Pediatric Cardiology最新文献

Pediatric cardiology fellowship programs in the United States (U.S.) follow the Accreditation Council for Graduate Medical Education (ACGME) guidelines, requiring scholarly activities for completion. However, detailed data on research experiences within these programs are scarce. This study sought to describe the state of research training and exposure in U.S. pediatric cardiology fellowship programs. An electronic survey was sent to 350 pediatric cardiology fellows across the U.S (Initially sent on 11/16/23 with a second request on 12/12/23). The survey assessed demographics, availability of research training resources, perceived barriers to research, expected research output, and satisfaction with research exposure. Fellowship programs were classified by size: small (1-5 fellows), medium (6-9 fellows), and large (10 + fellows). Statistical analyses included comparisons and logistic regression. We received 103 complete survey responses. Most respondents (89%) agreed with the statement: "I have been encouraged to pursue research training and experiences during my fellowship." However, only 31% agreed with the statement: "I am satisfied with the training on research methodology and statistics provided by my fellowship program." Commonly endorsed barriers to completing research projects included lack of statistical support (37%), lack of protected time for faculty to support fellow research (56%), lack of a formal research curriculum (38%), and lack of funding for fellow research (26%). Research training and experiences differed by program size, with larger programs providing more protected time and greater encouragement for research, while smaller programs were more likely to rely on online resources. Despite strong encouragement for research, satisfaction with research training in pediatric cardiology fellowship programs is limited. Addressing barriers such as statistical support, protected faculty time, and formal research curricula could enhance the research experience and outcomes for fellows.

The "nutmeg lung pattern" on fetal magnetic resonance imaging (MRI) indicates pulmonary lymphangiectasia. This is associated with adverse outcomes, particularly in fetuses with congenital heart defects and impaired pulmonary venous return. Whereas lymphedema is common in fetuses with Turner syndrome, pulmonary lymphangiectasia is not. A 26-year-old woman presented with a fetus with hypoplastic left heart syndrome (HLHS) without restrictive atrial septum (RAS). The family declined amniocentesis, yet non-invasive prenatal testing showed an increased risk for Turner syndrome. The patient underwent a fetal MRI as part of a blinded research protocol. Postnatal echocardiogram confirmed the fetal echocardiographic findings without evidence of RAS. Norwood stage I palliation was performed at two days of age. Significant neonatal respiratory morbidity including pneumonia, diaphragmatic and vocal cord pareses and chylothorax occurred. Subsequent review of fetal MRI revealed a prominent thoracic duct and mild pulmonary lymphangiectasia. Turner syndrome was confirmed by genetic testing. After one month, the patient was discharged to her home hospital with respiratory support, which was discontinued a few weeks later. Respiratory problems continued, but the vocal cord paresis resolved over time. This is a unique case of a fetus with HLHS/non-RAS with mild fetal pulmonary lymphangiectasia, and significant neonatal respiratory morbidity, probably in part due to Turner syndrome. The infant survived the neonatal period and underwent uneventful Glenn surgery. The patient's tolerance to the total cavopulmonary connection (TCPC) procedure is yet to be seen. An MRI lymphography should precede it to assess residual lymphatic abnormalities and serve as baseline for post-TCPC changes.

Congenital heart disease (CHD) is the most common congenital anomaly and a major cause of neonatal morbidity and mortality worldwide. A prenatal diagnosis of CHD is a psychologically distressing event that requires sensitive and effective counseling. However, no validated tool exists in Brazilian Portuguese to assess the quality of counseling provided to families following such diagnosis. To conduct the cross-cultural adaptation and content validation of "Kovacevic Questionnaire" for evaluating parental counseling after prenatal CHD diagnosis into Brazilian Portuguese. This cross-sectional study followed the standardized guidelines for cultural adaptation proposed by Beaton et al. The process included translation, synthesis, back-translation, expert review, pre-testing, and psychometric evaluation. Two Expert Committees assessed semantic, idiomatic, cultural, and conceptual equivalence. Content Validity Index (CVI), Prevalence- and Bias-Adjusted Kappa (PABAK), and Content Validity Coefficient (CVC) were calculated. The adapted instrument was pre-tested with ten pregnant women carrying fetuses diagnosed with CHD. The adaptation process resulted in a culturally relevant and linguistically accurate Brazilian version. Expert agreement was high, with most items achieving CVI and PABAK values close to 1.0 and CVC values ≥ 0.80. Minor linguistic refinements were made for clarity and cultural sensitivity. Pre-test participants rated all items as clear and relevant, with CVI and PABAK equal to 1.00. The Brazilian Portuguese version of "Kovacevic Questionnaire" demonstrated high validity and acceptability. It provides clinicians and researchers with a reliable tool to assess and enhance counseling quality for families facing prenatal CHD diagnoses, particularly in diverse and resource-constrained settings such as the Amazon region.

