Acute Liver Failure due to Altered Fontan Circulation in a Patient With Tricuspid Atresia: A Case Report.

Tricuspid atresia is a congenital malformation of the tricuspid valve resulting in a lack of blood flow between the right atrium and the right ventricle. Management generally involves staged surgical intervention enabling affected individuals to survive into adulthood. Although surgical intervention greatly improves morbidity and mortality in this patient population, there are many long-term complications associated with the creation of a surgical shunt. We report a case of a 33-year-old male with tricuspid atresia who underwent Fontan surgery as a child and presented to our hospital with acute liver failure.

[Stent ductal implant by axillary via in a newborn with ductal dependent pulmonary blood flow]. / Implante de stent ductal por vía axilar en neonato con cardiopatía congénita con flujo pulmonar dependiente del conducto arterioso.

INTRODUCTION: Ductus arteriosus stenting is a palliative alternative for neonates with ductal-dependent pulmonary flow. OBJECTIVE: To present an alternative of arterial access for percutaneous coronary intervention in neonates. CLINICAL CASE: A term neonate with low weight diagnosed with pulmonary atresia with intact ventricular septum and severe hypoplasia of the tricuspid valve with dependent coronary circu lation. Due to the surgical risk and femoral artery damage and the anatomy of the ductus arteriosus, a left axillary arterial puncture was decided where a 3.5-millimeter coronary stent was successfully placed. The patient developed an axillary spasm that resolved spontaneously. CONCLUSION: Alternati ve arterial access other than the femoral artery route is an option for neonates with high surgical risk and low birth weight.

Electro-vectorcardiographic and electrophysiological aspects of Ebstein's anomaly.

Ebstein's anomaly is a congenital heart disease where the most important anatomic feature is the inferior displacement of the tricuspid valve leaflets. Vectorcardiographic features are mainly forgotten and electrocardiographic features may be unrecognized by cardiologists handling adult patients.

Tricuspid valve dysplasia in a domestic feline: case report / Displasia valvar tricúspide em um felino doméstico: relato de caso

Tricuspid valve dysplasia (TVD) is a congenital heart defect described in dogs and cats; however, in Brazil there are no reports of this condition in cats. Therefore, our goal was to report a case of TVD in a domestic cat. A four-year-old, female, domestic short hair cat that was seen at the Cardiology Service of the Veterinary Teaching Hospital, University of São Paulo, for apathy, appetite loss and dyspnea for five days. During physical examination, dyspnea with a restrictive respiratory pattern due to pleural effusion was observed. Thoracocentesis was performed and 450 mL of serosanguineous fluid was drained. Two-dimensional echocardiography in the right parasternal short-axis plane at the level of the papillary muscles showed right ventricular dilatation and paradoxical septal motion. On the left parasternal apical four-chamber view, significant dilatation of the right chambers, loss of mobility of the septal leaflet of the tricuspid valve and a thickened mural leaflet chordae with anomalous insertion were observed. Based on clinical and echocardiographic aspects, a diagnosis of TVD was given. Treatment was initiated with enalapril 0.5 mg/kg, furosemide 0.5 mg/kg and pimobendan 0.3 mg/kg every 12 hours, all per os. The patient experienced remission of clinical manifestations and survived for 50 days after diagnosis.(AU)

