Chemical pancreatectomy in non-human primates ablates the acini and ducts and enhances beta-cell function.

Chronic pancreatitis is a debilitating disease affecting millions worldwide. These patients suffer from bouts of severe pain that are minimally relieved by pain medications and may necessitate major surgeries with high morbidity and mortality. Previously, we demonstrated that "chemical pancreatectomy," a pancreatic intraductal infusion of dilute acetic acid solution, ablated the exocrine pancreas while preserving the endocrine pancreas. Notably, chemical pancreatectomy resolved chronic inflammation, alleviated allodynia in the cerulein pancreatitis model, and improved glucose homeostasis. Herein, we extensively tested the feasibility of a chemical pancreatectomy in NHPs and validated our previously published pilot study. We did serial computed tomography (CT) scans of the abdomen and pelvis, analyzed dorsal root ganglia, measured serum enzymes, and performed histological and ultrastructural assessments and pancreatic endocrine function assays. Based on serial CT scans, chemical pancreatectomy led to the loss of pancreatic volume. Immunohistochemistry and transmission electron microscopy demonstrated exocrine pancreatic ablation with endocrine islet preservation. Importantly, chemical pancreatectomy did not increase pro-nociceptive markers in harvested dorsal root ganglia. Also, chemical pancreatectomy improved insulin secretion to supranormal levels in vivo and in vitro. Thus, this study may provide a foundation for translating this procedure to patients with chronic pancreatitis or other conditions requiring a pancreatectomy.

Pulmonary Nodules in Juvenile Systemic Sclerosis: A Case-Series from the National Registry for Childhood Onset Scleroderma (NRCOS).

BACKGROUND: Juvenile systemic sclerosis (jSSc) is a systemic inflammatory and fibrotic autoimmune disease. Adult guidelines recommend obtaining a screening high-resolution computed tomography scan (CT) at diagnosis. As these recommendations are adopted as standard of care for jSSc, increased screening with CT may lead to increased detection of nodules. The implications of nodules identified in jSSc are unclear and unreported. METHODS: A retrospective chart review was performed on the prospectively enrolled National Registry for Childhood-Onset Scleroderma (NRCOS) cohort over an enrollment period of 20 years. Clinical associations with presence of nodules and nodule characteristics were investigated. RESULTS: In this jSSc cohort, the prevalence of pulmonary nodules was 31% (n = 17 of 54). Nodule characteristics were heterogeneous, and most displayed stability over time. More participants with nodules had structural esophageal abnormalities, restriction, and reduced diffusing capacity on lung function tests, and follow-up imaging. Most participants had multiple nodules, and although most nodules were <5 mm, most participants had at least one nodule >5 mm. CONCLUSIONS: Pulmonary nodules are seen in children with jSSc and may be related to more severe disease and/or esophageal dysfunction. More work is needed to provide guidance on radiologic follow-up and clinical management of pulmonary nodules in jSSc.

Surrogates of Muscle Mass on Cardiac MRI Correlate with Exercise Capacity in Patients with Fontan Circulation.

