Elective and non-elective endomyocardial biopsy in heart transplant patients and procedural outcomes: An IMPACT registry analysis.

BACKGROUND: Endomyocardial biopsies are standard of care for transplant surveillance, however the procedural risks are not well established, especially in children. The purpose of the study was therefore to assess procedural risks and outcomes associated with elective (surveillance) biopsies and non-elective (clinically indicated) biopsies. METHODS: We used the NCDR IMPACT registry database for this retrospective analysis. Patients undergoing an endomyocardial biopsy were identified using the procedural code, with a diagnosis of heart transplantation required. Data regarding indication, hemodynamics, adverse events and outcomes was gathered and analyzed. RESULTS: A total of 32 547 endomyocardial biopsies were performed between 2012-2020; 31 298 (96.5%) elective and 1133 (3.5%) were non-elective biopsies. Non-elective biopsy was more commonly performed in infants and in those above 18 years of age, in female and in Black race patients and in those with non-private insurance (all p < .05) and showed hemodynamic derangements. Overall rate of complications was low. Combined major adverse events were more common in non-elective patients, with sicker patient profile, use of general anesthesia and femoral access with overall decline in these events over time. CONCLUSIONS: This large-scale analysis shows safety of surveillance biopsies and that non-elective biopsies carry a small but significant risk of major adverse event. Patient profile impacts the safety of the procedure. These data may serve as important comparison point for newer non-invasive tests and for bench marking, especially in children.

Current Postlaunch Implementation of State Mandates of Newborn Screening for Critical Congenital Heart Disease by Pulse Oximetry in U.S. States and Hospitals.

OBJECTIVE: Our objective was to gauge adherence to nationally endorsed protocols in implementation of pulse oximetry (POx) screening for critical congenital heart disease (CCHD) in infants after mandate by all states and to assess associated characteristics. STUDY DESIGN: Between March and October 2019, an online questionnaire was administered to nurse supervisors who oversee personnel conducting POx screening. The questionnaire used eight questions regarding performance and interpretation of screening protocols to measure policy consistency, which is adherence to nationally endorsed protocols for POx screening developed by professional medical societies. Multilevel linear regression models evaluated associations between policy consistency and characteristics of hospitals and individuals, state of hospital location, early versus late mandate adopters, and state reporting requirements. RESULTS: Responses from 189 nurse supervisors spanning 38 states were analyzed. Only 17% received maximum points indicating full policy consistency, and 24% selected all four options for potential hypoxia that require a repeat screen. Notably, 33% did not recognize ≤90% SpO2 as an immediate failed screen and 31% responded that an infant with SpO2 of 89% in one extremity will be rescreened by nurses in an hour rather than receiving an immediate physician referral. Lower policy consistency was associated with lack of state reporting mandates (beta = -1.23 p = 0.01) and early adoption by states (beta = -1.01, p < 0.01). CONCLUSION: When presented with SpO2 screening values on a questionnaire, a low percentage of nurse supervisors selected responses that demonstrated adherence to nationally endorsed protocols for CCHD screening. Most notably, almost one-third of respondents did not recognize ≤90% SpO2 as a failed screen that requires immediate physician follow-up. In addition, states without reporting mandates and early adopter states were associated with low policy consistency. Implementing state reporting requirements might increase policy consistency, but some inconsistency may be the result of unique protocols in early adopter states that differ from nationally endorsed protocols. KEY POINTS: · Low adherence to nationally endorsed protocols.. · Inconsistent physician follow-up to hypoxia.. · Reporting improved consistency with national policy..

Cardiac Effects of COVID-19 Infection, MIS-C, and the Vaccine in Infants and Children: What Is Known and Future Implications.

The cardiac effects of novel coronavirus disease 2019 (COVID-19) infection on the pediatric heart has become an area of particular interest as elevated cardiac enzymes and abnormalities on echocardiogram and electrocardiogram were seen in a portion of children affected by the virus. In this article, we review the cardiac manifestations of acute COVID-19 infection, multisystem inflammatory syndrome in children, and postvaccine myocarditis. The limited research on the effects of COVID-19 on neonates and infants is also reported. KEY POINTS: · Cardiac involvement from MIS-C is much higher than the risk of COVID-19 vaccine-induced myocarditis.. · Neonates and infants have overall been less affected by COVID-19 than adults and older children.. · At this point in time, there is limited research on the cardiac effects of COVID-19 in neonates..

