Prior work regarding counseling patients about congenital heart defects (CHD) has focused on their perceptions about accurate communication of cardiac anatomy, and the emotional support received from the provider. The objectives of this study were to identify the additional CHD counseling-specific challenges and areas for future intervention, using a practical communication framework. This is a secondary analysis of qualitative data provided by caretakers of infants who received congenital heart surgery from 2019 to 2020 in the Chicagoland area. While the survey in the primary study pertained to barriers in obtaining prenatal diagnosis, respondents with both prenatal and postnatal diagnosis reported challenges to effective counseling. Qualitative data measuring counseling challenges were collected from semi-structured phone interviews. Thematic analysis was performed using an inductive approach. Themes were organized into five domains using SPIKES (Setting, Perception, Invitation, Knowledge, Empathy, and Summarize/Strategy), a previously validated framework to help clinicians effectively break bad news. Among 160 survey respondents, 35 (21.9%) reported a challenge during CHD counseling that they received. In total, 12 challenges were identified and spanned all six SPIKES domains. The three most common challenges were as follows: perception of repeated imaging studies for accurate diagnosis or management (n = 19, Knowledge), the lack of cardiologist presence at the time of initial CHD detection (n = 8, Setting), and insufficient information provided about the CHD diagnosis (n = 7, Knowledge). Patients perceive counseling as a key component of prenatal diagnosis of CHD and identify the challenges that exist at all stages of the counseling process. These findings suggest that effective counseling extends beyond conveying information about anatomy and prognosis.

Background Mixed pulmonary disease with pulmonary regurgitation (PR) and stenosis (PS) in repaired tetralogy of Fallot (rTOF) can negatively impact ventricular health. Myocardial strain has been shown to be more sensitive at detecting occult ventricular dysfunction compared to right ventricular ejection fraction (RV EF). We hypothesize that rTOF patients with predominant PS will have lower RV global longitudinal strain (RVGLS) prior to and post-transcatheter pulmonary valve replacement (TPVR). Methods A retrospective cohort of rTOF patients who underwent cardiac magnetic resonance (CMR) and cardiac catheterization for right ventricular pressure (RVSP) measurement were analyzed at three time points: before valve implantation, at discharge and within 18 months post-TPVR. Patients were dichotomized into three groups based on RVSP: 0-49%, 50-74%, and >75%. RVGLS and left ventricular (LV) GLS by speckle tracking echocardiography (STE) were obtained from the apical 4-chamber using TomTec software (TOMTEC IS, Germany). Results Forty-eight patients were included. RV EF was not associated with a significant change in RV or LV GLS (p=0.7). RV GLS showed the greatest improvement immediately after valve implantation. Higher pre-implantation RVSP was found to correlate with worse strain (p=0.001). Overall, average RV strain magnitude was higher when pre-implantation RVSP was less than 50% and had greater improvement over the three time points. Higher post-implantation RVSP correlated with lower strain magnitude. Conclusion Patients with significant PS (>50%) may benefit from earlier PVR and not depend solely on RV size and EF. Myocardial strain may be a more sensitive marker of function; however, larger, prospective studies are needed.

Fetal arrhythmias are rare and carry significant morbidity and mortality without appropriate treatment. Initial reports exist of fetal arrhythmia in the setting of maternal COVID-19 infection. Our study sought to evaluate incidence of fetal arrhythmia before and during the COVID-19 pandemic at our institution. This retrospective cohort study from a tertiary care fetal cardiac center utilized the institutional REDCap database to search fetal arrhythmia diagnostic codes. Medical records of mother–fetus dyads were reviewed and data were collected on diagnoses, gestational age, treatment regimen, and postnatal outcomes. Patients were divided into pre-COVID and peri-COVID segments. 8368 total pregnancies were evaluated during the 7.3 years of study period. Forty-five patients (0.5%) had a significant fetal arrhythmia and were included in this study: 19 (42%) in the pre-COVID-19 group and 26 (58%) in the peri-COVID-19 group. No patients had associated congenital heart disease. There was a notable increase in the incidence of fetal supraventricular tachycardia (SVT) (1.82 per 1000 vs 2.65 per 1000 pregnancies) and complete heart block (1.04 per 1000 vs 1.77 per 1000 pregnancies) but no apparent change in other tachyarrhythmias during the COVID era. The proportion of antibody-mediated complete heart block increased from 50 to 87.5%. There was also an increase in the percentage of SVT patients requiring postnatal treatment during COVID-19 (53.8% vs 62.5%). Our experience shows an increased incidence of some fetal arrhythmia diagnoses during the COVID-19 pandemic. Additional multi-center studies will be necessary to fully evaluate the increased burden of fetal arrhythmias during the COVID-19 era as well as to elucidate etiology.

