Aortic Valve Abnormalities Research Articles

Analysis of NT-proBNP and uric acid due to left ventricle hypertrophy in the patients of aortic valve disease.

Objective:To evaluate the concentration of N terminal proBNP (NT-proBNP) and partially the serum uric acid in the severe condition of aortic valve dysfunction for assessment of left ventricle hypertrophy.Methods:The study was conducted in the signal transduction lab department of biochemistry Quaid-I-Azam University, Islamabad from September 2013 to February 2017. NT-proBNP and serum uric acid were measured in one hundred patients of aortic valve dysfunction. The patients were divided into three main groups: 1) Aortic stenosis, 2) Aortic regurgitation, and 3) Aortic stenosis with Aortic regurgitation. The results were compared between disease and controls groups.Results:High level of plasma NT-proBNP was detected in all the three disease groups of aortic valve (stenosis, p<0.001), (regurgitation, p<0.001) and (stenosis with regurgitation, p<0.001). In addition, non-significantly increased level of serum uric acid was also observed in left ventricle hypertrophy in all the three respective disease groups of aortic valve.Conclusion:Increased secretion of NT-proBNP during cardiac remodeling can be related to the severity of left ventricle hypertrophy due to aortic valve abnormality in all the disease groups of severe stenosis, severe regurgitation, and combine disease condition of severe stenosis and severe regurgitation. However, non-significant increase in uric acid concentration is also identified which may be due to one of the factors involved in left ventricle hypertrophy in all the three disease groups of aortic valve. The interaction of uric acid with NT-proBNP during cardiac remolding due to aortic valve dysfunction is still not clear.

Activation of valvular interstitial cells and remodeling of extracellular matrix in calcific aortic valve stenosis

Aim: Calcific aortic stenosis is the most common cause of aortic valve replacement in developed countries. The primary cell types in the aortic valve are valvular endothelial and interstitial cells (VICs). Abnormal aortic valve function likely results from extracellular matrix (ECM) remodeling associated with the disequilibrium between the synthesis of ECM components and their degradation.

A prospective study of electrocardiographic and echocardiographic changes in patients with cerebrovascular accidents

Background: Cerebrovascular accident also called as stroke is defined as the sudden onset of neurological deficit that can be attributable to a focal vascular cause. Many studies in the past have demonstrated the fact that neurological abnormalities produce Electrocardiographic changes without any underlying cardiac lesions. Present study is done to analyze different changes in Electrocardiogram and echocardiographic patterns in the cases of cerebrovascular accidents and to assess whether these different changes have got any prognostic significance in these patients.Methods: In this study 100 patients of acute stroke admitted between May 2011 to August 2012 in Vydehi institute of medical sciences and research centre were considered and subjected to CT scan of brain, Electrocardiogram and 2D echocardiography within 24 hours of admission. Follow-up was done within the hospital to know the prognosis of all the patients.Results: Out of 100 patients majority of them had ischemic stroke accounting for 80% and remaining 20% of patients had haemorrhagic stroke. Abnormal Electrocardiographic changes were seen in 64% patients and 58% patients showed abnormal Echocardiography. ECG changes seen are Tachycardia, QTc prolongation, T wave inversion, ST depression, U waves and Bradycardia. Commonly seen echocardiographic changes were LV dysfunction, mitral valve abnormality, aortic valve abnormality. Overall mortality in patients with abnormal ECG was 28.12% compared to 11.11% in patients with normal ECG and it was statistically not significant. Overall mortality in patients with abnormal 2D echocardiography was 31.03% compared to patients with normal 2D Echocardiography which was 4.76% and it was statistically significant.Conclusions: From this study we conclude that ECG abnormalities in stroke patients do not have any prognostic significance. But LV dysfunction has prognostic significance in predicting mortality in cerebrovascular accident.

