Cardiovascular Changes In Children Research Articles

Relation between the Epicardial Fat Thickness and the Cardiac Conduction System in Children and Adolescents with Diabetes

Introduction: Atherosclerosis in patients with type 1 diabetes starts early in childhood with subclinical abnormalities. The epicardial fat thickness (EFT) is a novel method for detecting these early changes. Furthermore, electrocardiographic markers may be altered in patients with diabetes owing to early cardiovascular changes. This study aimed to determine the relationship between EFT and electrocardiographic markers in children with type 1 diabetes mellitus. Methods: Children with type 1 diabetes who were followed up at the Alexandria University Children’s Hospital Diabetes Clinic were enrolled in this study. The study recruited three groups of participants, including 20 patients with a diabetes duration of less than 5 years, 20 patients with a diabetes duration of 5 years or more, and 20 healthy controls. All participants were evaluated with emphasis on anthropometric measurements, fasting blood glucose levels, and lipid profile. HbA1c levels were measured in the cohort with diabetes. All participants underwent electrocardiography for measurement of P-wave dispersion, corrected QT interval and its dispersion, and Tp-e measurement. Echocardiography was performed to measure the EFT. Results: Among all participants, EFT was significantly higher in children with a diabetes duration of ≥5 years (p = 0.009). Furthermore, P-wave dispersion was significantly prolonged in children with diabetes compared to that in nondiabetics (p = 0.041). There was a statistically significant correlation between EFT and P-wave dispersion in patients with diabetes aged ≥5 years (p = 0.021). Conclusions: Measurement of EFT by echocardiography is a novel and easy way to predict early cardiovascular changes in children with diabetes, including conduction system disorders.

Comorbid Cardiovascular Malformation and Type II Mucolipidosis: Clinical Case

Background . Type II mucolipidosis (I-cell disease, ICD) is one of the lysosomal storage diseases. It is very rare disease; the literature describes only few cases with confirmed diagnosis of mucolipidosis. Cardiovascular changes in children with such pathology are even less often. Clinical case description . The article describes the clinical case of type II mucolipidosis alongside with cardiovascular pathology — valvular heart apparatus defect with abdominal aortic hypoplasia and reversible myocardial dysfunction on the therapy of chronic heart failure (CHF). The patient has coarse face, gingival hyperplasia, macroglossia, dysostosis multiplex, diffuse muscular hypotonia, and mass of subcutaneous tissue. Arterial hypertension, heart cavities dilatation, left ventricular (LV) walls hypertrophy, and data of CT aortography let us to diagnosis abdominal aortic hypoplasia. Conclusion . Cardiovascular malformation in patients with mucolipidosis leads to severe, life-threatening conditions development. Untimely diagnosis can worsen the course of disease. Multidisciplinary approach is needed for the patient management.

Effect of Sleep Disorders on Blood Pressure and Hypertension in Children.

In this review, we aim to discuss the pathophysiologic basis of hypertension in sleep disorders and the current evidence in the medical literature linking sleep disorders and hypertension in children. The medical literature in adults is clear about the contribution of sleep disorders, poor sleep quality, and sleep deprivation to hypertension and increased cardiovascular risk. The literature on cardiovascular consequences of sleep disorders in children is not as robust, but there is some evidence of early cardiovascular changes in children with sleep deprivation and obstructive sleep apnea. Children with obstructive sleep apnea have increased sympathetic activation during sleep, blunted dipping, or elevated systolic or diastolic pressures. Although the literature on other sleep disorders such as narcolepsy and restless legs syndrome is scarce, there is evidence in adults and some recent supportive data in children.

Cardiovascular changes in children with obstructive sleep apnea and obesity after treatment with noninvasive ventilation.

Adults with obesity and obstructive sleep apnea (OSA) are at risk for cardiometabolic disease, and this risk likely extends to children with both conditions. Noninvasive ventilation (NIV; including continuous and bilevel positive airway pressure) is often used to treat OSA in children with obesity. The aim of this study was to examine the impact of NIV treatment on heart rate variability (HRV), as a marker of cardiovascular risk, in children with obesity and newly diagnosed OSA. A prospective multicenter cohort study was conducted in children with obesity prescribed NIV therapy for newly diagnosed moderate-severe OSA. Measurements of HRV were derived from polysomnography recordings at baseline and after 12 months of treatment. HRV parameters were examined by sleep stage, before and after arousal and oxygen desaturation events. HRV parameters were compared between time points using pair t tests as well as mixed model analysis. Twelve children had appropriate data for analysis at baseline and 12 months. Heart rate decreased by 4.5 beats/min after NIV treatment, with no change in HRV parameters. HRV parameters differed by sleep stage and showed an increase in arousal-related sympathetic-parasympathetic balance after 12 months of NIV treatment. HRV parameters did not differ before and after oxygen desaturation events. NIV for the treatment in children with obesity and OSA resulted in a small decrease in heart rate and an increase in arousal-related sympathetic-parasympathetic balance. These findings suggest small, potentially positive impacts of NIV on cardiovascular risk in children with concurrent obesity and OSA.

