-57.25). One patient required a rescue shunt procedure, with three others requiring early SPS (<30 days postprocedure). Twenty patients (54%) required reintervention with either balloon angioplasty, restenting, or both. At 6-month follow-up, right pulmonary artery growth (median z-score -1.16 to 0.01, p = 0.05) was greater than the left pulmonary artery (median z-score -0.9 to -0.64, p = 0.35). Serious adverse effects (SAEs) were seen in 18% (N = 7) of our cohort. One patient developed an SAE during planned reintervention There were no intraprocedural deaths, with one early procedure-related mortality, and three interstage mortalities not directly related to PDA stenting. CONCLUSIONS: PDA stenting in infants ≤2.5 kg is feasible and effective, promoting pulmonary artery growth. Reintervention rates are relatively high, though many are planned to allow for optimal growth before a definitive operation.

Measurement of Cardiac Output Using an Ultrasonic Cardiac Output Monitor (USCOM) in Patients with Single-Ventricle Physiology.

We evaluate the validity of cardiac index (CI) measurements utilizing the Ultrasonic Cardiac Output Monitor (USCOM), a non-invasive Doppler ultrasound device, by comparing measurements to cardiac catheterization-derived CI measurements in patients with single-ventricle physiology. USCOM measurements were repeated three times for each patient at the beginning of a cardiac catheterization procedure for twenty-six patients undergoing elective pre-Glenn or pre-Fontan catheterization. CI was measured by USCOM and was calculated from cardiac catheterization data using Fick's method. Bland-Altman analysis for CI showed bias of 0.95 L/min/m2 with the 95% limits of agreement of - 1.85 and 3.75. Pearson's correlation coefficient was 0.89 (p < 0.001) indicating a strong positive relationship between USCOM and cardiac catheterization CI measurements. When excluding two patients with significant dilation of the neo-aortic valve (z-score > + 5), the bias improved to 0.66 L/min/m2 with the 95% limits of agreement of - 1.38 and 2.70. Percent error of limits of agreement was 34%. There was excellent intra-operator reproducibility of USCOM CI measurements with an intra-class coefficient of 0.96. We demonstrate the use of USCOM to measure CI in patients with single-ventricle physiology for the first time, showing acceptable agreement of the CI measurements between USCOM and cardiac catheterization with a high intra-operator reproducibility.

Palliation Strategy to Achieve Complete Repair in Symptomatic Neonates with Tetralogy of Fallot.

Neonates with symptomatic tetralogy of Fallot (sTOF) may undergo palliations with varying physiology, namely systemic to pulmonary artery connections (SPC) or right ventricular outflow tract interventions (RVOTI). A comparison of palliative strategies based on the physiology created is lacking. Consecutive sTOF neonates undergoing SPC or RVOTI from 2005-2017 were reviewed from the Congenital Cardiac Research Collaborative. The primary outcome was survival with successful complete repair (CR) by 18 months. A variety of secondary outcomes were assessed including overall survival, hospitalization-related comorbidities, and interstage reinterventions. Propensity score adjustment was utilized to compare treatment strategies. The cohort included 252 SPC (surgical shunt = 226, ductus arteriosus stent = 26) and 68 RVOTI (balloon pulmonary valvuloplasty = 48, RVOT stent = 11, RVOT patch = 9) patients. Genetic syndrome (29 [42.6%] v 75 [29.8%], p = 0.04), weight < 2.5 kg (28 [41.2%] v 68 [27.0%], p = 0.023), bilateral pulmonary artery Z-score < - 2 (19 [28.0%] v 36 [14.3%], p = 0.008), and pre-intervention antegrade flow (48 [70.6%] v 104 [41.3%], p < 0.001) were more common in RVOTI. Significant center differences were noted (p < 0.001). Adjusted survival to CR by 18 months (HR = 0.87, 95% CI = 0.63-1.21, p = 0.41) and overall survival (HR = 2.08, 95% CI = 0.93-4.65, p = 0.074) were similar. RVOTI had increased interstage reintervention (HR = 2.15, 95% CI = 1.36-3.99, p = 0.001). Total anesthesia (243 [213, 277] v 328 [308, 351] minutes, p < 0.001) and cardiopulmonary bypass times (117 [103, 132] v 151 [143, 160] minutes, p < 0.001) favored RVOTI. In this multicenter comparison of physiologic palliation strategies for sTOF, survival to successful CR and overall survival were similar; however, reintervention burden was significantly higher in RVOTI.