Rethinking Pulse Oximetry Screening in the Level-IV Neonatal Intensive Care Unit.

OBJECTIVE: This study aimed to evaluate the outcomes of newborn pulse oximetry screening in a level IV, tertiary care neonatal intensive care unit (NICU). STUDY DESIGN: This is a retrospective cohort study of neonates who received newborn pulse oximetry screening after being admitted to a single-center, level-IV NICU between 2014 and 2021. Neonates with known critical congenital heart disease were excluded from the study. RESULTS: Of the 4,493 neonates who had pulse oximetry screening, there were three positive screens (fail rate of 0.067%, 0.67 per 1,000 screened). The average age of screening was 818 hours. There were no positive screens of newborns who were admitted during their initial birth hospitalization and were screened while off oxygen. There were no new diagnoses of critical congenital heart disease (true positives) and there were no known false negatives. CONCLUSION: The results bring into question whether pulse oximetry screening with the current AAP-endorsed algorithm should be re-evaluated for a level-IV NICU at a children's hospital. However, the results may not be generalizable to other NICU's where echocardiography and prenatal echocardiograms are not as readily available. KEY POINTS: · Pulse oximetry has been shown to be effective in decreasing delayed diagnosis of critical congenital heart disease (CCHD); however, there are limited prior studies on newborn pulse oximetry in the NICU.. · In our study of over 4,000 neonates admitted to a level IV tertiary care NICU, there were no true positives (no new diagnoses of CCHD).. · Special considerations may be needed for pulse oximetry screening in the NICU setting..

Global, regional, and national time trends in mortality for congenital heart disease, 1990-2019: An age-period-cohort analysis for the Global Burden of Disease 2019 study.

BACKGROUND: Congenital heart disease (CHD) is the leading cause of morbidity and mortality from birth defects worldwide. We report an overview of trends in CHD mortality in 204 countries and territories over the past 30 years and associations with age, period, and birth cohort. METHODS: Cause-specific CHD mortality estimates were derived from the Global Burden of Disease 2019 study. We utilised an age-period-cohort model to estimate overall annual percentage changes in mortality (net drifts), annual percentage changes from 0 to 4 to 65-69 years (local drifts), period and cohort relative risks (period/cohort effects) between 1990 and 2019. This approach allows for the examination and differentiation of age, period, and cohort effects in the mortality trends, with the potential to identify disparities and treatment gaps in cardiac care. FINDINGS: CHD is the leading cause of deaths from non-communicable diseases (NCDs) in those under 20 years. Global CHD deaths in 2019 were 217,000 (95% uncertainty interval 177,000-262,000). There were 129 countries with at least 50 deaths. India, China, Pakistan, and Nigeria had the highest mortality, accounting for 39.7% of deaths globally. Between 1990 and 2019, the net drift of CHD mortality ranged from -2.41% per year (95% confidence interval [CI] -2.55, -2.67) in high Socio-demographic Index (SDI) countries to -0.62% per year (95% CI: -0.82, -0.42) in low-SDI countries. Globally, there was an emerging transition in the age distribution of deaths from paediatric to adult populations, except for an increasing trend of mortality in those aged 10-34 years in Mexico and Pakistan. During the past 30 years, favourable mortality reductions were generally found in most high-SDI countries like South Korea (net drift = -4.0% [95% CI -4.8 to -3.1] per year) and the United States (-2.3% [-2.5 to -2.0]), and also in many middle-SDI countries like Brazil (-2.7% [-3.1 to 2.4]) and South Africa (-2.5% [-3.2 to -1.8]). However, 52 of 129 countries had either increasing trends (net drifts ≥0.0%) or stagnated reductions (≥-0.5%) in mortality. The relative risk of mortality generally showed improving trends over time and in successively younger birth cohorts amongst high- and high-middle-SDI countries, with the exceptions of Saudi Arabia and Kazakhstan. 14 middle-SDI countries such as Ecuador and Mexico, and 16 low-middle-SDI countries including India and 20 low-SDI countries including Pakistan, had unfavourable or worsening risks for recent periods and birth cohorts. INTERPRETATION: CHD mortality is a useful and accessible indicator of trends in the provision of congenital cardiac care both in early childhood and across later life. Improvements in the treatment of CHD should reduce the risk for successively younger cohorts and shift the risk for all age groups over time. Although there were gains in CHD mortality globally over the past three decades, unfavourable period and cohort effects were found in many countries, raising questions about adequacy of their health care for CHD patients across all age groups. These failings carry significant implications for the likelihood of achieving the Sustainable Development Goal targets for under-5 years and NCD mortality. FUNDING: Supported by the National Natural Science Foundation of China (81525002, 31971048, 82073573 to ZZ and HZ), Shanghai Outstanding Medical Academic Leader program (2019LJ22 to HZ), and Collaborative Innovation Program of Shanghai Municipal Health Commission (2020CXJQ01 to HZ), the Bill & Melinda Gates Foundation for the Global Burden of Disease Project (to NJK) and NHMRC fellowship administered through the University of Melbourne (to GCP).

