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B J Mulder

Publications and source records attributed to B J Mulder.

At least 19 recordsLinked to original sources
PubMed2004

[Hypertension in young adults: indication for possible aortic coarctation].

Three adult patients, a woman aged 37 and two men aged 22 and 23 years respectively, were admitted due to an unexplained hypertension. After a significant delay, the diagnosis of aortic coarctation was established for these patients. In two of them the abnormality was operatively corrected and the blood pressure subsequently normalised; the third patient is on the waiting list for the operation. In all three patients an earlier diagnosis could have been established, had accurate blood-pressure measurements of both arms and at least one leg been performed. Late detection and treatment of aortic coarctation have a profound detrimental effect on survival. Therefore it is extremely important to accurately measure the blood pressure in the limbs of young patients with hypertension.

Adult↗
PubMed2001

Natural history of cardiovascular manifestations in Marfan syndrome.

AIMS: To investigate the natural history of mitral valve and aortic abnormalities in patients with Marfan syndrome during childhood and adolescence. METHODS: Fifty two patients with Marfan syndrome were followed for a mean of 7.9 years. Occurrence of adverse cardiovascular outcomes was measured clinically and by ultrasound examination. RESULTS: Mitral valve prolapse (MVP) was diagnosed in 46 patients at a mean age of 9.7 years, more than 80% of whom presented as "silent MVP". Mitral regurgitation (MR) occurred in 25 patients, aortic dilatation in 43, and aortic regurgitation (AR) in 13. Both MVP and aortic dilatation developed at a constant rate during the age period 5-20 years. In 23 patients MVP was diagnosed before aortic dilatation, in 18 the reverse occurred, and in 11 patients the two abnormalities were diagnosed simultaneously. During follow up, 21 patients showed progression of mitral valve dysfunction; progression of aortic abnormalities occurred in 13. Aortic surgery was performed in 10; two died of subsequent complications. Mitral valve surgery was performed in six. In sporadic female Marfan patients the age at initial diagnosis of MVP, MR, aortic dilatation, and AR was lowest, the grade of MR and AR most severe, the time lapse between the occurrence of MVP and subsequent MR as well as between dilatation and subsequent AR shortest, and the risk for cardiovascular associated morbidity and mortality highest. CONCLUSIONS: During childhood and adolescence in Marfan syndrome, mitral valve dysfunction as well as aortic abnormalities develop and progress gradually, often without symptoms, but may cause considerable morbidity and mortality by the end of the second decade, especially in female sporadic patients.

Adolescent↗
PubMed2001

Increased brain and atrial natriuretic peptides in patients with chronic right ventricular pressure overload: correlation between plasma neurohormones and right ventricular dysfunction.

OBJECTIVE: To evaluate the role of plasma neurohormones in the diagnosis of asymptomatic or minimally symptomatic right ventricular dysfunction. SETTING: Tertiary cardiovascular referral centre. METHODS: Plasma brain natriuretic peptide (BNP) and atrial natriuretic peptide (ANP) concentrations were measured in 21 asymptomatic or minimally symptomatic patients with chronic right ventricular pressure overload caused by congenital heart disease, and in seven healthy volunteers. Right ventricular ejection fraction was determined using magnetic resonance imaging. RESULTS: Right ventricular ejection fraction in the volunteers was higher than in the patients (69.0 (8.2)% v 58.0(12.0)%, respectively; p < 0.006). Left ventricular ejection fraction was 72.3(7.8)% in volunteers and 68.1(11.0)% in patients (NS). There was a significant difference between patients and volunteers in the plasma concentrations of BNP (5.3 (3.5) v 2.3 (1.7) pmol/l, respectively; p < 0.009) and ANP (7.3 (4.5) v 3.6 (1.4) pmol/l; p < 0.05). In both patients and volunteers, mean plasma ANP was higher than mean plasma BNP. Right ventricular ejection fraction was inversely correlated with BNP and ANP (respectively, r = 0.65; p < 0.0002 and r = 0.61; p < 0.002). There was no correlation between left ventricular ejection fraction and BNP (r = 0.2; NS) or ANP (r = 0.52; NS). Similarly, no correlation was shown between the level of right ventricular systolic pressure and either plasma BNP (r = 0.20) or plasma ANP (r = 0.07). CONCLUSIONS: There was a significant inverse correlation between right ventricular ejection fraction and the plasma neurohormones BNP and ANP in asymptomatic or minimally symptomatic patients with right ventricular pressure overload and congenital heart disease. Monitoring changes in BNP and ANP may provide quantitative follow up of right ventricular dysfunction in these patients.

