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Die vorliegende Arbeit untersucht, inwieweit sich minimalinvasive Zugangswege gegenüber der medianen Sternotomie bei isolierten Aortenklappeneingriffen positiv auf die postoperative Schultergelenks- und Schultergürtelmobilität auswirken. Die Beurteilung erfolgt dabei postoperativ primär durch Anwendung des Constant-Murley-Score (CMS). Weiterhin werden der Disabilities of the Arm, Shoulder and Hand Fragebogen (DASH) sowie Untersuchungen von Gelenkbeweglichkeiten und Muskelfunktion (Verlängerbarkeit und Kraft) durchgeführt. Bis dato existiert kein gesondertes Testinstrument zur Beurteilung der funktionellen Einheit Schultergürtel / Schultergelenk in der Herzchirurgie. Existierende Untersuchungen deuten jedoch darauf hin, dass größere, insbesondere herzchirurgische Eingriffe sowohl lokal als auch systemisch Einfluss auf die Funktionalität von Muskulatur haben. Sei es durch direkte Affektion oder aufgrund postoperativer Immobilisation. Für den DASH gibt es ebenfalls bereits veröffentlichte Ergebnisse, die eine Verschlechterung der Funktion der oberen Extremität nach einer Herz-OP zeigen. Bei Betrachtung der Ergebnisse des CMS in dieser Arbeit haben die Patienten nach minimalinvasivem Aortenklappenersatz sowohl rechts (78,9±10,4 minimalinvasiv vs. 60,8±8,6 konventionell, p<0,01) als auch links (80,0±8,0 minimalinvasiv vs. 61,8±5,6 konventionell, p<0,01) eine signifikant bessere Schulterfunktion als nach medianer Sternotomie. Auch die Ergebnisse des DASH zeigen nach minimalinvasiver Operation eine signifikant bessere Funktion der oberen Extremität (40,9±13,6 minimalinvasiv vs. 62,5±22,1 konventionell, p=0,02). Die Beweglichkeitsuntersuchungen lassen bessere postoperative Ergebnisse bei den minimalinvasiv Operierten, insbesondere für nach vorne gerichteten Bewegungen, wie der Protraktion links (20±2,9° minimalinvasiv vs. 13±5,7° konventionell, p<0,01) und der horizontalen Adduktion rechts (115±9,8° minimalinvasiv vs. 103±9,7° konventionell, p=0,03), erkennen. Muskelverlängerbarkeit und -kraft hingegen offenbaren in diesem Setting keine signifikanten Unterschiede zwischen minimalinvasiver und konventioneller Gruppe. Ein Grund dafür kann unter anderem das lange Untersuchungsintervall postoperativ sein. Aufgrund der kleinen Anzahl eingeschlossener Patienten ist die statistische Aussagekraft dieser Arbeit eingeschränkt. Insgesamt wird jedoch die Annahme unterstützt, dass minimalinvasive Verfahren in der Aortenklappenchirurgie eine bessere Funktion der Schulter postoperativ ermöglichen, als dies durch die mediane Sternotomie der Fall ist. Diese bessere Funktion lässt sich durch etablierte Instrumente wie den Constant-Murley-Score sowie den DASH-Fragebogen darstellen.

Leaflet replacement in aortic valve repair (AVr) is associated with increased long-term repair failure. Hemodynamic performance and mechanical stress levels were investigated after porcine AVr with 5 types of clinically relevant replacement materials to ascertain which material(s) would be best suited for repair. Porcine aortic roots with intact aortic valves were placed in a left-heart simulator mounted with a high-speed camera for baseline valve assessment. Then, the non-coronary leaflet was excised and replaced with autologous porcine pericardium (APP), glutaraldehyde-fixed bovine pericardial patch (BPP; Synovis™), extracellular matrix scaffold (CorMatrix™), or collagen-impregnated Dacron (HEMASHIELD™). Hemodynamic parameters were measured over a range of cardiac outputs (2.5–6.5L/min) post-repair. Material properties of the above materials along with St. Jude Medical™ Pericardial Patch with EnCapTM Technology (SJM) were determined using pressurization experiments. Finite element models of the aortic valve and root complex were then constructed to verify the hemodynamic characteristics and determine leaflet stress levels. This study demonstrates that APP and SJM have the closest profiles to normal aortic valves; therefore, use of either replacement material may be best suited. Increased stresses found in BPP, HEMASHIELD™, and CorMatrix™ groups may be associated with late repair failure.

