Cor triatriatum sinister with left superior vena cava, unroofed coronary syndrome and valvular pulmonary stenosis / Cor triatriatum sinistrum con vena cava superior izquierda, síndrome coronario destechado y estenosis de la válvula pulmonar

Abstract A 25-year-old woman with a history of recent dyspnea and palpitation was admitted to our center. Transthoracic echocardiography (TTE) showed an echogenic and septal muscular ridge along the left atrium which were indicative of cor triatriatum sinister (CTS). Further evaluation with transesophageal echocardiography (TEE) showed that a lower chamber of divided left atrium receiving lower right inferior pulmonary vein, mitral valve, left superior vena cava (LSVC) and unroofed coronary sinus (CS). Also, the lower chamber had an unrestrictive communication with the right atrium. The upper accessory chamber receiving one left and one right upper pulmonary vein and communicated with the right atrium by a small atrial septal defect (ASD). However, upper and lower pulmonary venous systems separated directly from each other by a muscular ridge without the presence of any window or hole to allows blood flow between these two accessory chambers. Although the absence of septum associated with the presence of LSVC and unroofed CS makes our case a unique or very rare type of this complex anomaly in an adult case. In our case, surgical removal of dividing muscular membrane with redirection of LSVC and unroofed CS to the right atrium are warranted.

Resumen Se trata de una paciente de sexo femenino, de 25 años de edad, con antecedentes recientes de disnea y palpitaciones que ingresó a nuestro centro. Un ecocardiograma transtorácico (ETT) evidenció un reborde ecogénico muscular del tabique de la aurícula izquierda indicativo de cor triatriatum sinistrum (CTS). En un estudio más a fondo con ecocardiografía transesofágica (ETE) se evidenció que una cámara inferior de la aurícula izquierda dividida recibía la vena pulmonar inferior derecha, la válvula mitral, la vena cava superior izquierda (VCSI) y el seno coronario (SC) destechado. Además, la cámara inferior tenía comunicación libre con la aurícula derecha. La cámara superior secundaria recibía una vena pulmonar superior izquierda y una derecha y se comunicaba con la aurícula derecha a través de una comunicación interauricular (CIA) pequeña. Sin embargo, los sistemas venosos pulmonares superiores e inferiores se encontraban separados entre sí por un reborde muscular sin la presencia de alguna ventana u orificio que permitiera el flujo de sangre entre estas dos cámaras secundarias. La ausencia del tabique junto con la presencia de la VCSI y el SC destechado hacen que nuestro caso sea un tipo único o extremadamente inusual de esta anomalía compleja en un caso en un adulto. En este caso, se justifica la extirpación quirúrgica de la membrana muscular divisoria con redireccionamiento de la VCSI y el SC destechado a la aurícula derecha.

Triatriatum sinistrum em adulto assintomático: relato de caso / Cor triatriatum sinistrum in an asymptomatic adult: a case report

Cor triatriatum é um anomalia cardíaca congênita rara frequentemente diagnosticada na primeira infância. Este estudo de caso apresenta um adulto com um achado acidental de cor triatriatum sinistrum. Com base na apresentação clínica, o paciente foi tratado de forma conservadora. São apresentados achados de imagens ecocardiográficas de cor triatriatum sinistrum deste paciente juntamente de revisão narrativa da literatura sobre essa doença.(AU)

Cor triatriatum is a rare congenital heart anomaly often diagnosed in early childhood. This case study features an adult with an incidental finding of cor triatriatum sinistrum. Based on the clinical presentation, the patient was treated conservatively. Cor triatriatum sinistrum echocardiographic image findings of this patient are presented along with a narrative review of the literature about this disease. (AU)

Cor triatriatum sinister, una anomalía clásica pero un tanto olvidada: A propósito de un caso / Cor triatriatum sinister, a classic but somewhat forgotten anomaly: Apropos of a case

