Labyrinthine cor triatriatum sinister in fetal hypoplastic left heart syndrome is associated with poor outcomes.

OBJECTIVES: A subset of hypoplastic-left-heart-syndrome (HLHS) fetuses have a complex cor-triatriatum sinister that we named "labyrinthine-cor (L-cor)". We sought to determine the prevalence of L-cor in HLHS fetuses and hypothesized that it is associated with increased mortality. METHODS: This single-center retrospective cohort study included all HLHS fetuses from January 2010-December 2020. Fetuses with other hypoplastic-left-heart variants, inadequate images, lack of follow-up and fetal atrial-septal interventions were excluded. RAS was defined as the ratio of pulmonary-vein forward-to-reverse velocity-time-integral (VTI) ≤ 5 and severe-RAS defined as VTI-ratio <3. Kaplan-Meier survival-analysis was performed for the primary outcome of transplant-free survival for 62 weeks after gestational-age of 30 weeks (∼1 year). RESULTS: Of the 156 consecutive fetuses with HLHS, 11 (7.7%) had L-cor and 8/11 (72.7%) of these had RAS. When compared to HLHS-RAS without L-cor, fetuses with HLHS-RAS and L-cor were less likely to survive to 28 days (87% vs. 62.5%, p = 0.017) and to 1 year (69.6% vs. 25%, p = 0.029). When comparing by survival analysis, fetuses with severe-RAS with L-cor had lower survival compared severe-RAS without L-cor (p = 0.020). CONCLUSION: L-cor in fetal HLHS is associated with increased mortality. Recognition of this finding is important for prognostication and atrial-septal-intervention planning.

An extreme case of cor triatriatum mimicking hypoplastic left heart syndrome and combined pulmonary vein stenosis.

A 65-day-old girl presented to the emergency room with lethargy, requiring emergency venoarterial extracorporeal membrane oxygenation for refractory cardiogenic shock. Initially, hypoplastic left heart syndrome was suspected. However, cor triatriatum with a pinpoint opening on the membrane was diagnosed based on a detailed echocardiographic examination. After membrane resection, the left heart size was restored. However, follow-up echocardiography performed 4 months later showed occlusion of both upper pulmonary veins and stenosis in both lower pulmonary veins. Hybrid balloon angioplasty was performed in all pulmonary veins, and stents were inserted into the right upper and lower pulmonary veins. Despite repeated balloon angioplasty, all pulmonary vein stenosis progressed over 6 months and the patient expired while waiting for a heart-lung transplant. Even after successful repair of cor triatriatum, short-term close follow-up is required for detecting the development of pulmonary vein stenosis.

Cor triatriatum sinister diagnosed in a patient with postoperative cerebral infarction after beach chair position: A case report.

Cor triatriatum sinister is a rare congenital heart disease in which the left atrium is divided into two compartments by a fibromuscular membrane. In most cases, its symptoms appear in childhood, and it is rarely diagnosed in adulthood. Patients with cor triatriatum sinister are more prone to neurological diseases, such as cerebral infarction. Herein, we report the case of a patient whose cor triatriatum sinister went undiagnosed in the preoperative evaluation, but was diagnosed whilst investigating a cerebral infarction that occurred following a surgery in the beach chair position. It highlights the potential complications in otherwise healthy asymptomatic patients undergoing surgery in the beach chair position. Additionally, in high-risk patients, the provision of clear communication, in advance, regarding potential complications and their management, may reduce the patient's morbidity.

Corrected Transposition of Great Arteries with Cor Triatriatum and Atrial Septal Defect-Case Report.

A 37-year-old male patient with corrected transposition of great arteries (ccTGA) with cor triatriatum sinister (CTS), left superior vena cava, and atrial septal defects is reported in our case. None of these impacted the patient's growth or development, nor daily work until age 33. Later, the patient developed symptoms of obvious impaired heart function, which improved after medical treatment. However, the symptoms reappeared and gradually worsened two years later, and we decided to treat it with surgery. In this case, we selected tricuspid mechanical valve replacement, cor triatriatum correction, and atrial septal defect repair. During the follow-up of five years, the patient had no obvious symptoms, ECG did not change significantly from five years ago, and the cardiac color Doppler ultrasound showed RVEF 0.51.

Cor triatriatum sinister: Long-term surgical outcomes in children and a proposal for a new classification.

