MRI evaluation of right heart functions in children with mild cystic fibrosis.

BACKGROUND: This study aimed to assess the ventricular anatomy, function of the right ventricle, and the haemodynamic findings of pulmonary artery in children with cystic fibrosis using cardiac MRI. PATIENTS: This prospective study consisted of 32 children with mild cystic fibrosis and 30 age-matched healthy control participants. METHODS: Cardiac MRI was used to assess right ventricular volumes, anatomy, and function and to assessment of haemodynamic findings of pulmonary artery in the control and study groups. Haemodynamic findings of pulmonary arteries were determined using pulmonary arteries peak velocity (cm/s), and pulmonary arteries time-to-peak velocity (ms) and pulmonary artery systolic pressure. All data of children with mild cystic fibrosis were compared with those of 30 age-matched healthy control group participants. RESULTS: Our patients and their age-matched controls were aged from 6 to 17 years and from 7 to 15 years, respectively. We found that ejection fraction (%), cardiac output (L/ml), cardiac output (L/ml/m2), and systolic volume (ml/m2) were significantly lower in children with cystic fibrosis (p < 0.01). Right ventricular anterior wall thickness (mm) was significantly higher in children with cystic fibrosis (p = 0.01). No significant difference was observed between the haemodynamic parameters of pulmonary artery in the patient group. CONCLUSION: In our study, cardiac MRI was used to investigate whether the right ventricle was affected functionally and anatomically in children with mild cystic fibrosis. We detected a significant decrease in right ventricular systolic functions and notable alterations in the right ventricular geometry of children with mild cystic fibrosis. These alterations usually manifest themselves as hypertrophy of the right ventricle. Our study's results demonstrate no relationship between the development of pulmonary hypertension in mild cystic fibrosis children.

[Atypical form of cor triatrium in a 59-year-old man]. / Forme atypique de cœur triatrial chez un patient de 59ans.

Cor triatrium is a rare congenital heart disease typically diagnosed amongst very young patients. Delayed diagnosis in the elderly is much more unusual. We report the case of a 59-year-old male with unremarkable medical history in whom we have discovered, on an echocardiography performed for an exploration of transient ischemic accident with atrial fibrillation, a particular form of an isolated cor triatrium dextrum investigated with multimodality imaging.

[Giant pulmonary artery aneurysm: etiology and an exceptional 17 years natural course]. / Anévrisme géant de l'artère pulmonaire : aspects étiologiques et évolutifs exceptionnels sur 17ans.

True pulmonary artery aneurysm (AAP) is rare and represent less than 1% of intra-thoracic aneurysms. We report a case of a AAP in a patient with a likely cor triatrium sinister, with an obstructive membrane responsible for pulmonary hypertension, explaining AAP. The long-term evolution of 17 years is made to an uncomplicated myocardial infarction. The patient died eight months later suddenly probably due to the rupture of the PAA.