RESUMO
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 (â¼1 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.
Assuntos
Coração Triatriado , Síndrome do Coração Esquerdo Hipoplásico , Humanos , Feminino , Estudos Retrospectivos , Gravidez , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Coração Triatriado/complicações , Coração Triatriado/diagnóstico , Coração Triatriado/diagnóstico por imagem , Adulto , Ultrassonografia Pré-Natal , Estudos de CoortesRESUMO
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.
Assuntos
Coração Triatriado , Masculino , Humanos , Pré-Escolar , Adulto , Coração Triatriado/complicações , Coração Triatriado/diagnóstico por imagem , Coração Triatriado/cirurgia , Ecocardiografia , Ecocardiografia Transesofagiana , Átrios do Coração , Valva Mitral/diagnóstico por imagem , Valva Mitral/cirurgiaRESUMO
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.
Assuntos
Coração Triatriado , Síndrome do Coração Esquerdo Hipoplásico , Veias Pulmonares , Estenose de Veia Pulmonar , Feminino , Humanos , Coração Triatriado/complicações , Coração Triatriado/diagnóstico , Coração Triatriado/cirurgia , Ecocardiografia , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/cirurgia , Estenose de Veia Pulmonar/diagnósticoRESUMO
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.
Assuntos
Coração Triatriado , Humanos , Coração Triatriado/diagnóstico , Átrios do Coração , Infarto Cerebral/etiologia , Infarto Cerebral/complicaçõesRESUMO
Atrial fibrillation (AF) is the most common cardiac arrhythmia. However, the relation between congenital heart defects and the predisposition to AF is not fully understood. A 65-year-old male was admitted into the emergency department due to progressive dyspnea, orthopnea, palpitations, and edema. Transthoracic echocardiogram showed bi-atrial enlargement and dysplasia of the mitral leaflets with severe mitral regurgitation. Also, a membrane was noted in the LA, dividing the chamber into two parts, suggestive of cor triatriatum sinister. Coronary computed tomography angiography demonstrated a soft tissue septum in the left atrium. Multimodal evaluation is of vital importance for a complete approach, since, detected in time, it has an excellent prognosis.
Assuntos
Fibrilação Atrial , Cardiopatias Congênitas , Idoso , Humanos , Masculino , Fibrilação Atrial/complicações , Fibrilação Atrial/diagnóstico por imagem , Coração Triatriado/complicações , Coração Triatriado/diagnóstico por imagem , Ecocardiografia , Átrios do CoraçãoRESUMO
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.
Assuntos
Coração Triatriado , Cardiopatias Congênitas , Comunicação Interatrial , Transposição dos Grandes Vasos , Masculino , Humanos , Adulto , Coração Triatriado/complicações , Coração Triatriado/diagnóstico , Coração Triatriado/cirurgia , Veia Cava Superior/cirurgia , Comunicação Interatrial/complicações , Comunicação Interatrial/diagnóstico , Comunicação Interatrial/cirurgia , Transposição dos Grandes Vasos/complicações , Transposição dos Grandes Vasos/diagnóstico , Transposição dos Grandes Vasos/cirurgiaRESUMO
Cor triatriatum sinister is rare congenital heart disease. It is defined as the presence of an abnormal septum dividing the left atrium into two chambers, and in some cases may be associated with a total abnormal pulmonary venous connection. Prenatal diagnosis of cor triatriatum sinister may be possible with fetal echocardiography and the diagnosis can be made earlier than postnatal imaging techniques. In the fetal echocardiography performed in our patient, cor triatriatum sinister was present and the main pathology was the flow of right and left pulmonary vein return from the accessory atrium to the right atrium.
Assuntos
Coração Triatriado , Gravidez , Feminino , Humanos , Coração Triatriado/diagnóstico por imagem , Coração Triatriado/cirurgia , Ecocardiografia , Diagnóstico Pré-NatalRESUMO
Current classifications of cor triatriatum sinister (CTS) do not address the associated heart defects or single ventricle pathology. Therefore, these classifications are not prognostic classifications and only describe the anatomy and the pulmonary venous drainage. The proposed classification considered the associated congenital cardiac lesions and the single ventricle pathology, therefore, it could have prognostic value. Future multicenter studies are required to measure the performance of this classification and its prognostic value in patients with CTS.
Assuntos
Coração Triatriado , Cardiopatias Congênitas , Veias Pulmonares , Humanos , Coração Triatriado/diagnóstico por imagem , Coração Triatriado/cirurgiaRESUMO
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.