BACKGROUND: Cor Triatriatum Sinister (CTS) is a rare congenital anomaly with an estimated incidence of 0.4%, resulting from abnormal left atrial septation. It may present in isolation or in association with other heart defects. High percentage of patients require surgery with low mortality and recurrence rates. CTS has been classified in the past however we aim to describe a case series with more comprehensive and inclusive classification. METHODS: This was a single-center retrospective cohort study of 16 children with the diagnosis of CTS between 2000 and 2020. Medical records were reviewed for clinical presentations, hospital, and postoperative courses. RESULTS: Sixteen patients (63% female), with a median age at diagnosis of 4.3 months, five (31%) were neonates. Six (38%) had isolated CTS, two (13%) with functional single ventricle (SV), and the remaining eight patients (50%) had other associated heart defects (septal defects in three, coarctation of the aorta in another three, and anomalous pulmonary venous connections in three). Eight patients (50%) presented with obstructed CTS. Twelve patients (75%) underwent surgical intervention. Mortality occurred in three patients (19%) with two surgical (one with total anomalous pulmonary venous connection and another with SV) and one nonsurgical (septal defect with Fanconi anemia). The surgical median follow up was 4.7 years. Recurrence of the membrane occurred in two patients (17%). CONCLUSIONS: This study showed good long-term outcomes for patients with isolated and complex CTS. Complete and proper classification of CTS ensures appropriate diagnosis, surgical planning, and better family counseling which may correlate with outcomes.

Adult Obstructive Cor Triatriatum with Severe Mitral Regurgitation: A Case Report.

Adult cor triatriatum sinister associated with severe mitral regurgitation is extremely rare. As these obstructive cor triatriatum feature hemodynamics that mimic mitral stenosis, a pressure load is theoretically generated only on the left atrial proximal chamber, and therefore the left ventricle is less likely to suffer volume loading. Here, we report a surgical case with such rare hemodynamics. A 22-year-old man with obstructive cor triatriatum and severe mitral regurgitation received an anomalous membrane excision and mitral annuloplasty. An abnormal membrane with an orifice 7 mm in size was completely resected while a grossly dilated mitral annulus was repaired via annuloplasty ring. Mitral regurgitation was controlled well, and the postoperative course was uneventful. Even with obstructive cor triatriatum, severe mitral annular dilatation and subsequent left ventricular dilatation may occur, causing the progressive heart failure encountered in this case.

Mid-term outcomes of cor triatriatum repair: comparison of biventricular physiology and univentricular physiology.

BACKGROUND: Cor triatriatum is the rarest of all congenital cardiac diseases, accounting for 0.1-0.4% of congenital heart diseases. Atrial septal defect is the most common associated defect; however, cor triatriatum is sometimes associated with univentricular heart. METHODS: This single-centre retrospective study involved all patients who underwent the repair of cor triatriatum at Kobe Children's Hospital between 2000 and 2020. Twenty-four patients were required surgery. We conducted a survey of survival rate, early and late pulmonary vein stenosis in each group. RESULTS: The survival rate of 5 years after cor triatriatum resection was 100% in the biventricular group and 82.1% in the univentricular group, respectively. The free rate for pulmonary stenosis of 5 years after surgery was 100% in the biventricular group and 90.0% in the univentricular group, respectively. There was no statistical difference in survival rate and 5 years free rate for pulmonary stenosis after surgery. CONCLUSIONS: The results showed that surgical correction offers good early and mid-term outcomes for both cor triatriatum with biventricular and univentricular physiologies.

Loeffler's group 2 cor triatriatum sinistrum with mobile left atrial thrombus - a case report and literature review.

We report a case of a 25-year-old man diagnosed with an unusual case of cor triatriatum sinister with a mobile left atrial thrombus. He was hospitalised with aggravating dyspnoea. Transthoracic echocardiography revealed a membrane-like structure traversing the left atrial and a small orifice of about 7.1 mm. The mean pressure gradient was 12.94 mmHg across the orifice of the membrane-like structure and there was a mobile mass in the post-erosuperior chamber. The anomaly was rectified by a surgical resection. Timely diagnosis and surgical repair may prevent stroke in patients with unusual cor triatriatum sinister.

The strange case of congenital mitral stenosis in an adult man with cor triatriatum.

We report the case of a 74-year-old male, with a medical history of cor triatriatum, admitted with a 10-day history of intermittent fever. Three sets of blood cultures were positive for Providencia rettgeri. Transthoracic and transesophageal echocardiogram excluded infective endocarditis, but revealed a congenital accessory tissue adhering to the mitral valve, causing supravalvular mitral stenosis. Cor triatriatum sinistrum is a rare congenital cardiac anomaly, even more uncommon in adults, and quite exceptional when associated with mitral valve disease. Because the patient had no symptoms related to the heart valve disease, no surgical indication was given and he was managed conservatively.

Cor triatriatum sinister with left anomalous pulmonary venous return to innominate vein.

Cor triatriatum sinister is a rare congenital abnormality, with varying signs and symptoms related to the anatomic features and the components of the accompanying cardiac abnormalities. We present a 3-year-old girl who underwent successful simultaneous operation with complete loss of the membrane and ligation of vertical vein by anomalous venous return. The principles for surgical management of cor triatriatum can be summarised as radical resection of the membrane and correction of other intracardiac anomalies.

A Rare Cause of Shock in an Infant: Cor Triatriatum.

