The impact of specialty training and physician attitudes on fetal cardiac counseling.

OBJECTIVE: Fetal cardiac anomalies are the most commonly diagnosed structural anomalies. In these cases, Maternal-Fetal Medicine (MFM) specialists are tasked with counseling patients on a spectrum of diagnoses as well as their prognostic implications. A recent study of pediatric cardiologists demonstrated that personal beliefs regarding termination impact the counseling provided to patients. Our objective was to study whether the personal beliefs of MFMs impact counseling of patients with severe fetal cardiac anomalies and to compare these providers to their cardiology counterparts. METHODS: We conducted an anonymous cross-sectional survey of MFMs in New England that assessed personal beliefs and counseling practices when diagnosing hypoplastic left heart syndrome (HLHS). We subsequently compared these providers to the previously surveyed cardiologists. RESULTS: A total of 34 respondents representing a broad spectrum of age and experience across several states in New England were analyzed. When presented with the statement "some life is always better than no life at all," 79% (n = 27) of respondents disagreed and all respondents (n = 34) offered termination, palliative care, and treatment options when counseling patients with HLHS. Additionally, while 74% (n = 25) of providers would personally support a decision to terminate a pregnancy with HLHS, 94% (n = 32) would professionally support the decision to pursue termination.MFMs and cardiologists differed in their responses to "some life is better than no life" and the belief that termination should be offered, though differences did not reach statistical significance. However, with respect to the providers' personal and professional support of the decision to terminate the pregnancy, the groups of respondents varied significantly in their level of support, both professionally and personally with fewer cardiologists supporting this decision. CONCLUSION: When diagnosing a severe and potentially fatal congenital cardiac anomaly, counseling by MFMs was largely unaffected by personal beliefs regarding termination of pregnancy. While this is consistent with previously published data on counseling practices among pediatric cardiology specialists, some important differences between the specialties were seen.

Geospatial Distribution of Prenatally and Postnatally Diagnosed Congenital Heart Disease: Implications for Equitable Care from a Fetal Heart Society Research Collaborative Study.

OBJECTIVE: To characterize patterns in the geospatial distribution of pre- and postnatally diagnosed congenital heart disease (CHD) across 6 surgical centers. STUDY DESIGN: A retrospective, multicenter case series from the Fetal Heart Society identified patients at 6 centers from 2012 through 2016 with prenatally (PrND) or postnatally (PoND) diagnosed hypoplastic left heart syndrome (HLHS) or d-transposition of the great arteries (TGA). Geospatial analysis for clustering was done by the average nearest neighbor (ANN) tool or optimized hot spot tool, depending on spatial unit and data type. Both point location and county case rate per 10 000 live births were assessed for geographic clustering or dispersion. RESULTS: Of the 453 CHD cases, 26% were PoND (n = 117), and 74% were PrND (n = 336). PrND cases, in all but one center, displayed significant geographic clustering by the ANN. Conversely, PoND cases tended toward geographic dispersion. Dispersion of PoND HLHS occurred in 2 centers (ANN = 1.59, P < .001; and 1.47, P = .016), and PoND TGA occurred in 2 centers (ANN = 1.22, P < .05; and ANN = 1.73, P < .001). Hot spot analysis of all CHD cases (TGA and HLHS combined) revealed clustering near areas of high population density and the tertiary surgical center. Hot spot analysis of county-level case rate, accounting for population density, found variable clustering patterns. CONCLUSION: Geographic dispersion among postnatally detected CHD highlights the need for a wider reach of prenatal cardiac diagnosis tailored to the specific needs of a community. Geospatial analysis can support centers in improving the equitable delivery of prenatal care.

Labyrinthine cor triatriatum sinister in fetal hypoplastic left heart syndrome is associated with poor outcomes.

