Strikwerda, S.

Autosomal dominant inheritance of cardiac valves anomalies in two families: Extended spectrum of left-ventricular outflow tract obstruction (Article)

2009-02-01T00:00:00Z

Only a limited number of families with clear monogenic inheritance of nonsyndromic forms of congenital valve defects have been described. We describe two multiplex pedigrees with a similar nonsyndromic form of heart valve anomalies that segregate as an autosomal dominant condition. The first family is a three-generation pedigree with 10 family members affected with congenital defects of the cardiac valves, including six patients with aortic stenosis and/or aortic regurgitation. Pulmonary and/or tricuspid valve abnormalities were present in three patients, and ventricular septal defect (VSD) was present in two patients. The second family consists of 11 patients in three generations with aortic valve stenosis in seven patients, defects of the pulmonary valves in two patients, and atrial septal defect (ASD) in two patients. Incomplete penetrance was observed in both families. Although left-ventricular outflow tract obstruction was present in most family members, the co-occurrence with pulmonary valve abnormalities and septal defects in both families is uncommon. These families provide evidence that left-sided obstructive defects and thoracic aortic aneurysm may be accompanied by right-sided defects, and even septal defects. These families might be instrumental in identifying genes involved in cardiac valve morphogenesis and malformation.

Clinical events following excimer laser angioplasty or balloon angioplasty for complex coronary lesions: subanalysis of a randomised trial (Article)

1998-01-01T00:00:00Z

OBJECTIVES: To compare clinical outcome in patients with complex coronary lesions treated with either excimer laser coronary angioplasty (ELCA) or balloon angioplasty. PATIENTS AND DESIGN: 308 patients with stable angina and a coronary lesion of more than 10 mm in length were randomised to ELCA (151 patients, 158 lesions) or balloon angioplasty (157 patients, 167 lesions). The primary clinical end points were death, myocardial infarction, coronary bypass surgery, or repeated coronary angioplasty of the randomised segment during six months of follow up. Subanalysis was performed to identify a subgroup of patients with a beneficial clinical outcome following ELCA or balloon angioplasty. SETTING: Two university hospitals and one general hospital. RESULTS: There were no deaths. Myocardial infarction, coronary bypass surgery, and repeated angioplasty occurred in 4.6, 10.6, and 21.2%, respectively, of patients treated with ELCA compared with 5.7, 10.8, and 18.5%, respectively, of those treated with balloon angioplasty. ELCA did not yield a favourable clinical outcome in subgroups of patients with long (more than 20 mm) coronary lesions, calcified lesions, small diseased vessels (< or = 2.5 mm reference diameter), or total coronary occlusions. There was a worse clinical outcome in patients with tandem lesions treated with ELCA compared with balloon angioplasty (9/18 v 3/26 lesions; p = 0.01); while a trend towards an unfavourable clinical outcome was found in patients with vessels with a reference diameter of more than 2.5 mm (23/66 v 13/63 lesions, p = 0.07) and left circumflex coronary lesions (12/41 v 6/42 lesions, p = 0.08). CONCLUSIONS: The findings indicate a worse clinical outcome in patients with lesions of more than 10 mm treated with ELCA compared with balloon angioplasty who have tandem coronary lesions and in those with vessels with a reference diameter of more than 2.5 mm and left circumflex coronary lesions.

Differences in restenosis propensity of devices for transluminal coronary intervention. A quantitative angiographic comparison of balloon angioplasty, directional atherectomy, stent implantation and excimer laser angioplasty. CARPORT, MERCATOR, MARCATOR, PARK, and BENESTENT Trial Groups. (Article)

