Incidence and risk factors for pulmonary hemorrhage after percutaneous CT-guided pulmonary nodule biopsy: an observational study.

To evaluate the current incidence of pulmonary hemorrhage and the potential factors contributing to its increased risk after percutaneous CT-guided pulmonary nodule biopsy and to summarize the technical recommendations for its treatment. In this observational study, patient data were collected from ten medical centers from April 2021 to April 2022. The incidence of pulmonary hemorrhage was as follows: grade 0, 36.1% (214/593); grade 1, 36.8% (218/593); grade 2, 18.9% (112/593); grade 3, 3.5% (21/593); and grade 4, 4.7% (28/593). High-grade hemorrhage (HGH) occurred in 27.2% (161/593) of the patients. The use of preoperative breathing exercises (PBE, p =0.000), semiautomatic cutting needles (SCN, p = 0.004), immediate contrast enhancement (ICE, p =0.021), and the coaxial technique (CoT, p = 0.000) were found to be protective factors for HGH. A greater length of puncture (p =0.021), the presence of hilar nodules (p = 0.001), the presence of intermediate nodules (p = 0.026), a main pulmonary artery diameter (mPAD) larger than 29 mm (p = 0.015), and a small nodule size (p = 0.014) were risk factors for high-grade hemorrhage. The area under the curve (AUC) was 0.783. These findings contribute to a deeper understanding of the risks associated with percutaneous CT-guided pulmonary nodule biopsy and provide valuable insights for developing strategies to minimize pulmonary hemorrhage.

Effects of an intradialytic cardiovascular rehabilitation program on functional capacity and cardiac function: ICRP trial

Abstract Introduction: Chronic kidney disease (CKD) is a worldwide public health problem associated with an increased risk of death from cardiovascular complications. Although previous studies have described significant improvements in exercise in functional capacity and quality of life in patients with end-stage kidney disease (ESKD), there is a lack of studies that propose to assess its impact on cardiac function using transthoracic echocardiogram (ECHO). In addition, most of the intradialytic exercise protocols are inconsistent, and incomplete regarding their intensity prescription, time of intervention, and monitoring. Methods: The present study aims to evaluate the effects of an intradialytic cardiovascular rehabilitation protocol (ICRP) using medium intensity aerobic exercises, for 30 min. on cardiac function and functional capacity. In this 6-month longitudinal study, heart rate (HR), systolic (SBP) and diastolic (DBP) blood pressure, peripheral oxygen saturation (SpO2) and modified Borg scale will be analyzed in all HD sessions. The cardiac function will be evaluated by left ventricular ejection fraction (LVEF) through ECHO; functional capacity by the six-minute walk test (6MWT); quality of life through the SF-36 questionnaire and routine laboratory tests and KT/Vsp calculation before and after the ICRP. Conclusion: ICRP protocol will be examined and is expected to improve cardiac function, functional capacity, and quality of life in ESKD patients on hemodialysis.

Complementary Role of Oxytocin and Vasopressin in Cardiovascular Regulation.

The neurons secreting oxytocin (OXY) and vasopressin (AVP) are located mainly in the supraoptic, paraventricular, and suprachiasmatic nucleus of the brain. Oxytocinergic and vasopressinergic projections reach several regions of the brain and the spinal cord. Both peptides are released from axons, soma, and dendrites and modulate the excitability of other neuroregulatory pathways. The synthesis and action of OXY and AVP in the peripheral organs (eye, heart, gastrointestinal system) is being investigated. The secretion of OXY and AVP is influenced by changes in body fluid osmolality, blood volume, blood pressure, hypoxia, and stress. Vasopressin interacts with three subtypes of receptors: V1aR, V1bR, and V2R whereas oxytocin activates its own OXTR and V1aR receptors. AVP and OXY receptors are present in several regions of the brain (cortex, hypothalamus, pons, medulla, and cerebellum) and in the peripheral organs (heart, lungs, carotid bodies, kidneys, adrenal glands, pancreas, gastrointestinal tract, ovaries, uterus, thymus). Hypertension, myocardial infarction, and coexisting factors, such as pain and stress, have a significant impact on the secretion of oxytocin and vasopressin and on the expression of their receptors. The inappropriate regulation of oxytocin and vasopressin secretion during ischemia, hypoxia/hypercapnia, inflammation, pain, and stress may play a significant role in the pathogenesis of cardiovascular diseases.