Adult↗
PubMed2001

Biophysical properties of the normal-sized aorta in patients with Marfan syndrome: evaluation with MR flow mapping.

PURPOSE: To investigate the feasibility of magnetic resonance (MR) flow mapping in the assessment of aortic biophysical properties in patients with Marfan syndrome and to detect differences in biophysical properties in the normal-sized aorta distal to the aortic root between these patients and matched control subjects. MATERIALS AND METHODS: Seventy-eight patients with Marfan syndrome with aortic root dilatation and 23 matched control subjects underwent MR flow mapping in four locations in the normal-sized aorta (1, ascending aorta; 2, thoracic descending aorta; 3, descending aorta at the level of the diaphragm; and 4, abdominal descending aorta). Distensibility at each location and flow wave velocity between locations were calculated. RESULTS: Compared with the control subjects, patients with Marfan syndrome had decreased aortic distensibility at three of the four locations (levels 1, 2, and 4; P <.05) and increased flow wave velocity between all locations (P <.05) in the aorta. In patients with Marfan syndrome, flow wave velocity was also significantly increased along the entire aortic tract beyond the aortic root (from level 1 to level 4). CONCLUSION: MR imaging reveals abnormal biophysical properties of the normal-sized aorta in patients with Marfan syndrome. Monitoring of these properties is relevant for evaluating disease progression and treatment options.

Adolescent↗
PubMed2001

Quantitative assessment of dural ectasia as a marker for Marfan syndrome.

PURPOSE: To establish normal values for lumbosacral dural sac dimensions with magnetic resonance (MR) imaging and to use these values to assess the sensitivity and specificity of dural ectasia as a marker for Marfan syndrome. MATERIALS AND METHODS: MR imaging was performed to measure dural sac diameter (DSD) from L1 through S1 in 44 adult patients with Marfan syndrome and in 44 matched control subjects. DSD values were corrected for vertebral body size, yielding dural sac ratios (DSRs). The control subjects served to establish the upper limit of normal DSR values at the L1 through S1 levels. RESULTS: Cutoff values for normal DSRs for L1 through S1 were 0.64, 0.55, 0.47, 0.48, 0.48, and 0.57. Significant DSR differences were shown at all levels between patients with Marfan syndrome and control subjects (P <.001 at all levels). At L1 through S1, the sensitivity of dural ectasia as a marker for Marfan syndrome was 45%-77%, and the specificity was 95% or greater. By combining levels L3 and S1, dural ectasia as a marker for Marfan syndrome yielded a sensitivity of 95% (42 of 44 patients) and a specificity of 98% (43 of 44). The presence of dural ectasia excelled, compared with the presence of other Marfan syndrome manifestations in the patient population. CONCLUSION: Abnormal DSR values at L3 or S1 can be used to identify Marfan syndrome with 95% sensitivity and 98% specificity.

Adolescent↗
PubMed2001

Increased brain natriuretic peptide as a marker for right ventricular dysfunction in acute pulmonary embolism.

Right ventricular (RV) function is of major prognostic significance in patients with acute pulmonary embolism (PE). The aim of the present study was to evaluate the role of neurohormone plasma brain natriuretic peptide (BNP) in assessing RV function in patients with acute PE. BNP levels were measured in 16 consecutive patients with acute PE as diagnosed by high probability lung scintigraphy or pulmonary angiography. Twelve healthy age-matched volunteers served as controls. All 16 patients underwent standard echocardiography and blood tests during the first hour of presentation. In the patient group, survival was studied for a period of 30 days. Plasma BNP levels in patients with acute PE were higher than in controls (7.2 [95% CI 0.4 to 144.6] versus 1.4[95% CI 0.4 to 4.6] pmol/L, p = 0.0008). Plasma BNP was significantly higher in 5 patients with RV dysfunction compared to II patients with normal RV function (40.2 [95% CI 7.5 to 214.9] versus 3.3 [95% CI 0.4 to 24.9] pmol/L, p = 0.0003). RV systolic pressure was not significantly correlated with BNP (r = 0.42, p = ns). In conclusion, plasma BNP neurohormone levels might be of clinical importance as a supplementary tool for assessment of RV function in patients with acute PE.