Lessons Learned from Infants with Late Detection of Critical Congenital Heart Disease.

Late detection of critical congenital heart disease (CCHD) is multifactorial and ill defined. We investigated the results of pulse oximetry screening (POS) and points in the care chain that contribute to delayed detection of CCHD. The medical records of 13 infants with delayed detection at a single pediatric cardiac center between 2013 and 2016 were identified and reviewed. Left heart obstructive lesions were the most common diagnosis (n = 8; 62%) and included coarctation of the aorta (n = 6), interrupted aortic arch with ventricular septal defect (n = 1), and critical aortic stenosis (n = 1). Tetralogy of Fallot (TOF) (n = 2), truncus arteriosus (n = 1), pulmonary atresia with ventricular septal defect (n = 1), and total anomalous pulmonary venous drainage (n = 1) made up the remainder of the conditions. Routine prenatal care was reported in most infants (10/13). Infants with late detection had either a true negative POS (10/13 infants) or no POS performed (3/13 infants). At the time of detection, 5/6 (83%) infants with coarctation had normal pulse oximetry values, whereas 6/7 (86%) infants with other CCHD developed abnormal pulse oximetry values. At diagnosis, 11/13 (85%) infants had significant signs or symptoms of clinical deterioration; only 2 infants were completely asymptomatic. Late detection of CCHD is uncommon and multifactorial. Eliminating late detection is dependent upon improving detection on screening obstetrical ultrasounds, enforcement of universal POS, and attention to the neonatal physical exam.

The evolution of cardiac care for children in Washington, DC.

Cardiac surgery for CHD was pioneered in Washington, DC by Charles Hufnagel and Edgar Davis working at Georgetown University and Children's Hospital of the District of Columbia. Children's Hospital, now Children's National Hospital, had been established just 5 years after the end of the Civil War. In the 1950s, Davis and Hufnagel undertook many open-heart operations using the technique of surface cooling, hypothermia, and circulatory arrest. Hufnagel and Lewis Scott, who founded the cardiology department at Children's, were trained in Boston by Gross and Nadas. Judson Randolph, also a trainee of Gross, introduced cardiac surgery using cardiopulmonary bypass and established the General Pediatric Surgery department at Children's in the 1960s. The transition of hospital staffing from community-based private physicians to full-time hospital employees was often controversial but was complete by the turn of the millennium. The 21st century has seen continuing growth of the new Children's National Heart Institute and consolidation of several congenital cardiac programmes in Washington, DC.

Newborn Pulse Oximetry Screening at a Community Hospital: An 8-Year Experience.

OBJECTIVES: To evaluate newborn pulse oximetry screening (POS) outcomes at a large community hospital and the impact of the recommended revised POS algorithm. METHODS: A retrospective cohort study was performed to evaluate the results of POS in the well-infant nursery between 2012 and 2020. The POS results were obtained from an electronic platform. Chart review was completed for newborns with failed screens. The recommended revision to POS, no second rescreen, was applied to the data to evaluate screening outcomes. RESULTS: Of the total 65 414 infants admitted to the well-infant nursery during this 8-year period, >99% (n = 64 780) received POS. Thirty-one infants failed POS (4.6 per 10 000 screened). All infants who failed POS were found to have a disorder, with 12 (39%) having critical congenital heart disease (CCHD), 9 (29%) having non-CCHD requiring further follow-up, and 10 (32%) having noncardiac conditions. One false-negative screen result was identified through the Maryland Department of Health Newborn Screening Follow-up Program. The positive predictive value of POS for those screened was 39% for CCHD, with a specificity of 99.97%. Eliminating the second rescreen in the POS algorithm would have resulted in an additional 5 newborns without CCHD failing POS, increasing the false-positive rate from 0.03% to 0.04%. CONCLUSIONS: POS is an effective tool for identifying CCHD and secondary conditions. POS was successfully implemented with few missed screens and was highly specific. Elimination of the second rescreen in the pulse oximetry algorithm would have resulted in a minimal increase in false-positive results and faster evaluation of newborns with CCHD.

