Caracterización de las enfermedades valvulares congénitas en la edad pediátrica / Characterization the pediatric patients with diagnostic of illnesses congenital valvulares

Introducción: Las valvulopatías congénitas son un grupo de entidades en las cuales la principal alteración anatomofuncional está en las válvulas cardiacas. Objetivo: Caracterizar los pacientes pediátricos nacidos vivos con diagnósticos de valvulopatías congénitas. Métodos: Se realizó una investigación descriptiva retrospectiva a partir de los registros de valvulopatías congénitas del Servicio de Cardiología del Hospital Pediátrico Universitario José Luis Miranda en Santa Clara, Cuba, en el periodo de 2005 a 2016. La población estuvo conformada por los 147 niños nacidos vivos con diagnóstico de valvulopatías congénitas. Se empleó un muestreo no probabilístico intencional por criterios y la muestra quedó conformada por 143. La recogida de la información se realizó a través de la revisión documental de las historias clínicas. Resultados: La tasa de incidencia de la valvulopatías congénitas fue de 1,5 x 1 000 nacidos vivos, predominó la estenosis pulmonar que agrupó el 70,6 por ciento de los diagnosticados. Se encontró predominio del sexo masculino con 50,3 por ciento, se determinó que 45,5 por ciento de los casos se diagnosticaron antes de los 29 días de nacido, período que incluye un diagnóstico prenatal. Conclusiones: El desarrollo del diagnóstico prenatal de las cardiopatías congénitas ha posibilitado la disminución de la incidencia de las malformaciones más complejas. El conocimiento de las enfermedades congénitas del corazón contribuye a operar de forma segura y con resolución anatómica completa a muchos de estos niños en el período prenatal o cuando son lactantes pequeños, para evitar el deterioro global de estos cuando la cardiopatía no está resuelta(AU)

Introduction: The congenital valvulopatías is a group of entities in which the main alteration anatomofuncional is in the heart valves. Objective: To characterize the pediatric patients with diagnostic of born congenital valvulopatías. Methods: He/she was carried out a retrospective descriptive investigation starting from the registrations of congenital valvulopatías of the service of Cardiology of the Hospital Pediatric University student José Luis Miranda in the period of 2005 at the 2016. The population was conformed by the 147 alive born children with diagnostic of congenital valvulopatías, a sampling was not used intentional probabilístico by approaches, being conformed the sample by 143. The collection of the information was carried out through the documental revision of the clinical histories. Results: The rate of incidence of the congenital valvulopatías was of 1,5 x 1000 born alive, the lung estenosis prevailed containing to 70,6 percent of those diagnosed he/she was prevalence of the masculine sex in 50,3 percent, it was determined that 45,5 percent of the cases was diagnosed before the 29 days where a prenatal diagnosis is included. Conclusions: The development of the prenatal diagnosis of the congenital cardiopatías has facilitated the decrease of the incidence of the most complex malformations. The knowledge of the congenital illnesses of the heart contributes to operate in a sure way and with complete anatomical resolution to many of these children in the prenatal period or when they are small nurslings, to avoid the global deterioration of these when the cardiopatía is not resolved(AU)

Comparison of transthoracic echocardiography and right heart catheterization for assessing pulmonary arterial pressure in patients with congenital or valvular heart defects

Although right heart catheterization [RHC] has acceptable accuracy for the measurement of pulmonary arterial pressure [PAP], significant risks and cost issues are worrisome. Thus, a non-invasive technique such as echocardiography for assessing PAP would clearly be of great clinical value. We aimed to compare estimated systolic PAP [SPAP] by echocardiogram with the actual RHC measurements in the two groups of congenital and valvular heart diseases [CHD and VHD, respectively], in whom pulmonary hypertension [PHT] was clinically suspected. A total of 103 consecutive patients with confirmed CHD or VHD referred to our center between January and December 2009 were studied. Participants underwent transthoracic echocardiogaphy and RHC within 4 hours of each other. The mean SPAP in the CHD group was no different measured by RHC or echo [46.49 +/- 29.04 vs. 46.45 +/- 23 mmHg, p = 0.541]. The mean SPAP in the VHD group measured by RHC was significantly higher than that measured by echo [48.70 +/- 14.50 vs. 44.90 +/- 11.0 mmHg, p = 0.041]. Fifty-one [49.5%] patients were found to have PHT at RHC. Echocardiography correctly identified 48 of these patients [sensitivity=94.1%]. Nineteen of the 52 patients without PHT on RHC were correctly identified by echocardiography [specificity= 36.5%]. The positive and negative predictive values for echocardiography in assessing the presence or absence of PHT were 59.3% and 86.4%, respectively. Integration of hemodynamic data with the eho examination can appropriately provide comprehensive assessment of PHT with high sensitivity in individual patients with congenital or valvular heart defects

The bicuspid aortic valve and related disorders / A válvula aórtica bicúspide e desordens relacionadas

