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 Sinistrum Combined with Changes in Atrial Septum and Right Atrium in a 60-Year-Old Woman.

Background and Objectives: A rare case of cor triatriatum sinistrum in combination with anomalies in the atrial septum and in the right atrium of a 60-year-old female body donor is described here. Materials and Methods: In addition to classical dissection, ultrasound and magnetic resonance imaging, computer tomography and cinematic rendering were performed. In a reference series of 59 regularly formed hearts (33 men, 26 women), we looked for features in the left and right atrium or atrial septum. In addition, we measured the atrial and ventricular wall thickness in 15 regularly formed hearts (7 men, 8 women). Results: In the case described, the left atrium was partly divided into two chambers by an intra-atrial membrane penetrated by two small openings. The 2.5 cm-high membrane originated in the upper level of the oval fossa and left an opening of about 4 cm in diameter. Apparently, the membrane did not lead to a functionally significant flow obstruction due to the broad intra-atrial communication between the proximal and distal chamber of the left atrium. In concordance with this fact, left atrial wall thickness was not elevated in the cor triatriatum sinistrum when compared with 15 regularly formed hearts. In addition, two further anomalies were found: 1. the oval fossa was deepened and arched in the direction of the left atrium; 2. the right atrium showed a membrane-like structure at its posterior and lateral walls, which began at the lower edge of the oval fossa. It probably corresponds to a strongly developed eustachian valve (valve of the inferior vena cava). Conclusions: The case described suggests that malformations in the development of the atrial septum and in the regression of the valve of the right sinus vein are involved in the pathogenesis of cor triatriatum sinistrum.

Cor Triatriatum Sinistrum: A rare disease with a common presentation.

Cor triatriatum sinistrum (CTS) is a rare congenital cardiac anomaly characterised by an abnormal septum within the left atrium impairing blood flow to the left ventricle. We report the case of a two-month-old male infant who presented with symptoms of heart failure since the age of two weeks. He was admitted to a local hospital and was managed with antibiotics because of the impression of pneumonia. Due to persistent unresolved tachypnoea and tachycardia, he was referred to Sultan Qaboos University Hospital, Muscat, Oman, in 2019 for cardiac evaluation which confirmed a diagnosis of isolated CTS with severe stenosis and pulmonary hypertension. He underwent an urgent surgical excision of the membrane with uneventful recovery.

Serial cardiopulmonary exercise testing in an asymptomatic young female receiving percutaneous balloon dilatation for cor triatriatum sinistrum at an early age.

A young female was diagnosed as classic cor triatriatum sinistrum (CTS) at 38 months old incidentally and she received percutaneous catheter-based balloon dilatation twice at 41 and 48 months old. She took regular follow-up by echocardiography biannually with no re-stenosis of the orifice in the membrane between two chambers in the left atrium and she denied any cardiac-related symptoms. Serial cardiopulmonary exercise testing (CPET) by treadmill under Ramped-Bruce protocol was done at her 13, 19, and 23-year old. She could reach maximal effort and complete the three CPETs. No significant change of metabolic equivalent at anaerobic (MET) threshold, peak MET, and pulmonary function were noted in the serial CPETs and all of them were within normal limits comparing to the reference values of Chinese specific to her age. Our case report demonstrated that the concept of percutaneous catheter-based balloon dilatation of obstructive membrane for classic CTS without other associated congenital heart diseases is sound and feasible. The prognosis is well without re-obstruction and the cardiopulmonary fitness after that could be maintain as healthy peers for up to 18 years.

Cor triatriatum sinistrum diagnosed in the adulthood: a systematic review.

BACKGROUND: We performed a systematic review of cor triatriatum sinistrum (CTS) diagnosed in adults. The aim of this review was to describe the clinical presentation, natural history and management of this congenital heart disease. METHODS: A PubMed literature search for 'cor triatriatum sinistrum' published since 2005 was performed. Included patients were divided into those with and without obstructive membrane physiology. The clinical course differences were compared. RESULTS: A total of 171 published cases were included. The median age at diagnosis was 43 years (IQR, 30-60). Obstructive membrane physiology was observed in 70 (41%), and this patient group was younger at presentation (median age 39 (IQR, 28-52) vs 50 years (IQR, 32-64), p=0.003). Patients with obstructive membrane more frequently had associated cardiac defects (58.6% vs 42.4%, p=0.039). Overall, the most frequent clinical symptom was atrial fibrillation, as this was present in 56 (32.8%) of all patients. CTS-related symptoms were more frequent in patients with obstructive membrane: congestive heart failure (44.3% vs 15.2%, p<0.001), pulmonary hypertension (27.1% vs 6.1%, p<0.001), haemorrhage (8.6% vs 0%, p=0.004) and infections manifestation (8.6% vs 0%, p=0.004).A total of 71 (41.5%) patients with CTS required interventional treatment, mainly within patients with the obstructive membrane (86.8% vs 12.6%, p<0.001). CONCLUSION: The natural history of CTS most often manifests with symptoms of congestive heart failure. Patients with obstructive membrane most often have associated cardiac defects and higher risk for infections and haemorrhage. The interventional treatment of CTS remains the first choice for obstructive membrane.

Truncus arteriosus communis in combination with cor triatriatum sinsitrum.

Truncus arteriosus communis is a rare CHD, accounting for only 1% of all congenital cardiac abnormalities. It has been associated with other malformations of the heart, mainly truncal valve (bicuspid/quadricuspid) and aortic arch abnormalities such as right, interrupted, and hypoplastic aortic arch. Cor tratriatrum sinistrum is another rare CHD, and it has been associated with other cardiac defects such as anomalous pulmonary venous drainage, ventricular septal defect, coarctation of the aorta, and tetralogy of Fallot. The combination of truncus arteriosus communis and cor tratriatrum sinistrum has not been reported so far. This case study describes the diagnosis of a unique case, including these two very rare cardiac defects and the successful surgical treatment thereafter.

Coexistence of Cor triatriatum sinistrum and a prominent Eustachian valve mimicking a Cor triatriatum dextrum.

Cor triatriatum is among the rarest of all congenital cardiac abnormalities accounting for 0.1-0.4% of all congenital heart disease. Its coexistence with a very prominent Eustachian valve which mimics a Cor triatriatum dextrum is an exceptionally rare finding in an asymptomatic adult. We report the case of a 44 year old male who presented to our department on observing a pulse rate of 44 beats per minute during a home blood pressure check with his digital sphygmomanometer. Clinical examinationwas however, unremarkable and resting electrocardiography showed sinus rhythm with atrial premature complexes. The diagnosis was made on a two dimensional transthoracic echocardiography. Isolated atrial premature complexes and bradycardia may be a clinical presentation of Cor triatriatum in adult population. Although extremely rare, its coexistence with a prominent Eustachian valve may remain asymptomatic into adult life.

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.