OBJECTIVES: A subset of hypoplastic-left-heart-syndrome (HLHS) fetuses have a complex cor-triatriatum sinister that we named "labyrinthine-cor (L-cor)". We sought to determine the prevalence of L-cor in HLHS fetuses and hypothesized that it is associated with increased mortality. METHODS: This single-center retrospective cohort study included all HLHS fetuses from January 2010-December 2020. Fetuses with other hypoplastic-left-heart variants, inadequate images, lack of follow-up and fetal atrial-septal interventions were excluded. RAS was defined as the ratio of pulmonary-vein forward-to-reverse velocity-time-integral (VTI) ≤ 5 and severe-RAS defined as VTI-ratio <3. Kaplan-Meier survival-analysis was performed for the primary outcome of transplant-free survival for 62 weeks after gestational-age of 30 weeks (∼1 year). RESULTS: Of the 156 consecutive fetuses with HLHS, 11 (7.7%) had L-cor and 8/11 (72.7%) of these had RAS. When compared to HLHS-RAS without L-cor, fetuses with HLHS-RAS and L-cor were less likely to survive to 28 days (87% vs. 62.5%, p = 0.017) and to 1 year (69.6% vs. 25%, p = 0.029). When comparing by survival analysis, fetuses with severe-RAS with L-cor had lower survival compared severe-RAS without L-cor (p = 0.020). CONCLUSION: L-cor in fetal HLHS is associated with increased mortality. Recognition of this finding is important for prognostication and atrial-septal-intervention planning.

Novel application of the Diabolo technique to restrict pulmonary blood flow in a single ventricle patient with a right ventricle to pulmonary artery conduit.

Balancing pulmonary and systemic circulations in single ventricle patients with a conduit after Stage 1 palliation is challenging. A transcatheter intervention for excessive pulmonary blood flow would provide benefit. We report a case of a critically ill single ventricle patient with symptoms of excessive pulmonary blood flow after Stage 1 despite maximal medical therapy. The patient underwent percutaneous intraluminal downsizing of the right ventricle to pulmonary artery conduit using a novel application of the Diabolo-covered stent technique, with subsequent clinical improvement. A second catheterization was performed during the interstage period with successful dilation of the stent to achieve appropriate saturations. The Diabolo technique can be successfully employed in this population to restrict pulmonary blood flow and has the advantage of being adjusted during placement and in subsequent interventions.

Multimodality imaging for the diagnosis of left ventricular apical hypoplasia: A case presentation and review of the literatures.

Left ventricular apical hypoplasia is a rare malformation recently described congenital abnormality characterized by: (1) truncation of the left ventricle, with the septum projecting toward the right ventricle; (2) abnormal papillary muscle originating from the flattened left ventricular apex; (3) a narrow right ventricle encompassing the periapical area of the left ventricle; (4) fatty infiltration of the apex of the left ventricle. We reported a case of LVAH and reviewed the patient's clinical presentation. And its morphologic characteristics were revealed by multimodality imaging, including echocardiography and cardiac magnetic resonance imaging. Additionally, we reviewed 41 cases from 32 reports to summarize the pathogenesis and analyzed the imaging manifestations of LVAH in this study, aiming to provide new ideas for the diagnosis and clinical management of LVAH patients.

Cardiac fibroma presenting as hypoplastic left heart syndrome in a foetus: causal or coincidental?

Primary cardiac tumours are very rare. Cardiac tumours in the perinatal period are even more uncommon with a prevalence of 0.0017% to 0.28% in autopsy series. The majority of benign cardiac tumours are cardiac rhabdomyomas, followed by cardiac fibromas. Another rare congenital heart disease is hypoplastic left heart syndrome (HLHS). Here we present a 21-week-old foetus diagnosed antenatally with HLHS on foetal echocardiogram. An autopsy done on the foetus following medical termination of pregnancy revealed a cardiac fibroma in the ventricular septum. It is very uncommon to have a combination of two congenital heart diseases. An extensive literature review revealed only three cases that had rhabdomyoma and associated HLHS. This case presented with cardiac fibroma which in early gestation would have resulted in left ventricular outflow obstruction leading to the development of HLHS. Small cardiac tumours which are difficult to detect by echocardiogram in early gestation can lead to the development of HLHS. A thorough and hierarchical autopsy examination of such cases can help in a better understanding of the relationship between HLHS and cardiac tumours.