Selective serotonin reuptake inhibitors and the risk of congenital anomalies: a systematic review of current meta-analyses.

Objective: A review of current meta-analyses examining the relationship between maternal use of selective serotonin reuptake inhibitors (SSRIs) during pregnancy and congenital anomalies. Methods: PubMed was searched for meta-analyses published in English language between January 2010 and April 2020 by using the following combinations of key words: meta-analysis, pregnancy, antidepressant, SSRI, citalopram, escitalopram, fuloxetine, paroxetine, sertraline, fluvoxamine, neonatal outcome, birth outcome, congenital malformation, congenital anomaly, birth defect, cardiac malformation and heart defect. Results: A total of 15 meta-analyses met the search criteria. These meta-analyses consistently suggested a significant positive association between the use of SSRIs in general and paroxetine and fluoxetine in particular and the risk of major congenital anomalies. The data also showed a consistency in increased cardiovascular defects in infants due to maternal use of paroxetine. The risk of cardiovascular defects in infants of women using SSRIs in general and fluoxetine and sertraline in particular was controversial. Conclusion: Further large-scale prospective observational studies and meta-analyses on the effects of individual SSRIs other than paroxetine, especially escitalopram and fluvoxamine, are required to reach definitive conclusions.

Health Care Supervision for Children With Williams Syndrome.

This set of recommendations is designed to assist the pediatrician in caring for children with Williams syndrome (WS) who were diagnosed by using clinical features and with chromosome 7 microdeletion confirmed by fluorescence in situ hybridization, chromosome microarray, or multiplex ligation-dependent probe amplification. The recommendations in this report reflect review of the current literature, including previously peer-reviewed and published management suggestions for WS, as well as the consensus of physicians and psychologists with expertise in the care of individuals with WS. These general recommendations for the syndrome do not replace individualized medical assessment and treatment.

Circulatory emergencies in the delivery room.

The transition from fetal to neonatal life is a dramatic and complex process involving extensive physiologic changes, which are most obvious at the time of birth. Individuals who care for newly born infants must monitor the progress of the transition and be prepared to intervene when necessary. In the majority of births, this transition occurs without a requirement for any significant assistance. If newborns require assistance, the majority of the time respiratory support is all that is required. In some instances, however, there are circulatory emergencies that need to be rapidly identified or there may be dire consequences including death in the delivery room. This chapter will review various pathologies that are circulatory emergencies, and discuss how to assess them. We will also review new technologies which may help providers better understand the circulatory status or hemodynamic changes in the delivery room including heart rate, cardiac output, cerebral oxygenation and echocardiography.

The Influence of Occupational Noise Exposure on Cardiovascular and Hearing Conditions among Industrial Workers.

This study was conducted to estimate the current prevalence of hypertension, cardiovascular condition and hearing difficulty of workers exposure to occupational noise, and to analyze any associations between these abnormal signs and occupational noise exposure. The subjects included 5205 noise-exposed workers. Workers with high noise exposure were more likely to have a higher threshold value than low exposure ones (P < 0.05). Subjects in the high exposure group had a significantly higher risk of hypertension and hearing loss than the ones in low exposure group. Between the ages of 30 and 45, high-level occupational noise exposure led to a significantly raising risk of both hypertension (Adjusted OR = 1.59, 95% CI, 1.19-2.11) and hearing loss (Adjusted OR = 1.28, 95% CI, 1.03-1.60) when comparing to low-level noise exposure. In male workers, the prevalence of hearing difficulty in high exposure group was approximately 1.2 times worse than in low group (P = 0.006). In addition, exposure to high noise level demonstrated a significant association with hypertension and hearing loss when the duration time to occupational noise was longer than 10 years. Hypertension and hearing difficulty is more prevalent in the noise-exposed group (higher than 85 dB[A]). Steps to reduce workplace noise levels and to improve workplace-based health are thus urgently needed.

Pregnancy outcome following opioid exposure: A cohort study.