Fetal cardiology has evolved over the last 40 years and changed the timing of diagnosis and counseling of congenital heart disease, decision-making, planning for treatment at birth, and predicting future surgery from the postnatal to the prenatal period. Ethical issues in fetal cardiology transect multiple aspects of biomedical ethics including improvement in prenatal detection and diagnostic capabilities, access to equitable comprehensive care that preserves a pregnant person’s right to make decisions, access to all reproductive options, informed consent, complexity in shared decision-making, and appropriate use of fetal cardiac interventions. This paper first reviews the literature and then provides an ethical analysis of accurate and timely diagnosis, equitable delivery of care, prenatal counseling and shared decision-making, and innovation through in utero intervention.

Congenital complete heart block (CCHB) is associated with high intrauterine and post-natal mortality. Prenatal detection and management, as well as appropriate delivery planning, may improve the outcomes in CCHB. We describe a rare case of CCHB that initially presented with fetal ascites and high-grade second-degree heart block noted on fetal echocardiography. The mother was noted to be positive for anti-SSA antibodies, and treatment with maternal steroids was started in an effort to reverse the fetal cardiac conduction abnormality. However, the fetal cardiac rhythm progressed to complete heart block by the follow up evaluation and the fetus had a continual declination of heart rate throughout the pregnancy to a low fetal heart rate of 25 beats per minute (bpm). This case demonstrates the lowest fetal ventricular rate documented in the literature and illustrates a severe presentation of a rare disease process. An overview of the existing knowledge related to etiology, prenatal evaluation with fetal echocardiography and fetal magnetocardiography, prenatal management, and delivery planning in fetuses with prenatally detected CCHB is included.