Development of an international standard set of clinical and patient-reported outcomes for children and adults with congenital heart disease: a report from the International Consortium for Health Outcomes Measurement Congenital Heart Disease Working Group.

AIMS: Congenital heart disease (CHD) is the most common congenital malformation. Despite the worldwide burden to patient wellbeing and health system resource utilization, tracking of long-term outcomes is lacking, limiting the delivery and measurement of high-value care. To begin transitioning to value-based healthcare in CHD, the International Consortium for Health Outcomes Measurement aligned an international collaborative of CHD experts, patient representatives, and other stakeholders to construct a standard set of outcomes and risk-adjustment variables that are meaningful to patients. METHODS AND RESULTS: The primary aim was to identify a minimum standard set of outcomes to be used by health systems worldwide. The methodological process included four key steps: (i) develop a working group representative of all CHD stakeholders; (ii) conduct extensive literature reviews to identify scope, outcomes of interest, tools used to measure outcomes, and case-mix adjustment variables; (iii) create the outcome set using a series of multi-round Delphi processes; and (iv) disseminate set worldwide. The Working Group established a 15-item outcome set, incorporating physical, mental, social, and overall health outcomes accompanied by tools for measurement and case-mix adjustment variables. Patients with any CHD diagnoses of all ages are included. Following an open review process, over 80% of patients and providers surveyed agreed with the set in its final form. CONCLUSION: This is the first international development of a stakeholder-informed standard set of outcomes for CHD. It can serve as a first step for a lifespan outcomes measurement approach to guide benchmarking and improvement among health systems.

Mindful Mentors: Is a Longitudinal Mind-Body Skills Training Pilot Program Feasible for Pediatric Cardiology Staff?

BACKGROUND: Stress and burnout among medical professionals are common and costly, placing professionals, organizations, and patients at risk. OBJECTIVES: To determine feasibility and acceptability of a longitudinal mind-body skills training initiative to help staff decrease stress and burnout, improve well-being, and empower them to utilize basic mindfulness methods with coworkers, patients, and families. METHODS: Prospective cohort, mixed methods approach. Nurses, doctors, technicians, social workers, child life specialists were eligible to participate. The 12-month curriculum consisted of 16 hours of intensive education/practice over 2 days, with training in mindfulness skills, self-compassion, nonviolent communication, overcoming barriers to practice, and mindful listening/speaking, followed by monthly 1 hour booster/debriefing sessions. RESULTS: A total of 37 staff participated (RN = 18, MD = 5, Technician = 6, Social Worker = 3, Child life = 3, others = 2) in the initial training, and 24 (65%) completed the 3- and 12-month follow-up surveys. Compared with pretraining scores, there were significant improvements 3 to 12 months after the initial training in stress (P < .0001), distress (P ≤ .04), anxiety (P = .01), self-efficacy in providing non-drug therapies (P < .0001), mindfulness (P = .002), burnout (P < .0001), and confidence in providing compassionate care (P < .0001). In addition, 25 (67%) participants initiated projects incorporating what they learned into staff/patient wellness activities. CONCLUSION: This longitudinal pilot program was feasible and was associated with improvements in measures of psychological well-being over the 12-month intervention. The innovative approach of training participants to teach basic techniques to coworkers and other staff can increase the impact of this program beyond any individual participant. Future research will investigate the aspects of implementation and potential effects on patient care and experience.

Pulse Oximetry and Congenital Heart Disease Screening: Results of the First Pilot Study in Morocco.

Congenital heart disease (CHD) is the most common congenital malformation. Diagnosis of critical congenital heart disease (CCHD), the most severe type of congenital heart disease, in a newborn may be difficult. The addition of CCHD screening, using pulse oximetry, to clinical assessment significantly improves the rate of detection. We conducted a pilot study in Morocco on screening neonates for critical congenital heart disease. This study was conducted in the maternity ward of Mohammed VI University Hospital of Marrakesh, Morocco, and included asymptomatic newborns delivered between March 2019 and January 2020. The screening of CCHD was performed by pulse oximetry measuring the pre- and post-ductal saturation. Screening was performed on 8013/10,451 (76.7%) asymptomatic newborns. According to the algorithm, 7998 cases passed the screening test (99.82%), including one inconclusive test that was repeated an hour later and was normal. Fifteen newborns failed the screening test (0.18%): five CCHD, five false positives, and five CHD but non-critical. One false negative case was diagnosed at 2 months of age. Our results encourage us to strengthen screening for CCHD by adding pulse oximetry to the routine newborn screening panel.

