Early postoperative weight-based fluid overload is associated with worse outcomes after neonatal cardiac surgery.

OBJECTIVES: Evaluate the association of postoperative day (POD) 2 weight-based fluid balance (FB-W) > 10% with outcomes after neonatal cardiac surgery. METHODS: Retrospective cohort study of 22 hospitals in the NEonatal and Pediatric Heart and Renal Outcomes Network (NEPHRON) registry from September 2015 to January 2018. Of 2240 eligible patients, 997 neonates (cardiopulmonary bypass (CPB) n = 658, non-CPB n = 339) were weighed on POD2 and included. RESULTS: Forty-five percent (n = 444) of patients had FB-W > 10%. Patients with POD2 FB-W > 10% had higher acuity of illness and worse outcomes. Hospital mortality was 2.8% (n = 28) and not independently associated with POD2 FB-W > 10% (OR 1.04; 95% CI 0.29-3.68). POD2 FB-W > 10% was associated with all utilization outcomes, including duration of mechanical ventilation (multiplicative rate of 1.19; 95% CI 1.04-1.36), respiratory support (1.28; 95% CI 1.07-1.54), inotropic support (1.38; 95% CI 1.10-1.73), and postoperative hospital length of stay (LOS 1.15; 95% CI 1.03-1.27). In secondary analyses, POD2 FB-W as a continuous variable demonstrated association with prolonged durations of mechanical ventilation (OR 1.04; 95% CI 1.02-1.06], respiratory support (1.03; 95% CI 1.01-1.05), inotropic support (1.03; 95% CI 1.00-1.05), and postoperative hospital LOS (1.02; 95% CI 1.00-1.04). POD2 intake-output based fluid balance (FB-IO) was not associated with any outcome. CONCLUSIONS: POD2 weight-based fluid balance > 10% occurs frequently after neonatal cardiac surgery and is associated with longer cardiorespiratory support and postoperative hospital LOS. However, POD2 FB-IO was not associated with clinical outcomes. Mitigating early postoperative fluid accumulation may improve outcomes but requires safely weighing neonates in the early postoperative period. A higher resolution version of the Graphical abstract is available as Supplementary information.

A case report of successful percutaneous aspiration, injection, and re-aspiration (PAIR) technique for treatment of retrovesical pelvic hydatid cyst.

Pelvis is a rare location for occurrence of hydatid cyst with only a few cases reported in the literature. All the previous reports of pelvic hydatid cysts were managed with either surgical exploration and excision or laparoscopic intervention. In this case report, we describe the successful treatment of a large pelvic hydatid cyst located in the retrovesical space using the percutaneous aspiration, injection, and respiration (PAIR) technique. To the best of our knowledge, this is the first successful demonstration of the PAIR technique in the treatment of retrovesical pelvic hydatid cyst. Percutaneous treatment of hydatid cyst in this case yielded desirable reduction in the size of the cyst with subsequent involution and relief of the pressure symptoms on the urinary bladder and obviated the need for a surgical procedure.

Cumulative comorbid conditions influence mortality risk after staged palliation for hypoplastic left heart syndrome and variants.

OBJECTIVE: Prematurity, low birth weight, genetic syndromes, extracardiac conditions, and secondary cardiac lesions are considered high-risk conditions associated with mortality after stage 1 palliation. We report the impact of these conditions on outcomes from a prospective multicenter improvement collaborative. METHODS: The National Pediatric Cardiology Quality Improvement Collaborative Phase II registry was queried. Comorbid conditions were categorized and quantified to determine the cumulative burden of high-risk diagnoses on survival to the first birthday. Logistic regression was applied to evaluate factors associated with mortality. RESULTS: Of the 1421 participants, 40% (575) had at least 1 high-risk condition. The aggregate high-risk group had lower survival to the first birthday compared with standard risk (76.2% vs 88.1%, P < .001). Presence of a single high-risk diagnosis was not associated with reduced survival to the first birthday (odds ratio, 0.71; confidence interval, 0.49-1.02, P = .066). Incremental increases in high-risk diagnoses were associated with reduced survival to first birthday (odds ratio, 0.23; confidence interval, 0.15-0.36, P < .001) for 2 and 0.17 (confidence interval, 0.10-0.30, P < .001) for 3 to 5 high-risk diagnoses. Additional analysis that included prestage 1 palliation characteristics and stage 1 palliation perioperative variables identified multiple high-risk diagnoses, poststage 1 palliation extracorporeal membrane oxygenation support (odds ratio, 0.14; confidence interval, 0.10-0.22, P < .001), and cardiac reoperation (odds ratio, 0.66; confidence interval, 0.45-0.98, P = .037) to be associated with reduced survival odds to the first birthday. CONCLUSIONS: The presence of 1 high-risk diagnostic category was not associated with decreased survival at 1 year. Cumulative diagnoses across multiple high-risk diagnostic categories were associated with decreased odds of survival. Further patient accrual is needed to evaluate the impact of specific comorbid conditions within the broader high-risk categories.