The patient in this case was an otherwise healthy 12-year-old boy who presented to our hospital for monitoring after exposure to a pesticide. His medical history was significant only for attention-deficit/hyperactivity disorder, for which he took daily stimulant therapy. In the trailer he shared with his grandmother, an unknown amount of the pesticide tablets were accidentally spilled indoors. In an attempt to clean it up, they vacuumed up the substance, which reportedly caused the vacuum to explode, dispersing the pesticide in the air. When both the patient and his grandmother began vomiting a few hours later, they were taken to a local emergency department by family members. The grandmother experienced cardiac arrest and expired in the local emergency department within 12 hours of exposure. The patient was then transferred to our tertiary medical center for additional monitoring because of his pesticide exposure. He was initially monitored with no concerning symptoms. His heart rate was mildly elevated at rest, averaging 110 to 130 beats per minute, but his blood pressure was normal for age and height. Less than 48 hours after initial pesticide exposure, he developed a new, prominent gallop rhythm on physical examination. An echocardiogram was performed, which demonstrated a mildly dilated left ventricle with severely diminished left ventricular systolic function. The fractional shortening was 22%, and the ejection fraction was 36%. The electrocardiogram (ECG) was also abnormal, demonstrating sinus tachycardia, left axis deviation, poor R-wave progression, and diffuse T-wave flattening (Fig 1). All intervals, including QRS duration (88 msec) and QTc (444 msec), were within normal limits.As a whole, cardiomyopathy is a diverse class of cardiac diseases. Dilated cardiomyopathy is characterized by the development of dilated, poorly functional ventricles with normal wall thickness not precipitated by an abnormal cardiac preload or afterload.1,2 Dilated cardiomyopathy can be accompanied by cardiac arrhythmias and symptoms of heart failure. The ECGs of patients with dilated cardiomyopathy may be normal but may also demonstrate varying types of heart block, atrial fibrillation, or even ventricular arrhythmias.1,2 Echocardiography demonstrates increased ventricular dimensions, ventricular hypokinesia, and a decreased fractional shortening. Symptomatic relief can often be achieved through the use of diuretics to decrease the volume load placed on the heart. Angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, aldosterone antagonists, vasodilators, and β-blockers are all associated with increased survival and are used in almost all patients with dilated cardiomyopathy, from those with severe heart failure to those with asymptomatic left ventricular systolic dysfunction.2After an extensive literature review and discussion with several national toxicologists, we determined that this was a documented effect of exposure to aluminum phosphide pesticides. This was also supported by review of the patient’s admission laboratory values, which demonstrated a metabolic acidosis (bicarbonate 15 mEq/L) and mild hyperglycemia (glucose 147 mg/dL), both of which quickly resolved on subsequent testing. To ensure this was not a coincidental finding of cardiomyopathy of another cause, a cardiomyopathy evaluation was undertaken. Acute viral titers, including herpes simplex virus, Epstein-Barr virus, echovirus, cytomegalovirus, coxsackievirus, and adenovirus, as well as convalescent titers obtained 20 days later, were negative with the exception of a positive mumps immunoglobulin G, thus indicating an appropriate immune response to vaccines without any additional evidence of acute infection. The patient’s erythrocyte sedimentation rate at diagnosis was normal (8 mm/hr). C-reactive protein was only mildly elevated (1.063 mg/dL). Thyroid studies were normal, as were profiles of amino acids, acylcarnitines, and carnitines. An initial troponin was elevated (1.308 ng/mL), but this quickly normalized.Supportive treatment was then initiated. Several national toxicologists recommended placing the patient on intravenous (IV) fluids, which could aid in pesticide excretion. Although asymptomatic, he was also started on afterload reduction using lisinopril due to his diminished left ventricular systolic function. A 24-hour Holter monitor returned with normal findings, and a follow-up echocardiogram performed 3 days later demonstrated normal left ventricular systolic function with an improvement of his fractional shortening (32%) and ejection fraction (61.3%). Another echocardiogram done during his outpatient follow-up 20 days later continued to demonstrate normal left ventricular systolic function.Aluminum phosphide poisoning is a rarely reported entity in the United States. Most reports are due to job-related exposure or suicidal attempts occurring outside the United States.3–6 Childhood exposure is even less common, with the exception of older teenagers who ingest aluminum phosphide containing pesticide pellets with suicidal intent.4 The national poison control centers data from 2013 reported that 3.27% of reported exposures were due to pesticides in general, and only 0.006% were due to aluminum phosphide exposure, the majority of which occurred in adults.7 There have been rare case reports of unintentional aluminum phosphide poisoning of entire families that have reported fatalities due to acute cardiopulmonary collapse in children aged 15 months up to 6 years.8,9 Both of these reports demonstrated that the cardiac deterioration could occur far removed from the initial exposure. These reported families had repeated exposure to the aluminum phosphide-containing pesticides before the poisoning symptoms were recognized, which differs from our case.Aluminum phosphide is an inorganic compound commercially available as pesticides that are used to protect crops from insects and rodents.10 Commercial names for aluminum phosphide include Fumtoxin, L-Fume, Tri-Tox, Fumiphos, Phostoxin, Fumex, Gastoxin, and Quik-Fume. It is manufactured in dust, granular, and pellet forms. These readily react to form phosphine gas (PH3) after coming into contact with water, moisture, or the acidic content in the human stomach.3,10 Once inhaled or ingested, phosphine gas leads to the production and accumulation of reactive oxygen species (ROS) at the cellular level and subsequent lipid peroxidation. It is this extensive ROS generation that is believed to be responsible for the wide systemic toxicities of phosphine gas exposure. Specifically, these effects cause alterations in the cardiac transmembrane action potentials, which lead to dysrhythmias.3 They may also cause ischemic effects that can induce focal areas of necrosis as well as cardiac failure.3Phosphide exposure is known to rapidly affect multiple organ systems including the respiratory, cardiovascular, hepatic, renal, hematologic, and gastrointestinal systems. Early symptoms of acute exposure can vary on the exposure route and dose, but typically include fatigue, dyspnea, nausea, vomiting, abdominal pain, hypotension, and tachycardia.11–13 Progression of illness can be rapid and may lead to organ failure of any of the affected organs systems, but the most common effects include acute respiratory failure and cardiac shock.11–13 Other potential systemic results of exposure include acute hepatitis, acute renal failure, and disseminated intravascular coagulation. Multiple metabolic derangements have also been described including abnormalities in sodium, potassium, and/or magnesium levels, lactic acidosis, and hypo- or hyperglycemia.11–13From a cardiac perspective, phosphide exposure can present with a variety of complications, including peripheral circulatory collapse, dysrhythmias, and, rarely, acute cardiomyopathy.3,4,8,14 One study following 20 patients with aluminum phosphide exposure reported cardiac manifestations occurring within 12 hours in all patients, although there are case reports with presentation delayed up to 48 hours after exposure.4,5,9 Cardiac dysrhythmias have been reported at a rate of 50% in 1 study and may include atrial fibrillation or flutter, junctional rhythms, supraventricular tachycardia, ventricular tachycardia or fibrillation, QT prolongation, and varying degrees of heart block.4,5,15 Additional ECG findings of ST-segment elevation or depression and significant T-wave inversions may be seen.4,9 Direct cardiac toxicity and myocardial dysfunction may present as ventricular hypokinesia, cardiomyopathy, myocarditis, or pericarditis.4,13,16 One patient reportedly had prolonged ECG abnormalities attributed to subendocardial ischemia.4Perhaps because of the wide range of cytotoxic effects that phosphide exposure has, no antidote has yet been identified. If exposure occurs, the Centers for Disease Control recommends decontamination as soon as possible by experienced personnel.17 Treatment remains largely supportive, and several therapeutic strategies have been proposed to aid in the management of acute exposure. Maintaining renal perfusion with IV fluids may aid in phosphine elimination.5,18 Digoxin and other inotropic agents have had some effect in treating cardiovascular collapse and ventricular dysfunction in these cases.19 In 1 case report, calcium gluconate and magnesium sulfate were used for cardiac membrane stabilization.11 In 1 study of children after ingestion of aluminum phosphide pesticides, magnesium sulfate was associated with better survival.20 Other proposed treatments include high doses of vitamin C as an antioxidant or IV magnesium and/or N-acetylcysteine to replenish intracellular glutathione stores and reduce systemic ROS levels; however, none of these methods have as yet demonstrated themselves to be particularly effective.5,6,21 There is a report of using of an intra-aortic balloon pump successfully for a patient with myocarditis and cardiac shock whose clinical condition initially worsened despite inotropic support.22 Our patient received only minimal supportive treatment with IV fluids and lisinopril therapy before recovery. Several patients with phosphide-induced cardiomyopathy have reportedly experienced improvements of their left ventricular function over the course of their hospital stay, as our patient did.23 It is likely that this rapid recovery is due to clearance of phosphide from the patient’s system.Although aluminum phosphide exposure is rare in the United States, it presents a treatment dilemma given the paucity of definitive information available in the literature, especially in relation to children. Although there are no current consensus statements, supportive treatment appears to be the best choice for now. However, the best treatment is always the prevention of exposure by limiting usage of these pesticides, especially in the presence of children, and appropriately storing and disposing of them. If a pesticide spill occurs in the home or workplace, families should contact the national poison control hotline (1-800-222-1222) for instructions on appropriate cleanup and treatment as needed.