Cardiovascular changes in children with sickle cell crisis

Sickle cell anaemia is characterised by frequent, sometimes serious events referred to as "crisis". Cardiopulmonary consequences such as pulmonary hypertension and myocardial ischaemia may accompany a serious crisis. To determine the cardiovascular changes that occur during a severe sickle cell crisis. A cross-sectional comparative study of sickle cell anaemia in children (5-17 years) admitted during a severe crisis (cases) and those in steady state (controls) was conducted over a 2-year period. Effects of the crisis on the cardiopulmonary system were assessed. The diagnosis of myocardial ischaemia was made using electrocardiography and serological cardiac biomarkers, while cardiac dysfunction and the presence of pulmonary hypertension were determined using echocardiography. The presence of systemic hypertension and tachycardia was also evaluated. A total of 176 patients were recruited, 92 in steady state (male:female ratio, 1.2:1) and 84 in severe crisis (male:female ratio, 1.3:1). The mean age was 10.4 ± 3.2 years for steady state and 10.5 ± 3.4 years for those in crisis. The mean heart rate in crisis was higher than in steady state (p < 0.0001). The blood pressures (systolic, p < 0.0001, diastolic, p < 0.0001, mean, p < 0.0001) as well as myocardial ischaemia scores (p < 0.0001) were higher in patients with crisis than in those in steady state. Similarly, conduction abnormalities, pulmonary hypertension, and ventricular dysfunction were more prevalent in the crisis than in the steady state. The present data suggest that sickle cell crisis results in a derangement of clinical, electrocardiographical, and echocardiographical parameters in children with sickle cell anaemia. Further research on these cardiovascular events may improve the overall care of these patients.

Cardiovascular Changes in Children with Acute Lower Respiratory Tract Infections in Zagazig University Hospitals

Cardiovascular Changes in Children with Acute Lower Respiratory Tract Infections in Zagazig University Hospitals

Cardiovascular Changes in Children with Acute Lower Respiratory Tract Infection

Background: Acute Lower Respiratory Tract Infections (ALRI), particularly Pneumonia and Bronchiolitis, are important causes of death in childhood in Bangladesh. The cardiovascular and respiratory systems function as a single unit and alteration in cardiorespiratory interactions, can cause significant changes in cardiac function. The objective of this study was to find out any electrical and functional changes, myocardial injury, frequency of heart failure and the outcomes in these patients with ALRIs.&#x0D; Methodology: It was a prospective observational study carried out at DMCH from January to June 2012 on 35 consecutive children admitted with ALRI, which were further diagnosed as pneumonia or bronchiolitis using operational definitions. Heart failure cases were identified and all the cases were then evaluated for any cardiovascular changes.&#x0D; Results: Most of the patients were male. ECG changes occurred in the form of tachycardia. Abnormal echocardiographic findings were noted in the form of pulmonary hypertension, left ventricular systolic (LV) dysfunction and tricuspid regurgitation. 82.9% had raised CK-MB and was significantly higher in patients with tachycardia and having abnormal echocardiographic changes. Nine patients developed heart failure, 3 of them had pulmonary hypertension along with LV systolic dysfunction and one of pulmonary hypertension and LV systolic dysfunction each. CK-MB was raised significantly in all the patients with heart failure.&#x0D; Conclusion: Raised CK-MB, tachycardia out of proportion on ECG and pulmonary hypertension with left ventricular systolic dysfunction were common findings in patients with ALRI.&#x0D; Bangladesh J Child Health 2019; VOL 43 (1) :27-34

Preclinical cardiovascular changes in children with obesity: A real-time 3-dimensional speckle tracking imaging study.

