Search

Cor triatriatum is a rare congenital heart defect in which a thin, fibro-muscular membrane divides the left or right atrium into two chambers resulting in a triatrial heart. Subdivision of the left atrium named cor triatriatum sinister (CTS), is the more common form, whereas the right atrial equivalent called cor triatriatum dexter (CTD) is rarer. They account for up to 0.4% and 0.025% of the burden of congenital heart disease respectively. We present the case of CTD found incidentally with transthoracic echocardiography for a patient who underwent aortic valve replacement for symptomatic bicuspid aortic valve stenosis. [ABSTRACT FROM AUTHOR]

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.

Two patients with hypoplastic left heart syndrome (HLHS) concomitant with cor triatriatum underwent the staged Norwood procedure following hybrid palliation. Cor triatriatum was diagnosed after birth in both cases. Case 1 with aortic stenosis and mitral atresia underwent bilateral pulmonary artery banding at 2 days of age. Subsequently, balloon atrial septostomy was conducted at 21 days of age due to hypoxia and increased intra-atrial pressure gradient. At 2 months of age, concomitant Norwood operation, cor triatriatum repair, and tricuspid valve repair were successfully performed. She has suffered from branch pulmonary artery hypoplasia since then; thus, Fontan operation was out of indication. Case 2 with aortic stenosis and mitral stenosis underwent bilateral pulmonary artery banding at 6 days of age. After that, the patient's hemodynamic condition remained stable. The Norwood operation concomitant with cor triatriatum repair was performed at 2 months of age. However, during the procedure, massive pulmonary edema and hemorrhage occurred right after the initiation of cardiopulmonary bypass. She could not be weaned from cardiopulmonary bypass. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

This article discusses a case of Cor triatriatum sinistrum (CTS) syndrome, a rare congenital heart disease where a fibrous membrane divides the left atrium into two chambers. The case involves a 19-year-old woman who experienced symptoms of hypoxia in infancy but grew normally into adulthood, with pulmonary arterial hypertension being the only lasting effect. The article explains the haemodynamic adaptation that occurred in the patient, which alleviated her hypoxia symptoms. The patient underwent surgical correction for the syndrome, resulting in normalized haemodynamics and the resolution of symptoms. The article emphasizes the importance of detailed imaging diagnosis and haemodynamic evaluation in understanding the pathophysiological mechanisms of the disease and tailoring personalized treatment strategies for patients. [Extracted from the article]

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., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Domínguez-López et al.)

Introduction: Cor Triatriatum represents an extremely rare congenital cardiac malformation, accounting for 0.1%- 0.4% of all congenital heart defects, characterized by an intraatrial membrane dividing one of them into two distinct compartments. Left atrium is more frequently affected, and the upper cavity conduct to malformations of the pulmonary veins. Case Report: We present the case of a 1-year-old patient admitted to the 2nd Pediatric Department of the Emergency Clinical County Hospital of Craiova, in a severe general condition, hemodynamically unstable, with dyspnea, tachypnea, central and peripheral cyanosis, hepatomegaly, and peripheral edema that started in the last weeks. Maternal history reveals an uncomplicated pregnancy, delivered at 37 weeks by cesarean section. Following the echocardiographic examination, a cor triatriatum malformation, severe pulmonary hypertension, and NYHA class IV heart failure were diagnosed. Due to the potential risk of deterioration, the patient was transfered to the pediatric intensive care unit of the Cardiovascular Emergency Institute of Tg. Mures, where the patient goes into cardiorespiratory arrest and does not respond to resuscitation maneuvers, resulting in death. The autopsy revealed a complex cardiovascular malformation: a septated left atrium through a musculoconjunctival septum, subnumerary pulmonary veins due to agenesis/atresia of one of the right pulmonary veins, an ostium primum type atrial septal defect, eccentric right ventricular hypertrophy, and microscopic findings of myocardial interstitial edema, acute pulmonary edema, and chronic pulmonary congestion. Discussions : Cor triatriatum presents a wide spectrum of manifestations, from asymptomatic cases incidentally discovered during routine cardiac consultations or autopsies, to complications such as right heart failure, acute pulmonary edema, atrial fibrillation, and premature death. Diagnosis and treatment require a multidisciplinary team, as early correct diagnosis, in the absence of other malformations, has an excellent prognosis. Surgical intervention is curative, involving membrane resection followed by closure of the defect with a pericardial patch. Conclusions: The particularity of the case lies in the association of congenital heart malformations that decrease life expectancy and render surgical intervention impossible, with the evolution inevitably leading to death. [ABSTRACT FROM AUTHOR]

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.