Bicuspid aortic valve (BAV) is the most common congenital cardiac malformation, affecting 1-2 percent of the population, with strong male predominance. Individuals may have a normally functioning BAV, and may be unaware of its presence and the potential risk of complications. However, they may easily develop aortic valve disorders: either stenotic or regurgitant, or both. Today, BAV is recognized as a syndrome incorporating aortic valve disorders and aortic wall abnormalities, including aortic dilation, dissection or rupture. Congenital or hereditary diseases such as ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, Turner's syndrome, Marfan's syndrome etc., may frequently be associated with BAV. Infective endocarditis and occasionally thrombus formation may develop during the lives of BAV patients. Elevated cholesterol or C-reactive protein may be seen in laboratory findings of these patients. Beta-blockers and statins are the possibilities for medical treatment, and aortic valve repair/replacement and ascending aorta replacement are indicated for patients with a severely diseased aortic valve and aorta. Rigorous follow-up throughout life is mandatory after BAV has been diagnosed. The aim of the present article was to describe the implications of BAV and its associated disorders, and to discuss diagnostic and treatment strategies.

A válvula aórtica bicúspide (VAB) é a malformação cardíaca congênita, mais comum afetando 1-2 por cento da população, com forte predominância no sexo masculino. Indivíduos afetados podem exibir funcionamento normal da VAB, ignorando sua presença e o potencial risco de complicações associadas. Mas podem facilmente desenvolver disfunções de valva aórtica, com estenose, regurgitação, ou ambas. Hoje em dia, a VAB é reconhecida como uma síndrome incorporando alterações de valva aórtica e anormalidades da parede aórtica, inclusive dilação da aorta, dissecção ou ruptura. Doenças congênitas ou hereditárias, como defeito do septo ventricular, canal arterial patente, coartação de aorta, síndrome de Turner, síndrome de Marfan etc., podem estar frequentemente associadas com VAB. A endocardite infecciosa e ocasionalmente formação de trombo podem se desenvolver durante a vida nos pacientes com VAB. Elevação do colesterol ou proteína C-reativa pode ser observada nos resultados laboratoriais desses pacientes. Betabloqueador e estatinas são as opções de tratamento médico e reparação/substituição da valva aórtica e substituição da aorta ascendente são indicadas para pacientes com doença grave da valva aórtica e artéria aorta. Acompanhamento rigoroso por toda a vida é obrigatório quando do diagnóstico da VAB. O objetivo do presente artigo é descrever as implicações do VAB assim como suas desordens associadas e discutir as estratégias de diagnóstico e tratamento.

Clínica de enfermedad valvular cardíaca del Centro Cardiovascular Regional-Ascardio: Barquisimeto-Estado Lara / Clinic of valvular heart disease of the Regional Cardiovascular Center-Ascardio: Barquisimeto-Lara State

La enfermedad valvular cardíaca es un importante problema de salud que requiere programas de atención especializada. Este artículo señala la justificación de una consulta de patologías valvulares y presenta la consulta organizada en el Centro Cardiovascular Regional Ascardio, su estructura, sus objetivos, protocolo de trabajo, requisito para el ingreso de pacientes y los vínculos que tiene con otras unidades y servicios. Se concluye que la consulta de enfermedad vascular cardíaca representa una experiencia positiva que puede servir de referencia para otros centros similares que tengan la iniciativa de organizar consultas especializadas.

Valvular heart disease poses a critical health problem requiring specialized medical care programs. This article highlights why a valvular heart disease clinic is justified and presents the clinic set up at Ascardio Regional Cardiovascular Center, as well as its structure, objectives, work protocol, requirements for patients´enrollment, and links with other units and services. In conclusion, the valvular heart disease clinic is a positive experience and a useful reference for other similar centers who may be taking the initiative in setting up specialized clinics.

Padronização da técnica para cirurgia cardíaca videoassistida: experiência inicial / Standardization of video-assisted cardiac surgery technique: initial experience

INTRODUÇÃO: Cirurgias cardíacas videoassistidas minimamente invasivas têm sido realizadas em grandes centros mundiais, incluindo procedimentos valvares, coronários e congênitos. OBJETIVO: Apresentar nossa primeira casuística com videocirurgia cardíaca, por meio da experiência adquirida com cirurgia geral e torácica. MÉTODOS: Sempre que houve possibilidade de se realizar uma cirurgia cardíaca por acesso minimamente invasivo, esta foi a abordagem inicialmente utilizada. A toracoscopia esquerda foi utilizada em quatro casos: (1) ligadura de fistula coronário-pulmonar sintomática, (2) implante de eletrodo epicárdico em ventrículo esquerdo para ressincronização ventricular, (3) retirada de lipoma gigante de pericárdio com pedículo em átrio esquerdo e (4) ressecção de hemangio-lipoma de mediastino. Toracoscopia direita com bypass cardiopulmonar via artéria e veia femorais e parada cardíaca em fibrilação ventricular com hipotermia moderada, foram realizados nos seguintes casos: (5) paciente com estenose mitral pós-plastia, com anel de Carpentier 12 anos antes. Foi realizada comissurotomia anterior e posterior com sucesso, sem toracotomia, (6) paciente com miocardiopatia dilatada idiopática, fibrilação atrial de alta resposta e insuficiência mitral importante, realizada plastia mitral com anel de Gregori e ablação de veias pulmonares com cateter de radiofreqüência (MAZE modificada). Neste caso, uma minitoracotomia ântero-lateral direita de 6 cm foi utilizada. RESULTADOS: Nenhum óbito ocorreu nesta série e o resultado cirúrgico em todos os casos foi altamente satisfatório, sendo que todos os pacientes receberam alta hospitalar com média de 5,5 + 5 dias após a intervenção. CONCLUSÃO: Nossa experiência inicial neste campo demonstra a eficácia e exeqüibilidade de introdução deste tipo de técnica em nosso meio.

