Impact of ventricular assist device use on pediatric heart transplant waitlist mortality: Analysis of the scientific registry of transplant recipients database.

BACKGROUND: Children awaiting heart transplant (Tx) have a high risk of death due to donor organ scarcity. Historically, ventricular assist devices (VADs) reduced waitlist mortality, prompting increased VAD use. We sought to determine whether the VAD survival benefit persists in the current era. METHODS: Using the Scientific Registry of Transplant Recipients, we identified patients listed for Tx between 3/22/2016 and 9/1/2020. We compared characteristics of VAD and non-VAD groups at Tx listing. Cox proportional hazards models were used to identify risk factors for 1-year waitlist mortality. RESULTS: Among 5054 patients, 764 (15%) had a VAD at Tx listing. The VAD group was older with more mechanical ventilation and renal impairment. Unadjusted waitlist mortality was similar between groups; the curves crossed ~90 days after listing (p = .55). In multivariable analysis, infant age (HR 2.77, 95%CI 2.13-3.60), Black race (HR 1.57, 95%CI 1.31-1.88), congenital heart disease (HR 1.23, 95%CI 1.04-1.46), renal impairment (HR 2.67, 95%CI 2.19-3.26), inotropes (HR 1.28, 95%CI 1.09-1.52), and mechanical ventilation (HR 2.23, 95%CI 1.84-2.70) were associated with 1-year waitlist mortality. VADs were not associated with mortality in the first 90 waitlist days but were protective for those waiting ≥90 days (HR 0.43, 95%CI 0.26-0.71). CONCLUSIONS: In the current era, VADs reduce waitlist mortality, but only for those waitlisted ≥90 days. The differential effect by race, size, and VAD type is less clear. These findings suggest that Tx listing without VAD may be reasonable if a short waitlist time is anticipated, but VADs may benefit those expected to wait >90 days.

New races with wider virulence indicate rapid evolution of Puccinia striiformis f. sp. tritici in the Southern Cone of America.

Wheat yellow (stripe) rust, caused by Puccinia striiformis f. sp. tritici (Pst), is one of the most devastating diseases of wheat worldwide. Pst populations are composed of multiple genetic groups, each carrying one or more races characterized by different avirulence/virulence combinations. Since the severe epidemics in 2017, yellow rust has become the most economically important wheat foliar disease in Uruguay. A set of 124 Pst isolates collected from wheat fields in Uruguay between 2017 and 2021 were characterized phenotypically and 27 of those isolates were subsequently investigated in-depth by additional molecular genotyping and race phenotyping analyses. Three genetic groups were identified, i.e., PstS7, PstS10 and PstS13, the latter being the most prevalent. Two races previously reported in Europe, Warrior (PstS7) and Benchmark (PstS10), were detected in four and two isolates, respectively. A third race known as Triticale2015 (PstS13), first detected in Europe in 2015 and in Argentina in 2017, was detected at several locations. Additional virulence to Yr3, Yr17, Yr25, Yr27 or Yr32 was detected in three new race variants within PstS13. The identification of these new races, which have not been reported outside South America, provides strong evidence of the local evolution of virulence in Pst during the recent epidemic years.

Continuous Prostanoid Initiation in Severe Pulmonary Hypertension in the Pediatric Cardiac Intensive Care Unit.

OBJECTIVE: Limited data exists regarding prostanoid (PGI2) use in critically ill patients with pulmonary hypertension. (PH) in the pediatric cardiac intensive care unit (CICU) setting. MATERIALS AND METHODS: Single center, retrospective study of patients with diagnosis of PH who received continuous PGI2 and were admitted to CICU from January/2015 to April/2022. Data collected included patient demographics and clinical characteristics including diagnosis, etiology of PH, vasoactive and ventilatory support, length of stay, and survival. Type, initial, maximum, and final dose of PGI2 as well as hemodynamic data was obtained. Data reported as mean ± standard deviation. Significance taken p value < 0.05. RESULTS: 24 patients received PGI2 therapy at a mean age of 3.1 years, range (0-16.6 years). PGI2 was in the form of IV epoprostenol in 12 patients, IV treprostinil in 6, and SQ treprostinil in 6 patients. Mean initial dose was 2.79 ng/kg/min, max dose 18.75 ng/kg/min, and mean duration of therapy was 38.5 days. At PGI2 initiation, 21 (87.5%) were on vasoactive infusions, 19 (79.2%) mechanically ventilated (MV), and 6 (25%) were on extracorporeal membrane oxygenation (ECMO). The in-hospital mortality rate was 37.5% (n = 9). Patients MV and on ECMO support had higher risk of death (p = 0.04, and < 0.01, respectively). CONCLUSION: PGI2 therapy was tolerated in approximately 50% of patients with the most common side effect being hypotension leading to discontinuation in 1/3rd of patients. Ongoing evaluation of the benefits of PGI2 for patients in the CICU setting will help better identify patient selection, type, and dosing of PGI2.