Displasia valvar tricúspide (DVT) é um defeito cardíaco congênito descrito em cães e gatos. Entretanto no Brasil ainda não há relato desta cardiopatia em felinos. Desta forma, objetivou-se relatar um caso de DVT em um felino doméstico. Uma gata de pelo curto, com quatro anos de idade foi atendida no Serviço de Cardiologia do Hospital Veterinário da Universidade de São Paulo, apresentando apatia, perda de apetite e dispneia há cinco dias. Durante o exame físico observou-se dispneia com padrão respiratório restritivo devido a presença de efusão pleural. Foram drenados 450 mL de líquido serosanguinolento por meio de toracocentese. O ecocardiograma no modo bidimensional, pela janela paraesternal direita, ao eixo curto transversal ao nível dos músculos papilares, revelou hipertrofia excêntrica do ventrículo direito e movimento septal paradoxal. Pela vista apical quatro câmaras, na janela paraesternal esquerda, observou-se importante remodelamento de câmaras cardíacas direitas. A valva tricúspide apresentou-se com perda de mobilidade de sua cúspide septal e espessamento de cordoalha tendínea da cúspide mural, com inserção anômala. Com base nos aspectos clínicos e ecocardiográficos, instituiu-se o dianóstico de DVT. Iniciou-se o tratamento com enalapril (0,5 mg/kg), furosemida (0,5 mg/kg) e pimobendan (0,3 mg/kg), pela via oral a cada 12 horas. O paciente apresentou remissão das manifestações clínicas, sobrevivendo por 50 dias após o diagnóstico.(AU)

Insuficiência cardíaca congestiva secundária a displasia de tricúspide em cão - relato de caso / Congestive heart failure secondary to tricuspid dysplasia in dog - case report / Insuficiencia cardíaca congestiva secundaria a displasia tricúspide en perro - relato de caso

A displasia de tricúspide (DT) é uma afecção congênita caracterizada pelo subdesenvolvimento dessa valva. Quase a totalidade dos pacientes que apresentam essa condição são de raças grandes, sendo rara em cães sem raça definida (SRD), em especial de pequeno porte. A anamnese e o exame físico são essenciais para o diagnóstico, que deve ser confirmado por exames complementares. Relata-se o caso de uma cadela SRD de cinco meses que apresentava dispneia, ascite e sopro cardíaco. Suspeitou-se de cardiopatia congênita, razão pela qual foram requisitados exames complementares. Realizou- se um ecodopplercardiograma no qual se constatou dilatação de câmaras, hipertensão pulmonar e DT. A conduta terapêutica incluiu a administração de furosemida, benazepril, espironolactona, pimobendan e sildenafil. Houve ótima resposta à terapia, e a paciente continua estável após dezessete meses de tratamento.

Tricuspid dysplasia (TD) is a genetic ailment characterized by the underdevelopment of the tricuspid valve. The disease is diagnosed almost exclusively in large breed dogs, rarely occurring in small or mixed breed animals. Initial clinical suspicion is generally confirmed by laboratory and image examination. In this report, we describe the case of a five-month-old female mixed-breed dog that initially presented dyspnea, ascites, and cardiac murmur. Clinical suspicion led to complementary examination by doppler echocardiography, which revealed chamber dilation, pulmonary hypertension, and TD. Therapeutic conduct included administration of furosemide, benazepril, spironolactone, pimobendan and sildenafil. The patient responded well to treatment and is still stable seventeen months after therapy onset.

La displasia tricúspide (DT) es una enfermedad congénita caracterizada por falta de desarrollo de esa válvula. Casi todos los pacientes que presentan esta condición son de razas grandes, siendo rara en perros mestizos, y particularmente en aquellas razas pequeñas. La anamnesis y el examen físico son esenciales para et diagnóstico, que debe ser confirmado a través de exámenes complementarios. Se relata el caso de una perra mestiza de cinco meses que presentaba disnea, ascitis y soplo cardíaco. Se sospechó de una cardiopatia congénita, siendo indicada la realización de exámenes complementarios . Fue realizado un eco doppler cardiograma donde se comprobó la dilatación de las cámaras , hipertensión pulmonar y DT. El tratamiento consistió en la administración de furosemida, benazepril. Espironolactona, pimodenban y sildenafil. Hubo una excelente respuesta a la terapia, y la paciente continúa estable después de dezesiete meses de tratamiento.

