The Connection Between Anatomical Substrate and Clinical Severity in Fetal Ebstein Anomaly.

Ebstein anomaly (EA) is a rare congenital heart defect characterized by abnormal development of the tricuspid valve (TV) and right ventricular myocardium. This study documents 2 dramatic cases of fetal EA characterized by hydrops and cardiomegaly, leading to intrauterine or early neonatal death. These clinical outcomes were associated with morphological abnormalities including severe tricuspid regurgitation, unguarded TV orifice, pulmonary atresia, and flattened right ventricular myocardium. This study highlights that these adverse anatomical features may result in unfavorable clinical outcomes in fetal EA. While timely identification of such features by prenatal ultrasound is crucial for providing accurate prognostic stratification and guiding treatment decisions, fetopsy may be necessary to discern EA among the spectrum of right-heart anomalies.

Mental Health Care for Children with Heart Disease and Their Families: Practical Approaches and Considerations for the Pediatric and Pediatric Cardiology Clinician.

Mental health conditions are a common comorbidity among children living with heart disease. Children with congenital heart disease are more likely to have a mental health condition than their unaffected peers or peers with other chronic illnesses, and mental health risk persists across their lifetime. While poorer mental health in adults with congenital heart disease is associated with worse overall health outcomes, the association between mental health and cardiac outcomes for children with heart disease remains unknown. Despite this, it is suspected that mental health conditions go undiagnosed in children with heart disease and that many affected children and adolescents do not receive optimal mental health care. In this article, we review mental health in congenital heart disease across the lifespan, across domains of care, and across diagnoses. Further directions to support mental health care for children and adolescents with heart disease include practical screening and access to timely referral and mental health resources.

Homogenous access to fetal cardiac care in a heterogeneous state.

BACKGROUND: Timely prenatal diagnosis of CHD allows families to participate in complex decisions and plan for the care of their child. This study sought to investigate whether timing of initial fetal echocardiogram and the characteristics of fetal counselling were impacted by parental socio-economic factors. METHODS: Retrospective chart review of fetal cardiac patients from 1 January, 2017 to 31 December, 2018. We reviewed gestational age at first fetal echo, maternal age and ethnicity, zip code, rurality index, and hospital distance. Counselling was evaluated based on documentation regarding use of interpreter, time billed for counselling, and treatment option chosen. RESULTS: Total of 139 maternal-fetal dyads were included, and 29 dyads had single-ventricle heart disease. There was no difference in income, hospital distance or rurality index, and first fetal echo timing. There was no significant difference between maternal ethnicity and maternal age, gestational age at initial visit, or follow-up. Patients in rural areas had increased counselling time (p < .05). There was no difference between socio-economic factors and ultimate parental choices (termination, palliative delivery, or cardiac interventions). CONCLUSION: Oregon comprises a heterogeneous population from a large geographical catchment. While prenatal counselling and family decision-making are multifaceted, we demonstrated that dyads were referred from across the state and received care in a uniformly timely manner, and once at our centre received consistent counselling despite differences in parental socio-economic factors.

Clinically Suspected Myocarditis Temporally Related to COVID-19 Vaccination in Adolescents and Young Adults: Suspected Myocarditis After COVID-19 Vaccination.

