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.

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.