Diagnóstico prenatal de enfermedad de canal atrio-ventricular, análisis epidemiológico y experiencia CERPO entre 2003 y 2021 / Prenatal diagnosis of atrio-ventricular canal disease, epidemiological analysis and CERPO experience between 2003 and 2021

Antecedentes: En Chile, la mitad de los casos de mortalidad perinatal son atribuibles a anomalías congénitas, y un tercio de estas corresponde a cardiopatías congénitas. Aproximadamente un 35% de estos últimos requerirán cirugía antes del año de vida, por lo que la pesquisa prenatal impacta profundamente en el pronóstico. Objetivo: Dar a conocer los resultados perinatales de pacientes con diagnóstico prenatal de canal atrio-ventricular controlados en el Centro de Referencia Perinatal Oriente (CERPO) entre los años 2003 y 2021, su asociación a otras anomalías, características demográficas y pronóstico a un año. Métodos: En este estudio se puede apreciar que tanto el pronóstico como el plan terapéutico posnatal dependerán de la presencia de otras alteraciones morfológicas y del estudio genético. De los factores estudiados, se puede concluir que tanto la presencia de anomalías cardiacas asociadas, como el grado de insuficiencia valvular y el tipo de canal no son predictores de la sobrevida perinatal. Conclusiones: Finalmente, en relación con la sobrevida posnatal, en este estudio, la sobrevida a un año de los recién nacidos vivos fue de un 52%, pero al desglosarlo en los niños con cariotipo euploide y trisomía 21, estos valores se tornan muy distintos, 44 y 81% respectivamente.

Background: In Chile, half of the perinatal mortality cases are attributable to congenital anomalies, and one third of these correspond to congenital heart disease. Approximately 35% of the later will require surgery before one year of life, so prenatal screening has a profound impact on the prognosis. Objective: To present the perinatal results of patients with a prenatal diagnosis of atrio-ventricular canal controlled at Centro de Referencia Perinatal Oriente CERPO) between 2003 and 2021, its association with other anomalies, demographic characteristics, and 1-year prognosis. Methods: In this study it can be seen that both the prognosis and the postnatal therapeutic plan will depend on the presence of other morphological alterations and the genetic study. From the factors studied, it can be concluded that the presence of associated cardiac anomalies, the degree of valvular insufficiency, and the type of canal are not predictors of perinatal survival. Conclusions: Finally, in relation to postnatal survival, in this study, the 1-year survival of live newborns was 52%, but when broken down into children with euploid karyotype and trisomy 21, these values become very different, 44 and 81% respectively.

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)

Waiting for Cardiac Procedure in Congenital Heart Disease: Portrait of an a Hospital in the Amazonian Region / Fila de Espera para Tratamento de Pacientes com Cardiopatia Congênita: Retrato de um Centro de Referência Amazônico

Congenital heart disease is an important cause of morbidity and mortality in childhood, and in 50% of cases, surgery is required in the first year of life. A high deficit of surgical procedures is estimated in Northern Brazil. Objective: To analyze the waiting time for elective surgical treatment and/ or intervention in children with congenital heart disease in a Cardiology referral center, and to make considerations about heart diseases and forms of treatment in that institution. Methods: A cross-sectional study of all patients aged less than 14 years, with a diagnosis of congenital heart disease that were waiting for elective surgical or percutaneous cardiac treatment. Results: Among the 407 children with congenital heart defects, the most prevalent age group was > 2 to 6 years (34.0%). The average waiting time was 23.1 ± 18.3 months, with a median of 19. The most frequent heart disease was ventricular septal defect (28.98%), patent ductus arteriosus (18.42%) and atrial septal defect (11.05%). Most children (63.4%) were not from the metropolitan area. The percutaneous interventions represented only 27.84% of the catheterization procedures and 14,85% of all heart treatments. Approximately 60% of the pediatric surgeries occurred in children who were not previously registered due to urgency cases. Conclusion: Most of the children waiting for a cardiac procedure were not from the metropolitan area and had malformations potentially treatable by catheterization. It is necessary to increase the capacity of the single referral center in the state of Pará, as well as decentralize the high-complexity cardiological care in the metropolitan region