A prospective study of electrocardiographic and echocardiographic changes in patients with cerebrovascular accidents

Background: Cerebrovascular accident also called as stroke is defined as the sudden onset of neurological deficit that can be attributable to a focal vascular cause. Many studies in the past have demonstrated the fact that neurological abnormalities produce Electrocardiographic changes without any underlying cardiac lesions. Present study is done to analyze different changes in Electrocardiogram and echocardiographic patterns in the cases of cerebrovascular accidents and to assess whether these different changes have got any prognostic significance in these patients.Methods: In this study 100 patients of acute stroke admitted between May 2011 to August 2012 in Vydehi institute of medical sciences and research centre were considered and subjected to CT scan of brain, Electrocardiogram and 2D echocardiography within 24 hours of admission. Follow-up was done within the hospital to know the prognosis of all the patients.Results: Out of 100 patients majority of them had ischemic stroke accounting for 80% and remaining 20% of patients had haemorrhagic stroke. Abnormal Electrocardiographic changes were seen in 64% patients and 58% patients showed abnormal Echocardiography. ECG changes seen are Tachycardia, QTc prolongation, T wave inversion, ST depression, U waves and Bradycardia. Commonly seen echocardiographic changes were LV dysfunction, mitral valve abnormality, aortic valve abnormality. Overall mortality in patients with abnormal ECG was 28.12% compared to 11.11% in patients with normal ECG and it was statistically not significant. Overall mortality in patients with abnormal 2D echocardiography was 31.03% compared to patients with normal 2D Echocardiography which was 4.76% and it was statistically significant.Conclusions: From this study we conclude that ECG abnormalities in stroke patients do not have any prognostic significance. But LV dysfunction has prognostic significance in predicting mortality in cerebrovascular accident.

The great debate series: surgical treatment of aortic valve abnormalities in children.

This article is the latest in an EJCTS series entitled 'The Great Debates'. We have chosen the topic of aortic valve (AoV) surgery in children, with a focus on infants and neonates. The topic was selected due to the significant challenges that AoV problems in the young may present to the surgical team. There are many areas of active controversy, despite the vast accumulated world experience. We have tried to incorporate many of these issues in the questions posed, not claiming to be all-inclusive. The individuals invited to this debate are experts in paediatric valve surgery, with broad and successful clinical experiences on multiple continents. We hope that the facts and opinions presented in this debate will generate interest and discussion and perhaps prove useful in decision-making for future complex valve cases.

Abstract P025: Left Atrial Dilatation Predicts The Incidence Of Persistent Atrial Fibrillation In Japanese General Population; The Suita Study

Objective: Atrial fibrillation (AF) is an important risk factor for cardiovascular disease (CVD). Left atrial (LA) dilatation detected by ultrasonic cardiography (UCG) has been reported to be associated with the incidence of AF in Western countries. However, the relationship between LA dilatation and the AF incidence has not been well investigated in Asian countries, with lower incidence of AF and obesity and with higher incidence of hypertension and stroke. To investigate the relationship between LA dilatation and the AF incidence in Japanese general population, we estimated the multivariate-adjusted hazard ratios (HR) of LA dilatation assessed by UCG for the incidence of AF. Methods: Participants were 1,585 individuals without AF (665 men, 920 women) who are the participants of a prospective cohort study for CVD incidence in urban Japanese general population (the Suita study). The mean±SD age of the participants were 67.8±7.0 years. Transthoracic UCG was performed from July in 2007 to June in 2013, and LA dimension was determined in accordance with American Society of Echocardiography recommendations by M-mode echocardiograms. After the UCG, the electrocardiograms (ECG) were repeatedly performed until March in 2016. The physicians coded the ECG using Minnesota Code (MC), and AF code is defined as 8-3-1. The HRs of LA dilatation for the incidence of AF with 95% confidence interval (CI) were estimated by Cox proportional hazard model with the adjustment for the followings at the UCG test; age, sex, systolic blood pressure, body mass index, the presence of heavy drinking (more than 2 drinks/day), non-HDL cholesterol, and the presence of the moderate or severe mitral or aortic valve abnormality detected by UCG. Results: The mean follow-up period from UCG to the last ECG was 4.6±1.8 years, and 19 AF incidences were detected. The multivariate-adjusted HR of the LA dilatation (&gt;40 mm) for the incidence of AF was 8.19 (95% CI: 2.47-27.14). And the multivariate-adjusted HR of 5 mm increment of LA dimension was 2.95 (1.65-5.29), and that of the highest quartile of LA dimension compared to the lowest was 3.37 (0.64-17.78). Among 674 participants with left atrial volume index (LAVI) data (5 AF cases), the multivariate-adjusted HR of LAVI ≥ 32 (ml/m 2 ) for AF incidence was 3.93 (95%CI: 0.34-45.86), and it showed the highest HR among the co-adjusted factors. Conclusion: LA diameter assessed by UCG is a simple and non-invasive clinical data, and could be a useful factor for the prediction of AF incidence among Japanese general population. Although the follow-up period was not enough compared to the previous studies in Western population, the present study might indicate that individuals with LA dilation detected by UCG are the important candidate for careful and continuous check-ups about CVD risk factors, ECG and UCG in Asian population.

