Follow-Up of Secundum ASD, Muscular VSD, or PDA Diagnosed During Neonatal Hospitalization.

The ideal follow-up of neonates who have a secundum atrial septal defect (ASD), muscular ventricular septal defect (VSD), or patent ductus arteriosus (PDA) remains uncertain. Newborns with findings limited to a secundum ASD, muscular VSD, and/or PDA on their neonatal hospitalization discharge echocardiogram and at least one outpatient follow-up echocardiogram performed between 9-1-17 and 9-1-21 were evaluated and patient follow-up assessed through 9-1-23. 95 infants met inclusion criteria. 43 infants had a secundum ASD, 41 had a muscular VSD, and 54 had a PDA at newborn hospital discharge. 39/95 had more than one intracardiac shunt. 56 were discharged from care, 26 were still in follow-up and 13 were lost to recommended follow-up. No patients received intervention during the follow-up period of 2 to 6 years. Of the 43 infants with a secundum ASD, 16 (37.2%) had demonstrated closure of the ASD, and 13 (30.2%) were discharged from care with an ASD < 3.5 mm in diameter. 3/43 infants with secundum ASD had a defect with a diameter of more than 5 mm at their last echocardiogram. No infant discharged from their neonatal hospitalization with a secundum ASD, muscular VSD, or PDA needed any intervention from 2 to 6 years of follow-up. Ongoing follow-up with echocardiography of those infants with a secundum ASD is of greater value than of those with muscular VSD or PDA.

Preventive Medicine in Pediatric Cardiology Practice.

OBJECTIVES: The objective of the study was to assess contemporary practice patterns of pediatric cardiologists with respect to cholesterol disorders and smoking-related illness. STUDY DESIGN: We sent 2 anonymous surveys to the members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and the Pediheart online community. The surveys addressed training in and management of cholesterol disorders and smoking-related illness. RESULTS: There were 97 responses to the cholesterol disorders survey. A total of 51.6% reported little or no formal training on cholesterol disorders. A total of 56.4% underestimated the prevalence of familial hypercholesterolemia by at least twofold. A total of 54.7% were at least somewhat comfortable prescribing statins. In 5 clinical vignettes, respondents frequently gave clinical recommendations in line with the 2019 American Heart Association guidelines although both undertreatment and overtreatment were recommended. There were 90 responses to the survey on smoking-related illness. Little or no formal training in nicotine addiction (52.3%) or smoking cessation (60.5%) was reported by respondents. Respondents screened for tobacco use in less than a one-third of hospitalizations and less than two-thirds of outpatient clinic visits. Screening for exposure to secondhand smoke was even less common. Twenty-seven percent of respondents never recommend a household smoking ban for their patients. A total of 83.3% were uncomfortable prescribing medications for their patients for smoking cessation, and 65.5% rarely or never refer patients for smoking cessation assistance. CONCLUSION: Although positioned to address the childhood origins of adult heart disease, those cardiologists surveyed placed a limited emphasis on cholesterol disorders and smoking-related disease in their clinical practice.

A Survey on the Management of Anomalous Aortic Origins of the Coronary Arteries.

The management of patients with an anomalous aortic origin of a coronary artery (AAOCA) remains controversial despite the publication of the 2017 American Association for Thoracic Surgery (AATS) expert guidelines. We surveyed the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and the Pediheart.net online community regarding their care of patients with anomalous origins of the right or left coronary from the opposite cusp with inter-arterial courses and compared them to the AATS guidelines. We received 111 complete responses. Four notable variations from the AATS recommendations were identified. Respondents were more likely to use ECG exercise testing than the stress imaging recommended in the AATS guidelines. For a 16-year-old with AAOCA, recommendations for surgery generally followed the AATS guidelines. However, for asymptomatic left AAOCA without signs of ischemia on stress imaging, only 69.4% felt surgery was appropriate or somewhat appropriate. In the setting of a 16-year-old with right AAOCA free from signs or symptoms of ischemia, respondents were more likely to recommend surgery if the patient was a competitive athlete, a topic not directly addressed in the AATS guidelines. After surgical treatment of AAOCA, only 24% of respondents recommended lifelong antiplatelet therapy despite recommendations for this in the AATS guidelines. Respondents recommendations were generally consistent with the 2017 AATS guidelines but with important variations in the use of stress imaging, indications for surgery in asymptomatic left AAOCA, the impact of identification as a competitive athlete and duration of postoperative antiplatelet therapy.