As cardiopatias congênitas são importantes causas de morbimortalidade infantil e, em cerca de 50% dos casos, é necessária a intervenção cirúrgica no primeiro ano de vida. Estima-se alto défice de procedimentos na Região Norte do Brasil. Objetivo: Analisar o tempo de espera para realização de tratamento eletivo cirúrgico e/ou intervencionista de crianças portadoras de cardiopatias congênitas em um centro de referência cardiológico, e fazer considerações sobre as cardiopatias e suas formas de tratamento na referida instituição. Método: Estudo analítico, de caráter transversal do período de janeiro de 2012 a outubro de 2014, de pacientes com idade igual ou inferior a 14 anos diagnosticados com cardiopatias congênitas que estavam na fila de tratamento cardíaco eletivo cirúrgico ou percutâneo. Resultados: Das 407 crianças que aguardavam por tratamento, a faixa etária mais prevalente foi a de > 2 a 6 anos (34,0%). O tempo médio de espera, em meses, foi 23,1 ± 18,3, com mediana de 19. As cardiopatias mais frequentes foram comunicação interventricular (28,98%), persistência do canal arterial (18,42%) e comunicação interatrial (11,05%). A maioria das crianças (63,4%) não pertencia à região metropolitana. As intervenções percutâneas representaram somente 27,84% do total de cateterismos e 14,85% de todos os tratamentos cardíacos. Cerca de 60% do volume de cirurgias pediátricas ocorreu em crianças sem cadastro prévio no sistema, devido ao caráter de urgência. Conclusão: Grande parte das crianças que aguardam por procedimento cardíaco é procedente de fora da região metropolitana e tem malformações potencialmente tratáveis por cateterismo. É necessário aumentar a capacidade operacional do único centro de referência público do Estado, além de descentralizar o atendimento em alta complexidade cardiológica da região metropolitana

Fila de espera para tratamento de pacientes com cardiopatia congênita: relato de um Centro de Referência Amazônico / Waiting for cardiac procedure in congenital heart disease: portrait of an Amazonian Hospital

As cardiopatias congênitas são importantes causas de morbimortalidade infantil e, em cerca de 50% dos casos, é necessária a intervenção cirúrgica no primeiro ano de vida. Estima-se alto défice de procedimentos na Região Norte do Brasil.Analisar o tempo de espera para realização de tratamento eletivo cirúrgico e/ou intervencionista de crianças portadoras de cardiopatias congênitas em um centro de referência cardiológico, e fazer considerações sobre as cardiopatias e suas formas de tratamento na referida instituição. Estudo analítico, de caráter transversal do período de janeiro de 2012 a outubro de 2014, de pacientes com idade igual ou inferior a 14 anos diagnosticados com cardiopatias congênitas que estavam na fila de tratamento cardíaco eletivo cirúrgico ou percutâneo. Das 407 crianças que aguardavam por tratamento, a faixa etária mais prevalente foi a de > 2 a 6 anos (34,0%). O tempo médio de espera, em meses, foi 23,1 ± 18,3, com mediana de 19. As cardiopatias mais frequentes foram comunicação interventricular (28,98%), persistência do canal arterial (18,42%) e comunicação interatrial (11,05%). A maioria das crianças (63,4%) não pertencia à região metropolitana. As intervenções percutâneas representaram somente 27,84% do total de cateterismos e 14,85% de todos os tratamentos cardíacos. Cerca de 60% do volume de cirurgias pediátricas ocorreu em crianças sem cadastro prévio no sistema, devido ao caráter de urgência. Grande parte das crianças que aguardam por procedimento cardíaco é procedente de fora da região metropolitana e tem malformações potencialmente tratáveis por cateterismo. É necessário aumentar a capacidade operacional do único centro de referência público do Estado, além de descentralizar o atendimento em alta complexidade cardiológica da região metropolitana

Congenital heart disease is an important cause of morbidity and mortality in childhood, and in 50% of cases, surgery is required in the first year of life. A high deficit of surgical procedures is estimated in Northern Brazil. To analyze the waiting time for elective surgical treatment and/or intervention in children with congenital heart disease in a Cardiology referral center, and to make considerations about heart diseases and forms of treatment in that institution. A cross-sectional study of all patients aged less than 14 years, with a diagnosis of congenital heart disease that were waiting for elective surgical or percutaneous cardiac treatment. Among the 407 children with congenital heart defects, the most prevalent age group was > 2 to 6 years (34.0%). The average waiting time was 23.1 ± 18.3 months, with a median of 19. The most frequent heart disease was ventricular septal defect (28.98%), patent ductus arteriosus (18.42%) and atrial septal defect (11.05%). Most children (63.4%) were not from the metropolitan area. The percutaneous interventions represented only 27.84% of the catheterization procedures and 14,85% of all heart treatments. Approximately 60% of the pediatric surgeries occurred in children who were not previously registered due to urgency cases. Most of the children waiting for a cardiac procedure were not from the metropolitan area and had malformations potentially treatable by catheterization. It is necessary to increase the capacity of the single referral center in the state of Pará, as well as decentralize the high-complexity cardiological care in the metropolitan region

