David Garcia Dorado

Relationship between fibrillin-1 genotype and severity of cardiovascular involvement in Marfan syndrome

Background The effect of FBN1 mutation type on the severity of cardiovascular manifestations in patients with Marfan syndrome (MFS) has been reported with disparity results. Objectives This study aims to determine the impact of the FBN1 mutation type on aortic diameters, aortic dilation rates and on cardiovascular events (ie, aortic dissection and cardiovascular mortality). Methods MFS patients with a pathogenic FBN1 mutation followed at two specialised units were included. FBN1 mutations were classified as being dominant negative (DN; incorporation of non-mutated and mutated fibrillin-1 in the extracellular matrix) or having haploinsufficiency (HI; only incorporation of non-mutated fibrillin-1, thus a decreased amount of fibrillin-1 protein). Aortic diameters and the aortic dilation rate at the level of the aortic root, ascending aorta, arch, descending thoracic aorta and abdominal aorta by echocardiography and clinical endpoints comprising dissection and death were compared between HI and DN patients. Results Two hundred and ninety patients with MFS were included: 113 (39%) with an HI-FBN1 mutation and 177 (61%) with a DN-FBN1. At baseline, patients with HI-FBN1 had a larger aortic root diameter than patients with DN-FBN1 (HI: 39.3±7.2 mm vs DN: 37.3±6.8 mm, p=0.022), with no differences in age or body surface area. After a mean follow-up of 4.9±2.0 years, aortic root and ascending dilation rates were increased in patients with HI-FBN1 (HI: 0.57±0.8 vs DN: 0.28±0.5 mm/year, p=0.004 and HI: 0.59±0.9 vs DN: 0.30±0.7 mm/year, p=0.032, respectively). Furthermore, patients with HI-FBN1 tended to be at increased risk for the combined endpoint of dissection and death compared with patients with DN-FBN1 (HR: 3.3, 95% CI 1.0 to 11.4, p=0.060). Conclusions Patients with an HI mutation had a more severely affected aortic phenotype, with larger aortic root diameters and a more rapid dilation rate, and tended to have an increased risk of death and dissections compared with patients with a DN mutation.

Predictors of Moderate-to-Severe Pericardial Effusion, Cardiac Tamponade, and Electromechanical Dissociation in Patients With ST-Elevation Myocardial Infarction

Occurrence of moderate-to-severe pericardial effusion (PE; ≥10 mm), cardiac tamponade (CT), and sudden electromechanical dissociation (EMD) was investigated in 4,361 patients with ST-elevation myocardial infarction from 1993 to 2011 in 3 different periods: 1993 to 2000 (n: 1,488); 2001 to 2008 (n: 1,844); and 2009 to 2011 (n: 1,014). Their predictors, including the use of no reperfusion therapy (n: 1,186), thrombolysis (n: 1,607), or primary percutaneous coronary intervention (PPCI, n: 1,562), were also evaluated. Incidence of PE (8.7%, 6.8%, and 5.0%), CT (5.0%, 2.9%, and 1.9%), and EMD (3.7%, 1.7%, and 1.0%), declined over the 3 periods as did mortality (12.0% 8.2%, and 5.9%) with different rates of thrombolytic therapy (52%, 37%, and 14%) and PPCI (7%, 38%, and 76%; all p<0.001). In patients treated without reperfusion therapy, thrombolysis, and PPCI, incidence of PE (12.0%, 5.7%, and 4.3%), CT (6.0%, 3.0%, and 2.2%), and EMD (4.1%, 2.2%, and 0.8%) was different as was mortality (14.4%, 8.3%, and 5.9%; all p<0.001). Independent predictors of PE were lateral infarction (odds ratio [OR] 4.09, 95% confidence interval [CI] 2.57 to 6.49), increasing age (OR 1.05, 95% CI 1.04 to 1.07), number of electrocardiographic leads involved (OR 1.34, 95% CI 1.23 to 1.45), and admission delay (OR 1.01, 95% CI 1.01 to 1.02). Increasing ejection fraction (OR 0.97, 95% CI 0.96 to 0.98), thrombolysis (OR 0.53, 95% CI 0.37 to 0.75), and PPCI (OR 0.35, 95% CI 0.25 to 0.50), however, were protectors (all p<0.001). Lateral infarction, age, number of leads involved, ejection fraction, thrombolytic therapy, and PPCI were also predictors/protectors of CT and EMD. In conclusion, PE, CT, and EMD rates in patients with ST-elevation myocardial infarction have objectively fallen in the last 2 decades, and their predictors are lateral site, increasing age, number of leads involved, and lack of reperfusion therapy. Late hospital admission is also a relevant predictor of PE.

