Fig 3
Echocardiography with four chamber view showing a giant right atrium taking up most of the echo window with compression of other chambers
Source publication
Enlargement of right atrium is usually secondary to pulmonary hypertension due to valvular heart diseases or obstructive pulmonary disorders, atrial septal defect, tricuspid atresia or stenosis, pulmonary stenosis, primary pulmonary hypertension, Ebstein's anomaly. Congenital enlargement of right atrium is rare and it commonly presents in children....
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This review focuses on the utility of echocardiographic studies in the diagnosis of tricuspid atresia (TA) and in its management. Tricuspid atresia is a cyanotic congenital heart defect (CHD) accounting for nearly 1.5% of all CHDs. It is generally classified according to the morphology of the atretic tricuspid valve and associated heart defects. Fo...
Tracheal and bronchial varices are rarely found in children. However, they have been described in adults with failing Fontan circuits or secondary to vascular pathology, such as portal and pulmonary hypertension. We report the presentation of haemoptysis and bronchial varices in a child, six years after a Fontan procedure for tricuspid atresia. She...
Analysis of hemodynamics inside tricuspid atresia (TA) chamber is essential to the understanding of TA for optimal treatment. In this study, we introduced a combined computational fluid dynamics (CFD) to simulate blood flow in the left ventricle (LV) to study the diastolic flow changes in TA.
Real-time 3-dimentional echocardiography loops (ECHO) we...
Background:
Cardiac valvulogenesis is a highly conserved process among vertebrates and cause unidirectional flow of blood in the heart. It was precisely regulated by signal pathways such as VEGF, NOTCH, and WNT and transcriptional factors such as TWIST1, TBX20, NFATC1, and SOX9. Tricuspid atresia refers to morphological deficiency of the valve and...
Tricuspid atresia (TA) is a complex congenital heart disease that presents with cyanosis in the neonatal period. It is invariably fatal if left untreated and requires multiple stages of palliation. Early recognition and timely surgical intervention are therefore pivotal in the management of these infants. This literature review considers the pathop...
Citations
... Congenital giant right atrium may sometimes be mistaken as EA. 4 EA has variable clinical course. Long-term prognosis depends on the severity of TR and the right to left shunt. ...
Ebstein’s anomaly (EA) is a rare congenital heart disease. Paradoxical embolism (PDE) may occur in EA due to associated atrial septal defect (ASD). An 18 year old male with EA presented with left lower limb ischemia due to PDE to the limb. No features of cerebral embolism was present. PDE is a potential complication of EA. High level of awareness is needed for early diagnosis of PDE in EA to save a limb in case of embolism to the extremity. © 2016, Journal of Association of Physicians of India. All rights reserved.
Idiopathic enlargement of the right atrium (IERA) is a rare cardiac anomaly, and only sporadic cases have been reported. Little is known about its clinical relevance, and inconsistencies in medical and surgical management remain among different settings. In this paper, we systematically reviewed the published cases of the IERA in terms of clinical presentation , diagnosis, and management. A total of 153 cases of IERA were covered. Arrhythmia, dyspnea, and palpitation were found to be the most common clinical manifestations. It tends to be associated with life-threatening complications and sudden cardiac death. Diagnosis was mostly established by using echocardiography. Presenting symptoms, abnormal ECG findings, and therapeutic modalities were significantly related to the prognosis of IERA. Symptomatic patients were significantly more likely to have poor outcomes than asymptomatic patients (p = 0.044), and conservative treatment was more associated with adverse outcomes compared to surgical resection (p = 0.016). In conclusion, IERA, although rare, tends to be associated with potential life-threatening complications and sudden cardiac death. Echocardiography is the most common diagnostic modality. Surgical resection is indicated for symptomatic patients.
A 20‐year‐old Congolese woman presented with presyncope, dyspnea, and anasarca. Past medical history was unremarkable. Echocardiography revealed a rare combination of giant right atrium (RA), a dilated and hypertrophied right ventricle, subvalvular pulmonary stenosis (subPS), severe tricuspid regurgitation (TR), pericardial effusion and what appeared to be a spontaneously closed ventricular septal defect (VSD). Cardiac Magnetic Resonance and Cardiac Computed Tomography confirmed the findings excluding the presence of intra‐cardiac and extra‐cardiac shunt and other associated congenital anomalies. The patient underwent subPS resection, right atrioplasty, and tricuspid annuloplasty. Multimodality approach facilitated the detection of the abnormalities and provided clarity when determining the optimal surgical strategy.