Evaluation of Photosynthetic Efficiency in Photorespiratory Mutants by Chlorophyll Fluorescence Analysis.

Photosynthesis and photorespiration represent the largest carbon fluxes in plant primary metabolism and are necessary for plant survival. Many of the enzymes and genes important for photosynthesis and photorespiration have been well studied for decades, but some aspects of these biochemical pathways and their crosstalk with several subcellular processes are not yet fully understood. Much of the work that has identified the genes and proteins important in plant metabolism has been conducted under highly controlled environments that may not best represent how photosynthesis and photorespiration function under natural and farming environments. Considering that abiotic stress results in impaired photosynthetic efficiency, the development of a high-throughput screen that can monitor both abiotic stress and its impact on photosynthesis is necessary. Therefore, we have developed a relatively fast method to screen for abiotic stress-induced changes to photosynthetic efficiency that can identify uncharacterized genes with roles in photorespiration using chlorophyll fluorescence analysis and low CO2 screening. This paper describes a method to study changes in photosynthetic efficiency in transferred DNA (T-DNA) knockout mutants in Arabidopsis thaliana. The same method can be used for screening ethyl methanesulfonate (EMS)-induced mutants or suppressor screening. Utilizing this method can identify gene candidates for further study in plant primary metabolism and abiotic stress responses. Data from this method can provide insight into gene function that may not be recognized until exposure to increased stress environments.

Vitamin D and morbidity in children with Multisystem inflammatory syndrome related to Covid-19.

Background: Multisystem inflammatory syndrome (MIS-C) is a clinical presentation reported in children related to Coronavirus-19 infection who present with a toxic shock like syndrome. Vitamin D deficiency has been postulated to play a role with severity of coronavirus infection in adult patients and other viral respiratory infections. Objective: This study aims to investigate if severe vitamin D deficiency was associated with increased disease severity and cardiac involvement in MIS-C. Methods: This is a retrospective and single center study. We included hospitalized patients less than 18 years of age with diagnosis of MIS-C between March and July 2020. Severe vitamin D deficiency was defined as 25-OH vitamin D level < 10 ng/ml within 48 h of admission. The composite outcome severe disease included patients requiring inotropes, mechanical ventilation, and extracorporeal membrane oxygenation. Results: Of the 31 patients with MIS-C, 45% were male and 58% were African American. The median age was 8 (1-13) years. Ten patients had severe vitamin D deficiency with a mean level of 7.2 ng/ml. Ninety percent of patients with severe vitamin D deficiency had severe disease (P < 0.001). Patients with severe vitamin D deficiency had an increased risk of cardiac involvement (P < 0.001). Conclusions: We describe a potential association between severe vitamin D deficiency and severe disease in children presenting with MIS-C. Severe vitamin D deficiency predisposes patients for cardiovascular involvement and may play a critical role in the host immune response to COVID-19 infection. Future prospective studies at the basic science and clinical level should be pursued to better delineate this association.

Multimodality management of sinonasal teratocarcinosarcoma in a 76-year-old Alaska Native female during the COVID-19 pandemic.

Sinonasal teratocarcinosarcoma is a rare, highly aggressive tumor of the anterior skull base composed of malignant epithelial, mesenchymal, and neural tissue. Examination of cases in patients in minority populations is important in order to better understand the behavior of this neoplasm and outcomes of treatment in our nation's diverse population.

Whole-Genome Sequencing and RNA-Seq Reveal Differences in Genetic Mechanism for Flowering Response between Weedy Rice and Cultivated Rice.