Leaflet replacement in aortic valve repair (AVr) is associated with increased long-term repair failure. Hemodynamic performance and mechanical stress levels were investigated after porcine AVr with 5 types of clinically relevant replacement materials to ascertain which material(s) would be best suited for repair. Porcine aortic roots with intact aortic valves were placed in a left-heart simulator mounted with a high-speed camera for baseline valve assessment. Then, the non-coronary leaflet was excised and replaced with autologous porcine pericardium (APP), glutaraldehyde-fixed bovine pericardial patch (BPP; Synovis™), extracellular matrix scaffold (CorMatrix™), or collagen-impregnated Dacron (HEMASHIELD™). Hemodynamic parameters were measured over a range of cardiac outputs (2.5–6.5L/min) post-repair. Material properties of the above materials along with St. Jude Medical™ Pericardial Patch with EnCapTM Technology (SJM) were determined using pressurization experiments. Finite element models of the aortic valve and root complex were then constructed to verify the hemodynamic characteristics and determine leaflet stress levels. This study demonstrates that APP and SJM have the closest profiles to normal aortic valves; therefore, use of either replacement material may be best suited. Increased stresses found in BPP, HEMASHIELD™, and CorMatrix™ groups may be associated with late repair failure.

Thesis (MScEng)--Stellenbosch University, 2012. / ENGLISH ABSTRACT: Bioprosthetic heart valves (BHVs) for transcatheter aortic valve implantation (TAVI) have been rapidly developing over the last decade since the first valve replacement using the TAVI technique. TAVI is a minimally invasive valve replacement procedure offering lifesaving treatment to patients who are denied open heart surgery. The biomedical engineering research group at Stellenbosch University designed a 19 mm balloon expandable BHV for TAVI in 2007/8 for testing in animal trials. In the current study the valve was enlarged to 23 mm and 26 mm diameters. A finite element analysis was performed to aid in the design of the stents. New stencils were designed and manufactured for the leaflets using Thubrikar‟s equations as a guide. The 23 mm valve was manufactured and successfully implanted into two sheep. Fluid structure interaction (FSI) simulations constitute a large portion of this thesis and are being recognized as an important tool in the design of BHVs. Furthermore, they provide insight into the interaction of the blood with the valve, the leaflet dynamics and valve hemodynamic performance. The complex material properties, pulsating flow, large deformations and coupling of the fluid and the physical structure make this one of the most complicated and difficult research areas within the body. The FSI simulations, of the current valve design, were performed using a commercial programme called MSC.Dytran. A validation study was performed using data collected from a cardiac pulse duplicator. The FSI model was validated using leaflet dynamics visualisation and transvalvular pressure gradient comparison. Further comparison studies were performed to determine the material model to be used and the effect of leaflet free edge length and valve diameter on valve performance. The results from the validation study correlated well, considering the limitations that were experienced. However, further research is required to achieve a thorough validation. The comparative studies indicated that the linear isotropic material model was the most stable material model which could be used to simulate the leaflet behaviour. The free edge length of the leaflet affects the leaflet dynamics but does not greatly hinder its performance. The hemodynamic performance of the valve improves with an increase in diameter and the leaflet dynamics perform well considering the increased surface area and length. Many limitations in the software prevented more accurate material models and flow initiation to be implemented. These limitations significantly restricted the research and confidence in the