School age and adolescent heart failure following the Norwood procedure.

BACKGROUND: Heart failure results in significant morbidity and mortality for young children with hypoplastic left heart syndrome (HLHS) following the Norwood procedure. The trajectory in later childhood is not well described. METHODS: We studied the outcome into adolescence of participants enrolled in the Single Ventricle Reconstruction trial who underwent the Fontan procedure or survived to 6 years without having undergone Fontan procedure. The primary outcome was heart failure events, defined as heart transplant listing or death attributable to heart failure. Symptomatic heart failure for participants surviving 10 or more years was also assessed utilizing the Pediatric Quality of Life Inventory (PedsQL). RESULTS: Of the 345 participants who underwent a Fontan operation or survived to 6 years without Fontan, 25 (7.2%) had a heart failure event before the age of 12 years. Among these, 21 were listed for heart transplant, and 4 died from heart failure. Nineteen participants underwent heart transplant, all of whom survived to age 12 years. Factors associated with a heart failure event included longer Norwood hospital length of stay, aortic atresia, and no Fontan operation by age 6 years. Assessment of heart failure symptoms at 12 years of age revealed that 24 (12.2%) of 196 PedsQL respondents "often" or "almost always" had difficulty walking more than one block. CONCLUSIONS: Heart failure events occur in over 5% of children with palliated HLHS between preschool age and adolescence. Outcomes for children listed for transplant are excellent. However, a substantial portion of palliated HLHS children have significant symptoms of heart failure at 12 years of age.

Inpatient Resource Utilization for Hypoplastic Left Heart Syndrome from Birth Through Fontan.

Completing 3-stage palliation for hypoplastic left heart syndrome requires significant resources. An analysis of recent data has not been performed. We aimed to determine total charges necessary to complete all 3 stages of single-ventricle palliation, including interstage encounters. We also aimed to determine overall resource utilization, including hospital days, interstage admissions, and interstage procedures. We performed a retrospective cohort study using data from the Pediatric Health Information System database between 2016 and 2021, including all patients who completed 3-stage palliation for hypoplastic left heart syndrome. We identified 199 patients who underwent 3-stage palliation of hypoplastic left heart syndrome between 2016 and 2021. Median total adjusted charges (interquartile range, IQR) over the course of 3-stage palliation were $1,475,800 ($1,028,900-2,191,700). Median adjusted charges (IQR) for stage 1, 2, and 3 hospitalizations were $604,300 ($419,000-891,400), $234,000 ($164,300-370,800), and $256,260 ($178,300-345,900), respectively. Median hospital length of stay (IQR) for stages 1, 2, and 3 was 36 (26,53), 9 (6,17), and 10 (7,14) days, respectively. Pulmonary artery stenosis was the most common admitting diagnosis for interstage hospitalizations (3.4% of hospitalizations). Cardiac catheterization (24.1% of procedures) and feeding tube placement (10.0% of procedures) were the most common principal procedures during interstage hospitalizations. Total inpatient charges incurred throughout 3-stage palliation of hypoplastic left heart syndrome are substantial and have risen since prior studies. Gastrointestinal comorbidities and feeding optimization contribute considerably to this resource utilization.

Endovascular rehabilitation of acutely thrombosed fontan conduit in a patient with multiorgan failure.

A 32-year-old man with history of hypoplastic left heart syndrome status post-Fontan palliation (20-mm aortic homograft conduit) had previously undergone Fontan conduit (FC) and left pulmonary artery (LPA) stenting to relieve conduit obstruction.

Neighborhood Social Vulnerability and Interstage Weight Gain: Evaluating the Role of a Home Monitoring Program.