RESUMEN Un corazón con tres aurículas, que sumaba cinco cámaras en total, fue un hecho que sorprendió a la medicina hace ya más de un siglo. Este raro defecto cardíaco congénito tiene muy baja incidencia, lo cual hace que sea muy poco conocido por las jóvenes generaciones de médicos dedicados a las enfermedades cardiovasculares. El cor triatriatum sinister generalmente se diagnostica en la temprana infancia y es considerado una causa congénita de estenosis mitral. La conducta quirúrgica es de elección para poder liberar el obstáculo al adecuado flujo sanguíneo a través de la aurícula izquierda. Se presenta el caso de un adulto joven con síntomas típicos de estenosis mitral, sin aparentes elementos claves al examen físico y con un ecocardiograma que evidencia esta anomalía, apenas vista y publicada en nuestro medio en pacientes adultos.

ABSTRACT A heart with three atria, totaling five chambers in all, was a fact that surprised medicine more than a century ago. This rare congenital heart defect has a very low incidence, which makes it very little known to the young generations of doctors dedicated to cardiovascular diseases. The cor triatriatum sinister is usually diagnosed in early childhood, and it is considered a congenital cause of mitral stenosis. Surgical approach is the choice to release the obstacle in order to adequate blood flow through the left atrium. We present the case of a young adult with typical symptoms of mitral stenosis, without apparent key elements on the physical examination and that the echocardiogram showed this anomaly, hardly seen and published in our field in adult patients.

Cor triatriatum dexter y comunicación interauricular tipo seno venoso: Una asociación infrecuente / Cor triatriatum dexter and sinus venosus atrial septal defect: An infrequent association

RESUMEN El cor triatriatum es una malformación congénita poco frecuente, con una prevalecia de un 0,1% entre todas las anomalías congénitas cardíacas, donde el corazón queda dividido en tres atrios o aurículas. En el cor triatriatum dexter la aurícula derecha queda dividida, por una membrana fibromuscular, en dos partes, una proximal y otra distal, que se comunican o no entre sí. En la evolución natural de la enfemedad, los pacientes pueden permanecer asintomáticos hasta su diagnóstico o presentar manifectaciones cardiovasculares secundarias a insuficiencia cardíaca derecha y trastornos del ritmo. En el caso particular de aquellos que se encontraran sintomáticos, el tratamiento de elección sería la corrección quirúrgica del defecto y, en ocasiones, la rotura percutánea de la membrana; mientras que en los que se encuentran asintomáticos, el seguimiento y tratamiento oportuno de sus complicaciones parecería la alternativa más viable.

ABSTRACT Cor triatriatum is a rare congenital condition with a 0.1% prevalence among all cardiac congenital anomalies in which the heart is partitioned into three atria. In cor triatriatum dexter the right atrium is divided by a fibromuscular membrane into two parts, a proximal and a distal one, which may or may not communicate with each other. In the natural course of the condition, patients may remain asymptomatic until diagnosis or present with cardiovascular manifestations secondary to right heart failure and rhythm disorders. In the specific case of symptomatic patients, the treatment of choice would be surgical correction of the anomaly and sometimes percutaneous rupture of the membrane; while in asymptomatic patients, timely follow-up and treatment of their complications would seem to be the best alternatives.

Caso clínico de cor triatriatum en el adulto asintomático: diagnóstico ecocardiográfico / Clinical case of cor triatriatum in the asymptomatic adult: cardiac ultrasound diagnosis

Resumen El cor triatriatum es una anomalía congénita rara, que representa el 0,4% de todas las malformaciones cardíacas. En esta malformación la aurícula es separada en dos compartimientos por una membrana fibromuscular, que puede ser enteramente intacta o contener fenestraciones de diámetros variables. La aparición de la sintomatología está en relación con las propiedades obstructivas que mimetizan la estenosis mitral y la presencia de una comunicación interauricular. La ecocardiografía bidimensional y el Doppler color son las modalidades diagnósticas no invasivas de elección para el diagnóstico preciso del cor triatriatum y de su repercusión hemodinámica. Se reporta un caso de cor triatriatum en un paciente adulto mayor, en quien se hizo el diagnóstico tardío y casual de esta malformación.