BACKGROUND: Sarcopenia is an increasingly recognized marker of frailty in cardiac patients. Patients with a history of congenital heart disease and Fontan procedure have a higher risk of developing progressive muscle wasting. Our objective was to determine if we could use routine cardiac MRI (CMR) for the surveillance of muscle wasting. METHODS: A retrospective study of all Fontan patients (n = 75) was conducted at our institution, with CMR performed from 2010 to 2022 and exercise stress testing performed within 12 months (4.3 ± 4.2 months). The skeletal muscle area (SMA) for the posterior paraspinal and anterior thoracic muscles were traced and indexed for body surface area (BSA). Patients were stratified by percentile into the upper and lower quartiles, and the two groups were compared. Multivariable regression was performed to control for sex and age. RESULTS: There was a significant positive association of both anterior (r = 0.34, p = 0.039) and paraspinal (r = 0.43, p = 0.007) SMA to peak VO2. Similarly, paraspinal but not anterior SMA was negatively associated with the VE/VCO2 (r = -0.45, p = 0.006). The upper quartile group had significantly more males (18/19 vs. 8/20; p = 0.0003) and demonstrated a significantly higher peak VO2 (32.2 ± 8.5 vs. 23.8 ± 4.7, p = 0.009), a higher peak RER (1.2 ± 0.1 vs. 1.1 ± 0.04, p = 0.007), and a significantly lower VE/VCO2 (32.9 ± 3.6 vs. 40.2 ± 6.2, p = 0.006) compared to the lowest quartile. The association of SMA to VO2 peak and VE/VCO2 was redemonstrated after controlling for sex and age. CONCLUSION: Thoracic skeletal muscle area may be an effective surrogate of muscle mass and is correlated to several measures of cardiorespiratory fitness post-Fontan. CMR would be an effective tool for the surveillance of sarcopenia in post-Fontan patients given its accessibility and routine use in these patients.

Chemical pancreatectomy in non-human primates ablates the acini and ducts and enhances beta-cell function.

Chronic pancreatitis is a debilitating disease affecting millions worldwide. These patients suffer from bouts of severe pain that are minimally relieved by pain medications and may necessitate major surgeries with high morbidity and mortality. Previously, we demonstrated that "chemical pancreatectomy," a pancreatic intraductal infusion of dilute acetic acid solution, ablated the exocrine pancreas while preserving the endocrine pancreas. Notably, chemical pancreatectomy resolved chronic inflammation, alleviated allodynia in the cerulein pancreatitis model, and improved glucose homeostasis. Herein, we extensively tested the feasibility of a chemical pancreatectomy in NHPs and validated our previously published pilot study. We did serial computed tomography (CT) scans of the abdomen and pelvis, analyzed dorsal root ganglia, measured serum enzymes, and performed histological and ultrastructural assessments and pancreatic endocrine function assays.  Based on serial CT scans, chemical pancreatectomy led to the loss of pancreatic volume. Immunohistochemistry and transmission electron microscopy demonstrated exocrine pancreatic ablation with endocrine islet preservation. Importantly, chemical pancreatectomy did not increase pro-nociceptive markers in harvested dorsal root ganglia. Also, chemical pancreatectomy improved insulin secretion to supranormal levels in vivo and in vitro. Thus, this study may provide a foundation for translating this procedure to patients with chronic pancreatitis or other conditions requiring a pancreatectomy.

Pediatric mediastinal masses.

Mediastinal masses are categorized based on the International Thymic Malignancy Interest Group (ITMIG) classification into prevascular, visceral and paravertebral compartments. The schema is based on cross-sectional imaging, mainly CT, and helps with generating a differential diagnosis based on location of the mass. Up to half of all pediatric mediastinal tumors are malignant. In this review we describe mediastinal masses that are relevant to the pediatric population, as well as the role of MR imaging of mediastinal masses and its advantages.

Long-term metformin adherence in the Diabetes Prevention Program Outcomes Study.