BACKGROUND: Understanding the clinical course and short-term outcomes of suspected myocarditis after the coronavirus disease 2019 (COVID-19) vaccination has important public health implications in the decision to vaccinate youth. METHODS: We retrospectively collected data on patients <21 years old presenting before July 4, 2021, with suspected myocarditis within 30 days of COVID-19 vaccination. Lake Louise criteria were used for cardiac MRI findings. Myocarditis cases were classified as confirmed or probable on the basis of the Centers for Disease Control and Prevention definitions. RESULTS: We report on 139 adolescents and young adults with 140 episodes of suspected myocarditis (49 confirmed, 91 probable) at 26 centers. Most patients were male (n=126, 90.6%) and White (n=92, 66.2%); 29 (20.9%) were Hispanic; and the median age was 15.8 years (range, 12.1-20.3; interquartile range [IQR], 14.5-17.0). Suspected myocarditis occurred in 136 patients (97.8%) after the mRNA vaccine, with 131 (94.2%) after the Pfizer-BioNTech vaccine; 128 (91.4%) occurred after the second dose. Symptoms started at a median of 2 days (range, 0-22; IQR, 1-3) after vaccination. The most common symptom was chest pain (99.3%). Patients were treated with nonsteroidal anti-inflammatory drugs (81.3%), intravenous immunoglobulin (21.6%), glucocorticoids (21.6%), colchicine (7.9%), or no anti-inflammatory therapies (8.6%). Twenty-six patients (18.7%) were in the intensive care unit, 2 were treated with inotropic/vasoactive support, and none required extracorporeal membrane oxygenation or died. Median hospital stay was 2 days (range, 0-10; IQR, 2-3). All patients had elevated troponin I (n=111, 8.12 ng/mL; IQR, 3.50-15.90) or T (n=28, 0.61 ng/mL; IQR, 0.25-1.30); 69.8% had abnormal ECGs and arrhythmias (7 with nonsustained ventricular tachycardia); and 18.7% had left ventricular ejection fraction <55% on echocardiogram. Of 97 patients who underwent cardiac MRI at a median 5 days (range, 0-88; IQR, 3-17) from symptom onset, 75 (77.3%) had abnormal findings: 74 (76.3%) had late gadolinium enhancement, 54 (55.7%) had myocardial edema, and 49 (50.5%) met Lake Louise criteria. Among 26 patients with left ventricular ejection fraction <55% on echocardiogram, all with follow-up had normalized function (n=25). CONCLUSIONS: Most cases of suspected COVID-19 vaccine myocarditis occurring in persons <21 years have a mild clinical course with rapid resolution of symptoms. Abnormal findings on cardiac MRI were frequent. Future studies should evaluate risk factors, mechanisms, and long-term outcomes.

Prenatal Congenital Heart Disease-It Takes a Multidisciplinary Village.

Prenatal diagnosis of congenital heart disease (CHD) allows for thoughtful multidisciplinary planning about location, timing, and need for medical interventions at birth. We sought to assess the accuracy of our prenatal cardiac diagnosis, and postnatal needs for patients with CHD utilizing a multidisciplinary approach. We performed a retrospective chart review of fetal CHD patients between 1/1/18 and 4/30/19. Maternal and infant charts were reviewed for delivery planning, subspecialty care needs, genetic evaluation, prenatal and postnatal cardiac diagnoses, need for prostaglandin (PGE) and neonatal cardiac intervention. 82 maternal-fetal dyads met inclusion criteria during the study period and delivered at a median of 38w2d gestation. 32 (39%) dyads had CHD and other anomalies or genetic abnormalities. All dyads met with a genetic counselor and neonatologist. 11 patients delivered at outside hospitals as planned (all with isolated CHD not requiring neonatal intervention), and 5 chose a palliative delivery. 30 patients were counseled to expect a neonatal cardiac intervention and 25 (83%) underwent an intervention within the expected time period. No neonates required an uncounseled cardiac intervention. 29 patients planned for PGE at birth and 31 received PGE. Of the 79 postnatal echocardiograms, 60 (76%) were entirely consistent with the fetal diagnosis. A multidisciplinary approach to the prenatal diagnosis of CHD in maternal-fetal dyads is optimal and utilizing this method we were able to accurately predict postnatal physiology and ensure that patients delivered in the correct location with an appropriate supportive structure in place.

Evaluating How Physician Attitudes May Affect Practice in Fetal Cardiac Counseling.

Advances in fetal cardiac imaging over the last few decades have allowed for increased prenatal detection and detailed counseling of congenital heart disease (CHD). When CHD is detected, fetal cardiologists are faced with the challenge of providing nuanced prenatal counseling. Studies in other specialties have shown that differences in physician attitudes exist around termination of pregnancy and correlate with variations in the counseling provided to parents. We conducted an anonymous cross-sectional survey of fetal cardiologists in New England (n = 36) regarding attitudes toward termination of pregnancy and the counseling provided to parents with a fetal diagnosis of hypoplastic left heart syndrome. Using a screening questionnaire, there was no significant difference in the counseling provided to parents regardless of the physician's personal or professional views on termination of pregnancy, age, gender, location, type of practice, or years of experience. There were, however, differences among physicians on reasons to consider termination and their perceived professional responsibility to the fetus or mother. Further investigation on a larger geographic scale may reveal additional insights on variations in physician beliefs and whether such beliefs affect variability in counseling practices.