A Survey of Pediatric Cardiologists Regarding Non-emergent Echocardiographic Findings in Asymptomatic Newborns.

Echocardiography is often used to assess for significant heart disease in newborns, but there is little information on how to best manage non-emergent echocardiographic findings in asymptomatic babies. We reviewed the literature regarding the natural history of a patent foramen ovale (PFO), atrial septal defect (ASD), ventricular septal defect (PFO), and patent ductus arteriosus (PDA). We surveyed pediatric cardiologists to determine their recommendations for ten echocardiographic findings (PFO, 3 mm ASD, 6 mm ASD, small muscular VSD, small perimembranous VSD, small PDA with left to right shunting, small PDA with bidirectional shunting, trivial mitral insufficiency, trivial aortic insufficiency, and a normally functioning bicuspid aortic valve) in an asymptomatic one-day old with a heart murmur. These ten findings were set in three clinical contexts (an otherwise normal term baby, a baby born at 34 weeks gestation, and a term baby with trisomy 21). 149 survey responses were evaluated. Follow-up was universally recommended for those babies with a 6 mm ASD, a perimembranous VSD and a bicuspid aortic valve and frequently recommended for newborns with a 3 mm ASD, a small muscular VSD and any PDA. Depending on the context, between 17.5 and 23% of respondents recommended follow-up for an isolated PFO. Follow-up typically included repeat echocardiography. Some form of follow-up, typically with repeat echocardiography was recommended for many asymptomatic day-old newborns who had echocardiographic findings which were unlikely to be clinically significant. Given the wide range of recommendations, a consensus guideline could prove useful to clinicians.

Variation in management of paediatric isolated bicuspid aortic valve: current practice survey.

BACKGROUND: Prior to the recent release of appropriate use criteria for imaging valvulopathies in children, follow-up of valvular lesions, including isolated bicuspid aortic valve, was not standardised. We describe current follow up, treatment, and intervention strategies for isolated bicuspid aortic valve with varying degrees of stenosis, regurgitation, and dilation in children up to 18 years old and compare them with newly released appropriate use criteria. METHODS: Online survey was sent to members of the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery and PediHeartNet. RESULTS: Totally, 106 responses with interpretable data were received. For asymptomatic patients with isolated BAV without stenosis, regurgitation, or dilation follow-up-intervals increased from 7+/-4 months in the newborn period to 28 +/- 14 months at 18 years of age. Respondents recommended more frequent follow-up for younger patients and those with greater disease severity. More than 80% of respondents treat aortic regurgitation or aortic dilation in the setting of bicuspid aortic valve medically. In general, intervention was recommended once stenosis or regurgitation became severe (stenosis of >4 m/s; regurgitation with LV Z score 4) regardless of age, but was not routinely recommended for younger children (newborn - age 6 years) with severe dilation. Exercise was restricted at 38+/-11 mmHg echocardiographic mean gradient. CONCLUSIONS: Current follow-up, treatment, and intervention strategies for isolated bicuspid aortic valve deviate from appropriate use criteria. Differences between the two highlight the need to better delineate the disease course, clarify recommendations for care, and encourage wider adoption of guidelines.

Critical Congenital Heart Disease Newborn Screening Implementation: Lessons Learned.

Introduction The purpose of this article is to present the collective experiences of six federally-funded critical congenital heart disease (CCHD) newborn screening implementation projects to assist federal and state policy makers and public health to implement CCHD screening. Methods A qualitative assessment and summary from six demonstration project grantees and other state representatives involved in the implementation of CCHD screening programs are presented in the following areas: legislation, provider and family education, screening algorithms and interpretation, data collection and quality improvement, telemedicine, home and rural births, and neonatal intensive care unit populations. Results The most common challenges to implementation include: lack of uniform legislative and statutory mandates for screening programs, lack of funding/resources, difficulty in screening algorithm interpretation, limited availability of pediatric echocardiography, and integrating data collection and reporting with existing newborn screening systems. Identified solutions include: programs should consider integrating third party insurers and other partners early in the legislative/statutory process; development of visual tools and language modification to assist in the interpretation of algorithms, training programs for adult sonographers to perform neonatal echocardiography, building upon existing newborn screening systems, and using automated data transfer mechanisms. Discussion Continued and expanded surveillance, research, prevention and education efforts are needed to inform screening programs, with an aim to reduce morbidity, mortality and other adverse consequences for individuals and families affected by CCHD.

