Swati Choudhry

Percutaneous carotid artery access in infants < 3 months of age

Carotid artery access in infants with congenital heart disease undergoing cardiac catheterization via a surgical cut down has been well described. There is a paucity of information regarding percutaneous carotid artery (CA) access in infants <3 months.

Rapid resolution of cardiac rhabdomyomas following everolimus therapy

Cardiac rhabdomyoma is the most common primary cardiac tumour during childhood and is usually associated with tuberous sclerosis complex (TSC). These tumours are generally considered benign, and spontaneous regression occurs commonly. However, when the tumours cause significant symptoms, the current standard treatment is surgical resection. Everolimus is an mammalian target of rapamycin (mTOR) complex 1 inhibitor that has been successfully used to treat subependymal giant cell astrocytomas and renal angiomyolipomas associated with TSC. A few case reports have described the effectiveness of everolimus therapy in treating cardiac rhabdomyomas as well. We report a case of a newborn who had near complete resolution of multiple rhabdomyomas within a month of receiving everolimus therapy for non-cardiac masses. To the best of our knowledge, this is the fastest resolution of cardiac rhabdomyomas associated with everolimus therapy to date. Everolimus may be a promising alternative for high-risk surgical candidates with haemodynamically significant cardiac rhabdomyomas.

Normative Left Ventricular M-Mode Echocardiographic Values in Preterm Infants up to 2 kg

Background: There is a paucity of normative echocardiographic data in preterm infants. The objectives of this study were to (1) derive left ventricular (LV) M‐mode reference values and (2) compare the performance of alternative methods of indexing LV dimensions and LV mass (LVM) in preterm infants. The authors propose that indexing LV measures to weight in preterm infants is a practical approach given the variability associated with tape‐measure length measurement in infants. Methods: In this retrospective study, LV M‐mode echocardiographic measurements of end‐diastolic interventricular septal thickness, end‐diastolic LV posterior wall thickness, LV end‐diastolic and end‐systolic dimensions, LVM, and relative wall thickness were remeasured in 503 hospitalized preterm infants ≤2 kg (372 from a retrospective sample and 131 prospectively enrolled). Measures for all variables did not differ between retrospective and prospective samples, so results were pooled. LV dimensions and LVM indexed for weight, length, and body surface area sex‐specific centile curves and corresponding Z scores were generated using Coles lambda‐mu‐sigma method. Threshold limits (10th and 80th percentiles) were used to generate the normative range for relative wall thickness. Results: Sex‐specific centile curves using LVM, end‐diastolic interventricular septal thickness, end‐diastolic LV posterior wall thickness, LV end‐diastolic dimension, and LV end‐systolic dimension indexed to weight were similar to the curves generated using length and body surface area. The mean normal range for relative wall thickness was 0.33 (10th percentile, 0.26; 80th percentile, 0.38). Conclusions: From this large cohort of preterm infants, LV M‐mode dimension and LVM centile curves indexed to weight were developed as a practical method to assess LV morphology in preterm infants. HighlightsLV M‐mode echocardiographic measurements were performed in 503 preterm infants.Sex‐specific centile curves using LV M‐mode dimension indexed to weight are a practical method to assess LV morphology in preterm infants.Normative range for LV relative wall thickness in preterm infants provides an additional tool to classify LV remodeling.

Evidence of Echocardiographic Markers of Pulmonary Vascular Disease in Asymptomatic Infants Born Preterm at One Year of Age

Objective To test the hypothesis that echocardiographic markers of pulmonary vascular disease (PVD) exist in asymptomatic infants born preterm at 1‐year corrected age. Study design We conducted a prospective cohort study of 80 infants born preterm (<29 weeks of gestation) and 100 age‐ and weight‐matched infants born at term and compared broad‐based conventional and quantitative echocardiographic measures of pulmonary hemodynamics at 1‐year corrected age. Pulmonary artery acceleration time (PAAT), a validated index of pulmonary vascular resistance, arterial pressure, and compliance, was used to assess pulmonary hemodynamics. Lower PAAT is indicative of PVD. Subanalyses were performed in infants with bronchopulmonary dysplasia (BPD, n = 48, 59%) and/or late‐onset pulmonary hypertension (n = 12, 15%). Results At 1 year, there were no differences between conventional measures of pulmonary hypertension in the infants born at term and preterm. All infants born preterm had significantly lower values of PAAT than infants born at term (73 ± 8 milliseconds vs 98 ± 5 milliseconds, P < .001). Infants born preterm with BPD had even lower PAAT than those without BPD (69 ± 5 milliseconds vs 79 ± 4 milliseconds, P < .01). The degree of PVD at 1‐year corrected age was inversely related to gestation in all infants born preterm. Data analysis included adjustment for ventricular function and other confounding factors. Conclusions In comparison with infants born at term, infants born preterm exhibit abnormal PAAT at 1‐year corrected age irrespective of neonatal lung disease status, suggesting the existence of PVD beyond infancy. PAAT measurements offer a reliable, noninvasive tool for screening and longitudinal monitoring of pulmonary hemodynamics in infants.

Markers of maternal and infant metabolism are associated with ventricular dysfunction in infants of obese women with type 2 diabetes

BackgroundTo test the hypothesis that infants born to obese women with pre-gestational type 2 diabetes mellitus (IBDMs) have ventricular dysfunction at 1 month that is associated with markers of maternal lipid and glucose metabolism.MethodsIn a prospective observational study of IBDMs (OB+DM, n=25), echocardiographic measures of septal, left (LV) and right ventricular (RV) function, and structure were compared at 1 month of age with those in infants born to OB mothers without DM (OB, n=24) and to infants born to non-OB mothers without DM (Lean, n=23). Basal maternal lipid and glucose kinetics and maternal plasma and infant (cord) plasma were collected for hormone and cytokine analyses.ResultsRV, LV, and septal strain measures were lower in the OB+DM infants compared with those in other groups, without evidence of septal hypertrophy. Maternal hepatic insulin sensitivity, maternal plasma free-fatty-acid concentration, and cord plasma insulin and leptin most strongly predicted decreased septal strain in OB+DM infants.ConclusionIBDMs have reduced septal function at 1 month in the absence of septal hypertrophy, which is associated with altered maternal and infant lipid and glucose metabolism. These findings suggest that maternal obesity and DM may have a prolonged impact on the cardiovascular health of their offspring, despite the resolution of cardiac hypertrophy.