Structured Framework for Multidisciplinary Parent Counseling and Medical Interventions for Fetuses and Infants with Trisomy 13 or Trisomy 18.

OBJECTIVE: Trisomy 13 (T13) and 18 (T18) are aneuploidies associated with multiple structural congenital anomalies and high rates of fetal demise and neonatal mortality. Historically, patients with either one of these diagnoses have been treated similarly with exclusive comfort care rather than invasive interventions or intensive care, despite a wide phenotypic variation and substantial variations in survival length. However, surgical interventions have been on the rise in this population in recent years without clearly elucidated selection criterion. Our objective was to create a standardized approach to counseling expectant persons and parents of newborns with T13/T18 in order to provide collaborative and consistent counseling and thoughtful approach to interventions such as surgery. STUDY DESIGN: This article describes our process and presents our resulting clinical care guideline. RESULTS: We formed a multi- and interdisciplinary committee. We used published literature when available and otherwise expert opinion to develop an approach to care featuring individualized assessment of the patient to estimate qualitative mortality risk and potential to benefit from intensive care and/or surgeries centered within an ethical framework. CONCLUSION: Through multidisciplinary collaboration, we successfully created a patient-centered approach for counseling families facing a diagnosis of T13/T18. Other institutions may use our approach as a model for developing their own standardized approach. KEY POINTS: · Trisomy 13 and trisomy 18 are associated with high but variable morbidity and mortality.. · Research on which patients are most likely to benefit from surgery is lacking.. · We present our institution's framework to counsel families with fetal/neonatal T13/T18..

mRNA Coronavirus Disease 2019 Vaccine-Associated Myopericarditis in Adolescents: A Survey Study.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Paediatrician's guide to post-operative care for functionally univentricular CHD: a review.

IMPORTANCE: Single ventricle CHD affects about 5 out of 100,000 newborns, resulting in complex anatomy often requiring multiple, staged palliative surgeries. Paediatricians are an essential part of the team that cares for children with single ventricle CHD. These patients often encounter their paediatrician first when a complication arises, so it is critical to ensure the paediatrician is knowledgeable of these issues to provide optimal care. OBSERVATIONS: We reviewed the subtypes of single ventricle heart disease and the various palliative surgeries these patients undergo. We then searched the literature to detail the general paediatrician's approach to single ventricle patients at different stages of surgical palliation. CONCLUSIONS AND RELEVANCE: Single ventricle patients undergo staged palliation that drastically changes physiology after each intervention. Coordinated care between their paediatrician and cardiologist is requisite to provide excellent care. This review highlights what to expect when these patients are seen by their paediatrician for either well child visits or additional visits for parental or patient concern.

Paediatrician's guide to post-operative care for biventricular CHD: a review.

IMPORTANCE: Paediatricians play an integral role in the lifelong care of children with CHD, many of whom will undergo cardiac surgery. There is a paucity of literature for the paediatrician regarding the post-operative care of such patients. OBSERVATIONS: The aim of this manuscript is to summarise essential principles and pertinent lesion-specific context for the care of patients who have undergone surgery or intervention resulting in a biventricular circulation. CONCLUSIONS AND RELEVANCE: Familiarity with common issues following cardiac surgery or intervention, as well as key details regarding specific lesions and surgeries, will aid the paediatrician in providing optimal care for these patients.

Low prenatal detection rate of valvar pulmonary stenosis: What are we missing?

OBJECTIVES: Critical pulmonary stenosis (PS) and pulmonary atresia with intact ventricular septum (PAIVS) require urgent neonatal intervention. Since PS may be more insidious than PAIVS during gestation, we hypothesized that neonates with PS would have lower rates of prenatal detection than PAIVS. METHODS: We performed a retrospective chart review of all neonates who underwent diagnostic or interventional cardiac catheterization between 2000 and 2014 for critical PS or PAIVS. The rates of prenatal diagnosis were calculated for PS and PAIVS. Prenatal and postnatal echocardiographic data were reviewed. RESULTS: 178 patients met inclusion criteria: 91 with critical PS and 87 with PAIVS. The prenatal diagnosis rate for critical PS was lower than for PAIVS at 37% (34/91) vs 60% (52/87) (P = .003). At the time of diagnosis at a median gestational age of 25 weeks, the median TV z-score for patients with critical PS was larger than in PAIVS (-0.15 vs -3.0 P = .004). CONCLUSION: Critical PS had a lower prenatal detection rate than PAIVS, likely due to a relatively normal 4-chamber view at the time of routine second trimester screening in patients with PS. Color flow Doppler of the outflow tracts may improve detection, since outflow tracts may appear normal by 2D imaging.