Paediatrician's guide to post-operative care for functionally univentricular CHD: a review.

IMPORTANCE: Single ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care. OBSERVATIONS: We reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician's approach to single ventricle patients at different stages of surgical palliation. CONCLUSIONS AND RELEVANCE: Single ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern.

Paediatrician's guide to post-operative care for biventricular CHD: a review.

IMPORTANCE: Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients. OBSERVATIONS: The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation. CONCLUSIONS AND RELEVANCE: Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients.

Balloon expandable covered stents as primary therapy for hemodynamically stable traumatic aortic injuries in children.

OBJECTIVES: To expand on the limited available literature regarding the use of balloon expandable covered stents for the treatment of traumatic aortic injuries (TAI) in the pediatric population. BACKGROUND: Although endovascular grafts have largely replaced surgery for TAI repair, there are significant limitations to the use of these grafts in pediatric patients. METHODS: Multicenter, retrospective chart review of pediatric patients with TAI following blunt chest wall trauma. Procedural characteristics, follow-up, and reinterventions are described. RESULTS: Six covered stents implanted in five patients. Median patient age was 12 years (11-13 years) and median weight 50 kg (44-54 kg). Procedural success was achieved in all cases. No procedural or postprocedural complications were noted. Median follow-up time was 24 months (11-36 months). CONCLUSIONS: Balloon expandable covered stent treatment of pediatric patients with TAI is a feasible alternative to open surgical repair, and preferred over endovascular grafts due to graft size limitations and the large delivery systems.

Pulsus alternans in a child with dilated cardiomyopathy.

A previously healthy 21-month-old presented with new-onset dilated cardiomyopathy. Evaluation noted pulsus alternans, with beat-to-beat alternations in aortic pulse wave amplitude, both clinically and on diagnostic studies. Pulsus alternans is an infrequent, complex pathophysiologic sign often associated with severe heart failure. The mechanisms are incompletely understood, but theorised aetiologies include beat-to-beat changes in left ventricular loading conditions, variations in myocardial oxygen supply/demand, and alternations in myocardial contractility. Recognition of pulsus alternans is important as it provides significant clinical information, may suggest suboptimal medical management, and may be the first warning sign of severe cardiac dysfunction.

Concomitant percutaneous treatment of aortic coarctation and associated intercostal aneurysms: pre-procedural recognition is key.

Intercostal aneurysms are associated with aortic coarctation. Their aetiology is not well-understood but may be related to intrinsic vascular pathology and altered flow dynamics through the intercostal artery. We present the cases of two patients with coarctation and intercostal aneurysms. The aneurysms were recognised on pre-catheterisation imaging studies and were selectively occluded during the same procedure to treat the coarctation. There were no complications; both the patients have no residual coarctation at the most recent follow-up. Intercostal aneurysms associated with coarctation can have significant consequences including late rupture, paralysis, and even death. These aneurysms are common with an incidence of up to 40% with adult-diagnosed coarctation; one treatment plan is to treat both the coarctation and aneurysm during a single catheterisation. Pre-catheterisation CT or MRI may play a role in this strategy.

Quality metrics in cardiac catheterization for congenital heart disease: utility of 30-day mortality.

OBJECTIVES: To characterize the frequency and attributability of death among patients who died within 30 days of their cardiac catheterization (30-day mortality). BACKGROUND: 30-day postprocedure mortality is commonly used as a quality outcome metric in national cardiac catheterization registries. It is unclear if this parameter is sufficiently specific to meaningfully capture mortality attributable to cardiac catheterization in patients with congenital heart disease (CHD). METHODS: Multicenter cohort study with 3 participating centers. Records were retrospectively reviewed for patients who died within 30 days of catheterization (06/2007-06/2012). Attributability of death was assigned to each case. RESULTS: A total of 14,707 cardiac catheterization procedures were performed during the study period. Death occurred within 30 days in 279/14,707 (1.9%) of cases. Among the patients who died, 53% of cases were emergent or urgent cases. The median age was 4 mos (1 day-45 years). Death was attributable to the catheterization procedure in 29/279 (10%) of cases. Death was attributable to cardiac surgery in 14%, precatheterization clinical status in 34%, postcatheterization clinical status in 22%, and noncardiac comorbidity in 19%. In 1%, death attributability could not be established. CONCLUSIONS: While valuable in adult settings, 30-day mortality is inadequate as a quality metric among patients with CHD undergoing cardiac catheterization. To derive the optimal benefit from catheterization registry data, more robust methodologies to capture procedure-related mortality are needed. © 2014 Wiley Periodicals, Inc.

