Prenatal Predictors for Pulmonary Balloon Valvuloplasty in the Newborn.

Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1-58) days and 11 (22%) underwent outpatient PBV at 51.8 (11-174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02-94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS.

Association Between Remote Monitoring and Interstage Morbidity and Death in Patients With Single-Ventricle Heart Disease Across Socioeconomic Groups.

BACKGROUND: Despite improvements in survival over time, the mortality rate for infants with single-ventricle heart disease remains high. Infants of low socioeconomic status (SES) are particularly vulnerable. We sought to determine whether use of a novel remote monitoring program, the Cardiac High Acuity Monitoring Program, mitigates differences in outcomes by SES. METHODS AND RESULTS: Within the Cardiac High Acuity Monitoring Program, we identified 610 infants across 11 centers from 2014 to 2021. All enrolled families had access to a mobile application allowing for near-instantaneous transfer of patient information to the care team. Patients were divided into SES tertiles on the basis of 6 variables relating to SES. Hierarchical logistic regression, adjusted for potential confounding characteristics, was used to determine the association between SES and death or transplant listing during the interstage period. Of 610 infants, 39 (6.4%) died or were listed for transplant. In unadjusted analysis, the rate of reaching the primary outcome between SES tertiles was similar (P=0.24). Even after multivariable adjustment, the odds of death or transplant listing were no different for those in the middle (odds ratio, 1.7 [95% CI, 0.73-3.94) or highest (odds ratio, 0.997 [95% CI, 0.30, 3.36]) SES tertile compared with patients in the lowest (overall P value 0.4). CONCLUSIONS: In a large multicenter cohort of infants with single-ventricle heart disease enrolled in a digital remote monitoring program during the interstage period, we found no difference in outcomes based on SES. Our study suggests that this novel technology could help mitigate differences in outcomes for this fragile population of patients.

Associations of Home Monitoring Data to Interventional Catheterization for Infants with Recurrent Coarctation of the Aorta and Hypoplastic Left Heart Syndrome.

The post-Norwood interstage period for infants with hypoplastic left heart syndrome is a high-risk time with 10-20% of infants having a complication of recurrent coarctation of the aorta (RCoA). Many interstage programs utilize mobile applications allowing caregivers to submit home physiologic data and videos to the clinical team. This study aimed to investigate if caregiver-entered data resulted in earlier identification of patients requiring interventional catheterization for RCoA. Retrospective home monitoring data were extracted from five high-volume Children's High Acuity Monitoring Program®-affiliated centers (defined as contributing > 20 patients to the registry) between 2014 and 2021 after IRB approval. Demographics and caregiver-recorded data evaluated include weight, heart rate (HR), oxygen saturation (SpO2), video recordings, and 'red flag' concerns prior to interstage readmissions. 27% (44/161) of infants required interventional catheterization for RCoA. In the 7 days prior to readmission, associations with higher odds of RCoA included (mean bootstrap coefficient, [90% CI]) increased number of total recorded videos (1.65, [1.07-2.62]) and days of recorded video (1.62, [1.03-2.59]); increased number of total recorded weights (1.66, [1.09-2.70]) and days of weights (1.56, [1.02-2.44]); increasing mean SpO2 (1.55, [1.02-2.44]); and increased variation and range of HR (1.59, [1.04-2.51]) and (1.71, [1.10-2.80]), respectively. Interstage patients with RCoA had increased caregiver-entered home monitoring data including weight and video recordings, as well as changes in HR and SpO2trends. Identifying these items by home monitoring teams may be beneficial in clinical decision-making for evaluation of RCoA in this high-risk population.

Ability of Video Telemetry to Predict Unplanned Hospital Admissions for Single Ventricle Infants.