Repair of double orifice left av valve (dolavv) with endocardial cushion defect in adult

Abstract Double orifice left atrioventricular valve (DOLAVV) or double orifice mitral valve (DOMV) is a rare congenital cardiac anomaly manifesting either as an isolated lesion (mitral stenosis or mitral insufficiency) or in association with other congenital cardiac defects. Signs of mitral valve disease are usually present along with the symptoms of associated coexistent congenital heart diseases. Mitral insufficiency due to annular dilatation is seen when DOLAVV is associated with endocardial cushion defects. Surgical intervention like mitral valve repair or replacement is required in 50% of patients and yields good results. We report a case of a 56-year-old lady who successfully underwent surgical correction of DOLAVV with partial atrioventricular canal defect.

Defeito do septo átrio ventricular em adulto portador de trissomia do 21 / Case report defect of the septure ventricular atrial in adult trissomy carrier of 21

Introdução: Dos pacientes com Síndrome de Down que possuem cardiopatias congênitas, metade possuem defeitos no septo atrioventricular, em associação de comunicação interatrial, comunicação interventricular e a persistência do canal arterial. Descrição do caso: M.N.S,42 anos, portador de trissomia do 21. Encaminhado para a cardiologia,dia 07/09/16, para realização do cateterismo cardíaco após dor torácica recorrente e sopro sistólico. Na admissão, referiu precordialgia, com intensidade forte e duração de um dia, sem irradiações e fatores desencadeantes. Possui histórico de internações recorrentes de dor precordial e história familiar marcante de cardiopatias. Na avaliação clínica, encontrou-se sopro sistólico panfocal acentuado pela manobra de Rivero-Carvallo e diminuído com a manobra de Valsalva, confirmando sobrecarga de câmaras direitas. Realizou-se CATE de câmaras direitas e esquerdas no dia 09/09, onde conclui-se a presença de defeito septo AV de forma intermediária, hipertensão arterial pulmonar,hiperfluxo pulmonar,dilatação moderada da artéria pulmonar, aumento das dimensões do átrio esquerdo e constatou-se que o retorno venoso dáse para átrio esquerdo por uma comunicação interatrial ampla. Foi submetido à correção cirúrgica no dia 19/09/16, onde foi realizado plastia da valva mitral, correção da comunicação interatrial ostium primum com remendo pericárdico bovino e o fechamento do forame oval. Após a cirurgia, foi encaminhado para o CTI, onde permaneceu estável, sendo extubado no dia 21/09. Recebeu alta do CTI no dia 26/09 e encaminhado para a enfermaria para cuidados pós-cirúrgicos. Conclusão: É possível concluir que o despreparo de pequenos centros urbanos faz com que o serviço de saúde se direcione para os grandes centros urbanos e como muitas vezes isso não é possível, o diagnóstico e a terapêutica se dão tardiamente. (AU)

Introduction: Of the patients with Down syndrome who have congenital heart defects,half have defects in the atrioventricular septum,in the association of atrial septal defect, ventricular septal defect and persistence of the ductus arteriosus. Case description: MNS,42 years old,with trisomy 21. Referred to cardiology,day 07/09/16, to perform cardiac catheterization after recurrent chest pain and systolic murmur. On admission, he reported precordialgia,with strong intensity and duration of one day, without irradiation and triggering factors. He has a history of hospitalizations recurrence of precordial pain and marked family history of cardiopathies. In the clinical evaluation, a panfocal systolic murmur accentuated by the Rivero-Carvallo maneuver and decreased with the Valsalva maneuver, confirming overhead of right chambers. CATE of right chambers and on the 09/09, where the presence of an AV septal defect is found in pulmonary arterial hypertension, pulmonary hyperflow, moderate dilation of the pulmonary artery, enlargement of the left atrium, and venous return to the left atrium by extensive atrial septal defect. It was submitted to surgical correction on day 19 / 09, where mitral valve repair was performed,correction of the ostium primum atrial septal defect with bovine pericardial patch, and closure of the oval foramen. After surgery, the patient was referred to the ICU where he remained stable and was extubated on September 21. He was discharged from the ICU on September 26 and was sent to the ward for post-surgical care. Conclusion: It is possible to conclude that the lack of preparation of small urban centers causes the health service to be directed to large urban centers and how often this not possible, diagnosis and therapy are delayed. (AU)