1995-01-01T00:00:00Z

With the increasing clinical application of new devices for percutaneous coronary revascularization, maximization of the acute angiographic result has become widely recognized as a key factor in maintained clinical and angiographic success. What is unclear, however, is whether the specific mode of action of different devices might exert an additional independent effect on late luminal renarrowing. The purpose of this study was to investigate such a difference in the degree of provocation of luminal renarrowing (or 'restenosis propensity') by different devices, among 3660 patients, who had 4342 lesions successfully treated by balloon angioplasty (n = 3797), directional coronary atherectomy (n = 200), Palmaz-Schatz stent implantation (n = 229) or excimer laser coronary angioplasty (n = 116) and who also underwent quantitative angiographic analysis pre- and post-intervention and at 6-month follow-up. To allow valid comparisons between the groups, because of significant differences in coronary vessel size (balloon angioplasty = 2.62 +/- 0.55 mm, directional coronary atherectomy = 3.28 +/- 0.62 mm, excimer laser coronary angioplasty = 2.51 +/- 0.47 mm, Palmaz-Schatz = 3.01 +/- 0.44 mm; P < 0.0001), the comparative measurements of interest selected were the 'relative loss' in luminal diameter (RLoss = loss/vessel size) to denote the restenosis process, and the 'relative lumen at follow-up' (RLfup = minimal luminal diameter at follow up/vessel size) to represent the angiographic outcome. For consistency, lesion severity pre-intervention was represented by the 'relative lumen pre' (RLpre = minimal luminal diameter pre/vessel size) and the luminal increase at intervention was measured as 'relative gain' (relative gain = gain/ vessel size). Differences in restenosis propensity between devices was evaluated by univariate and multivariate analysis. Multivariate models were constructed to determine relative loss and relative lumen at follow-up, taking account of relative lumen pre-intervention, lesion location, relative gain, vessel size and the device used. In addition, model-estimated relative loss and relative lumen at follow-up at given relative lumen pre-intervention relative gain and vessel size, were compared among the four groups. Significant differences were detected among the groups both with respect to these estimates, as well as in the degree of influence of progressively increasing relative gain, on the extent of renarrowing (relative loss) and angiographic outcome (relative lumen at follow-up), particularly at higher levels of luminal increase (relative gain). Specifically, lesions treated by balloon angioplasty or Palmaz-Schatz stent implantation (the predominantly 'dilating' interventions) were associated with more favourable angiographic profiles than directional atherectomy or excimer laser (the mainly 'debulking' interventions). Significant effects of lesion severity and location, as well as the well known influence of luminal increase on both luminal renarrowing and late angiographic outcome were also noted. These findings indicate that propensity to restenosis after apparently successful intervention is influenced not only by the degree of luminal enlargement achieved at intervention, but by the device used to achieve it. In view of the clinical implications of such findings, further evaluation in larger randomized patient populations is warranted.

Quantitative angiographic comparison of elastic recoil after coronary excimer laser-assisted balloon angioplasty and balloon angioplasty alone. (Article)

1995-01-01T00:00:00Z

Response of conductance and resistance coronary vessels to scalar concentrations of acetylcholine: Assessment with quantitative angiography and intracoronary Doppler echography in 29 patients with coronary artery disease (Article)

1994-01-01T00:00:00Z

Abnormal vasoreactivity of the large conductance arteries has been observed in the presence of impaired endothelial function. More recently, experimental and clinical reports have shown that in early coronary atherosclerosis the impairment of the endothelium-mediated vasodilatation also involves the resistance arteries. The aim of this study is the correlation of endothelium-dependent vasodilatation of conductance and resistance vessels in coronary arteries without significant stenoses. In 29 patients (aged 57 +/- 9 years, 24 men and 5 women) undergoing coronary angioplasty, a Doppler guide wire and a perfusion catheter were introduced into the proximal segment of an artery with less than 30% diameter stenosis. Selective infusions of papaverine (bolus of 7 mg), acetylcholine (continuous infusion of 0.036, 0.36, and 3.6 micrograms/ml at a flow rate of 2 ml/min), and isosorbide dinitrate (bolus of 3 mg) were sequentially performed. Heart rate, aortic blood pressure, and blood flow velocity were continuously measured. Mean cross-sectional areas of a proximal and a distal arterial segment were measured in baseline conditions, at the end of each infusion of acetylcholine, and at the peak effect of isosorbide dinitrate with quantitative angiography (CAAS System; Pie Medical Data, Maastricht, The Netherlands). Coronary blood flow was calculated from the time-averaged flow velocity and the cross-sectional area at the site of the Doppler sample volume. Coronary flow resistance was calculated as mean aortic pressure divided by coronary flow. All of the concentrations of acetylcholine induced a significant vasoconstriction of the studied artery. At the maximal concentration of acetylcholine all but three patients (90%) showed a reduction of cross-sectional area (-24% +/- 20% and -22% +/- 20% for the proximal and distal segments, respectively, p < 0.00001). Flow velocity showed a significant increase only with the two highest concentrations of acetylcholine. The maximal concentration induced a 105% +/- 138% increase from the baseline flow velocity (p < 0.001). The coronary flow changes after acetylcholine showed a large interpatient variability, with a mean increase from baseline after the highest dose of +43% +/- 85% (range, -60% +/- 239%), with the presence of a flow reduction in 10 patients (35%). No clinical or angiographic variables showed a significant correlation with the cross-sectional area, flow velocity, and flow changes after infusion of acetylcholine.(ABSTRACT TRUNCATED AT 400 WORDS)

Intracoronary pressure and flow velocity with sensor-tip guidewires: a new methodologic approach for assessment of coronary hemodynamics before and after coronary interventions (Article)

