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Maarten Groenink

Publications and source records attributed to Maarten Groenink.

11 recordsLinked to original sources

Obstetric complications in Marfan syndrome.

OBJECTIVE: To investigate the obstetric maternal and neonatal outcome of pregnancy in women with Marfan syndrome. METHODS: Retrospective observational multi-center study based on congenital heart disease registry. RESULTS: Sixty-three of the 122 enrolled women with Marfan syndrome had been pregnant 142 times (including 111 pregnancies>20 weeks, 28 (20%) miscarriages and 3 elective abortions). In 40% of all completed pregnancies an obstetric and/ or neonatal complication occurred. The most important complications were an increased percentage of premature deliveries (n=17, 15%) mainly due to preterm premature rupture of membranes and cervical incompetence and a markedly increased combined fetal and neonatal mortality of 7.1%. An obstetric and/or neonatal complication occurred in a similar proportion of pregnancies in women with a diagnosis of Marfan syndrome before pregnancy versus women with a diagnosis of Marfan syndrome after pregnancy (39% vs. 41%, p=0.85, respectively). CONCLUSION: In addition to cardiovascular complications, pregnancy in women with Marfan syndrome is associated with a high rate of premature deliveries, preterm premature rupture of membranes and increased mortality in the offspring.

Abortion, Spontaneous↗

Magnetic resonance imaging analysis of right ventricular pressure-volume loops: in vivo validation and clinical application in patients with pulmonary hypertension.

BACKGROUND: The aims of this study were to validate MRI-derived right ventricular (RV) pressure-volume loops for assessment of RV myocardial contractility and then to apply this technique in patients with chronic RV pressure overload for assessment of myocardial contractility, ventricular pump function, and VA coupling. METHODS AND RESULTS: Flow-directed catheters were guided under MR fluoroscopy (1.5 T) into the RV for invasive pressure measurements. Simultaneously, ventricular volumes and myocardial mass were assessed from cine MRI. From sampled data, RV pressure-volume loops were constructed, and maximal ventricular elastance indexed to myocardial mass (E(max_i)) was derived by use of a single-beat estimation method. This MRI method was first validated in vivo (6 swine), with conductance techniques used as reference. Bland-Altman test showed good agreement between methods (E(max_i)=5.1+/-0.5 versus 5.8+/-0.7 mm Hg x mL(-1) x 100 g(-1), respectively; P=0.08). Subsequently, the MRI method was applied in 12 subjects: 6 control subjects and 6 patients with chronic RV pressure overload from pulmonary hypertension. In these patients, indexes of RV pump function (cardiac index), E(max_i), and VA coupling (E(max)/E(a)) were assessed. In patients with pulmonary hypertension, RV pump function was decreased (cardiac index, 2.2+/-0.5 versus 2.9+/-0.4 L x min(-1) x m(-2); P<0.01), myocardial contractility was enhanced (E(max_I), 9.2+/-1.1 versus 5.0+/-0.9 mm Hg x mL(-1) x 100 g(-1); P<0.01), and VA coupling was inefficient (E(max)/E(a), 1.1+/-0.3 versus 1.9+/-0.4; P<0.01) compared with control subjects. CONCLUSIONS: RV myocardial contractility can be determined from MRI-derived pressure-volume loops. Chronic RV pressure overload was associated with reduced RV pump function despite enhanced RV myocardial contractility. The proposed MRI approach is a promising tool to assess RV contractility in the clinical setting.

Adolescent↗

Aortic stiffness and diameter predict progressive aortic dilatation in patients with Marfan syndrome.

AIM: Patients with Marfan syndrome may develop dissection due to progressive dilatation in the entire aorta, which is not always predictable by mere anatomic assessment of the aortic diameter, especially of the descending aorta. The aim of this study was to identify the predictive value of aortic stiffness on the occurrence of dissection and progressive aortic dilatation. METHODS AND RESULTS: In 78 non-operated patients with Marfan syndrome (mean age 31+/-8 years, mean aortic root diameter 43+/-6 mm, range 31-55 mm) aortic stiffness and diameters were assessed by magnetic resonance imaging (MRI) at multiple levels. After a median follow-up of 71 months (25-75%: 68-72 months) a second MRI was performed and the incidence of aortic dissection and progressive aortic dilatation, defined as mean aortic diameter increase >1 mm/year was determined. During follow-up, 4 (5%) of 78 patients developed an aortic dissection (1 type A, 2 type B, and 1 infra-renal dissection). Twenty (26%) of the 78 patients had progressive aortic root dilatation. There were 5 (6%) patients with progressive descending thoracic aortic dilatation and 6 (7%) with progressive abdominal aortic dilatation. Multivariate logistic regression analysis revealed that local distensibility was an independent predictor of progressive thoracic descending aortic dilatation (OR=4.14, 95% CI, 1.02-16.7). For progressive aortic root and abdominal aortic dilatation local initial diameter appeared to be the major predictor (OR=1.37, 95% CI, 1.16-1.62; OR=1.36, 95% CI, 1.09-1.69, respectively). CONCLUSION: In patients with Marfan syndrome both aortic diameter and aortic distensibility are independent predictors of progressive aortic dilatation. For optimal risk assessment and monitoring of patients with Marfan syndrome, both aortic stiffness and diameter should be assessed at least annually.