Acute Disease↗
PubMed2000

Aortic root asymmetry in marfan patients; evaluation by magnetic resonance imaging and comparison with standard echocardiography.

BACKGROUND: Patients with Marfan syndrome may develop aortic root dissection despite only mild aortic root dilation as shown by standard echocardiography, which may be due to aortic root asymmetry. Purpose of the present study was to investigate aortic root asymmetry by magnetic resonance (MR) imaging in patients with Marfan syndrome and to compare these measurements with standardly performed echocardiography. METHODS: Eighty-seven Marfan patients (mean age 31 +/- 8 years) underwent MR imaging. From this population, 15 patients (mean age 29 +/- 3 years) were selected in whom both echocardiography and MR imaging had been performed within 3 months. With echocardiography, the aortic root was measured according to the recommendations of the American Society of Echocardiography. With MR imaging, a short axis view of the aortic root was obtained to measure distances between the noncoronary, right coronary and left coronary cusps and the aortic root area. Correlations between aortic root area and diameters were assessed, and 95% confidence intervals (95% CIs) calculated. RESULTS: No difference in the standardly measured noncoronary to right coronary cusp diameter between MR imaging and echocardiography was shown (42 +/- 6 mm). Largest aortic root diameter on the MR images was the right to left coronary cusp diameter (46 +/- 7 mm, p < 0.02). For a given noncoronary to right coronary cusp diameter, 95% confidence intervals revealed a variation of -20 to +20% in the aortic root area. CONCLUSIONS: The majority of Marfan patients show asymmetric dilation of the aortic root by MR imaging. This phenomenon may go unnoticed when standard echocardiography is performed. The asymmetry of the aortic root might be of clinical importance in unexpected aortic root dissection.

Adult↗
PubMed2000

Dobutamine-induced increase of right ventricular contractility without increased stroke volume in adolescent patients with transposition of the great arteries: evaluation with magnetic resonance imaging.

OBJECTIVE: Prognosis in patients with surgically corrected (Senning or Mustard) transposition of the great arteries (TGA) depends mainly on right ventricular (RV) function and RV functional reserve. We examined the role of dobutamine stress in the early detection of RV dysfunction in asymptomatic or slightly symptomatic patients with TGA using magnetic resonance imaging (MRI). DESIGN AND PATIENTS: Twelve asymptomatic or slightly symptomatic patients with chronic RV pressure overload, surgically corrected (Mustard or Senning) TGA (age 22.8 (+/- 3.4) years; New York Heart Association (NYHA) class I/II) and nine age matched healthy volunteers (age 27.3 (+/- 4.4) years) were included. MRI was applied both at baseline and during dobutamine stress (start dose 5 microg/kg/min to maximum dose 15 microg/kg/min) to determine RV and left ventricular (LV) stroke volumes (SV) and ejection fraction (EF). RESULTS: At baseline only RVEF was significantly higher in controls than in patients (71 (+/- 9) vs. 57 (+/- 10)%, p < 0.001), other RV parameters were not significantly different between the two examined groups: RVSV (86 (+/- 21) vs. 72 (+/- 27) ml, p = ns), RV end-diastolic volume (EDV) (123 (+/- 37) vs. 123 (+/- 33) ml, p = ns), and heart rate (61 (+/- 10) vs. 69 (+/- 14) bpm, p = ns), respectively. During dobutamine stress RVEF increased significantly both in controls and patients (20 (+/- 16) vs. 17 (+/- 18)%, p < 0.01 and p < 0.02 vs. rest, respectively), but stress RVEF was significantly higher in controls than in patients (85 (+/- 3) vs. 66 (+/- 7)%, p < 0.0001). RVSV increased significantly in controls (22 (+/- 19)%, p < 0.02), and there was no significant increase in RVSV in patients (-10 (+/- 28)%, p = ns). The controls showed no change in RVEDV (2 (+/- 17)%, p = ns), but in patients a significant decrease in RVEDV (-24 (+/- 15)%, p < 0.001) was observed. Maximal heart rate was significantly higher in patients than in controls (122 (+/- 20) vs. 101 (+/- 14) bpm, p < 0.02). CONCLUSION: In asymptomatic or slightly symptomatic patients with surgically corrected TGA dobutamine had a positive inotropic effect on RV, but the increased contractility was not accompanied by an appropriate increase in SV. Our data suggest inadequate RV filling in this category of patients, possibly due to rigid atrial baffles and compromised atrial function or decreased compliance due to RV hypertrophy.