Abstract Cor triatriatum is a rare congenital anomaly that represents 0.4% of all cardiac malformations. In this malformation the atrium is separated into two compartments by a fibrous-muscular membrane that may be completely intact or contain fenestrations with variable diameters. The appearance of symptoms is associated with obstruction-like properties that mimic mitral stenosis and an atrial septal defect. Two-dimensional cardiac ultrasound and colour Doppler are non-invasive methods of choice for the accurate diagnosis of cor triatriatum and its haemodynamic repercussions. A case of cor triatriatum is reported in an elderly patient, in whom a delayed and casual diagnosis was made of this malformation.

Complete cor triatriatum sinister misdiagnosed as atresia of the common pulmonary vein on prenatal ultrasound / 中华围产医学杂志

Cor triatriatum sinister (CTS) is characterized by the presence of a fibromuscular membrane dividing the left atrium into two chambers. The postero-superior and proximal chamber of CTS receives blood from four pulmonary veins, whereas the antero-inferior and distal chamber (true left atrium) connects to mitral valve and left atrial appendage (LAA). CTS can be classified into two types, complete and partial CTS. We reported a case of fetal autopsy-confirmed complete CTS that had been misdiagnosed as atresia of the common pulmonary vein on prenatal ultrasound. At 25 gestational weeks, fetal sonography revealed that the pulmonary venous angle on the top of the left atrium disappeared and no vertical vein was found around the common pulmonary venous cava formed by bilateral pulmonary veins. Thus, atresia of the common pulmonary venous cava was considered. Fetal autopsy after induction further confirmed the case was complete CTS.

Complete cor triatriatum sinister misdiagnosed as atresia of the common pulmonary vein on prenatal ultrasound / 中华围产医学杂志

Cor triatriatum sinister (CTS) is characterized by the presence of a fibromuscular membrane dividing the left atrium into two chambers. The postero-superior and proximal chamber of CTS receives blood from four pulmonary veins, whereas the antero-inferior and distal chamber (true left atrium) connects to mitral valve and left atrial appendage (LAA). CTS can be classified into two types, complete and partial CTS. We reported a case of fetal autopsy-confirmed complete CTS that had been misdiagnosed as atresia of the common pulmonary vein on prenatal ultrasound. At 25 gestational weeks, fetal sonography revealed that the pulmonary venous angle on the top of the left atrium disappeared and no vertical vein was found around the common pulmonary venous cava formed by bilateral pulmonary veins. Thus, atresia of the common pulmonary venous cava was considered. Fetal autopsy after induction further confirmed the case was complete CTS.

Cor triatriatum sinester. A propósito de un caso / Cor triatriatum sinester. Apropos of a case

RESUMEN El cor triatriatum es una anomalía congénita rara. Su prevalencia es de un 0,1% entre todos los pacientes con cardiopatías congénitas. La aurícula queda dividida en dos partes por una membrana fibromuscular; una cámara proximal y otra distal que se comunican entre sí por dos o más orificios con distintos grados de obstrucción. Es más frecuente encontrarlo en la aurícula izquierda (cor triatriatum sinester). Se diagnostica generalmente en la infancia o durante la edad adulta, muchas veces de manera fortuita mediante un ecocardiograma de rutina. Las manifestaciones clínicas en esta rara enfermedad dependerán del grado de obstrucción de la membrana en la aurícula, así como de las cardiopatías congénitas asociadas.

ABSTRACT Cor triatriatum is a rare congenital defect, (estimated incidence of 0.1% of all patients with congenital heart diseases). The atrium is divided into two compartments by a fibromuscular membrane; a proximal and a distal chamber that communicate with each other through two or more perforations with varying degrees of obstruction. It is more commonly found in the left atrium (cor triatriatum sinester) and usually diagnosed in childhood or during adulthood, often incidentally by a routine echocardiogram. Clinical symptoms in this rare disease depend on the severity of obstruction of the membrane in the atrium, as well as the associated congenital heart diseases.