The aims of this study were (1) to quantify changes in 3-dimensional (3D) strain in obese children using real-time 3D echocardiography (RT3DE) and 3D speckle tracking echocardiography (3DSTE), and (2) to investigate the utility of left ventricular (LV) strain variables in measuring early cardiovascular changes in children with obesity. A total of 181 obese children (study group) aged 4–18 years old were prospectively enrolled and compared with 229 healthy subjects (control group). We acquired demographic, clinical, biochemical, and 2D echocardiography/Doppler data. Also, RT3DE and 3DSTE were performed to measure LV volume, left ventricular ejection fraction (LVEF), LV mass (LVM), LV peak systolic global longitudinal strain (GLS), radial strain (GRS), circumferential strain (GCS), and global strain (GS). There were significant differences in anthropometric measurements, blood pressures, Cholesterol, C-reactive protein (CRP), Intima-media thickness (IMT), left atrium end-systolic dimension (LASD), interventricular septal end-diastolic dimension (IVSD), LV posterior wall end-diastolic dimension (LVPWD), LV end-diastolic dimension (LVEDD), LV end-systolic dimension (LVESD), LV end-diastolic volumes (LVEDV), and LV end-systolic volumes (LVESV), E and A velocities, E/A,e’, e’/a’, E/e’, LVM, LV mass index (LVMI), GLS, GRS, GCS, and GS between the study and control groups. The receiver operating characteristic curves (ROC) for the statistically significant echocardiographic variables showed that the range of areas of ROC curves varied from 0.76 (GLS), 0.74 (GRS), 0.72 (LASD), to 0.58 (LVESD), respectively. In conclusion, LV 3D strain variables by RT3DE and 3DSTE decrease in obese children. LV 3D strain is more sensitive than other echocardiographic and vascular ultrasound variables in detecting cardiovascular changes in children with obesity.

Postnatal persistence of fetal cardiovascular remodelling associated with assisted reproductive technologies: a cohort study.

To assess the postnatal persistence of fetal cardiovascular remodelling associated with assisted reproductive technologies (ART) in children at 3 years of age. A cohort study of children conceived by ART. Maternal-Fetal Medicine Unit, Hospital Clinic Barcelona, Spain. Eighty singleton pregnancies conceived by ART and 80 spontaneously conceived (controls) followed from fetal life up to childhood. Cardiovascular evaluation was performed at 3 years of corrected age, including echocardiography, carotid intima-media (cIMT) by ultrasound, and blood pressure. Postnatal persistence of cardiovascular changes in children conceived by ART. Compared with controls, children conceived by ART showed larger atria (right atrial area: control 4.9 cm2 (0.9) versus ART 5.5 cm2 (0.9), P < 0.001), more globular ventricles (right ventricular sphericity index: control mean 1.8 (SD 0.5) versus ART 1.6 (0.2), P < 0.001), and signs of systolic (tricuspid annular plane systolic excursion: control 18 mm (2) versus ART 16 mm (3), P < 0.001) and diastolic dysfunction (isovolumic relaxation time: control 68 ms (12) versus ART 79 ms (12), P < 0.001). ART children also presented increased systolic blood pressure (control 90 mmHg (6) versus ART 94 mmHg (5), P < 0.003) and cIMT (control 0.52 μm (0.14) versus ART 0.60 μm (0.16), P < 0.001) as compared with those spontaneously conceived. Cardiovascular changes previously reported in ART fetuses persist postnatally at 3 years of age. These results underscore the importance of future studies for assessing the long-term cardiovascular health associated with ART. Cardiovascular changes described in fetuses conceived by ART, persist in children at 3 years of age.

Dyslipidemia and cardiovascular changes in children.

In this review, we firstly highlight the role of dyslipidemia as a trigger in the initiation and progression of endothelial dysfunction, considered the earliest atherosclerotic lesion and patent in children with risk factors.In this context, we also revise methods that reflect the impact of endothelial dysfunction not only on arterial stiffness but also on cardiovascular morphology, namely, the common carotid intima-media thickness and the ventricular geometry. In view of its atherogenic burden, the most widely studied lipoprotein has been low density lipoprotein cholesterol. However, the smaller, denser, low density lipoprotein cholesterol particles, the nonhigh density lipoprotein cholesterol fraction, appear to be more atherogenic and a more sensitive cardiovascular risk marker. Studies have shown that in children, atherogenic lipids have also been linked to cardiovascular morphological changes, such as the common carotid intima-media thickness and the ventricular geometry, both independent cardiovascular risk markers. In infancy, atherosclerosis is a preclinical disorder in which dyslipidemia plays a crucial role. Due to its impact on cardiovascular structures, potentially reversible during childhood, dyslipidemia ought to be managed aggressively to prevent further disease progression that will ultimately culminate in cardiac disease, a leading cause of mortality in adults.

