ABSTRACT
Criança com 12 anos de idade, encaminhada para ecocardiografia transtorácica e transesofágica 2D e 3D para controle pós-operatório de correção de estenose subaórtica, que evidenciou um grande pseudoaneurisma da fibrosa intervalvar mitro-aórtica, associado à perfuração da base do folheto anterior da valva mitral. Tratava-se de um caso de coartação da aorta (CoAo) neonatal associada à comunicação interventricular (CIV), que necessitou de múltiplas intervenções cirúrgicas na infância devido ao não tratamento adequado das estruturas predisponentes de estenose subvalvar aórtica. Neste relato, são discutidas as particularidades anatômicas que predispõem a lesões obstrutivas esquerdas e a provável causa de desenvolvimento dessas lesões iatrogênicas.
Twelve year-old child referred for echocardiographic evaluation after surgical treatment of subaortic stenosis. Transthoracic and transesofageal (2D and real time 3D) showed a large pseudoaneurysm of the mitral-aortic intervalvular fibrosa associated with a small perforation at the base of the anterior leaflet of the mitral valve. She had neonatal coarctation of the aorta associated with ventricular septal defect and required multiple surgical procedures in infancy due to an inappropriate treatment of the predisposing anatomical structures of subvalvular aortic stenosis. In this report, the mechanisms of these iatrogenic lesions and the abnormal anatomical features that predispose to left sided obstructive lesions are discussed.
Subject(s)
Humans , Child , Aortic Coarctation/surgery , Aortic Coarctation/complications , Heart Septal Defects, Ventricular/surgery , Echocardiography/methods , Aortic Stenosis, Subvalvular/surgery , Aortic Stenosis, Subvalvular/complications , Aneurysm, False/therapyABSTRACT
El Síndrome de Williams (SW) es un síndrome genético generado por la deleción del gen de la Elastina y genes contiguos del cromosoma 7q11.23. Tiene una incidencia de 1:7500-20.000 recién nacidos vivos. Se caracteriza por un conjunto de síntomas y signos con compromiso multiorgánico y un fenotipo conductual distintivo. Objetivo: Describir la clínica del SW en relación a tres casos clínicos. Método: Estudio descriptivo retrospectivo de fichas clínicas de pacientes estudiados y tratados entre el 2006 y 2012. Resultados: Tres varones con rango de edad entre 4 y 6 años. Todos presentaron dismorfias características y se asociaron a cardiopatía congénita: estenosis aórtica supravalvular. En los rasgos de personalidad destacaron alta sociabilidad y habilidades en lenguaje expresivo, RM leve a moderado y mala coordinación motora. Conclusión: Todos nuestros pacientes presentaron características concordantes con las descritas para SW. Existen alteraciones funcionales cerebrales en pacientes con SW que tienen relación con el perfil cognitivo observado.
Williams Syndrome (WS) is a genetic disorder caused by deletion of elastine gene and contiguous genes of chromosome 7q11.2. It has an incidence of 1:7.500-20.000 newly born. It is characterized by a group of symptoms and signs with multiorganic involvement and a distinctive behavioural phenotype. Objective: To describe the clinical manifestations of WS in relation to three case reports. Method: review of clinical reports of patients diagnosed and treated between 2006 and 2012. Results: Three boys aged 5-9 years, all of them presented distinctive appearance, associated to congenital heart disease: aortic supravalvular stenosis. Behavioral features included high sociability and expressive language skills, mental retardation and poor motor coordination. Conclusions: All of our patients had clinical characteristics corresponding to the ones described for WS in the literature. The peculiar cognitive profile is presumed to be related to functional brain alterations described in WS.
