Adélaïde Richard

Interest of β-blockers in patients with right ventricular systemic dysfunction.

BACKGROUND β-blockers improve the prognosis of patients with cardiac failure due to left ventricular systolic dysfunction. The aim of this study was to assess the efficacy of β-blockers in patients with dysfunctional systemic right ventricle. METHODS Fourteen patients with systemic right ventricle following a Mustard or a Senning operation for the transposition of the great arteries, or congenitally corrected transposition were included in the study. All had a decreased systemic right ventricular ejection fraction despite having standard cardiac failure therapy. Quality of life, New York Heart Association class, aerobic capacity, and systemic right ventricular function were assessed before treatment with β-blockers and at the end of the follow-up period, mean of 12.8 months with a range from 3 to 36 months. RESULTS Change in New York Heart Association class was significant (p = 0.016). Quality of life improved significantly throughout the study from a median grade 2 with a range from 1 to 3 to a median grade 1 with a range from 1 to 2 (p = 0.008). Systemic right ventricular ejection fraction assessed by radionuclide ventriculography improved significantly from a median of 41% (range: 29-53%) to 49% (range: 29-62%; p = 0.031). However, the change in thee ejection fraction assessed by magnetic resonance imaging was not significant from a median of 29% (range: 12-47%) to 32% (range: 22-63%; p = 0.063). CONCLUSION In patients with cardiac failure due to systemic right ventricular dysfunction, β-blockers improve New York Heart Association class, quality of life, and systemic right ventricular ejection fraction assessed by radionuclide ventriculography.

How Pregnancy Impacts Adult Cyanotic Congenital Heart Disease

Survival into adulthood of patients with unrepaired cyanotic congenital heart defects (CHDs) is possible when cyanotic CHDs are deemed unsuitable for radical surgical repair but are compatible with survival. These situations include, for example, complex pulmonary atresia with aortopulmonary collaterals and single-ventricle hearts (with and without earlier palliation). This is also the case when patients with cyanotic CHDs reach adulthood without serious symptoms requiring surgery, such as those with mild tetralogy of Fallot, Ebstein’s anomaly, and some cases of corrected transposition of the great arteries with pulmonary stenosis and ventricular septal defect. Many women with these heart conditions wish to become pregnant, which creates a situation of high maternal and fetal risks of complications.1 Management of these patients before, during, and after pregnancy has improved, with an earlier recognition of the underlying disease, improved understanding of cardiopulmonary physiopathology, better prenatal and peri-partum obstetric/anesthetic management, and the introduction of a multidisciplinary approach.2 We retrospectively reviewed the charts of all pregnant women with cyanotic CHDs (n=51) who were followed in 11 adult CHD referral …

0323: Mid-term follow-up and quality of life in patients after Fontan surgery

Background The Fontan procedure (atriopulmonary Fontan) and total cavo-pulmonary connection are designed to treat univentricular heart. Whereas peri-operative mortality has declined, the current challenge is long-term outcome. Objective To evaluate the outcome and quality of life of survivors with Fontan circulation. Methods This retrospective monocentric study aimed patients who had follow-up after Fontan surgery at the University Hospital of Lille. Data were collected on medical records. The quality of life was evaluated between June and October 2014 by two scales: Paediatric Quality of Life Inventory TM (PedsQL) before 26 years of age and Medical Outcome Study Short Form 36 (MOS SF 36) after 26 years. Results Among 96 patients who underwent Fontan procedure, median follow-up was 9.6 (6.1-12.5) years after the last intervention. Nine-year global survival was 93%. 95% of patients had total cavo-pulmonary connection and 5% had atrio-pulmonary connection. Arrhythmia occurred in 27.1%, single ventricle dysfunction in 87.4%, leak of the atrio-ventricular valve in 58.9%. Protein-losing enteropathy affected 4.2% of patients and thromboembolic events appeared in 17.7%. Total score of quality of life was 66.5% according to the PedsQL and 62.5% to the MOS SF36. Conclusion This French cohort of survivors with Fontan circulation has the same initial characteristics than which described in the literature. The level of quality of life was comparable to general population. The question of global rehabilitation of these patients must be raised.