Ray Lowery

Hyperoxia Is Associated With Poor Outcomes in Pediatric Cardiac Patients Supported on Venoarterial Extracorporeal Membrane Oxygenation.

Objectives: Patients who require venoarterial extracorporeal membrane oxygenation because of cardiac failure frequently have supranormal blood oxygen tensions (hyperoxia). Recent studies have suggested worse outcomes in patients with hyperoxia after resuscitation from cardiac or respiratory arrests, presumably because of oxidative stress. There are limited data regarding the effect of hyperoxia on outcomes in pediatric patients on venoarterial extracorporeal membrane oxygenation. Design: Retrospective chart review. Setting: Pediatric cardiothoracic ICU. Patients: Cardiac surgery patients less than 1 year old requiring venoarterial extracorporeal membrane oxygenation in the postoperative period from 2007 to 2013. Measurements and Main Results: In 93 infants (median time on extracorporeal membrane oxygenation, 5 d), mortality at 30 days post surgery (primary outcome) was 38%. Using a receiver operating characteristic curve, a mean PaO2 of 193 mm Hg in the first 48 hours of extracorporeal membrane oxygenation was determined to have good discriminatory ability with regard to 30-day mortality. Univariate analysis identified a mean PaO2 greater than 193 mm Hg (p = 0.001), longer cardiopulmonary bypass times (p = 0.09), longer duration of extracorporeal membrane oxygenation (p < 0.0001), and higher extracorporeal membrane oxygenation pump flows (p = 0.052) as possible risk factors for 30-day mortality. In multivariable analysis controlling for the variables listed above, a mean PaO2 greater than 193 mm Hg remained an independent risk factor for mortality (p = 0.03). In addition, a mean PaO2 greater than 193 mm Hg was associated with the need for renal dialysis (p = 0.02) but not with neurologic injury (p = 0.41) during the hospitalization. Conclusions: In infants with congenital heart disease who are placed on venoarterial extracorporeal membrane oxygenation postoperatively, hyperoxia (defined as a mean PaO2 > 193 mm Hg in the first 48 hr of extracorporeal membrane oxygenation) was an independent risk factor for 30-day mortality after surgery. Future studies are needed to delineate the causative or associative role of hyperoxia with outcomes, especially in children with baseline cyanosis who may be more susceptible to the effects of oxidative stress.

Twenty years of experience with intraoperative pulmonary artery stenting

To describe our 20‐year experience with intraoperative pulmonary artery (PA) stent placement and evaluate long‐term patient outcomes, specifically the need and risk factors for reintervention.

Functional Status and Quality of Life in Survivors of Extracorporeal Membrane Oxygenation After the Norwood Operation

BACKGROUND Infants who require extracorporeal membrane oxygenation (ECMO) support after a Norwood operation are at increased risk for early and late death compared with patients who do not require ECMO post-Norwood. Little is known about the effect that ECMO post-Norwood has on functional status and quality of life among long-term survivors. METHODS We prospectively evaluated functional status and health-related quality of life in 12 surviving patients (cases) and 19 corresponding patients (controls) from a previous retrospective case-control assessment of long-term survival in patients requiring ECMO post-Norwood. Functional status was assessed with the Vineland Adaptive Behavior Scale-II, and health-related quality of life was assessed with the Pediatric Quality of Life Inventory (PedsQL) core and cardiac modules. RESULTS There were no differences in demographics, extracardiac or genetic anomalies, or age at follow-up assessment between ECMO cases and non-ECMO controls. The Vineland Adaptive Behavior Scale-II scores were comparable between groups, with both groups demonstrating function in the normal range in all four domains tested. The only difference in PedsQL scores between cases and controls was perceived physical appearance, which was lower among ECMO survivors by both patient and proxy report. PedsQL scores of both groups were comparable to published scores for patients with single-ventricle congenital heart disease but generally lower than scores for the healthy population. CONCLUSIONS The requirement for ECMO support after a Norwood operation does not appear to significantly affect functional status or quality of life among the subset of patients who achieve long-term survival.

