Prospective Evaluation of High Titer Autoantibodies and Fetal Home Monitoring in the Detection of Atrioventricular Block Among Anti-SSA/Ro Pregnancies.

OBJECTIVE: This prospective study of pregnant patients, Surveillance To Prevent AV Block Likely to Occur Quickly (STOP BLOQ), addresses the impact of anti-SSA/Ro titers and utility of ambulatory monitoring in the detection of fetal second-degree atrioventricular block (AVB). METHODS: Women with anti-SSA/Ro autoantibodies by commercial testing were stratified into high and low anti-52-kD and/or 60-kD SSA/Ro titers applying at-risk thresholds defined by previous evaluation of AVB pregnancies. The high-titer group performed fetal heart rate and rhythm monitoring (FHRM) thrice daily and weekly/biweekly echocardiography from 17-26 weeks. Abnormal FHRM prompted urgent echocardiography to identify AVB. RESULTS: Anti-52-kD and/or 60-kD SSA/Ro met thresholds for monitoring in 261 of 413 participants (63%); for those, AVB frequency was 3.8%. No cases occurred with low titers. The incidence of AVB increased with higher levels, reaching 7.7% for those in the top quartile for anti-60-kD SSA/Ro, which increased to 27.3% in those with a previous child who had AVB. Based on levels from 15 participants with paired samples from both an AVB and a non-AVB pregnancy, healthy pregnancies were not explained by decreased titers. FHRM was considered abnormal in 45 of 30,920 recordings, 10 confirmed AVB by urgent echocardiogram, 7 being second-degree AVB, all <12 hours from normal FHRM and within another 0.75 to 4 hours to echocardiogram. The one participant with second/third-degree and two participants with third-degree AVB were diagnosed by urgent echocardiogram >17 to 72 hours from an FHRM. Surveillance echocardiograms detected no AVB when the preceding interval FHRM recordings were normal. CONCLUSION: High-titer antibodies are associated with an increased incidence of AVB. Anti-SSA/Ro titers remain stable over time and do not explain the discordant recurrence rates, suggesting that other factors are required. Fetal heart rate and rhythm (FHRM) with results confirmed by a pediatric cardiologist reliably detects conduction abnormalities, which may reduce the need for serial echocardiograms.

Pediatric Cardiology Condolence Letter Writing: Does a Fellowship Curriculum Impact Practice?

CONTEXT: Condolence letter (CL) writing after the death of a child is an important opportunity for humanism. Pediatric cardiology fellowship training now recognizes the importance of palliative care, but rarely includes CL education, despite its fragile patient population. OBJECTIVES: To address this professionalism gap, a formal CL writing curriculum was created and implemented in a pediatric cardiology fellowship. This study investigated the impact of the curriculum on pediatric cardiology CL writing, and broader CL practices and beliefs. METHODS: Pediatric cardiology fellows at a high volume urban academic program from 2000 to 2022 were divided into two cohorts (exposure to CL curriculum [2014-2022] vs. no exposure [2000-2013]) and responded by anonymous electronic multiple choice and open ended survey to assess the CL curriculum and describe current CL practices and beliefs. Impact of curriculum elements was determined by ordinal ranking. A 5-point Likert scale was used to report physician behaviors. Chi-square tests of independence were utilized for group comparisons. RESULTS: The overall survey response rate was 59% (63/107). Cardiologists who participated in the curriculum (64%, 35/55) were more likely to report writing CLs (80% vs. 40%; P < 0.01). Impactful curriculum elements included the opportunity for all fellows to contribute to a CL (78%) and identifying a primary fellow to write the CL (66%). A majority (>75%) of curriculum participants agreed that formal teaching increased their frequency, ability, and comfort in writing CLs. CONCLUSION: Development of condolence expression educational programs in pediatric cardiology training should be expanded.

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.

Care of the Fetus With Congenital Cardiovascular Disease: From Diagnosis to Delivery.