The Utility of Echocardiography in Pediatric Patients with Structurally Normal Hearts and Suspected Endocarditis.

The objective of this study was to evaluate the utility of transthoracic echocardiography (TTE) in children with structurally normal hearts suspected of having infective endocarditis (IE). We hypothesized that the diagnostic yield of TTE is minimal in low-risk patients with normal hearts. We performed a retrospective chart review of TTEs performed for concern for endocarditis at a pediatric tertiary care referral center in Portland, Oregon. Three hundred patients met inclusion criteria (< 21 years old, completed TTE for IE from 2005 to 2015, no history of congenital heart disease or endocarditis). We recorded findings that met the modified Duke criteria (MDC) including fever, positive blood culture, and vascular/immunologic findings; presence of a central line; whether or not patients were diagnosed with IE clinically; and if any changes to antibiotic regimens were made based on TTE. Ten patients (3%) had echocardiograms consistent with IE. When compared to the clinical diagnosis of IE, the positive predictive value (PPV) of one positive blood culture without other major/minor MDC was 0. Similarly, the PPV of two positive blood cultures without other major/minor criteria was 0.071. Patients should be evaluated using the MDC to assess the clinical probability of IE prior to performing a TTE. Patients with a low probability for IE should not undergo TTE as it has a low diagnostic yield and patients are unlikely to be diagnosed with disease.

Telehealth for Pediatric Cardiology Practitioners in the Time of COVID-19.

Due to the COVID-19 pandemic, there has been an increased interest in telehealth as a means of providing care for children by a pediatric cardiologist. In this article, we provide an overview of telehealth utilization as an extension of current pediatric cardiology practices and provide some insight into the rapid shift made to quickly implement these telehealth services into our everyday practices due to COVID-19 personal distancing requirements. Our panel will review helpful tips into the selection of appropriate patient populations and specific cardiac diagnoses for telehealth that put patient and family safety concerns first. Numerous practical considerations in conducting a telehealth visit must be taken into account to ensure optimal use of this technology. The use of adapted staffing and billing models and expanded means of remote monitoring will aid in the incorporation of telehealth into more widespread pediatric cardiology practice. Future directions to sustain this platform include the refinement of telehealth care strategies, defining best practices, including telehealth in the fellowship curriculum and continuing advocacy for technology.

Provider perspective on the role of palliative care in hypoplastic left heart syndrome.

OBJECTIVE: Hypoplastic left heart syndrome is a single ventricle defect. While staged surgical palliative treatments have revolutionised care, patients with hypoplastic left heart syndrome continue to have significant morbidity and mortality. In 2017, the National Pediatric Cardiology Quality Improvement Collaborative recommended all single ventricle patients to receive a prenatal palliative care consult. This study aimed to elucidate provider perspectives on the implementation of prenatal palliative care consults for families expecting a child with hypoplastic left heart syndrome. METHODS: An online survey was administered to obstetric and paediatric providers of relevant disciplines to assess their experience with palliative care involvement in hypoplastic left heart syndrome cases. RESULTS: Nearly, all physicians (97%) and most registered nurses (79%) agreed that the initial palliative care consult for patients with hypoplastic left heart syndrome should occur during the prenatal period. Respondents also indicated that prenatal palliative care consults should also be offered in a variety of other CHD conditions. Participants believed positive aspects of this new referral protocol included an expanded support network for families, decreased family stress during the postnatal period, increased patient education about what to expect during the postnatal period, and continuity of care. CONCLUSION: Multidisciplinary healthcare professionals believe that prenatal palliative care consults provide a variety of benefits for patients and families with hypoplastic left heart syndrome. Additional, multi-centre research is necessary to evaluate whether prenatal palliative care consults should become standard of care for families expecting a child with a single ventricle defect.

Cerebrovascular blood flow in patients with tetralogy of fallot: prediction for early surgical intervention.