Background Our Cardiac High Acuity Monitoring Program (CHAMP) uses home video telemetry (HVT) as an adjunct to monitor infants with single ventricle during the interstage period. This study describes the development of an objective early warning score using HVT, for identification of infants with single ventricle at risk for clinical deterioration and unplanned hospital admissions (UHA). Methods and Results Six candidate scoring parameters were selected to develop a pragmatic score for routine evaluation of HVT during the interstage period. We evaluated the individual and combined ability of these parameters to predict UHA. All infants with single ventricle monitored at home by CHAMP between March 2014 and March 2018 were included. Videos obtained within 48 hours before UHA were compared with videos obtained at baseline. We used binary logistic regression models and receiver operating characteristic curves to evaluate the parameters' performance in discriminating the outcome of interest. Thirty-nine subjects with 64 UHA were included. We compared 64 pre-admission videos to 64 paired baseline videos. Scoring was feasible for a mean of 91.6% (83.6%-98%) of all observations. Three different HVT score models were proposed, and a final model composed of respiratory rate, respiratory effort, color, and behavior exhibited an excellent discriminatory capability with an area under the receiver operating characteristic curve of 93% (89%-98%). HVT score of 5 was associated with specificity of 93.8% and sensitivity of 88.7% in predicting UHA. Conclusions We developed a feasible and reproducible HVT score that can serve as a tool to predict UHA in infants with single ventricle. Future directions involve prospective, multicenter validation of this tool.

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.

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.

Long-Term Transplant-Free Survival After Repair of Total Anomalous Pulmonary Venous Connection.

BACKGROUND: Long-term survival, risk of transplantation, and causes of death after repair of total anomalous pulmonary venous connection (TAPVC) remain unknown. By linking the Pediatric Cardiac Care Consortium with the National Death Index and the United Network for Organ Sharing, we evaluated long-term transplant-free survival in children undergoing repair of TAPVC. METHODS: We identified 777 infants within the Pediatric Cardiac Care Consortium who underwent TAPVC repair (median 21 days; interquartile range, 5 to 80) and had sufficient personal identifiers for linkage with the National Death Index and United Network for Organ Sharing. Sixty-six deaths, ten cardiac transplantations, and one bilateral lung transplantation had occurred by the end of 2014. Data collected included age and weight at time of procedure, TAPVC type, associated cardiac lesions, and postoperative length of stay. The study cohort was divided into simple and complex TAPVC based on the presence of an associated cardiac lesion. Parametric survival plots were constructed, and risk factor analyses were performed to identify demographic and clinical characteristics associated with long-term outcomes. RESULTS: Mortality or need for transplantation was 9.7% with a median follow-up of 18.4 years and a median age of death or transplant of 0.74 years. The risk of mortality and transplant after TAPVC repair was highest during the first 18 months after hospital discharge. Cardiac causes accounted for the majority of deaths. Multivariate regression models for transplant-free survival demonstrated that complex TAPVC, mixed TAPVC, and postoperative length of stay were associated with increased risk of death/transplant. CONCLUSIONS: Transplant-free survival after TAPVC repair is excellent, with most deaths or transplant events occurring early. Factors associated with the worst long-term outcomes included complex TAPVC, mixed TAPVC, and prolonged postoperative length of stay.

Recurrent Coarctation After Neonatal Univentricular and Biventricular Norwood-Type Arch Reconstruction.

BACKGROUND: Previous studies have evaluated recurrent coarctation after the Norwood procedure (NP) in patients with single-ventricle (SV) anatomy. Extended patch augmentation of the aorta, a Norwood-type arch reconstruction (NTAR), is also used in patients with 2 ventricles and a diffusely hypoplastic arch; however, outcomes after NTAR in these patients are not well described. METHODS: A retrospective review was performed of neonates with SV and 2-ventricle (2V) anatomy who underwent an NP or NTAR at our institution from 2000 to 2010. The incidence of recurrent coarctation requiring intervention and associated risk factors were evaluated. RESULTS: Overall, 101 patients with 2V anatomy and 361 patients with SV anatomy were included. Eighteen patients with 2V anatomy (17.8%) and 35 patients with SV anatomy (9.7%) required intervention for recurrent coarctation at a median of 0.5 years (interquartile range [IQR], 0.3-1.2 years) after the initial operation. Independent risk factors associated with recurrent coarctation in patients with 2V anatomy included weight less than 2.5 kg (hazard ratio [HR], 6.05; p = 0.001) and peak aortic arch gradient (PAAG) on the discharge echocardiogram greater than 10 mm Hg (HR, 3.07; p = 0.03). In patients with SV anatomy, shunt type (HR, 6.42; p < 0.0001 for right ventricle to pulmonary artery [RV-PA] shunt compared with others) and peak gradient on the discharge echocardiogram greater than 10 mm Hg were found to be significant (HR, 7.40; p < 0.0001). There was no survival difference in patients with and those without recurrent coarctation. CONCLUSIONS: Recurrent coarctation is common after NTAR. Small patient size and shunt type were found to be independent risk factors for recurrent coarctation in the 2V and SV populations, respectively, and discharge aortic arch gradient was a significant risk factor in both populations.