BACKGROUND: The majority of congenital cardiovascular disease including structural cardiac defects, abnormalities in cardiac function, and rhythm disturbances can be identified prenatally using screening obstetrical ultrasound with referral for fetal echocardiogram when indicated. METHODS: Diagnosis of congenital heart disease in the fetus should prompt assessment for extracardiac abnormalities and associated genetic abnormalities once maternal consent is obtained. Pediatric cardiologists, in conjunction with maternal-fetal medicine, neonatology, and cardiothoracic surgery subspecialists, should counsel families about the details of the congenital heart defect as well as prenatal and postnatal management. RESULTS: Prenatal diagnosis often leads to increased maternal depression and anxiety; however, it decreases morbidity and mortality for many congenital heart defects by allowing clinicians the opportunity to optimize prenatal care and plan delivery based on the specific lesion. Changes in prenatal care can include more frequent assessments through the remainder of the pregnancy, maternal medication administration, or, in selected cases, in utero cardiac catheter intervention or surgical procedures to optimize postnatal outcomes. Delivery planning may include changing the location, timing or mode of delivery to ensure that the neonate is delivered in the most appropriate hospital setting with the required level of hospital staff for immediate postnatal stabilization. CONCLUSIONS: Based on the specific congenital heart defect, prenatal echocardiogram assessment in late gestation can often aid in predicting the severity of postnatal instability and guide the medical or interventional level of care needed for immediate postnatal intervention to optimize the transition to postnatal circulation.

Loss of Consciousness in the Young Child.

In the very young child (less than eight years of age), transient loss of consciousness represents a diagnostic and management dilemma for clinicians. While most commonly benign, syncope may be due to cardiac dysfunction which can be life-threatening. It can be secondary to an underlying ion channelopathy, cardiac inflammation, cardiac ischemia, congenital heart disease, cardiomyopathy, or pulmonary hypertension. Patients with genetic disorders require careful evaluation for a cardiac cause of syncope. Among the noncardiac causes, vasovagal syncope is the most common etiology. Breath-holding spells are commonly seen in this age group. Other causes of transient loss of consciousness include seizures, neurovascular pathology, head trauma, psychogenic pseudosyncope, and factitious disorder imposed on another and other forms of child abuse. A detailed social, present, past medical, and family medical history is important when evaluating loss of consciousness in the very young. Concerning characteristics of syncope include lack of prodromal symptoms, no preceding postural changes or occurring in a supine position, after exertion or a loud noise. A family history of sudden unexplained death, ion channelopathy, cardiomyopathy, or congenital deafness merits further evaluation. Due to inherent challenges in diagnosis at this age, often there is a lower threshold for referral to a specialist.

Right Atrial Conduit Phase Emptying Predicts Risk of Adverse Events in Pediatric Pulmonary Arterial Hypertension.

BACKGROUND: Idiopathic pulmonary arterial hypertension (PAH) is a severe disease associated with a 20% 5-year mortality, often due to right heart failure. Recent studies suggest that compensatory changes in right atrial (RA) function may precede other clinical and echocardiographic evidence of right ventricular dysfunction. No prior prospective study has evaluated the role of RA emptying pattern as a prognostic marker of adverse clinical events in pediatric PAH. OBJECTIVE: To demonstrate whether RA fractional emptying indices will prospectively predict risk of adverse clinical outcomes in pediatric PAH patients. METHODS: Single-center, prospective cohort analysis of 41 patients with idiopathic or heritable PAH and 1:1 age and sex-matched controls with normal echo and electrocardiogram. Right atrial area (RAA) was measured just prior to tricuspid valve opening (RAAmax), at electrical p wave (RAAp), and just after tricuspid valve closing (RAAmin). Right atrial conduit fraction percent (RA cF%) was defined as the percentage of total RAA change happening prior to the electrical p wave = (RAAmax - RAAp)/(RAAmax - RAAmin) ∗ 100. Clinical worsening was analyzed with a predefined composite adverse event outcome. RESULTS: RA measurements were technically feasible in all study participants. The PAH patients (median age 11.9 years) had decreased RA cF% compared with controls (P < .0001), and PAH subjects with lower RA cF% demonstrated higher right ventricular systolic (R = -0.49, P = .019) and end-diastolic (R = -0.52, P = .012) pressure than those with higher RA cF%. Sixteen subjects had a clinical event. Right atrial cF% (hazard ratio = 0.09; P < .001) was highly prognostic for risk of adverse clinical event with area under the curve = 0.90 on receiver operating characteristic curve analysis (median 3.2-year follow-up). CONCLUSIONS: Right atrial conduit phase emptying is significantly altered in pediatric PAH. Within the PAH population, decreased RA cF% was prognostic for risk of clinical worsening. The combination of accuracy and ease of measurement could make RA cF% a clinically useful, noninvasive biomarker of early right heart failure and risk of disease progression in pediatric PAH.

Implications of Atrial-Level Shunting by Echocardiography in Newborns with Congenital Diaphragmatic Hernia.

