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BACKGROUND: Cor triatriatum dexter (CTD) is an extremely rare pathology, with an incidence of < 0.4%. Its main characteristic is a partitioning of the right atrium by the persistence of the embryonic valve of the right sinus venosus. CLINICAL CASE: In this report, we describe the case of a 7-day-old newborn who presented with persistent cyanosis associated with feeding and crying. The diagnosis of CTD was made after an echocardiogram and confirmed using cardiac magnetic resonance imaging. The patient underwent successful surgery on day 14 with a favorable outcome and without complications. CONCLUSION: The importance of our case lies in the identification of rare heart disease as a cause of cyanosis and desaturation in a neonatal patient in the first days of life who did not present signs of heart failure and whose condition improved with supplemental oxygen. We also demonstrate that early diagnosis with echocardiography and surgical resolution resulted in clear clinical improvement and avoided future complications.

INTRODUCCIÓN: El cor triatriatum dexter es una cardiopatía muy rara, caracterizada por la división parcial del atrio derecho en dos cavidades por la persistencia de una membrana que embriológicamente representa la valva derecha del seno venoso. CASO CLÍNICO: En este reporte de caso, presentamos el caso de un neonato en su día 7 de vida que acude a valoración por presentar desaturación persistente con cianosis al llanto. El diagnóstico se realizó con ecocardiograma posterior al cual se decidió la resección quirúrgica de la membrana, procedimiento que fue llevado a cabo el día 14 de vida con éxito sin complicaciones. CONCLUSIONES: La importancia de este caso clínico radica en la identificación de una cardiopatía rara como causa de cianosis y desaturación en un paciente en etapa neonatal, el cual no presentaba datos de compromiso hemodinámico. También se muestra como un diagnóstico y tratamiento quirúrgico oportuno permitieron una resolución de los síntomas sin complicaciones futuras.

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Introduction and importance: Cor triatriatum sinister (CTS) is an uncommon heterogeneous congenital cardiac defect that may manifest in adulthood when symptomatic blockage manifests due to a change in hemodynamic physiology or when a condition such as atrial fibrillation (AF) arises. The incidence of cor triatriatum with cardiomyopathy and congenital heart illness ranges from 0.1 to 0.4%. Case presentation: The Department of Cardiology examined a 10-year-old girl for a diastolic murmur. The patient's medical history was ordinary, and no previously known co-morbid illnesses were present. Pneumonia was the patient's original medical condition. A cardiac murmur was also discovered during the physical examination, and the patient was forwarded for more research. Physical examination revealed just a diastolic murmur, which was noteworthy. The patient's care plan includes routine echocardiographic monitoring. Since the deformity was not clinically significant, surgical repair was not advised. Clinical discussion: It is unclear what caused this flaw. A full, incomplete, or fenestrated septum may be the result of the common pulmonary vein failing to merge with the left atrium during embryonic development. The existence of a fibromuscular membrane, which separates the left atrium (LA) into two chambers-the proximal chamber receiving the pulmonary veins (PVs) and the distal chamber contains the mitral valves and LA appendages defines the disease. Conclusion: The diagnostic procedure should be carried out in the optimal settings, however, in cases of frail health systems, employing accessible alternatives might help the early diagnosis. Early referral to a cardiologist is required in case of CTS suspicion.

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Levoatriocardinal vein has rarely been described with cor triatriatum. We report a case of a newborn with respiratory distress who was found to have cor triatriatum sinister with a decompressing levoatriocardinal vein on transthoracic echocardiography. The pulmonary venous confluence received all pulmonary veins and drained to the proximal left atrial chamber through significant communication. Moreover, the cor triatriatum membrane separated the left atrial body into proximal and distal left atrial chambers with a restrictive opening in the membrane, causing severe flow limitation to the cardiac output, severe pulmonary arterial hypertension, and significant right ventricular dilatation. In addition, a sizeable levoatriocardinal vein decompressed the pulmonary venous confluence drained superiorly to the left innominate vein. She had a successful surgical repair by resectioning the cor triatriatum membrane and ligating the levoatriocardinal vein. The patient had improved pulmonary arterial pressures and right ventricular dimensions at the one-month follow-up. The case highlights the rare association of the levoatriocardinal vein and cor triatriatum, and its presence signifies severe obstruction at the level of cor triatriatum.

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Cor triatriatum (CT) or a triatrial heart is a rare congenital anomaly in which one of the atrial chambers is divided by a fibromuscular membrane. Of the two variants, CT dexter (right-sided CT) is still further rare than CT sinister (left-sided CT). Although CT sinister presents with features of left heart obstructive disease mimicking mitral stenosis, CT dexter is usually asymptomatic and is found incidentally on imaging. Here, we present a patient with an unusual case of complete heart block who was found to have CT dexter along with right ventricular noncompaction on imaging.