Subject(s)
Humans , Male , Child , Williams Syndrome/complications , Williams Syndrome/physiopathology , Williams Syndrome/psychology , Chromosome Deletion , Cognition , Intellectual Disability/etiology , Aortic Stenosis, Subvalvular/etiology , Social Behavior , Williams Syndrome/genetics , Motor Skills Disorders/etiologySubject(s)
Humans , Heart Septal Defects, Ventricular , Heart Septal Defects, Atrial , Ductus Arteriosus, Patent , Aortic Stenosis, Subvalvular , Aortic Stenosis, Supravalvular , Aortic Coarctation , Pulmonary Valve Stenosis , Tetralogy of Fallot , Transposition of Great Vessels , Hypoplastic Left Heart Syndrome , Truncus Arteriosus , Ebstein AnomalySubject(s)
Child, Preschool , Humans , Male , Aortic Coarctation , Aortic Stenosis, Subvalvular , Heart Septal Defects, Ventricular , Mitral Valve Stenosis , Mitral Valve/abnormalities , Aortic Coarctation/complications , Aortic Stenosis, Subvalvular/complications , Diagnosis, Differential , Heart Septal Defects, Ventricular/complications , Mitral Valve Stenosis/complications , Radiography, ThoracicABSTRACT
It is a case of a 28-year-female with severe subaortic stenosis due to accesory mitral valve tissue of the type of an accesory medial valve belove the normal anterior one. The diagnosis was performed by echocardiography.
Subject(s)
Adult , Female , Humans , Aortic Stenosis, Subvalvular , Mitral Valve/abnormalities , Aortic Stenosis, Subvalvular , Mitral ValveABSTRACT
Immediate results after operative treatment of subaortic membrane have been generally good with low operative mortality and good preservation of systolic function Residual gradients after operation may be due to inadequate resection, but dynamic outflow obstruction almost contributes significantly to this finding. The hypothesis that discrete subaortic stenosis [SAS] is a dynamic, progressive disorder of the LVOT is supported serial hemodynamic and angiographic investigations. These data support the concept that in patients, who develop SAS, there may be a preexisting nidus that is stimulated by hemodynamic forces. Resection of extensive subaortic fibrous tissue may still leave behind tissue that has a propensity for forming a recurrent obstruction. To find the best surgical management of the subaortic membrane in an attempt to reach best results, minimal gradient, with least residual complications or risk of recurrence. This study is prospective trial that was conducted in the National Heart Institute in Cairo, Egypt in the period between January 2003 and January 2005, 105 patients with isolated congenital subaortic membrane in the study. All patients were evaluated preoperatively and monitored postoperatively in the intensive care unit, in the ward until discharge, 15 days, 1 month, and 6 months after surgery. All residual complications or recurrences were noted. Surgical complete resection of congenital subaortic membrane is a successful operation; with statistically significant improvement in dyspnea functional class, decrease in systolic gradient across the LV naorta, and gradual decrease in septal wall thickness. But it still carries the risk of residual LVOT restenosis and risk of recurrence. So it is preferable to do at least a septal myotomy to decrease these complications. Complete resection of congenital subaortic membrane has generally good late post operative results. It is preferable to perform at least myotomy as routine, and in cases of severe septal hypertrophy to do myomectomy [this seems to be a more aggressive approach]. But it acceptable to decrease the risk of residual or recurrence of the LVOT
Subject(s)
Humans , Male , Female , Thoracic Surgery , Echocardiography , Aortic Stenosis, Subvalvular , Postoperative Complications , Prospective StudiesABSTRACT
OBJETIVO: Estudar o comportamento clínico da estenose subaórtica associada a comunicação interventricular perimembranosa. MÉTODOS: Foram acompanhadas, de janeiro 1979 a junho 2000, quanto às características anatômicas, caráter evolutivo e eventos clínicos, 36 crianças com comunicação interventricular perimembranosa e estenose subaórtica fixa. RESULTADOS: A idade de diagnóstico da estenose subaórtica fixa variou de seis meses a 170 meses, sendo abaixo de 1 ano apenas em duas crianças. Quanto ao sexo a distribuição foi de 2:1 com grande predomínio do masculino. A comunicação interventricular era de tamanho pequeno em 61,00 por cento dos casos, médio em 30,56 por cento e grande em 8,40 por cento, apresentando diminuição do tamanho da comunicação durante o acompanhamento em 30,56 por cento (11 casos). Em todos os pacientes a estenose subaórtica era fixa, em membrana. Durante o tempo de acompanhamento, 23 pacientes apresentaram progressão da estenose. Foi realizado tratamento cirúrgico em 21 casos, sendo um paciente reoperado por reestenose. Endocardite bacteriana ocorreu em dois casos, um deles faleceu. CONCLUSÃO: A estenose subaórtica ocorre na história natural da comunicação interventricular geralmente após o 1° ano de vida, apresentando caráter progressivo e necessitando de cirurgia na maioria dos casos.
Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Infant , Male , Aortic Stenosis, Subvalvular/etiology , Heart Septal Defects, Ventricular/complications , Angiocardiography , Aortic Stenosis, Subvalvular/diagnosis , Aortic Stenosis, Subvalvular/surgery , Echocardiography , Follow-Up Studies , Heart Septal Defects, Ventricular/surgery , Severity of Illness IndexABSTRACT
BACKGROUND: Discrete subaortic stenosis is known to recur frequently even after surgical resection. We retrospectively reviewed the preoperative and postoperative changes in pressure gradient through left ventricular outflow tract, and the recurrence rate. MATERIAL AND METHOD: Between September 1984 and December 2004, 34 patients underwent surgical treatment. Mean age of patients was 17.1+/-15.2 years and 19 patients (55.9%) were male. 16 patients (47.1%) had previous operations and associated diseases were aortic regurgitation (11), coarctation of aorta (3), and others. RESULT: Immediate postoperative peak pressure gradient was significantly lower than preoperative peak pressure gradient (21.8 mmHg vs 75.8 mmHg, p<0.01). Peak pressure gradient measured after 50.3 months of follow up was 20.2 mmHg, which was also significantly lower than that of preoperative value but not significantly different from that of immediate postoperative value. There was no surgical mortality but one patient developed cerebral infarction. Mean follow up duration was 69.8+/-54.6 months. During this period, 5 patients (14.7%) had reoperation, 3 (8.8%) of whom were due to recurred subaortic stenosis. We found no risk factors for recurrence and survival for free from reoperation was 76.4%. CONCLUSION: Excision of subaortic membrane combined with or without myectomy in discrete subaortic stenosis showed sufficient relief of left ventricular outflow tract obstruction with low mortality and morbidity, but careful long term follow up is necessary for recurrence, since it is not predictable.
Subject(s)
Humans , Male , Aortic Coarctation , Aortic Stenosis, Subvalvular , Aortic Valve Insufficiency , Cerebral Infarction , Constriction, Pathologic , Discrete Subaortic Stenosis , Follow-Up Studies , Membranes , Mortality , Recurrence , Reoperation , Retrospective Studies , Risk FactorsABSTRACT
A case of subaortic membrane with coronary artery disease in a 48-year-old man is described. He was referred to our hospital for cardiac murmur, which was discovered on routine clinical examination. He had no significant past medical history apart from dizziness while exercising. Subaortic membrane was totally excised; left internal thoracic artery was anastomosed to left anterior descending artery by end-to-side technique. The postoperative 2-year course of the patient was uneventful
Subject(s)
Humans , Male , Aortic Stenosis, Subvalvular/congenital , Coronary Disease/complications , EchocardiographyABSTRACT
Discrete subvalvular aortic stenosis is a relatively rare condition in adults. It is often diagnosed during first decade of life especially in association with other congenital malformations. Isolated form of discrete subvalvular aortic stenosis may however silently progress from innocent murmurs of childhood and adolescence to symptomatic left ventricular outflow tract obstruction in adults. Certain overt and subtle morphological abnormalities may underlie the initial expression as well as high recurrence rates after surgical resection of sub aortic membrane. Though surgical resection is the only treatment available, debate on the surgical technique and appropriate timing of surgery continues. Close followup with serial echocardiographic examinations in patients detected to have functional murmurs during childhood may be helpful in early detection of subvalvular aortic stertosis.