Flowering is a key agronomic trait that influences adaptation and productivity. Previous studies have indicated the genetic complexity associated with the flowering response in a photoinsensitive weedy rice accession PSRR-1 despite the presence of a photosensitive allele of a key flowering gene Hd1. In this study, we used whole-genome and RNA sequencing data from both cultivated and weedy rice to add further insights. The de novo assembly of unaligned sequences predicted 225 genes, in which 45 were specific to PSRR-1, including two genes associated with flowering. Comparison of the variants in PSRR-1 with the 3K rice genome (RG) dataset identified unique variants within the heading date QTLs. Analyses of the RNA-Seq result under both short-day (SD) and long-day (LD) conditions revealed that many differentially expressed genes (DEGs) colocalized with the flowering QTLs, and some DEGs such as Hd1, OsMADS56, Hd3a, and RFT1 had unique variants in PSRR-1. Ehd1, Hd1, OsMADS15, and OsMADS56 showed different alternate splicing (AS) events between genotypes and day length conditions. OsMADS56 was expressed in PSRR-1 but not in Cypress under both LD and SD conditions. Based on variations in both sequence and expression, the unique flowering response in PSRR-1 may be due to the high-impact variants of flowering genes, and OsMADS56 is proposed as a key regulator for its day-neutral flowering response.

Dilated cardiomyopathy associated with a mutation in the dispatched RND transporter family member 1 gene.

Dilated cardiomyopathy is the most common presentation of cardiomyopathy in children with 20-35% of patients having an identified genetic component. There are more than 30 genes implicated in the pathogenesis of dilated cardiomyopathy. We present the first report of a female infant with dilated cardiomyopathy with a genetic variant in the dispatched RND transporter family member 1 gene.

Is there an association of near-infrared spectroscopy with low cardiac output and adverse outcomes in single-ventricle patients after stage 1 palliation?

Objective: Our primary objective study was to evaluate the association between near-infrared spectroscopy (NIRS) and low cardiac output (LCO) in patients with single-ventricle physiology after stage 1 palliation. Methods: In this retrospective study, infants ≤6 months of age with single-ventricle physiology who underwent stage 1 palliation were included. Cerebral and renal NIRS values at various time intervals after surgery were compared between patients with low and normal cardiac output. LCO within the first 48 after surgery was defined as per the pediatric cardiac critical care consortium database. NIRS values were also compared with other adverse outcomes such as cardiac arrest, need for extracorporeal membrane oxygenation and mortality. The receiver operative characteristic curve was generated to determine an optimal cut-off NIRS value for detecting LCO. Results: Ninety-one patients with median (Interquartile range) age of 10 days (6-26) and weight of 3.3 kg (3-3.5) were included in the study. Cerebral NIRS at 1 h (41.2 vs. 49.5; P = 0.002), 6 h (44 vs. 52.2; P = 0.001), and 12 h (51.8 vs. 56; P = 0.025) was significantly lower in the grouP with LCO compared to no LCO. Cerebral NIRS at 6 h was independently associated with LCO (P = 0.018), and cerebral NIRS at 6 h ≤57% had 91% sensitivity and 72% specificity to detect LCO. Conclusions: Cerebral NIRS ≤57% at 6 h after surgery detected LCO after stage 1 palliation in single-ventricle patients. Cerebral or renal NIRS was not associated with adverse outcomes and therefore, may not be useful in predicting adverse outcomes in this population.

Promoting the Selectivity of Pt/m-ZrO2 Ethanol Steam Reforming Catalysts with K and Rb Dopants.

The ethanol steam reforming reaction (ESR) was investigated on unpromoted and potassium- and rubidium-promoted monoclinic zirconia-supported platinum (Pt/m-ZrO2) catalysts. Evidence from in situ diffuse reflectance infrared Fourier transform spectroscopy (DRIFTS) characterization indicates that ethanol dissociates to ethoxy species, which undergo oxidative dehydrogenation to acetate followed by acetate decomposition. The acetate decomposition pathway depends on catalyst composition. The decarboxylation pathway tends to produce higher overall hydrogen selectivity and is the most favored route at high alkali loading (2.55 wt.% K and higher or 4.25 wt.% Rb and higher). On the other hand, decarbonylation is a significant route for the undoped catalyst or when a low alkali loading (e.g., 0.85% K or 0.93% Rb) is used, thus lowering the overall H2 selectivity of the process. Results of in situ DRIFTS and the temperature-programmed reaction of ESR show that alkali doping promotes forward acetate decomposition while exposed metallic sites tend to facilitate decarbonylation. In previous work, 1.8 wt.% Na was found to hinder decarbonylation completely. Due to the fact that 1.8 wt.% Na is atomically equivalent to 3.1 wt.% K and 6.7 wt.% Rb, the results show that less K (2.55% K) or Rb (4.25% Rb) is needed to suppress decarbonylation; that is, more basic cations are more efficient promoters for improving the overall hydrogen selectivity of the ESR process.