Cor Triatriatum sinester / Cor Triatriatum Sinester

ResumenEl cor triatriatum sinester es una anormalidad cardiaca congénita poco frecuente, que constituye del 0,1 al 0,4% de todas las malformaciones cardiacas. Se caracteriza por una división anómala de la aurícula izquierda por una membrana fibromuscular, la cual genera dos cavidades que se comunican por uno o más orificios con distinto grado de obstrucción.Se asocia con otras anomalías cardiacas, principalmente con comunicación interauricular. Se puede presentar en la infancia, cuando suele ser fatal, o más tardíamente en el adulto, como insuficiencia cardiaca, o incluso cursar de forma asintomática, dependiendo del tamaño de los orificios y del gradiente de presión entre las cavidades. En este artículo se presenta el caso de un masculino de 30 años, sin patologías crónicas, quien consulta por disnea de grandes esfuerzos en los últimos 6 meses, a quien se le diagnostica por medio del ecocardiograma transtorácico y transesofágico un cor triatriatum sinister.

AbstractCor Triatriatum Sinister is a rare congenital heart defect, representing 0.1 to 0.4% of all cardiac malformations. This condition is characterized for an abnormal division of the left atrium by a fibromuscular membrane that generates two cavities, which are connected by one or more orifices with different degrees of obstruction. This condition is associated with other cardiac abnormalities mainly related to atrial septal defects. Its presentation in pediatric patients is often lethal, in adults it may appear as heart failure symptoms or as an asymptomatic finding in echocardiography depending on the pressure gradient between cavities. We present a case report of a 30 years old male without chronic conditions, who complained of dyspnea with strenuous physical activity occurring in the last 6 months, who was diagnosed with a cor triatriatum sinister by the use of transthoracic and transesophageal echocardiogram.

A Case of Cor Triatriatum with Severe Mitral Regurgitation and Atrial Fibrillation in an Adult / 日本心臓血管外科学会雑誌

<p>Cor triatriatum is a rare congenital cardiac anomaly in which the accessory chamber is separated from the left atrium by an anomalous septum. We report a rare case of cor triatriatum with severe mitral regurgitation and atrial fibrillation in an adult. The patient was a 65-year old woman who developed congestive heart failure 3 years previously, and received medical follow-up with mitral regurgitation, atrial fibrillation and cor triatriatum since then. She developed congestive heart failure again and was referred to our hospital for operation for progressed mitral regurgitation, tricuspid regurgitation and atrial fibrillation. Mitral valve plasty (Physio ring II 28 mm, cleft closure, edge to edge repair for PMC), tricuspid annuloplasty (Physio tricuspid ring 28 mm), resection of the anomalous septum and maze procedure was performed. All of the pulmonary veins were connected to the accessory chamber. There was only one hole on the anomalous septum, and the hole was large, about 3.0 cm in diameter. The patient regained sinus rhythm without mitral and tricuspid regurgitation after the operation. Even though the duration of atrial fibrillation was long and left atrium diameter was large, complete excision of the anomalous septum and maze procedure were effective for the patient diagnosed cor triatriatum.</p>

Experience of surgical repair for cor triatriatum sinister in chirdren / 中华胸心血管外科杂志

Objective To share the experience of surgical repair for cor triatriatum in chidren.Methods Between July 2008 and June 2013,24 children with cor triatriatum underwent surgical correction in Cardiac Center of Children's Hospital of Fudan University.The minimum age at the time of operation was 1 month,median age was 5 month;the minimum body weight at the time of operation was 3.5 kg,median body weight was 7.4 kg.Retrospectively analyzed their cardiac anatomy,clinical data,surgical procedures and follow-up data.Results Of all 24 patients,there were 4 patients diagnosed semplice cor triatriatum,and 20 patients diagnosed cor triatriatum associated with other malformation includes cardiac and non-cardiac issues.Procedures were performed on 22 patients to correct all cardiac defects in one stage.1 patient underwent surgical repair for cor triatriatum and Glenn procedure as well.1 patient,diagnosed as cor triatriatum and functional single ventricle S/P Glenn,underwent surgical repair for cor tiratriatum only.2 patients recovered from pulmonary artery hypertension crisis after operation.1 patient dead 17 days post procedure caused by respiratory failure.23 patients discharged from hospital.The longest follow-up was 73 months,at least 12 months after discharge.All alive patients were in New York Heart Association's function class Ⅰ-Ⅱ.Echo assessment revealed normal size of left atriums of almost all alive patients one year after discharge.Till April 2015,no postoperation intervention,no blood flow obstruction at level of pulmonary veins,left atrium or mitral valve.Conclusion Pediatric patients diagnosed cor tiratriatum sinister need intervention,no matter hemodynamic change occurs or not.Right time of surgery is important for successful treatment.