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BACKGROUND: Alström syndrome (ALMS), a rare recessively inherited ciliopathy caused by mutations in ALMS1, is characterized by retinal dystrophy, childhood obesity, sensorineural hearing loss, and type 2 diabetes mellitus. The majority of pathogenic variants in ALMS1 are nonsense and frameshift mutations, which would lead to premature protein truncation, whereas copy number variants are seldom reported. METHODS: Herein, we present a 10-year-old Chinese girl with ALMS. The potential causative genetic variant was confirmed through whole genome sequencing, quantitative real-time PCR analysis, and Sanger sequencing. Additionally, breakpoint analysis was performed to determine the exact breakpoint site of the large deletion and elucidate its probable formation mechanism. RESULTS: The patient had a cor triatriatum sinister (CTS) structure. Genetic analysis identified novel compound heterozygous variants in the patient, consisting of a frameshift variant c.4414_4415delGT (p.V1472Nfs*26) in ALMS1 and a novel large deletion at chr2:73,612,355-73,626,339, which encompasses exon 1 of the ALMS1 gene. Moreover, breakpoint analysis revealed that the large deletion probably formed through the microhomology-mediated end joining (MMEJ) mechanism due to the 6-bp microhomologies (TCCTTC) observed at both ends of the breakpoints. CONCLUSIONS: In this study, novel compound heterozygous variants in the ALMS1 gene were identified in an ALMS patient with a CTS structure. The molecular confirmation of these variants expands the mutational spectrum of ALMS1, while the manifestation of ALMS in the patient provides additional clinical insights into this syndrome.

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BACKGROUND: Cor triatriatum sinister (CTS) is an uncommon congenital cardiac anomaly. Atrial fibrillation (AF) is commonly the initial symptom in patients with CTS, occurring in approximately 32% of the cases. The complexity of performing AF catheter ablation, particularly in cases with persistent AF, increases in patients with CTS due to its unique structural challenges. CASE PRESENTATION: We report the treatment course of a 60-year-old male patient diagnosed with CTS, who underwent catheter ablation of drug-refractory, persistent AF. The complex anatomical structure of the condition made catheter ablation of AF challenging. To navigate these challenges, we performed comprehensive assessments using transthoracic echocardiography and transesophageal echocardiography, along with cardiac computed tomography angiography, prior to treatment initiation. The intricate anatomy of CTS was further clarified during the procedure via intracardiac echocardiography (ICE). Additionally, the complexity of catheter manipulation was further reduced with the aid of the VIZIGO sheath and the vein of Marshall ethanol infusion to achieve effective mitral isthmus blockage, thereby circumventing the impact of the CTS membrane. CONCLUSIONS: This case underscores the complexity and potential of advanced ablation techniques in managing cardiac arrhythmias associated with unusual cardiac anatomies. During the procedure, ICE facilitated detailed modeling of the left atrium, including the membranous structure and its openings, thus providing a clearer understanding of CTS. It is noteworthy that the membrane within the CTS may serve as a potential substrate for arrhythmias, which warrants further validation through larger sample studies.

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A 25-year-old male with no prior medical history presented with a one-month history of nausea, weight loss, and dyspnea that progressed to syncope. The initial echocardiogram showed a dilated right ventricle with signs of systolic failure. The patient was admitted for suspected pulmonary embolism, but chest computed tomography (CT) revealed interstitial pneumonia. A transthoracic echocardiogram on day 6 of admission diagnosed cor triatriatum sinister (CTS), severe pulmonary hypertension, chronic cor pulmonale, and reduced right ventricular function. The patient was managed conservatively in the intensive care unit (ICU) without the need for mechanical ventilation and discharged after clinical improvement. This case highlights the importance of the early diagnosis of rare congenital heart defects such as cor triatriatum sinister, which can present with nonspecific symptoms and rapidly progress to right heart failure.

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Cor triatriatum is a rare congenital heart abnormality in which a membrane separates the left atrium (LA; sinister) or the right atrium (dexter) into two compartments. It is also a long-forgotten cause of atrial fibrillation (AF) and substantially higher rates of blood stagnation, particularly proximal to the additional septum in the LA. In this case report, we faced a CHA2DS2-VASc score of 1 in patients with non-valvular AF due to Cor triatriatum sinister (CTS). The decision to start anticoagulants in this particular case was controversial, so we reviewed the literature to assess and address it. We present our case and discuss the indication of anticoagulants in this unique clinical scenario, accompanied by a literature review. Facing this dilemma of starting anticoagulants in special cases of CTS and AF should be individualized and need more investigation. However, till this moment, based on similar reports, it seems to be rational to consider CTS Per se as an additional risk stratification marker beyond the CHA2DS2-VASc score start anticoagulant until the surgical resection. Considering CTS as the sole indication of anticoagulant in patients with normal sinus rhythm is a complex matter that needs further investigation.