Insuficiência cardíaca congestiva secundária a displasia de tricúspide em cão - relato de caso / Congestive heart failure secondary to tricuspid dysplasia in dog - case report / Insuficiencia cardíaca congestiva secundaria a displasia tricúspide en perro - relato de caso

A displasia de tricúspide (DT) é uma afecção congênita caracterizada pelo subdesenvolvimento dessa valva. Quase a totalidade dos pacientes que apresentam essa condição são de raças grandes, sendo rara em cães sem raça definida (SRD), em especial de pequeno porte. A anamnese e o exame físico são essenciais para o diagnóstico, que deve ser confirmado por exames complementares. Relata-se o caso de uma cadela SRD de cinco meses que apresentava dispneia, ascite e sopro cardíaco. Suspeitou-se de cardiopatia congênita, razão pela qual foram requisitados exames complementares. Realizou- se um ecodopplercardiograma no qual se constatou dilatação de câmaras, hipertensão pulmonar e DT. A conduta terapêutica incluiu a administração de furosemida, benazepril, espironolactona, pimobendan e sildenafil. Houve ótima resposta à terapia, e a paciente continua estável após dezessete meses de tratamento.(AU)

Tricuspid dysplasia (TD) is a genetic ailment characterized by the underdevelopment of the tricuspid valve. The disease is diagnosed almost exclusively in large breed dogs, rarely occurring in small or mixed breed animals. Initial clinical suspicion is generally confirmed by laboratory and image examination. In this report, we describe the case of a five-month-old female mixed-breed dog that initially presented dyspnea, ascites, and cardiac murmur. Clinical suspicion led to complementary examination by doppler echocardiography, which revealed chamber dilation, pulmonary hypertension, and TD. Therapeutic conduct included administration of furosemide, benazepril, spironolactone, pimobendan and sildenafil. The patient responded well to treatment and is still stable seventeen months after therapy onset.(AU)

La displasia tricúspide (DT) es una enfermedad congénita caracterizada por falta de desarrollo de esa válvula. Casi todos los pacientes que presentan esta condición son de razas grandes, siendo rara en perros mestizos, y particularmente en aquellas razas pequeñas. La anamnesis y el examen físico son esenciales para et diagnóstico, que debe ser confirmado a través de exámenes complementarios. Se relata el caso de una perra mestiza de cinco meses que presentaba disnea, ascitis y soplo cardíaco. Se sospechó de una cardiopatia congénita, siendo indicada la realización de exámenes complementarios . Fue realizado un eco doppler cardiograma donde se comprobó la dilatación de las cámaras , hipertensión pulmonar y DT. El tratamiento consistió en la administración de furosemida, benazepril. Espironolactona, pimodenban y sildenafil. Hubo una excelente respuesta a la terapia, y la paciente continúa estable después de dezesiete meses de tratamiento.(AU)

[Morphometric and morphologic studies in right ventricular hypoplasia]. / Hipoplasia ventricular derecha. Estudios morfométrico y morfológico.

In hypoplastic right ventricle the dysplasia of the tricuspid valve (TV) has repercussions on the development of the right ventricle. This paper documents the results of an anatomopathologic and morphometric studies of the tricuspid valve and the right ventricle in 25 hearts with this cardiopathy, which were morphologically analyzed using the segmentary sequential system. The following measurements were made: the annulus of the tricuspid valve, the thickness of the walls of the right ventricle, the ventricular septum and the distances from the atrioventricular-apex (AV-A) and from the apex-pulmonary valve (A-PV). The values obtained were compared with those of normal hearts of equivalent ages; the morphologic features of the tricuspid valve and the right ventricle were described. The thickness of the anterior and posterior walls of righ ventricle was between 2 and 10 times greater than normal. The tickness of the ventricular septum was 1 to 3 times greater than normal and the anterior wall of the infundibulum from 1 to 4 times greater than normal; the AV-A and A-PV distances were reduced in a half of the normal length. The great reduction of the right ventricular cavity was found to be related to the great hypertrophy of the right ventricular walls. The TV was dysplastic with Ebstein's anomaly (72%). The size of the tricuspid valve was small and it was a good indicator for the size of the right ventricle. Morphometric studies are useful because they quantitatively document alterations in the dimensions of valves, walls and diameters of the cardiac chambers in congenital heart disease.