results. Further investigation regarding the implementation of FSI simulations of a heart valve using the commercial software is recommended. / AFRIKAANSE OPSOMMING: Bio-prostetiese hartkleppe (Bioprosthetic Heart Valves - BHVs) wat gebruik word vir transkateter aortaklep-inplantings (Transcatheter Aortic Valve Implantation - TAVI) het geweldig vinnige ontwikkeling getoon in die afgelope tien jaar sedert die eerste klepvervanging wat van die TAVI prosedure gebruik gemaak het. TAVI is ʼn minimaal indringende klepvervangingsprosedure wat lewensreddende behandeling bied aan pasiënte wat ope-hart sjirurgie geweier word. Die Biomediese Ingenieurswese Navorsingsgroep (BERG) by Stellenbosch Universiteit het in 2007/8 ʼn 19 mm ballon-uitsetbare BHV vir TAVI ontwerp vir eksperimente met diere, en hierdie tesis volg op die vorige projekte. In die huidige studie is die klep vergroot na 23 mm en 26 mm in deursnee. ʼn Eindige element analise is gedoen om by te dra tot die ontwerp van die rekspalke vir die klep. Nuwe stensils is ontwerp en vervaardig vir die klepsuile, deur gebruik te maak van Thubrikar se vergelykings. Die 23 mm klep is vervaardig en suksesvol in twee skape ingeplant. Vloeistruktuur interaksie (Fluid Structure Interaction (FSI)) simulasies vorm ‟n groot deel van die tesis en word gesien as ʼn noodsaaklike hulpmiddel in die ontwerp van BHVs. Die simulasies verskaf ook insig in die interaksie van die bloed met die klep, die klepsuil-dinamika en die klep se hemodinamiese werkverrigting. Die komplekse materiaal eienskappe, polsende vloei, grootskaalse vervorming, die verbinding van die vloeistof en die fisiese struktuur maak van hierdie een van die mees gekompliseerde voorwerpe om te simuleer. Die FSI simulasies van die huidige ontwerp, is uitgevoer deur van kommersiële sagteware, MSC.Dytran, gebruik te maak. ʼn Geldigheidstudie wat data gebruik het vanaf die hartklop-nabootser, is uitgevoer. Die FSI model word geverifieer deur klepsuil dinamika visualisering en ʼn vergelyking van die drukgradiënt gebruik te maak. Verdere vergelykende studies is uitgevoer om te bepaal watter materiaal model om te gebruik, asook die uitwerking van die klepsuil-vrye rand en klepdeursnee op die klep se werkverrigting. Die resultate van die studie korreleer goed, in ag genome die beperkings wat ervaar is. Verdere navorsing is egter nodig vir ʼn volledige geldigheidstudie. Vergelykende studies het getoon dat die liniêre isotropiese materiaalmodel die meer stabiele materiaalmodel is wat kan gebruik word om klepsuilgedrag te simuleer. Die vrye-rand lengte van die klepsuil affekteer die dinamika van die klepsuil, maar belemmer nie die werkverrigting grootliks nie. Die hemodinamiese werkverrigting van die klep verbeter met die toename in deursnee en die klepsuil-dinamika vertoon goed in ag genome die verhoogde oppervlak area en lengte. Die vele beperkings in die sagteware het die implementering van meer akkurate materiaalmodelle verhoed. Hierdie beperkings het ʼn verminderde vertroue in die resultate tot gevolg gehad. Verdere ondersoek rakende die implementering van die FSI simulasies van ʼn hartklep deur kommersieel beskikbare sagteware te gebruik, word aanbevel.

La sténose valvulaire aortique (SVA) est une pathologie associée au vieillissement et aux facteurs de risque cardiovasculaire. Afin d’étudier la SVA et d’explorer de nouvelles thérapies, plusieurs modèles animaux ont été récemment développés, mais la plupart de ces modèles ciblent les mécanismes de développement de la SVA reliés à l’hypercholestérolémie. Le syndrome de Werner (WS) est une maladie caractérisée par un vieillissement prématuré. Récemment, il a été découvert que des souris mutantes ayant une délétion du domaine hélicase du gène Werner, responsable du WS, démontraient un profile hémodynamique typique de la SVA. De ce fait, nous avons émis l’hypothèse que ces souris pourraient développer une SVA plus rapidement que des souris de type sauvage. Nous avons donc étudié les effets cette mutation chez des souris WrnΔhel/Δhel, en comparant le taux de progression d’une SVA entre des souris