Newborn Critical Congenital Heart Disease Screening Using Pulse Oximetry: Value and Unique Challenges in Developing Regions.

Newborn screening for critical congenital heart disease (CCHD) is recommended for implementation in many developed countries as the standard of care. Efforts to implement this point of care screen in developing regions face unique barriers, and present important opportunities. The First Pan-African Newborn Screening Conference, held in Rabat in June 2019, incorporated a workshop dedicated specifically to identifying and discussing CCHD screening issues in the Middle East Northern Africa (MENA) region. The issues explored may be beneficial as part of the greater discussion of CCHD screening's growing importance in developing regions around the world. Screening experts presented education and lessons learned from previous CCHD implementations, including a hands-on technical demonstration of CCHD screening. Children's HeartLink, The Newborn Foundation, and Children's National Hospital each presented on their experiences working with teams and pilot projects from around the world. Experience in implementation from Children's Hospital Marrakesh was presented and highlighted some of the unique findings, challenges, and experiences of screening in Morocco. As developing regions investigate the implementation of CCHD screening using pulse oximetry either as part of research studies, pilots, regional studies, or as part of a nationally supported program, data to inform policymakers on the benefits of screening and specific needs for infrastructure development and resources are essential. This special issue contains initial lessons learned on newborn CCHD screening from a select number of developing countries, including Saudi Arabia and Morocco and regions such as Latin America.

Updated Strategies for Pulse Oximetry Screening for Critical Congenital Heart Disease.

Seven years after its addition to the US Recommended Uniform Screening Panel, newborn screening for critical congenital heart disease (CCHD) using pulse oximetry became mandatory in the United States. Although CCHD newborn screening reduces morbidity and mortality, there remain important opportunities to improve. An expert panel convened for a 1-day meeting in September 2018, including subject matter experts and representatives from stakeholder organizations. Presentations on CCHD outcomes, variations in approach to screening, and data and quality improvement helped identify improvement opportunities. The expert panel concluded that sufficient evidence exists to recommend modifying the current American Academy of Pediatrics algorithm by (1) requiring an oxygen saturation of at least 95% in both (formerly either) the upper and lower extremities to pass and (2) requiring only 1 repeat screen instead of 2 for cases that neither pass nor fail initially. The panel underscored the importance of improving public health reporting by further specifying the targets of screening and criteria for reporting outcomes (false-negative and false-positive cases). The panel also highlighted the need to ensure sufficient public health funding for CCHD newborn screening and opportunities for education and global implementation. Newborn screening for CCHD using pulse oximetry has led to significant improvements in child health outcomes. However, further important work is required to understand and improve the effectiveness and efficiency of screening.

Quality Improvement Interventions to Improve Critical Congenital Heart Disease Screening.

Newborn screening for critical congenital heart disease (CCHD) using pulse oximetry improves detection and is associated with decreased related infant mortality. In 2015, the Healthy Hearts of Babies Act required hospitals to screen all newborns in the District of Columbia for CCHD using pulse oximetry and to provide documentation of individual screening results to the Department of Health. A regulatory report from the electronic health record revealed an opportunity to improve both documentation and protocol adherence within our hospital. We aimed to reduce documentation errors and protocol violations by 75% and sustain this improvement for 6 months. METHODS: In February of 2014, our center, a large free-standing children's hospital, implemented CCHD screening in the neonatal intensive care unit on all infants without known congenital heart disease or receiving supplemental oxygen. During the intervention period (January 2016 to December 2018), an interdisciplinary team engaged in regular review and analysis of reports, monthly closed-loop feedback, and iterative refinements to the electronic health record. Statistical process control charts were used to compare a baseline period to the intervention period and track monthly progress. RESULTS: Between February 2014 and December 2018, we screened 2,214 infants for CCHD. The average percentage of documentation errors decreased from 23.5% during the baseline period to 1.2% during the intervention period, a sustained reduction for over 2 years. Protocol violations occurred at an average of 2.1% in the baseline period, with a sustained decrease to 0.6% during the intervention period. CONCLUSIONS: This multimodal quality improvement project demonstrated a sustained reduction of CCHD screening documentation errors and protocol violations.