Evaluation of Cardiovascular Changes in Children with BAVs.

The objectives of this study were to investigate left ventricular (LV) function, aortic dilation, and atherosclerosis in children with mildly deteriorated isolated bicuspid aortic valve (BAV) function using echocardiographic studies and biochemical markers of atherosclerosis and to correlate results with normal children. Biochemical analyses indicating cardiovascular risk of atherosclerosis and vascular changes in the aorta in relation to BAV were performed in 41 children aged 5-15 years old with isolated BAV and in 25 children with tricuspid aortic valves. Evaluations of aortic valve structures and functions; examinations of the LV M-mode and ascending aorta Doppler; and measurements of the LV Tei index (MPI), propagation velocity, ascending aorta at four levels, and carotid intima-media thickness (CIMT) were performed. There were no statistically significant differences in CIMTs, plasma matrix metalloproteinase-9, tissue metalloproteinase inhibitor-1 levels, or other biochemical parameters indicating cardiovascular risk or atherosclerosis between study and control groups. Deterioration of LV function, which could not be seen with M-mode echocardiography, was evident by MPI. MPI values in the study versus control groups were 0.46 ± 0.080 versus 0.40 ± 0.086 (p < 0.05). Diameters of the aorta in the study and control groups were 19.7 ± 4.7 and 17.2 ± 2.8 mm (p < 0.05) at the sinotubular junction level and 20.6 (14.4-40.5) and 18.3 (12.4-24) mm at the ascending aorta level (p < 0.05). Increased aortic valve insufficiency was related to increased aortic diameter. No sign of atherosclerosis was detected in children with BAV. Deterioration of LV function was seen using MPI, and aortic dilation was related to the severity of aortic valve insufficiency.

Cardiovascular change in children with dengue shock syndrome.

To determine the cardiovascular changes in children with dengue shock syndrome. Echocardiography was performed in 8 children (5 females) with dengue shock syndrome, median age 6.5, 4.2-13.7 yr and weight 34, 12-66 kg. All had massive bleeding with low initial hematocrit in most cases (median 31%), thrombocytopenia (median platelet 37,000/μL), and coagulopathy with massive pleural effusion. Seven (87.5%) developed acute renal failure and hepatic failure. All patients were in either compensate or decompensate shock with alteration of consciousness, tachycardia, poor tissue perfusion, and prolonged capillary refill (>4 s) with mean arterial pressure 65, 39-94 mm Hg. The cardiac dimension was normal to low normal except one had dilated left ventricle. Seven patients had normal left ventricular systolic function (5 with inotrope infusion). One patient had impaired systolic function even with inotrope. All had normal cardiac index (4.14, 3.51-6.37 L/min/m2) with increased heart rate (141.5, 110-160/min) but low stroke volume index (30.72, 25.37-42.49 mL/m2) and low systemic vascular resistance index (1,072, 223-2,880 dyne/sec/cm-5/m2). Decreased preload from bleeding and vascular leakage into the third space play an important role in shock in Dengue. However, decreased stroke volume and low systemic vascular resistance may be additional causes of shock.

CARDIOVASCULAR LESIONS IN CHILDREN WITH OBESITY

The dominance of cardiovascular diseases in the structure of mortality and disability in developed countries as well as the increasing incidence of cardiovascular disorders led to development of conception of cardiovascular continuum based on the clinical evaluation of the risk factors for cardiovascular complications. In this regard studying of the initial stages of this process, i. e. childhood and adolescence, is of great interest. It is now apparent that childhood obesity may be regarded as a cardiovascular risk factor in adulthood. This fact also corresponds to the accumulated information on the cardiovascular disease in children and adolescents with obesity and its similarity with the changes found in adults. What also facilitates the importance of this issue is a steady worldwide increase of the percentage of infants with excess body weight, which may lead to possible increase in cardiovascular disease in the near future. The review covers early manifestations of cardiovascular disease in children with obesity, the development of endothelial dysfunction, remodeling of the artery wall and the progression of atherosclerotic lesions in the aorta and peripheral vessels. The review addresses the distinction of the hypertension development and course as well as the indication of cardiac remodeling and diagnostic criteria of left ventricular hypertrophy. The review provides directions in prevention and treatment of cardiovascular changes in children and adolescents with obesity.