WrnΔhel/Δhel (WrnΔhel) et des souris de type sauvage comme groupe contrôle. À la suite d’une diète riche en sucre et en gras sur une période de 24 semaines, les souris WrnΔhel ont démontré une diminution plus prononcée de leur aire de valve aortique (mesures échocardiographiques) que les souris contrôles, supportée par les analyses histologiques concernant la fibrose des valves aortiques. Les souris n’ont toutefois développé aucun signe évident d’athérosclérose comme l’infiltration de lipides ou l’inflammation, bien que certaines caractéristiques liées à la dysfonction endothéliale semblent être augmentées chez les souris WrnΔhel. D’autres mesures échocardiographiques indiquant une SVA, comme une hypertrophie du ventricule gauche dans le groupe WrnΔhel, ont été obtenues. Nous avons aussi observé des indices de vieillissement plus marqués quant aux analyses sanguines et de la moelle osseuse des souris WrnΔhel en comparaison avec les souris contrôles. Par conséquent, ce modèle expérimental de vieillissement pourrait être utilisé pour les études futures sur la SVA sans les principaux effets athérogéniques des autres modèles expérimentaux. / Aortic valve stenosis (AVS) is associated with aging and classical cardiovascular risk factors. Different animal models were recently developed to study AVS and explore new therapies, however, most of these models rely almost exclusively on hypercholesterolemia-related mechanisms for AVS development. Werner syndrome (WS) is a disorder characterized by premature aging. It was recently demonstrated that mutant mice with a deletion of the helicase domain of the Werner gene, the gene responsible for WS, showed hemodynamic profile typical of AVS. We therefore hypothesized that mice with the WrnΔhel deletion could develop AVS earlier than wild-type (WT) mice. We studied the effect of the WrnΔhel mutation by comparing the rate of progression of AVS in homozygous mutant versus WT mice. By twenty-four weeks on a high-fat/high-carbohydrate diet, WrnΔhel/Δhel (WrnΔhel) mice showed a stronger decrease of the aortic valve area measured by serial echocardiography than WT mice, supported by histological analyses of valve fibrosis but without developing major signs of atherosclerosis such as lipid infiltration or increased inflammation. Some features linked to endothelial dysfunction also appeared to be increased in WrnΔhel mice. Other echocardiographic measurements were typical of AVS, such as left ventricle hypertrophy in the WrnΔhel group. We also observed stronger aging properties from WrnΔhel mice bone marrow and blood analyses compared to the WT group. Consequently, this experimental aging model could be used for AVS research without the major confounding atherogenic effects of other experimental models.

(v AJ) Introduction to the topic and importance of the topic: Aortic valve disease is the third most frequent cardiovascular disease and also the most frequently operated valvular disease in adulthood. The natural process of the disease is unfavorable, almost half of the patients suffer from symptoms of heart failure (Branny et al. 2012). This is the reason why I focused my thesis on the assessment of the quality of life of patients diagnosed of aortic stenosis. Aim of the thesis and research questions: The aim of my thesis is to evaluate the quality of life patients with diagnosed aortic valve disease, within one month before the planned surgery and in the postoperative period when patients will be checked at a cardiac surgery clinic. The partial objective at the same time was to compare the quality of life and health status of patients with aortic valve disease who have completed a spa therapy with those who have rejected it. Methodology (method, research sample): The research work will be carried out as a quantitative research in a group of 29 respondents. The HRQoL construct it means questionnaire SF-36 were used to obtain the data. The questionnaire was anonymous. For the completed questionnaires, a closed box was set up, which I didn't open until the poll was complete. The survey was...