Subject(s)
Adult , Aorta/diagnostic imaging , Aortic Stenosis, Subvalvular/surgery , Diagnosis, Differential , Echocardiography, Doppler , Female , Heart Ventricles/diagnostic imaging , Humans , Male , RecurrenceABSTRACT
Se presenta un caso clínico de un paciente joven, inmunocompetente que en el posoperatorio de corrección de estenosis subvalvular aórtica congénita presentó endocarditis infecciosa de comienzo tardío (3 meses); de etiología micótica a un germen excepcional: Exserohilum rostratum. El diagnóstico etiológico de infección micótica por Exserohilum rostratum, se estableció por el estudio antomopatológico y micológico de la vegetación extraída. El tratamiento consistió en la intervención quirúrgica de limpieza y reemplazo valvular aórtico con prótesis mecánica, y el uso de anfotericina B. El paciente se mantiene asintomático hasta el momento actual. Se revisa la literatura a propósito de infecciones micóticas por Exserohilum y se analiza en particular algunos aspectos de la historia clínica de este paciente
Subject(s)
Humans , Male , Adolescent , Endocarditis/etiology , Endocarditis/therapy , Mycoses/diagnosis , Mycoses/therapy , Amphotericin B/therapeutic use , Aortic Stenosis, Subvalvular/surgeryABSTRACT
Subvalvular aortic stenosis developed after patch closure of perimembranous VSD is rarely reported. A 18-month-old, 8 kg child with this complication after VSD closure 8 months ago in other hospital has been treated medically and was admitted to this hospital because of severe cardiomegaly and sign of heart failure. Cardiac catheterization revealed 55 mmHg of pressure gradient between aorta and LV cavity. We report one successful redo case of surgically relieved subvalvular aortic stenosis in a child after patch closure of perimembranous VSD.
Subject(s)
Child , Humans , Infant , Aorta , Aortic Stenosis, Subvalvular , Cardiac Catheterization , Cardiac Catheters , Cardiomegaly , Heart FailureABSTRACT
Se studió a una joven de 15 años de edad por la presencia de un soplo sistólico en foco aórtico. El ecocardiograma reveló la presencia de una estructura móvil, de características semejantes a una valva, fija a la superficie ventricular de la valva anterior de la mitral, y por cuerdas tendinosas al músculo papilar. Esta estructura se dirigía hacia el tracto de salida del ventrículo izquierdo durante la sístole, ocupando el área subaórtica. El eco convencional Doppler demostró obstrucción del tracto de salida del ventrículo izquierdo, causado por el tejido valvular mitral accesorio, lo que producía un gradiente de presión de 25 mmHg entre la aorta y el ventrículo izquierdo. El diagnóstico no fue obvio clínicamente y fue basado en los datos ecocardiográficos. El ecocardiograma convencional y Doppler es el método de elección y el más seguro para establecer este diagnóstico
Subject(s)
Adolescent , Humans , Female , Heart Defects, Congenital/diagnosis , Echocardiography/statistics & numerical data , Endocardial Cushion Defects/diagnosis , Aortic Stenosis, Subvalvular/diagnosis , Mitral Valve/abnormalities , Heart Ventricles/abnormalitiesABSTRACT
El tratamiento quirúrgico del la estenosis subvalvular aórtica es complejo, en la forma localizada ha propuesto el tratamiento quirúrgico con miotomía-miectomía (procedimiento de Morrow) con buenos resultados. Sin embargo, para la forma difusa dicho procedimiento no resulta adecuado. Pos eso se ha propuesto la colocación de tubos valvados de la punta del ventrículo izquierdo a la aorta descendente, así como el procedimiento de Konno-Rastan que requiere la colocación de una válvula protésica. Desde 1984 se ha propuesto el abordaje aortoseptal con reconstrucción directa del tracto de salida del ventrículo izquierdo preservando la válvula aórtica, para la correción total de esta patología extrema. Se presenta la experiencia de 5 pacientes durante 10 años con esta alternativa de tratamiento quirúrgico