Cardiovascular changes in children with coarctation of the aorta treated by endovascular stenting

The aim of this study was to investigate the echocardiographic, biochemical short- and mid-term effects of the stenting procedure on left ventricular function, aortic stiffness, elasticity and systemic hypertension in children with coarctation of the aorta (CoA). Fifteen patients with native or recurrent CoA and 30 healthy controls who were sex and age matched were included in the study. The blood pressure values, echocardiographic measurements, elastic functions of ascending aorta and serum N-Terminal ProBNP (NT-ProBNP) levels were recorded prospectively before and at the first and sixth month after stenting. The mean arterial pressure recorded before stenting was 134.4±16.3 mm Hg; at the sixth month it was 115.5±9.5 mm Hg and in the control group it was 107.3±9.4 mm Hg. Although blood pressure levels were lower compared with the pre-stenting measurements (P<0.05), they were still significantly higher compared with the control group (P<0.05). Although a significant reduction was detected in the LVMIz at the end of the sixth month (50.4±14.3 g m(-2.7)) compared with the baseline (66.6±17.9 g m(-2.7); P<0.05), it was still higher compared with the control group (35.7±6.2 g m(-2.7); P<0.05). The baseline aortic elasticity (6.4±3.4 cm(2) dyn(-1) 10(-6)) was lower compared with the control group (10.0±1.7 cm(2) dyn(-1) 10(-6); P<0.05), and prestenting aortic stiffness was higher than that of the control group (5.6±1.6 dyn(-1) 10(-6); 2.5±0.45 dyn(-1) 10(-6); P<0.05). A statistically significant negative correlation was detected between the pressure gradient at the lesion site and aortic elasticity (r: -0.53, P: 0.04). Although resolution of the coarctation by endovascular stenting led to a reduction in the arteriopathy that had already begun before treatment, it was demonstrated that these children did not completely return to normal.

Cardiovascular Changes in Children with Snoring and Obstructive Sleep Apnoea

Adults with obstructive sleep apnoea (OSA) are well documented to be at high risk for cardiovascular abnormalities. Growing evidence suggests that OSA is also associated with cardiovascular consequences in children. The purpose of this review is to examine the available data on this association in children. Primary studies were extracted from a MEDLINE search limited to those published between 1970 and 2008. The keywords used included child, sleep disordered breathing, sleep apnoea, snoring, blood pressure and hearts. The relevant articles were selected by consensus between 2 authors. The results suggested that OSA was consistently associated with hypertension. Meta-analysis of risk of hypertension in those with high apnoea-hypopnoea index was undertaken. A combined odds ratio equal to 3.15 was found (95% confidence interval, 2.01 to 4.93). There was evidence for increased sympathetic activation, decreased arterial distensibility and ventricular hypertrophy in children with OSA. Childhood OSA is associated with blood pressure dysregulation. The association of OSA with other cardiovascular morbidities requires further study in view of the limited data available currently.

Cardiovascular changes in children with mucopolysaccharide disorders

The aims of this study were to evaluate cardiac involvement, assess risk factors and mortality, and define the outcome of cardiac abnormalities with age in the different types of mucopolysaccharidoses (MPS). The echocardiograms of 99 patients with MPS, aged 1–24 y (median age 10.3 y) were reviewed between 1978 and 2000. Mitral regurgitation (MR) was detected in 29 patients (29%). MR was more frequent in types IH [n= 11 (38%)], II [n= 10 (24%)] and III [n= 4 (20%)]. Sixteen patients (16%) developed aortic regurgitation (AR), seen mostly in types II [n = 9 (56%)] and IV [n= 4 (24%)]. AR and/or MR was detected in 37 patients and 8 had both abnormalities of borderline significance (odds ratio 2.95,95% confidence interval 1.0–8.85, p= 0.05). Of 99 patients, 47 had a normal study on their first echocardiogram, whereas only 7 had a normal study on subsequent echocardiograms. Fifty‐four (54%) had a single echocardiogram. Of these, 27 (50%) were abnormal and 27 normal. Forty‐five patients had more than one echocardiogram, of which 25 (56%) were abnormal and 20 normal. In 13/20 (65%) a cardiac abnormality developed on a subsequent echocardiogram which was statistically significant (p= 0.002). Overall mitral and aortic valve abnormalities showed a positive association with age. Univariate analysis of risk factors showed that increasing age, MPS I and ejection fraction were significant risk factors for death. However, left ventricular hypertrophy, mitral valve abnormalities and type II MPS were not significant risk factors for death, with borderline significance for aortic valve abnormalities. Conclusion: This study demonstrates the evaluation of ventricular function, which is a significant risk factor for death, along with increasing age and MPS I, and outlines the borderline significance of aortic valve abnormalities, which has not been mentioned in previous studies. It also shows that mitral valve lesions, commonly seen in MPS, were not a significant risk factor for death. The results emphasize the importance of performing serial echocardiograms in patients with MPS to assess ventricular function and the progression of cardiac abnormalities with age.