INTRODUCTION: Opioids constitute a cornerstone of pain relief treatment. However, opioid safety during pregnancy has not been well established. Recent studies reported an association between in utero opioid exposure and spina bifida. METHODS: In order to further evaluate the association of opioids exposure during pregnancy with adverse pregnancy outcomes, we conducted a large historical cohort by linking four databases: medications dispensations, births, pregnancy terminations for medical reasons and infant hospitalizations during the years of 1999-2009. Confounders that were controlled for included maternal age, ethnicity, maternal diabetes, smoking status, parity, obesity, year and folic acid intake. A secondary analysis for total major malformations and for spina bifida was performed using propensity score matching for first trimester exposure. RESULTS: Of the 101,586 women included in the study, 3003 were dispensed opioids during the first trimester. Intrauterine exposure to opioids was not associated with overall major malformations (adjusted odds ratio (aOR) 0.97, 95% CI 0.83-1.13), cardiovascular malformations (aOR = 0.89, 95% CI 0.70-1.13) other malformations by systems or spina bifida in particular. However, the risk for spina bifida among newborns and abortuses who were exposed to codeine was four times higher than that of the unexposed (aOR = 4.42, 95% CI 1.60-12.23). This association remained significant in a secondary analysis using propensity score matching. Third trimester exposure to opioids was not associated with low birth weight (aOR = 1.08, 95% CI 0.77-1.52), perinatal death (aOR = 1.38, 95% CI 0.64-2.99) and other adverse pregnancy outcomes. CONCLUSIONS: These findings suggest that opioids exposure (as a homogenous group) is not a significant risk factor for overall major malformations. Exposure to codeine during the first trimester was found to be associated with increased risk of spina bifida. However, this finding was based on a small number of cases and need to be verified in future work.

Constitutive activation of the PI3K-AKT pathway and cardiovascular abnormalities in an individual with Kosaki overgrowth syndrome.

Kosaki overgrowth syndrome is a recently described syndrome characterized by distinctive facial features, brain white matter lesions, and developmental delay. Germline activating heterozygous PDGFRB mutations have been reported in this condition. Systemic connective tissue-type findings have been described in some individuals. We describe a 19-year-old Caucasian female with a history of hydrocephalus, Dandy-Walker malformation, cervical spine arachnoid cyst, progressive scoliosis, and overgrowth. Her physical exam included distinctive craniofacial dysmorphism, as well as soft and hyperextensible skin. Cardiovascular imaging during adolescence revealed saccular aneurysms in both coronary artery systems and subtle tortuosity of the cervical vertebral arteries. Exome sequencing trio analysis identified a de novo previously reported pathogenic variant in PDGFRB, c.1696T>C (p.[Trp566Arg]). Further functional studies included platelet-derived growth factor cellular metabolic pathway activity that confirmed the variant causes a constitutive activation of the PI3K-AKT pathway. This is the first report to characterize the activating nature of this PDGFRB variant. We also highlight the connective tissue findings seen in Kosaki overgrowth syndrome and recommend baseline echocardiographic evaluation in all individuals with this condition with particular emphasis on coronary arteries.

Pericardial Disease, Myocardial Disease, and Great Vessel Abnormalities in Horses.

Pericardial, myocardial, and great vessel diseases are relatively rare in horses. The clinical signs are often nonspecific and vague, or related to the underlying cause. Physical examination usually reveals tachycardia, fever, venous distension or jugular pulsation, a weak or bounding arterial pulse, ventral edema, and abnormal cardiac auscultation such as arrhythmia, murmur, or muffled heart sounds. The prognosis depends on the underlying cause and the disease progression, and ranges from full recovery to poor prognosis for survival. This article focuses on the etiology, diagnosis, prognosis, and treatment of pericarditis, pericardial mass lesions, myocarditis, cardiomyopathy, and great vessel aneurysm or rupture.

Cardiac disorders and structural brain abnormalities are commonly associated with hypospadias in children with neurodevelopmental disorders.

The objective of our study was to use an established cohort of boys to investigate common patterns of malformations in those with hypospadias. We performed a retrospective review of the phenotype of participants in the Deciphering Developmental Disorders Study with neurodevelopmental delay and an 'Abnormality of the genital system'. This group was divided into two subgroups: those with hypospadias and without hypospadias. Associated phenotypes of the two subgroups were compared and analysed. Of the 166 Deciphering Developmental Disorders participants with hypospadias and neurodevelopmental delay, 47 (28%) had cardiovascular and 40 (24%) had structural brain abnormalities. The rate of cardiovascular abnormalities in those with neurodevelopmental delay and genital abnormalities other than hypospadias (N = 645) was lower at 19% (P = 0.001). In addition, structural brain malformations were higher at 24% in the hypospadias group versus 15% in the group without hypospadias (P = 0.002). The constellation of these features occured at a higher rate in the hypospadias group versus the no hypospadias group (P = 0.038). In summary, this is the first study to indicate that cardiovascular and brain abnormalities are frequently encountered in association with hypospadias in children with neurodevelopmental disorders. Not only do these associations provide insight into the underlying aetiology but also they highlight the multisystem involvement in conditions with hypospadias.