Rearrangements in the nuclear protein in testis (NUT) gene cause carcinomas that represent a rare but aggressive tumor type that often present at advanced stages in midline structures. Survival rarely exceeds 12 months from the time of diagnosis. There have been no reports of a primary cardiac presentation, and few studies have reported on the numerous treatment strategies. Given their aggressive and invasive nature, NUT midline carcinomas present a therapeutic dilemma. Treatment may include surgical resection, chemotherapy, or radiotherapy, but no consistently successful treatment has been established. Surgical resection is indicated to reduce symptomatic mass effect whenever present. Novel therapies with bromodomain extra-terminal inhibitors may be associated with potential survival benefit. Here, we describe an unusual presentation of this tumor. Literature review with management considerations is underlying.

Case: The patient was a 12-year-old African American male who presented to our hospital from a community hospital for further care of a worsening left-sided pneumonia. His mother reported a 3-week history of intermittent cough and fever at home before his community hospital admission. She had been treating these symptoms with over-the-counter cough remedies at home but became concerned because his symptoms were not improving. The patient also had diagnoses of idiopathic bronchiectasis and selective immunoglobulin (Ig) A deficiency (reported according to history). He had required hospital admission for bacterial pneumonia several times during his life, and he was well below the 1% mark for height, weight, and BMI for his age. At the community hospital, he was treated aggressively with weight-appropriate doses of ceftriaxone and vancomycin. Despite treatment, the patient’s symptoms had not improved. Repeat imaging at the community hospital demonstrated worsening status of his pneumonia. On admission to our hospital, he was not in marked respiratory distress requiring invasive respiratory support. However, it was noted that he was extremely thin, had decreased to no air movement at the left lung base, had severe digital clubbing, and was coughing frequently with dyspnea on minimal exertion. His chest radiograph (Fig 1) and chest computed tomography scan (Fig 2) from the community hospital were interpreted by our pediatric radiologists to show a left-sided necrotizing pneumonia with concern for empyema. FIGURE 1 Chest radiograph at admission to the community hospital showing opacification of much of the left hemithorax with areas of cystic lucency that were thought to represent necrotizing pneumonia. FIGURE 2 Chest radiograph from the community hospital showing evidence of severe necrotizing pneumonia on left with multiple fluid collections and gas collections throughout the consolidated left lung. A large, complex pleural fluid collection is also seen with areas of gas and enhancing septations concerning for …