Cardiac findings and long-term thromboembolic outcomes following pulmonary embolism in children: a combined retrospective-prospective inception cohort study.

In paediatric pulmonary embolism, cardiac findings and thromboembolic outcomes are poorly defined. We conducted a mixed retrospective-prospective cohort study of paediatric pulmonary embolism at the Children's Hospital Colorado between March, 2006 and January, 2011. A total of 58 consecutive children - age less than or equal to 21 years - with acute pulmonary embolism were enrolled. Data collection included clinical and laboratory characteristics, treatments, serial echocardiographic and electrocardiographic findings, and outcomes of pulmonary embolism non-resolution and recurrence. The median age was 16.5 years ranging from 0 to 21 years. The most prevalent clinical risk factors were oral contraceptive pill use (52% of female patients), presence of a non-infectious inflammatory condition (21%), and trauma (21%). Thrombophilias included heterozygous factor V Leiden in 21%; antiphospholipid antibody syndrome was established in 31% overall. Proximal pulmonary artery involvement was present in 34%. At presentation, nearly half of the patients had hypoxaemia and 37% had tachycardia. The classic electrocardiographic finding of S1Q3T3 was present in 12% acutely; tricuspid regurgitation greater than 3 metres per second, septal flattening, and right ventricular dilation were each present on acute echocardiogram in 25%. Nearly all patients received therapeutic anticoagulation, with initial systemic tissue plasminogen activator administered in 16% for occlusive iliofemoral deep venous thrombosis and/or massive pulmonary embolism. Pulmonary embolism resolution was observed in 82% by 6 months. Recurrent pulmonary embolism occurred in 9%. There were no pulmonary embolism-related deaths. Right ventricular dysfunction was rare in follow-up. These data indicate that acute heart strain is common, but chronic cardiac dysfunction is rare, following aggressive management of acute pulmonary embolism in children.

Prospective validation of the vasoactive-inotropic score and correlation to short-term outcomes in neonates and infants after cardiothoracic surgery.

PURPOSE: Prospective validation of the vasoactive-inotropic score (VIS) and inotrope score (IS) in infants after cardiovascular surgery. METHODS: Prospective observational study of 70 infants (≤90 days of age) undergoing cardiothoracic surgery. VIS and IS were assessed at 24 (VIS24, IS24), 48 (VIS48, IS48), and 72 (VIS72, IS72) h after surgery. Maximum VIS and IS scores in the first 48 h were also calculated (VIS48max and IS48max). The primary outcome was length of intubation. Additional outcomes included length of intensive care (ICU) stay and hospitalization, cardiac arrest, mortality, time to negative fluid balance, peak lactate, and change in creatinine. RESULTS: Based on receiver-operating characteristic (ROC) analysis, the area under the curve (AUC) was highest for VIS48 to identify prolonged intubation time. AUC for the primary outcome was higher for VIS than IS at all time points assessed. On multivariate analysis VIS48 was independently associated with prolonged intubation (OR 22.3, p = 0.002), prolonged ICU stay (OR 8.1, p = 0.017), and prolonged hospitalization (OR 11.3, p = 0.011). VIS48max, IS48max, and IS48 were also associated with prolonged intubation, but not prolonged ICU or hospital stay. None of the scores were associated with time to negative fluid balance, peak lactate, or change in creatinine. CONCLUSION: In neonates and infants, a higher VIS at 48 h after cardiothoracic surgery is strongly associated with increased length of ventilation, and prolonged ICU and total hospital stay. At all time points assessed, VIS is more predictive of poor short-term outcome than IS. VIS may be useful as an independent predictor of outcomes.