Cierre de defectos Interauriculares en adultos en forma ambulatoria. Una palabra de alerta? / Transcatheter closure of atrial septal defects in adults on an outpatient basis. A word of caution

En esta edición de la Revista Colombiana de Cardiología, Villalobos y colaboradores (1) reportan los resultados del cierre de defectos del tabique interauricular de 56 pacientes pertenecientes a un Hospital Universitario, durante un período de seis años, en donde se indica que el defecto interauricular tipo ostium secundum (CIA) estuvo presente en 42% de los casos y el foramen oval permeable (FOP) en los restantes, y que el dispositivo más utilizado fue el Amplatzer Septal Occluder. Se precisa, además, que hubo éxito en el implante en 52/56 pacientes (93%) y la causa de falla fue un diámetro distendido de más de 40 mm debido a bordes laxos. No hubo complicaciones significativas y todos los pacientes, excepto uno, presentaban cierre completo de los defectos en el seguimiento ecocardiográfico de por lo menos un mes. Hubo, así mismo, mejoría significativa de los síntomas después del procedimiento, en especial de la presencia de disnea y palpitaciones. Dados estos resultados, que se corroboran con los previamente publicados en la literatura y ratifican la seguridad y efectividad del método, los autores merecen felicitaciones. Sin embargo, llaman la atención varios aspectos, entre ellos la estrategia adoptada en referencia a que en el 75% de los casos el procedimiento se realizó bajo anestesia local y de forma ambulatoria con alta hospitalaria después de cuatro horas del implante. De igual forma, no está claro qué tipo de monitoreo ecocardiográfico se utilizó durante el procedimiento o si no se utilizó este método. Los casos se seleccionaron previamente con el uso de la ecocardiografía transtorácica. El diámetro de la comunicación interauricular en este último método tuvo una variación entre 1 y 35 mm con una media de 13 ± 8,3 mm, que definen una población con defectos relativamente pequeños. Por otra parte, el tamaño del dispositivo utilizado osciló entre 14-35 mm con un promedio de 23 mm, que es un tamaño mayor a 50% que el diámetro nativo del defecto. Además, no está claro si los diámetros de los dispositivos utilizados se refieren a la totalidad de los dispositivos incluyendo CIA y FOP o sólo los de CIA. Como bien se sabe, el tamaño de los dispositivos de CIA se define por el diámetro de su cintura central, mientras que el tamaño del dispositivo de FOP por el diámetro del disco mayor, que es el derecho. Dichas prácticas merecen algunos comentarios y palabras de alerta.

Fístula aorta-átrio direito, simulando CIV, com fechamento percutâneo por prótese CERA / Percutaneous closure of aorta to right atrial fistula,simulating VSD, with CERA occluder

Fístulas entre a aorta e o átrio direito podem ser de origem congênita ou adquirida. As fístulas aortocamerais congênitas são raras anomalias do coração que podem ser confundidas com outros defeitos como comunicações interventriculares ou interatriais. São raros canais vasculares extra cardíacos e sua história natural, apresentação clínica e tratamento adequado ainda são obscuros. As fístulas adquiridas são, em geral, decorrentes de ruptura do seio de valsalva pós-infecciosa. Neste relato apresentamos um caso de difícil diagnóstico ecocardiográfico, no qual o diagnóstico de fístula aorta-átrio direito foi confundido com o de comunicação interventricular perimenbranosa e só confirmado após estudo hemodinâmico.

