Transcatheter and Surgical Ductus Arteriosus Closure in Very Low Birth Weight Infants: 2018-2022.

BACKGROUND AND OBJECTIVE: The optimal patent ductus arteriosus (PDA) closure method in very low birth weight (VLBW) infants is uncertain. In 2019, the first transcatheter occlusion device was approved in the United States for infants ≥700 g. We described survival and short-term outcomes among VLBW infants who underwent transcatheter or surgical PDA closure (2018-2022). METHODS: Vermont Oxford Network members submitted data on infants born from 401 to 1500 g or 22 to 29 weeks' gestational age. Adjusted risk ratios (aRR) for survival, length of stay (LOS), prematurity complications, and discharge support were used to compare transcatheter versus surgical closure. Subgroup analyses were conducted for infants with birth weight ≥700 g and born in 2020-2022. RESULTS: Overall, 6410 of 216 267 infants at 726 hospitals received invasive PDA treatment. Transcatheter closure increased from 29.8% in 2018 to 71.7% in 2022. VLBW infants undergoing transcatheter closure had higher survival (adjusted rate ratio [aRR] 1.03; 1.02-1.04) with similar LOS (aRR 1.00; 0.97-1.03), neonatal complications (aRR 1.00; 0.98-1.01), and receipt of discharge support (aRR 0.94; 0.89-1.01). In subgroup analyses, survival (aRR 1.02; 1.00-1.04) and discharge support (aRR 0.90; 0.81-1.01) were similar between groups, whereas selected neonatal complications (aRR 0.95; 0.93-0.98) and LOS (aRR 0.95; 0.90-0.99) were lower after transcatheter closure. CONCLUSIONS: Transcatheter PDA closure in VLBW infants was increasingly used after 2018. Selected short-term outcomes for infants receiving transcatheter closure may be more favorable, compared with surgical, and warrants further clinical investigation.

Improvement Science Increases Routine Lipid Screening in General Pediatric Cardiology.

OBJECTIVE: To evaluate the effectiveness of patient education, physician counseling, and point-of-care (POC) testing on improving adherence to lipid screening national guidelines in a general pediatric cardiology practice (2017-2023). STUDY DESIGN: Regional primary care providers were surveyed regarding lipid screening practices. Key drivers were categorized (physician, patient, and system) with corresponding interventions. Pediatric cardiologists started offering lipid screening during regular visits by providing families with preventive cardiovascular education materials and lab phlebotomy testing. System redesign included educational posters, clinical intake protocol, physician counseling, electronic health record integration, and POC testing. Run charts and statistical process control charts measured screening rates and key processes. RESULTS: The primary care survey response rate was 32% (95/294); 97% supported pediatric cardiologists conducting routine lipid screening. Pediatric cardiology mean baseline lipid screening rate was 0%, increased to 7% with patient education, and to 61% after system redesign including POC testing. Screening rates among 1467 patients were similar across age groups (P = .98). More patients received lipid screening by POC (91.7%) compared with phlebotomy (8.3%). Lipid abnormalities detected did not differ by screening methodology (P = .49). CONCLUSION: Patient education, counseling, and POC testing improved adherence to national lipid screening guidelines, providing a possible model for primary care implementation.

Obstetric imaging practice characteristics associated with prenatal detection of critical congenital heart disease in a rural US region over 20 years.

OBJECTIVE: To identify clinical practice characteristics associated with the frequency of prenatal critical congenital heart disease (CCHD) detection (i.e., the number of liveborn infants with postnatally confirmed CCHD identified on prenatal sonography) over 20 years in a rural setting comprised of 11 primarily low-volume obstetric hospitals and the single tertiary academic hospital to which they refer. METHODS: This was a retrospective cohort study of all patients in the referral region with an initial prenatal and/or postnatal diagnosis of CCHD from 01/01/2002 to 12/31/2021. The frequency of prenatal CCHD detection at the time of an obstetric ultrasound was reported, as was the change in detection over time. Critical congenital heart disease detection was assessed as a function of cardiac lesion type, practice setting, and practice characteristics. RESULTS: There were 271 cases with a confirmed postnatal CCHD diagnosis, of which 49% were identified prenatally. The majority of community practices each averaged <10 CCHD cases in total over the study period. Prenatal detection at the tertiary academic hospital's obstetric ultrasound unit was 64%, compared to 22% at the combined referring community practices (p < 0.001), though CCHD detection improved over time in both settings. Professional accreditation by the American Institute of Ultrasound in Medicine, image interpretation by radiology or Maternal Fetal Medicine, and use of video clips of ventricular outflow tracts were associated with improved prenatal CCHD detection. CONCLUSIONS: Our data demonstrate the infrequency of CCHD cases at small-volume, rural hospitals and the substantial variation in prenatal CCHD detection across practice settings. Our methods allowed for the identification of practice characteristics associated with prenatal CCHD detection.

