Idiopathic Dilatation of the Right Atrium: Clinical and Diagnostic Pitfalls – A Series of 3 Cases

Srinivasa, K; Kharge, Jayashree; Patil, Shivanand; Rangan, Kapil; Ramalingam, Rangaraj

doi:10.1111/echo.12312

Cited by 10 publications

(8 citation statements)

References 6 publications

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“…Altogether 13 patients had the thrombus formation within the enlarged RA [13-17]. Sudden cardiac death was reported in 6 patients, primarily due to refractory arrhythmia or RHF except 2 who died because of other associated anomalies [14, 18-22]. Altogether 6 patients developed tricuspid regurgitation, and 1 child had RA wall dissection [23].…”

Section: Resultsmentioning

confidence: 99%

Clinical Presentation, Diagnosis, and Management of Idiopathic Enlargement of the Right Atrium: An Analysis Based on Systematic Review of 153 Reported Cases

Zhang

et al. 2020

Cardiology

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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 (<i>p</i> = 0.044), and conservative treatment was more associated with adverse outcomes compared to surgical resection (<i>p</i> = 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.

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Section: Resultsmentioning

confidence: 99%

Clinical Presentation, Diagnosis, and Management of Idiopathic Enlargement of the Right Atrium: An Analysis Based on Systematic Review of 153 Reported Cases

Zhang

et al. 2020

Cardiology

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“…Reported symptoms of enlarged RA include dyspnea, palpitations, chest pain, right heart failure, syncope, and fatigue. However, half of patients with enlarged RA are asymptomatic at the time of diagnosis [ 1 – 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…The approach to treatment of isolated severe TR with dilatation of the RA is controversial. Surgical treatment is recommended in the presence of arrhythmias, congestive heart failure, or LV compression, which may cause sudden death [ 1 ]. In the majority of cases, asymptomatic patients can be treated conservatively.…”

Section: Discussionmentioning

confidence: 99%

“…Massive dilatation of the right atrium (RA) with tricuspid regurgitation (TR) is frequently diagnosed by accidental recognition of an enlarged cardiac silhouette during routine chest radiography. Although some patients are asymptomatic, TR is associated with arrhythmias and thrombus formation leading to pulmonary embolism, stroke, and, rarely, sudden death due to left ventricular (LV) compression [ 1 ]. TR may rarely cause massive dilatation of the RA.…”

Section: Introductionmentioning

confidence: 99%

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Surgical repair of massive dilatation of the right atrium with tricuspid regurgitation

Okada

Watanuki

Sugiyama

et al. 2018

J Cardiothorac Surg

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BackgroundMassive dilatation of the right atrium with tricuspid regurgitation is frequently diagnosed by accidental recognition of an enlarged cardiac silhouette during routine chest radiography. Although some patients are asymptomatic, enlargement of the right atrium can cause secondary tricuspid regurgitation due to dilatation of the tricuspid annulus, associated with arrhythmias and thrombus formation leading to pulmonary embolism, stroke, and, rarely, sudden death due to left ventricular compression.Case presentationA 76-year-old woman was followed up due to atrial fibrillation and tricuspid regurgitation for 8 years. A follow-up echocardiogram showed progressive dilatation of the right atrium. Because of the development of shortness of breath, right atrial plication and tricuspid valve repair were performed. Tricuspid annuloplasty was performed on the beating heart with the use of a 28-mm Carpentier-Edwards Physio tricuspid annuloplasty ring. Plication of the enlarged right atrium was performed at the interatrial septum, the free right atrium wall including the appendage, and the space between the inferior vena cava and the tricuspid ring. Closure of the left atrial appendage was performed from outside to prevent left atrial thrombus formation. Postoperative X-ray and computed tomography showed reduced cardiac silhouette and right atrial volume. The patient was discharged uneventfully and returned for follow-up visits with improved symptoms.ConclusionsAn adult case of massive dilatation of the right atrium of unknown etiology is reported. The patient’s symptoms were relieved by our operative procedure.

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“…[8][9][10] The clinical presentation ranges from no symptoms to arrhythmias, heart failure, and even sudden death. [11][12][13][14][15][16][17] Treatment is controversial in asymptomatic patients, especially in children. Prophylactic antiplatelet treatment has been suggested to prevent the risk of thrombus formation, 18 and surgical reduction has been performed in some symptomatic patients or in asymptomatic patients at high risk of progression, including initial severe right atrial dilatation, significant enlargement over a short period, compression of adjacent structures, or uncontrolled arrhythmias.…”

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confidence: 99%

Idiopathic dilatation of the right atrium: a not so benign entity

et al. 2020

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AbstractIdiopathic dilatation of the right atrium is an isolated enlargement of the right atrium in the absence of other cardiac lesions. This rare anomaly has a clinical spectrum ranging from asymptomatic to heart failure or even sudden death. It can be associated with atrial arrhythmias and thrombus formation. Antiplatelet therapy is prescribed in most cases reported in the literature, and reduction plasty is indicated when there is rapid growth of the right atrium, compression of adjacent structures, or refractory arrhythmias. We report four cases of idiopathic dilatation of the right atrium diagnosed during prenatal screening. We describe the intrauterine course and management in postnatal life until early childhood.

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Idiopathic Dilatation of the Right Atrium: Clinical and Diagnostic Pitfalls – A Series of 3 Cases

Cited by 10 publications

References 6 publications

Clinical Presentation, Diagnosis, and Management of Idiopathic Enlargement of the Right Atrium: An Analysis Based on Systematic Review of 153 Reported Cases

Clinical Presentation, Diagnosis, and Management of Idiopathic Enlargement of the Right Atrium: An Analysis Based on Systematic Review of 153 Reported Cases

Surgical repair of massive dilatation of the right atrium with tricuspid regurgitation

Idiopathic dilatation of the right atrium: a not so benign entity

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