Echocardiographic Indicators Associated with Adverse Clinical Course and Cardiac Sequelae in Multisystem Inflammatory Syndrome in Children with Coronavirus Disease 2019.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 causes significant cardiovascular involvement, which can be a determinant of clinical course and outcome. The aim of this study was to investigate whether echocardiographic measures of ventricular function were independently associated with adverse clinical course and cardiac sequelae in patients with MIS-C. METHODS: In a longitudinal observational study of 54 patients with MIS-C (mean age, 6.8 ± 4.4 years; 46% male; 56% African American), measures of ventricular function and morphometry at initial presentation, predischarge, and at a median of 3- and 10-week follow-up were retrospectively analyzed and were compared with those in 108 age- and gender-matched normal control subjects. The magnitude of strain is expressed as an absolute value. Risk stratification for adverse clinical course and outcomes were analyzed among the tertiles of clinical and echocardiographic data using analysis of variance and univariate and multivariate regression. RESULTS: Median left ventricular apical four-chamber peak longitudinal strain (LVA4LS) and left ventricular global longitudinal strain (LVGLS) at initial presentation were significantly decreased in patients with MIS-C compared with the normal cohort (16.2% and 15.1% vs 22.3% and 22.0%, respectively, P < .01). Patients in the lowest LVA4LS tertile (<13%) had significantly higher C-reactive protein and high-sensitivity troponin, need for intensive care, and need for mechanical life support as well as longer hospital length of stay compared with those in the highest tertile (>18.5%; P < .01). Initial LVA4LS and LVGLS were normal in 13 of 54 and 10 of 39 patients, respectively. There was no mortality. In multivariate regression, only LVA4LS was associated with both the need for intensive care and length of stay. At median 10-week follow-up to date, seven of 36 patients (19%) and six of 25 patients (24%) had abnormal LVA4LS and LVGLS, respectively. Initial LVA4LS < 16.2% indicated abnormal LVA4LS at follow-up with 100% sensitivity. CONCLUSION: Impaired LVGLS and LVA4LS at initial presentation independently indicate a higher risk for adverse acute clinical course and persistent subclinical left ventricular dysfunction at 10-week follow-up, suggesting that they could be applied to identify higher risk children with MIS-C.

Sustained ventricular tachycardia in a paediatric patient with acute COVID-19 myocarditis.

Although rare, children with active coronavirus disease 2019 are at risk of developing malignant arrhythmia. Herein, we present the first paediatric case of refractory ventricular tachycardia from acute fulminant myocarditis secondary to acute COVID-19 infection. This 5-year-old boy required venoarterial extracorporeal membrane oxygenation support, but made a complete recovery without significant morbidity.

Inferior Vena Cava Collapsibility Index to Assess Central Venous Pressure in Perioperative Period Following Cardiac Surgery in Children.

The inferior vena cava collapsibility index (IVCCI) is an ultrasound method used to assess central venous pressure (CVP). Our objective was to evaluate the correlation between IVCCI and CVP in children during the early period following surgery for congenital heart disease (CHD). Prospective study performed in a single tertiary care center. Patients ≤ 18 years old, who underwent cardiopulmonary bypass surgery for CHD, were enrolled. Ultrasound images of the inferior vena cava (IVC) were obtained at two time points; the first was within 2 h of arrival to the CICU and the second was 12-18 h from the first measurement. CVP measured by catheter placed during surgery was recorded within minutes of performing ultrasound. Maximum and minimum IVC diameters were measured by 2D images and M mode method. Seventy patients (47.1% males), with median age 7 months (IQR 4-47 months) and weight 6.9 kg (IQR 4.8-13.5 kg), were evaluated. The 2D IVCCI had inverse correlation with CVP in patients breathing spontaneously; r = - 0.76 (p < 0.01) and r = - 0.73 (p < 0.01), during the first and second measurements, respectively. The 2D IVCCI ≤ 0.24 had sensitivity, specificity, and negative predictive value of 94%, 79%, and 88.9% , respectively, to detect CVP ≥ 10 mmHg. No correlation was found between IVCCI and CVP during positive pressure ventilation. There is a significant inverse correlation between 2D IVCCI and CVP in spontaneously breathing children after surgery for CHD. Use of 2D IVCCI for monitoring CVP could reduce the frequency and duration of CVP catheters and their inherent complications.