INTRODUCTION: To investigate long-term metformin adherence in the Diabetes Prevention Program Outcomes Study (DPPOS) by examining: (1) predictors of long-term adherence to study metformin and (2) whether metformin adherence was associated with incident type 2 diabetes. RESEARCH DESIGN AND METHODS: DPPOS was an open-label continuation of the randomized clinical trial (Diabetes Prevention Program (DPP)) in which eligible participants randomized to the metformin group were offered study metformin and followed over 11 years. A brief structured adherence interview was administered semiannually. Metformin adherence was assessed by pill counts. Predictors of metformin adherence were examined in multivariate regression models. Incident diabetes associated with metformin adherence and other variables was assessed in Cox proportional hazards models. RESULTS: Of 868 participants eligible to continue taking study metformin, 664 (76%) took at least some metformin over 11 years, with 478 of them reporting problems with adherence. DPPOS cumulative adherence showed significant associations of higher adherence (≥80%) with early adherence at 3 months in DPP (p<0.001) and lower depression scores during DPPOS (p<0.001); significant differences were also seen by race/ethnicity (p<0.004). Predicting adherence by multivariate modeling showed odds of adherence significantly lower for Black participants and for participants reporting more than one barrier. Odds for adherence were significantly higher for those adherent early in DPP and those reporting at least one planned strategy to improve adherence. Higher metformin adherence was significantly associated with a lower diabetes risk (p=0.04), even after adjustment for demographic variables, depression, and anxiety scores. CONCLUSIONS: In this long-term diabetes prevention study, early metformin adherence and planned strategies to promote adherence improved long-term adherence over 11 years; higher adherence to metformin was related to lower diabetes incidence. Incorporating strategies to promote adherence when initially prescribing metformin and counseling to support adherence over time are warranted.

Liver Transplantation for Pediatric Liver Cancer.

Unresectable hepatocellular carcinoma (HCC) was first removed successfully with total hepatectomy and liver transplantation (LT) in a child over five decades ago. Since then, children with unresectable liver cancer have benefitted greatly from LT and a confluence of several equally important endeavors. Regional and trans-continental collaborations have accelerated the development and standardization of chemotherapy regimens, which provide disease control to enable LT, and also serve as a test of unresectability. In the process, tumor histology, imaging protocols, and tumor staging have also matured to better assess response and LT candidacy. Significant trends include a steady increase in the incidence of and use of LT for hepatoblastoma, and a significant improvement in survival after LT for HCC with each decade. Although LT is curative for most unresectable primary liver sarcomas, such as embryonal sarcoma, the malignant rhabdoid tumor appears relapse-prone despite chemotherapy and LT. Pediatric liver tumors remain rare, and diagnostic uncertainty in some settings can potentially delay treatment or lead to the selection of less effective chemotherapy. We review the current knowledge relevant to diagnosis, LT candidacy, and post-transplant outcomes for these tumors, emphasizing recent observations made from large registries or larger series.

Pediatric ovarian volumes measured at ultrasound after contralateral unilateral oophorectomy.

BACKGROUND: Changes that occur in the remaining ovary after contralateral oophorectomy are not well described. OBJECTIVE: To determine average ovarian volume in pediatric patients after contralateral oophorectomy compared to age-matched controls with two normal ovaries. MATERIALS AND METHODS: We performed a retrospective review of ultrasound examinations and electronic medical records of patients ages 0-18 years who had unilateral oophorectomy from 2000 to 2017 (n=64). We used 384 consecutive normal age-matched ovaries for comparison, analyzing mean ovarian volumes. RESULTS: Higher mean ovarian volume (mL) was observed in patients who had oophorectomy compared to controls in the first decade of life (P<0.003) and second decade of life (P<0.0003). Higher mean ovarian volume was seen in both premenarchal and menstruating patients with prior oophorectomy when compared to controls (P<0.05 and P<0.0001, respectively). When comparing volume during menstrual cycle, we saw higher mean ovarian volumes in the oophorectomy group compared to the control group for the follicular (P<0.0001), pre-ovulatory (P=0.0005) and luteal phases (P<0.0003). We provide an updated reference of normal ovarian volumes for pediatric patients, with values similar to those already reported in the literature. CONCLUSION: Ovarian volume is higher in pediatric patients with one normal ovary following contralateral oophorectomy. The provided normative volumes can be used in evaluating these patients.

Pediatric liver transplantation for hepatocellular cancer and rare liver malignancies: US multicenter and single-center experience (1981-2015).

