Serge Willoteaux

The vascular phenotype in Pseudoxanthoma elasticum and related disorders: contribution of a genetic disease to the understanding of vascular calcification.

Vascular calcification is a complex and dynamic process occurring in various physiological conditions such as aging and exercise or in acquired metabolic disorders like diabetes or chronic renal insufficiency. Arterial calcifications are also observed in several genetic diseases revealing the important role of unbalanced or defective anti- or pro-calcifying factors. Pseudoxanthoma elasticum (PXE) is an inherited disease (OMIM 264800) characterized by elastic fiber fragmentation and calcification in various soft conjunctive tissues including the skin, eyes, and arterial media. The PXE disease results from mutations in the ABCC6 gene, encoding an ATP-binding cassette transporter primarily expressed in the liver, kidneys suggesting that it is a prototypic metabolic soft-tissue calcifying disease of genetic origin. The clinical expression of the PXE arterial disease is characterized by an increased risk for coronary (myocardial infarction), cerebral (aneurysm and stroke), and lower limb peripheral artery disease. However, the structural and functional changes in the arterial wall induced by PXE are still unexplained. The use of a recombinant mouse model inactivated for the Abcc6 gene is an important tool for the understanding of the PXE pathophysiology although the vascular impact in this model remains limited to date. Overlapping of the PXE phenotype with other inherited calcifying diseases could bring important informations to our comprehension of the PXE disease.

Portosystemic collateral vessels in liver cirrhosis: a three-dimensional MDCT pictorial review

PurposePortosystemic collateral vessels (PSCV) are a consequence of the portal hypertension that occurs in chronic liver diseases. Their prognosis is strongly marked by the risk of digestive hemorrhage and hepatic encephalopathy.Materials and methodsCT was performed with a 16-MDCT scanner. Maximum intensity projection and volume rendering were systematically performed on a workstation to analyze PSCV.ResultsWe describe the PSCV according to their drainage into either the superior or the inferior vena cava. In the superior vena cave group, we found gastric veins, gastric varices, esophageal, and para-esophageal varices. In the inferior vena cava group, the possible PSCV are numerous, with different sub groups: gastro and spleno renal shunts, paraumbilical and abdominal wall veins, retroperitoneal shunts, mesenteric varices, gallbladder varices, and omental collateral vessels. Regarding clinical consequences esophageal and gastric varices are most frequently involved in digestive bleeding; splenorenal shunts often lead to hepatic encephalopathy; the paraumbilical vein is an acceptable derivation pathway for natural decompression of the portal system.ConclusionKnowledge of precise cartography of PSCV is essential to therapeutic decisions. MDCT is the best way to understand and describe the different types of PSCV.

Planned caesarean in the interventional radiology cath lab to enable immediate uterine artery embolization for the conservative treatment of placenta accreta.

AIM To evaluate the feasibility and efficacy of routine uterine artery embolization (UAE) immediately after planned caesareans performed in the cath lab for conservative treatment of placenta accreta. MATERIALS AND METHODS A retrospective study included all patients who had a planned caesarean in the cath lab for conservative treatment of placenta accreta at Angers University Hospital, which is a tertiary care centre, from April 2001 to September 2010. Twelve patients underwent UAE immediately after caesarean with the placenta left partially or totally in situ. The success rate of embolization, blood loss, and complications were reported. RESULTS Diagnosis of abnormal placentation was confirmed by caesarean findings in 14 cases. Four patients had a percreta form with bladder invasion. In seven cases blood loss was insignificant and UAE was prophylactic; no secondary haemorrhage was observed in this group. Postpartum haemorrhage occurred in five cases: control of immediate postpartum bleeding by embolization was successful in three and failed in two leading to hysterectomy. In one case uterine necrosis occurred 6 weeks after embolization, requiring a hysterectomy. Delayed complications resulted in hysterectomy and partial bladder resection 3 months after delivery for one of the patients with placenta percreta. CONCLUSION UAE immediately after a caesarean performed in the cath lab is a feasible therapeutic option for conservative treatment of placenta accreta. Advantages include reducing stress and risks associated with transferring women with potentially unstable haemodynamics.

Longitudinal Strain Is a Marker of Microvascular Obstruction and Infarct Size in Patients with Acute ST-Segment Elevation Myocardial Infarction

