Copyright © 2005, The European Society of Cardiology
Spontaneous echo contrast of unexpected etiology
Prof. Dr. C.C. Iliescu Institute of Cardiovascular Diseases, 258 Fundeni Str., 022328 Bucharest, Romania
Received 7 February 2005; received in revised form 4 May 2005; accepted after revision 18 May 2005.
ioanagh2001{at}yahoo.com
silviavilcu{at}xnet.ro
mefinbuc{at}fx.ro
cosmin.c{at}xnet.ro
pavelplaton{at}yahoo.com
apetrei{at}fx.ro
* Corresponding author. Tel.: +40 7450 22299; fax: +40 2124 08963. marinelaserban{at}yahoo.com
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Abstract |
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Cor triatriatum sinister is a rare congenital anomaly characterized by the presence of a fibromuscular membrane dividing the left atrium into two chambers: one entering the four pulmonary veins, the other connecting to the mitral valve. The extent of the communication between the two chambers and the presence of associated lesions determine the severity of symptoms and the complications. We report the case of a 20-year-old man firstly diagnosed with obstructive cor triatriatum sinister and severe pulmonary hypertension.
Keywords: Cor triatriatum; Spontaneous echo contrast; Pulmonary hypertension
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Case report |
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A 20-year-old previously healthy man was admitted to our hospital with a two-month history of progressive exertional dyspnoea and haemoptysis. Clinical examination revealed orthopnea, central cyanosis, pronounced elevation of jugular venous pressure, pulsatile hepatomegaly (6cm) and moderate peripheral edema. There was an accentuated second heart sound and tricuspid regurgitation. ECG showed right ventricular hypertrophy. Severe pulmonary arterial hypertension was seen on chest radiography.
Transthoracic echocardiography revealed the presence of a tensed membrane dividing the left atrium. Transoesophageal echocardiography (TEE) was performed to complete morphological characteristics. It showed a membrane with calcifications at its attachment to the atrial septum. Laterally it was attached to the junction between the left upper pulmonary vein and left atrial appendage. TEE also showed intense spontaneous echo contrast into the proximal chamber (Fig. 1), where all pulmonary veins were draining (Fig. 1).
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Colour Doppler showed an aliased high-velocity flow jet, with proximal isovelocity surface area, across the diaphragm (Fig. 2). CW Doppler was used to assess the severity of the obstruction: the mean transmembrane gradient was estimated at 18mmHg (Fig. 3). Severe pulmonary hypertension was also demonstrated by 2D and CW Doppler echo. Systolic pulmonary arterial pressure (PAP) estimated by the Bernoulli equation of the tricuspid regurgitant jet was 128mmHg. A dilated right ventricle compressed the left ventricle and the curvature of the interventricular septum was reversed in both systole and in early mid-diastole.
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Discussion |
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TopAbstractCase reportDiscussionReferences |
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Cor triatriatum sinister is a rare cardiac malformation consisting of a fibromuscular diaphragm dividing the left atrium into two chambers. The postero-superior proximal chamber usually receives the pulmonary veins. The antero-inferior or distal chamber includes the left atrial appendage and connects to the mitral orifice.
The extent of the communication between the two chambers and the presence of associated lesions determine the severity of symptoms and the complications.1
In our case cor triatriatum was an isolated finding, with all four pulmonary veins draining into upper posterior chamber and a restrictive communication between chambers. Despite the long-term evolution of pulmonary hypertension, the patient was asymptomatic for the first two decades of life.
The ultrasonographic aspect is quite particular. The increased pressure against the membrane induces its convex shape towards the mitral valve. Blood stagnation in the proximal chamber causes a very intense spontaneous contrast. At first sight, the association gives the impression of an exogenous compression of the left atrium. Atypically located pericardial cysts compressing the left atrium and the outflow of pulmonary veins were occasionally reported, with similar echocardiographic aspect.2
Thinking of a surgical procedure, a right cardiac catheterism was performed. Systolic and mean pulmonary artery pressure was 146mmHg and 110mmHg, respectively. Pulmonary capillary wedge pressure was 36mmHg. Total pulmonary vascular resistance was 56.3Wood units, whereas arterial pulmonary resistance was 37.8Wood units. The systemic vascular resistance was 36.9Wood units. Haemodynamic parameters did not suffer any significant changes after oxygen supplementation. Unfortunately, we did not have either prostacyclin or nitric oxide with which to do an acute vasoreactivity test. Under these circumstances the operative risk was estimated to be unacceptably high. We considered an alternative interventional procedure (balloon atrial septostomy), but the emboligenic risk, due to stasis and the intense spontaneous contrast, was also considered too high.
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References |
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TopAbstractCase reportDiscussionReferences |
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- Buchholz S., Jenni R. Doppler echocardiographic findings in 2 identical variants of a rare cardiac anomaly, ‘subtotal’ cor triatriatum: a critical review of the literature. J Am Soc Echocardiogr (2001) 14:846–849.[CrossRef][Web of Science][Medline]
- Antonini-Canterin F., Piazza R., Ascione L., Pavan D., Nicolosi G.L. Value of transesophageal echocardiography in the diagnosis of compressive atypically located pericardial cysts. J Am Soc Echocardiogr (2002) 15:192–194.[CrossRef][Web of Science][Medline]
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