Hospital and 4-Year Mortality Predictors in Patients With Acute Pulmonary Edema With and Without Coronary Artery Disease.

Background Long‐term prognosis of acute pulmonary edema (APE) remains ill defined. Methods and Results We evaluated demographic, echocardiographic, and angiographic data of 806 consecutive patients with APE with (CAD) and without coronary artery disease (non‐CAD) admitted from 2000 to 2010. Differences between hospital and long‐term mortality and its predictors were also assessed. CAD patients (n=638) were older and had higher incidence of diabetes and peripheral vascular disease than non‐CAD (n=168), and lower ejection fraction. Hospital mortality was similar in both groups (26.5% vs 31.5%; P=0.169) but APE recurrence was higher in CAD patients (17.3% vs 6.5%; P<0.001). Age, admission systolic blood pressure, recurrence of APE, and need for inotropics or endotracheal intubation were the main independent predictors of hospital mortality. In contrast, overall mortality (70.0% vs 57.1%; P=0.002) and readmission for nonfatal heart failure after a 45‐month follow‐up (10–140; 17.3% vs 7.6%; P=0.009) were higher in CAD than in non‐CAD patients. Age, peripheral vascular disease, and peak creatine kinase MB during index hospitalization, but not ejection fraction, were the main independent predictors of overall mortality, whereas coronary revascularization or valvular surgery were protective. These interventions were mostly performed during hospitalization index (294 of 307; 96%) and not intervened patients showed a higher risk profile. Conclusions Long‐term mortality in APE is high and higher in CAD than in non‐CAD patients. Considering the different in‐hospital and long‐term mortality predictors herein described, which do not necessarily involve systolic function, it is conceivable that a more aggressive interventional program might improve survival in high‐risk patients.

Acute cardiac syndromes without significant coronary stenosis: differential features between myocardial infarction and apical-ballooning syndrome.

ObjectiveAmong patients with acute cardiac syndromes without coronary stenosis, the clinical, electrocardiographic, echocardiographic, and angiographic features of those with a first acute myocardial infarction (AMI) were compared with those with apical-ballooning syndrome (ABS). MethodsData of consecutive patients admitted with a first AMI (n=30) or ABS (n=45) were reviewed. ResultsPatients with ABS were older (72 vs. 56 years; P=0.001) and presented a higher frequency of female sex (91 vs. 43%; P=0.001), triggering emotional or physical stress (47 vs. 17%; P=0.003) and a lower rate of tobacco smoking (27 vs. 50%; P=0.051) than those with the first AMI. They also presented a greater number of leads (5.5 vs. 3.6; P=0.01) and more anterior or anterior+inferior involvement (96 vs. 40%; P<0.001), more depressed ejection fraction (45 vs. 57%; P=0.001), more proportion of akinesia or diskinesia (89 vs. 27%; P=0.001) that extended beyond the boundaries of a single-vessel territory, and a greater rate of left ventricular outflow obstruction (29 vs. 0%; P=0.001) and heart failure (38 vs. 10%; P=0.015). Frequency of nonsignificant coronary stenosis or smooth vessels, however, was similar in both groups. ConclusionPatients with ABS were older and more frequently were women than those with first AMI without significant coronary stenosis and had larger hypocontractile areas. The preponderance of tobacco smoking, pain without triggers, and hypocontractility limited to one-vessel territory in the latter, however, may suggest a transient thrombotic/vasospastic event as their underlying mechanism as opposed to patients with ABS.

Left ventricular dynamic gradient and pericardial effusion. A life threatening combination in patients with apical ballooning syndrome

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¿Son iguales todas las prótesis? ¿Prótesis diferentes para indicaciones diferentes?

Transcatheter aortic valve implantation has become an accepted treatment option for patients with severe symptomatic aortic stenosis who are at a high surgical risk. In particular, randomized trials have shown that the technique is associated with lower mortality in inoperable patients than medical treatment and is noninferior to conventional surgery in patients at a high surgical risk. From its inception, transcatheter aortic valve implantation has shown great potential and, consequently, considerable effort has been devoted to designing new devices that can provide better outcomes and that can expand indications to ever more complex anatomies and to patients with multiple comorbidities. This review describes developments in the design of novel transcatheter prostheses and discusses recent findings with the application of these new-generation prostheses.