OBJECTIVES: Children with tetralogy of Fallot (TOF) require initial intervention at varying times reflecting a spectrum of obstruction to pulmonary blood flow. In utero, fetal patients with right sided obstructive lesions demonstrate increased middle cerebral artery (MCA) pulsatility index (PI). We sought to determine whether MCA flow patterns are associated with the degree of pulmonary obstruction and the consequential need for early surgical intervention (ESI). METHODS: We performed a multi-center retrospective study evaluating patients with a prenatal diagnosis of TOF. All echocardiographic fetal studies performed from 16 weeks' gestational age through delivery were reviewed between 2001 and 2015 with subsequent postnatal medical chart review. RESULTS: 82 patients met inclusion criteria. Patients who ultimately required ESI (n=30) demonstrated an increased MCA PI z-score (-0.68 vs -1.32, p=0.02) compared to those who did not (n=52). An elevated MCA PI in patients with TOF was associated with ESI after 24 weeks' gestational age (-0.36 vs -1.22, p=0.02). CONCLUSIONS: Fetal patients with TOF demonstrate altered MCA flow that correlates with need for ESI. This effect is blunted earlier in gestation which may reflect cerebral autoregulation. MCA PI may be a beneficial tool in helping determine perinatal management and counseling for patients with TOF.

Determination of right ventricular function with adjusted systolic to diastolic duration ratio after transannular patch repair of tetralogy of Fallot.

BACKGROUND: Early detection of right ventricular dysfunction after transannular patch for tetralogy of Fallot (TOF-TAP) is essential for management. OBJECTIVES: To evaluate echocardiographic metrics of ventricular function correlate with functional MRI measurements, in patients with TOF-TAP. METHODS: A retrospective review of patients with TOF-TAP between 2007 and 2017 who had an echocardiogram and MRI within six months were analyzed. Systolic to diastolic ratio (SD ratio) was measured from the tricuspid regurgitation and adjusted for heart rate. Tricuspid Annular Plane Systolic Excursion (TAPSE), Fractional Area Change (FAC), and shortening fraction (SF) were additionally measured. Echocardiographic measurements were correlated with MRI assessment of right ventricular ejection fraction (RVEF), left ventricular ejection fraction (LVEF), right ventricular end-diastolic volume index (RVEDi), and right to left ventricle volume ratio (RV/LV). RESULTS: Of the 53 patients (mean age 12.8 years) that met inclusion criteria, 45 (85%) had available TR jets for SD ratio analysis. The HR adjusted SD ratio negatively correlated with RVEF (r = -.359, P = .016), LVEF (r = -.317, P = .038) and positively with RV/LV EDV ratio (r = .347, P = .024). TAPSE, FAC, and SF measurements did not show significant correlation. CONCLUSION: In patients with TOF-TAP, there is a moderate negative correlation between heart rate adjusted SD ratio and MRI metrics of ventricular function, suggesting that decreased filling time is a marker for reduced right ventricular function. The SD ratio may be a useful echocardiographic tool for serial evaluation of in this population.

Paediatric cardiology fellowship training: effect of work-hour regulations on scholarly activity.

BACKGROUND: In 2003, work-hour regulations were implemented by the Accreditation Council for Graduate Medical Education. Much has been published regarding resident rest and quality of life as well as patient safety. There has been no examination on the effect of work-hour restrictions on academic productivity of fellows in training. Paediatric subspecialty fellows have a scholarly requirement mandated by the American Board of Pediatrics. We have examined the impact of work-hour restrictions on the scholarly productivity of paediatric cardiology fellows during their fellowship. METHODS: We conducted a literature search for all paediatric cardiology fellows between 1998 and 2007 at a single academic institution as first or senior authors on papers published during their 3-year fellowship and 3 years after completion of their categorical fellowship (n=63, 30 fellows before 2003 and 33 fellows after 2003). The numbers of first- or senior-author fellow publications before and after 2003 were compared. We also collected data on final paediatric cardiology subspecialty career choice. RESULTS: There was no difference in the number of fellow first-author publications before and after 2003. Before work-hour restrictions, the mean number of publications per fellow was 2.1 (±2.2), and after work-hour restrictions it was 2.0 (±1.8), (p=0.89). By subspecialty career choice, fellows who select electrophysiology, preventative cardiology, and heart failure always published within the 6-year time period. CONCLUSIONS: Since the implementation of work-hour regulations, total number of fellow first-authored publications has not changed. The role of subspecialty choice may play a role in academic productivity of fellows in training.

