The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results

INTRODUCTION: We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. METHODS: A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. RESULTS: A total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of <6%. Thirteen percent received an implantable cardioverter-defibrillator. After a median follow-up of 3.3 years (interquartile range [P25-P75] 1.3-6.5 years), 31% were asymptomatic. All-cause mortality was 1.19%/year and cardiovascular mortality 0.65%/year. The incidence of heart failure-related death was 0.25%/year, of sudden cardiac death 0.22%/year and of stroke-related death 0.04%/year. Heart failure-related death plus heart transplantation occurred in 0.27%/year and sudden cardiac death plus equivalents occurred in 0.53%/year. CONCLUSIONS: Contemporary HCM in Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for disease-specific treatments that impact the natural history of the disease

Stent for Life in Portugal: This initiative is here to stay

AIMS: Portugal has one of the lowest rates of primary percutaneous coronary intervention (p-PCI) in Western Europe. This study assessed the progress of Portuguese p-PCI performance indicators one year after Portugal joined the Stent for Life (SFL) initiative. METHODS AND RESULTS: Two national surveys were carried out, each covering a period of one month: the first when Portugal joined the SFL in 2011 (Moment Zero), and the second one year later (Moment One). A total of 397 consecutive patients with probable ST-segment elevation myocardial infarction were enrolled (201 at Moment Zero and 196 at Moment One) from 15 centers. During this period, the number of patients who arrived at a local hospital without p-PCI decreased (62-47%; p=0.004) and transportation to a p-PCI hospital by the National Institute for Medical Emergencies (INEM) increased significantly (13-37%; p<0.001). Shorter times to revascularization were observed, due to shorter patient delay (118-102 min; p=0.008). Door-to-balloon delay and system delay remained unchanged. CONCLUSIONS: Improvements in performance indicators for p-PCI demonstrate the success of the first year of the local SFL plan, which was mainly focused on raising public awareness of the need to use the INEM emergency services, which has reduced patient delay, and on improving secondary transportation

Phytoestrogen intake and other dietary risk factors for low motile sperm count and poor sperm morphology

Background Few potentially modifiable risk factors of male infertility have been identified, and while different diets and food groups have been associated with male infertility, evidence linking dietary factors including phytoestrogens and semen quality is limited and contradictory. Objectives To study the associations between phytoestrogen intake and other dietary factors and semen quality. Materials and Methods A case‐referent study was undertaken of the male partners, of couples attempting conception with unprotected intercourse for 12 months or more without success, recruited from 14 UK assisted reproduction clinics. A total of 1907 participants completed occupational, lifestyle and dietary questionnaires before semen quality (concentration, motility and morphology) were assessed. Food intake was estimated by a 65‐item food frequency questionnaire (FFQ) covering the 12 months prior to recruitment. Analyses of dietary risk factors for low motile sperm concentration (MSC: <4.8 × 106/mL) and poor sperm morphology (PM: <4% normal morphology) used unconditional logistic regression, accounting for clustering of subjects within the clinics, first without, and then with, adjustment for confounders associated with that outcome. Results High consumption of daidzein (≥13.74 μg/d), a phytoestrogen found in soy products, was a protective factor for MSC with an odds ratio (95%CI) of 0.58 (0.42‐0.82) after adjustment for clustering and potential confounding. Dietary risk factors for PM after similar adjustment showed that drinking whole milk (OR 0.67, 95%CI 0.47‐0.96) and eating red meat were protective with an OR 0.67 (0.46‐0.99) for eating red meat >3 times/wk. Discussion In this case‐referent study of men attending an infertility clinic for fertility diagnosis, we have identified that low MSC is inversely associated with daidzein intake. In contrast, daidzein intake was not associated with PM but eating red milk and drinking whole milk were protective. Conclusions Dietary factors associated with semen quality were identified, suggesting that male fertility might be improved by dietary changes

Cystic fibrosis and pregnancy. Report from French data (1980–1999)

Objective To study the consequences of pregnancy on women affected by cystic fibrosis and to clarify the impact of the disease on maternal and newborn health. Design Retrospective study. Setting Pregnancy survey from the French Cystic Fibrosis Registry. Population Women with cystic fibrosis having a pregnancy between 1980 and 1999. Methods During the 1980–1999 period, 90 pregnancies in 80 French female patients were registered in the pregnancy survey of the French Cystic Fibrosis Registry. General and clinical data before pregnancy were noted. Outcome of the pregnancy was described. Variations of pulmonary function and body weight during pregnancy were evaluated. Comparison between a group of pregnant women and a group of non-pregnant cystic fibrotic women of same age and genotype, followed in the same care centre network, was made. Main outcome measures Spirometric and nutritional parameters, vital status and perinatal health indicators. Results The outcome was identified for 75 cases: 64 delivered babies (45 at term and 10 prematurely, prematurity rate: 18%), 10 abortions (five spontaneous and five therapeutic or medical), and one maternal death during pregnancy. The proportion of newborns with low weight was 29.8%. Mean maternal weight gain during pregnancy was 5.5 kg. Four affected children were diagnosed after birth. A decline in the forced expiratory volume in one second (FEV1) and forced vital capacity was observed between the beginning of pregnancy and the year following the delivery. However, no significant difference was found when comparing the variation in the pulmonary function during pregnancy between cases and controls. Moreover, the pulmonary status before pregnancy was better than the status of non-pregnant women. Among 12 deaths recorded after pregnancy, only three happened in the year following the pregnancy. All three women had an FEV1 <50% before pregnancy. Conclusion Pregnancy only has a slight adverse effect on maternal health if the women are in good general condition before starting pregnancy. Women with a better health status are more inclined to initiate and successfully complete a pregnancy. Complete collaboration between cystic fibrosis practitioners and obstetricians should be observed to allow women to deliver children in the best conditions