A tenth of all pediatric liver transplantations (LTs) are performed for unresectable liver malignancies, especially the more common hepatoblastoma (HBL). Less understood are outcomes after LT for the rare hepatocellular carcinoma, nonhepatoblastoma embryonal tumors (EMBs), and slow growing metastatic neuroendocrine tumors of childhood. Pediatric LT is increasingly performed for rare unresectable liver malignancies other than HBL. We performed a retrospective review of outcomes after LT for malignancy in the multicenter US Scientific Registry of Transplant Recipients (SRTR; n = 677; 1987-2015). We then reviewed the Children's Hospital of Pittsburgh (CHP; n = 74; 1981-2014) experience focusing on LT for unresectable hepatocellular cancer (HCC), EMBs, and metastatic liver tumors (METS). HBL was included to provide reference statistics. In the SRTR database, LT for HCC and HBL increased over time (P < 0.001). Compared with other malignancies, the 149 HCC cases received fewer segmental grafts (P < 0.001) and also experienced 10-year patient survival similar to 15,710 adult HCC LT recipients (51.6% versus 49.6%; P = 0.848, not significant [NS], log-rank test). For 22 of 149 cases with incidental HCC, 10-year patient survival was higher than 127 primary HCC cases (85% [95% confidence interval (CI), 70.6%-100%] versus 48.3% [95% CI, 38%-61%]; P = 0.168, NS) and similar to 3392 biliary atresia cases (89.9%; 95% CI, 88.7%-91%). Actuarial 10-year patient survival for 17 EMBs, 10 METS, and 6 leiomyosarcoma patients exceeded 60%. These survival outcomes were similar to those seen for HBL. At CHP, posttransplant recurrence-free and overall survival among 25 HCC, 17 (68%) of whom had preexisting liver disease, was 16/25 or 64%, and 9/25 or 36%, respectively. All 10 patients with incidental HCC and tumor-node-metastasis stage I and II HCC survived recurrence-free. Only vascular invasion predicted poor survival in multivariate analysis (P < 0.0001). A total of 4 of 5 EMB patients (80%) and all patients with METS (neuroendocrine-2, pseudopapillary pancreatic-1) also survived recurrence-free. Among children, LT can be curative for unresectable HCC confined to the liver and without vascular invasion, incidental HCC, embryonal tumors, and metastatic neuroendocrine tumors. Liver Transplantation 23 1577-1588 2017 AASLD.

Canal of Nuck hernia: a multimodality imaging review.

Canal of Nuck abnormalities are a rare but important cause of morbidity in girls, most often those younger than 5 years of age. The canal of Nuck, which is the female equivalent of the male processus vaginalis, is a protrusion of parietal peritoneum that extends through the inguinal canal and terminates in the labia majora. The canal typically obliterates early in life, but in some cases the canal can partially or completely fail to close, potentially resulting in a hydrocele or hernia of pelvic contents. Recognition of this entity is especially important in cases of ovarian hernia due to the risk of incarceration and torsion. We aim to increase awareness of this condition by reviewing the embryology, anatomy and diagnosis of canal of Nuck disorders with imaging findings on US, CT and MRI using several cases from a single institution.

Pediatric neuroendocrine carcinoid tumors: Management, pathology, and imaging findings in a pediatric referral center.