Dyslipidemia Among Adults With Congenital Heart Disease.

Background: Atherosclerotic disease is an important cause of morbidity among adults with congenital heart disease (CHD). Prevalence of dyslipidemia in this group is poorly described. Objectives: This study aimed to describe the prevalence of dyslipidemia among adults with CHD. Methods: A prospective, outpatient screening study was conducted among adults aged ≥18 years at 4 New England ambulatory congenital cardiology centers. Participants were surveyed regarding cardiovascular risk factors. Nonfasting fingerstick samples were obtained for analysis using a point-of-care lipid analyzer. Results: Lipid screening was completed on 186 participants (median age 30 [range 18-71] years, 50% female). Eighteen (10%) had simple CHD anatomy, and 63 (34%) had complex anatomy. Only 15% of 169 respondents reported history of high cholesterol. Eighty-five (46%) participants met National Cholesterol Education Program definition of dyslipidemia with 60 (32%), 62 (34%), and 37 (20%) having low high-density lipoprotein cholesterol (HDL-C <40 mg/dL), high non-HDL-C (≥130 mg/dL), and high total cholesterol (TC ≥200 mg/dL), respectively. TC was higher among participants with simple CHD than among those with moderate and complex lesions (mean 178.4 ± 48.7 vs 170.1 ± 35.0 vs 157.6 ± 34.5 mg/dL; P = 0.03). HDL-C was lower among participants with complex CHD than among those with simple and moderate lesions (mean 44.1 ± 13.5 vs 46.9 ± 12.5 vs 49.8 ± 15.3 mg/dL; P = 0.05). Conclusions: Dyslipidemia is highly prevalent among our cohort of adults with CHD, despite <15% reporting a prior diagnosis. Low HDL-C was more common in complex CHD, and high TC was more common in simple or moderate CHD. Lipid screening should be part of preventive health maintenance for all adults with CHD.

Variation of Patent Ductus Arteriosus Treatment in Very Low Birth Weight Infants.

BACKGROUND: Patent ductus arteriosus (PDA) treatment is common among very low birth weight (VLBW) infants. Given limitations in evidence, controversy exists regarding treatment risks and benefits. In this study, we describe PDA treatment trends and variation in a large, US, multicenter VLBW infant cohort. METHODS: Data were collected through Vermont Oxford Network on 291 292 VLBW infants born 2012-2019 at 806 US NICUs. PDA diagnosis and treatment rates, further categorized as pharmacologic, invasive, or combined, were determined. NICUs were classified as capable versus noncapable of invasive PDA treatment. Infant and hospital characteristics were examined by NICU type and treatment quartile. Geographic NICU distribution and treatment rates were described in 9 US census divisions. RESULTS: Of all infants, 24.6% were diagnosed with and 20.5% were treated for PDA. Diagnosis and treatment rates decreased over the study period. Treatment was predominantly pharmacologic. Treatment rates varied widely among NICUs (0% to 67%) despite similar infant characteristics. The median treatment rate was higher at NICUs capable of pharmacologic and invasive treatment (20.3%, interquartile range 13.3-28.6) than at NICUs capable of only pharmacologic treatment (8.9%, interquartile range 2.9-14.8). Treatment rates were highest in the northeast and lowest in the west. Invasive treatment was more common in the west. CONCLUSIONS: PDA diagnosis and treatment rates are trending downward. Wide variation exists in PDA treatment despite a largely uniform VLBW infant population. This variation correlates with differences in hospital treatment capabilities and geography. Further understanding of the effects of treatment disparity could aid in guiding clinical management.

Outcomes for Ectopia Cordis.