Updates in Congenital Heart Disease: Can Outcomes Improve?

Despite numerous advances in medical and surgical management, congenital heart disease (CHD) remains the number one cause of death in the first year of life from congenital malformations. The current strategies used to approach improving outcomes in CHD are varied. This article will discuss the recent impact of pulse oximetry screening for critical CHD, describe the contributions of advanced cardiac imaging in the neonate with CHD, and highlight the growing importance of quality improvement and safety programs in the cardiac intensive care unit.

IMPACT Registry and National Pediatric Cardiology Quality Improvement Collaborative: Contributions to Quality in Congenital Heart Disease.

The IMproving Pediatric and Adult Congenital Treatments (IMPACT) Registry and the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) are two efforts initiated to improve outcomes in the congenital heart disease field. The IMPACT Registry is focused on evaluating the use, risks, adverse events (AEs), and outcomes associated with diagnostic and common interventional catheterization procedures in all children and adults with congenital heart disease. Utilizing a modular approach, the common procedures include diagnostic cardiac catheterization, atrial septal defect device closure, patent ductus arteriosus device closure, pulmonary valvuloplasty, aortic valvuloplasty, balloon and stent angioplasty of coarctation of the aorta, pulmonary artery balloon stent angioplasty, transcatheter pulmonary valve replacement, and electrophysiology procedures including radiofrequency ablation. To date, important observations on the common procedures have been made and a risk stratification methodology has been created to allow comparisons between centers in AEs and quality improvement activity. The registry is open to international participation. The NPC-QIC was developed to reduce mortality and improve the quality of life of infants with Hypoplastic Left Heart Syndrome (HLHS) during the interstage period between discharge from the Norwood operation and admission for the bidirectional Glenn procedure. Mortality in the interstage has been reduced by 44%. The IMPACT Registry and the NPC-QIC have demonstrated value to the congenital heart disease community. The IMPACT Registry, however, has not yet demonstrated an impact on patient outcomes. The NPC-QIC, which combines both a registry with a learning collaborative with specific aims, key drivers, and change strategies, has made more significant gains with reductions in variation, growth failures, and mortality.

Surgery for Congenital Heart Disease: Improvements in Outcomes.

Cardiac surgery for congenital heart disease has changed dramatically since the first surgery in 1938. During the early era, children underwent surgery at older ages often with palliative procedures before their corrective operation. Not surprisingly, in the early era, there was considerably higher early and late mortality, including the additive risks of having more than one procedure and a long period of living with an unphysiological palliated circulation. Over time with advances in noninvasive diagnosis, surgical approach, cardiopulmonary bypass techniques, and team-based care, outcomes have improved. Children now undergo corrective surgery at a younger age and have fewer palliative procedures. Short-term outcome as measured by the commonly used metric "procedural early mortality" (i.e., death before hospital discharge or less than 30 days following a surgical procedure) is now as low as 1 or 2% for many low-to-moderate complexity procedures. Late outcomes have also improved with long-term survival of hospital survivors for simple lesions being close to population controls. Late outcomes for more complex defects still show diminishing survival relative to a control population. Examination of the causes of death provides insights into areas in which clinical improvements may further improve the outlook for children with complex congenital heart disease.

Asymptomatic atresia of the anomalous pulmonary vein in a patient with scimitar syndrome presenting in childhood.

An asymptomatic 6-year-old boy with a history of right lung hypoplasia was referred for cardiology evaluation. Echocardiography demonstrated right pulmonary artery hypoplasia with flow reversal in that vessel. The right pulmonary veins were not visualised in the echocardiogram. Cardiac catheterisation confirmed the diagnosis of scimitar syndrome with a characteristic large vertical vein; however, the right pulmonary veins were found to be atretic with no connection to the heart with decompression through the azygos vein. In all, four systemic to pulmonary arterial collaterals were identified, supplying the right lung, which were occluded using embolization coils. This case demonstrates the potential for progressive stenosis and atresia of the so-called "scimitar vein" without previous surgical instrumentation, and that this can occur without haemodynamic embarrassment or development of pulmonary vascular disease.