Computational study of aortic hemodynamics for patients with an abnormal aortic valve: The importance of secondary flow at the ascending aorta inlet.

Blood flow in the aorta is helical, but most computational studies ignore the presence of secondary flow components at the ascending aorta (AAo) inlet. The aim of this study is to ascertain the importance of inlet boundary conditions (BCs) in computational analysis of flow patterns in the thoracic aorta based on patient-specific images, with a particular focus on patients with an abnormal aortic valve. Two cases were studied: one presenting a severe aortic valve stenosis and the other with a mechanical valve. For both aorta models, three inlet BCs were compared; these included the flat profile and 1D through-plane velocity and 3D phase-contrast magnetic resonance imaging derived velocity profiles, with the latter being used for benchmarking. Our results showed that peak and mean velocities at the proximal end of the ascending aorta were underestimated by up to 41% when the secondary flow components were neglected. The results for helical flow descriptors highlighted the strong influence of secondary velocities on the helical flow structure in the AAo. Differences in all wall shear stress (WSS)-derived indices were much more pronounced in the AAo and aortic arch (AA) than in the descending aorta (DAo). Overall, this study demonstrates that using 3D velocity profiles as inlet BC is essential for patient-specific analysis of hemodynamics and WSS in the AAo and AA in the presence of an abnormal aortic valve. However, predicted flow in the DAo is less sensitive to the secondary velocities imposed at the inlet; hence, the 1D through-plane profile could be a sufficient inlet BC for studies focusing on distal regions of the thoracic aorta.

Echocardiographic Screening of Rheumatic Heart Disease in American Samoa.

While rheumatic heart disease (RHD) is a treatable disease nearly eradicated in the United States, it remains the most common form of acquired heart disease in the developing world. This study used echocardiographic screening to determine the prevalence of RHD in children in American Samoa. Screening took place at a subset of local schools. Private schools were recruited and public schools underwent cluster randomization based on population density. We collected survey information and performed a limited physical examination and echocardiogram using the World Heart Federation protocol for consented school children aged 5-18years old. Of 2200 students from two private high schools and two public primary schools, 1058 subjects consented and were screened. Overall, 133 (12.9%) children were identified as having either definite (3.5%) or borderline (9.4%) RHD. Of the patients with definitive RHD, 28 subjects had abnormal mitral valves with pathologic regurgitation, three mitral stenosis, three abnormal aortic valves with pathologic regurgitation, and seven borderline mitral and aortic valve disease. Of the subjects with borderline disease, 77 had pathologic mitral regurgitation, 12 pathologic aortic regurgitation, and 7 at least two features of mitral valve disease without pathologic regurgitation or stenosis. Rheumatic heart disease remains a major cause of morbidity and mortality worldwide. The prevalence of RHD in American Samoa (12.9%) is to date the highest reported in the world literature. Echocardiographic screening of school children is feasible, while reliance on murmur and Jones criteria is not helpful in identifying children with RHD.

