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Archives of cardiovascular diseases
Volume 103, n° 8-9
pages 489-490 (août 2010)
Doi : 10.1016/j.acvd.2009.12.008
Received : 15 November 2009 ;  accepted : 7 December 2009
A case of curable pulmonary hypertension
Une hypertension artérielle pulmonaire curable
 

Patrice Guérin a, , b , Phuong Hoang b, Olivier Baron c
a Inserm UMR 915, IRT-1, institut du thorax, 8, quai Moncousu, BP 70 721, 44007 Nantes cedex 1, France 
b Service d’hémodynamique, CHU de Nantes, institut du thorax, 44093 Nantes cedex 1, France 
c Service de chirurgie thoracique, CHU de Nantes, institut du thorax, 44093 Nantes cedex 1, France 

Corresponding author. Fax: +2 28 08 01 30.

Keywords : Pulmonary hypertension, Triatrial heart

Mots clés : Hypertension artérielle pulmonaire, Coeur triatrial


A 16-year-old girl was admitted for breathlessness. Her only medical history was asthma treated with a bronchodilator and steroids. Examination revealed hepatomegaly, lower limb oedema and a soft systolic murmur with loud second heart sound. Chest radiography revealed cardiomegaly with pulmonary congestion, and the electrocardiogram was suggestive of right ventricular hypertrophy (Figure 1A). Echocardiography showed an abnormal dilated right atrium and right ventricle and an estimated pulmonary artery systolic pressure, on tricuspid regurgitation, of 124mmHg (supra-systemic pulmonary hypertension) (Figure 1B). In addition, a thick membrane was noted in the left atrium, separating it into two chambers (Figure 1B, white arrow). This had an eccentrically placed orifice nearer to the septal margin of the membrane on transesophageal echography (Figure 1C, white arrow), with a significant diastolic gradient (38mmHg) (Figure 1B), causing postcapillary pulmonary hypertension (Supplementary data). A diagnosis of cor triatriatum was made (without atrial septal defect or anomalous pulmonary venous connection) and the findings of pulmonary hypertension were attributed to the restrictive membrane, mimicking mitral stenosis. A decision was made to proceed to surgical resection of the obstructing atrial membrane. The left atrium was divided by a thick, fibromuscular membrane, with a 3mm eccentrically placed orifice. The membrane was excised completely; the anatomy of the left atrium was inspected and showed no other abnormalities. The patient made an uneventful recovery following surgery; nevertheless, pulmonary hypertension persisted (80mmHg), and was treated by an endothelin receptor antagonist (bosentan) and oral anticoagulation. Two years later, pulmonary pressures were normalized and treatment interrupted definitively.



Figure 1


Figure 1. 

Chest radiography revealing a cardiomegaly with pulmonary congestion and ECG suggestive of right ventricular hypertrophy (A). Echocardiography showing a dilated right atrium and right ventricle and a pulmonary hypertension on tricuspid regurgitation (B). TTE showing a membrane in the left atrium (B, white arrow) with an eccentrically placed restrictive orifice on TOE (C, white arrow) responsible of a significant diastolic gradient (38mmHg) (B).

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Conflict of interest

None.


Appendix A. Supplementary data

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