Background Poor interstage weight gain is a risk factor for adverse outcomes in infants with hypoplastic left heart syndrome. We sought to examine the association of neighborhood social vulnerability and interstage weight gain and determine if this association is modified by enrollment in our institution's Infant Single Ventricle Management and Monitoring Program (ISVMP). Methods and Results We performed a retrospective single-center study of infants with hypoplastic left heart syndrome before (2007-2010) and after (2011-2020) introduction of the ISVMP. The primary outcome was interstage weight gain, and the secondary outcome was interstage growth failure. Multivariable linear and logistic regression models were used to examine the association between the Social Vulnerability Index and the outcomes. We introduced an interaction term into the models to test for effect modification by the ISVMP. We evaluated 217 ISVMP infants and 111 pre-ISVMP historical controls. The Social Vulnerability Index was associated with interstage growth failure (P=0.001); however, enrollment in the ISVMP strongly attenuated this association (P=0.04). Pre-ISVMP, as well as high- and middle-vulnerability infants gained 4 g/d less and were significantly more likely to experience growth failure than low-vulnerability infants (high versus low: adjusted odds ratio [aOR], 12.5 [95% CI, 2.5-62.2]; middle versus low: aOR, 7.8 [95% CI, 2.0-31.2]). After the introduction of the ISVMP, outcomes did not differ by Social Vulnerability Index tertile. Infants with middle and high Social Vulnerability Index scores who were enrolled in the ISVMP gained 4 g/d and 2 g/d more, respectively, than pre-ISVMP controls. Conclusions In infants with hypoplastic left heart syndrome, high social vulnerability is a risk factor for poor interstage weight gain. However, enrollment in the ISVMP significantly reduces growth disparities.

Use of extracorporeal membrane oxygenation and cerebral oximetry in a stage 1 norwood repair for hypoplastic left heart syndrome.

The role of extracorporeal membrane oxygenation (ECMO) and its indications in stage I Norwood palliation are controversial. The decision to initiate ECMO and its timing remains difficult with no definitive cut-off points or evidence-based guidelines. It varies on a case-to-case basis. We report a case where the use of ECMO was beneficial after stage I Norwood palliation with severe ventricular dysfunction. The systemic-to-pulmonary artery shunt was kept open to balance the systemic and pulmonary circulations. Cerebral oximetry can be useful as an additional monitoring modality to guide management, monitor cerebral perfusion, and help detect cerebral steal.

[Perinatal outcome and five-year survival in children with prenatal diagnosis of hypoplastic left heart syndrome]. / Evolución perinatal y sobrevida a cinco años en niños con diagnóstico prenatal de síndrome de hipoplasia de ventrículo izquierdo.

Hypoplastic left heart syndrome (HLHS) is a congenital heart disease of low prevalence and high lethality. OBJECTIVE: to determine the perinatal outcome and survival at one and five years of fetuses with a prenatal diagnosis of HLHS. PATIENTS AND METHOD: Prospective cohort study of all the fetuses with HLHS from the Perinatal Reference Center (CERPO) born between January 2008 and December 2017. Demographic and clinical perinatal data were obtained from the CERPO database. At one and five years of age, a telephone survey was conducted to determine the surgical treatment and survival. RESULTS: 1,573 patients were admitted to the CERPO, 899 with congenital heart diseases (CHD), confirming the prenatal diagnosis of HLHS in 7% (110/1,573). The mean gestational age at diagnosis and the median at admission were 26+3 and 32+3 weeks, respectively. 89% were born alive, 90% at term, and 57% delivered by cesarean section. The median birth weight was 3,128 grams. 89% survive the prenatal period, 50% the early neonatal period, 33% the late neonatal period, 19% the first year, and 17% at 5 years. CONCLUSIONS: In this center, the one-year and five-year survival of fetuses with prenatal diagnosis of HLHS was 19% and 17%, respectively. It is important for prenatal counseling to consider publications based on local casuistry, that include patients with prenatal and postnatal diagnoses and those who underwent surgery, in order to provide more precise information to parents.

Association of Home Monitoring and Unanticipated Interstage Readmissions in Infants With Hypoplastic Left Heart Syndrome.

