Caso clínico: adulto con anomalía de Ebstein / Clinical case: adult with Ebstein anomaly

Introducción: la anomalía de Ebstein se caracteriza por la implantación baja de las valvas septal y posterior de la tricúspide; por lo tanto, la aurícula derecha es amplia y su ventrículo es pequeño. Al ser una patología bastante rara en nuestro medio se encuentra subdiagnosticada y no recibe el tratamiento oportuno. Por ende, la importancia de conocerla para no retrasar su manejo clínico quirúrgico.Caso clínico: paciente de 46 años de edad, masculino, con antecedente de hipoacusia congénita, presentó disnea (clase funcional III) más palpitaciones y dolor torácico atípico hace 1 año aproximadamente. Al examen físico evidenció soplo holosistólico en foco tricuspídeo y uso moderado de musculatura accesoria. Al ecocardiograma transesofágico, se observó arritmia auricular más implante bajo de las valvas de la tricúspide y se inició tratamiento clínico más ablación del flutter. En la evolución presentó mejoría de la sintomatología y dependiendo del deterioro de la clase funcional, tratamiento quirúrgico Conclusiones: en el presente caso se ajustó tratamiento clínico y ablación del flutter de acuerdo a sintomatología del paciente, mostrando mejoría (AU);

Introduction: Ebstein's anomaly is characterized by low implantation of the septal and posterior tricuspid leaflets; therefore, the right atrium is wide and its ventricle is small. Being a rather rare pathology in our environment, it is underdiagnosed and does not receive the appropriate treatment. Therefore, it is important to know it to avoid a delay in its clinical surgical management.Clinical case: The patient is 46-year-old male, with a history of congenital hearing loss, who presented dyspnea (functional class III) plus palpitations and atypical chest pain approximately 1 year ago. In the physical examination, he revealed a holosystolic murmur in the tricuspid focus and moderate use of accessory muscles. A transesophageal echocardiogram revealed atrial arrhythmia plus implantation of the tricuspid leaflets and it was started a clinical treatment plus flutter ablation. In the evolution he presented improvement of the symptoms and depending on the deterioration of the functional class, surgical treatment.Conclusions: in this case, clinical treatment and flutter ablation were adjusted according to the patient's symptoms, showing improvement (AU);

Ebstein's anomaly: "The one and a half ventricle heart"

Abstract Objective: Ebstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV). Methods: 22 consecutive patients undergoing surgery for Ebstein's anomaly were studied. Echocardiography was performed to assess the type and severity of the disease, tricuspid annular dimension and its 'Z' score. Patients were operated by a modification of the cone repair, with addition of annuloplasty, bidirectional cavopulmonary shunt (BCPS) and right reduction atrioplasty to provide a comprehensive repair. TTA 'Z' score was correlated later with postplication indexed residual RV volume. Results: There was one (4.5%) early and no late postoperative death. There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P<0.001). Residual RV volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation (rho −0.83) between TTA 'Z' score and indexed residual RV volume. During the follow-up of 20.54±7.62 months, the functional class improved from 2.59±0.7 to 1.34±0.52 (P<0.001). Conclusion: In Ebstein's anomaly, a higher TTA 'Z' score correlates with a lower postplication indexed residual RV volume. Hence, a complete trileaflet repair with offloading of RV by BCPS (when the TTA 'Z' score is >2) is recommended. The short-term outcomes of our technique are promising.

Anomalía de Ebstein en diagnóstico prenatal / Ebstein's anomaly in prenatal diagnosis

Se presentó una gestante de 26 años de edad, mestiza, con antecedente de salud aparente evaluada en consulta de asesoramiento genético comunitaria con riesgo incrementado por exposición a teratógenos y mutágenos, que en la ecografía fetal del segundo trimestre se identificaron alteraciones cardiovasculares que pudieron estar relacionadas con anomalía de Ebstein. Este diagnóstico fue confirmado en el Centro Provincial de Genética Médica de Guantánamo y, posteriormente, en Santiago de Cuba. A pesar de ser una cardiopatía congénita poco frecuente, se consideraron: la intensificación de las acciones en la atención prenatal precoz de las gestantes en la comunidad, la realización y cumplimiento de los programas prenatales existentes en nuestro país, principalmente los ultrasonidos prenatales que continúan siendo el examen complementario de elección y ofrecer el asesoramiento genético específico en las familias afectadas(AU)