Antiphospholipid Antibodies and Heart Valve Disease in Systemic Lupus Erythematosus

Evaluation of antiphospholipid antibodies (aPL) and correlation with heart valve abnormalities among patients with systemic lupus erythematosus (SLE). Nested case-control study was conducted with 70 patients with SLE selected from a longitudinal database based on levels of aPL and presence or absence of valve disease by echocardiogram. Valvular abnormalities observed were regurgitation (52), other (14), artificial valves (4), stenosis (2), thickening (2) and no Libman-Sacks endocarditis (0). The mitral valve was the most commonly affected (30 abnormalities), followed by the tricuspid (20 abnormalities). Multivariate logistic regression for those with and without an aPL value ≥20 units/mL, adjusted for disease duration and age, showed significant differences for any valve abnormality (odds ratio [OR] = 3.1; 95% CI: 1.0-8.9; P = 0.041) and individually for the tricuspid valve (OR = 3.3; 95% CI: 1.0-11.1; P = 0.052) but not for the mitral valve (OR = 2.1; 95% CI: 0.68-6.45; P = 0.195). Levels of aPL ≥20 units/mL showed no association with aortic (P = 0.253), pulmonic (P = 1.000), tricuspid (P = 0.127), or mitral (P = 0.249) valve abnormalities. Levels of aPL correlate with certain valvular abnormalities among patients with SLE.

The complex genetics of hypoplastic left heart syndrome.

Congenital heart disease (CHD) affects up to 1% of live births. Although a genetic etiology is indicated by an increased recurrence risk, sporadic occurrence suggests that CHD genetics is complex. Here, we show that hypoplastic left heart syndrome (HLHS), a severe CHD, is multigenic and genetically heterogeneous. Using mouse forward genetics, we report what is, to our knowledge, the first isolation of HLHS mutant mice and identification of genes causing HLHS. Mutations from seven HLHS mouse lines showed multigenic enrichment in ten human chromosome regions linked to HLHS. Mutations in Sap130 and Pcdha9, genes not previously associated with CHD, were validated by CRISPR-Cas9 genome editing in mice as being digenic causes of HLHS. We also identified one subject with HLHS with SAP130 and PCDHA13 mutations. Mouse and zebrafish modeling showed that Sap130 mediates left ventricular hypoplasia, whereas Pcdha9 increases penetrance of aortic valve abnormalities, both signature HLHS defects. These findings show that HLHS can arise genetically in a combinatorial fashion, thus providing a new paradigm for the complex genetics of CHD.

Automatic cardiac phase detection of mitral and aortic valves stenosis and regurgitation via localization of active valves

Abstract Heart auscultation is a primary way of heart diagnosis and monitoring. A critical step of heart sound analysis is dividing the sound into its basic phases, known as S1, systole, S2 and diastole. Heart sound phase detection becomes a challenging issue when more than one heart valve problems are simultaneously existed. In this paper, a powerful approach is proposed that properly determine phases of heart sounds with Mitral and Aortic stenosis and Regurgitation murmurs. In this approach, heart valves spatial-temporal information investigated by a multiple channel data recording system is employed to determine different heart phases. Since heart valves are very near to each other and are located in the reverberant environment of chest, according to its anatomy, the group-delay information of Recursively Applied and Projected-Multiple Signal Classification spectra (RAP-MUSIC) is utilized to localize active heart valves. The proposed segmentation algorithm is applied to some normal and abnormal (mitral and aortic valve malfunctions) heart sounds recorded by a rectangular microphone array consisted of six sensors. To evaluate the benefits of the proposed method, it is compared with the basic and Tunable Q wavelet and S-transform based segmentation methods, by conducting some proper experiments. The obtained results show that the proposed algorithm is considerably superior to the mentioned methods The average positive predictive value, sensitivity and accuracy results from the proposed segmentation algorithm are 97.4%, 95.5%, and 93%, respectively.