Vasoactive-ventilation-renal score: a reliable prognostic index for perioperative outcomes following congenital heart surgery in adults.

INTRODUCTION: The number of adults requiring surgeries for CHD is increasing. We sought to evaluate the utility of the vasoactive-ventilation-renal (VVR) score as a predictor of prolonged length of stay in adults following CHD surgery. METHODS: This is a retrospective review of 158 adult patients who underwent CHD surgery involving cardiopulmonary bypass. VVR score was calculated upon arrival to ICU and every 6 hours for the first 48 hours post-operatively. Our primary outcome was prolonged length of stay defined as hospital length of stay greater than 75th percentile for the cohort (≥8 days). RESULTS: The study cohort had a median age of 25.6 years (18-60 years), and 83 (52.5%) were male. The groups with and without prolonged length of stay were comparable in age, gender, race, and surgical severity score. VVR score was significantly higher at all time points in the group with prolonged length of stay. The first post-operative day peak VVR score ≥13 had a sensitivity of 81% and specificity of 75% for predicting prolonged length of stay (p = 0.0001). On regression analysis, peak VVR score during the first day was independently associated with prolonged length of stay. CONCLUSIONS: Peak VVR score during the first post-operative day was a strong predictor of prolonged length of stay in adults following CHD surgery.

A Novel Mutation of the NARROW LEAF 1 Gene Adversely Affects Plant Architecture in Rice (Oryza sativa L.).

Plant architecture is critical for enhancing the adaptability and productivity of crop plants. Mutants with an altered plant architecture allow researchers to elucidate the genetic network and the underlying mechanisms. In this study, we characterized a novel nal1 rice mutant with short height, small panicle, and narrow and thick deep green leaves that was identified from a cross between a rice cultivar and a weedy rice accession. Bulked segregant analysis coupled with genome re-sequencing and cosegregation analysis revealed that the overall mutant phenotype was caused by a 1395-bp deletion spanning over the last two exons including the transcriptional end site of the nal1 gene. This deletion resulted in chimeric transcripts involving nal1 and the adjacent gene, which were validated by a reference-guided assembly of transcripts followed by PCR amplification. A comparative transcriptome analysis of the mutant and the wild-type rice revealed 263 differentially expressed genes involved in cell division, cell expansion, photosynthesis, reproduction, and gibberellin (GA) and brassinosteroids (BR) signaling pathways, suggesting the important regulatory role of nal1. Our study indicated that nal1 controls plant architecture through the regulation of genes involved in the photosynthetic apparatus, cell cycle, and GA and BR signaling pathways.

Three Cases of Pediatric Multisystem Inflammatory Syndrome Associated with COVID-19 Due to SARS-CoV-2.

BACKGROUND Coronavirus disease 2019 (COVID-19) infection commonly presents as fever, cough, and shortness of breath in adults. Children are thought to have milder respiratory symptoms and to recover more quickly. We describe a new presentation of COVID-19 infection in children consisting of multisystem inflammation with decreased left ventricular function and evidence of lung disease. CASE REPORT Three children presented with fever, conjunctivitis, dry and cracked lips, rash, and/or cervical lymphadenopathy for at least 5 days. Two of these children required mechanical ventilation, and 1 of the 2 needed extracorporeal membrane oxygenation (ECMO) to support cardiorespiratory function. All of these children had moderate to severe hyponatremia and lymphopenia, which is usually seen in COVID-19. They were treated with intravenous immunoglobulin and high-dose aspirin. All of the children recovered. CONCLUSIONS Early recognition of children with multisystem inflammation is important because they are at increased risk for deterioration. Treatment with intravenous immunoglobulin and aspirin was used because this regimen has been shown to be beneficial in vasculitis of Kawasaki disease. The development of shock due to cardiac involvement may require ECMO.

Comparative Transcriptomics of Rice Genotypes with Contrasting Responses to Nitrogen Stress Reveals Genes Influencing Nitrogen Uptake through the Regulation of Root Architecture.

