[Evaluation of ventricular asynchronism by Doppler tissue imaging in patients with idiopathic dilated cardiomyopathy]. / Evaluation de l'asynchronisme ventriculaire par échocardiographie Doppler tissulaire chez les patients atteints d'une cardiomyopathie dilatée idiopathique à QRS fins.

OBJECTIVES: To study ventricular asynchronism with tissue Doppler imaging in patients with primitive dilated cardiomyopathy and narrow QRS. PATIENTS AND METHODS: We compared a group of patients with DCM and QRS < 120 ms (gr 1, n=25, 52+/-14 yrs, LVEF: 25+/-9%) with a group of normal patients (gr 2, n=16, 36+/-20 yrs). We measured the delays between the beginning of QRS and the beginnings of aortic (QA), mitral (QM), tricuspid (QT) and pulmonary (QP) flows, and of systolic (QSm) and protodiastolic (QEm) wall motion waves recorded with TDI in the basal portion of interventricular septum (IVS) and LV and RV free walls. We then calculated the differences QA-QP, QM-QT, the interparietal differences for QSm and QEm, and the maximal interparietal systolic (QSm max) and diastolic (QEm max) delays. RESULTS: QA, QP, QM and QT were significantly lengthened in group 1 patients but there were no difference between both groups for QA-QP and QM-QT. There was a trend toward a lengthening of QSm and QEm in group 1 patients. Interparietal differences of QSm and QEm were similar in both groups; however, QSm max and QEm max were significantly longer in group 1 patients than in group 2. CONCLUSION: Doppler study of patients with DCM and narrow QRS shows a lengthening of all electromechanical delays and suggests some degree of ventricular asynchronism by showing a significant increase in maximal interparietal systolic and diastolic delays.

[Familial diffuse interstitial fibrosis of prolonged onset worsening during pregnancy]. / Pneumopathie interstitielle diffuse fibrosante familiale d'évolution prolongée aggravée au cours d'une grossesse.

INTRODUCTION: Familial idiopathic interstitial pulmonary fibrosis is rare. In this case report the diagnosis was confirmed histologically in four members of the same family. CASE REPORT: A woman whose father and two paternal uncles had developed pulmonary fibrosis was hospitalised from birth on account of delayed growth and dyspnoea. At the age of one year an increase in dyspnoea and the development of hypoxaemia and diffuse interstitial shadowing led to a surgical lung biopsy. The histological diagnosis was idiopathic interstitial fibrosis. Immunosuppressive treatment for one year led to clinical improvement with relief of the hypoxaemia but persistence of the interstitial shadowing. A pneumothorax at the age of 15 required pleurectomy. The clinical state remained stable with a restrictive ventilatory defect up to the age of 26 when respiratory insufficiency developed in the course of pregnancy. The outcome following delivery was severe respiratory failure complicated by pulmonary arterial hypertension leading to death the following year. CONCLUSIONS: This case is distinguished by a histological diagnosis in four members of a family of whom one was an infant, the prolonged stabilisation after immunosuppressive therapy and the possible role of pregnancy in the progression.