Carsten Rickers

Utility of Cardiac Magnetic Resonance Imaging in the Diagnosis of Hypertrophic Cardiomyopathy

Background—Two-dimensional echocardiography is currently the standard test for the clinical diagnosis of hypertrophic cardiomyopathy (HCM). The present study was undertaken to determine whether cardiac MRI (CMR) affords greater accuracy than echocardiography in establishing the diagnosis and assessing the magnitude of left ventricular (LV) hypertrophy in HCM. Methods and Results—Forty-eight patients (age 34±16 years) suspected of having HCM (or with a confirmed diagnosis) were imaged by both echocardiography and CMR to assess LV wall thickness in 8 anatomic segments (total n=384 segments) and compared in a blinded fashion. Maximum LV thickness was similar by echocardiography (21.7±9.1 mm) and CMR (22.5±9.6 mm; P=0.21). However, in 3 (6%) of the 48 patients, echocardiography did not demonstrate LV hypertrophy, and CMR identified otherwise undetected areas of wall thickening in the anterolateral LV free wall (17 to 20 mm), which resulted in a new diagnosis of HCM. In the overall study group, compared with CMR, echocardiography also underestimated the magnitude of hypertrophy in the basal anterolateral free wall (by 20±6%; P=0.001), as well as the presence of extreme LV wall thickness (≥30 mm) in 10% of patients (P<0.05). Conclusions—CMR is capable of identifying regions of LV hypertrophy not readily recognized by echocardiography and was solely responsible for diagnosis of the HCM phenotype in an important minority of patients. CMR enhances the assessment of LV hypertrophy, particularly in the anterolateral LV free wall, and represents a powerful supplemental imaging test with distinct diagnostic advantages for selected HCM patients.

Outcome of the Norwood operation in patients with hypoplastic left heart syndrome: a 12-year single-center survey.

OBJECTIVE Recent advances in perioperative care have led to a decrease in mortality of children with hypoplastic left heart syndrome undergoing the Norwood operation. This study aimed to evaluate the outcome of the Norwood operation in a single center over 12 years and to identify clinical and anatomic risk factors for adverse early and longer term outcome. METHODS Full data on all 157 patients treated between 1996 and 2007 were analyzed. RESULTS Thirty-day mortality of the Norwood operation decreased from 21% in the first 3 years to 2.5% in the last 3 years. The estimated exponentially weighted moving average of early mortality after 157 Norwood operations was 2.3%. Risk factors were an aberrant right subclavian artery, the use and duration of circulatory arrest, and the duration of total support time. The anatomic subgroup mitral stenosis/aortic atresia and female gender tended to show an increased early mortality. In the group of patients who required postoperative cardiopulmonary resuscitation, the ascending aorta was significantly smaller than in the remainder (3.03 +/- 1.05 vs 3.63 +/- 1.41 mm). Interstage mortality was 15% until the initiation of a home surveillance program in 2005, which has zeroed it so far. It was significantly higher in the mitral stenosis/aortic atresia subgroup and tended to be higher in patients who required cardiopulmonary resuscitation after the Norwood operation. The best actuarial survival was observed in the mitral atresia/aortic atresia subgroup. CONCLUSION The Norwood operation can now be performed with low mortality. Patients with mitral stenosis/aortic atresia still constitute the most challenging subgroup.

Magnetic Resonance Image-Guided Transcatheter Closure of Atrial Septal Defects

Background—Recent developments in cardiac MRI have extended the potential spectrum of diagnostic and interventional applications. The purpose of this study was to test the ability of MRI to perform transcatheter closures of secundum type atrial septal defects (ASD) and to assess ASD size and changes in right cardiac chamber volumes in the same investigation. Methods and Results—In 7 domestic swine (body weight, 38±13 kg), an ASD (Qp:Qs=1.7±0.2) was created percutaneously by balloon dilation of the fossa ovalis. The ASD was imaged and sized by both conventional radiography and MRI. High-resolution MRI of the ASD diameters correlated well with postmortem examination (r =0.97). Under real-time MR fluoroscopy, the introducer sheath was tracked toward the left atrium with the use of novel miniature MR guide wires. The defect was then closed with an Amplatzer Septal Occluder. In all animals, it was possible to track and interactively control the position of the guide wire within the vessels and the heart, including the successful deployment of the Amplatzer Septal Occluder. Right atrial and ventricular volumes were calculated before and after the intervention by using cine-MRI. Both volumes were found to be significantly reduced after ASD closure (P <0.005). Conclusions—These in vivo studies demonstrate that catheter tracking and ASD device closure can be performed under real-time MRI guidance with the use of intravascular antenna guide wires. High-resolution imaging allows accurate determination of ASD size before the intervention, and immediate treatment effects such as changes in right cardiac volumes can also be measured.

