Cor triatriatum

Treatment of
non-restrictive cor triatriatum sinister during concomitant cardiac surgery. Apical 4-chamber on magnetic resonance. RA: right atrium; MV: mitral valve; CTS: cor triatriatum sinister; PC: proximal chamber of left atrium; DC: distal chamber of left atrium (see text for description)
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Cor
triatriatum sinistrum in adult female patient. Left atrial enlargement divided into two compartments by a membrane.
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Cor
triatriatum sinistrum in adult female patient. Left atrial enlargement divided into two compartments by a membrane.
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Cor
triatriatum sinistrum in adult female patient. Left atrial enlargement divided into two compartments by a membrane.
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Cor
triatriatum sinistrum in adult female patient. Moderate left atrial dilation divided into two compartments by a membrane of 2mm thickness.
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Cor
triatriatum sinistrum in adult female patient. Moderate left atrial dilation divided into two compartments by a membrane of 2mm thickness.
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Cor
triatriatum sinistrum in adult female patient. Membrane of 2mm thickness divided left atrial.
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Cor
triatriatum sinistrum in adult female patient. Membrane of 2mm thickness divided left atrial.
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Treatment of
non-restrictive cor triatriatum sinister during concomitant cardiac surgery. Post-operative echography showing minimal residual fenestration in left atrium
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A
six-chambered heart: cor triatriatum sinister with double-chambered right ventricle in association with ventricular septal defect. Transthoracic echocardiography. A Parasternal short axis view showing the hypertrophied muscle bands (arrow) with distal infundibular chamber (*). B Apical four-chamber view showing the membranous partition in the LA (arrow) with turbulent flow across its aperture. C Parasternal long axis view showing the perimembranous VSD (arrow). (Ao-aorta)
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A
six-chambered heart: cor triatriatum sinister with double-chambered right ventricle in association with ventricular septal defect. CT Angiography. A Coronal section showing double SVC draining into RA. B Four-chamber view showing cor triatriatum, VSD (*) and double SVC. C Two-chamber view showing the infundibular chamber and the hypertrophied obstructing muscle bands (arrow) dividing the RV into two chambers. 1—Right atrium, 2—posterior chamber of left atrium, 3—anterior chamber of left atrium, 4—right ventricle, 5—left ventricle, 6—right superior vena cava, 7—left superior vena cava, 8—infundibular chamber
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Cor
triatriatum sinister: a rare underlying cause of pulmonary hemosiderosis. Bilateral lung markings thickening and prominent pulmonary artery segment (white arrow)
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Cor
triatriatum sinister: a rare underlying cause of pulmonary hemosiderosis. A 15 mm long high density band dividing the left atrium into true chamber and accessory chamber, with a 4-mm orifice connecting two chambers
springerplus.springeropen.com • CC-by-4.0
Cor
triatriatum sinister: a rare underlying cause of pulmonary hemosiderosis. a The cross section of CT shows bilaterally increased pulmonary vasculature, especially in the right side. b The coronal plane shows bilaterally increased pulmonary vasculature, especially in the right side
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Cor
triatriatum sinister: a rare underlying cause of pulmonary hemosiderosis. The left atrium’s division is visible, as well as left pulmonary veins draining into true chamber, right pulmonary veins draining into accessory chamber
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Cor
triatriatum sinister: a rare underlying cause of pulmonary hemosiderosis. Right pulmonary venous confluence distention (black arrow)
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Radiopaedia • CC-by-nc-sa 3.0 • de

Cor triatriatum is an extremely rare and serious congenital cardiac anomaly.

Epidemiology

It is thought to account for ~0.1% of all congenital cardiac anomalies .

Clinical presentation

Clinical presentation depends on:

  • degree of stenosis in the fibromuscular membrane
  • the integrity of the interatrial septum
  • presence of associated cardiovascular malformations.

It can be a cause of unexplained pulmonary hypertension in children. Typical presentation includes dyspnea, heart failure, and failure to thrive .

Pathology

There are two recognized subtypes:

  • classic cor triatriatum (cor triatriatum sinistrum / sinister): commoner
  • cor triatriatum dextrum/dexter

In the classical type, there is abnormal incorporation of pulmonary venous structures into the left atrium with an unnecessary fibromuscular membraneous subdivision through the atrial chamber.

With cor triatriatum dexter a similar scenario is seen through the right atrium.

Cor triatriatum exists along a spectrum from an imperforate membrane to a membrane with fenestrations, which may allow some passage of blood flow. A highly fenestrated membrane in cor triatrum dexter is sometimes referred to as a Chiari network.

Radiographic features

Plain radiograph

An isolated cor triatriatum demonstrates chest x-ray changes identical to that of mitral stenosis: the heart is normal sized with changes of chronic interstitial edema.

Echocardiography

The intra-atrial membrane characteristic of classic cor triatriatum is often best visualized in the transthoracic apical 4 chamber view, with the following features often observed ;

  • echogenic, linear density subdividing the left atrium into proximal (inferior) and distal (superior) chambers
    • extends from the posteroinferior to the anterosuperior atrial wall
    • proximal chamber will typically receive the pulmonary venous drainage
    • distal chamber usually communicates with the mitral valve and atrial appendage
  • use of color flow Doppler allows confirmation of the communication between atrial subdivisions
  • common associations which should be sought include
MRI

May allow direct visualization of membrane +/- accessory chamber in greater detail.

Treatment and prognosis

A cor triatriatum is usually fatal within the first two years of life. The membrane can be surgically resected. The prognosis is related to timely surgical intervention, the degree of obstruction between the two left atria, and the presence or absence of associated anomalies .

History and etymology

The word cor triatriatum essentially means "heart with three atria" due to the accessory chamber created by the membrane appearing as an extra atrium.

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu Cor triatriatum:
pulmonale
Hypertonie
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fibromuskuläres
Band im linken Vorhof
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