Introdução: O papel do sistema nervoso simpático (SNS) na remodelação ventricular esquerda na insuficiência aórtica crônica (IAo) é pouco conhecido. O aumento da atividade do SNS tem associação com remodelamento ventricular e mau prognóstico na insuficiência cardíaca (IC) não valvar, fazendo do seu bloqueio farmacológico importante conduta terapêutica. A despeito de similaridades na evolução clínica da IAo com IC não valvar, não há estudos com mensuração direta da atividade do SNS em IAo. Objetivo: Quantificar a atividade nervosa simpática muscular (ANSM) em pacientes com IAo importante em três situações clínicas, representativas da história natural dessa doença. Métodos: Trata-se de estudo transversal, unicêntrico, incluindo 30 pacientes com IAo importante que foram alocados em três grupos: (I) assintomáticos (n = 10, 70% homens, 37,4 ± 13,6 anos), (II) sintomáticos em pré-operatório de troca de valva aórtica (TVA) (n = 10, 70% homens, 42,2 ± 12,1 anos) e (III) - pós-operatório de TVA (n = 10, 80% homens, 41,2 ± 15,4 anos). Grupo controle formado por voluntários saudáveis sem doença cardíaca estrutural (n = 10) correspondentes para idade, sexo e IMC também foram avaliados. Variáveis clínicas, ecocardiográficas e BNP (peptídeo natriurético atrial) foram analisadas nos grupos. Apenas a pressão arterial sistólica era significativamente menor no grupo III. A ANSM foi mensurada utilizando a técnica padrão de microneurografia muscular (MM). A variável desfecho foi a Resumo média do número de espículas obtidas num registro contínuo de 10 minutos de MM. Resultados: Os grupos IAo não diferiram em relação às características demográficas, antropométricas e ecocardiográficas, assim como etiologia e BNP. A média de espículas obtidas pela MM, representativa da ANSM, nos grupos I, II, III e controle foi, respectivamente, 25,5 ± 4,1, 25,1 ± 3,6, 28,6 ± 6,5 e 15,6 ± 1,5 (p=0,001). Houve apenas diferença estatística entre os grupos IAo e o grupo controle. Conclusão: Houve aumento significativo da ANSM em pacientes com IAo importante associado ao remodelamento ventricular esquerdo em relação a indivíduos sem doença cardíaca estrutural. A ANSM foi similar em pacientes com IAo importante assintomáticos, sintomáticos e em pós-operatório de TVA. A participação da ação do SNS na IAo deve estar associada ao remodelamento ventricular, mas sem correlação com mudanças clínicas / Introduction: The role of sympathetic nervous system (SNS) in the left ventricle remodeling of severe aortic regurgitation (AR) remains poorly understood. The increase in SNS activity is associated with ventricular remodeling and poor prognosis in non-valvular heart failure (HF), making its pharmacological blockade an important therapeutic approach. Despite similarities in the clinical evolution of AR with non-valvular HF, there are no studies with direct measurement of SNS activity in AR. Aims: The present study aimed to quantify muscular sympathetic nervous activity (MSNA) in patients with severe AR in three clinical situations: asymptomatic, symptomatic before aortic valve replacement (AVR), and patients submitted to AVR. Methods: Thirty patients with severe AR were allocated to three groups: (I) asymptomatic patients (n=10, 70% men, age: 37.4 ± 13.6), (II) symptomatic patients before AVR (n=10, 70% men, age: 42.2 ± 12.1), and (III) patients submitted to AVR (n=10, 80% men, age: 41.2 ± 15.4). Healthy volunteers (n = 10) matched for age, sex, and BMI were also assessed. The AR groups did not differ in relation to etiology, demographic, anthropometric or echocardiographic data. Only systolic blood pressure was significantly lower in group III. MSNA was recorded using microneurography, with a spike per minute result. Results: The means of 10-minute recordings in groups I, II, III and control were 23.2 ± 6.4, 25.5 ± 4.1, 25.1 ± 3.6 and 15.6 ± 1.5, respectively (p=0.001). Only the AR and control groups differed from each other. Conclusions: AR is associated with relatively higher SNS activity, which is similar across different stages of the disease (asymptomatic, symptomatic and postoperative). The role of the SNS in AR must be associated with ventricular remodeling, but without correlation with clinical change