Normal values of aortic dimensions, distensibility, and pulse wave velocity in children and young adults: a cross-sectional study

BackgroundAortic enlargement and impaired bioelasticity are of interest in several cardiac and non-cardiac diseases as they can lead to cardiovascular complications. Cardiovascular magnetic resonance (CMR) is increasingly accepted as a noninvasive tool in cardiovascular evaluation. Assessment of aortic anatomy and bioelasticity, namely aortic distensibility and pulse wave velocity (PWV), by CMR is accurate and reproducible and could help to identify anatomical and bioelastic abnormalities of the aorta. However, normal CMR values for healthy children and young adults are lacking.MethodsSeventy-one heart-healthy subjects (age 16.4 ± 7.6 years, range 2.3 - 28.3 years) were examined using a 3.0 Tesla CMR scanner. Aortic cross-sectional areas and aortic distensibility were measured at four positions of the ascending and descending thoracic aorta. PWV was assessed from aortic blood flow velocity measurements in a aortic segment between the ascending aorta and the proximal descending aorta. The Lambda-Mu-Sigma (LMS) method was used to obtain percentile curves for aortic cross-sectional areas, aortic distensibility and PWV according to age.ResultsAortic areas, PWV and aortic distensibility (aortic cross-sectional areas: r = 0.8 to 0.9, p < 0.001; PWV: r = 0.25 to 0.32, p = 0.047 to 0.009; aortic distensibility r = -0.43 to -0.62, p < 0.001) correlated with height, weight, body surface area, and age. There were no significant sex differences.ConclusionsThis study provides percentile curves for cross-sectional areas, distensibility and pulse wave velocity of the thoracic aorta in children and young adolescents between their 3rd and 29th year of life. These data may serve as a reference for the detection of pathological changes of the aorta in cardiovascular disease.

New self-expanding patent foramen ovale occlusion device

Our purpose was to evaluate a new self‐expanding device for closure of the patent foramen ovale (PFO). A transeptal catheter passage through the flap of the fossa ovalis was performed with a transeptal needle inside a catheter, creating a PFO in two minipigs. In an additional five animals, a naturally occurring PFO was found. The device is made from 0.005 inch nitinol wire mesh with polyester fabric inside, similar in construction to the Amplatzer atrial septal occluder. However, the left atrial disc is smaller (18 mm) than the right atrial disc (26 mm). Both discs are connected by a very short flexible waist (3 mm) that allows free movement of both retention discs. Pulmonary and right atrial angiography were performed after placement, at 1 month, and at 3 months follow‐up. Placement of the device was technically successful in six animals. One animal died from ventricular fibrillation during placement. Pulmonary angiography and echocardiography showed complete occlusion of the PFO in six animals. Two animals were sacrificed after 1 month and four animals after 3 months. In the animals sacrificed at 1 month, histopathological examination showed partial (n = 2) endothelialization, and in the 3 months follow‐up group (n = 4) endothelialization was complete. The device appears to be highly effective for occlusion of PFOs. This procedure may be performed as an outpatient procedure due to the small 7 Fr delivery system sheath. Cathet. Cardiovasc. Intervent. 47:370–376, 1999.

Transcatheter closure of membranous ventricular septal defects with a new nitinol prosthesis in a natural swine model.

Transcatheter closure of a membranous ventricular septal defect (MVSD) is much more difficult than closure of other intracardiac defects because of the proximity to the aortic and tricuspid valves and their relatively large size in small children. In this report, transcatheter closure of naturally occurring membranous VSDs was attempted in 12 Yucatan minipigs. The prosthesis is constructed from fine Nitinol wires in the shape of two buttons and a connecting waist filled with polyester fiber. Two kinds of prosthesis were used in this study: concentric and eccentric left‐sided retention disks. A 6 or 7 Fr delivery sheath was advanced across the membranous VSD over a wire from femoral vein. The prosthesis was inserted through the sheath by pushing the delivery cable to deploy a button into left ventricle and the second button was then deployed into right ventricle by withdrawing the sheath. Successful implantation of the device was achieved in all animals except one. Complete closure rate was 58.3% immediately after placement, 100% at 1 week, 90.9% at 1 month and 3 months, and 100% at 6 months. An associated aneurysm of the membranous septum increased significantly in size in two of three animals using the concentric device, and in none of the animals using the eccentric device. A trace to mild aortic regurgitation was present in two of the three animals using the concentric device, and only in one of the eight animals using the eccentric device. Five animals developed a trace to mild tricuspid regurgitation. Pathologic examination showed all devices to be covered by smooth neoendothelium at 3 months. This report presents the first experimental study where closure of membranous ventricular septal defects in a swine model was attempted by specially constructed devices. Procedural success and occlusion rates are very encouraging but overall results cannot equal surgery. Further experimentation is needed with devices that are redesigned according to the experience gained from this study. Cathet. Cardiovasc. Intervent. 50:502–509, 2000.

Magnetic resonance imaging of myocardial contrast enhancement with MS-325 and its relation to myocardial blood flow and the perfusion reserve.

To determine with an intravascular contrast agent the relation between the rate of myocardial signal enhancement during the first pass (upslope) and myocardial blood flow (MBF), and to derive and validate a corrected perfusion reserve (PR) index from the upslope parameter.

