Food insecurity in children with heart disease.

PURPOSE OF REVIEW: This review discusses the epidemiology of food insecurity (FI) and its consequences in children with congenital heart disease. We aimed to highlight current interventions to screen and address food insecurity in the context of pediatric cardiology and to offer strategies for providers to engage in this meaningful work. RECENT FINDINGS: Food insecurity is consistently associated with poor health outcomes in children. In the United States, 17.3% of households with children experience FI. Nonwhite and single-parent families are disproportionately affected. Interestingly, because of a low-quality diet, FI is associated with childhood obesity, putting affected children at increased risk for cardiovascular morbidity and mortality over time. Children with congenital heart disease are susceptible to poor outcomes due to unique altered metabolic demands, increased risk for growth impairment, frequent need for specialized feeding regimens, and additional morbidity associated with heart surgery in underweight children. SUMMARY: Today, the burden of screening for FI is most commonly placed on general pediatricians. Considering the importance of nutrition to cardiovascular health and general wellbeing, and the ease with which screening can be performed, pediatric cardiologists and other subspecialists should take a more active role in FI screening.

Fetal Diagnosis of Supravalvular Aortic Stenosis and Pulmonary Stenosis in a Family with Non-Syndromic Elastin Mutation.

Supravalvular aortic stenosis (SVAS) has been well described in Williams-Beuren Syndrome and non-syndromic elastin (ELN) mutations. Non-syndromic ELN mutations are inherited in an autosomal dominant pattern with incomplete penetrance and variable expressivity. ELN haploinsufficiency leads to progressive arteriopathy, typically affecting the aortic sinotubular junction. Multi-level pulmonary stenosis has also been reported and biventricular obstruction may portend a worse prognosis. Fetal presentation of ELN mutation with SVAS has not been previously reported in the literature. We present a case of fetal diagnosis of SVAS and multi-level pulmonary stenosis in a family with a known pathogenic ELN mutation (Exon 6, c.278del [p.Pro93Leufs*29]). On the fetus' initial fetal echo, there was only mild flow acceleration through the aortic outflow tract, however, she went on to develop progressive bilateral obstruction. In the early post-natal period, the child was clinically asymptomatic and showed similar mild SVAS and mild valvar and supravalvular pulmonary stenosis. Our case highlights the need for serial monitoring of fetuses with suspected or confirmed ELN arteriopathy.

Congenital Bronchial Stenosis Presenting as Neonatal Respiratory Distress: A Case Report.

BACKGROUND: Neonatal respiratory distress has a broad differential that includes cardiac, pulmonary, anatomic, and infectious etiologies. Congenital stenotic lesions of the trachea and bronchus are rare and can occur anywhere along the tracheobronchial tree. Patients with tracheobronchial stenosis typically present in the neonatal period with respiratory distress. CASE REPORT: We present a case of a 10-day-old term female who presented to the emergency department (ED) with tachypnea and increased work of breathing. She was found to have congenital bronchial stenosis of her right mainstem bronchus. She was stabilized in the ED and remained in the neonatal intensive care unit until successful slide tracheoplasty was performed. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Congenital bronchial stenosis is a rare etiology of respiratory distress in a neonate. Anatomic lower airway abnormalities are an important cause of neonatal tachypnea and must remain on the differential. In addition to respiratory stabilization with noninvasive or invasive support, evaluation should be directed at determining the location and anatomic characteristics of the area of stenosis.

Long-Term Survival of Patients With Coarctation Repaired During Infancy (from the Pediatric Cardiac Care Consortium).