Background The impact of home monitoring on unanticipated interstage readmissions in infants with hypoplastic left heart syndrome has not been previously studied. We sought to examine the association of our institution's Infant Single Ventricle Management and Monitoring Program (ISVMP) with readmission frequency, cumulative readmission days, and readmission illness severity and to identify patient-level risk factors for readmission. Methods and Results We performed a retrospective single-center cohort study comparing infants with hypoplastic left heart syndrome enrolled in ISVMP (December 2010-December 2019) to historical controls (January 2007-November 2010). The primary outcome was number of readmissions per interstage days. Secondary outcomes were cumulative interstage readmission days and occurrence of severe readmissions. Inverse probability weighted and multivariable generalized linear models were used to examine the association between ISVMP and the outcomes. We compared 198 infants in the ISVMP to 128 historical controls. Infants in the ISVMP had more than double the risk of interstage readmission compared with controls (adjusted incidence rate ratio, 2.38 [95% CI, 1.50-3.78]; P=0.0003). There was no difference in cumulative interstage readmission days (adjusted incidence rate ratio, 1.02 [95% CI, 0.69-1.50]; P=0.90); however, infants in the ISVMP were less likely to have severe readmissions (adjusted odds ratio, 0.28 [95% CI, 0.11-0.68]; P=0.005). Other factors independently associated with number of readmissions included residing closer to our center, younger gestational age, genetic syndrome, and discharge on exclusive enteral feeds. Conclusions Infants in the ISVMP had more frequent readmissions but comparable readmission days and fewer severe unanticipated readmissions. These findings suggest that home monitoring can reduce interstage morbidity without increasing readmission days.

Factors influencing the choice-of-care pathway and survival in the fetus with hypoplastic left heart syndrome in New Zealand: a population-based cohort study.

OBJECTIVES: To better understand the relative influence of fetal and maternal factors in determining the choice-of-care pathway (CCP) and outcome in the fetus with hypoplastic left heart syndrome (HLHS). DESIGN: A retrospective, population-based study of fetuses with HLHS from a national dataset with near-complete case ascertainment from 20 weeks' gestation. Fetal cardiac and non-cardiac factors were recorded from the patient record and maternal factors from the national maternity dataset. The primary endpoint was a prenatal decision for active treatment after birth (intention-to-treat). Factors associated with a delayed diagnosis (≥24 weeks' gestation) were also reviewed. Secondary endpoints included proceeding to surgical treatment, and 30-day postoperative mortality in liveborns with an intention-to-treat. SETTING: New Zealand population-wide. PARTICIPANTS: Fetuses with a prenatal diagnosis of HLHS between 2006 and 2015. RESULTS: Of 105 fetuses, the CCP was intention-to-treat in 43 (41%), and pregnancy termination or comfort care in 62 (59%). Factors associated with intention-to-treat by multivariable analysis included a delay in diagnosis (OR: 7.8, 95% CI: 3.0 to 20.6, p<0.001) and domicile in the maternal fetal medicine (MFM) region with the most widely dispersed population (OR: 5.3, 95% CI: 1.4 to 20.3, p=0.02). Delay in diagnosis was associated with Maori maternal ethnicity compared with European (OR: 12.9, 95% CI: 3.1 to 54, p<0.001) and greater distance from the MFM centre (OR: 3.1, 95% CI: 1.2 to 8.2, p=0.02). In those with a prenatal intention-to-treat, a decision not to proceed to surgery was associated with maternal ethnicity other than European (p=0.005) and the presence of major non-cardiac anomalies (p=0.01). Thirty-day postoperative mortality occurred in 5/32 (16%) and was more frequent when there were major non-cardiac anomalies (p=0.02). CONCLUSIONS: Factors associated with the prenatal CCP relate to healthcare access. Anatomic characteristics impact treatment decisions after birth and early postoperative mortality. The association of ethnicity with delayed prenatal diagnosis and postnatal decision-making suggests systemic inequity and requires further investigation.

Prenatal care coordination, racial and socioeconomic inequities, and pre- and post-operative outcomes in hypoplastic left heart syndrome.