Assessment of fluid balance after neonatal cardiac surgery: a description of intake/output vs. weight-based methods.

BACKGROUND: Fluid overload associates with poor outcomes after neonatal cardiac surgery, but consensus does not exist for the most clinically relevant method of measuring fluid balance (FB). While weight change-based FB (FB-W) is standard in neonatal intensive care units, weighing infants after cardiac surgery may be challenging. We aimed to identify characteristics associated with obtaining weights and to understand how intake/output-based FB (FB-IO) and FB-W compare in the early postoperative period in this population. METHODS: Observational retrospective study of 2235 neonates undergoing cardiac surgery from 22 hospitals comprising the NEonatal and Pediatric Heart and Renal Outcomes Network (NEPHRON) database. RESULTS: Forty-five percent (n = 998) of patients were weighed on postoperative day (POD) 2, varying from 2 to 98% among centers. Odds of being weighed were lower for STAT categories 4 and 5 (OR 0.72; 95% CI 0.53-0.98), cardiopulmonary bypass (0.59; 0.42-0.83), delayed sternal closure (0.27; 0.19-0.38), prophylactic peritoneal dialysis use (0.58; 0.34-0.99), and mechanical ventilation on POD 2 (0.23; 0.16-0.33). Correlation between FB-IO and FB-W was weak for every POD 1-6 and within the entire cohort (correlation coefficient 0.15; 95% CI 0.12-0.17). FB-W measured higher than paired FB-IO (mean bias 12.5%; 95% CI 11.6-13.4%) with wide 95% limits of agreement (- 15.4-40.4%). CONCLUSIONS: Weighing neonates early after cardiac surgery is uncommon, with significant practice variation among centers. Patients with increased severity of illness are less likely to be weighed. FB-W and FB-IO have weak correlation, and further study is needed to determine which cumulative FB metric most associates with adverse outcomes. A higher resolution version of the Graphical abstract is available as Supplementary information.

Redefining the Relationship: Palliative Care in Critical Perinatal and Neonatal Cardiac Patients.

Patients with perinatal and neonatal congenital heart disease (CHD) represent a unique population with higher morbidity and mortality compared to other neonatal patient groups. Despite an overall improvement in long-term survival, they often require chronic care of complex medical illnesses after hospital discharge, placing a high burden of responsibility on their families. Emerging literature reflects high levels of depression and anxiety which plague parents, starting as early as the time of prenatal diagnosis. In the current era of the global COVID-19 pandemic, the additive nature of significant stressors for both medical providers and families can have catastrophic consequences on communication and coping. Due to the high prognostic uncertainty of CHD, data suggests that early pediatric palliative care (PC) consultation may improve shared decision-making, communication, and coping, while minimizing unnecessary medical interventions. However, barriers to pediatric PC persist largely due to the perception that PC consultation is indicative of "giving up." This review serves to highlight the evolving landscape of perinatal and neonatal CHD and the need for earlier and longitudinal integration of pediatric PC in order to provide high-quality, interdisciplinary care to patients and families.

Pediatric Palliative Care in the Heart Failure, Ventricular Assist Device and Transplant Populations: Supporting Patients, Families and Their Clinical Teams.

Heart failure is a life-changing diagnosis for a child and their family. Pediatric patients with heart failure experience significant morbidity and frequent hospitalizations, and many require advanced therapies such as mechanical circulatory support and/or heart transplantation. Pediatric palliative care is an integral resource for the care of patients with heart failure along its continuum. This includes support during the grief of a new diagnosis in a child critically ill with decompensated heart failure, discussion of goals of care and the complexities of mechanical circulatory support, the pensive wait for heart transplantation, and symptom management and psychosocial support throughout the journey. In this article, we discuss the scope of pediatric palliative care in the realm of pediatric heart failure, ventricular assist device (VAD) support, and heart transplantation. We review the limited, albeit growing, literature in this field, with an added focus on difficult conversation and decision support surrounding re-transplantation, HF in young adults with congenital heart disease, the possibility of destination therapy VAD, and the grimmest decision of VAD de-activation.