Patients who undergo coarctation repair during infancy have excellent early survival but long-term survival is unknown. We aimed to describe the long-term survival of patients with coarctation repaired during infancy and determine predictors of mortality. We performed a retrospective cohort study using data from the Pediatric Cardiac Care Consortium for patients with coarctation who underwent surgical repair before 12 months of age between 1982 and 2003. Long-term transplant-free survival was obtained by linkage with the National Death Index and the Organ Sharing Procurement Network. Kaplan Meier survival plots were constructed, and univariate and multivariable analyses were performed to determine predictors of mortality. We identified 2,424 coarctation patients who met inclusion criteria. At 20 years postoperatively, 94.5% of all patients and 95.8% of those discharged after initial operation remained alive, respectively. Significant multivariable predictors of mortality included surgical weight <2.5 kg (hazard ratio [HR] 3.70, 95% confidence interval [CI] 2.19 to 6.24), presence of a genetic syndrome (HR 2.40, 95% CI 1.13 to 5.10), and repair before 1990 (HR 1.91, 95% CI 1.09 to 3.34). None of the other factors examined including age at repair, gender, coarctation type, or surgical approach were found to be statistically significant. Over half of the deaths were due to the underlying congenital heart disease or other cardiovascular etiology. Overall long-term survival of patients who undergo coarctation repair during infancy is excellent. However, patients do experience small continued survival attrition throughout early adulthood. Ongoing monitoring of this cohort is necessary to assess late mortality risk.

Long-Term Survival After Arterial Versus Atrial Switch in d-Transposition of the Great Arteries.

BACKGROUND: The arterial switch operation (ASO) became the procedure of choice for dextro-transposition of the great arteries (d-TGA) nearly 30 years ago, but the long-term results of this operation are unknown. We aimed to compare the long-term transplant-free survival of patients with d-TGA who underwent ASO versus atrial switch in the Pediatric Cardiac Care Consortium. METHODS: We performed a retrospective cohort study of d-TGA patients undergoing ASO or atrial switch in the United States between 1982 and 1991. Long-term transplant-free survival was obtained by linking Pediatric Cardiac Care Consortium data with the National Death Index and the Organ Procurement and Transplant Network. Kaplan-Meier survival plots were constructed, and multivariable regression was used to compare long-term transplant-free survival. RESULTS: Of 554 d-TGA patients who underwent ASO (n = 259) or atrial switch (n = 295), the 20-year overall transplant-free survival was 82.1% for those undergoing ASO and 76.3% for those who had atrial switch procedure. Adjusted overall transplant-free survival beyond 10 years after operation was superior for ASO compared with atrial switch (hazard ratio 0.07, 95% confidence interval: 0.01 to 0.52, p = 0.009). During this period, the ASO had higher in-hospital mortality than the atrial switch (21.6% versus 12.9%, p = 0.007). After excluding patients with in-hospital mortality, the transplant-free survival 20 years after repair was 97.7% for the ASO patients versus 86.3% for the atrial switch patients. CONCLUSIONS: Despite initial higher in-hospital mortality for ASO during the study period, there is a significant long-term transplant-free survival advantage for ASO as compared with atrial switch for d-TGA surgery. Ongoing monitoring is required to assess late risk of cardiovascular disease.

Osseous injuries of the foot: an imaging review. Part 3: the hindfoot.

Injuries to the foot are a common cause for presentation to the emergency department, and imaging is often used to aid in the diagnosis. The foot can be divided into three distinct anatomic regions: the forefoot, midfoot and hindfoot. Our manuscripts comprise a three-part imaging review in which we address the use of radiography as well as advanced imaging modalities. We provide pearls to radiographic interpretation and discuss prognostic implications and classification systems. Part 1 addresses forefoot injuries, part 2 reviews midfoot injuries and part 3 covers the hindfoot.

Osseous injuries of the foot: an imaging review. Part 1: the forefoot.

Injuries to the foot are a common cause for presentation to the ED, and imaging is often used to aid in the diagnosis. The foot can be divided into three distinct anatomical regions: the forefoot, midfoot and hindfoot. Our manuscripts comprise a three-part imaging review in which we address the use of radiography as well as advanced imaging modalities. We provide pearls to radiographic interpretation and discuss prognostic implications and classification systems. Part 1 addresses forefoot injuries, part 2 reviews midfoot injuries and part 3 covers the hindfoot.

Osseous injuries of the foot: an imaging review. Part 2: the midfoot.

Injuries to the foot are a common cause for presentation to the emergency department (ED), and imaging is often used to aid in the diagnosis. The foot can be divided into three distinct anatomic regions: the forefoot, midfoot and hindfoot. Our manuscripts comprise a three-part imaging review in which we address the use of radiography as well as advanced imaging modalities. We provide pearls to radiographic interpretation and discuss prognostic implications and classification systems. Part 1 addressed forefoot injuries, Part 2 reviews midfoot injuries and Part 3 covers the hindfoot.