Hans Romkes

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.

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 μg/kg/min to maximum dose 15 μg/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.

Relation between exercise-induced hypertension and sustained hypertension in adult patients after successful repair of aortic coarctation.

Objectives To investigate whether exercise-induced hypertension in successfully repaired adult post-coarctectomy patients is associated with hypertension on 24-h blood pressure measurement and increased left ventricular mass. Methods One hundred and forty-four consecutive post-coarctectomy patients (mean age 31.5 years, range 17–74 years; mean age at repair 7.9 years, range 0–45 years) from three tertiary referral centres were studied using ambulatory blood pressure monitoring, treadmill exercise testing and echocardiography. Results Of the 144 patients, 27 (19%) were known to have sustained hypertension, based on their history, and all were on antihypertensive medication. However, 32 (27%) of the remaining 117 patients showed elevated mean daytime systolic blood pressure readings at 24-h ambulatory blood pressure monitoring (systolic blood pressure ⩾ 140 mmHg). Of the remaining 85 patients with normal mean daytime systolic blood pressure, 18 patients (21%) had exercise-induced hypertension (maximal exercise systolic blood pressure > 200 mmHg). Mean daytime systolic blood pressure was higher in the exercise-induced hypertensive patients compared to the normotensive patients with normal exercise blood pressure (134 ± 5 versus 129 ± 7 mmHg, P = 0.008). By multivariate analysis, both maximal exercise systolic blood pressure (P = 0.007) and resting systolic blood pressure (P < 0.0001) were independently associated with mean daytime systolic blood pressure. Maximal exercise systolic blood pressure had no independent predictive value for left ventricular mass (P = 0.132). Conclusions In adult post-coarctectomy patients, maximal exercise systolic blood pressure is independently associated with mean daytime systolic blood pressure at ambulatory blood pressure monitoring. In this study no independent predictive value of maximal exercise systolic blood pressure for left ventricular mass could be demonstrated.

Quantitative assessment of the pressure and volume overloaded right ventricle: imaging is a real challenge

Physicians are facing an increasing number of patients with right ventricular (RV) overload due to congenital heart disease. Adult patients with congenital heart disease are emerging as a new and continuously growing population. Improvements in medical care, surgical techniques, and closer follow-up have dramatically improved life expectancy in this group of patients. However, in a substantial part of these patients, the RV is or has been subjected to chronic pressure and/or volume overload (Figure 1). The long-term prognosis of these patients is unknown and mainly dependent on factors such as RV (dys)function, the occurrence of RV failure [1], and rhythm disorders [2–4]. There is much controversy regarding the long term outcome of RV function under these circumstances [4–6]. Some patients are asymptomatic [3, 7, 8], while others are in need of frequent medical care [9]. Accordingly, their life expectancy is highly variable and expressed over a wide range [7, 10– 13]. Because assessment of RV function is a difficult task, current evaluation of these patients is mainly based on qualitative parameters [14]. The lack of quantitative determinants for RV function hampers physicians to estimate the appropriate time for surgical or medical intervention in order to prevent irreversible RV failure. Management of these patients would be improved by establishing accurate noninvasive quantitative RV function determinants and to relate them to the already existing qualitative RV function determinants. The combined information could thus be implemented in daily clinical practice. This paper will review the imaging modalities for RV function determination in congenital heart disease.

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.

Frequency of coronary ostial aneurysms after aortic root surgery in patients with the Marfan syndrome

Coronary ostial aneurysm is a common finding in patients with Marfans syndrome after aortic root surgery, especially in patients who are greater than or equal to35 years of age at time of surgery