Adolescent↗

Decreased plasma neurohormones and improved cardiac performance after surgical treatment of chronic pulmonary embolism.

The findings of this case report suggest that quantitative assessment of plasma neurohormones and magnetic resonance imaging functional parameters in patients with right ventricular pressure overload due to chronic pulmonary embolism might be used as indicators for right ventricular function before and after intervention. Monitoring of changes in these parameters may provide quantitative follow-up of right ventricular function in these patients.

Biomarkers↗

Right ventricular function in congenital cardiac disease: noninvasive quantitative parameters for clinical follow-up.

Right ventricular function is of great importance in patients with both acute and chronic ventricular overload. The early detection of right ventricular dysfunction may have an impact on therapeutic decision making, helping to prevent or further delay functional deterioration of the right ventricle. In patients with right ventricular overload due to congenital cardiac diseases, dobutamine stress testing combined with magnetic resonance imaging, electrocardiographic changes, and monitoring of concentrations of plasma brain natriuretic peptide are very suitable parameters for the early detection of ventricular dysfunction, and should therefore be used in the follow-up of these patients. It is apparent that no single measurement of anatomy or function can ever adequately describe the form or performance of the right ventricle. Rather, we should be looking more towards an integrated approach of different parameters for right ventricular function. The quantitative parameters described in this study can serve this purpose. The strong correlation found between these non-invasive and independent parameters encourages their clinical implementation.

Child↗

Usefulness of magnetic resonance imaging dobutamine stress in asymptomatic and minimally symptomatic patients with decreased cardiac reserve from congenital heart disease (complete and corrected transposition of the great arteries and subpulmonic obstruction).

We explored the effect of dobutamine stress and its possible clinical implications in different groups of asymptomatic patients with chronic right ventricular (RV) pressure overload due to congenital heart disease. Forty-seven asymptomatic and minimally symptomatic patients with chronic RV pressure overload were studied: 24 patients with systemic right ventricles (16 surgically corrected transposition of the great arteries (TGA) (Mustard or Senning), 8 congenitally corrected TGA), 23 patients with chronic pressure overloaded subpulmonic right ventricles, and 11 age- and sex-matched healthy volunteers. Magnetic resonance imaging (MRI) was performed both at baseline and during dobutamine stress to determine RV volumes and ejection fraction. At baseline, RV ejection fraction in patients with surgically corrected TGA was significantly lower than in controls (58 +/- 10% vs 70 +/- 8%, p = 0.02). During dobutamine stress, RV ejection fraction increased significantly in controls and patient groups except for patients with pressure overloaded subpulmonic right ventricles. RV stroke volume increased in controls (21 +/- 21%, p = 0.008); RV stroke volume remained unchanged in patients with congenitally corrected TGA and surgically corrected TGA (2 +/- 17%, p = NS; -8 +/- 29%, p = NS). A significant RV stroke volume decrease was observed in patients with subpulmonic right ventricles (-15 +/- 16%, p = 0.0002). The changes in RV stroke volume were accompanied by a significant decrease in RV end-diastolic volume (-13 +/- 14%, p = 0.001) in patients with subpulmonic right ventricles and in patients with surgically corrected TGA (-23 +/- 16%, p = 0.0001). In controls and in patients with congenitally corrected TGA there was no change in RV end-diastolic volume (3 +/- 15%, p = NS; -5 +/- 11%, p = NS). There is a clear heterogeneity in response to MRI dobutamine stress between different groups of patients with chronic RV pressure overload. Our data suggest impaired filling in surgically corrected TGA and decreased contractility in patients with chronic pressure overloaded subpulmonic right ventricles. Dobutamine stress MRI may facilitate follow-up of RV (dys)function in patients with chronic RV pressure overload due to congenital heart disease.

Adult↗

Current insights in diagnosis and management of the cardiovascular complications of Marfan's syndrome.

Marfan's syndrome is an inherited disorder of connective tissue, caused by mutations in the fibrillin-1 gene located on chromosome 15. Diagnosis is still based on a combination of major and minor clinical features. Prognosis is mainly determined by the cardiovascular complications. Advances in surgical and medical treatment for these complications have dramatically improved the prognosis of the syndrome.

Cardiovascular Diseases↗