Adolescent↗
PubMed1999

Worse clinical outcome but similar graft patency in women versus men one year after coronary artery bypass graft surgery owing to an excess of exposed risk factors in women. CABADAS. Research Group of the Interuniversity Cardiology Institute of The Netherlands. Coronary Artery Bypass graft occlusion by Aspirin, Dipyridamole and Acenocoumarol/phenoprocoumon Study.

OBJECTIVES: This retrospective study sought to assess differences in graft patency and clinical outcome between women and men after coronary artery bypass graft surgery (CABG). BACKGROUND: A less favorable clinical outcome has been reported in women as compared with men. Its relation to graft patency has not been studied. METHODS: We analyzed one-year follow-up data of 912 patients (120 women) who entered a randomized clinical drug trial. All patients received vein grafts; in 494 patients (56 women) internal mammary artery (IMA) grafts were also used. Graft patency was assessed by coronary angiography at one year. Primary clinical end points were myocardial infarction, revascularization procedures and death; secondary clinical end points included recurrent angina, heart failure and arrhythmias. RESULTS: Occlusion rates of vein grafts were 16.7% in women and 12.4% in men (odds ratio [OR] 1.62, 95% confidence interval [CI] 0.88 to 3.00, p = 0.12); occlusion rates of IMA grafts were 3.4% and 5.7% in women and men, respectively (OR 0.56, 95% CI 0.08 to 3.96, p = 0.56). Primary clinical end points were observed in 16.7% of women and 9.2% of men (OR 1.97, 95% CI 1.10 to 3.34, p = 0.022), and any clinical end point in 41.7% of women and 25.8% of men (OR 2.06, 95% CI 1.39 to 3.04, p = 0.0004). Myocardial infarction (15% vs. 7.6%, OR 2.15, 95% CI 1.24 to 3.75, p = 0.013) and recurrent angina (26.7% vs. 15.4%, OR 2.00, 95% CI 1.28 to 3.11, p = 0.004) occurred most frequently. Multivariate regression analysis did not identify gender as an independent risk factor for graft occlusion or the clinical end points. Graft occlusion was an independent predictor of the composite primary clinical end point (OR 2.75, 95% CI 1.59 to 4.75, p = 0.0003) and each of the secondary clinical end points. The observed differences were due to an imbalance of risk factors at baseline and to surgical and graft characteristics. CONCLUSIONS: One-year occlusion rates of vein and IMA grafts were comparable in women and men. Clinical outcome was related to graft patency and was less favorable in women owing to their uneven distribution of risk factors among both groups.

Aged↗
PubMed1999

The influence of aging and aortic stiffness on permanent dilation and breaking stress of the thoracic descending aorta.