Símdrome de Silver-Russell (hemihipertrofia) y cor triatriatum en un neonato / Silver-Russell syndrome (hemihypertrophy) and cor triatriatum in a newborn

Tanto el síndrome de hemihipertrofia como el cor triatriatum son patologías sumamente infrecuentes. La hemihipertrofia se define como el sobrecrecimiento completo o parcial de uno de los hemicuerpos. El cor triatriatum es una cardiopatía congénita caracterizada por una membrana que divide la aurícula izquierda en dos cámaras; si esta membrana tiene un orificio restrictivo, provoca obstrucción al pasaje sanguíneo desde las venas pulmonares hacia el ventrículo izquierdo, lo que genera hipertensión y edema pulmonar. En este contexto, el ductus arterioso permeable puede actuar como vía de descompresión del circuito pulmonar al permitir el pasaje sanguíneo desde la arteria pulmonar hacia la aorta. Presentamos a un paciente con diagnóstico de síndrome de Silver-Rusell (hemihipertrofia), cor triatriatum y ductus arterioso con flujo invertido. Hasta donde conocemos, esta asociación de patologías infrecuentes y forma de presentación no se han descrito anteriormente.

Hemihypertrophy syndrome and cor triatriatum are extremely rare pathologies. Hemihypertrophy is defined as complete or partial overgrowth of one of the hemibodies. Cor triatriatum is a congenital heart disease characterized by a membrane which separates the left atrium into two chambers; if that membrane has a restrictive hole, it causes obstruction to blood passage from the pulmonary veins into the left ventricle causing hypertension and pulmonary edema. In this context, the patent ductus arteriosus can act as a means of decompression of the pulmonary circuit, because it allows the blood passage from the pulmonary artery to the aorta. We report a patient with Silver-Rusell syndrome (hemihypertrophy), cor triatriatum and ductus arteriosus with reverse flow. To our knowledge, this association of rare pathologies and this clinical presentation have not been described previously.

Anesthesia and cor triatriatum.

Aims and Objectives: Cor triatriatum sinistrum (CTS) and cor triatriatum dextrum (CTD) are rare congenital anomalies characterized by the presence of a perforated septum which divides the respective atrium into a proximal and distal chamber. This report reviews the perioperative course of patients with uncorrected cor triatriatum (CT) undergoing procedures requiring anesthesia. In addition, we performed a literature search that examines the experience of others regarding the peri‑operative course of patients with CT. Materials and Methods: A computerized search of a medical record database was conducted to identify patients with a clinical diagnosis of uncorrected CTD and CTS undergoing surgical procedures. Descriptive statistics were used. Results: We identified 12 adult patients with asymptomatic CTS (n = 7) and CTD (n = 5) who underwent 23 anesthetics. There were no perioperative complications which could be attributed directly to the anatomy of CT. Conclusions: Our observation and review of the literature suggest that patients with asymptomatic CT typically tolerate anesthesia and surgical procedures well.

Unusual Case of Cor Triatriatum Sinister

No abstract available.

Incidental discovery of an unusual right atrial membrane in an adult patient.