OBJECTIVES: To utilize a large multicenter neonatal cohort to describe survival and clinical outcomes of very low birth weight (VLBW) or preterm infants with ectopia cordis. STUDY DESIGN: Data were prospectively collected on 2 211 262 infants (born 2000-2017) from 845 US centers. Both VLBW (401-1500 g or 22-29 weeks of gestation) and non-VLBW (>1500 g and >29 weeks) infants had diagnoses or anatomic descriptors consistent with ectopia cordis and/or pentalogy of Cantrell. The primary outcome was neonatal survival, defined as hospital discharge or initial length of stay of ≥12 months. RESULTS: In total, 180 infants had ectopia cordis, 135 (76%) with findings of pentalogy of Cantrell. VLBW infants comprised 52% of the population. VLBW mortality was 96% with 79% dying within 12 hours, compared with 59% and 36%, respectively, for non-VLBW. One-third of VLBW infants received life support compared with 65% of non-VLBW. Surgery was reported for 34% of VLBW and 68% of non-VLBW infants. Congenital heart disease was reported in 8% of VLBW and 36% of non-VLBW, with conotruncal abnormalities most common. Survival exceeded 50% for infants >2500 g and >37 weeks of gestation. CONCLUSIONS: Survival of VLBW infants with ectopia cordis was poor and substantially worse compared with non-VLBW, with notable discrepancies in resuscitative efforts and surgical interventions. Although gestational age and weight strongly influence current survival, more detailed information regarding the severity of cardiac and noncardiac abnormalities is required to fully determine prognosis and inform counseling.

Identification of Critical Congenital Heart Disease in Vermont: The Role of Universal Pulse Oximetry Screening in a Rural State.

OBJECTIVE: Pulse oximetry screening (POS) is becoming the standard of care in screening for critical congenital heart disease (CCHD). Our objective was to characterize the historical diagnostic pattern in a rural tertiary care children's hospital and explore how universal POS might affect morbidity, mortality, and care delivery. PATIENTS AND METHODS: We identified patients <6 months of age in the Vermont Children's Hospital echocardiogram database with CCHD diagnosed between 2002 and 2011. Charts were reviewed to characterize timing of diagnosis, course, and outcome. The medical examiner was consulted to identify deaths due to undetected CCHD during the study period. RESULTS: Of 60 329 live births, 73 (0.12 %) were diagnosed with CCHD. Of these, 31 (42%) were diagnosed prenatally, 34 (47%) were diagnosed by clinical examination in the nursery, 7 (9.6%) were diagnosed after nursery discharge, and 1 (1.4%) was born at home. The 8 patients not diagnosed by prenatal ultrasound or in the nursery were considered cases of undetected CCHD. Three had normal oxygen saturation (>95%) at diagnosis. Three presented with cardiovascular compromise. None died and all were well at the most recent follow-up. Review of autopsy reports from patients <6 months found no deaths from undetected CCHD during the study period. CONCLUSIONS: Over a 10-year period at our institution, universal POS could have identified 5 patients with undetected CCHD, possibly avoiding the need for resuscitation in 3. Examination of local diagnostic data may affect the cost/benefit considerations of universal POS initiatives in a setting of limited healthcare resources.

Distribution of and mortality from serious congenital heart disease in very low birth weight infants.

OBJECTIVE: To characterize serious congenital heart disease in very low birth weight (VLBW) infants (born at <1500 g or a gestational age of 22-29 weeks) in a large, international database. PATIENTS AND METHODS: We analyzed a database of 99 786 VLBW infants born or treated at 703 NICUs between calendar years 2006 and 2007. We defined serious congenital heart disease as 1 of 14 specific lesions or any other structural congenital heart disease that required surgical or medical treatment by initial hospital discharge or by the age of 1 year. We reviewed records for all infants with cardiac diagnoses and other genetic syndromes and associations to determine which had serious congenital heart disease. We excluded nonstructural disease as well as isolated and untreated atrial or ventricular septal defects. We determined the frequency of serious congenital heart disease, compared overall mortality rates of those with and without serious congenital heart disease, and determined the distribution of specific lesions and mortality for each diagnosis. RESULTS: Of 99 786 VLBW infants studied, 893 had serious congenital heart disease (8.9 per 1000). The most common lesions were tetralogy of Fallot (n = 166 [18.6% of those with serious congenital heart disease]), aortic coarctation (n = 103 [11.5%]), complete atrioventricular canal (n = 81 [9.1%]), pulmonary atresia (n = 73 [8.2%]), and double-outlet right ventricle (n = 68 [7.6%]). The mortality rate of those with serious congenital heart disease was 44%, compared with 12.7% in those without serious congenital heart disease (P < .0001). CONCLUSIONS: Serious congenital heart disease is probably more frequent in VLBW infants treated in NICUs than in the general live-born population, and the distribution reflects lesions associated with extracardiac malformations. VLBW infants with serious congenital heart disease have higher a mortality rate than those without, independent of other risk factors.