A 26-year-old mixed-race pregnant woman with a history of apparent health evaluated in a community genetic counseling clinic with increased risk due to exposure to teratogens and mutagens was presented. Fetal echography of the second trimester identified cardiovascular alterations that may have been related to Ebstein's anomaly. Diagnosis that was confirmed in the Provincial Center of Medical Genetics of Guantanamo later was confirmed in Santiago de Cuba. Despite being a rare congenital heart disease, the following were considered: the intensification of the actions in the prenatal care of pregnant women in the community, the realization and fulfillment of the prenatal programs existing in our country, mainly the prenatal ultrasounds that continue being the complementary examination of choice and offer specific genetic counseling in affected families(AU)

Anomalía de Ebstein en diagnóstico prenatal / Anomalia de Ebstein no diagnóstico pré-natal / Ebstein's anomaly in prenatal diagnosis

Se presentó una gestante de 26 años de edad, mestiza, con antecedente de salud aparente evaluada en consulta de asesoramiento genético comunitaria con riesgo incrementado por exposición a teratógenos y mutágenos, que en la ecografía fetal del segundo trimestre se identificaron alteraciones cardiovasculares que pudieron estar relacionadas con anomalía de Ebstein. Este diagnóstico fue confirmado en el Centro Provincial de Genética Médica de Guantánamo y, posteriormente, en Santiago de Cuba. A pesar de ser una cardiopatía congénita poco frecuente, se consideraron: la intensificación de las acciones en la atención prenatal precoz de las gestantes en la comunidad, la realización y cumplimiento de los programas prenatales existentes en nuestro país, principalmente los ultrasonidos prenatales que continúan siendo el examen complementario de elección y ofrecer el asesoramiento genético específico en las familias afectadas(AU)

A 26-year-old mixed-race pregnant woman with a history of apparent health evaluated in a community genetic counseling clinic with increased risk due to exposure to teratogens and mutagens was presented. Fetal echography of the second trimester identified cardiovascular alterations that may have been related to Ebstein's anomaly. Diagnosis that was confirmed in the Provincial Center of Medical Genetics of Guantanamo later was confirmed in Santiago de Cuba. Despite being a rare congenital heart disease, the following were considered: the intensification of the actions in the prenatal care of pregnant women in the community, the realization and fulfillment of the prenatal programs existing in our country, mainly the prenatal ultrasounds that continue being the complementary examination of choice and offer specific genetic counseling in affected families(AU)

A presentou-se uma gestante de 26 anos de idade, mestiza, com antecedente de saúde aparente avaliada em consulta de assessoramento genético comunitária com risco incrementado por exposição a teratógenos e mutágenos, que na ecografia fetal do segundo trimestre se identificaram alterações cardiovasculares que puderam estar relacionadas com anomalia de Ebstein. Diagnóstico que foi confirmado no Centro Provincial de Genética Médica de Guantánamo e, posteriormente, em Santiago de Cuba. Apesar de ser uma cardiopatía congênita pouco frequente, consideraram-se: a intensificação das ações na atenção prenatal precoz das gestantes na comunidade, a realização e cumprimento dos programas prenatales existentes em nosso país, principalmente os ultrasonidos prenatales que continuam sendo o exame complementar de eleição e oferecer o assessoramento genético específico nas famílias afetadas(AU)

Anomalia de Ebstein: relato de caso / Ebstein Anomaly: a case report

Justificativa e objetivos: a anomalia de Ebstein é a doença congênita mais comum da valva tricúspide. É caracterizada por aderência de porção variável das cúspides posterior e septal de uma valva tricúspide redundante para o interior da parede ventricular direita e implantada mais caudalmente em direção ao ápice. Suas manifestações clínicas bem como seu tratamento são variáveis conforme o grau de anomalia valvar. Relato do caso: paciente de 11 anos com diagnóstico de anomalia de Ebstein desde o período pós-natal imediato. Apresentava percentil 10 de peso e estatura; desenvolvimento normal. Foi indicada intervenção cirúrgica devido a quadro de dispneia aos médios esforços, piora da cianose e diaforese. Submetido à plicatura do VD atrializado, plastia da valva tricúspide com diminuição do anel tricúspide, correção da CIA e CIV sob anestesia geral balanceada associada à peridural torácica. Antes de entrar em CEC, apresentou TSV após manipulação do AD. A CEC durou 89 min e o CAo, 60 min. Saiu de CEC com adrenalina a 0,3 mcg/kg/min e milrinona a 0,75 mcg/kg/min. Aproximadamente 15 minutos após receber uma bolsa de concentrado de hemácias, duas unidades de plaquetas e 10 mL de gluconato de cálcio 10 mL a 10%, cursou com TV sem pulso revertida a ritmo juncional após a segunda desfibrilação. Foi transferido para o CTI em ritmo juncional, FC: 130 BPM, PIA: 56/40 mmHg. Conclusões: portadores de anomalia de Ebstein com mais de 10 anos frequentemente apresentam arritmias cardíacas. Há forte associação com vias de condução acessórias ­ fenômenos de pré-excitação ­ WPW (ressalta-se que no caso em questão o estudo eletrofisiológico pré-CEC não evidenciou qualquer via anômala ou fenômeno de pré-excitação). É comum a coexistência de defeitos septais. O tratamento clínico-cirúrgico deve ser individualizado, sendo mandatório conhecer alterações cardiovasculares e hemodinâ- micas associadas para otimizar o ato anestésico-cirúrgico