Radiation Reduction Capabilities of a Next-Generation Pediatric Imaging Platform.

The aims of this study were to quantify patient radiation exposure for a single interventional procedure during transition from an adult catheterization laboratory to a next-generation imaging system with pediatric settings, and to compare this radiation data to published benchmarks. Radiation exposure occurs with any X-ray-directed pediatric catheterization. Technologies and imaging techniques that limit dose while preserving image quality benefit patient care. Patient radiation dose metrics, air kerma, and dose-area product (DAP) were retrospectively obtained for patients <20 kg who underwent patent ductus arteriosus (PDA) closure on a standard imaging system (Group 1, n = 11) and a next-generation pediatric imaging system (Group 2, n = 10) with air-gap technique. Group 2 radiation dose metrics were then compared to published benchmarks. Patient demographics, procedural technique, PDA dimensions, closure devices, and fluoroscopy time were similar for the two groups. Air kerma and DAP decreased by 65-70% in Group 2 (p values <0.001). The average number of angiograms approached statistical significance (p value = 0.06); therefore, analysis of covariance (ANCOVA) was conducted that confirmed significantly lower dose measures in Group 2. This degree of dose reduction was similar when Group 2 data (Kerma 28 mGy, DAP 199 µGy m(2)) was compared to published benchmarks for PDA closure (Kerma 76 mGy, DAP 500 µGy m(2)). This is the first clinical study documenting the radiation reduction capabilities of a next-generation pediatric imaging platform. The true benefit of this dose reduction will be seen in patients requiring complex and often recurrent catheterizations.

Pulse oximetry screening for critical congenital heart disease in planned out-of-hospital births.

OBJECTIVES: To describe the use of pulse oximetry screening (POS) for critical congenital heart disease (CCHD). STUDY DESIGN: This observational study of Wisconsin out-of-hospital births was performed from January to November, 2013. Licensed midwives, Amish birth attendants, and public health nurses were trained in the use of pulse oximetry to detect CCHD, supplied with pulse oximeters, and reported screening results and clinical outcomes. RESULTS: Results of POS in 440 newborns were reviewed; 173/440 births were from Amish or Mennonite communities. Prenatal ultrasonography was performed in less than one-half of the pregnancies and in only 13% of Amish and Mennonite women. A total of 432 babies passed the screening, 5 babies were incorrectly assigned to have passed or failed, and 3 babies failed the screening. Two of the babies who failed the screening were treated for sepsis and the third had congenital heart disease. There was 1 false negative result (coarctation of the aorta and ventricular septal defect). CONCLUSIONS: This study provides information on the use of POS for CCHD in out-of-hospital births and shows that POS can be successfully implemented outside the hospital setting. Although the failure rate in this small sample was higher than reported in studies of hospital births, those babies failing the screening had significant disease processes that were identified more rapidly because of the screening.

Blood pressure screening for critical congenital heart disease in neonates.

Pulse oximetry (POx) screening for critical congenital heart disease (CCHD) in neonates is less effective in identifying aortic arch obstruction than in detecting other forms of CCHD. This study was performed to assess the use of neonatal blood pressure (BP) screening to detect CCHD. A retrospective review of BP and POx measurements performed at the age of 24 h or before discharge in asymptomatic term neonates was undertaken. The charts of infants readmitted younger than 30 days with a diagnosis of CCHD also were reviewed to identify infants with a missed diagnosis. The screening process was completed for 10,012 of 10,436 infants. Because of an abnormal initial result, 164 neonates required a repeat screening (139 due to abnormal BP). A total of 12 infants failed the BP screening component, and 1 infant failed both the BP and Pox components. The average final right arm-to-leg BP gradient was 25 mmHg in these 13 babies. For nine infants, CCHD was excluded by echocardiography. Three patients were normal at their 1-year well-child exam, and one patient was lost to follow-up evaluation. No infants were identified who had been discharged home with a missed diagnosis of CCHD. Neonatal BP screening to detect CCHD was responsible for more inappropriately performed screenings, repeated screenings, and screening failures than the POx component of the screening protocol and had a highest possible positive predictive value of 1 in 13. These data suggests that BP screening at the time of routine newborn hospital discharge is of limited value in the detection of unrecognized CCHD.