OBJECTIVE: To assess the influence of aging and aortic stiffness on the extent of irreversible deformation and breaking stress of the human thoracic aorta. METHODS: From 14 human heart valve donors without aortic disease (mean age 35 years, range 8-59 years), 14 intact segments of the thoracic descending aorta were studied within 48 h after cardiac arrest. In an experimental setup, the segments were submitted to increasing hydrostatic pressure loads, both statically and dynamically, while radius and wall thickness were monitored echocardiographically. Pressure-radius curves were constructed. Radius and wall thickness were determined at a pressure of 100 mmHg. Radius at elastin resting length and collagen recruitment pressure (Pcol, mmHg) were derived from the pressure-radius relationship and stress-strain curves were constructed to yield Young's moduli of elastin and collagen. Distensibility (D, mmHg-1) was determined while loading the segment with a sinusoidal pressure wave of 120/50 mmHg at both 0.5 and 1 Hz. Subsequently increasing static pressure loads of 400, 800, 1200 and 1600 mmHg were applied. After each pressure load, the increase in aortic radius at a pressure of 100 mmHg (Rinc) was determined. The experiment continued until rupture occurred and breaking stress (sigma break, N m-2) was calculated, donor age and aortic stiffness were correlated with Rinc and sigma break of the aortic segments. RESULTS: Mean breaking stress of the 14 segments was 2.7 x 10(6) N m-2. Breaking stress was negatively correlated with age (r2 = 0.66) and positively with D (r2 = 0.44) and with Pcol (r2 = 0.18). Seven segments survived a pressure load of 800 mmHg, in these vessels, the extent of irreversible dilation was positively correlated with age (r2 = 0.42) and negatively with D (r2 = 0.40) and Pcol (r2 = 0.40). CONCLUSION: Permanent deformation and rupture of the human thoracic aorta following pressure overload are influenced by age, distensibility and collagen recruitment pressure.

Adolescent↗
PubMed1999

Postsurgical course of patients with congenitally corrected transposition of the great arteries.

Because congenitally corrected transposition is a rare congenital anomaly, little is known about the prognosis of patients with this syndrome. The present study evaluated the functional status and clinical course of 73 patients (42 male and 31 female) aged 10 days to 73 years. Follow-up was 1 week to 37 years (mean 12.7 years). Particular attention was paid to the systemic right ventricle, considering the current controversies about long-term right ventricular function. Survival of patients with this condition in general was significantly below normal. The total mortality rate was 11% (8 patients) after a mean follow-up of 12.7 years (range 10 days to 37 years). Mean age at death was 18.5 years (range 6 days to 63 years). The mean age of the survivors at latest follow-up was 21.4 years (range 4 months to 73 years). In most patients, right ventricular function deteriorated and tricuspid valve regurgitation increased, which began at a very young age. Patients without associated lesions developed complications at a higher age compared with the total group. Right ventricular and tricuspid valvular function deteriorated more frequently in patients following intracardiac operation (28% and 52%, respectively) compared with patients undergoing palliative intervention (16%) or no surgery at all (28%). We conclude that survival of patients with this condition is substantially reduced compared with the natural history of an age- and gender-matched general population. This could be explained by the associated cardiac defects, but also by the anatomic condition itself.

Adolescent↗
PubMed1999

Abnormalities in liver function and coagulation profile following the Fontan procedure.

OBJECTIVE: To investigate liver function and coagulation disorders in patients with a Fontan circulation at different time intervals after surgery. DESIGN: Retrospective analysis of clinical data and cross sectional study relating liver function and coagulation profile to time since surgery, in 28 surviving patients after the modified Fontan procedure. PATIENTS: 20 patients (71%) with atriopulmonary anastomosis, seven (25%) with atrioventricular anastomosis, and one (4%) with total cavopulmonary connection. Follow up ranged from 2.0 to 21.8 years (mean 11.1). RESULTS: Abnormal liver function tests, mainly reflecting cholestasis, were present in 21 patients who had a significantly longer follow up (p < 0.01). Protein synthesis was normal in almost all patients. Coagulation profile showed abnormalities in 22 patients. "Procoagulant" abnormalities-that is, decreased plasminogen and protein C activity-were found in 11 and five patients, respectively. The extent of these abnormalities was less in patients with a longer follow up. Anticoagulant abnormalities were factor V deficiency in 16 patients and factor VII deficiency in 17, resulting in a prolonged prothrombin time in 19 patients. Thirteen patients had both pro- and anticoagulant abnormalities. A prethrombotic state was present in five patients, with a significantly longer mean time interval since surgery (p = 0.05). Thus, although the individual procoagulant indices decreased with increasing time intervals since surgery, a prethrombotic state was found particularly in patients with a long term follow up. CONCLUSIONS: Mild cholestasis was mainly present in Fontan patients with a long duration of follow up. Along with laboratory procoagulant abnormalities indicating a prethrombotic state, anticoagulant abnormalities were also present. The coagulation profile varied at different time intervals after surgery. Thus detailed evaluation should be performed regularly, and the use of anticoagulants should be considered in every patient. Long term prospective studies are needed to evaluate the individual fluctuations of coagulation profile over time following a Fontan procedure.