BACKGROUND: While neuroendocrine (carcinoid) tumors are increasingly recognized in the adult population, they are often not suspected in children. PROCEDURE: Retrospective review of all well-differentiated neuroendocrine (carcinoid) tumors was performed based on pathology reports from a quaternary pediatric medical center between January 2003 and June 2016. Clinical presentations, treatment approaches, imaging findings, and outcomes were reviewed and analyzed. RESULTS: A total of 45 cases of pathology-proven carcinoid tumor were reported with an average age of 14.1 years (range: 7-21 years, SD: 2.8 years). Of these cases, 80% (36) were appendiceal, 11% (5) bronchial, 2% (1) colonic, 2% (1) gastric, 2% (1) enteric, and 2% (1) testicular. Metastases were observed in one (3%) appendiceal, one (100%) enteric, and two (40%) bronchial cases. No recurrence was demonstrated in any appendiceal carcinoid cases. Recurrence was seen in one of three extra-appendiceal gastrointestinal tumors. Tumor site and size significantly correlated with metastases and recurrence. CONCLUSIONS: Contrary to recent epidemiological investigations in adults, appendiceal carcinoid tumors remain the most common site for pediatric carcinoid tumors. Appendiceal carcinoid tumors exhibited benign clinical courses without recurrence during short-term follow-up. Extra-appendiceal gastrointestinal carcinoid tumors exhibited much more aggressive behavior with greater metastases and recurrence. Bronchial carcinoid tumors demonstrated good clinical response to resection even in cases with mediastinal lymph node involvement. While increased use of urine 5-HIAA levels and somatostatin receptor-specific imaging might improve detection and guide management of extra-appendiceal carcinoid tumors, longer-term follow-up is needed.

Pediatric Neuroendocrine Carcinoid Tumors: Review of Diagnostic Imaging Findings and Recent Advances.

OBJECTIVE: Although rare, pediatric neuroendocrine tumors including carcinoid tumor have increased in incidence over the past few decades. Despite increased recognition, survival rates have not significantly improved. Earlier detection of these tumors is necessary to improve clinical outcomes. This article discusses imaging approaches for detecting pediatric carcinoid tumors including anatomic imaging methods such as CT and MRI as well as functional imaging methods targeting the somatostatin receptor including 111In-labeled octreotide and newer 68Ga-based radiotracers that may hold promise in hybrid PET/CT or PET/MRI. CONCLUSION: Improvements in functional imaging with novel somatostatin receptor-specific radiotracers along with fused functional and anatomic imaging have substantially improved the clinical detection of carcinoid tumors. Although rare, these tumors are encountered in children, and an awareness of the appropriate use of various imaging methods is essential for pediatric specialists. Further research is needed to ascertain the diagnostic value of newer imaging methods and radiotracer-based treatment approaches, especially in the pediatric population.

Salivary cortisol protocol adherence and reliability by socio-demographic features: the Multi-Ethnic Study of Atherosclerosis.

Collection of salivary cortisol has become increasingly popular in large population-based studies. However, the impact of protocol compliance on day-to-day reliabilities of measures, and the extent to which reliabilities differ systematically according to socio-demographic characteristics, has not been well characterized in large-scale population-based studies to date. Using data on 935 men and women from the Multi-Ethnic Study of Atherosclerosis, we investigated whether sampling protocol compliance differs systematically according to socio-demographic factors and whether compliance was associated with cortisol estimates, as well as whether associations of cortisol with both compliance and socio-demographic characteristics were robust to adjustments for one another. We further assessed the day-to-day reliability for cortisol features and the extent to which reliabilities vary according to socio-demographic factors and sampling protocol compliance. Overall, we found higher compliance among persons with higher levels of income and education. Lower compliance was significantly associated with a less pronounced cortisol awakening response (CAR) but was not associated with any other cortisol features, and adjustment for compliance did not affect associations of socio-demographic characteristics with cortisol. Reliability was higher for area under the curve (AUC) and wake up values than for other features, but generally did not vary according to socio-demographic characteristics, with few exceptions. Our findings regarding intra-class correlation coefficients (ICCs) support prior research indicating that multiple day collection is preferable to single day collection, particularly for CAR and slopes, more so than wakeup and AUC. There were few differences in reliability by socio-demographic characteristics. Thus, it is unlikely that group-specific sampling protocols are warranted.

A non-invasive method of quantifying pancreatic volume in mice using micro-MRI.

