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1.
Pediatr Dev Pathol ; : 10935266241250235, 2024 May 19.
Article in English | MEDLINE | ID: mdl-38762771

ABSTRACT

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.

2.
Prenat Diagn ; 2024 Aug 30.
Article in English | MEDLINE | ID: mdl-39215461

ABSTRACT

OBJECTIVE: Impairments in the maternal-fetal environment are associated with adverse postnatal outcomes among infants with congenital heart disease. Therefore, we sought to investigate placental anomalies as they related to various forms of fetal congenital heart disease (FCHD). METHODS: We reviewed the placental pathology in singleton pregnancies with and without FCHD. FCHD was divided into separate categories (transposition physiology, obstructive left, obstructive right, biventricular without obstruction, and others). Exclusion criteria included other prenatally known structural malformations and/or aneuploidy. The significance threshold was set at p < 0.05 or False Discovery rate q < 0.05 when multiple tests were performed. RESULTS: The cohort included 215 FCHD and 122 non-FCHD placentas. FCHD placentas showed increased rates of maternal vascular malperfusion (24% vs. 5%, q < 0.001) and cord anomalies (27% vs. 1%, q < 0.001). Placentas with fetal TGA demonstrated a lower rate of hypoplasia when compared with other FCHD types (1/39 vs. 51/176, Fisher's exact p = 0.015). CONCLUSION: Placental maternal vascular malperfusion is increased in FCHD. The prevalence of vascular malperfusion did not differ by FCHD type, indicating that CHD type does not predict the likelihood of placental vascular dysfunction. Further investigation of the placental-fetal heart axis in FCHD is warranted given the importance of placental health.

3.
Pediatr Cardiol ; 2024 May 16.
Article in English | MEDLINE | ID: mdl-38753034

ABSTRACT

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.

4.
Cardiol Young ; 34(3): 500-504, 2024 Mar.
Article in English | MEDLINE | ID: mdl-37485827

ABSTRACT

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.


Subject(s)
Fetus , Prenatal Care , Child , Female , Pregnancy , Humans , Retrospective Studies , Gestational Age , Prenatal Diagnosis
5.
BMC Med Educ ; 24(1): 1092, 2024 Oct 07.
Article in English | MEDLINE | ID: mdl-39375650

ABSTRACT

BACKGROUND: Pediatric cardiology fellows often deliver serious news to families. Effective clinician-patient communication is the basis of strong therapeutic relationships and improves health outcomes, increases patient adherence, and enhances patient satisfaction. Communication training improves physicians' communication skills, ability to deliver serious news, and meet the informational and emotional needs of patients and family members. However, there is little data surrounding pediatric cardiology fellows competencies or training in communication skills. METHODS: Pediatric cardiology fellows participated in a 3-hour communication training session. The session used VitalTalk methodology and was facilitated by two VitalTalk facilitators. Fellows spent 1 h learning the skills of delivering serious news and responding to emotion and 2 h in role play with standardized actors followed by a brief group wrap-up activity. Participants took an anonymous, electronic pre- and post-survey and an 8-month follow-up survey via REDCap. Participants were asked about their preparedness and comfort performing certain communication skills and leading challenging conversations specific to pediatric cardiology. Response options used a combination of 0 (low comfort/preparedness) to 100 (high comfort/preparedness) point scales and multiple choice. RESULTS: 9 fellows participated in the training and 100% completed all three surveys. Eight were first-year fellows and 1 was a third-year fellow. Finding the right words, balancing honesty with hope, and clinical and prognostic uncertainty were the top three factors that contributed to making conversations difficult. Following the course, there was a significant increase in fellow preparedness to communicate a new diagnosis of congenital heart disease, discuss poor prognoses, check understanding, and respond to emotion and an increase in fellow comfort responding to emotions. Four fellows reported using the skills from this training course in various clinical settings at 8-month follow up. CONCLUSIONS: Communicating serious news effectively is a skill that can be learned in a sustainable way and is essential in the field of pediatric cardiology. Our study demonstrates that an interactive, VitalTalk course can improve preparedness and comfort to deliver serious news in a cohort of pediatric cardiology trainees. Future studies are needed to evaluate translation of skills to clinical practice and durability of these skills in larger cohorts.


Subject(s)
Cardiology , Communication , Pediatrics , Physician-Patient Relations , Humans , Pilot Projects , Cardiology/education , Pediatrics/education , Fellowships and Scholarships , Female , Male , Clinical Competence , Education, Medical, Graduate
6.
Circulation ; 145(5): 345-356, 2022 02.
Article in English | MEDLINE | ID: mdl-34865500

ABSTRACT

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.


Subject(s)
COVID-19 Vaccines/adverse effects , COVID-19/prevention & control , Myocarditis/diagnostic imaging , Myocarditis/physiopathology , Adolescent , Child , Electrocardiography/methods , Female , Humans , Magnetic Resonance Imaging, Cine/methods , Male , Myocarditis/blood , Myocarditis/etiology , Retrospective Studies , Time Factors , Young Adult
7.
Pediatr Cardiol ; 44(5): 1050-1056, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37186174

ABSTRACT

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.


Subject(s)
Fetal Diseases , Heart Defects, Congenital , Pregnancy , Infant , Female , Infant, Newborn , Humans , Retrospective Studies , Ultrasonography, Prenatal/methods , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/therapy , Prenatal Diagnosis
8.
Pediatr Cardiol ; 2023 Jun 19.
Article in English | MEDLINE | ID: mdl-37335356

ABSTRACT

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.

9.
Am J Perinatol ; 2023 Aug 24.
Article in English | MEDLINE | ID: mdl-37619598

ABSTRACT

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..