Adolescent↗
PubMed1999

Survival and complication free survival in Marfan's syndrome: implications of current guidelines.

OBJECTIVE: To evaluate survival and complication free survival in patients with Marfan's syndrome and to assess the possible influence of recently revised guidelines for prophylactic aortic root replacement in these patients. METHODS: 130 patients who had been attending one institution over 14 years were evaluated. Kaplan-Meier analysis was performed in 125 patients who did not present with aortic root dissection as the first sign of Marfan's syndrome, with the end points: death, aortic root dissection, and prophylactic aortic root replacement after diagnosis. In the patients developing aortic root dissection, current guidelines for prophylactic aortic root replacement were retrospectively applied to investigate the number of dissections that could theoretically have been prevented. The guidelines were: (1) aortic root diameter >/= 55 mm, (2) positive family history of aortic dissections and aortic root diameter >/= 50 mm, and (3) aortic root growth >/= 2 mm/year. Outcomes following emergency surgery (15 patients) and prophylactic surgery of the aortic root (30 patients) were compared. RESULTS: Five and 10 year survival after diagnosis was 95% and 88%, and the five and 10 year complication free survival was 78% and 66%, respectively. Thirteen patients developed dissection, 30 underwent prophylactic repair, and 82 had an uncomplicated course. Eleven dissections could theoretically have been prevented by application of the current guidelines. Five year survival following emergency and prophylactic repair of the aortic root was 51%, and 97%, respectively. CONCLUSIONS: Survival in the Marfan's syndrome in the past 14 years seems satisfactory; with application of current guidelines, it has probably even improved. However, because of the high fatality rate in Marfan patients developing aortic root dissection, more extensive screening for Marfan's syndrome and a search for additional risk factors are desirable.

Adolescent↗
PubMed1998

Changes in aortic distensibility and pulse wave velocity assessed with magnetic resonance imaging following beta-blocker therapy in the Marfan syndrome.

It has been shown that beta-adrenergic blocking agents may reduce the rate of aortic root dilation and the development of aortic complications in patients with the Marfan syndrome. This may be due to beta-blocker-induced changes in aortic stiffness, of which distensibility and pulse wave velocity are in vivo measurable derivatives. We studied changes in distensibility at 4 levels of the aorta and pulse wave velocity along the entire aorta after 2 weeks of beta-blocker therapy in 6 Marfan syndrome patients and in 6 healthy volunteers, using magnetic resonance imaging (MRI) combined with brachial artery blood pressure measurements. In both groups, mean blood pressure decreased significantly (Marfan: 86 +/- 6 vs 78 +/- 5 mm Hg, p <0.05; control: 80 +/- 8 vs 73 +/- 3 mm Hg, p <0.05) (all data expressed as mean +/- 1 SD). At baseline, the Marfan syndrome patients exhibited decreased distensibility at the level of the ascending aorta (2 +/- 1 vs 6 +/- 2 10(-3)mm Hg(-1), p <0.01) and increased pulse wave velocity (6.2 +/- 0.4 vs 3.9 +/- 0.4 ms(-1), p <0.01) compared with control subjects. Only the Marfan syndrome patients had a significant increase in aortic distensibility at multiple levels and a significant decrease in pulse wave velocity after beta-blocker therapy (ascending aorta distensibility: 2 +/- 1 vs 4 +/- 1 10(-3)mm Hg(-1), p <0.05; abdominal aorta distensibility: 5 +/- 2 vs 8 +/- 3 10(-3)mm Hg(-1), p <0.05; pulse wave velocity: 6.2 +/- 0.4 vs 5.0 +/- 1.0 ms(-1), p <0.05). Thus, aortic stiffness in Marfan syndrome, together with mean blood pressure, is reduced by beta-blocker therapy, and MRI is well suited to detect these changes by measuring distensibility and pulse wave velocity.