IMPACT OF EARLY PALLIATIVE CARE INTERVENTION ON MATERNAL STRESS IN MOTHERS OF INFANTS PRENATALLY DIAGNOSED WITH SINGLE VENTRICLE HEART DISEASE: A RANDOMIZED CLINICAL TRIAL

Children with single ventricle (SV) cardiac defects requiring staged palliation have a high risk of mortality and receive invasive and complex care, resulting in significant maternal stress. In other complex, life-limiting illnesses among children, pediatric palliative care (PPC) may mitigate

Single Ventricle and Total Anomalous Pulmonary Venous Connection: Implications of Prenatal Diagnosis:

Background: Single ventricle (SV) patients with total anomalous pulmonary venous connection (TAPVC) are at high risk. Given the limited published data available, we examined outcomes and the implications of a prenatal diagnosis of SV/TAPVC. Methods: A single-center, retrospective review was performed in neonates with SV/TAPVC from 1998 to 2014, identified through institutional databases. Patient demographic, perioperative, and follow-up data were collected. Results: Thirty-four eligible infants with SV/TAPVC were identified (mean birth weight: 3.0 kg). The TAPVC types were supracardiac (59%), infracardiac (21%), mixed (12%), and cardiac (9%). Heterotaxy syndrome was present in 25 (74%) infants. A prenatal diagnosis of SV was made in 26 (76%) infants, with TAPVC identified in 12 (35%). Seventeen (50%) had obstructed TAPVC within the first 48 hours of life; 7 of these patients had obstructed TAPVC identified prenatally. There were two preoperative deaths. Overall survival for the cohort was 65% at 1 year and 50% at 3 years. Survival in the obstructed group was significantly worse compared to the unobstructed group (47% vs 81% at 1 year; 27% vs 73% at 3 years, P = .01). Obstructed TAPVC and a prenatal prediction of obstructed TAPVC were significantly associated with postoperative mortality (P = .01 and .03, respectively). Conclusions: Patients with SV/TAPVC remain a high-risk group, with obstructed TAPVC a significant risk factor for mortality. Prenatal diagnosis of TAPVC in SV patients is challenging, but given those with obstructed TAPVC are especially at high risk, improved prenatal diagnostic techniques in this group may enhance counseling/delivery planning.

Similar Interstage Outcomes for Single Ventricle Infants Palliated With an Aortopulmonary Shunt Compared to the Norwood Procedure

Background: Interstage outcomes for single ventricle infants following Norwood operation have been well studied, showing significant mortality. Other single ventricle infants require only an aortopulmonary shunt. The aim of the study was to describe the interstage outcomes of this group compared to Norwood patients and identify risk factors for mortality. Methods: A single-center retrospective cohort review was performed in patients who underwent a Norwood operation (Norwood) or aortopulmonary shunt (Shunt) during 2000 to 2011 and survived to discharge. Hybrid or pulmonary artery banding patients were excluded. Univariate comparison was made between Norwood and Shunt patients as well as a Shunt subgroup analysis. Results: A total of 486 patients (368 Norwood and 118 Shunt) were included. Norwood and Shunt patients were similar in terms of preterm birth, surgery weight, and stage 1 complications. Shunt patients were more likely to be female, have an extracardiac or genetic anomaly, and have a shorter hospital length of stay compared to the Norwood patients (all P < .0001). No significant difference in interstage mortality was seen between the Shunt and Norwood patients (6.8% vs 11.1%, respectively; P = .17). Stage 2 mortality was also similar (Shunt 4.6% vs Norwood 7.8%; P = .25). In the Shunt patients, those who died during interstage weighed less at surgery (2.7 [0.7] kg vs 3.3 [0.7] kg, P = .03) and were more likely to have arrhythmias (50% vs 12%, P = .01), compared to survivors. Conclusions: Shunt patients have an interstage mortality that is not significantly less than Norwood patients. Lower weight at surgery and arrhythmias are risk factors for interstage death in Shunt patients.

Fontan-associated protein-losing enteropathy and post-heart transplant outcomes: a multicenter study

BACKGROUND The influence of Fontan-associated protein-losing enteropathys (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality. METHODS This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought. RESULTS Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors. CONCLUSIONS PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.

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.

KOMMERELL DIVERTICULUM ACROSS THE AGES: DESTINED FOR DILATION?

Background: A Kommerell Diverticulum (KD) is the dilated proximal portion of an aberrant subclavian artery as a consequence of abnormal embryonic vascular development. Concern has arisen for aneurysmal KD dilation, dissection and rupture, with a trend toward invasive prophylactic intervention. This

DIFFERENTIAL MYOCARDIAL MECHANICS IN VOLUME AND PRESSURE LOADED RIGHT VENTRICLES DEMONSTRATED BY CARDIAC MAGNETIC RESONANCE

Echocardiographic studies suggest an adaptive shift to circumferential (CS) rather than longitudinal strain (LS) in patients with a systemic right ventricle. We aimed to evaluate myocardial mechanics in pressure-loaded, volume-loaded and control right ventricles by cardiac magnetic resonance. This