10.
J Pediatr ; 243: 208-213.e3, 2022 04.
Article in English | MEDLINE | ID: mdl-34952008

ABSTRACT

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.


Subject(s)
COVID-19 , Myocarditis , Adolescent , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Humans , Myocarditis/epidemiology , Myocarditis/etiology , RNA, Messenger
11.
Cardiol Young ; 32(11): 1721-1727, 2022 Nov.
Article in English | MEDLINE | ID: mdl-36165406

ABSTRACT

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.


Subject(s)
Pediatricians , Child , Humans
12.
Cardiol Young ; 32(10): 1547-1553, 2022 Oct.
Article in English | MEDLINE | ID: mdl-36148873

ABSTRACT

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.


Subject(s)
Heart Defects, Congenital , Univentricular Heart , Humans , Child , Infant, Newborn , Heart Defects, Congenital/surgery , Palliative Care , Postoperative Care , Pediatricians , Heart Ventricles/surgery , Retrospective Studies
13.
Prenat Diagn ; 40(8): 966-971, 2020 07.
Article in English | MEDLINE | ID: mdl-32314369

ABSTRACT

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.


Subject(s)
Heart Defects, Congenital/diagnosis , Pulmonary Atresia/diagnosis , Pulmonary Valve Stenosis/diagnosis , Ultrasonography, Prenatal/statistics & numerical data , Adult , Echocardiography/methods , Echocardiography/statistics & numerical data , Female , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/therapy , Humans , Infant Care/statistics & numerical data , Infant, Newborn , Male , Postnatal Care/statistics & numerical data , Pregnancy , Pulmonary Atresia/epidemiology , Pulmonary Atresia/therapy , Pulmonary Valve Stenosis/epidemiology , Pulmonary Valve Stenosis/therapy , Reproducibility of Results , Retrospective Studies , Ultrasonography, Prenatal/methods , United States/epidemiology
14.
Pediatr Cardiol ; 41(1): 62-68, 2020 Jan.
Article in English | MEDLINE | ID: mdl-31673735

ABSTRACT

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.


Subject(s)
Echocardiography, Transesophageal , Endocarditis, Bacterial/diagnostic imaging , Adolescent , Adult , Child , Endocarditis, Bacterial/blood , Endocarditis, Bacterial/physiopathology , Female , Humans , Male , Predictive Value of Tests , Retrospective Studies , Young Adult
15.
Pediatr Cardiol ; 41(6): 1081-1091, 2020 Aug.
Article in English | MEDLINE | ID: mdl-32656626

ABSTRACT

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.


Subject(s)
Cardiology , Coronavirus Infections , Heart Diseases/therapy , Monitoring, Physiologic , Pandemics , Pediatrics , Pneumonia, Viral , Remote Consultation , Telemedicine , Betacoronavirus , COVID-19 , Cardiology/education , Cardiology/trends , Child , Curriculum , Forecasting , Heart Diseases/diagnosis , Humans , Monitoring, Physiologic/methods , Monitoring, Physiologic/trends , Pediatrics/education , Pediatrics/trends , Remote Consultation/methods , Remote Consultation/trends , SARS-CoV-2 , Telemedicine/methods , Telemedicine/trends
16.
Cardiol Young ; 30(3): 377-382, 2020 Mar.
Article in English | MEDLINE | ID: mdl-32146916

ABSTRACT

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.


Subject(s)
Hypoplastic Left Heart Syndrome/diagnosis , Hypoplastic Left Heart Syndrome/therapy , Palliative Care/methods , Palliative Care/psychology , Parents/psychology , Attitude of Health Personnel , Female , Humans , Infant, Newborn , Oregon , Patient Care Team/organization & administration , Pregnancy , Prenatal Diagnosis , Professional-Family Relations , Quality Improvement , Referral and Consultation/statistics & numerical data , Surveys and Questionnaires
17.
Prenat Diagn ; 39(13): 1184-1190, 2019 12.
Article in English | MEDLINE | ID: mdl-31525255

ABSTRACT

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.


Subject(s)
Cerebrovascular Circulation , Tetralogy of Fallot/physiopathology , Echocardiography , Female , Humans , Middle Cerebral Artery/physiopathology , Pregnancy , Retrospective Studies , Tetralogy of Fallot/diagnostic imaging , Tetralogy of Fallot/surgery , Ultrasonography, Prenatal
18.
Echocardiography ; 36(9): 1706-1712, 2019 09.
Article in English | MEDLINE | ID: mdl-31490578

ABSTRACT

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.


Subject(s)
Echocardiography/methods , Magnetic Resonance Imaging/methods , Tetralogy of Fallot/surgery , Ventricular Dysfunction, Right/diagnostic imaging , Ventricular Dysfunction, Right/physiopathology , Child , Diastole , Female , Humans , Male , Retrospective Studies , Stroke Volume , Systole
19.
Cardiol Young ; 27(1): 69-73, 2017 Jan.
Article in English | MEDLINE | ID: mdl-26956034

ABSTRACT

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.


Subject(s)
Accreditation , Cardiology/education , Career Choice , Education, Medical, Graduate/methods , Internship and Residency/methods , Pediatrics/education , Child , Female , Humans , Male , Quality of Life , United States
20.
Cardiol Young ; 26(8): 1514-1521, 2016 Dec.
Article in English | MEDLINE | ID: mdl-28148335

ABSTRACT

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.


Subject(s)
Cardiology/education , Clinical Competence/standards , Fellowships and Scholarships/standards , Pediatrics/education , Program Evaluation/standards , Curriculum , Education, Medical , Humans
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