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The diagnosis of atrial septal defect (ASD) may be delayed until adulthood or even later in life as it is a well-tolerated congenital heart disease. If patients are not examined and investigated well in childhood, the diagnosis may be delayed until later in adulthood when patients present with palpitations and sometimes dyspnea due to the right chambers dilatation from right ventricular volume overload. In this report, we present a case of a 50-year-old female patient with symptoms of heart failure and atrial fibrillation who was found to have dilated right cardiac chambers, dilated pulmonary artery, severe tricuspid regurgitation, pulmonary hypertension, and a pulmonary-to-systemic flow ratio (Qp/Qs) of more than 1.5 by transthoracic echocardiography and Doppler, indicating left to right shunt at the atrial level. However, transthoracic echocardiography could not visualize the defect, and two-dimensional (2D) transesophageal echocardiography was done in this patient and documented the presence of a sinus venosus ASD with an incomplete cor triatriatum dexter membrane; all four pulmonary veins were identified going to the left atrium. Since the presence of an incomplete cor triatriatum dexter membrane (despite causing no symptoms) makes the percutaneous closure of the sinus venosus ASD and the percutaneous repair of tricuspid regurgitation very difficult, we decided to advise surgical ASD closure and tricuspid valve repair for the patient.

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Cor triatriatum sinister an interesting and relatively rare congenital condition in which the left atrium is bisected by a fibromuscular membrane into 2 distinct chambers. Classically, patients are diagnosed at an early age, although in some cases they remain asymptomatic until adulthood and this is mainly due to differences in the degree of obstruction to pulmonary venous return and the presence of associated lesions. However, some patients may remain asymptomatic into adolescence or late adulthood due to incomplete membranes or other favorable circumstances. Although there are only a few cases where cardiac MRI has been used to diagnose cor triatriatum sinister, it is very suitable for making the diagnosis. important to carefully evaluate cases of cor triatriatum sinister, as it resembles the physiology of mitral stenosis. Such cases in elderly individuals accompanied by mitral and tricuspid regurgitation are very rare. We want to present a case of a diagnosed cor triatriatum with mitral and tricuspid regurgitation in a 73-year-old male, who was hospitalized due to signs of heart failure. The diagnosis was made using MRI.

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Cor triatriatum is a rare congenital heart defect that occurs when a fibromuscular membrane divides the atrium into two chambers, which may impair blood flow to the ventricle. When it does, the symptoms usually manifest during infancy or early childhood. In this E-challenge, though, the case of a 40-year-old man is reviewed whose symptoms of shortness of breath progressed over the years and were attributed to the diminished mitral valve inflow due to the restricted cor triatriatum sinister associated with pulmonary hypertension, tachycardia-bradycardia syndrome, and atrial fibrillation. Despite routine preoperative evaluation, intraoperative transesophageal echocardiography was used to more accurately evaluate cor triatriatum sinister's morphology, hemodynamic significance, and associated anomalies.

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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.

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Necropsy of a 52-day-old Camborough pig revealed numerous cardiac malformations. The positional relationship of the atria, ventricles and great vessels was a mirror image type (I, L and L): inverted arrangement of the atria, with a left-sided right atrium and right-sided left atrium (situs inversus); inverted arrangement of the ventricles, with a left-sided morphological right ventricle and right-sided morphological left ventricle (L-loop); and aortic valve to the front left relative to the pulmonary valve (L-malposed). The major malformations included an ostium secundum atrial septal defect, cor triatriatum sinister (CTS), a subpulmonary ventricular septal defect and a bicuspid pulmonary valve. Histological examination revealed myocyte hypertrophy, focal myocardial necrosis and calcification in the left morphological right ventricle of the heart. To the best of our knowledge, this is the first report of CTS in pigs. Although the individual malformations found in the present case are not unique, an unusual combination of these cardiac malformations has not been described in animals.

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Atrial transseptal catheterisation is a fundamental skill of any interventional electrophysiologist. In this review, various scenarios that pose unique challenges to atrial transseptal catheterisation are discussed. These scenarios include post-surgical or congenital malformations of the interatrial septum, presence of interatrial septal closure devices, absent or obstructed inferior vena cava and complex congenital heart disease after palliative surgery. Transseptal catheterisation in all of the above situations is feasible and can be performed safely with the aid of dedicated tools and specific techniques.

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Cor triatriatum dexter (CTD) is an uncommon congenital cardiac anomaly in dogs. This case report describes successful membranectomy for CTD via partial venous inflow occlusion under mild hypothermia in a dog. A 7-month-old intact male mixed-breed dog weighing 20.5 kg presented with a history of abdominal distention, lethargy, and anorexia. Clinical examination, radiography, echocardiography, microbubble testing, and computed tomography revealed a remnant right atrium membrane obscuring the venous blood inflow from the vena cava. Considering the potential risk of re-stenosis following interventional treatment, curative resection involving surgical membranectomy via venous inflow occlusion was performed. By performing partial venous inflow occlusion under mild hypothermia (34.5 °C), sufficient time was obtained to explore the defect and resect the remnant membrane. The dog recovered without any complications, and the clinical signs were relieved. This case illustrates that partial venous inflow occlusion under mild hypothermia is feasible for achieving curative resection of cor triatriatum dexter in dogs.

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Cor triatriatum is a very rare cardiac malformation characterized by the presence of an abnormal interatrial membrane separating either the left or right atrial chamber into two compartments. It can be associated with other cardiac defects and is often symptomatic in childhood. The signs depend on the size and position of the interatrial membrane and other associated malformations. Here we report a case of right-sided cor triatriatum associated with an ostium primum-type interatrial septum defect and left-sided opening of the coronary sinus in a fetus. The cause of intrauterine death was asphyxia due to total placental abruption.