In experimental models of pancreatic growth and recovery, changes in pancreatic size are assessed by euthanizing a large cohort of animals at varying time points and measuring organ mass. However, to ascertain this information in clinical practice, patients with pancreatic disorders routinely undergo non-invasive cross-sectional imaging of the pancreas using magnetic resonance imaging (MRI) or computed tomography (CT). The aim of the current study was to develop a thin-sliced, optimized sequence protocol using a high field MRI to accurately calculate pancreatic volumes in the most common experimental animal, the mouse. Using a 7 Telsa Bruker micro-MRI system, we performed abdominal imaging in whole-fixed mice in three standard planes: axial, sagittal, and coronal. The contour of the pancreas was traced using Vitrea software and then transformed into a 3-dimensional (3D) reconstruction, from which volumetric measurements were calculated. Images were optimized using heart perfusion-fixation, T1 sequence analysis, and 0.2 to 0.4 mm thick slices. As proof of principle, increases in pancreatic volume among mice of different ages correlated tightly with increasing body weight. In summary, this is the first study to measure pancreatic volumes in mice, using a high field 7 Tesla micro-MRI and a thin-sliced, optimized sequence protocol. We anticipate that micro-MRI will improve the ability to non-invasively quantify changes in pancreatic size and will dramatically reduce the number of animals required to serially assess pancreatic growth and recovery.

Associations of socioeconomic and psychosocial factors with urinary measures of cortisol and catecholamines in the Multi-Ethnic Study of Atherosclerosis (MESA).

BACKGROUND: Stress hormones have been hypothesized to contribute to the social patterning of cardiovascular disease but evidence of differences in hormone levels across social groups is scant. PURPOSE: To examine the associations of socioeconomic and psychosocial factors with urinary levels of cortisol and catecholamines and determine whether these associations are modified by race/ethnicity. METHODS: Measures of cortisol, epinephrine, norepinephrine and dopamine were obtained on 12-h overnight urine specimens from 942 White, African American and Hispanic participants in the Multi-Ethnic Study of Atherosclerosis (MESA). Linear regression was used to examine associations of income-wealth index, education, depression, anger, anxiety and chronic stress with the four hormones after adjustment for covariates. RESULTS: Higher income-wealth index was associated with lower levels of urinary cortisol, epinephrine, norepinephrine and dopamine, after adjustment for age, sex, race/ethnicity, medication use, body mass index, smoking, and alcohol use. Education and psychosocial factors were not associated with urinary stress hormone levels in the full sample. However, there was some evidence of effect modification by race: SES factors were more strongly inversely associated with cortisol in African Americans than in other groups and anger was inversely associated with catecholamines in African Americans but not in the other groups. CONCLUSIONS: Lower SES as measured by income-wealth index in a multi-ethnic sample is associated with higher levels of urinary cortisol and catecholamines. Heterogeneity in these associations by race/ethnicity warrants further exploration.

Single coronary artery anomaly: classification and evaluation using multidetector computed tomography and magnetic resonance angiography.

The aim of this study was to use multidetector computed tomography (MDCT) and magnetic resonance (MRA) angiography to illustrate the classification and clinical characteristics of single coronary artery anomaly (SCAA). Retrospective evaluation of 22 adult and pediatric patients with SCAA by way of a medical archiving system was performed between June 2001 and August 2012. Imaging modalities used for coronary artery evaluation included MRA and MDCT angiography. Of the 22 patients, the majority (n = 8; 36%) showed an interarterial course, the subtype having the worst prognosis. The retroaortic course (n = 3; 14%) and course anterior to the pulmonary trunk (n = 3; 14%) were the next most frequent patterns. Additional types (n = 8; 36%) included the following: L-I, R-III, septal, and combined. Four patients (18%) showed atherosclerotic involvement. SCAA anomaly was diagnosed as an incidental finding in the majority of patients evaluated initially for cardiovascular diseases (n = 19; 86%). Two patients (9%) required surgical interventions solely for their anomaly. Nine patients (41%) were found to have coexisting congenital heart disease. Although conventional catheter angiography is responsible for the current classification of SCAA, advanced imaging modalities are useful in the evaluation of morphological and clinical characteristics of single coronary arteries.