OBJECTIVE: We sought to identify associations between prenatal care coordination (PNC) and outcomes in hypoplastic left heart syndrome (HLHS). STUDY DESIGN: We hypothesized that suboptimal PNC is associated with worse pre-operative status. HLHS patients from 2016 through 2019 were identified using a multicenter registry. Optimal PNC was defined as (1) a completed interdisciplinary conference and (2) closed-loop communication with the obstetric team. Associations between PNC and outcomes were identified. RESULTS: Of 1441 patients, 1242 (86%) had prenatal diagnosis. Among those with a prenatal diagnosis, PNC was achieved in only 845 (68%). Suboptimal PNC was associated with adverse events (50% vs 40%, p < 0.001), inotrope need (19% vs 13%, p = 0.007), mechanical ventilation (22% vs 16%, p = 0.016), and parenteral feeding (60% vs 46%, p < 0.001). African-American race and non-commercial insurance were associated with a lower likelihood of optimal PNC (p = 0.006 and p < 0.001, respectively). CONCLUSION: Improving PNC and overcoming racial and socioeconomic barriers are important targets to improve HLHS perinatal care.

Milrinone for treatment of elevated lactate in the pre-operative newborn with hypoplastic left heart syndrome.

BACKGROUND: There is a paucity of information reported regarding the use of milrinone in patients with hypoplastic left heart syndrome prior to the Norwood procedure. At our institution, milrinone is initiated in the pre-operative setting when over-circulation and elevated serum lactate levels develop. We aimed to review the responses associated with the administration of milrinone in the pre-operative hypoplastic left heart syndrome patient. Second, we compared patients who received high- versus low-dose milrinone prior to Norwood procedure. METHODS: Single-centre retrospective study of patients diagnosed with hypoplastic left heart syndrome between January 2000 and December 2019 who underwent Norwood procedure. Patient characteristics and outcomes were compared. RESULTS: During the study period, 375 patients were identified; 79 (21%) received milrinone prior to the Norwood procedure with median lactate 2.55 mmol/l, and SpO2 93%. Patients who received milrinone were older at the time of Norwood procedure (6 vs. 5 days) and were more likely to be intubated and sedated. In a subset analysis stratifying patients to low- versus high-dose milrinone, median lactate decreased from time of initiation (2.39 vs 2.75 to 1.6 vs 1.8 mmol/l) at 12 hours post-initiation, respectively. Repeated measures analysis showed a significant decrease in lactate levels by 4 hours following initiation of milrinone, that persisted over time, with no significant difference in mean arterial pressure. CONCLUSIONS: The use of milrinone in the pre-operative over-circulated hypoplastic left heart syndrome patient is well tolerated, is associated with decreased lactate levels, and was not associated with significant hypotension or worsening of excess pulmonary blood flow.

New approach in stage 1 surgery for hypoplastic left heart syndrome: preliminary outcomes.

OBJECTIVES: We present the short-term results of an alternative method in stage 1 surgery for hypoplastic left heart syndrome. METHODS: Data of 16 consecutive patients who were treated with the novel method in our clinic between February 2019 and March 2021 were analysed retrospectively. Preoperative data and postoperative follow-up were recorded. RESULTS: Of the 16 operated patients, 12 were diagnosed with hypoplastic left heart syndrome, while four were diagnosed with hypoplastic left heart syndrome variants. Seven patients died during early postoperative period. One patient died at home waiting stage 2 surgery. Three patient underwent stage 2 surgery. Pulmonary artery reconstruction was performed in one patient due to left pulmonary artery distortion. CONCLUSIONS: We believe that our method can be an effective alternative in the surgery of hypoplastic left heart syndrome and its variants. It is hoped that with increasing number of studies and more experience better outcome will be achieved.

Hospitalisations of children and adults with Hypoplastic Left Heart Syndrome in Texas from 2009 to 2019.