A Narrative Review of Cardiovascular Abnormalities After Spontaneous Intracerebral Hemorrhage.

BACKGROUND: The recommended cardiac workup of patients with spontaneous intracerebral hemorrhage (ICH) includes an electrocardiogram (ECG) and cardiac troponin. However, abnormalities in other cardiovascular domains may occur. We reviewed the literature to examine the spectrum of observed cardiovascular abnormalities in patients with ICH. METHODS: A narrative review of cardiovascular abnormalities in ECG, cardiac biomarkers, echocardiogram, and hemodynamic domains was conducted on patients with ICH. RESULTS: We searched PubMed for articles using MeSH Terms "heart," "cardiac," hypertension," "hypotension," "blood pressure," "electro," "echocardio," "troponin," "beta natriuretic peptide," "adverse events," "arrhythmi," "donor," "ICH," "intracerebral hemorrhage." Using Covidence software, 670 articles were screened for title and abstracts, 482 articles for full-text review, and 310 extracted. A total of 161 articles met inclusion and exclusion criteria, and, included in the manuscript. Cardiovascular abnormalities reported after ICH include electrocardiographic abnormalities (56% to 81%) in form of prolonged QT interval (19% to 67%), and ST-T changes (19% to 41%), elevation in cardiac troponin (>0.04 ng/mL), and beta-natriuretic peptide (BNP) (>156.6 pg/mL, up to 78%), echocardiographic abnormalities in form of regional wall motion abnormalities (14%) and reduced ejection fraction. Location and volume of ICH affect the prevalence of cardiovascular abnormalities. Prolonged QT interval, elevated troponin-I, and BNP associated with increased in-hospital mortality after ICH. Blood pressure control after ICH aims to preserve cerebral perfusion pressure and maintain systolic blood pressure between 140 and 179 mm Hg, and avoid intensive blood pressure reduction (110 to 140 mm Hg). The recipients of ICH donor hearts especially those with reduced ejection fraction experience increased early mortality and graft rejection. CONCLUSIONS: Various cardiovascular abnormalities are common after spontaneous ICH. The workup of patients with spontaneous ICH should involve 12-lead ECG, cardiac troponin-I, as well as BNP, and echocardiogram to evaluate for heart failure. Blood pressure control with preservation of cerebral perfusion pressure is a cornerstone of hemodynamic management after ICH. The perioperative implications of hemodynamic perturbations after ICH warrant urgent further examination.

Differences in Cardiovascular Manifestation of Marfan Syndrome Between Children and Adults.

Marfan syndrome (MFS) is a connective tissue disorder characterized by a broad range of clinical manifestations. Cardiovascular involvement is the most life-threatening aspect of the syndrome. Although abnormalities within the cardiovascular system in adults are well documented, there is still a paucity of data regarding manifestation of MFS in childhood. The aim of the study was to compare cardiovascular manifestation of MFS between children and adults. The study population consisted of 236 patients (144 children and 92 adults), who were referred to our department with suspicion of MFS. All patients underwent complete clinical evaluation in order to confirm the diagnosis of MFS according to the modified Ghent criteria. MFS was diagnosed in 101 (44 children and 57 adults) out of the 236 patients. The other patients were diagnosed with Ehlers-Danlos syndrome, Loeys-Dietz syndrome, MASS phenotype, ectopia lentis syndrome, marfanoid habitus and other rare syndromes. The most common cardiovascular abnormality was aortic root dilatation (81.19% of patients). It was found that both adults and children had similar high rates of aortic root dilatation. Similarly, there was no significant difference with regard to the prevalence of aortic valve regurgitation and mitral valve prolapse among children and adults. These findings equivocally indicate that the aforementioned abnormalities develop in early childhood, therefore, they may be used in the early identification of patients with MFS. Other assessed abnormalities, which included mitral valve regurgitation, pulmonary artery dilation, aneurysms of aortic arch, descending thoracic aorta and abdominal aorta were found mostly in adults, and thus, are of less use in the early detection of MFS.

Scimitar syndrome associated with aberrant right subclavian artery, diaphragmatic hernia, and urinary anomalies - case report and review of the literature.