Assuntos
Procedimentos Cirúrgicos Cardíacos , Coração Triatriado , Recém-Nascido , Criança , Humanos , Feminino , Lactente , Masculino , Coração Triatriado/diagnóstico , Coração Triatriado/cirurgia , Estudos Retrospectivos , Átrios do Coração , Procedimentos Cirúrgicos Cardíacos/métodos , Resultado do TratamentoRESUMO
Anomalous pulmonary veins drain into the right side of the left atrium is an uncommon variety of anomalous pulmonary venous return. Rarely, anomalous pulmonary venous drainage combined with cor triatriatum and atrial septal defect. We presented the imaging findings of a male patient who had anomalous pulmonary venous drainage which has not previously been described.
Assuntos
Doenças Cardiovasculares , Coração Triatriado , Comunicação Interatrial , Veias Pulmonares , Síndrome de Cimitarra , Humanos , Masculino , Coração Triatriado/diagnóstico por imagem , Coração Triatriado/cirurgia , Comunicação Interatrial/diagnóstico por imagem , Comunicação Interatrial/cirurgia , Veias Pulmonares/diagnóstico por imagem , Veias Pulmonares/anormalidades , Síndrome de Cimitarra/diagnóstico por imagem , Síndrome de Cimitarra/cirurgiaRESUMO
BACKGROUND: Quadricuspid aortic valve and cor triatriatum are rare congenital cardiac anomalies. METHODS: We describe a rare case of quadricuspid aortic valve with dividing membranes in the left and right atria. RESULTS: The patient underwent surgery with resection of the dividing membrane in the left atrium and aortic valve replacement. CONCLUSION: Management is based on symptoms of obstruction due to the dividing membranes in the atria and the severity of aortic valve dysfunction. This case also highlights the limitations in current nomenclature when describing atrial partitions.
Assuntos
Insuficiência da Valva Aórtica , Coração Triatriado , Válvula Aórtica Quadricúspide , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Valva Aórtica/cirurgia , Insuficiência da Valva Aórtica/cirurgia , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/cirurgia , HumanosRESUMO
Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly representing <0.1% of all congenital cardiac malformations. It is characterized by the presence of a left atrial (LA) membrane that leads to left ventricular inflow obstruction. Uncorrected, obstructed CTS may have significant sequelae such as pulmonary hypertension or arrhythmias. Transcatheter balloon decompression has been described as a successful alternative to surgical resection of the obstructing membrane. Our review of the literature revealed no reported cases utilizing radiofrequency (RF) energy to perforate the CTS membrane prior to balloon decompression. This manuscript describes two patients with CTS who were treated successfully with a transcatheter technique using RF energy to perforate the obstructing membrane prior to balloon angioplasty and decompression of the LA.
Assuntos
Coração Triatriado , Cardiopatias Congênitas , Coração Triatriado/complicações , Coração Triatriado/diagnóstico por imagem , Coração Triatriado/cirurgia , Descompressão , Ventrículos do Coração , Humanos , Resultado do TratamentoRESUMO
INTRODUCTION: Cor triatriatum sinister (CTS) is a rare congenital heart defect characterized by fibromuscular septation of the left atrium associated with atrial fibrillation (AF). The incidence of hemodynamically insignificant CTS in the AF ablation population and effect on ablation success are not known. Furthermore, little is known about the potential effect of CTS on arrhythmogenic substrate. OBJECTIVE: We define the incidence of hemodynamically insignificant CTS in patients undergoing AF ablation with RF and cryoballoon ablation, the technical challenges created by the left atrial partitioning, and the potentially arrhythmogenic effects of the membrane. We also review the literature of CA in patients with CTS. METHODS: First-time AF ablation cases at our institution over a 10-year period were screened to identify patients with CTS. Retrospective review was performed to obtain clinical characteristics and ablation data. RESULTS: Of the 3953 consecutive patients undergoing initial AF ablation during the study period, four patients (0.10%) had CTS. Ablation was successful acutely in all patients. One patient had recurrent AF and required repeat ablation for a single procedure success rate of 75% and multi-procedure success rate of 100%. The CTS membrane was associated with low voltage zones in the two patients in whom it was measured and with substrate for macro-reentrant atrial tachycardia in one of these patients. CONCLUSION: The incidence of hemodynamically insignificant CTS in patients undergoing CA for AF is very low, but does not serve as a significant barrier to successful ablation as long as directed access to the superoposterior chamber is obtained.