RBC Transfusion Induced ST Segment Variability Following the Norwood Procedure.

The transfusion of stored RBCs decreases nitric oxide bioavailability, which may have an adverse effect on vascular function. We assessed the effects of RBC transfusion on coronary vascular function by evaluating the relationship between myocardial oxygen delivery and demand as evidenced by ST segment variability. DESIGN: Retrospective case-control study. SETTING: Nine-hundred seventy-three-bed pediatric hospital with a 54-bed cardiovascular ICU. PATIENTS: Seventy-three neonates with hypoplastic left heart syndrome following the Norwood procedure, 38 with a Blalock-Taussig shunt and 35 with a right ventricle to pulmonary artery shunt. INTERVENTIONS: RBC transfusion. MATERIALS AND MAIN RESULTS: High-frequency physiologic data were captured 30 minutes prior to the initiation of (baseline) and during the 120 minutes of the transfusion. A rate pressure product was calculated for each subject and used as an indicator of myocardial oxygen demand. Electrocardiogram leads (aVL, V1, II) were used to construct a 3D ST segment vector to assess ST segment variability and functioned as a surrogate indicator of myocardial ischemia. One-hundred thirty-eight transfusions occurred in the Blalock-Taussig shunt group and 139 in the right ventricle to pulmonary artery shunt group. There was no significant change in the rate pressure product for either group; however, ST segment variability progressively increased for the entire cohort during the transfusion, becoming statistically significant by the end of the transfusion. Upon subgroup analysis, this finding was noted with statistical significance in the Blalock-Taussig shunt group and trending toward significance in the right ventricle to pulmonary artery shunt group. CONCLUSIONS: We found a significant increase in the ST segment variability and evidence of myocardial ischemia temporally associated with RBC transfusions in neonates following the Norwood procedure, specifically among those in the Blalock-Taussig shunt group, which may impact immediate and long-term outcomes.

Predicting and Surviving Prolonged Critical Illness After Congenital Heart Surgery.

OBJECTIVES: Prolonged critical illness after congenital heart surgery disproportionately harms patients and the healthcare system, yet much remains unknown. We aimed to define prolonged critical illness, delineate between nonmodifiable and potentially preventable predictors of prolonged critical illness and prolonged critical illness mortality, and understand the interhospital variation in prolonged critical illness. DESIGN: Observational analysis. SETTING: Pediatric Cardiac Critical Care Consortium clinical registry. PATIENTS: All patients, stratified into neonates (≤28 d) and nonneonates (29 d to 18 yr), admitted to the pediatric cardiac ICU after congenital heart surgery at Pediatric Cardiac Critical Care Consortium hospitals. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: There were 2,419 neonates and 10,687 nonneonates from 22 hospitals. The prolonged critical illness cutoff (90th percentile length of stay) was greater than or equal to 35 and greater than or equal to 10 days for neonates and nonneonates, respectively. Cardiac ICU prolonged critical illness mortality was 24% in neonates and 8% in nonneonates (vs 5% and 0.4%, respectively, in nonprolonged critical illness patients). Multivariable logistic regression identified 10 neonatal and 19 nonneonatal prolonged critical illness predictors within strata and eight predictors of mortality. Only mechanical ventilation days and acute renal failure requiring renal replacement therapy predicted prolonged critical illness and prolonged critical illness mortality in both strata. Approximately 40% of the prolonged critical illness predictors were nonmodifiable (preoperative/patient and operative factors), whereas only one of eight prolonged critical illness mortality predictors was nonmodifiable. The remainders were potentially preventable (postoperative critical care delivery variables and complications). Case-mix-adjusted prolonged critical illness rates were compared across hospitals; six hospitals each had lower- and higher-than-expected prolonged critical illness frequency. CONCLUSIONS: Although many prolonged critical illness predictors are nonmodifiable, we identified several predictors to target for improvement. Furthermore, we observed that complications and prolonged critical care therapy drive prolonged critical illness mortality. Wide variation of prolonged critical illness frequency suggests that identifying practices at hospitals with lower-than-expected prolonged critical illness could lead to broader quality improvement initiatives.

Diagnostic errors in paediatric cardiac intensive care.