Scimitar syndrome is a form of a partially or totally right pulmonary venous return to the inferior vena cava, which may associate variably right lung hypoplasia, right pulmonary artery hypoplasia, pulmonary sequestration together with the presence of aortopulmonary collaterals from the descending aorta towards the right lung. In many cases, there are also other cardiac anomalies associated. We present a unique association of a partially anomalous pulmonary venous return to the inferior vena cava with other vascular and thoracic anomalies: inferior sinus venosus and secundum atrial septal defect, retroesophageal right subclavian artery, obstructed accessory right bronchus, diaphragmatic hernia with ectopic liver, "S"-type thoracic scoliosis and malformations of the urinary tract (duplication of the right ureter and of the left basinet). The patient had a reimplantation of the "scimitar" vein to the left atrium and closure of the inferior sinus venosus and secundum atrial septal defect.

Blood Products Transfusion and Mid-Term Outcomes of Lung Transplanted Patients Under Extracorporeal Membrane Oxygenation Support.

INTRODUCTION: Extracorporeal membrane oxygenation (ECMO) is considered a reliable technique in lung transplantation requiring cardiorespiratory support. However, the impact of this technology on blood product transfusion rate and outcomes compared to off-pump lung transplantation has been rarely investigated. METHODS: Between January 2012 and June 2015, 52 elective adult lung transplants were performed at our institution. Of these, 15 recipients required intraoperative venoarterial extracorporeal support and 37 did not. We compared blood product consumption and other outcome variables between the 2 groups. RESULTS: We found comparable in-hospital (86.7% vs 97.3%, P = .14) and 6-month (86.7% vs 91.9%, P = .56) survival between patients with and without extracorporeal support, respectively. Survival at 30 days was lower in the ECMO group (86.7% vs 100%, P = .02). Although patients who underwent ECMO received more intraoperative transfusions, postoperative transfusion rate was similar between the 2 groups. The ECMO group experienced longer mechanical ventilation (median 3 vs 2 days, P = .02) and intensive care unit stay (median 7 vs 5 days, P = .02), besides more cardiogenic shock and deep vein thrombosis. However, we observed no difference in other major and minor in-hospital complications and 6-month complications. CONCLUSIONS: In our experience, despite the higher need for intraoperative transfusions, lung transplantation performed with ECMO support is comparable to the off-pump procedure as to short-term survival and outcomes.

Effects of 2-day calorie restriction on cardiovascular autonomic response, mood, and cognitive and motor functions in obese young adult women.

Although long-term energy restriction has been widely investigated and has consistently induced improvements in health and cognitive and motor functions, the responses to short-duration calorie restriction are not completely understood. The purpose of this study was to investigate the effects of a 2-day very low-calorie diet on evoked stress, mood, and cognitive and motor functions in obese women. Nine obese women (body fatness > 32%) aged 22-31 years were tested under two randomly allocated conditions: 2-day very low-calorie diet (511 kcal) and 2-day usual diet. The perceived stressfulness of the diet, cardiovascular autonomic response, and cognitive and motor performances were evaluated before and after each diet. The subjective stress rating of the calorie-restricted diet was 41.5 ± 23.3. Calorie restriction had no detectable effects on the heart rate variability indices, mood, grip strength, or psychomotor functions. By contrast, calorie restriction increased (p < 0.05) spatial processing and visuospatial working memory accuracy, and decreased (p < 0.05) accuracy of cognitive flexibility. In conclusion, our results demonstrate that although a 2-day calorie restriction evoked moderate stress in obese women, cardiovascular autonomic function was not affected. Calorie restriction had complex effects on cognition: it declined cognitive flexibility, and improved spatial processing and visuospatial working memory, but did not affect mood or motor behavior.

Exposure to Nitrogen Oxide in the First Trimester and Risk of Cardiovascular-Related Malformations: A Dose-Response Meta-Analysis of Observational Studies.

Nitrogen oxide (NO x ) is produced during combustion at high temperature, which is a major constituent of air pollutants. Recent studies suggested inconsistent results on the association between NO x exposure and cardiovascular-related malformations. We aimed to assess aforementioned association in pregnant women in the first trimester and cardiovascular-related malformations of infants. A systematic literature review identified studies for observational studies about NO x exposure and cardiovascular-related malformation in PubMed. Random-effect models were used to estimate summary odds ratio (SOR) and 95% confidence intervals (CIs) for aforementioned association. Finally, nine studies met the inclusion criteria. Overall, the SOR of cardiovascular-related malformation per 10 ppb increment in NO x and NO2 concentration was 1.01 (95% CI: 0.98-1.04; I2 = 38.6%, P = 0.09) and 0.99 (95% CI: 0.95-1.04; I2 = 37.8%, P = 0.13), respectively. Stratifying by study design, geographic locations, and confounded adjustments, the majority of strata showed negative results, which were consistent with the main findings. However, we found that exposure to NO x and NO2 in the first trimester increased the risk of coarctation of the aorta (COA) malformation by 13% and 19%, respectively. Our study provided limited evidence regarding the association between NO x exposure in the first trimester and cardiovascular-related malformations in infants.

