Chest

Chest

Volume 117, Issue 6, June 2000, Pages 1740-1748
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Review
Congenital Malformations of the Right Atrium and the Coronary Sinus

https://doi.org/10.1378/chest.117.6.1740Get rights and content

Study objectives

Congenital malformations of the right atrium (RA) and the coronary sinus (CS) are rare, and only sporadic cases have been reported. Little is known about the clinical relevance of this disorder. We report on two patients, one with a giant RA diverticulum, the other with a diverticulum of the CS, and review 103 cases of such malformations that have been reported previously.

Design

A MEDLINE search was performed to collect all cases of congenital malformations of the RA and the CS reported in the literature between 1955 and 1998. Cases were classified into the following categories: (1) congenital enlargement of the RA; (2) single diverticulum of the RA; (3) multiple diverticula of the RA; and (4) diverticulum of the CS. Clinical presentation and outcome of the different types of malformations were analyzed.

Results

The patients most frequently presenting with symptoms were those with diverticula of the CS (n = 28) followed by those with single diverticula of the RA (n = 13), multiple diverticula (n = 4), and congenital enlargements of the RA (n = 60). The percentages of symptomatic patients were 93, 84, 75, and 53%, respectively. Symptoms were frequently caused by arrhythmias. Supraventricular tachycardia (SVT) was found in 42 of the patients (40%) and was most common in patients with diverticula of the CS (24 of 28 patients) and multiple atrial diverticula (3 of 4 patients). Sudden cardiac death was reported more frequently in patients with diverticula of the CS (18%) compared to those with congenital enlargement of the RA (5%) or single or multiple diverticula of the RA (6%). All seven patients with diverticula of the CS who were not treated with catheter or surgical ablation eventually died.

Conclusion

Congenital malformations of the RA and the CS frequently are associated with arrhythmias. SVT and sudden cardiac death have been reported in a significant percentage of patients with diverticula of the CS.

Section snippets

Case 1

A 40-year-old white man presented with atrial fibrillation and chest discomfort. A chest radiograph made during childhood had revealed a grossly enlarged heart, but no definite diagnosis had been made. The patient had never been seriously ill. An ECG revealed atrial fibrillation with a ventricular rate of 100 beats/min, right axis deviation, and nonspecific T-wave abnormalities. Chest radiography (Fig 1) showed a markedly enlarged cardiac silhouette. Laboratory findings were normal.

Data Sources

We performed a search of the international literature (MEDLINE) from 1955 through November 1998. Articles without individual case data and duplicate reports were excluded. In addition, cases with possible secondary causes of RA enlargement such as atrial septal defects, anomalous pulmonary venous return, Ebstein's anomaly, and other lesions of the tricuspid or pulmonary valve, and pulmonary hypertension were excluded.

Data Analysis

The cases were classified into the following categories: (1) congenital

Data Sources

The MEDLINE search revealed 141 patients reported in the literature. Thirty-eight patients had to be excluded. Thus, 103 patients that were reported on in 77 articles were analyzed. With the addition of the two cases from our institution, a total of 105 patients were studied.

Classification

The most commonly reported type of malformation was a congenital enlargement of the RA (n = 60) followed by diverticulum of the CS (n = 28), single diverticulum of the RA (n = 13), and multiple diverticula of the RA (n = 4).

Discussion

There are four distinct types of malformations of the CS and the RA: congenital enlargement of the RA; single RA diverticulum; multiple diverticula of the RA; and diverticula of the CS. It is difficult to estimate the true incidence of the various subgroups since smaller diverticula, especially in asymptomatic patients, easily could be missed. Particularly for patients with diverticula of the CS in whom diagnosis generally requires angiography of the CS, the diagnostic procedure will be

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