Antibiotic Prophylaxis for Infective Endocarditis: A Survey of Practice Among Pediatric Cardiology Providers.

The 2007 American Heart Association (AHA) guidelines limited antibiotic prophylaxis (AP) for infective endocarditis (IE) to fewer patients with predisposing cardiac conditions (PCC). We surveyed the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery (AAP SOCCS) on their recommendations for AP for a number of PCC and procedures. We report on those 173 respondents who follow the 2007 AHA guidelines. AP rates for high-risk PCCs clearly meeting AHA criteria ranged from 70.5-89.8%. Conversely, for PCCs which did not meet AHA criteria, prescribing rates varied from <1% to 29.5%. PCC for which AP indication was unclear per guidelines, AP rates similarly varied from 9.9-39.8%. Similar variability is noted in AP for various procedures in setting of high-risk PCC. There is variability in AP prescribing practices among pediatric cardiologists based on both underlying PCC and noncardiac procedures in the setting of underlying cardiac disease.

Etiology of chest pain in children and adolescents referred to cardiology clinic.

OBJECTIVE: To determine the proportion of children referred to pediatric cardiology clinic for chest pain diagnosed with a cardiac cause for the pain. DESIGN: Medical records of patients evaluated for chest pain at the University of Wisconsin Children's Hospital from 2004 to 2006 were reviewed, including the studies performed and final diagnosis. RESULTS: A total of 135 patients, including 78 boys, ranging from 4 to 17 years were evaluated. Eighty-four (62%) patients had pain for at least 1 month. All patients had an electrocardiogram (ECG), and most had an echocardiogram performed. Only 1 patient (0.7%) was found to have a cardiac cause for the pain. In 6 patients (4.3%), there was possible supraventricular tachycardia based on history, but no evidence of abnormality on subsequent testing. Ninety-five percent of the patients were diagnosed with noncardiac chest pain. CONCLUSION: The incidence of cardiac chest pain in our study population is less than previously reported. Many patients were referred to cardiology clinic despite having had normal testing by the referring physician. Primary care physicians should be reassured when patients have a normal history, physical examination, and testing. Referral to pediatric cardiology usually is not necessary under these circumstances.

Transpulmonary passage of 99mTc macroaggregated albumin in healthy humans at rest and during maximal exercise.

We have demonstrated that 50-mum-diameter arteriovenous pathways exist in isolated, healthy human and baboon lungs, ventilated and perfused under physiological pressures. These findings have been confirmed and extended by demonstrating the passage of 25-microm microspheres through the lungs of exercising dogs, but not at rest. Determination of blood flow through these large-diameter intrapulmonary arteriovenous pathways would be an important first step to establish a physiological role for these vessels. Currently, we sought to estimate blood flow through these arteriovenous pathways using technetium-99m ((99m)Tc)-labeled macroaggregated albumin (MAA) in healthy humans at rest and during maximal treadmill exercise. We hypothesized that the percentage of (99m)Tc MAA able to traverse the pulmonary circulation (%transpulmonary passage) would increase during exercise. Seven male subjects without patent foramen ovale were injected with (99m)Tc MAA at rest on 1 day and during maximal treadmill exercise on a separate day (>6 days). Within 5 min after injection, subjects began whole body imaging in the supine position. Six of the seven subjects showed an increase in transpulmonary passage of MAA with maximal exercise. Using two separate analysis methods, percent transpulmonary passage significantly increased with exercise from baseline to absolute values of 1.2 +/- 0.8% (P = 0.008) and 1.3 +/- 1.0% (P = 0.016), respectively (means +/- SD; paired t-test). We conclude that MAA may be traversing the pulmonary circulation via large-diameter intrapulmonary arteriovenous conduits in healthy humans during exercise. Recruitment of these pathways may divert blood flow away from pulmonary capillaries during exercise and compromise the lung's function as a biological filter.