INTRODUCTION: Minimally invasive cardiac surgery has been performed in major worldwide centers, including procedure such as valves, coronary and congenital surgery. OBJECTIVE: To demonstrate our first works with noninvasive cardiac surgery by mean of the experience gained with general and thoracic surgery. METHODS: Whenever possible to carry out a minimally invasive cardiac surgery, this was the approach of choice. The left thoracoscopy was used in four cases: (1) symptomatic coronary-pulmonary fistula ligation; (2) implant of an epicardial electrode into the left ventricle for resynchronization; (3) excision of pericardial giant lipoma in the left atrium, and (4) resection of hemangiolipoma in the mediastinum. Right thoracoscopy with extracorporeal circulation through cardiopulmonary bypass via femoral vein and artery and cardiac arrest in ventricular fibrillation with moderate hypothermia were carried out in the following cases: (5) patient with mitral stenosis after surgical repair with Carpentier ring 12 years before. An anterior and posterior commissurotomy without thoracotomy was successfully made; (6) patient with idiopathic dilated cardiomyopathy, high-response atrial fibrillation, and severe mitral insufficiency, underwent mitral repair surgery with Gregori's ring and ablation of the pulmonary veins with radio-frequency catheter. (MAZZE modified). RESULT: No death occurred in this series and the surgical result in all cases was highly satisfactory. All patients were discharged from hospital with a mean time of 5.5 ± 5 days after intervention. CONCLUSION: Our initial experience in this field proves the effectiveness and the viability in introducing this type of technique in Brazil.

Ventricular septal defect with congenital mitral valve disease: long-term results of corrective surgery.

BACKGROUND: A retrospective analysis of the mortality, morbidity and long-term follow-up of patients undergoing corrective surgery for ventricular septal defect and congenital mitral valve disease is presented. METHODS AND RESULTS: Between January 1991 and December 2000, 69 consecutive patients aged 2 months to 45 years (median 18 months) underwent repair of ventricular septal defect and associated mitral valve disease. In 52 patients (75%), the ventricular septal defects were located in the perimembranous and subarterial area. Forty-six patients had congenital mitral incompetence and 23 had congenital mitral stenosis. The ventricular septal defect was repaired through the right atrium in all. Sixty-five patients underwent reconstruction of the mitral valve and 4 underwent primary mitral valve replacement. Another 4 patients underwent mitral valve replacement after a failed repair. Associated procedures included: patent ductus arteriosus ligation (n=12), aortic valve replacement (n=6), coarctation repair (n=13), interrupted aortic arch repair (n=1), atrial septal defect closure (n=17) and Takeuchi repair (n=1). There were 6 early deaths (8.6%). Three deaths were due to pulmonary arterial hypertensive crisis and one due to residual mitral stenosis. One death was due to intractable congestive heart failure. Another patient died due to persistent low cardiac output. Follow-up ranged from 6 months to 120 months (mean 64.4+/-33.6 months). Reoperation was required in 22 patients, mainly for recurrent/residual mitral valve dysfunction or hemodynamically significant left ventricular outflow tract obstruction. There were 4 late deaths, 2 due to residual mitral stenosis and the other 2 as a result of a thrombosed prosthetic valve. At 10 years, the actuarial survival rate was 850+/-5.0%, and freedom from reoperation was 45%+/-10.0%. CONCLUSIONS: Reconstruction of the mitral valve along with closure of VSD is possible in most cases. However, careful follow-up is recommended to detect changes in the mitral valve status over a course of time.

Congenitally unguarded tricuspid valve orifice with a giant right atrium and a massive clot in an asymptomatic adult.

Congenitally unguarded tricuspid valve orifice, a variant of tricuspid valve dysplasia, is a rare malformation with protean manifestations. This report describes an asymptomatic adult who, on echocardiographic examination ordered in view of an abnormal 12-lead surface electrocardiogram and plain chest X-ray, was found to have an unguarded tricuspid valve orifice with a giant right atrium (12 x 10 cm), intense spontaneous echo contrast and a large right atrial clot.

Spectrum of tricuspid atresia--a study of 23 cases.

The present report describes the clinical and angio-cardiographic features of 23 cases of tricuspid atresia. Nineteen patients had type I anatomy, 2 patients had type II anatomy and 2 patients had type III anatomy. In addition to the standard features, there were some uncommon associations observed. These included double outlet right ventricle, double outlet left ventricle, single coronary artery, complete heart block, right axis deviation, ostium primum atrial septal defect and coarctation of the aorta.