Prevalence and prognostic impact of left-sided valve thickening in systemic light-chain amyloidosis.

Left heart valve thickening (LVT) was described in patients with light-chain amyloidosis (AL). This phenomenon reflects likely infiltration of the valve by amyloid proteins. However, the prevalence of LVT and its prognostic value have not been investigated in patients with AL. Comprehensive transthoracic echocardiography was performed at baseline in 150 patients [median age 68 (33-87) years; 59% male] with confirmed AL. The presence of abnormal mitral and/or aortic valve thickening (>3mm) was assessed in all included patients. Overall, 42% had LVT at the time of diagnosis. Compared to patients without LVT, those with LVT were older and had a more advanced NYHA functional class (63% in patients with NYHA III-IV vs. 33% in NYHA I-II, p<0.001). They also had higher left ventricular (LV) wall thickness and mass, larger left atrium, higher mitral annulus E/E' ratio and systolic pulmonary artery pressures, and lower LV ejection fraction (all p<0.05). Patients with more advanced Mayo Clinic stage had a higher incidence of LVT: 58% in stage III vs. 45% in stage II and 5% in stage I (p<0.001). During a median follow-up of 2years, 79 deaths occurred. The presence of LVT was significantly associated with reduced 5-year survival (32±7 vs. 64±6%). In multivariate analysis, after adjusting for age, gender, NYHA functional class, and LV ejection fraction, LVT remained significantly associated with higher all-cause mortality (hazard ratio 1.90, 95% CI 1.10-3.34, p=0.02). Left heart valve thickening is common in patients with AL and is associated with worse functional class, LV systolic and diastolic function, and more advanced stage of the disease. In addition, LVT appears to be a powerful marker of all-cause mortality.

Prenatal Sonographic Predictors of Neonatal Coarctation of the Aorta.

To identify practical prenatal sonographic markers for the postnatal diagnosis of coarctation of the aorta. We reviewed the fetal echocardiograms and postnatal outcomes of fetal cases of suspected coarctation of the aorta seen at a single institution between 2010 and 2014. True- and false-positive cases were compared. Logistic regression analysis was used to determine echocardiographic predictors of coarctation of the aorta. Optimal cutoffs for these markers and a multivariable threshold scoring system were derived to discriminate fetuses with coarctation of the aorta from those without coarctation of the aorta. Among 35 patients with prenatal suspicion of coarctation of the aorta, the diagnosis was confirmed postnatally in 9 neonates (25.7% true-positive rate). Significant predictors identified from multivariate analysis were as follows: Z score for the ascending aorta diameter of -2 or less (P = < .001), Z score for the mitral valve annulus of -2 or less (P= .033), Zscore for the transverse aortic arch diameter of -2 or less (P= .028), and abnormal aortic valve morphologic features (P= .026). Among all variables studied, the ascending aortic Z score had the highest sensitivity (78%) and specificity (92%) for detection of coarctation of the aorta. A multivariable threshold scoring system identified fetuses with coarctation of the aorta with still greater sensitivity (89%) and only mildly decreased specificity (88%). The finding of a diminutive ascending aorta represents a powerful and practical prenatal predictor of neonatal coarctation of the aorta. A multivariable scoring system, including dimensions of the ascending and transverse aortas, mitral valve annulus, and morphologic features of the aortic valve, provides excellent sensitivity and specificity. The use of these practical sonographic markers may improve prenatal detection of coarctation of the aorta.

Simulation of motor current waveforms in monitoring aortic valve state during ventricular assist device support.

Monitoring of aortic valve (AV) opening and closure during left ventricular assist device (LVAD) heart pump support is crucial in preventing AV abnormalities and remodeling caused by anomalous resirculation. In this study, simulations of LVAD motor current waveforms were undertaken to investigate AV response to rotary blood pump assistance, as well as to detect AV open and close status under heart failure conditions. A two-dimensional fluid-structure interaction finite-element model is presented to predict AV state during LVAD outflow. The data will be useful in the development of a pump speed controller for optimal management of pump outflow.