Hipoplasia ventricular derecha: estudios morfométrico y morfológico / Morphometric and morphologic studies in right ventricular hypoplasia

In hypoplastic right ventricle the dysplasia of the tricuspid valve (TV) has repercussions on the development of the right ventricle. This paper documents the results of an anatomopathologic and morphometric studies of the tricuspid valve and the right ventricle in 25 hearts with this cardiopathy, which were morphologically analyzed using the segmentary sequential system. The following measurements were made: the annulus of the tricuspid valve, the thickness of the walls of the right ventricle, the ventricular septum and the distances from the atrioventricular-apex (AV-A) and from the apex-pulmonary valve (A-PV). The values obtained were compared with those of normal hearts of equivalent ages; the morphologic features of the tricuspid valve and the right ventricle were described. The thickness of the anterior and posterior walls of righ ventricle was between 2 and 10 times greater than normal. The tickness of the ventricular septum was 1 to 3 times greater than normal and the anterior wall of the infundibulum from 1 to 4 times greater than normal; the AV-A and A-PV distances were reduced in a half of the normal length. The great reduction of the right ventricular cavity was found to be related to the great hypertrophy of the right ventricular walls. The TV was dysplastic with Ebstein's anomaly (72%). The size of the tricuspid valve was small and it was a good indicator for the size of the right ventricle. Morphometric studies are useful because they quantitatively document alterations in the dimensions of valves, walls and diameters of the cardiac chambers in congenital heart disease.

Electrocardiographic characteristics of patients with Ebstein's anomaly before and after ablation of an accessory atrioventricular pathway.

UNLABELLED: The abnormal development of the tricuspid valve in patients with Ebstein's anomaly results in several activation abnormalities including delayed intraatrial conduction, right bundle branch block (RBBB), and ventricular preexcitation. The aim of the present study was to define the ECG characteristics before and after ablation of an accessory A-V pathway (AP) in patients with Ebstein's anomaly. METHODS: A series of 226 consecutive patients with Ebstein's anomaly was studied. Sixty-four patients (28%) had documented tachycardia. Thirty-three patients with recurrent tachycardia were found to have a single right-sided AP that was successfully ablated (study group). Thirty patients without tachycardia served as the control group. RESULTS: Only 21 of 33 patients (62%) had a typical ECG pattern of preexcitation. In addition, none of the patients had an ECG pattern of RBBB during sinus rhythm. In contrast, 28 of 30 (93%) patients in the control group had RBBB (P < 0.001). Radiofrequency catheter ablation resulted in appearance of RBBB in 31 of 33 (94%) patients. The absence of RBBB in patients with Ebstein's anomaly and recurrent tachycardia had a 98% sensitivity and 92% specificity for the diagnosis of an AP. The positive predictive value was 91% (0.77, 0.97 CI 95%) and the negative predictive value was 98% (0.85, 0.99 CI 95%). CONCLUSION: One-third of patients with Ebstein's anomaly and symptomatic tachyarrhythmias have minimal or absent ECG features of ventricular preexcitation. In these patients, the absence of RBBB pattern is a strong predictor of an AP.

Thrombolytic treatment as first option in recurrent tricuspid prosthetic valve thrombosis and Ebsteins anomaly

Ebsteins anomaly is the most frequent cause of congenital tricuspid regurgitation. The coexistence of a mechanical heart prosthesis in a low-pressure circuit and poor compliance in the anticoagulant therapy contributed decisively to the appearance of recurrent mechanical heart valve thrombosis in these patients. A 49 years old female patient is reported where thrombolytic therapy with recombinant Streptokinase (TT-rSK) was the first treatment choice in seven recurrent episodes of prosthetic valve thrombosis(AU)

La anomalía de Ebstein es la causa más frecuente de regurgitación tricúspide congénita. La coexistencia de una prótesis mecánica en un centro de baja presión del circuito y los pobres en el cumplimiento de la terapia anticoagulante contribuido de forma decisiva a la aparición recurrente de válvulas cardíacas mecánicas trombosis en estos pacientes. A 49 años de edad del paciente es informado de que el tratamiento trombolítico con estreptoquinasa recombinante (TT-RSK) fue el primer tratamiento de elección en siete episodios recurrentes de trombosis de válvula protésica

Thrombolytic treatment as first option in recurrent tricuspid prosthetic valve thrombosis and Ebstein's anomaly.