Aorta to right atrium fistula can be a congenital or condition. Congenital fistulas are uncommon heart diseases and can be mistaken by other heart lesions like interventricular septal defect or atrial septal defect. They seem to be are extra-cardiac vascular channels and their natural history, clinical presentation and treatment remain uncertain. Acquired fistula between aorta and right atrium is usually due to a post infeccious rupture of sinus of valsalva. The authors present a case of aorta-right atrium fistula that had been mistaken by perimenbranous interventricular septal defect during echocardiography examination and confirmed by cardiac catheterization.

Defecto del canal auriculoventricular, aurícula única y atresia tricuspídea como parte de un caso de síndrome de Ellis-Van Creveld / Atrioventricular canal defect, single atrium and tricuspid atresia as part of a case of Ellis-Van Creveld syndrome

El síndrome de Ellis-Van Creveld (SEVC) o displasia condroectodérmica se debe a una mutación de transmisión autosómica recesiva en el brazo corto del cromosoma 4 y afecta múltiples órganos. Descrito como una tétrada clásica de condrodisplasia, displasia ectodérmica, polidactilia y defectos cardíacos congénitos, sólo se conoce a partir de informes y series de casos. Se describe el caso de un varón de 3 meses, sin antecedentes familiares de importancia, que presentó un cuadro de condrodisplasia, labio superior fusionado al paladar, simpolidactilia posaxial bilateral en las manos, displasia del desarrollo de las caderas, tórax estrecho con costillas cortas y compromiso cardíaco. El presente caso sería la primera comunicación del SEVC en el Perú.

Ellis-Van Creveld Syndrome or chondrectodermal dysplasia is produced by an autosomal recessive inheritance secondary to mutation in the short arm of chromosome 4. The syndrome affects multiple organs. It is described as a clinical tetrad that involves chondrodysplasia, ectodermal dysplasia, polydactyly and congenital heart defects. It is only known from reports and case series. We present a three months old male, without relevant family history, who presented chondrodysplasia, upper lip merged to palate, bilateral sinpolydactyly in the hands, developmental dysplasia of the hip, narrow chest with short ribs, and heart defects. This case is the first report of EVC in Peruvian literature.

Cierre de comunicaciones interauriculares en adultos vía percutánea con dispositivo amplatzer. Primera serie reportada en Honduras / Percutaneous closure of atrial septal defects in adults with Amplatzer device. frst serie reported in Honduras

Antecedentes: La Comunicación Interauricular es una de las cardiopatías congénitas más frecuentes. En Honduras en pacientes con indicación de cierre, el método tradicional es la cirugía, otra opción es a través de dispositivos insertados por procedimiento de intervencionismo cardíaco. Pacientes y Métodos: Estudio descriptivo en el cual se incluyeron 27 pacientes con diagnóstico de comunicación interauricular tipo Ostium Secundum con cortocircuito intracardíaco (Qp/Qs) mayor de 1.5: 1, con adecuado tamaño de los bordes, únicas y sin hipertensión arterial pulmonar severa. Resultados: De 27 pacientes, en 24 (90%) se obtuvo éxito en cerrar la comunicación interauricular con dispositivos Amplatzer, sin encontrar complicaciones a corto ni a largo plazo. Conclusión: El cierre de comunicación Interauricular tipo Ostium Secundum a través de dispositivos Amplatzer es un procedimiento seguro y con alta tasa de éxito.

Un cas decouvert tardivement de canal atrio-ventriculaire complet avec hypertension arterielle pulmonaire importante

Malformation congenitale complexe; rare; a shunt gauche droit; le canal atrio-ventriculaire dans sa forme complete est grave par la complexite des lesions et le risque d'Hypertension Arterielle Pulmonaire fixee. Son pronostic depend de la precocite de son diagnostic et de sa prise en charge therapeutique. Les auteurs relatent un cas decouvert assez tardivement a l'age de 15 mois et corrigee chirurgicalement a l'age de 24 mois

Quantification of AS and AR.

Trans-esophageal echocardiography (TEE) is routinely used in valvular surgery in most institutions. The popularity of TEE stems from the fact that it can supplement or confirm information gained from other methods of evaluation or make completely independant diagnoses. Quantitative and qualitative assessment permits informed decisions regarding surgical intervention, type of intervention, correction of inadequate surgical repair and re-operation for complications. This review summarizes the various methods for quantification of aortic regurgitation and stenosis on TEE. The application of Doppler echo (pulsed wave, continuous wave and color) with two-dimensional echo allows the complete evaluation of AV lesions.