Introdução: as mucopolissacaridoses (MPSs) são doenças lisossômicas de depósito, caracterizadas pela degradação enzimática deficiente dos glicosaminoglicanos (GAGs): ácido hialurônico, condroitin sulfato, dermatan sulfato, heparan sulfato e queratan sulfato. A classificação baseia-se na enzima comprometida, tendo sido descritos sete tipos com manifestações clínicas heterogêneas: MPS tipo I, II, III, IV, VI, VII e IX. O comprometimento cardiovascular é variável, porém a falência cardiopulmonar contribui significativamente para a morbidade e mortalidade. Lesões valvares esquerdas e a hipertrofia do ventrículo esquerdo são os achados mais citados, ainda que não haja concordância quanto à relação entre o comprometimento cardíaco e o tipo de MPS. Especula-se que o acometimento é mais grave em pacientes cujo defeito enzimático traz acúmulo do dermatan sulfato (MPS tipos I, II, VI e VII), visto que esse GAG predomina naturalmente em válvulas e vasos sanguíneos. Frente à perspectiva de tratamento específico dessas patologias através de reposição enzimática, torna-se fundamental conhecer o comprometimento cardiovascular inicial, para determinar com segurança o impacto destas terapêuticas sobre as crianças a elas submetidas. O propósito deste estudo foi caracterizar as alterações ecocardiográficas de pacientes pediátricos com MPSs, além de testar a associação entre o acúmulo de dermatan sulfato e a gravidade das lesões cardiovasculares. Métodos: foram analisados retrospectivamente os prontuários e os ecocardiogramas de 28 pacientes (15M: 13F) entre 2 e 14 anos (9 ± 3 anos), acompanhados no Ambulatório de Genética do Instituto da Criança de setembro de 2003 a novembro de 2005: 6 com MPS tipo I, 2 com tipo II, 7 com tipo III, 6 com tipo IV, 5 com tipo VI e 2 com tipo VII. No período estudado nenhum paciente realizava terapia de reposição enzimática. Um único ecocardiografista executou 53 avaliações, visto que 17 indivíduos submeteram-se a múltiplos exames, com intervalo de 10,3 ± 5,6 meses. Todos os ecocardiogramas foram realizados segundo as normas da Sociedade Americana de Ecocardiografia. Os pacientes foram analisados quanto aos aspectos clínicos e parâmetros xvi ecocardiográficos, sendo realizada em seguida a comparação entre o grupo que acumula (D+) e o que não acumula dermatan sulfato (D-). O grupo D+ incluiu os tipos I, II, VI e VII e o grupo D-, os tipos III e IV. O programa estatístico utilizado foi o Statistical Package for Social Sciency e os testes aplicados foram o Exato de Fisher e o de Correlação de Spearman, com um valor de p significativo 0,05. Resultados: 26 (93%) pacientes exibiram alterações ecocardiográficas ao exame final. No entanto, em apenas 16 (57%) havia registro de ausculta anormal e em 6 (21%) alguma queixa cardiovascular. Hipertrofia de septo e de parede posterior foram detectadas em 12 pacientes (43%) e em 5 (18%) ocorreu hipertrofia septal isolada. Somente 2 (7%) apresentaram dilatação ventricular. Em 22 casos foi possível avaliar a função diastólica de ventrículo esquerdo. Destes, 6 (27%) apresentaram disfunção de grau leve. Todos apresentaram função sistólica preservada. Detectou-se hipertensão pulmonar em 10 pacientes (36%). Quatro foram admitidos à Unidade de Terapia Intensiva e dois evoluíram a óbito, todos por agravamento de hipertensão pulmonar. Valva mitral normal foi o achado em 5 (17,8%) e espessamento sem disfunção em 6 (21,4%). Espessamento valvar com disfunção ocorreu em 17 pacientes (60,8%): 12 (42,8%) com insuficiência, 2 (7,2%) com estenose e 3 (10,8%) com dupla lesão. A valva aórtica foi normal em 5 (17,8%) e espessada sem disfunção em 13 (46,4%). Espessamento com disfunção ocorreu em 10 pacientes (35,8%): todos com insuficiência de grau leve ou moderado. Verificou-se forte associação entre o acúmulo de dermatan sulfato e a presença de: disfunção valvar mitral (p = 0,0003), disfunção valvar aórtica (p = 0,006) e hipertensão pulmonar (p = 0,006). Entre os 17 indivíduos com múltiplos exames, 14 (82%) mostraram piora ecocardiográfica justificada por: surgimento (4/14) ou agravamento (6/14) de lesões valvares, surgimento (5/14) ou progressão (6/14) da hipertrofia ventricular, desenvolvimento de disfunção diastólica (1/14) e de hipertensão pulmonar (4/14). Conclusões: as alterações ecocardiográficas em pacientes pediátricos com mucopolissacaridoses são freqüentes e têm caráter progressivo, enquanto os sinais e sintomas são escassos. Lesões valvares esquerdas, hipertrofia ventricular e hipertensão pulmonar foram os achados mais comuns, havendo associação significativa entre o acúmulo de dermatan sulfato e o comprometimento cardiovascular. Diferentemente do que é descrito em adultos, a hipertensão pulmonar foi a causa mais importante de óbito e não a disfunção sistólica de ventrículo esquerdo. / Introduction: mucopolysaccharidosis (MPSs) are lysosomal storage diseases, characterized by deficient enzymatic degradation of