Subject(s)
Adult , Humans , Male , Female , Aortic Stenosis, Subvalvular/surgery , Surgical Procedures, Operative , Aortic Valve/surgeryABSTRACT
Discrete subvalvular aortic stenosis is a relatively uncommon cause of the left ventricular outflow obstruction, requiring early intervention. Conventional transthoracic echocardiography may fail in some patients due to insufficient imaging quality. In particular, in patients with a discrete fibrous membrane close to the aortic valve without narrowing of the left ventricular outflow tract, the echocardiographic detection of the membrane may be difficult. Transesophageal echocardiography allows a clear visualization of the aortic valve and the left ventricular outflow tract in virtually all patients, it can be performed rapidly with almost no risk, and it may therfore be helpful in establishing the diagnosis of discrete subaortic stenosis, in particular in patients where the conventional transthoracic approach fails. We have experienced two cases of discrete subaortic stenosis. One case of them was combined with hypertrophic obstructive cardiomyopathy in this report we discussed the utility of multiplane transesophageal echocardiography in patients with discrete subvalvular aortic stenosis.
Subject(s)
Humans , Aortic Stenosis, Subvalvular , Aortic Valve , Cardiomyopathy, Hypertrophic , Diagnosis , Discrete Subaortic Stenosis , Early Intervention, Educational , Echocardiography , Echocardiography, Transesophageal , Membranes , Ventricular Outflow ObstructionABSTRACT
Percutaneous balloon dilatation [PBD] for discrete subaortic stenosis [DSS] has been attempted in 10 patients. Mean age 10 year [range 6-20], 4 patients were male and 6 were females. The balloon diameter used ranged from 15-23 mm. 7 patients had thin membrane [m] [group 1], and 3 patients had thick fibromuscular ring [TFR] [group 2]. In group 1 the peak to peak hemodynamic gradient [PPHG] decreased From 75 +/- 21 mmHg to 30 +/- 23 mmHg, and the left ventricular systolic pressure [LVSP] has slightly decreased from 171.8 +/- 80.5 mmHg to 126.3 +/- 21.5 mmHg. In group 2 the [PPHG] decreased slightly from 75.5 +/- 26.3 to 63.5 +/- 18.4 mmHg and the [LVSP] has slightly decreased from 180.5 +/- 32.5 mmHg to 156 +/- 9.3 mmHg. Two patients developed grade I aortic regurgitation, the other 8 patients with pre existent grade one aortic regurgitation, showed no aggravation. One patient developed thrombosis of the right femoral artery, successfully treated by thrombectomy. A mean echocardiographic follow up after 6 months showed stabilization of the post dilatation gradient in group 1. In conclusion, percutaneous balloon dilatation for discrete subaortic stenosis of membraneous etiology offers a good immediate and short term result and constitutes an excellent alternative palliative treatment to surgery, while surgery is indicated for patients with thick firbomuscular ring
Subject(s)
Humans , Aortic Stenosis, Subvalvular/therapy , Echocardiography/instrumentationABSTRACT
Patient with subaortic stenosis due to the insertion of anomalous chordae tendineae of the mitral valve in the interventricular septum (mitral web) associated with mitral stenosis with diagnosis made by bidimensional echocardiographic study with Doppler and confirmed by hemodynamic study. The patient was submitted to surgical treatment. The anomalous cords were resected and the mitral valve with severe malformation was replaced by a bioprosthesis. The patient had a uneventful recovery and echocardiographic evaluation showed an outflow tract without obstruction, with no gradient, with remnants of the papillary muscles and normo-functioning bioprosthesis