MR myocardial perfusion imaging

Contrast material-enhanced myocardial perfusion imaging by using cardiac magnetic resonance (MR) imaging has, during the past decade, evolved into an accurate technique for diagnosing coronary artery disease, with excellent prognostic value. Advantages such as high spatial resolution; absence of ionizing radiation; and the ease of routine integration with an assessment of viability, wall motion, and cardiac anatomy are readily recognized. The need for training and technical expertise and the regulatory hurdles, which might prevent vendors from marketing cardiac MR perfusion imaging, may have hampered its progress. The current review considers both the technical developments and the clinical experience with cardiac MR perfusion imaging, which hopefully demonstrates that it has long passed the stage of a research technique. In fact, cardiac MR perfusion imaging is moving beyond traditional indications such as diagnosis of coronary disease to novel applications such as in congenital heart disease, where the imperatives of avoidance of ionizing radiation and achievement of high spatial resolution are of high priority. More wide use of cardiac MR perfusion imaging, and novel applications thereof, are aided by the progress in parallel imaging, high-field-strength cardiac MR imaging, and other technical advances discussed in this review.

Maladaptive Aortic Properties in Children After Palliation of Hypoplastic Left Heart Syndrome Assessed by Cardiovascular Magnetic Resonance Imaging

Background— The status of the reconstructed aorta in hypoplastic left heart syndrome is considered an important determinant of long-term prognosis. Therefore, we assessed the anatomy, elastic properties, and viability of the aorta and right ventricular function in patients with hypoplastic left heart syndrome by cardiovascular magnetic resonance imaging. Methods and Results— Cardiovascular magnetic resonance imaging was performed in 40 patients with hypoplastic left heart syndrome (age, 6.0±2.2 years) and 13 control subjects (age, 6.6±2.2 years). Aortic dimensions and distensibility were calculated at different locations of the aorta using gradient-echo cine imaging at 3.0 T. Additionally, pulse-wave velocity, right ventricular ejection fraction, and aortic late gadolinium enhancement for viability assessment were measured. Compared with control subjects, patients with hypoplastic left heart syndrome had increased axial diameters of the aortic root (36.0±5.5 versus 24.1±2.7 mm/m2; P<0.01), ascending aorta (32.0±5.0 versus 21.3±1.5 mm/m2; P<0.01), and transverse aortic arch (22.7±5.2 versus 18.7±2.5 mm/m2; P<0.01). Wall distensibility was reduced in the ascending aorta (4.1±2.4 versus 13.5±7.2 10−3 mm Hg−1; P<0.01) and transverse aortic arch (5.4±3.6 versus 10.3±3.5 10−3 mm Hg−1; P<0.01). Pulse-wave velocity trended higher in patients (P=0.06). Reduced distensibility in the ascending aorta correlated with the amount of late gadolinium enhancement in a volume that included the aortic root and the ascending aorta (r=−0.72, P<0.01), and both parameters correlated with decreased right ventricular ejection fraction. Conclusions— Adverse aortic properties post palliation of hypoplastic left heart syndrome manifest themselves by aortic dilatation, decreased distensibility, and increased volume of nonviable aortic wall tissue. The negative association between aortic late gadolinium enhancement and right ventricular ejection fraction suggests unfavorable aortic-ventricular coupling. The potential impact of these findings on long-term right ventricular function should be evaluated in future studies.

Insulin resistance, subclinical left ventricular remodeling, and the obesity paradox: MESA (Multi-Ethnic Study of Atherosclerosis).

OBJECTIVES This study assessed whether impaired fasting glucose (IFG), insulin resistance, and waist-to-hip ratio (WHR) had effects on cardiac remodeling, independent of obesity, in the MESA (Multi-Ethnic Study of Atherosclerosis) trial. BACKGROUND Recent studies have suggested that central obesity and insulin resistance may be primary mediators of obesity-related cardiac remodeling independent of body mass index (BMI). METHODS We investigated 4,364 subjects without diabetes in the MESA trial. IFG (100 to 125 mg/dl) or insulin resistance (by homeostatic model assessment of insulin resistance [HOMA-IR]) and WHR were used for cardiometabolic phenotyping. Multivariate linear regression analysis was used to determine the effects of the cardiometabolic markers on left ventricular (LV) remodeling, assessed primarily through the LV mass-to-volume ratio obtained by cine cardiac magnetic resonance imaging. RESULTS Individuals with IFG were more likely to be older and hypertensive, with increased prevalence of cardiometabolic risk factors regardless of BMI. In each quartile of BMI, subjects with above-median HOMA-IR, above-median WHR, or IFG had a higher LV mass-to-volume ratio (p < 0.05 for all). HOMA-IR (p < 0.0001), WHR (p < 0.0001), and the presence of IFG (p = 0.04), but not BMI (p = 0.24), were independently associated with LV mass-to-volume ratio after adjustment for age, sex, hypertension, race, and dyslipidemia. CONCLUSIONS Insulin resistance and WHR were associated with concentric LV remodeling independent of BMI. These results support the emerging hypothesis that the cardiometabolic phenotype, defined by insulin resistance and central obesity, may play a critical role in LV remodeling independently of BMI.