We sought to determine if the COVID-19 pandemic was associated with changes in Kawasaki disease (KD) phenotype and cardiac manifestations. Patients hospitalized with acute KD and enrolled into the International KD Registry were categorized into time periods based on admission date: during the pandemic (January 1, 2020 - September 30, 2022, 33 months) and after the pandemic (October 1, 2022 - September 30, 2025, 36 months). Only patients with verified KD diagnoses as per American Heart Association criteria with no evidence of preceding COVID-19 exposure were included. Demographics, clinical features, management, and cardiac manifestations were compared between time periods. From across 45 sites, 726 during pandemic and 813 after pandemic KD patients were included. During pandemic patients were younger (median 2.6 vs. 3.3 years; p < 0.001), more commonly had incomplete KD (15 vs. 9%; p < 0.001), were less likely to have cough (34 vs. 43%; p < 0.001) and sore throat (15 vs. 22%; p < 0.001), and there were no significant differences in immunomodulatory treatments received. Left ventricular ejection fraction was normal and maximal coronary artery Z scores (median, interquartile range 25-75%, 1.38 [0.77, 2.30] vs. 1.41[0.42, 2.37]; p = 0.08) were similar, including coronary artery aneurysm Z score categories. During the pandemic, KD patients were younger, more likely to present as incomplete KD, and less likely to have respiratory symptoms, with no differences in immunomodulatory treatments received. Cardiac manifestations were however similar. These findings suggest that the COVID-19 pandemic had minimal impact on KD phenotype especially cardiac manifestations.

Ventriculoarterial coupling (VAC), the ratio of arterial elastance (Ea) to LV end-systolic elastance (Ees), measures the relationship between ventricular performance and arterial stiffness. We hypothesized that VAC is abnormal in muscular dystrophy (MD) patients and worsens with disease progression. MD patients < 21 years were included. Demographics, MD phenotype, medications, medical history, and clinical status were collected. Initial and last follow up echocardiograms were reviewed to determine Ea, Ees, and VAC and compared between MD and age-matched males and between the initial and last follow up studies in the MD patients. Univariable and multivariable regression analyses was performed to determine factors independently associated with VAC. Forty-six MD patients, median age at diagnosis 9.8 years (IQR 7 -12.2), followed for a median of 4.1 years (IQR 3-6) were included for study. 41% patients were wheelchair-bound, 67% were on steroids, and 65% on afterload reducing medications. Ejection Fraction (EF) < 55% was found in 28% of patients at diagnosis and in 44% at the last follow-up. EF and Ees were significantly lower and VAC ratio was significantly higher (worse) in the MD patients compared to controls & showed significant worsening from diagnosis to last follow up. Ambulation status was independently associated with EF and Ees at last follow up. Cardiac performance, as measured by VAC ratio, is impaired in patients with MD compared with a control population and worsens with longitudinal follow-up. Ambulation status is independently associated with this impairment, offering mechanistic insight into the development and progression of cardiac dysfunction.

Tricuspid regurgitation (TR) is common in single right ventricle (RV) anomalies and affects outcomes during staged surgical palliations. The Pediatric Heart Network's (PHN) Single Ventricle Reconstruction trial followed children with single RV to age 6 years. This study compared serial echocardiographic measures of TR during staged palliations and determine the impact of TR and tricuspid valve (TV) intervention on transplant-free survival in the PHN cohort. Study echocardiograms were performed at 6 pre-determined times: baseline, post-Norwood, pre-stage II, age 14 months, pre-Fontan, and age 6 years. TR was categorized as ≤ mild or > mild. McNemar's test compared TR across time; t-tests compared RV size/function by TR category; and survival analysis assessed the effect of > mild TR on transplant-free survival. TR was assessed in 543 participants prior to Norwood. From baseline to post-Norwood, those with > mild TR increased from 12 to 24% (p < 0.001). Thereafter, the proportion of surviving participants with > mild TR was stable. Participants with > mild TR had increased baseline TV annular area, and increased RV volume but not decreased function. Transplant-free survival at 6 years was lower with > mild TR at any time except pre-Fontan. 78 TV interventions occurred in 66 participants. Those with TV intervention at the Norwood and/or Stage II had a higher rate of death/transplant than those without or those who had a later intervention. At baseline, post Norwood and pre-stage II, > mild TR is associated with decreased transplant-free survival. TV intervention at the Norwood or Stage II operations was not associated with improved survival.Clinical Trial Registration: NCT00115934.

Material hardship is the degree to which families experience housing, food, energy and transportation insecurity. It has been shown to affect various pediatric outcomes. However, it is unclear what role it plays in outcomes in children with congenital heart disease (CHD). To examine the association between material hardship and mortality and healthcare utilization among infants with CHD starting at prenatal diagnosis. We enrolled pregnant patients at the time of prenatal diagnosis of fetal CHD. We collected level of material hardship and sociodemographic factors using validated surveys at the time of prenatal diagnosis and when the infant was 6 months of age. We assessed outcomes at birth hospitalization and during the first six months of life. The cohort include 58 families with a diverse racial and socioeconomic make-up. There was an overall mortality rate of 8.6% in the first 6 months. At baseline 36.1% of the families had at least one type of material hardship compared to 27.6% at 6 months. Families with income at < 200% of the federal poverty line were 27.6% baseline compared to 20.7% at 6 months. Income < 200% of the federal (financial insecurity) at baseline was associated with increased mortality (p = 0.05). There was an increased length of stay those with housing insecurity (median LOS 28 vs. 16 days, p = 0.032) and in those with any type of material hardship (median LOS 28 vs. 16 days, p = 0.047). Increased levels of material hardship and financial insecurity were associated with increased mortality and length of stay in infants with CHD. Further research is needed to assesses the benefit of interventions designed to identify and address these gaps early since they may reduce morbidity and healthcare utilization. Keywords: Material Hardship, Health Equity, Prenatal Diagnosis.