Cor Triatriatum: Operative Results in 20 Patients

Qethamy, Howaida O Al; Aboelnazar, Saber; Faraidi, Yahya Al; Jubair, Khalid Al; Gadura, Mohamed; Fagih, Mohamed Al

doi:10.1177/021849230601400103

Cited by 19 publications

(18 citation statements)

References 8 publications

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“…2 Anomalous venous return is a frequently associated condition in 11-53% of other congenital cardiac anomalies associated with cor triatriatum. [2][3][4] Many types of partial -left or right -or total anomalous pulmonary venous connections are described to the azygos vein, the innominate vein, and the coronary sinus. 1,5 Lucas, in 1995, combined elements of several classifications of cor triatriatum into a comprehensive classification.…”

Section: Commentmentioning

confidence: 99%

Cor triatriatum with a left upper anomalous pulmonary venous return to the innominate vein

Galoin-Bertail

Gouton

2013

Cardiol Young

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Cor triatriatum sinister is an uncommon congenital cardiac anomaly accounting for 0.1% of all congenital cardiac malformations, with an anomalous venous return being a frequently associated condition. We present the case of a 14-year-old girl misdiagnosed as having an atrial septal defect with pulmonary hypertension who actually had a cor triatriatum with a left upper anomalous pulmonary venous return to the innominate vein.

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Section: Commentmentioning

confidence: 99%

Cor triatriatum with a left upper anomalous pulmonary venous return to the innominate vein

Galoin-Bertail

Gouton

2013

Cardiol Young

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“…Two of them (25%) had moderate reduction in PA pressures with RV dysfunction in postoperative period. Huang et al and Al Qethamy et al also reported zero mortality after a mean follow‐up duration of 52 months and 31 months, respectively. These excellent results may be because of the surgical repair done in the current era with better intraoperative and perioperative management techniques and facilities and also may be due to the less complexity of the associated lesions.…”

Section: Discussionmentioning

confidence: 90%

Surgical experience with cor triatriatum repair beyond infancy

et al. 2019

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Background: Cor triatriatum is an exceedingly rare congenital heart defect which can present at any age depending upon the presence of associated cardiac anomalies and the size of communication between the common chamber and the left atrium. Our aim is to present 10 years surgical outcome of cor triatriatum repair in patients who present beyond infancy.Methods: On going through our institute's surgical database from January 2009 to June 2019 for cor triatriatum, 14 patients were found to have undergone the surgical correction. Their demographic profile and midterm outcome in terms of mortality, functional status, and cardiac function was assessed.Results: There were total 14 patients with slight female predominance (57.14%) who underwent cor triatriatum surgical repair. The mean age at the time of surgery was 12.14 ± 9.97 years ranging from 1 to 29 years. Associated congenital cardiac defects were seen in 12 (85.71%) patients with atrial septal defect being the most common.Partial anomalous pulmonary venous connection was seen in 4 (28.57%) patients.Eight (57.14%) patients had severe pulmonary hypertension preoperatively. All but two patients showed marked reduction in pulmonary artery pressures postoperatively. All the patients underwent excision of the membrane and repair of associated cardiac defects simultaneously. There was no early or midterm mortality or any reintervention over a mean follow-up period of 58.06 ± 30.73 months (range 20-120 months). Conclusion:The early and midterm surgical outcome of cor triatriatum is excellent even in patients who present beyond infancy with reversible pulmonary artery hypertension. K E Y W O R D S common chamber, cor triatriatum sinister, left atrium, triatriatum 1 | INTRODUCTION Cor triatriatum sinister is a very rare disease seen in <1 in 1000 patients with congenital cardiac defects. 1 In cor triatriatum the left atrium is typically divided by a fibromuscular membrane, resulting in a postero-superior positioned proximal cavity and an anterio-inferior positioned distal cavity containing the left atrial appendage. It has to be differentiated from the supramitral ring or membrane which lies distal to the left atrial appendage. Clinical presentation is dependent on the degree of functional pulmonary venous obstruction and the presence of associated congenital cardiac lesions. Early presentation resulting in early treatment is the usual management of the patients with cor triatriatum. When the communication between the pulmonary venous chamber and the left atrium is small (<3 mm), almost 75% of the patients die in infancy if left untreated. 2 The naturally selected patients who survive infancy are those with either lesser degree of stenosis at the communication site and/or with a large ASD which protects the pulmonary venous vasculature for some time. However, as the age increases, their pulmonary artery pressure rises and they become symptomatic and present with signs and symptoms of mitral stenosis and/or large ASD. 2 Our aim was to assess the surgical outcome in patient...

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“…Only a few studies on CTS in infancy are available, and most paediatric patients are identified early due to severe membrane obstruction. CHF, recurrent respiratory tract infections and failure to thrive are the most frequent findings in children 27 29–33. According to the literature data, the prevalence of asymptomatic children within the CTS population varies from 0% to 75% 2 23 24 26 29–34…”

Section: Discussionmentioning

confidence: 99%

Cor triatriatum sinistrum diagnosed in the adulthood: a systematic review

Rudienė

Hjortshøj

Glaveckaitė

et al. 2019

Heart

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BackgroundWe performed a systematic review of cor triatriatum sinistrum (CTS) diagnosed in adults. The aim of this review was to describe the clinical presentation, natural history and management of this congenital heart disease.MethodsA PubMed literature search for ‘cor triatriatum sinistrum’ published since 2005 was performed. Included patients were divided into those with and without obstructive membrane physiology. The clinical course differences were compared.ResultsA total of 171 published cases were included. The median age at diagnosis was 43 years (IQR, 30–60). Obstructive membrane physiology was observed in 70 (41%), and this patient group was younger at presentation (median age 39 (IQR, 28–52) vs 50 years (IQR, 32–64), p=0.003).Patients with obstructive membrane more frequently had associated cardiac defects (58.6% vs 42.4%, p=0.039). Overall, the most frequent clinical symptom was atrial fibrillation, as this was present in 56 (32.8%) of all patients. CTS-related symptoms were more frequent in patients with obstructive membrane: congestive heart failure (44.3% vs 15.2%, p<0.001), pulmonary hypertension (27.1% vs 6.1%, p<0.001), haemorrhage (8.6% vs 0%, p=0.004) and infections manifestation (8.6% vs 0%, p=0.004).A total of 71 (41.5%) patients with CTS required interventional treatment, mainly within patients with the obstructive membrane (86.8% vs 12.6%, p<0.001).ConclusionThe natural history of CTS most often manifests with symptoms of congestive heart failure. Patients with obstructive membrane most often have associated cardiac defects and higher risk for infections and haemorrhage. The interventional treatment of CTS remains the first choice for obstructive membrane.

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Cor Triatriatum: Operative Results in 20 Patients

Cited by 19 publications

References 8 publications

Cor triatriatum with a left upper anomalous pulmonary venous return to the innominate vein

Cor triatriatum with a left upper anomalous pulmonary venous return to the innominate vein

Surgical experience with cor triatriatum repair beyond infancy

Cor triatriatum sinistrum diagnosed in the adulthood: a systematic review

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