Adolescent↗
PubMed1998

Marfan syndrome in children and adolescents: an adjusted nomogram for screening aortic root dilatation.

OBJECTIVE: To construct an adjusted nomogram for the echocardiographic screening of aortic root diameter in children with possible Marfan disease. DESIGN: In 91 children (42 boys, 49 girls, age range 3.2 to 18.4 years) undergoing Marfan screening from 1983 until 1996, the diagnosis Marfan syndrome and any other aortic pathology was definitely ruled out. These served as a control population to set appropriate reference standards. RESULTS: Compared with a standard Dutch reference population, body surface area of the control subjects (mean (SD)) was above the 50th centile (boys 0.09 (0.20) m2, range -0.28 to 0.69 m2; girls 0.09 (0.17) m2, range -0.17 to 0.69 m2). Echocardiographically determined aortic root diameter and body surface area showed a linear relation and a greater variability of aortic root diameter in these relatively tall subjects (n = 91, R2 = 0.62) than in the standard nomogram (n = 56, R2 = 0.93). In 24% of cases (n = 22), the aortic root exceeded the upper limit of normal in the standard nomogram, by 2.2 (2.0) mm. An adjusted nomogram was constructed with a higher upper limit. CONCLUSIONS: A Marfan screening population differs from the unselected population in body surface area and aortic root size variability. An adjusted nomogram should therefore be used to detect a truly enlarged aortic root.

Adolescent↗
PubMed1998

Marfan syndrome in children and adolescents: predictive and prognostic value of aortic root growth for screening for aortic complications.

OBJECTIVE: To assess and measure the diagnostic and prognostic value of individual aortic root growth in children and adolescents with Marfan syndrome. DESIGN: From 1983 to 1996, 250 children were screened for Marfan syndrome. Serial echocardiographic aortic root measurements of 123 children (57 Marfan, 66 control) were available for evaluation of aortic root growth. Aortic root diameters were correlated with body surface area. Based on individual growth of the aortic root a discrimination formula was derived to distinguish Marfan patients from control subjects. RESULTS: Sensitivity and specificity of this method, which were dependent on the number of measurements, were 84% and 73%, respectively, for three serial measurements. The discrimination score also predicted a five year complication free survival in all patients. CONCLUSIONS: In children and adolescents with Marfan syndrome, aortic root growth differs significantly from subjects in whom Marfan syndrome was definitely ruled out during screening. Measurement of individual aortic root growth may serve to establish diagnosis at an early stage and is of prognostic value for occurrence of aortic complications.

Adolescent↗
PubMed1996

Aprotinin in aortocoronary bypass surgery: increased risk of vein-graft occlusion and myocardial infarction? Supportive evidence from a retrospective study.

To assess the thrombotic risk of aprotinin in aortocoronary bypass surgery, we retrospectively analyzed the results of a trial, originally designed to compare the effects of one-year treatment with various antithrombotic drugs in the prevention of vein-graft occlusion. Graft patency at one year was assessed by angiography. Myocardial infarction, thromboembolism, major bleeding, and death were clinical endpoints. Of 948 randomized patients, 42 received aprotinin, all enrolled by one of the participating centres. Occlusion rates of distal anastomoses were 20.5% in the aprotinin group and 12.7% in the non-aprotinin group (p = 0.091). The proportions of patients with occluded grafts were 44.1% versus 26.3% (p = 0.029). Perioperative myocardial infarction occurred in 14.3% and 7.0%, respectively (p = 0.12). Mean postoperative blood loss was 451 ml in the aprotinin group compared with 1039 ml in the non-aprotinin group (p < 0.0001). Mean transfusion requirements were 1.1 U versus 2.1 U of red blood cells (p = 0.004). Aprotinin decreases blood loss and transfusion requirement. Our data suggest that this benefit may be associated with a reduction of graft patency and an increased risk of myocardial infarction.

Aprotinin↗