Justification and objectives: Ebstein anomaly is the most common congenital disorder in the tricuspid valve. It is characterized by a variable portion of the posterior and septal leaflets of a redundant tricuspid valve adhering the right ventricular wall and caudally towards the apex. Its clinical manifestations and treatments vary according to the degree of valve anomaly. Case report: An eleven-year-old patient diagnosed with Ebstein anomaly since the immediate postnatal period. The patient was in the 10th percentile of height and weight, and had normal development. Surgery was indicated due to effort dyspnea, worsening cyanosis, and diaphoresis. The patient underwent plicature of the atrialized right ventricle; tricuspid valve plastic surgery to reduce the tricuspid ring, correction of interatrial and interventricular communication with the aid of balanced, general anesthesia associated with thoracic epidural. Before initial- izing extracorporeal circulation (ECC), the patient had supraventricular tachycardia upon manipulation of the right atrium. ECC and aortic clamping lasted 89 and 60 minutes respectively. CEC stopped with 0.3 mcg/ kg/min adrenalin and 0.75 mcg/kg/min milrinone. Approximately 15 minutes after receiving a red blood cell concentrate bag, two plaque units and 10 mL of 10 % calcium gluconate, he had ventricular tachycardia without pulse that was reverted to junctional rhythm upon the second defibrillation.The patient was transferred to ICU in junctional rhythm, heart rate: 130 BPM; intra-abdominal pressure: 56/40 mmHg. Conclusions: Elbstein anomaly patients aging over 10 years old frequently have cardiac arrhythmias. There is strong association with accessory conduction pathways ­ pre-excitation phenomena ­ WPW (in the case under scrutiny, a pre-CEC electrophysiological study did not point to any anomalous pathway or pre-excitation phenomena). Coexistence of septal defects is common. Clinical and surgical treatment should be customized, and the practitioners must be aware of the associated cardiovascular and hemodynamic alterations in order to optimize both anesthetic and surgical procedures.

Normal offset of the tricuspid septal leaflet in Thai fetuses.

OBJECTIVE: To quantify the correlation between gestational age and the normal distance between the insertion of the medial leaflets of the mitral valve and tricuspid valve in Thai fetuses. MATERIAL AND METHOD: A prospective study was constructed using an apical 4-chamber view at end diastole to measure the mitral valve-tricuspid valve distance (MTD) in 169 confirmed-dated singleton normal fetuses between 18 and 40 weeks of gestation at King Chulalongkorn Memorial Hospital from August 2005 to July 2006. The recorded distances were plotted against gestational ages. The 5th, 50th, and 95th centile of MTD were calculated and the relationship between MTD and gestational age was determined with regression model. One hundred and forty-seven measurements were validated for analyses. RESULTS: Regression analysis demonstrated a linear correlation of MTD with gestational age with each increase of 1 week in gestational age, there was an increase of 0.12 mm of MTD (p <. 001). The linear regression curve that correlated MTD with gestational age showed a gradual slope (r = 0.84; p < 0.001). The MTD range from 1.3 to 5.1 mm (mean +/- SD = 2.6 +/- 0.9). A significant difference in MTD was noted on comparing to the data in Western study. Intra-observer reliability was 0.92. CONCLUSION: The present findings support the positive correlation between MTD and advancing gestational age. However, clinically significant differences of MTD between the presented population and Caucasian fetuses underline the need for employing a locally derived data on assigning the fetal cardiac abnormality.

Transvenous ventricular pacing options in Ebstein's anomaly.

Transvenous pacing in patients with Ebstein's anomaly is challenging due to anatomical abnormalities of the tricuspid valve and right heart chambers. This paper describes the various transvenous ventricular lead placement options for permanent pacing in patients with Ebstein's anomaly. In Ebstein's anomaly, stable long-term ventricular pacing can be achieved by positioning the lead either in the atrialised right ventricle, true right ventricle or the cardiac venous system. The pitfalls and advantages of pacing from these sites with the electrocardiographic and chest X-ray appearances are described.