A Pediatric Cardiology Fellowship Boot Camp improves trainee confidence.

Introduction New paediatric cardiology trainees are required to rapidly assimilate knowledge and gain clinical skills to which they have limited or no exposure during residency. The Pediatric Cardiology Fellowship Boot Camp (PCBC) at Boston Children's Hospital was designed to provide incoming fellows with an intensive exposure to congenital cardiac pathology and a broad overview of major areas of paediatric cardiology practice. METHODS: The PCBC curriculum was designed by core faculty in cardiac pathology, echocardiography, electrophysiology, interventional cardiology, exercise physiology, and cardiac intensive care. Individual faculty contributed learning objectives, which were refined by fellowship directors and used to build a programme of didactics, hands-on/simulation-based activities, and self-guided learning opportunities. RESULTS: A total of 16 incoming fellows participated in the 4-week boot camp, with no concurrent clinical responsibilities, over 2 years. On the basis of pre- and post-PCBC surveys, 80% of trainees strongly agreed that they felt more prepared for clinical responsibilities, and a similar percentage felt that PCBC should be offered to future incoming fellows. Fellows showed significant increase in their confidence in all specific knowledge and skills related to the learning objectives. Fellows rated hands-on learning experiences and simulation-based exercises most highly. CONCLUSIONS: We describe a novel 4-week-long boot camp designed to expose incoming paediatric cardiology fellows to the broad spectrum of knowledge and skills required for the practice of paediatric cardiology. The experience increased trainee confidence and sense of preparedness to begin fellowship-related responsibilities. Given that highly interactive activities were rated most highly, boot camps in paediatric cardiology should strongly emphasise these elements.

Left Ventricular Dysfunction Following Neonatal Pulmonary Valve Balloon Dilation for Pulmonary Atresia or Critical Pulmonary Stenosis.

Pulmonary valve (PV) balloon dilation (BD) is the primary therapy for infants born with critical pulmonary stenosis (PS) or membranous pulmonary atresia with intact ventricular septum (PAIVS). We observed left ventricular (LV) dysfunction in patients following BD and sought to determine its incidence, clinical course and associated risk factors. Clinical, echocardiographic and catheterization data for all patients who underwent neonatal (<2 weeks age) PV BD for critical PS or PAIVS between January 2000 and February 2014 were retrospectively analyzed (n = 129). Post-procedure LV dysfunction was defined as ejection fraction (EF) <54 %. Median age at PV BD was 1 day. Most (71 %) patients had critical PS. Median PV diameter pre-BD was 6.0 mm with PV z-scores -4.1 to 0.9, median LV EF pre-BD was 58 %. Post-BD LV dysfunction developed in 45 patients (35 %); 15 patients had LV EF ≤40 %. Median time to normalization of LV EF was 10 days (range 2-72). In univariate analysis, diagnosis (critical PS or PAIVS), right ventricle to LV pressure ratio pre-BD, acute procedural complication and post-BD inotropic support were not associated with post-BD LV dysfunction. In multivariable analysis, the predictors of post-procedure LV dysfunction were lower PV z-score (OR 1.81, p 0.04), tricuspid regurgitation pre-BD ≥ moderate (OR 3.73, p 0.008) and larger right ventricular apical area (OR 1.99, p 0.04). LV dysfunction post-neonatal PV BD develops in a significant number of patients (35 %) and can be severe, but resolves. The risk of developing LV dysfunction post-PV BD is highest in patients with larger right ventricles.

The Evolving Role of Genetic Evaluation in the Prenatal Diagnosis and Management of Congenital Heart Disease.

Congenital heart disease (CHD) is increasingly diagnosed prenatally and the ability to screen and diagnose the genetic factors involved in CHD have greatly improved. The presence of a genetic abnormality in the setting of prenatally diagnosed CHD impacts prenatal counseling and ensures that families and providers have as much information as possible surrounding perinatal management and what to expect in the future. This review will discuss the genetic evaluation that can occur prior to birth, what different genetic testing methods are available, and what to think about in the setting of various CHD diagnoses.