INTRODUCTION: Hypoplastic Left Heart Syndrome accounts for a significant proportion of CHD morbidity and mortality, despite improvements in care and improved survival. This study evaluates number of, reasons for, and trends in discharges of patients with hypoplastic left heart syndrome over 11 years in Texas. METHODS: The Texas Inpatient Discharge Dataset Public Use File captures almost all discharges in Texas and was reviewed from 2009 to 2019. Discharges of patients ≥5 years of age and diagnosis codes for Hypoplastic Left Heart Syndrome were included. The admitting and principle diagnoses were categorised and all discharges were evaluated for procedures performed. Descriptive and univariate statistical analyses were performed. RESULTS: A total of 1024 discharges were identified with a 16.9% annual increase over the study period. Median length of stay was 4 [IQR: 2-8] and there were 17 (1.7%) in-hospital mortalities with no differences across age groups. Seven (17.1%) discharges of patients 25+ years were uninsured, higher than other age groups (p < 0.001). The most common admitting diagnosis was CHD and 224 (21.9%) of discharges included a procedure, including 23 heart transplants. Discharges occurred from 67 different hospitals with 4 (6.0%) representing 71.4% of all discharges. CONCLUSIONS: Discharges of Hypoplastic Left Heart Syndrome have increased rapidly, particularly in the older age groups and were spread over a large number of hospitals. Further work is needed to understand the interplay between Hypoplastic Left Heart Syndrome and other conditions and care experiences that occur within the general population, which will become more common as this population ages and grows.

Cardiovascular intensive care unit variables inform need for feeding tube utilization in infants with hypoplastic left heart syndrome.

OBJECTIVE: Feeding strategies in infants with hypoplastic left heart syndrome (HLHS) following stage 1 palliation (S1P) include feeding tube utilization (FTU). Timely identification of infants who will fail oral feeding could mitigate morbidity in this vulnerable population. We aimed to develop a novel clinical risk prediction score for FTU. METHODS: This was a retrospective study of infants with HLHS admitted to the Boston Children's Hospital cardiovascular intensive care unit for S1P from 2009 to 2019. Infants discharged with feeding tubes were compared with those on full oral feeds. Variables from early (birth to surgery), mid (postsurgery to cardiovascular intensive care unit transfer), and late (inpatient transfer to discharge) hospitalization were analyzed in univariate and multivariable models. RESULTS: Of 180 infants, 66 (36.7%) discharged with a feeding tube. In univariate analyses, presence of a genetic disorder (early variable, odds ratio, 3.25; P = .014) and nearly all mid and late variables were associated with FTU. In the mid multivariable model, abnormal head imaging, ventilation duration, and vocal cord dysfunction were independent predictors of FTU (c-statistic 0.87). Addition of late variables minimally improved the model (c-statistic 0.91). A risk score (the HV2 score) for FTU was developed based on the mid multivariable model with high specificity (93%). CONCLUSIONS: Abnormal head imaging, duration of ventilation, and presence of vocal cord dysfunction were associated with FTU in infants with HLHS following S1P. The predictive HV2 risk score supports routine perioperative head imaging and vocal cord evaluation. Future application of the HV2 score may improve nutritional morbidity and hospital length of stay in this population.

Management of Hypoplastic Left Heart Syndrome in Low-Resource Settings and the Ethics of Decision-Making.

Hypoplastic left heart syndrome (HLHS) is possibly the most challenging congenital heart defect to confront in any setting. The highly specialized infrastructure and resources needed to treat HLHS is not available in many low-resource settings. However, low-resource settings must not be assumed to be synonymous with low- and middle-income countries as national income is not necessarily indicative of a country's prioritization of healthcare resources. Besides, a low-resource setting may be institution-specific as well as country-specific. We have stratified institutional capabilities for addressing the requirements of treatment for HLHS into five levels based on the capacity for diagnosis, intervention, and post-discharge monitoring. Depending on institutional capabilities, children born with HLHS in low-resource settings experience a spectrum of outcomes ranging from death without diagnosis to the hybrid or Norwood stage 1 palliation. The decision-making is ethically challenging when resources are scarce and economic efficiency must be considered in the context of distributive justice. Even in settings that would be classified as resource-rich where survival after surgery and quality of life afterward keep improving, not every parent would choose surgical intervention for their hypothetical child with HLHS.