1993-01-01T00:00:00Z

The use of miniaturized pressure and velocity sensors mounted on angioplasty guidewires allows the simultaneous measurement of coronary blood flow velocity and transstenotic pressure gradient, 2 parameters that, combined, should perfectly characterize stenosis hemodynamics. The aim of this article is assessment of the changes in coronary blood flow velocity observed with a Doppler-tipped angioplasty guidewire in 35 patients undergoing balloon angioplasty. We also report our initial experience in 16 patients with the combined use of sensor-tip pressure and Doppler guidewires, and we discuss the application of new methodologic approaches for the study of the coronary circulation allowed by these techniques, such as the instantaneous assessment of the flow velocity/pressure and pressure gradient/flow velocity relations. Before and after angioplasty, flow velocity measurements were obtained distal to the stenosis, both in baseline conditions and after intracoronary injection of 8-12.5 mg of papaverine. The Doppler guidewire was left in place during the dilation procedure and the Doppler signal was continuously recorded during balloon inflation and after deflation to monitor the development of collateral flow, the restoration of flow after balloon deflation, the phase of postocclusive reactive hyperemia, and, incidently, the development of flow-limiting complications. Merits and pitfalls of several flow velocity parameters (average peak velocity, coronary flow velocity reserve, diastolic/systolic velocity ratio), as well as of parameters derived from the combination of pressure and velocity measurements (transstenotic pressure gradient/flow velocity relation and instantaneous diastolic hyperemic flow velocity/pressure relation) were evaluated in 35 patients with, and 37 without, significant coronary stenoses.(ABSTRACT TRUNCATED AT 250 WORDS)

Excimer laser coronary angioplasty in the Netherlands: preamble for a randomized study (Article)

1993-01-01T00:00:00Z

The immediate outcome of ELCA by XeCl excimer laser radiation is described in 53 patients who were selected to undergo ELCA from December 1990 to September 1991 in two centers that are currently performing ELCA in the Netherlands. Immediate success rates on the basis of visual assessment of the angiogram were as follows. Laser success (> 20% reduction of diameter stenosis after ELCA alone) was observed in 77% of patients, procedural success (< 50% residual stenosis after ELCA with or without adjunctive balloon dilatation [PTCA]) in 91%, and clinical success (procedural success without clinical complications) in 83% of patients. Quantitative coronary angiography by automated contour detection was performed in 31 patients who underwent ELCA in the Thoraxcenter. The minimal luminal diameter (mean +/- SD) of the treated coronary segments increased from 0.77 +/- 0.41 mm to 1.24 +/- 0.25 mm after ELCA and further to 1.67 +/- 0.29 mm after adjunctive PTCA in 25 patients. The present experience is put in perspective of results initially reported by other centers and compared with data from multicenter registries of ELCA. Finally, a short description is given of the design of a prospective, randomized trial of ELCA versus conventional PTCA (AMRO trial).

Restenosis after directional coronary atherectomy and balloon angioplasty: comparative analysis based on matched lesions (Article)

1993-01-01T00:00:00Z

OBJECTIVES. Late lumen narrowing after directional coronary atherectomy was assessed by quantitative coronary angiography and compared with that after balloon angioplasty. BACKGROUND. Directional coronary atherectomy has been introduced as an alternative technique for balloon angioplasty and may reduce the incidence of restenosis. METHODS. A prospectively collected consecutive series of 87 native coronary artery lesions successfully treated with atherectomy were matched with 87 coronary artery lesions selected from a consecutive series of lesions that had been successfully dilated by balloon angioplasty. Late angiographic analysis was performed in 158 lesions. The net gain index represents the ultimate gain in minimal lumen diameter at follow-up study, normalized for the vessel size. This index is the result of the relative gain attained during the procedure (the ratio of the change in minimal lumen diameter and reference diameter) and the relative loss observed during the follow-up period (the ratio of the change in minimal lumen diameter during the follow-up period and the reference diameter). RESULTS. Matching for clinical and angiographic variables resulted in two comparable groups with similar baseline stenosis characteristics. Atherectomy resulted in a more pronounced increase in minimal lumen diameter than did balloon angioplasty (mean +/- SD 1.17 +/- 0.29 to 2.44 +/- 0.42 mm vs. 1.21 +/- 0.38 to 2.00 +/- 0.36 mm, p < 0.001). However, this favorable immediate result was subsequently lost during late angiographic follow-up, so that the minimal lumen diameter at follow-up and the net gain index did not differ significantly between the two groups (1.76 +/- 0.62 vs. 1.77 +/- 0.59 mm, p = 0.93, and 0.18 +/- 0.19 vs. 0.17 +/- 0.17, p = 0.70). Consequently, the relative gain and relative loss were higher in the atherectomy group. For both techniques, the relative gain was linearly related to the relative loss but the slope of the regression line was steeper for atherectomy, suggesting that the relative loss in the atherectomy group is proportionally even larger for a given relative gain compared with that in the angioplasty group. CONCLUSIONS. In matched groups of patients, atherectomy induces a greater initial gain in minimal lumen diameter than does balloon angioplasty. However, the vascular wall injury induced by the device is of a different nature (debulking vs. dilating) that leads to more relative loss over the follow-up period in the atherectomy group.

Percutaneous directional atherectomy for discrete coronary lesions in cardiac transplant patients (Article)

1992-01-01T00:00:00Z