End-stage renal disease after pediatric heart transplantation: A 25-year national cohort study

BACKGROUND End-stage renal disease (ESRD), defined as the need for chronic dialysis and/or kidney transplantation (KTx), is a known complication after heart transplant (HTx). However, factors associated with ESRD are not well elucidated. The objectives of this study were to determine the prevalence, risk factors, and outcomes associated with ESRD after pediatric HTx. METHODS Scientific Registry of Transplant Recipients data were linked, using direct identifiers, to the United States Renal Data System to identify patients (aged ≤ 18 years) who underwent primary HTx between 1989 and 2013. Risk factors for ESRD and death were analyzed using Cox regression analysis. RESULTS Combining the above 2 databases identified ~25% additional HTx patients who developed ESRD that were not captured by either database alone. During a median follow-up of 11.8 years, ESRD developed in 276 of 6,901 patients (4%). The actuarial risk of developing ESRD after HTx was 3% at 10 years and 16% at 20 years. Age at HTx > 1 year, African-American race, year of HTx before 2000, hypertension, diabetes mellitus, re-HTx, acute dialysis, graft failure, and hospitalized infection were significant risk factors for ESRD development. Those who remained on chronic dialysis had higher risk of death than those who received KTx (hazard ratio, 31.4; 95% confidence interval, 20.8-48.4; p < 0.0001). CONCLUSIONS ESRD after pediatric HTx is more prevalent in HTx survivors than documented by a transplant database alone. A number of factors develop at or after HTx that increase the risk for developing ESRD. Use of KTx in post-HTx ESRD is associated with improved survival.

Risk factors and prognostic significance of altered left ventricular geometry in preterm infants

Objective:Left ventricular (LV) hypertrophy (LVH) predicts adverse cardiac events in adults. We sought to determine the risk factors and prognostic significance of altered LV geometry in preterm infants.Study design:In an echocardiographic, single-center, retrospective case–control study we investigated the risk factors and outcomes in patients with altered LV geometry (either increased left ventricular mass index (LVMI) or increased relative wall thickness (RWT)) from a cohort of 503 preterm infants ≤2 kg.Result:Altered LV geometry was seen in 180 patients and was predicted by postnatal steroids and small for gestational age. Hospital stay was longer in the elevated RWT cases. Altered LV geometry resolved in 129 of the 131 cases with follow-up echocardiogram. Fifteen of 94 patients with elevated RWT died compared to 3/90 controls (P = 0.004).Conclusion:Altered LV geometry in preterm infants is associated with postnatal steroid use and small for gestational age. Elevated RWT is associated with longer hospital stay and increased mortality.

Early stroke post–heart transplant is associated with decreased survival in children

BACKGROUND Major neurologic events (MNEs) after heart transplantation (HTx) and their effect on survival have not been well described in children. In this study we aimed to characterize early MNEs (stroke, isolated seizures not from stroke and posterior reversible leukoencephalopathy [PRES] within 1 year after primary pediatric HTx) and evaluate their impact on 1-year post-HTx survival. We hypothesized that early an MNE after HTx is associated with decreased 1-year patient survival. METHODS We performed a pediatric, single-center, retrospective analysis of 345 consecutive patients aged 0 to 22 years who underwent primary HTx during the period from November 1, 1994 to October 31, 2015. Characteristics were compared between patients with and without early MNEs. RESULTS Nineteen percent (65 of 345) of patients had an MNE within 1 year after HTx (median 9 days, interquartile range [IQR] 4 to 23 days). Freedom from early MNE was 97%, 85% and 80% at 1, 6 and 12 months, respectively. Of the total 65 events, stroke comprised 55.4% (n = 36), isolated seizure 29.2% (n = 19) and PRES 15.4% (n = 10). With multiple logistic regression, previous neurologic disease, infection requiring intravenous antibiotic therapy and post-operative drug-treated hypertension were found to be significant risk factors for early MNEs. Stroke (hazard ratio 4.1, IQR 2.3 to 7.6, p < 0.0001), but not seizures and PRES, was associated with decreased 1-year patient survival. CONCLUSIONS Major neurologic events are common after pediatric HTx and usually occur within the first few weeks. Early stroke was associated with decreased 1-year survival. Potentially modifiable factors, including prior neurologic event, drug-treated hypertension and infection, were associated with increased risk of developing early MNEs.

Dilated cardiomyopathy with cardiogenic shock in a child with Kearns-Sayre syndrome

Kearns-Sayre syndrome (KSS) is a mitochondrial myopathy resulting from mitochondrial DNA deletion. This syndrome primarily involves the central nervous system, eyes, skeletal muscles and the heart. The most well-known cardiac complications involve the conduction system; however, there have been case reports describing cardiomyopathy. We describe a case of a child with KSS who presented with decompensated cardiac failure from dilated cardiomyopathy representing cardiomyocyte involvement of KSS. Our patient had a rapidly progressing course, despite maximal medical management, requiring emergent institution of extracorporeal membrane oxygenation and transition to a ventricular assist device. To the best of our knowledge, this is the youngest patient in the literature to have dilated cardiomyopathy in KSS.