Single Nucleotide Polymorphisms in the G-Protein Coupled Receptor Kinase 5 (GRK5) Gene are associated with Plasma LDL-Cholesterol Levels in Humans.

Genetically modified mice models suggest an important role for G-protein-coupled receptor kinase 5 (GRK5) in the pathophysiology of obesity and related disorders. We investigated whether single nucleotide polymorphisms (SNPs) in the gene encoding GRK5 affect cardiometabolic traits in humans. We genotyped 3 common SNPs in intron 1 (rs1980030, rs10466210, rs9325562) and one SNP in intron 3 (rs10886471) of GRK5 in 2332 subjects at risk for type 2 diabetes. Total- and visceral fat mass were measured by magnetic resonance (MR) tomography and liver fat content by 1H-MR spectroscopy. Insulin secretion and sensitivity were estimated during an OGTT and measured during the euglycemic, hyperinsulinemic clamp (n = 498). Carriers of the minor allele of rs10466210 and rs1980030 had higher total- and LDL-cholesterol levels (p = 0.0018 and p = 0.0031, respectively, for rs10466210; p = 0.0035 and p = 0.0081, respectively, for rs1980030), independently of gender, age, BMI and lipid-lowering drugs. The effects of rs10466210 withstood Bonferroni correction. Similar associations were observed with apolipoprotein B levels (p = 0.0034 and p = 0.0122, respectively). Carriers of the minor allele of rs10466210 additionally displayed a trend for higher intima-media thickness of the carotid artery (p = 0.075). GRK5 may represent a novel target for strategies aiming at lowering LDL-cholesterol levels and at modifying cardiovascular risk.

Natural history of echocardiographic abnormalities in mucopolysaccharidosis III.

BACKGROUND: Mucopolysaccharidosis (MPS) type III, Sanfilippo Syndrome, is an autosomal recessive lysosomal storage disorder. MPS I and II patients often develop cardiac involvement leading to early mortality, however there are limited data in MPS III. The objective of this study is to describe cardiac abnormalities in a large group of MPS III patients followed in a longitudinal natural history study designed to determine outcome measures for gene transfer trials. METHODS: A single center study of MPS III patients who were enrolled in the Nationwide Children's Hospital natural history study in 2014. Two cardiologists reviewed all patient echocardiograms for anatomic, valvular, and functional abnormalities. Valve abnormalities were defined as abnormal morphology, trivial mitral regurgitation (MR) with abnormal morphology or at least mild MR, and any aortic regurgitation (AR). Abnormal left ventricular (LV) function was defined as ejection fraction < 50%. Group comparisons were assessed using two-sample t-tests or Wilcoxon rank sum tests for continuous variables and chi-square or Fisher's exact tests for categorical variables. RESULTS: Twenty-five patients, 15 Type A and 10 Type B MPS III, underwent 45 echocardiograms. Fifteen patients (60%) demonstrated an abnormal echocardiographic finding with age at first abnormal echocardiogram within the study being 6.8 ±â€¯2.8 years. Left-sided valve abnormalities were common over time: 7 mitral valve thickening, 2 mitral valve prolapse, 16 MR (8 mild, 8 trivial), 3 aortic valve thickening, and 9 AR (7 mild, 2 trivial). Two patients had asymmetric LV septal hypertrophy. No valvular stenosis or ventricular function abnormalities were noted. Incidental findings included: mild aortic root dilation (2), bicommissural aortic valve (1), and mild tricuspid regurgitation (3). CONCLUSIONS: Individuals with Sanfilippo A and B demonstrate a natural history of cardiac involvement with valvular abnormalities most common. In short-term follow up, patients demonstrated only mild progression of abnormalities, none requiring intervention. Valvular disease prevalence is similar to MPS I and II, but appears less severe. These findings raise no specific concerns for gene transfer trials in patients in this age range.