Congenital heart disease can be difficult to diagnose in infants because they often present with nonspecific symptoms. Their clinical presentation can resemble that of respiratory infections or sepsis, yet typical treatments for these conditions such as fluid resuscitation may worsen their course. We report a case of an 8-week-old boy who had several weeks of viral symptoms and eventually presented to the emergency department in shock. An echocardiogram revealed the diagnosis of cor triatriatum, a rare congenital heart disease that requires urgent surgical repair. In this article, we review the evaluation and management of such patients and emphasize the importance of considering congenital heart disease in the differential diagnosis for an infant in shock.

A Rare Case of Cor Triatriatum Leading to Respiratory Failure and Shock in an 8-Month-Old Boy.

An 8-month-old boy presents with 1 week of gradually worsening respiratory distress. After chest radiograph shows bilateral airspace opacities, he is treated for pneumonia with antibiotics and intravenous fluids. Shortly after this, he decompensates requiring intubation, followed by cardiopulmonary support with pressors and eventually extracorporeal membrane oxygenation. Bedside ultrasound then shows an additional congenital membrane in the left atrium obstructing pulmonary venous return and thought to have caused his original presentation and subsequent decompensation. After surgical correction, he is decannulated from extracorporeal membrane oxygenation, makes a quick recovery, and is discharged from the hospital in good condition within 2 weeks. This rare case of cor triatriatum highlights the importance of considering congenital heart disease even with common presentations in the pediatric population.

Cor triatriatum dexter with right ventricular hypoplasia: Role of multimodality imaging in decision making.

Cor Triatriatum Dexter (CTD) is a rare congenital anomaly involving the systemic venous valves. Failure of regression of the right-sided sinus venosus valve leads to abnormal septation of the right atrium and a variety of right atrial and tricuspid valve obstructive lesions. The presentation can be varied ranging from asymptomatic to persistent neonatal cyanosis. We describe a late diagnosis of CTD in a 10-month-old male with persistent hypoxia despite balloon valvuloplasty for mild pulmonic valve stenosis with a large secundum atrial septal defect and a mildly hypoplastic right ventricle.

Cor triatriatum sinister with an intact interatrial septum and a decompressing vein in a toddler.

Cor triatriatum sinister is a very rare cardiac anomaly that may lead to pulmonary hypertension, right ventricular dilation, and eventually right heart failure. We report a case of a toddler who presented with respiratory distress and cardiomegaly and was found to have cor triatriatum sinister with a restrictive communication, decompressing vertical vein, pulmonary hypertension, severe tricuspid regurgitation, and severe right ventricular dysfunction. She underwent a successful surgical repair, with normalisation of right ventricular function and pulmonary artery pressure.

Association of a unique form of cor triatriatum with tetralogy of Fallot.

Cor triatriatum is a rare cardiac anomaly sometimes associated with tetralogy of Fallot (TOF) and persistent left superior vena cava (PLSVC). We present the case of a 2-year-old girl who was diagnosed with a unique form of cor triatriatum that was associated with TOF and PLSVC. In this case, the abnormal membrane that arose from the posterior wall of the left atrium encircled the left superior vena cava (LSVC) in the left atrial cavity. Here, we discuss this rare case as well as the imaging studies and surgical strategy adopted.

Catheter ablation of persistent atrial fibrillation in a patient with cor triatriatum sinister demonstrating a total common trunk of the pulmonary vein.

A 57-year-old male with persistent atrial fibrillation (AF) was referred for catheter ablation. Multidetector computed tomography (MDCT) revealed that a membrane divided the left atrium into two chambers, thus indicating the presence of cor triatriatum sinister. A 3D image reconstructed by MDCT showed that the accessory atrium received the left common and the right side PVs, as if it were a total common trunk, and this then flowed into the main atrium. After isolation of the pulmonary vein and posterior wall from the left atrium, AF could not be induced by any programmed pacing. The patient has remained free from AF during the 1 year of follow-up.

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.

Exercise-induced hypoxia secondary to an atrial septal defect and cor triatriatum dexter.

A 14-year-old boy presented to us with a diagnosis of severe asthma and oxygen desaturation of 76% on a 6-minute-walk test. A contrast echocardiogram revealed echocontrast in the left and right atria simultaneously. A secundum atrial septal defect and partial cor triatriatum dexter were diagnosed, and the atrial defect was closed by cardiac catheterisation.

Cor triatriatum dexter and coarctation of the aorta--a rare association in a 7-year-old child with type 1 neurofibromatosis.

Cor triatriatum dexter is an extremely rare congenital anomaly of the right atrium. It occurs because of the persistence of the right sinus venosus valve, resulting in partitioning of the right atrium. Most of the described cases of cor triatriatum dexter in the literature were incidental findings on echocardiogram or at necropsy. We present a case report of a 7-year-old girl who was referred to us for further assessment, with a possible diagnosis of coarctation of the aorta. Initial investigations confirmed not only the presence of a long segment coarctation of the aorta, but also a large obstructive membrane in the right atrium. A catheter intervention was performed to stent the coarctation segment, and the fibro-muscular shelf in the right atrium was surgically resected.