The indiscriminate use of nitrogenous fertilizers continues unabated for commercial crop production, resulting in air and water pollution. The development of rice varieties with enhanced nitrogen use efficiency (NUE) will require a thorough understanding of the molecular basis of a plant's response to low nitrogen (N) availability. The global expression profiles of root tissues collected from low and high N treatments at different time points in two rice genotypes, Pokkali and Bengal, with contrasting responses to N stress and contrasting root architectures were examined. Overall, the number of differentially expressed genes (DEGs) in Pokkali (indica) was higher than in Bengal (japonica) during low N and early N recovery treatments. Most low N DEGs in both genotypes were downregulated whereas early N recovery DEGs were upregulated. Of these, 148 Pokkali-specific DEGs might contribute to Pokkali's advantage under N stress. These DEGs included transcription factors and transporters and were involved in stress responses, growth and development, regulation, and metabolism. Many DEGs are co-localized with quantitative trait loci (QTL) related to root growth and development, chlorate-resistance, and NUE. Our findings suggest that the superior growth performance of Pokkali under low N conditions could be due to the genetic differences in a diverse set of genes influencing N uptake through the regulation of root architecture.

Does obesity affect the short-term outcomes after cardiothoracic surgery in adolescents with congenital heart disease?

BACKGROUND: Obesity is a modifiable, independent risk factor for mortality and morbidity after cardiovascular surgery in adults. Our objective was to evaluate the impact of obesity on short-term outcomes in adolescents undergoing surgery for congenital heart disease (CHD). METHODS: This retrospective chart review included patients 10-18 years of age who underwent CHD surgery. Our exclusion criteria were patients with a known genetic syndrome, heart transplantation, and patients with incomplete medical records. The clinical data collected included baseline demographics and multiple perioperative variables. Charting the body mass index in the Centers for Disease Control and Prevention growth curves, the entire cohort was divided into three categories: obese (>95th percentile), overweight (85th-95th percentile), and normal weight (<85th percentile). The composite outcome included survival, arrhythmias, surgical wound infection, acute neurologic injury, and acute kidney injury. RESULTS: The study cohort (n = 149) had a mean standard deviation (SD), body mass index (BMI) of 22.6 ± 6.5 g/m2, and 65% were male. There were 27 obese (18.1%), 24 overweight (16.1%), and 98 normal weight (65.8%) patients. Twenty-seven (18%) patients had composite adverse outcomes. Overweight and obese patients had significantly higher adverse outcomes compared with normal weight patients (odds ratio (OR): 2.9; confidence interval (CI): 1-8.5, p = 0.04 and OR: 3; CI: 1-8.5, p = 0.03, respectively). In multivariate analysis, obesity was an independent predictor of adverse outcome in our cohort (p = 0.04). CONCLUSIONS: Obesity is associated with short-term adverse outcome and increased health resource utilisation in adolescents following surgery for CHD. Further studies should evaluate if intervention in the preoperative period can improve outcomes in this population.

Noonan syndrome associated with anomalous left coronary artery from the pulmonary artery in a patient with the rare RAF1 mutation: A case report and review of literature.

We present the case of a 7-week-old male infant diagnosed with anomalous left coronary artery from the pulmonary artery (ALCAPA) who underwent repair by left coronary artery reimplantation, followed by an eventful postoperative period including need for venous arterial extracorporeal membrane oxygenation and mitral valve replacement due to mitral calcification and severe insufficiency. He also required heart transplant due to severe rapidly progressive biventricular hypertrophy. The pathology examination of the explanted heart showed massive cardiomegaly. Subsequently, the infant's cardiomyopathy panel was positive for RAF1 mutation, consistent with diagnosis of a rare form of Noonan syndrome. To our knowledge, this autosomal dominant condition in association with ALCAPA has not been previously reported in the literature.

Intravascular Papillary Endothelial Hyperplasia in the Coronary Artery: An Unusual Cause of Massive Myocardial Infarction in Hypoplastic Left Heart Syndrome.

Background: Intravascular papillary endothelial hyperplasia (IPEH) is a benign vascular lesion that usually involves the head and neck or extremities. Involvement of the coronary arterial system is unreported. Case: A 1-month-old patient born with hypoplastic left heart syndrome died from a massive myocardial infarction shortly after first stage palliation with Norwood/Sano. Autopsy demonstrated a massive univentricular hemorrhagic infarction with complete occlusion of the left main coronary artery and its intramural branches by intraluminal papillary endothelial hyperplasia (IPEH). Immunostaining with CD34 and CD31 confirmed the diagnosis. The inferior and superior mesenteric artery branches also had IPEH. Conclusion: IPEH can involve the coronary vasculature, can be multifocal and can occur in the newborn.