Superior vena cava obstruction after the Glenn procedure

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.

Diagnóstico prenatal de atresia tricuspidea / Prenatal diagnosis of tricuspid atresia

La atresia tricuspidea es una cardiopatía rara caracterizada por oclusión de la válvula tricúspide y asociada a hipoplasia de ventrículo derecho y estenosis pulmonar. Clásicamente se presenta con un defecto en septun ventricular. El diagnóstico puede establecerse con ecocardiografía fetal y usualmente se sospecha ante una imagen de cuatro cámaras anormal.

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.

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.(AU)

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.(AU)

Abordaje simplificado para la ablación de la taquicardia por reentrada intranodal en paciente con atresia tricuspídea y Fontan extracardiaco / Simplified approach for ablation of nodal reentrant tachycardia in a patient with tricuspid atresia and extracardiac fontan palliation

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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].

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.

Tromboembolismo pulmonar masivo en paciente previamente anticoagulado y con antecedente de intervención de Fontan / Massive pulmonary thromboembolism in patient treaty with anticoagulant previously and with intervention of Fontan

Presentamos el caso de un varón de 18 años sometido a los tres años de vida a la intervención de Fontan por atresia tricuspídea, que ingresó en nuestro servicio de Cardiología por descompensación de insuficiencia cardíaca. Tras estudio exhaustivo se diagnosticó de tromboembolismo pulmonar masivo pese a estar anticoagulado. Hemos realizado una búsqueda bibliográfica de casos similares y aportamos nuestra opinión sobre las recomendaciones sobre anticoagulación en estos pacientes

We present the case of a 18 years-old male subjected to at the age of three years old to the Fontan operation for tricuspid atresia, that entered in our Cardiology Unit for descompensated heart failure. After exhaustive study, a massive pulmonary thromboembolism was diagnosed in spite of the patient was treated with anticoagulant drugs previously. We have carried out a bibliographical search of similar cases and we contribute our opinion on the recommendations about anticoagulation in these patients

Dupla via de saída de ventriculo esquerdo. Diagnóstico ecocardiográfico

This is a case report of a double-outlet left ventricle associated with tricuspid atresia and hypoplasia of the right ventricle, diagnosed during echocardiography with color-flow imaging, in a three-month-old child who presented with fatigue and cyanosis. The child underwent palliative pulmonary arterial banding without an invasive procedure, and showed sustained improvement during follow-up

Anomalia de Ebstein com valva tricúspide imperfurada diagnóstico pré-natal / Ebstein's anomaly with imperforate tricuspid valve. Prenatal diagnosis

Ebstein's anomaly is an uncommon congenital heart defect, with a prevalence of 0.3-0.5 percent. Its association with an imperforate tricuspid valve is an even more rare situation (less than 10 percent of cases). Prenatal diagnosis of this association by means of fetal echocardiography has not been reported. We describe here this association diagnosed before birth and confirmed after birth. The diagnostic potential and importance of fetal echocardiography during prenatal evaluation of cardiac malformations allows for adequate perinatal planning and management, with an obvious impact on morbidity and mortality