The Summer Food Service Program and the Ongoing Hunger Crisis in Mississippi.

Food insecurity is simply defined as uncertain access to adequate food. Nearly 50 million Americans, 16 million of whom are children, are food insecure. Mississippi has 21% food insecure citizens, and has the most food insecure county in the nation. Our state's school system's National Breakfast and Lunch Programs help combat food insecurity, but a gap still exists. This gap widens during the summer. In this paper, we describe the Mississippi Summer Food Service Program. While the program has had success in our state, it still faces challenges. Organized action by physicians in Mississippi and the Mississippi State Medical Association could significantly increase participation in these programs that are vital to our state.

Ductal spasm during performance of transcatheter ductal occlusion.

OBJECTIVES: Transcatheter patent ductus arteriosus (PDA) occlusion is a staple of pediatric catheterization laboratories. We present the phenomenon of significant PDA spasm to prevent failure to occlude a hemodynamically significant duct. BACKGROUND: Transcatheter techniques have evolved, allowing safe and effective occlusion of PDAs in younger and smaller patients. Neonatal care is evolving with increasing survival at younger gestational ages. Premature infants often have PDAs, so the proportion of formerly premature children referred for transcatheter ductal occlusion will likely rise. METHODS: We reviewed all transcatheter PDA occlusions performed at our institution since 2001 (N = 331). Retrospective data included: gestational age, age at catheterization, precatheterization echocardiographic parameters, PDA size (after spasm relief), device specifications, and most recent follow-up data. RESULTS: Seven cases were identified. Median age was 12 months, median gestational age was 28 weeks. All were born prematurely. All PDAs were restrictive and six had left-heart volume overload. All patients were examined by the interventional cardiologist and had ductal murmurs. When reauscultated (three of seven), murmurs were absent during spasm. Once spasm relieved, PDA diameters ranged from 1.5 to 8 (median 2) mm. All patients accommodated a 6-mm-or larger-Amplatzer device. No significant complications occurred and all patients were well at follow-up. CONCLUSIONS: Ductal spasm occurs during transcatheter occlusion and may be an unrecognized cause of procedural failure. The phenomenon seems to occur in children born prematurely, and can occur after infancy. Loss of a continuous murmur confirms the diagnosis. Care should be taken to avoid device under-sizing when spasm occurs.

Catheterization for Congenital Heart Disease Adjustment for Risk Method II.

BACKGROUND: Current metrics used to adjust for case mix complexity in congenital cardiac catheterization are becoming outdated due to the introduction of novel procedures, innovative technologies, and expanding patient subgroups. This study aims to develop a risk adjustment methodology introducing a novel, clinically meaningful adverse event outcome and incorporating a modern understanding of risk. METHODS: Data from diagnostic only and interventional cases with defined case types were collected for patients ≤18 years of age and ≥2.5 kg at all Congenital Cardiac Catheterization Project on Outcomes participating centers. The derivation data set consisted of cases performed from 2014 to 2017, and the validation data set consisted of cases performed from 2019 to 2020. Severity level 3 adverse events were stratified into 3 tiers by clinical impact (3a/b/c); the study outcome was clinically meaningful adverse events, severity level ≥3b (3bc/4/5). RESULTS: The derivation data set contained 15 224 cases, and the validation data set included 9462 cases. Clinically meaningful adverse event rates were 4.5% and 4.2% in the derivation and validation cohorts, respectively. The final risk adjustment model included age <30 days, Procedural Risk in Congenital Cardiac Catheterization risk category, and hemodynamic vulnerability score (C statistic, 0.70; Hosmer-Lemeshow P value, 0.83; Brier score, 0.042). CONCLUSIONS: CHARM II (Congenital Heart Disease Adjustment for Risk Method II) risk adjustment methodology allows for equitable comparison of clinically meaningful adverse events among institutions and operators with varying patient populations and case mix complexity performing pediatric cardiac catheterization.