Prevalence of aortic valve dystrophy and insufficiency in a cohort of 255 HIV-positive patients followed-up in a cardiology department between 2012 and 2014

ObjectivesTo study valve appearance and the presence of valve disease in a cohort of people living with HIV (PLHIV). DesignA prospective study of PLHIV examined at the cardiology department of the Clermont Ferrand university hospital group (CHU) between January 1, 2012, and December 31, 2014. Were excluded those with a history of infection associated with a possible endocarditis. MethodsDemographic, medical characteristics and cardiovascular disease risk factors at time of cardiovascular examination and Doppler-echocardiography were recorded and analyzed. ResultsIn total, 903 PLHIV were examined in the infectious diseases department, 255 of whom were included. These consisted of 67 women (26.3%) and 188 men, of a mean age of 51.2±9.7years, in whom coronary artery disease was diagnosed in 18 patients (7.0%), two women and 16 men, representing a prevalence of 3.0% in females and 8.5% in males. The appearance of the aortic cusps was considered dystrophic in 14.1% of cases (36/255), dysplastic in two cases (0.8%), exhibiting a bicuspid deformity in one case. The prevalence of aortic valve abnormality was therefore 6.0% in the women (4/67) and 17.0% in the men (32/188). On facing off this data with the Kora Monica study findings, an increase in prevalence appears only to truly manifest after 50years of age. We registered 35 aortic insufficiency cases (13.7%), representing a higher incidence than that of the Framingham cohort, with age and masculine gender being the determining factors. ConclusionValve disease, along with coronary artery disease, should be closely monitored in PLHIV.

Intima-media thickness of the descending aorta in patients with bicuspid aortic valve

ObjectiveA bicuspid aortic valve (BAV) is associated with accelerated aortic valve disease (AVD) and abnormalities in aortic elasticity. We investigated the intima-media thickness of the descending aorta (AoIMT) in patients with AVD with or without an ascending aortic aneurysm (AscAA), in relation to BAV versus tricuspid aortic valve (TAV) phenotype, type of valve disease, cardiovascular risk factors, and single-nucleotide polymorphisms (SNPs) with a known association with carotid IMT. Methods and results368 patients (210 with BAV, 158 with TAV,); mean age 64±13years) were examined using transesophageal echocardiography (TEE) before valvular and/or aortic surgery. No patient had a coronary disease (CAD). The AoIMT was measured on short-axis TEE images of the descending aorta using a semi-automated edge-detection technique. AoIMT was univariately (P<0.05) related to age, blood pressure, smoking, creatinine, highly sensitive C-reactive protein, HDL, valve hemodynamics and BAV. In the TAV subgroup it was also associated with the rs200991 SNP. Using multivariate regression analysis, age was the main determinant for AoIMT (P<0.001), followed by male gender (P=0.02), BAV was no longer a significant predictor of AoIMT. AoIMT was still related to the rs200991 SNP in TAV (P=0.034), and to creatinine in BAV (P=0.019), when other variables were accounted for. ConclusionsIntima-media thickness of the descending aorta is not affected by aortic valve morphology (BAV/TAV); age is the main determinant of AoIMT. Genetic markers (SNPs) known to influence IMT in the carotid artery seem to correlate to IMT in the descending aorta only in patients with TAV.

Neurocardiogenic Syncope and Supraventricular Tachycardia in Association with a Rare Congenital Aortic Valve Abnormality.