Ebstein's anomaly is the most frequent cause of congenital tricuspid regurgitation. The coexistence of a mechanical heart prosthesis in a low-pressure circuit and poor compliance in the anticoagulant therapy contributed decisively to the appearance of recurrent mechanical heart valve thrombosis in these patients. A 49 years old female patient is reported where thrombolytic therapy with recombinant Streptokinase (TT-rSK) was the first treatment choice in seven recurrent episodes of prosthetic valve thrombosis.

Perforación tricuspídea traumática: presentación de un caso / Traumatic tricuspid valve perforation: case report

Esta comunicación describe el caso de un paciente que ingresa en la guardia del hospital con una herida de arma de fuego a nivel precordial, en paro cardiorrespiratorio. La lesión interesó el ventrículo derecho, la aurícula derecha y el pulmón derecho. Dichos daños se repararon quirúrgicamente por cardiorrafia y drenaje del hemotórax derecho. El ecocardiograma en el posoperatorio mediato descubrió una perforación de una valva de la tricúspide, que produjo insuficiencia tricuspídea leve. Se realizan diferentes consideraciones referentes a los traumatismos cardíacos

Perforación tricuspídea traumática: presentación de un caso / Traumatic tricuspid valve perforation: case report

Esta comunicación describe el caso de un paciente que ingresa en la guardia del hospital con una herida de arma de fuego a nivel precordial, en paro cardiorrespiratorio. La lesión interesó el ventrículo derecho, la aurícula derecha y el pulmón derecho. Dichos daños se repararon quirúrgicamente por cardiorrafia y drenaje del hemotórax derecho. El ecocardiograma en el posoperatorio mediato descubrió una perforación de una valva de la tricúspide, que produjo insuficiencia tricuspídea leve. Se realizan diferentes consideraciones referentes a los traumatismos cardíacos (AU)

Insuficiência tricúspide pós trauma associada a agnesia de músculo papilar anterior / Post-traumatic tricuspid insufficiency associated with agenesis of the anterior papillary muscle

Relatamos o caso de paciente do sexo masculino com 36 anos de idade com sinais e sintomas de insuficiência cardíaca direita. A história revelou trauma torácico há aproximadamente cinco anos. Submetido a operaçäo para tratamento de insuficiência tricúspide, notou-se ausência do músculo papilar anterior da valva tricúspide, fenda na cúspide anterior e dilataçäo do anel tricuspídeo. Foi realizada sutura da fenda localizada na cúspide anterior e feita sua sustentaçäo utilizando-se tira de pericárdio bovino fixada na face atrial e base do músculo papilar posterior. A operaçäo foi completada com anuloplastia de Revuelta. O paciente obteve nítida melhora dos sintomas no pós-operatório imediato, mantendo-se em classe funcional I (NYHA), após 22 meses de evoluçäo

Cambios hemodinámicos y gasométricos en la anestesia para fístula de Blalock Taussing modificada / Hemodynamic and gasometric changes during anesthesia for Blalock Taussing Fistula

La fístula de Blalock Taussing modificada es un procedimiento quirúrgico cuya finalidad es aumentar la circulación pulmonar, en pacientes con cardiopatías congénitas complejas con flujo pulmunar disminuído. Durante su realización se han reportado complicaciones letales, por lo que se implementó un seguimiento hemodinámico y gasométrico en ocho pacientes con atresia tricuspídea, durante su periodo transanestésico. Se observó al término del estudio que la disminución de la presión arterial, frecuencia cardiaca, pH sanguíneo, así como desaturación y retención de CO2, se presentan de manera significativa y progresiva durante los minutos del pinzamiento de la arteria pulmonar, por lo que es éste el momento crítico de la cirugía, que debe vigilarse más estrecha y eficazmente para una intervensión terapéutica oportuna