Right- and left-ventricular strain evaluation in repaired pediatric Tetralogy of Fallot patients using magnetic resonance tagging.

Residual pulmonary insufficiency in post-repair Tetralogy of Fallot (rToF) patients often mediates biventricular dysfunction which is associated with long-term adverse clinical outcomes. The objective of this study was to demonstrate the presence of impaired left ventricle (LV) circumferential strain (CS) in pediatric rToF patients as compared to controls using cardiac magnetic resonance imaging (CMRI). Additionally, bivariate analysis between right ventricle (RV) and LV functional measures in rToF patients was performed to further characterize the interventricular interactions thought to mediate LV dysfunction secondary to RV volume overload. The medical records of 12 rToF patients (mean age 13.3 years) and 9 controls (mean age 10.9 years) were analyzed. LV global CS was significantly decreased in rToF patients versus controls (p = 0.04). This impairment was differentially distributed within the LV, with only the LV anterior and anterior lateral walls significantly decreased versus controls (p = 0.04, p = 0.03). Bivariate analysis revealed a significant correlation between RV mean CS and LV EF (r = 0.71, p = 0.01), RV infundibulum CS and LV EF (r = 0.70, p = 0.01), RV infundibulum CS and LV anterolateral wall CS (r = 0.59, p = 0.04), and RV infundibulum CS and pulmonary regurgitation fraction (r = -0.63, p = 0.03). These findings support existing research implicating interventricular interactions in the development of LV dysfunction. Furthermore, the segment specific CS impairment in the LV suggests a possible spatial component to these interactions. The success of this study in identifying regional myocardial strain impairment indicates CMRI based techniques may be useful in localizing otherwise undetectable myocardial dysfunction.

Isolated intramuscular fibrous band masquerading antral web in a premature neonate. A case report.

Isolated fibrous gastric antral band in a neonate causing partial gastric obstruction and mimicking prepyloric antral web is not reported in the English literature, and we would like to report the first case. A premature neonate with feeding difficulties and bilious aspirate underwent upper gastrointestinal contrast examination. The upper gastrointestinal series showed a thin, radiolucent, circumferential band in the antrum with delayed gastric emptying. Radiographic diagnosis of prepyloric antral web with partial gastric obstruction was made. Intraoperatively, a circumferential fibrous cord was found in the muscle layer (tunica muscularis) of the antrum, which was completely divided. Clinically and radiographically, the 2 conditions could not be differentiated but require 2 separate surgical techniques for their management.

Noncompaction cardiomyopathy in children with congenital heart disease: evaluation using cardiovascular magnetic resonance imaging.

Noncompaction of the left ventricle, a genetic cardiomyopathy with a reported incidence of 0.05% to 0.24%, can lead to sudden cardiac death, particularly among children, if left undetected. Because the diagnosis of isolated noncompaction cardiomyopathy (NCM) can be overlooked, its association with other congenital heart diseases (CHDs) makes the diagnosis of NCM even more difficult. This study aimed to assess the impact of NCM on the cardiovascular physiology of children with coexisting CHDs evaluated by cardiovascular magnetic resonance imaging. A case-control study was performed with 12 children (6 patients with combined NCM and CHD and 6 control subjects with isolated CHD). The mean left ventricular end-diastolic and end-systolic volume indices were significantly higher in the CHD patients presenting with NCM than in the CHD patients with no NCM (P = 0.028). However, no differences were observed for right ventricular end-diastolic and end-systolic volume indices, biventricular ejection fractions, stroke volumes and indices, left ventricular wall thickness, left ventricular fractional shortening, cardiac output, or cardiac index. This study suggests that NCM in children with CHDs increases left ventricular volumes, and larger studies are required to demonstrate other changes (e.g., ejection fraction, stroke volume) that were close to being significant.