We report a case of a 26-year-old woman who presented with multiple episodes of syncope over a five-months period of time. Transthoracic echocardiogram had shown a normal functioning quadricuspid aortic valve (QAV) which was also confirmed on a transesophageal echocardiogram. Computed tomographic angiography of heart and coronary arteries showed the QAV with equal size of all aortic cusps and normal coronary arteries. Intermittent chest pain and palpitations warranted an exercise stress test. The stress test revealed normal aerobic exertion, with achievement of 101% of maximal peak heart rate. However, during peak stress, we noted a drop in her blood pressure significantly resulting in dizziness. No arrhythmias were noted during the stress test. With recurrent syncope episodes and palpitations, Holter monitoring was done, revealing supraventricular tachycardia (SVT). We discuss current available literature and coassociations with QAV. New association of QAV with SVT needs further analysis.

Large Cryptic Derivative Chromosome 8 Detected by SNP Chromosomal Microarray

Large Cryptic Derivative Chromosome 8 Detected by SNP Chromosomal Microarray

Streamlining NGS Workflows Using Cancer Samples by the Application of Tthe DNA Integrity Number (DIN) from the Genomic DNA Screentape Assay

Streamlining NGS Workflows Using Cancer Samples by the Application of Tthe DNA Integrity Number (DIN) from the Genomic DNA Screentape Assay

A Case of Reye’s-Like Syndrome in a 68-Day Old Infant: Water Damaged Home, Mold, Bacteria and Aflatoxins.

Introduction: Reye’s Syndrome is characterized by acute encephalopathy, hepatic injury accompanied with elevated serum ammonia, serum fatty acids, amino acids and triglycerides; hypoglycemia, prolonged prothrombin time, fatty infiltration of the liver, and mitochondrial pathology. We present an infant that died at 68 days of age with Reye ’s syndrome. The infant and parents were exposed to airborne fungi, bacteria and toxic bioaerosols resulting from water intrusion in the home. The purpose of this study was to investigate and, if possible, to determine the cause of death of the infant with respect to the fungi, bacteria and their toxins present in the families water-damaged home. Materials and Methods: Health and genetic history was done on the family (father, mother and two siblings). Environmental evaluation was carried out to identify airborne fungi and bacteria in the home. Clinical testing of the baby while in the hospital provided data on blood chemistry and EEG results. Mitochondrial studies on skin fibroblasts and skeletal muscle were carried out testing for functions of Complexes I-IV and mitochondrial DNA mutations. Light and E.M. microscopy were done on liver biopsy material. Immunoaffinity column and fluorometry were used to detect aflatoxins (B1, B2, G1 and G2) in liver autopsy material. The mother’s breast milk and urine was tested for mycotoxins: trichothecenes, aflatoxins and ochratoxin. Results: Medical and genetic history were negative for familial diseases similar to Reye’s syndrome and for mitochondrial DNA mutations. Aortic and pulmonic valve abnormalities were observed. Environmental testing revealed the presence of elevated levels of several species of fungi and bacteria in the infant’s bedroom and other rooms of the home. Clinical diagnostic tests of the infant revealed metabolic acidosis, elevated serum ammonia, triglycerides, pyruvic and lactic acids, serum alanine, and beta-hydroxybutyrate. Mitochondrial studies showed decreased function of complexes I-IV and the absence of known mutations associated with mitochondrial diseases. Microscopy (light and E.M.) of biopsies demonstrated the accumulation of glycogen in muscle and fatty droplets in the liver. Aflatoxins were detected in the infant’s liver (2.1 ppb), and the mother’s breast milk (15 ppb), while maternal urine was positive for trichothecenes (4.76 ppb) and ochratoxin (3.4 ppb). Conclusions: After a review of the peer reviewed literature, we conclude that the infant died of a Reye’s-Like Syndrome at the age of 68 days (all 22 cited criteria were met). Clinical and autopsy findings were consistent with this disease process. The valvular abnormalities found are associated with actinomycetes exposure. The medical and genetic histories were negative for any familial diseases of a similar nature. The infant had mitochondrial dysfunction of complexes I-IV, suggesting mitochondrial disease consistent with alflatoxin toxicity. The presence of aflatoxins in the liver supports the causal role of this mycotoxin in the illness of this infant. We have discussed the role of other factors in the indoor environment that may also lead to similar conditions.