Assuntos
Fibrilação Atrial/cirurgia , Ablação por Cateter/métodos , Coração Triatriado/cirurgia , Fibrilação Atrial/complicações , Coração Triatriado/complicações , HumanosRESUMO
Cor triatriatum is a rare congenital heart disease. A 57-year-old woman had cor triatriatum with severe mitral valve regurgitation (MR) and atrial fibrillation (AF). We performed mitral valve repair, left atrial appendage resection, and maze procedure by resection of the anomalous septum in the left atrium. As a result, MR was controllable and AF disappeared after the operation. Although there is no established maze procedure with cor triatriatum, removing the septum was effective to complete it.
Assuntos
Fibrilação Atrial , Procedimentos Cirúrgicos Cardíacos , Coração Triatriado , Insuficiência da Valva Mitral , Adulto , Fibrilação Atrial/etiologia , Fibrilação Atrial/cirurgia , Coração Triatriado/complicações , Coração Triatriado/diagnóstico por imagem , Coração Triatriado/cirurgia , Feminino , Átrios do Coração , Humanos , Pessoa de Meia-Idade , Insuficiência da Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/etiologia , Insuficiência da Valva Mitral/cirurgiaRESUMO
BACKGROUND AND AIM: The morphological variations when one, or both, of the atrial chambers is subdivided, are many and varied. We sought to address clinical presentations, potentially misdiagnosed cases, diagnostic modalities, surgical approaches, and outcomes of this "family" of uncommon lesions. METHODS: A total of 193 published investigations were synthesized. Diagnostic information was provided by clinical presentation, and multimodality imaging studies. RESULTS: Almost three-quarters of patients with divided atrial chambers present during infancy with severe pulmonary hypertension and cardiac failure. Associated cardiac and extra-cardiac defects are present in between half and nine-tenths of cases. Acquired division of the left atrium has been reported after the Fontan operation, orthotopic cardiac transplantation, and complicated aortic valvar infective endocarditis. Surgery under cardiopulmonary bypass remains the definitive treatment. Balloon dilation may be considered in anatomically compatible variants in the setting of cardiac failure and pregnancy as a bridge todefinitive treatment. Overall, mortality has been cited between nil to 29%. Presentation during infancy, associated congenital anomalies, pulmonary hypertension, and surgery in the previous era, have been the reported causes of death. The operative survivors have long-term favourable outcomes, with near normal cardiac dimensions and low risk of recurrence. While asymptomatic patients with division of the right atrium do not need treatment, surgical resection of the dividing partition under cardiopulmonary bypass is recommended in symptomatic patients with complex anatomy, the spinnaker malformation, or associated cardiac anomalies. Balloon dilation may be considered in uncomplicated patients with less obstructive lesions. Hybrid intervention and endoscopic robotic correction also have been performed. CONCLUSIONS: Resection of the dividing shelf allows the survivors to regain near normal dimensions with a low risk of recurrence. We submit that an increased appreciation of the anatomic background to division of the atrial chambers will contribute to improved surgical management.
Assuntos
Coração Triatriado , Técnica de Fontan , Cardiopatias Congênitas , Ponte Cardiopulmonar , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/cirurgia , HumanosRESUMO
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.
Assuntos
Procedimentos Cirúrgicos Cardíacos/métodos , Coração Triatriado/complicações , Insuficiência da Valva Mitral/complicações , Valva Mitral/cirurgia , Coração Triatriado/diagnóstico , Coração Triatriado/cirurgia , Ecocardiografia Transesofagiana , Humanos , Masculino , Valva Mitral/diagnóstico por imagem , Insuficiência da Valva Mitral/diagnóstico , Insuficiência da Valva Mitral/cirurgia , Adulto JovemRESUMO
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.
Assuntos
Coração Triatriado , Comunicação Interatrial , Criança , Coração Triatriado/diagnóstico , Coração Triatriado/cirurgia , Humanos , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
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.
Assuntos
Apêndice Atrial , Coração Triatriado , Trombose , Adulto , Coração Triatriado/diagnóstico , Coração Triatriado/diagnóstico por imagem , Ecocardiografia , Átrios do Coração/diagnóstico por imagem , Átrios do Coração/cirurgia , Humanos , Masculino , Trombose/diagnóstico por imagemRESUMO
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.
Assuntos
Coração Triatriado , Adulto , Pré-Escolar , Coração Triatriado/diagnóstico por imagem , Coração Triatriado/terapia , Dilatação , Ecocardiografia , Teste de Esforço , Feminino , Átrios do Coração/diagnóstico por imagem , Humanos , Adulto JovemRESUMO
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.