IntroductionDiagnostic errors cause significant patient harm and increase costs. Data characterising such errors in the paediatric cardiac intensive care population are limited. We sought to understand the perceived frequency and types of diagnostic errors in the paediatric cardiac ICU. METHODS: Paediatric cardiac ICU practitioners including attending and trainee physicians, nurse practitioners, physician assistants, and registered nurses at three North American tertiary cardiac centres were surveyed between October 2014 and January 2015. RESULTS: The response rate was 46% (N=200). Most respondents (81%) perceived that diagnostic errors harm patients more than five times per year. More than half (65%) reported that errors permanently harm patients, and up to 18% perceived that diagnostic errors contributed to death or severe permanent harm more than five times per year. Medication side effects and psychiatric conditions were thought to be most commonly misdiagnosed. Physician groups also ranked pulmonary overcirculation and viral illness to be commonly misdiagnosed as bacterial illness. Inadequate care coordination, data assessment, and high clinician workload were cited as contributory factors. Delayed diagnostic studies and interventions related to the severity of the patient's condition were thought to be the most commonly reported process breakdowns. All surveyed groups ranked improving teamwork and feedback pathways as strategies to explore for preventing future diagnostic errors. CONCLUSIONS: Paediatric cardiac intensive care practitioners perceive that diagnostic errors causing permanent harm are common and associated more with systematic and process breakdowns than with cognitive limitations.

A randomised trial of early palliative care for maternal stress in infants prenatally diagnosed with single-ventricle heart disease.

Children with single-ventricle disease experience high mortality and complex care. In other life-limiting childhood illnesses, paediatric palliative care may mitigate maternal stress. We hypothesised that early palliative care in the single-ventricle population may have the same benefit for mothers. In this pilot randomised trial of early palliative care, mothers of infants with prenatal single-ventricle diagnoses completed surveys measuring depression, anxiety, coping, and quality of life at a prenatal visit and neonatal discharge. Infants were randomised to receive early palliative care - structured evaluation, psychosocial/spiritual, and communication support before surgery - or standard care. Among 56 eligible mothers, 40 enrolled and completed baseline surveys; 38 neonates were randomised, 18 early palliative care and 20 standard care; and 34 postnatal surveys were completed. Baseline Beck Depression Inventory-II and State-Trait Anxiety Index scores exceeded normal pregnant sample scores (mean 13.76±8.46 versus 7.0±5.0 and 46.34±12.59 versus 29.8±6.35, respectively; p=0.0001); there were no significant differences between study groups. The early palliative care group had a decrease in prenatal to postnatal State-Trait Anxiety Index scores (-7.6 versus 0.3 in standard care, p=0.02), higher postnatal Brief Cope Inventory positive reframing scores (p=0.03), and a positive change in PedsQL Family Impact Module communication and family relationships scores (effect size 0.46 and 0.41, respectively). In conclusion, these data show that mothers of infants with single-ventricle disease experience significant depression and anxiety prenatally. Early palliative care resulted in decreased maternal anxiety, improved maternal positive reframing, and improved communication and family relationships.

Outcomes of infants weighing three kilograms or less requiring extracorporeal membrane oxygenation after cardiac surgery.

BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is the most common form of cardiac support for postcardiotomy cardiac failure in children. While its benefits have been thoroughly explored in infants weighing more than 3 kg, similar analyses are lacking in lower weight neonates. This single-center study investigated outcomes and risks for poor survival among infants weighing 3 kg or less. METHODS: A retrospective review of infants 3 kg or less who required postcardiotomy ECMO support was performed (January 1, 1999 to December 31, 2010). Primary outcome was 30-day survival after decannulation. Factors analyzed for association with poor outcome included demographics, cardiac anatomy, bypass and circulatory arrest times, total ECMO support time, postoperative lactate, inotrope use, and need for renal replacement therapy. RESULTS: During the study period, 64 patients weighing 3 kg or less required postcardiotomy ECMO. Median gestational age and age at ECMO initiation were 38 weeks (interquartile range, 36 to 39) and 7 days (interquartile range, 4 to 9), respectively, with median ECMO support time of 164 hours (interquartile range, 95 to 231). Overall 30-day survival after decannulation was 33%. Factors associated with poor outcome were longer duration of support (231 hours or more, 12% survival, versus less than 231 hours, 40% survival; p = 0.05) and renal replacement therapy (n = 36, survival 17% versus 54%; p = 0.002). Multivariable regression analysis identified renal replacement therapy as the only independent factor associated with poor survival (odds ratio 4.3, 95% confidence interval: 1.3 to 14.9, p = 0.02). CONCLUSIONS: For infants weighing 3 kg or less, 30-day survival after decannulation after cardiac ECMO is poor. Factors associated with poor prognosis were need for renal replacement therapy and longer duration of ECMO support. These findings may provide a useful guide for medical decision making among this unique, high-risk patient group.