glycosaminoglycanes (GAGs): hyaluronic acid, chondroitin sulfate, dermatan sulfate, heparan sulfate and keratan sulfate. The classification is based on the defective enzyme and seven types with heterogeneous clinical manifestations have been described: MPS type I, II, III, IV, VI, VII and IX. The cardiovascular involvement is variable, but the cardiopulmonary failure contributes significantly towards the morbidity and mortality. Left valve lesions and left ventricle hypertrophy are the most commented findings, although there is still no agreement about the relationship between the heart involvement and the type of MPS. It is speculated that the lesions are more severe in patients whose enzymatic defect lead to the accumulation of dermatan sulfate (MPS types I, II, VI and VII), because this GAG prevails naturally in valves and blood vessels. Due to the perspective of specific treatment for the pathology through enzymatic replacement, it is essential to know the initial cardiovascular abnormalities to determine the impact of this therapeutics on pediatric patient. The purpose of this study was to characterize the echocardiographic alterations of the pediatric patients with MPSs, besides testing the association between the accumulation of dermatan sulfate and the severity of the cardiovascular lesions. Methods: the medical records and echocardiograms of 28 patients (15M: 13F) aged 2 to 14 (9 ± 3 years), seen at the Genetic Clinic between September 2003 and November 2005, were retrospectively analyzed: 6 with MPS type I, 2 with type II, 6 with type III, 7 with type IV, 5 with type VI and 2 with type VII. During the period of study no patient had enzymatic replacement. A single pediatric cardiologist executed 53 echocardiograms, since 17 individuals underwent multiple exams, with an interval of 10.3 ± 5.6 months. All the echocardiograms were performed according to the recommendations of the American Society of Echocardiography. Patients were analyzed according to both clinical and echocardiographic parameters, and then a comparison was made among the group that accumulates (D+) and the one that does not xviii accumulate dermatan sulfate (D-). The group D+ included the types I, II, VI and VII and the group D included types III and IV. The statistical program used was the Statistical Package for Social Science and the applied tests were the Fisher\'s exact test and the Spearman correlation, where a p-value < 0.05 was considered significant. Results: echocardiographic alterations were detected in 26 patients (93%), whereas 16 (57%) had abnormal auscultation, and only 6 (21%) presented cardiovascular complaint. Septum and posterior wall hypertrophy were diagnosed in 12 patients (43%) and five (18%) showed signs of isolated septal hypertrophy. Only 2 (7%) presented ventricular dilation. In 22 patients it was possible to evaluate the diastolic function of the left ventricle. Of these, 6 presented mild dysfunction. However, all patients had preserved systolic function. Pulmonary hypertension was detected in 10 patients (36%). 4 patients were admitted in the Intensive Care Unit and 2 died, due to aggravation of pulmonary hypertension. Normal mitral valve was found in 5 (17.8%) and thickening without dysfunction in 6 cases (21.4%). Valve thickening with dysfunction occurred in 17 (60.8%): 12 (42.8%) with regurgitation, 2 (7.2%) with stenosis and 3 (10.8%) with double lesion. The aortic valve was normal in 5 (17.8%) and thickened without dysfunction in 13 cases (46.4%). Thickening with dysfunction happened in 10 patients (35.8%): all with mild or moderate aortic regurgitation. A strong association was observed between accumulation of dermatan sulfate and presence of mitral valve dysfunction (p = 0.0003), aortic valve dysfunction (p = 0.006) and pulmonary hypertension (p = 0.006). Among 17 individuals with multiple exams, 14 (82%) had a worsening evolution justified by the appearance (4/14) or aggravation (6/14) of valve lesions, appearance (5/14) or progression (6/14) of ventricular hypertrophy, development of left ventricle diastolic dysfunction (1/14) and of pulmonary hypertension (4/14). Conclusions: echocardiographic alterations in pediatric patients with Mucopolysaccharidosis are frequent and have a progressive character. Left valve lesions, ventricular hypertrophy and pulmonary hypertension were the most common findings and there was association between accumulation of dermatan sulfate and cardiovascular involvement. Unlike in adults, pulmonary hypertension was the main cause of death, not left ventricle systolic dysfunction.