SummaryBackgroundCongenital heart disease (CHD) is frequently described in patients with Down syndrome (DS) and is the main cause of death in this population during the first two years of life. The spectrum of CHD patterns in DS varies widely worldwide; this variation could be due to sociodemographic, genetic and geographic factors.MethodsA six-year retrospective, descriptive study was carried out from December 2008 to October 2014, based on the Paediatric Unit CHD registry of Ibn Rochd University Hospital. Clinical, echocardiographic and outcomes data were collected and sorted according to confirmation of the syndrome.ResultsAmong 2 156 patients with CHD, 128 were identified with Down syndrome. The genders were equally represented (gender ratio 1) and the median age at diagnosis was 9.5 months (2 days to 16 years). The median age of mothers at delivery was 39 years (16–47). Of the 186 CHD lesions reported, the most common was atrioventricular septal defect (AVSD, 29%), followed by ventricular septal defect (VSD,21.5%) and atrial septal defect (ASD, 19.9%). The most common associations of CHD were AVSD + ASD (10%) and VSD + ASD (7.8%). Surgery was the most common modality of treatment (54.3%). The overall mortality rate was 14.1%.ConclusionOur study confirmed that the profile and type of CHD in DS in the Moroccan setting exhibited slight differences in the distribution of these CHDs compared with European neighbours and other Western countries. Further studies are needed to determine which variables have an impact on these differences.
Development of direct-acting antiviral agents against hepatitis C virus (HCV) has changed the management of chronic HCV infection. We report three cases of newly diagnosed or exacerbated pulmonary arterial hypertension (PAH) in patients treated with sofosbuvir. All patients had PAH-associated comorbidities (HIV coinfection in two, portal hypertension in one) and one was already being treated for PAH. At admission, all patients presented with syncope, World Health Organization functional class IV, right-sided heart failure, and extremely severe hemodynamic parameters. After specific PAH therapy, the clinical and hemodynamic properties for all patients were improved. Severity and acuteness of PAH, as well as chronology, could suggest a causal link between HCV treatment and PAH onset. We hypothesize that suppression of HCV replication promotes a decrease in vasodilatory inflammatory mediators leading to worsening of underlying PAH. The current report suggests that sofosbuvir-based therapy may be associated with severe PAH.
BackgroundIsolated cleft mitral valve (ICMV) may occur alone or in association with
other congenital heart lesions. The aim of this study was to describe the
profile of cardiac lesions associated with ICMV and their potential impact
on therapeutic management.MethodsWe conducted a descriptive study with data retrieved from the Congenital
Heart Disease (CHD) single-center registry of our institution, including
patients with ICMV registered between December 2008 and November 2014.ResultsAmong 2177 patients retrieved from the CHD registry, 22 (1%) had ICMV. Median
age at diagnosis was 5 years (6 days to 36 years). Nine patients (40.9%) had
Down syndrome. Seventeen patients (77.3%) had associated lesions, including
11 (64.7%) with accessory chordae in the left ventricular outflow tract
(LVOT) with no obstruction, 15 (88.2%) had ventricular septal defect (VSD),
three had secundum atrial septal defect, and four had patent ductus
arteriosus. Thirteen patients (59.1%) required surgical repair. The decision
to proceed with surgery was mainly based on the severity of the associated
lesion in eight patients (61.5%) and on the severity of the mitral
regurgitation in four patients (30.8%). In one patient, surgery was decided
based on the severity of both the associated lesion and mitral
regurgitation.ConclusionOur study shows that ICMV is rare and strongly associated with Down syndrome.
The most common associated cardiac abnormalities were VSD and accessory
chordae in the LVOT. We conclude that cardiac lesions associated with ICMV
are of major interest, since in this study patients with cardiac lesions
were diagnosed earlier. The decision to operate on these patients must take
into account the severity of both mitral regurgitation and associated
cardiac lesions.
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