OBJECTIVE: To determine whether postnatal echocardiographic indices of left ventricular (LV) size and function are associated with atrial-level shunt direction and investigate whether a relationship exists between LV function and LV size in patients with congenital diaphragmatic hernia (CDH). STUDY DESIGN: This was a single-center retrospective study of 51 neonates with CDH evaluated at Children's Hospital Colorado. Initial postnatal echocardiograms were analyzed for cardiac dimensions, valvar integrity, cardiac time intervals, and biventricular function. Comparisons were made between neonates with left-to-right vs right-to-left atrial-level shunting, as well as between those with decreased (ejection fraction <55%) vs normal (ejection fraction ≥55%) LV function. RESULTS: The majority (82.4%) of infants with CDH had a left-to-right atrial-level shunt, and 84.3% had systemic or suprasystemic pulmonary artery pressure. Decreased LV function was demonstrated in 37.2% and was associated with smaller LV volumes and worse outcomes, including the need for extracorporeal membrane oxygenation (ECMO) and survival. CONCLUSIONS: Left-to-right atrial-level shunting is common in neonates with severe CDH, even in the presence of suprasystemic pulmonary artery pressure. LV dysfunction correlates with decreased LV volumes and is associated with adverse neonatal outcomes, including increased need for ECMO and decreased survival.

Risk Stratification of Fetal Cardiac Anomalies in an Underserved Population Using Telecardiology.

OBJECTIVE: To evaluate a fetal telecardiology program in a medically underserved area. METHODS: We conducted a prospective case series of pregnant women at 18-38 weeks of gestation with risk factors for fetal congenital heart disease. Obstetric ultrasonographers performed fetal echocardiograms (local site) that were read in real time. The results were given to the mother by a fetal cardiologist at a children's hospital 243 miles and two mountain passes away (distant site). We evaluated the fetal telecardiology program in five domains: 1) education of obstetric ultrasonographers before initiation of telecardiology services, 2) process and efficiency, 3) patient satisfaction, 4) economic effects, and 5) accuracy of cardiac diagnosis and success of risk stratification. RESULTS: The program was initiated on November 12, 2015, and here we describe its first 37 months. Over the initial training period of 3 months and about 70 examinations, obstetric ultrasonographers improved their identification of fetal congenital heart disease. Telecardiology was performed once a week and also for suspected fetal congenital heart disease or arrhythmia outside clinic hours, for a total of 455 examinations. All mothers preferred having their fetal cardiac evaluations performed locally as opposed to traveling to the distant center. The estimated cost to parents for fetal cardiac evaluation at the distant center was nine times greater than that of telecardiology ($581 vs $61). Congenital heart disease or arrhythmia was diagnosed in 28 and 15 fetuses, respectively; there was one false-negative result. All fetuses were correctly risk-stratified with respect to delivery location. CONCLUSIONS: Neither diagnostic quality nor patient satisfaction were sacrificed with telecardiology. The program was feasible, empowered the local health care providers and ultrasonographers, offered strong economic advantages to families, and offered the benefit of timely standard-of-care, face-to-face consultation without travel. Based on the success of this program, further studies are warranted to assess its replicability.

Neonatal Palliative Care for Complicated Cardiac Anomalies: A 10-Year Experience of an Interdisciplinary Program at a Large Tertiary Cardiac Center.

OBJECTIVES: To report the outcomes of a Neonatal Palliative Care (NPC) Program at a large tertiary cardiac center caring for a subset of fetuses and neonates with life-limiting cardiac diagnoses or cardiac diagnoses with medical comorbidities leading to adverse prognoses. STUDY DESIGN: The Neonatal Comfort Care Program at New York-Presbyterian Morgan Stanley Children's Hospital/Columbia University Medical Center is an interdisciplinary team that offers the option of NPC to neonates prenatally diagnosed with life-limiting conditions, including single ventricle (SV) congenital heart disease (CHD) or less severe forms of CHD complicated by multiorgan dysfunction or genetic syndromes. RESULTS: From 2008 to 2017, the Neonatal Comfort Care Program cared for 75 fetuses or neonates including 29 with isolated SV CHD, 36 with CHD and multiorgan dysfunction and/or severe genetic abnormalities, and 10 neonates with a prenatal diagnosis of isolated CHD and postnatal diagnoses of severe conditions who were initially in intensive care before transitioning to NPC because of a poor prognosis. CONCLUSIONS: At New York-Presbyterian Morgan Stanley Children's Hospital/Columbia University Medical Center, a large tertiary cardiac center, 13.5% of parents of fetuses or neonates with isolated SV CHD opted for NPC. Twenty-six of 29 newborns with SV CHD treated with NPC died. Of the remaining, 2 neonates with mixing lesions are alive at 3 and 5 years of age, and 1 neonate was initially treated with NPC and then pursued surgical palliation. These results suggest that NPC is a reasonable choice for neonates with SV CHD.