Hyperoxia prevents exercise-induced intrapulmonary arteriovenous shunt in healthy humans.

The 100% oxygen (O(2)) technique has been used to detect and quantify right-to-left shunt for more than 50 years. The goal of this study was to determine if breathing 100% O(2) affected intrapulmonary arteriovenous pathways during exercise. Seven healthy subjects (3 females) performed two exercise protocols. In Protocol I subjects performed an incremental cycle ergometer test (60 W + 30 W/2 min; breathing room air, FIO2 = 0.209) and arteriovenous shunting was evaluated using saline contrast echocardiography at each stage. Once significant arteriovenous shunting was documented (bubble score = 2), workload was held constant for the remainder of the protocol and FIO2 was alternated between 1.0 (hyperoxia) and 0.209 (normoxia) as follows: hyperoxia for 180 s, normoxia for 120 s, hyperoxia for 120 s, normoxia for 120 s, hyperoxia for 60 s and normoxia for 120 s. For Protocol II, subjects performed an incremental cycle ergometer test until volitional exhaustion while continuously breathing 100% O(2). In Protocol I, shunting was seen in all subjects at 120-300 W. Breathing oxygen for 1 min reduced shunting, and breathing oxygen for 2 min eliminated shunting in all subjects. Shunting promptly resumed upon breathing room air. Similarly, in Protocol II, breathing 100% O(2) substantially decreased or eliminated exercise-induced arteriovenous shunting in all subjects at submaximal and in 4/7 subjects at maximal exercise intensities. Our results suggest that alveolar hyperoxia prevents or reduces blood flow through arteriovenous shunt pathways.

Intrapulmonary shunting and pulmonary gas exchange during normoxic and hypoxic exercise in healthy humans.

Exercise-induced intrapulmonary arteriovenous shunting, as detected by saline contrast echocardiography, has been demonstrated in healthy humans. We have previously suggested that increases in both pulmonary pressures and blood flow associated with exercise are responsible for opening these intrapulmonary arteriovenous pathways. In the present study, we hypothesized that, although cardiac output and pulmonary pressures would be higher in hypoxia, the potent pulmonary vasoconstrictor effect of hypoxia would actually attenuate exercise-induced intrapulmonary shunting. Using saline contrast echocardiography, we examined nine healthy men during incremental (65 W + 30 W/2 min) cycle exercise to exhaustion in normoxia and hypoxia (fraction of inspired O(2) = 0.12). Contrast injections were made into a peripheral vein at rest and during exercise and recovery (3-5 min postexercise) with pulmonary gas exchange measured simultaneously. At rest, no subject demonstrated intrapulmonary shunting in normoxia [arterial Po(2) (Pa(O(2))) = 98 +/- 10 Torr], whereas in hypoxia (Pa(O(2)) = 47 +/- 5 Torr), intrapulmonary shunting developed in 3/9 subjects. During exercise, approximately 90% (8/9) of the subjects shunted during normoxia, whereas all subjects shunted during hypoxia. Four of the nine subjects shunted at a lower workload in hypoxia. Furthermore, all subjects continued to shunt at 3 min, and five subjects shunted at 5 min postexercise in hypoxia. Hypoxia has acute effects by inducing intrapulmonary arteriovenous shunt pathways at rest and during exercise and has long-term effects by maintaining patency of these vessels during recovery. Whether oxygen tension specifically regulates these novel pathways or opens them indirectly via effects on the conventional pulmonary vasculature remains unclear.

Heart murmurs and echocardiography findings in the normal newborn nursery.