Cardiovascular changes in children with mucopolysaccharide disorders.

The aims of this study were to evaluate cardiac involvement, assess risk factors and mortality, and define the outcome of cardiac abnormalities with age in the different types of mucopolysaccharidoses (MPS). The echocardiograms of 99 patients with MPS, aged 1-24 y (median age 10.3 y) were reviewed between 1978 and 2000. Mitral regurgitation (MR) was detected in 29 patients (29%). MR was more frequent in types IH [n = 11 (38%)], II [n = 10 (24%)] and III [n = 4 (20%)]. Sixteen patients (16%) developed aortic regurgitation (AR), seen mostly in types II [n = 9 (56%)] and IV [n = 4 (24%)]. AR and/or MR was detected in 37 patients and 8 had both abnormalities of borderline significance (odds ratio 2.95, 95% confidence interval 1.0-8.85, p = 0.05). Of 99 patients, 47 had a normal study on their first echocardiogram, whereas only 7 had a normal study on subsequent echocardiograms. Fifty-four (54%) had a single echocardiogram. Of these, 27 (50%) were abnormal and 27 normal. Forty-five patients had more than one echocardiogram, of which 25 (56%) were abnormal and 20 normal. In 13/20 (65%) a cardiac abnormality developed on a subsequent echocardiogram which was statistically significant (p = 0.002). Overall mitral and aortic valve abnormalities showed a positive association with age. Univariate analysis of risk factors showed that increasing age, MPS I and ejection fraction were significant risk factors for death. However, left ventricular hypertrophy, mitral valve abnormalities and type II MPS were not significant risk factors for death, with borderline significance for aortic valve abnormalities. This study demonstrates the evaluation of ventricular function, which is a significant risk factor for death, along with increasing age and MPS I, and outlines the borderline significance of aortic valve abnormalities, which has not been mentioned in previous studies. It also shows that mitral valve lesions, commonly seen in MPS, were not a significant risk factor for death. The results emphasize the importance of performing serial echocardiograms in patients with MPS to assess ventricular function and the progression of cardiac abnormalities with age.

Cardiovascular changes in children with mucopolysaccharide storage diseases and related disorders--clinical and echocardiographic findings in 64 patients.

Cardiovascular abnormalities were evaluated in 64 children aged between 1 year 9 months and 25 years with mucopolysaccharidoses (MPS) and related disorders. A heart murmur was heard in 18 patients, but in only 6 was it characteristic for specific valvular lesions. Echocardiography was performed in 63 children. In one girl cardiac lesions were diagnosed on autopsy. In 46 patients (72%), valvular lesions and/or different types of cardiomyopathy were detected. There were no characteristic changes for different types of MPS. In the majority of children in whom dermatan sulphate accumulated, cardiac involvement was the most frequent (88%) and severe. The most common lesion, regardless of MPS type, was thickening of the mitral valve (66%), with regurgitation or stenosis in 28 (44%). Aortic valve thickening was detected in 17 patients (27%), asymmetric septal hypertrophy or hypertrophic cardiomyopathy in 18, congestive cardiomyopathy in 1 and endocardial thickening in 13 patients. Cardiac involvement was less frequent in children with Sanfilippo disease. Two or more echocardiographic examinations were performed in 23 patients. In 19 of them (83%) cardiac changes were more severe during the second examination. One 7-year-old boy with Hunter disease underwent successful mitral valve replacement. CONCLUSIONS; Cardiac involvement is present in most patients with MPS although there are few clinical signs and symptoms. The most common and severe changes are in Hurler, Hunter, Maroteaux-Lamy and I-cell disease, rarely in Sanfilippo disease. Mitral valve deformation is most frequent in all patients. The cardiac lesions are progressive.