Mumbai stroke registry (2005-2006)--surveillance using WHO steps stroke instrument--challenges and opportunities.

BACKGROUND: India will face enormous socioeconomic burden because life expectancy is increasing placing larger numbers of older people at risk of stroke and other chronic diseases. In order to plan prevention strategies, reliable information on stroke epidemiology is required. For uniform data collection (population based), WHO recommends use of STEPS Stroke instrument. STUDY: A well-defined community (H-ward) with verifiable census data, and representative of population structure of Mumbai (Bombay), was selected. The manual on WHO STEPwise approach to stroke surveillance (STEPS; http://www.who.int/chp/steps/Manual.pdf) was the operational protocol. RESULTS: During the two year study period (Jan 2005 to Dec 2006), 521 new stroke (CVD) cases (males--275 and females--246) were identified; of which 456 (238 males and 218 females) had "first ever stroke"(FES) indicating an annual incidence of 145 per 100,000 persons (CI 95%: 120-170); age adjusted Segi rate: 152/100,000/year (CI 95% 132-172). Two thirds of the FES cases were admitted to health care facilities (Step I: "in-hospital" cases), the remaining 150 (32.8%) either died outside of hospital or were treated at home or nursing homes (Step II: Fatal events in community and Step III: Non-fatal events in community). CVD Diagnosis was supported by CT (Computed Tomography) in 407 (89%) of 456 FES cases: 366 (80.2%) had Ischaemic CVD, 81 (17.7%) had hemorrhagic CVD and 9 (1%) were of unspecified category. The mean age was 66 yrs SD +/- 13.60 and women were older compared with men (mean age 68.9 yrs SD +/- 13.12 versus 63.4 yrs SD +/- 13.53). Hypertension (BP more than 140/90 mm Hg) alone or in various combinations was present in 378 ( 82.8%) cases. Case fatality at 28 days after the FES stroke was 29.8%. Of 320 surviving patients 38.5% had moderate to severe disability. CONCLUSIONS: WHO STEPs stroke surveillance Instrument is simple to use and, practical for community surveys. The data are useful for planning stroke prevention campaigns on public awareness and education with regard to diet, exercise, blood pressure control and early symptoms of minor strokes.

Antithrombotic agents in cerebral ischaemia.

The current evidence suggests that aspirin is treatment of choice when compared to anticoagulants for patients with non-cardioembolic stroke. The usefulness of combination therapy (aspirin vs. with or without warfarin) is still debated. Likewise the combination of Aspirin with clopidogrel has no added advantage (MATCH Trial). However anticoagulant therapy significantly benefits high-risk patients with atrial fibrillation in the elderly subjects whereas aspirin may still be the drug of choice in stroke prevention in low risk group in the younger age. There is dire need for well planned randomized double blind controlled studies to define the role of Antithrombotic agents in "cryptogenic stroke" (PFO/ASD related) antiphospholipid antibody syndrome, arterial dissections and intraluminal clot syndromes. Evaluation and treatment of associated risk factors in all categories needs greater emphasis.

Structural and functional characteristics of lung macro- and microvascular endothelial cell phenotypes.

Lung macro- and microvascular endothelial cells exhibit unique functional attributes, including signal transduction and barrier properties. We therefore sought to identify structural and functional features of endothelial cells that discriminate their phenotypes in the fully differentiated lung. Rat lung macro- (PAEC) and microvascular (PMVEC) endothelial cells each exhibited expression of typical markers. Screening for reactivity with nine different lectins revealed that Glycine max and Griffonia (Bandeiraea) simplicifolia preferentially bound microvascular endothelia whereas Helix pomatia preferentially bound macrovascular endothelia. Apposition between the apical plasmalemma and endoplasmic reticulum was closer in PAECs (8 nm) than in PMVECs (87 nm), implicating this coupling distance in the larger store operated calcium entry responses observed in macrovascular cells. PMVECs exhibited a faster growth rate than did PAECs and, once a growth program was initiated by serum, PMVECs sustained growth in the absence of serum. Thus, PAECs and PMVECs differ in their structure and function, even under similar environmental conditions.