Peripartum cardiomyopathy as a part of familial dilated cardiomyopathy.

BACKGROUND: Anecdotal cases of familial clustering of peripartum cardiomyopathy (PPCM) and familial occurrences of PPCM and idiopathic dilated cardiomyopathy (DCM) together have been observed, suggesting that genetic factors play a role in the pathogenesis of PPCM. We hypothesized that some cases of PPCM are part of the spectrum of familial DCM, presenting in the peripartum period. METHODS AND RESULTS: We reviewed our database of 90 DCM families, focusing specifically on the presence of PPCM patients. Then, in a reverse approach, we reviewed 10 PPCM patients seen in our clinic since the early 1990s and performed cardiological screening of the first-degree relatives of 3 PPCM patients who did not show a full recovery. Finally, we analyzed the genes known to be most commonly involved in DCM in the PPCM patients. We identified a substantial number (5 of 90, 6%) of DCM families with PPCM patients. Second, cardiological screening of first-degree relatives of 3 PPCM patients who did not show full recovery revealed undiagnosed DCM in all 3 families. Finally, genetic analyses revealed a mutation (c.149A>G, p.Gln50Arg) in the gene encoding cardiac troponin C (TNNC1) segregating with disease in a DCM family with a member with PPCM, supporting the genetic nature of disease in this case. CONCLUSIONS: Our findings strongly suggest that a subset of PPCM is an initial manifestation of familial DCM. This may have important implications for cardiological screening in such families.

Intracoronary versus intravenous administration of abciximab in patients with ST-segment elevation myocardial infarction undergoing primary percutaneous coronary intervention with thrombus aspiration: the comparison of intracoronary versus intravenous abciximab administration during emergency reperfusion of ST-segment elevation myocardial infarction (CICERO) trial.

BACKGROUND: administration of the glycoprotein IIb/IIIa inhibitor abciximab is an effective adjunctive treatment strategy during primary percutaneous coronary intervention for ST-segment elevation myocardial infarction. Although small-scale studies have suggested beneficial effects of intracoronary over intravenous administration of abciximab, this has not been investigated in a medium-scale randomized clinical trial. METHODS AND RESULTS: a total of 534 ST-segment elevation myocardial infarction patients undergoing primary percutaneous coronary intervention with thrombus aspiration within 12 hours of symptom onset were randomized to either an intracoronary or an intravenous bolus of abciximab (0.25 mg/kg). Patients were pretreated with aspirin, heparin, and clopidogrel. The primary end point was the incidence of restored myocardial reperfusion, defined as complete ST-segment resolution. Secondary end points included myocardial reperfusion as assessed by myocardial blush grade, enzymatic infarct size, and major adverse cardiac events at 30 days. The incidence of complete ST-segment resolution was similar in the intracoronary and intravenous groups (64% versus 62%; P=0.562). However, the incidence of myocardial blush grade 2/3 was higher in the intracoronary group than in the intravenous group (76% versus 67%; P=0.022). Furthermore, enzymatic infarct size was smaller in the intracoronary than in the intravenous group (P=0.008). The incidence of major adverse cardiac events was similar in both groups (5.5% versus 6.1%; P=0.786). CONCLUSIONS: in ST-segment elevation myocardial infarction patients undergoing primary percutaneous coronary intervention with thrombus aspiration, intracoronary administration of abciximab compared with intravenous administration does not improve myocardial reperfusion as assessed by ST-segment resolution. However, intracoronary administration is associated with improved myocardial reperfusion as assessed by myocardial blush grade and a smaller enzymatic infarct size.

Late onset of new conduction disturbances requiring permanent pacemaker implantation following TAVI.

BACKGROUND: The timing of onset and associated predictors of late new conduction disturbances (CDs) leading to permanent pacemaker implantation (PPI) following transcatheter aortic valve implantation (TAVI) are still unknown, however, essential for an early and safe discharge. This study aimed to investigate the timing of onset and associated predictors of late onset CDs in patients requiring PPI (LCP) following TAVI. METHODS AND RESULTS: We performed retrospective analysis of prospectively collected data from five large volume centres in Europe. Post-TAVI electrocardiograms and telemetry data were evaluated in patients with a PPI post-TAVI to identify the onset of new advanced CDs. Early onset CDs were defined as within 48 hours after procedure, and late onset CDs as after 48 hours. A total of 2804 patients were included for analysis. The PPI rate was 12%, of which 18% was due to late onset CDs (>48 hours). Independent predictors for LCP were pre-existing non-specific intraventricular conduction delay, pre-existing right bundle branch block, self-expandable valves and predilation. At least one of these risk factors was present in 98% of patients with LCP. Patients with a balloon-expandable valve without predilation did not develop CDs requiring PPI after 48 hours. CONCLUSIONS: Safe early discharge might be feasible in patients without CDs in the first 48 hours after TAVI if no risk factors for LCP are present.

Higher patient perceived side effects related to higher daily doses of inhaled corticosteroids in the community: a cross-sectional analysis.

UNLABELLED: The range and extent of inhaled corticosteroid (ICS) side effects experienced by patients in the general community are likely to be underestimated. AIMS: To identify the side effects of ICS perceived by patients in the community and, through the use of a self-report questionnaire, measure their intensity, prevalence and relationship with daily medication dose. METHODS: Focus groups and in-depth interviews were conducted to identify side effects that patients associated with their use of ICS. In an international multicentre cross-sectional survey, 395 inhaler users from community pharmacy (mean age 50, 53% female), divided into 4 daily dosage groups (beta2-agonist without ICS n=66, beclometasone dipropionate (BDP) equivalent ICS low dose 400 microg, n=109; mid dose 401-800 microg, n=151; and high dose>800 microg, n=69) reported how much they were affected by these side effects on a 7-point Likert scale. RESULTS: Focus groups and interviews revealed 57 side effects that were associated with ICS use. Cross-sectional survey results showed significant differences in side effect perception between the four dosage groups for 31 items (all P0.01) and a rising intensity with increasing ICS dose for total side effect score (P<0.001). For ICS users reporting the most bothersome side effects (scoring 3 on 0-6 scale) there was a rising prevalence as ICS dose increased for 34 items. A multivariate model confirmed that mid and high ICS dosages were statistically significantly associated with side effect perception after controlling for the other factors and covariates. CONCLUSIONS: Higher daily ICS doses were associated with a higher intensity and a higher prevalence of many patient perceived side effects, lending support to the call for dose titration in clinical practice. Results indicate the usefulness of patient self-report scales for understanding the burden of side effects of ICS in the community.

Percutaneous aortic valve replacement using a subclavian artery graft.

Over the past few years transcatheter heart valve implantation (THI) has become an alternative treatment for aortic valve replacement. The THI does not require a midline sternotomy or cardiopulmonary bypass and can be performed through a transfemoral or a transapical approach. In case of severe peripheral vascular disease the transapical route is usually chosen. However, when the use of a small anterolateral thoracotomy is not preferred due to comorbidities, the subclavian artery can be considered as a third alternative route. This case report describes an approach for THI through the subclavian artery, by using a Dacron graft.