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Pulmonary homografts are standard alternatives to right ventricular outflow tract reconstruction in congenital heart surgery. Unfortunately, shortage and conduit failure by early calcifications and shrinking are observed for small-sized homografts in younger patients. In neonates, Contegra® 12 mm (Medtronic Inc., Minneapolis, Minnesota, United States of America) could be a valuable alternative, but conflicting evidence exists. There is no published study considering only newborns with heterogeneous pathologies. We retrospectively compared the outcomes of these two conduits in this challenging population.
Patients who underwent a right ventricular outflow tract reconstruction between January 1992 and December 2014 at the Hôpital Universitaire des Enfants Reine Fabiola were included. We retrospectively collected and analysed demographic, echocardiographic, surgical, and follow-up data.
Of the 53 newborns who benefited from a right ventricular outflow tract reconstruction during the considered period, 30 received a Contegra 12 mm (mean age 15 ± 8 days), and 23 a small (9–14 mm) pulmonary homograft (mean age 10 ± 7 days). Overall mortality was 16.6% with Contegra versus 17.4% in the pulmonary homograft group (p = 0.98 log-rank). Operative morbidity and early re-operation for conduit failure were not significantly different between the two groups. Mean follow-up in this study is 121 ± 74 months. Survival free from re-operation was not different between the two groups (p = 0.15). Multivariable analysis showed that weight and significant early gradient were factors associated with anticipated conduit failure.
Contegra 12 mm is a valid alternative to small pulmonary homografts in a newborn patient population. Trial registration: NCT03348397.
Women with congenital heart disease are often considered to be restricted in their obstetrical life and even their marital life. Our single-centre study aimed to determine the real-life situation of these women with regard to successful family life and any pregnancy complications they may experience.
From our database of adults with congenital heart disease, 160 of 178 women completed a questionnaire and had their files reviewed. They were classified into three groups according to their pregnancy risk – “good condition” group, no pregnancy restriction; “at-risk” group, pregnancy allowed with close follow-up at a tertiary centre; and “contraindicated” group, pregnancy inadvisable.
The proportion of women in a relationship was 46% with no difference between the three groups. In the groups where pregnancy was allowed, 55% of women conceived a child. The total incidence of spontaneous abortion was 21%. The rate of caesarean section was 15%. The incidence of cardiac failure was 4.7%, arrhythmia 1.2%, endocarditis 1.2%, hypertension 2.4%, and preeclampsia 1.2%. Foetal complications included prematurity and/or low birth weight (9.5%) and one foetal malformation (0.82%).
Women with severe congenital heart disease are willing to start a family and are successful in this enterprise. Although the complication rate during pregnancy in congenital heart disease remains high, with good monitoring these pregnancies occur without severe complications and a low rate of medical abortion or caesarean section.
To investigate the effect of bosentan in patients with a failing Fontan circulation.
A multicentric open label, non-controlled study.
5 tertiary care centres for congenital cardiology.
We included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks.
Main measures of outcomes
We assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded.
Of the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued.
Our study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.
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