Intra-operative trans-esophageal echocardiography in congenital heart disease.

Intra-operative trans-esophageal echocardiography (TEE) is an important monitoring and diagnostic tool used during surgery for repair of congenital heart disease. In several studies,TEE has been shown to provide additional intra-cardiac anatomic information. Its ability to be used intra- operatively before and after cardiac repair makes it a unique tool. Before TEE was available for intra-operative use, significant residual abnormalities were frequently not detected. The result was often substantial post-operative morbidity and mortality and sometimes the need for re-operation. According to practice guidelines established by the Society of Cardiovascular Anesthesiologists and the American Society of Anesthesiologists, there is strong evidence for the usefulness of TEE in surgery for congenital heart disease because it significantly improves the clinical outcome of these patients. Before surgical correction, TEE helps confirm diagnosis and spot any additional lesion, while after the surgical correction, it provides baseline parameters for comparison after the surgical correction.

Clinical analysis of unroofed coronary sinus syndrome with atrioventricular canal defect / 中华外科杂志

<p><b>OBJECTIVE</b>To analyze symptoms, associated anomalies, diagnostic approach, and surgical procedures in patients with unroofed coronary sinus syndrome and atrioventricular canal defect.</p><p><b>METHODS</b>The clinical data of 20 patients with unroofed coronary sinus syndrome from September 1999 to October 2007 were reviewed retrospectively. There were 10 male and 10 female patients. The age ranged from 6 months to 38 years old, with a mean of (11.4 +/- 11.0) years old. The body weight ranged from 6.7 to 73.0 kg, with a mean of (28.4 +/- 21.3) kg. There were 18 cases of partial atrioventricular canal defect, 2 cases of complete atrioventricular canal defect, and 12 cases of common atrium. The initial diagnosis of unroofed coronary sinus syndrome was made by the surgeon at repair of a partial or complete atrioventricular canal defect in 20 patients. Complex unroofed coronary sinus with left superior vena cava (LSVC) directly draining into the left atrium was found in 11 cases, 1 case of LSVC was ligated, 10 cases were reconstructed the intraatrial baffle or the intracardiac tunnel to drain LSVC to right atrium. The other 9 patients with simple unroofed coronary sinus were repaired with other procedures. The associated cardiac lesions were corrected concomitantly.</p><p><b>RESULTS</b>Death occurred in 1 patient with complex congenital cardiac disease due to pulmonary infection. In the 14 early survivors, who had been followed up from 4 months to 3 years, there was no death and severe complications.</p><p><b>CONCLUSIONS</b>When associated with a partial or complete atrioventricular canal defect, LSVC and a common atrium, unroofed coronary sinus syndrome should be considered as a possible additional finding. Repair according to the type of unroofed coronary sinus syndrome is effective.</p>

Case Report of Partial Endocardial Cushion Defect with Mild Pulmonary Hypertension in Old Age / 대한흉부외과학회지

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.

Case Report of Partial Endocardial Cushion Defect with Mild Pulmonary Hypertension in Old Age / 대한흉부외과학회지

The partial endocardial cushion defect including ostium primum atrial septal defect and anterior mitral leaflet cleft, presents less significant clinical symptoms than complete endocardial cushion defect. But, as mitral insufficiency develops, cardiomegaly, congestive heart failure, pulmonary arterial hypertension appear. So, partial endocardial cushion defect has poor prognosis and is rarely seen in elderly patients. A 67 years old woman admitted at our hospital for operative treatment with partial endocardial cushion defect. She had increased pulmonary pressure of 45/22 mmHg, mean 32 mmHg. She had repair of ostium primum defect with patch, and the mitral valve was treated with valve replacement. Because advanced atrioventricular block developed postoperatively, she received permanent pacemaker.

Hallazgos y resultados de los defectos septales auriculoventriculares diagnosticados en la vida fetal / Findings and results of atrioventricular septal defects diagnosed in fetal life

Los hallazgos y resultados de los casos con defectos septales auriculoventriculares diagnosticados en la vida fetal comunicados en un estudio previo se compararon con nuestra nueva serie de casos y con otros informes bibliográficos respecto de los resultados prenatales y posnatales de esta lesión.

Features and outcome of cases with atrioventricular septal defect diagnosed in fetal life reported in a previous study are compared with our new cases and with other reports in the literature regarding prenatal and postnatal outcome in this lesion.