Paciente portador de estenose subaórtica por cordas anômalas da valva mitral, inserindo no septo interventricular ("mitral Web") e estenose da valva mitral com diagnóstico feito através de estudo ecocardiográfico bidimensional com Doppler e confirmado pelo cateterismo cardíaco. O paciente foi submetido a tratamento cirúrgico, tendo sido encontrada dupla lesão mitral com predomínio de estenose, implantação de cordas anômalas da cúspide anterior da valva mitral no septo interventricular, obstruindo parcialmente a via de saída do ventrículo esquerdo. Foi realizada ressecção das cordas anômalas, aliviando a estenose subaórtica e, devido a má-formação da valva mitral, foi necessária a sua substituição por bioprótese. O paciente teve evolução pós-operatória sem intercorrências.O estudo ecocardiográfico pós-operatório mostrou resquício do aparelho subvalvar mitral e ausência de gradiente pressórico entre o ventrículo esquerdo e sua via de saída
Subject(s)
Humans , Male , Middle Aged , Aortic Stenosis, Subvalvular/etiology , Chordae Tendineae/abnormalities , Mitral Valve/abnormalities , Aortic Stenosis, Subvalvular/surgery , Aortic Stenosis, Subvalvular/diagnosis , Echocardiography, Doppler , Chordae Tendineae/surgery , Electrocardiography , Mitral Valve/surgery , Ventricular Function, Left/physiologyABSTRACT
Paciente de 14 anos, branco, sexo masculino, com diagnóstico de estenose subaórtica severa no pós-operatório tardio de correçäo total de um defeito do septo atrioventricular, forma completa. O diagnóstico clínico foi comprovado pelo estudo ecocardiográfico e cateterismo cardíaco, sendo o paciente submetido a reoperaçäo para tratamento da nova condiçäo, com ressecçäo do diafragma subaórtico. A via de saída do ventrículo esquerdo, estreita e deformada nos casos de defeito do septo atrioventricular, é suscetível de obstruçäo natural ou no pós-operatório, em decorrência do próprio ato cirúrgico ou das novas condiçöes estabelecidas. Torna-se, portanto, recomendável pesquisar possível obstruçäo tanto no pré-operatório como na sala de cirurgia após a correçäo,e no pós-operatório tardio
White male patient, 14 years old, who developed a severe subaortic stenosis in the late postoperative period of a total correction of atrioventricular septal defect. Clinical diagnosis was conf rmed by echocardiography and cardiac cathoterization. The patient underwent reoperation for treatment of the new condition, with ressection of subaortic stenosis. Atrioventricular septal defects show a narrow and deformed leit ventricle outflow tract, susceptible to obstruction before or after operation. It is, therefore, advisable to investigate a possible obstruction before the operation at the operation room and in the late postoperative period
Subject(s)
Humans , Male , Adolescent , Aortic Stenosis, Subvalvular/etiology , Heart Septal Defects, Ventricular/surgery , Postoperative Complications , Aortic Stenosis, Subvalvular/surgery , Aortic Stenosis, Subvalvular , Echocardiography , Electrocardiography , ReoperationABSTRACT
In this study 29 cases of subaortic stenosis were analyzed to evaluate the value of septal myectomy in surgical management of subaortic stenosis. They were classified into 2 groups: Group I included 16 cases without myectomy and group II 13 cases for whom myectomy [in 10 cases] or myotomy [in 3 cases] was performed with excision of subaortic membrane of fibromuscular ridge. Immediate and late postoperative pressure gradient measurements across LVOT, showed smaller gradient in group II. There was more regression of the gradient during follow up visits in patients, for whom septal myectomy or myotomy was performed. Septal myectomy is recommended in cases of subaortic stenosis. The amount of resection is adjusted according to the degree of septal hypertrophy