Pretransport and posttransport characteristics and outcomes of neonates who were admitted to a cardiac intensive care unit.

OBJECTIVE: The objective for this study was to characterize the impact and the safety of transporting neonates with known or suspected cardiac abnormalities. METHODS: We reviewed retrospectively the charts and computerized records of 192 admissions to a cardiac ICU in 2002. Patients were included when they were < 28 days of age at admission and were transported from adjacent obstetric facilities (local N = 70) or other inpatient medical facilities (transport N = 122). Demographic, clinical, pharmacologic, laboratory, and diagnostic information was obtained before transport (when available) and within 3 hours of arrival. Arrival status was considered optimal when measured metabolic and clinical parameters all were within range. Outcome variables included days on ventilator, days in ICU, days in hospital, and death. RESULTS: Of local admissions, 31 (44%) patients had 61 suboptimal arrival values, including pH < 7.25 (n = 11), saturation < 70% (n = 12), and temperature < 36 degrees C (n = 9). There were 69 undocumented values in 39 patients. Of transported patients, 55 (45%) had 86 suboptimal arrival values, including pH < 7.25 (n = 8), saturation < 70% (n = 14), and temperature < 36 degrees C (n = 13). There were 98 undocumented values in 53 patients. No in-transport deaths or catastrophic events occurred. Local admissions were more likely to have a prenatal diagnosis of heart disease and had more complex disease and higher mortality. Other outcome parameters were not significantly different between the 2 groups. Low admission arterial saturation, pH, and core temperature were not correlated with adverse outcome measures. CONCLUSIONS: Although we did not encounter major transport complications, opportunities exist to optimize arrival status and improve surveillance and documentation.

A novel method for selective delivery of drugs to the pulmonary arteries.

Drug treatment of pulmonary hypertension may be limited by systemic hypotension. Selective action of a vasodilator drug in pulmonary arteries could be achieved by administering a vasodilator gas into systemic venous blood so that it dilates pulmonary arteries before immediate first-pass elimination via exhalation. This article presents in vivo data to show that a pharmacologically active gas can be delivered safely into systemic venous blood where it has a distribution pattern and physiologic effects similar to those observed when the gas is inhaled into pulmonary venous (systemic arterial) blood. This is a first step toward development of first-pass pulmonary clearance as a mechanism to concentrate drugs in pulmonary arteries.

Prenatal role of the ductus arteriosus in absent pulmonary valve syndrome.

Absent pulmonary valve with ventricular septal defect is associated with ductal agenesis and markedly dilated main and branch pulmonary arteries. The less common variant with intact ventricular septum generally exhibits a patent ductus and smaller branch pulmonary arteries, and may be associated with tricuspid atresia. We identified 7 patients with the prenatal diagnosis of absent pulmonary valve, 5 with ventricular septal defect (Group 1) and 2 with an intact ventricular septum (Group 2). Imaging, color Doppler, and pulsed-Doppler recordings were analyzed. The branch and main pulmonary arteries were measured and expressed as a ratio with the descending aorta. Pulmonary regurgitation time (PRT) and diastolic acceleration time (DAT) were derived, and DAT/PRT was calculated to characterize diastolic pulmonary flow. Group 1 patients all had a large ventricular septal defect, normal biventricular size and function, and dilated main and branch pulmonary arteries. Group 2 patients had dilated main but smaller branch pulmonary arteries, moderate right ventricular dilation with severe dysfunction, and limited or absent tricuspid inflow. Group 1 demonstrated shorter acceleration time and earlier peak velocity, resulting in a smaller DAT/RT ratio. We speculate that free communication between the fetal aorta and the ventricles may limit atrial inflow and elevate diastolic pressure, affecting cardiac output, ventricular function, and atrioventricular valve development. With an intact ventricular septum, these physiologic and anatomic repercussions are limited to the right ventricle, but with a ventricular septal defect, both ventricles would experience similar consequences and cardiac performance could be critically impaired.