We describe presence of an unusual right atrial membrane in a 30-year old female with end stage renal disease, hypertension and peripheral vascular disease. The patient was scheduled for midline sternotomy and pericardiotomy and removal of a migrated vascular stent in the right pulmonary artery. An intraoperative transesophageal echocardiogram (TEE) revealed an unusual membranous structure with fenestrations that stretched across the right atrium with attachments superiorly at the free wall and inferiorly at the inter-atrial septum. There was no evidence of flow obstruction across the tricuspid valve. Some of the considerations for the likely diagnosis of this structure were a prominent Eustachian valve, persistent Chiari network, aneurysmal inter-atrial septum, an inter-atrial septal cyst or Cor triatriatum dexter (CTD).

Cor triatriatum dexter: Uso de resonancia magnética cardiovascular en su diagnóstico a propósito de un caso / Useful of cardiovascular magnetic resonance in diagnosis of cor triatriatum dexter: Case report

El Cor triatriatum dexter es una patología rara, los principales datos obtenidos se han presentado únicamente como reporte de casos posterior al diagnóstico postmortem, así al encontrar esta patología durante el estudio de resonancia magnética de un paciente con estenosis pulmonar severa, que es enviado para valoración de la función ventricular derecha, sin reporte de Cor triatriatum en otros estudios de imagen, parte el interés del análisis y revisión de la literatura. La incidencia de Cor triatriatum dexter comprende aproximadamente el 0.025% de los casos de las cardiopatías congénitas y es el resultado de la persistencia de las valvas embriónicas del seno venoso. Durante el estudio de resonancia en imágenes de spin-eco se observa la membrana que divide la aurícula derecha, además que durante un primer paso de contraste se corrobora que esta membrana está fenestrada al observar flujo turbulento entre la aurícula verdadera y la falsa, además de que la resonancia magnética permitió la adecuada evaluación de la función ventricular derecha. Aunque el Cor triatriatum es poco común, suele acompañar a la estenosis pulmonar, la resonancia magnética permitió su adecuada evaluación gracias a su resolución, con lo que podemos confirmar su importancia en la valoración de cardiopatías congénitas.

The Cor triatriatum dexter is a strange pathology, the main data have been obtained only like case report postmortem study, when we find this pathology during the magnetic resonance study of a patient with severe pulmonary stenosis, who were send for evaluation of right ventricular function, without report of Cor triatriatum in other image studies, it increase the interest of the analysis and revision of the literature. The incidence of Cor triatriatum dexter is approximately 0.025% of the cases of the congenital heart disease and it is the result of the persistence of the embryonic valve of the venous sine. During the magnetic resonance study, in images of spin-echo is observed the membrane that divides the right atrium, also that during a first-pass contrast is corroborated that this membrane is fenestrated when observing turbulent flow among the true atrium and the false one, besides that the magnetic resonance allowed the appropriate evaluation of the right ventricular function. Although the Cor triatriatum is not very common it usually accompanies the pulmonary stenosis, the magnetic resonance allowed its appropriate evaluation thanks to its resolution, we can confirm its importance in the valuation of congenital heart disease.

A Case of Acute Cerebral Infarction in Adult Cor Triatriatum Sinistrum / 대한내과학회지

Cor triatriatum is a rare congenital malformation of the heart characterized by a fibromuscular membrane dividing the atrium into two distinct chambers. In the majority of cases, it is diagnosed in early childhood, whereas adult cases are extremely rare [1,2]. The hemodynamics of cor triatriatum are similar to those of mitral stenosis, which sometimes cause embolic infarction. We describe an unusual case of cor triatriatum sinistrum in a 48-year-old man who presented with relapsed embolic infarction.

Anesthetic management of non-cardiac surgery with adult onset type of cor triatriatum sinister: A case report / 대한마취과학회지

A 45-year-old woman with cor triatriatum sinister was admitted for laparoscopic resection of an ovarian tumor. Her medical history was benign with the exception of a single episode of syncope one year ago. A 1.5-cm membrane fenestration was found on echocardiography, but there were no other cardiac structural anomalies. General anesthesia was established with etomidate, sevoflurane, and remifentanil; no notable events occurred during the anesthesia. As cor triatriatum shows a clinical picture of mitral stenosis (MS), careful anesthetic management is required.