OBJECTIVE: To determine the clinical findings and management implications of echocardiograms performed on infants with murmurs in the nursery. DESIGN: Retrospective cohort study conducted from January 2008 through December 2015. Patients in the study were followed by chart review for up to 5 years. In addition, a survey of nursery providers was conducted in February 2016. SETTING: A single community hospital associated with a university. PATIENTS: All 26 573 infants who received care in the normal newborn nursery were eligible for inclusion in the study. Infants with echocardiograms were analyzed. The survey was sent by e-mail to all 135 physicians who work in the nursery. OUTCOME MEASURES: The primary outcomes include the specific findings on echocardiogram and whether the findings required an acute change in management, outpatient follow up, or were incidental findings. The primary survey question was how physicians would manage an otherwise asymptomatic newborn with a heart murmur. RESULTS: Four hundred ninety-nine infants had echocardiograms, and over the study period the utilization of echocardiography increased from 1.02% to 2.56% (P < .001) of all infants. Three hundred fifty-four babies had echocardiography performed because of a heart murmur. One hundred sixty-three (46.0%) of these echocardiograms were normal and 160 (45.2%) had findings that did not require additional care. Twenty-three neonates (6.5%) had echocardiographic findings that necessitated outpatient follow-up and 8 neonates (2.3%) required neonatal intensive care due to the findings on their echocardiogram. In total, 14 infants (4%) would go on to require heart surgery or interventional cardiac catheterization. 63/135 (47%) physicians completed the survey, with wide variations in the management of newborns with heart murmurs. CONCLUSIONS: The use of echocardiography in the normal newborn nursery has increased with time despite improved prenatal detection of heart disease and the use of pulse oximetry screening, and identifies significant heart disease in a small but important number of infants.

Excessive gas exchange impairment during exercise in a subject with a history of bronchopulmonary dysplasia and high altitude pulmonary edema.

A 27-year-old male subject (V(O2 max)), 92% predicted) with a history of bronchopulmonary dysplasia (BPD) and a clinically documented case of high altitude pulmonary edema (HAPE) was examined at rest and during exercise. Pulmonary function testing revealed a normal forced vital capacity (FVC, 98.1% predicted) and diffusion capacity for carbon monoxide (D(L(CO)), 91.2% predicted), but significant airway obstruction at rest [forced expiratory volume in 1 sec (FEV(1)), 66.5% predicted; forced expiratory flow at 50% of vital capacity (FEF(50)), 34.3% predicted; and FEV(1) /FVC 56.5%] that was not reversible with an inhaled bronchodilator. Gas exchange worsened from rest to exercise, with the alveolar to arterial P(O2) difference (AaD(O2)) increasing from 0 at rest to 41 mmHg at maximal normoxic exercise (VO(2) = 41.4 mL/kg/min) and from 11 to 31 mmHg at maximal hypoxic exercise (VO(2) = 21.9 mL/kg/min). Arterial P(O2) decreased to 67.8 and 29.9 mmHg at maximal normoxic and hypoxic exercise, respectively. These data indicate that our subject with a history of BPD is prone to a greater degree of exercise-induced arterial hypoxemia for a given VO(2) and F(I(O2)) than healthy age-matched controls, which may increase the subject's susceptibility to high altitude illness.

Development of quality metrics for ambulatory pediatric cardiology: Chest pain.

OBJECTIVE: As part of the American College of Cardiology Adult Congenital and Pediatric Cardiology Section effort to develop quality metrics (QMs) for ambulatory pediatric practice, the chest pain subcommittee aimed to develop QMs for evaluation of chest pain. DESIGN: A group of 8 pediatric cardiologists formulated candidate QMs in the areas of history, physical examination, and testing. Consensus candidate QMs were submitted to an expert panel for scoring by the RAND-UCLA modified Delphi process. Recommended QMs were then available for open comments from all members. PATIENTS: These QMs are intended for use in patients 5-18 years old, referred for initial evaluation of chest pain in an ambulatory pediatric cardiology clinic, with no known history of pediatric or congenital heart disease. RESULTS: A total of 10 candidate QMs were submitted; 2 were rejected by the expert panel, and 5 were removed after the open comment period. The 3 approved QMs included: (1) documentation of family history of cardiomyopathy, early coronary artery disease or sudden death, (2) performance of electrocardiogram in all patients, and (3) performance of an echocardiogram to evaluate coronary arteries in patients with exertional chest pain. CONCLUSIONS: Despite practice variation and limited prospective data, 3 QMs were approved, with measurable data points which may be extracted from the medical record. However, further prospective studies are necessary to define practice guidelines and to develop appropriate use criteria in this population.