Objectives We assessed the value of speckle tracking imaging performed early after a first ST-segment elevation myocardial infarction (STEMI) in order to predict infarct size and functional recovery at 3-month follow-up. Methods 44 patients with STEMI who underwent revascularization within 12 h of symptom onset were prospectively enrolled. Echocardiography was performed 3.9±1.2 days after myocardial reperfusion, assessing circumferential (CGS), radial (RGS), and longitudinal global (GLS) strains. Late gadolinium-enhanced cardiac magnetic imaging (CMR), for assessing cardiac function, infarct size, and microvascular obstruction (MVO), was conducted 5.6±2.5 days and 99.4±4.6 days after myocardial reperfusion. Results GLS was evaluable in 97% of the patients, while CGS and RGS could be assessed in 85%. Infarct size significantly correlated with GLS (R = 0.601, p<0.001), RGS (R = −0.405, p = 0.010), CGS (R = 0.526, p = 0.001), ejection fraction (R = −0.699, p<0.001), wall motion score index (WMSI) (R = 0.539, p = 0.001), and left atrial volume (R = 0.510, p<0.001). Baseline ejection fraction and GLS were independent predictors of 3-month infarct size. MVO mass significantly correlated with GLS (R = 0.376, p = 0.010), WMSI (R = 0.387, p = 0.011), and ejection fraction (R = −0.389, p = 0.011). In multivariate analysis, GLS was the only independent predictor of MVO mass (p = 0.015). Longitudinal strain >−6.0% within the infarcted area exhibited 96% specificity and 61% sensitivity for predicting the persistence of akinesia (≥3 segments) at 3-month follow-up. Conclusions Speckle-tracking strain imaging performed early after a STEMI is easy-to-use as a marker for persistent akinetic territories at 3 months. In addition, GLS correlated significantly with MVO and final infarct size, both parameters being relevant post-MI prognostic factors, usually obtained via CMR.

Quantification of the Calcification Phenotype of Abcc6-Deficient Mice with Microcomputed Tomography

Pseudoxanthoma elasticum in humans and dystrophic cardiac calcification in mice are heritable disorders characterized by dystrophic calcification of soft connective tissues related to the defective function of the ABCC6 (human)/Abcc6 (mouse) transporter. Of particular interest is the finding of calcified vibrissae in Abcc6(-/-) mice, which facilitates the study of dystrophic calcification by histological techniques. We aimed to determine whether mice prone to dystrophic cardiac calcification (C3H/HeOuJ and DBA/2J strains) presented similar vibrissae changes and to evaluate the value of microcomputed tomography to quantify the extent of mystacial vibrissae calcifications. These calcifications were absent in DBA/2J and C57BL/6J control mice. In both Abcc6(-/-) and C3H/HeOuJ mice, calcifications progressed in a caudal-rostral direction with aging. However, the calcification process was delayed in C3H/HeOuJ mice, indicating an incomplete expression of the calcification phenotype. We also found that the calcification process in the cephalic region was not limited to mystacial vibrissae but was also present in other periorbital sensorial vibrissae. The vibrissae calcification was circular and encompassed the medial region of the vibrissae capsule, adjacent to the ring and cavernous sinuses (the areas adjacent to blood and lymphatic vessels). Collectively, our findings confirm that Abcc6 acts as an inhibitor of spontaneous chronic mineralization and that microcomputed tomography is a valuable noninvasive tool for the assessment of the calcification phenotype in Abcc6-deficient mice.

CMR assessment after a transapical-transcatheter aortic valve implantation

AIMS To describe the time course of myocardial scarring after transapical-transcatheter aortic valve implantation (TA-TAVI) with the Edwards SAPIEN XT™ and the Edwards SAPIEN™ prosthesis in a 3-month follow-up study using cardiac magnetic resonance imaging (CMR). METHODS In 20 TA-TAVI patients, CMR was performed at discharge and 3 months (3M). Cine-MRI was used for left ventricular (LV) functional assessment, and late gadolinium enhancement (LGE) imaging was employed for detecting the presence of myocardial scarring. Special attention was given to any artifacts caused by the prosthesis, which were consequently defined using a three-grade artifact scale. RESULTS We systematically reported the presence of small LGE hyperintensity relating to the apical segment, with no variation found between discharge and 3M (2.8±1.6g vs. 2.35±1.1g). LV ejection fraction, end-diastolic, and end-systolic volumes did not significantly vary. A small area of apical akinesia was observed, with no improvement at follow-up. Whereas the Edwards SAPIEN XT™ prosthesis and the Edwards SAPIEN™ prosthesis are both constituted by metallic stenting structure, the Edwards SAPIEN™ was responsible for a larger signal void, thus potentially limiting the diagnostic performance of CMR. CONCLUSIONS CMR may be performed safely in the context of TA-TAVI. The presence of a very small apical infarction correlating with focal akinesia was observed. As expected, the Edwards SAPIEN XT™ prosthesis was shown to be particularly suitable for CMR assessment.

The Contribution of Arterial Calcification to Peripheral Arterial Disease in Pseudoxanthoma Elasticum

Background and aims The contribution of arterial calcification (AC) in peripheral arterial disease (PAD) and arterial wall compressibility is a matter of debate. Pseudoxanthoma elasticum (PXE), an inherited metabolic disease due to ABCC6 gene mutations, combines elastic fiber fragmentation and calcification in various soft tissues including the arterial wall. Since AC is associated with PAD, a frequent complication of PXE, we sought to determine the role of AC in PAD and arterial wall compressibility in this group of patients. Methods and Results Arterial compressibility and patency were determined by ankle-brachial pressure index (ABI) in a cohort of 71 PXE patients (mean age 48±SD 14 yrs, 45 women) and compared to 30 controls without PAD. Lower limb arterial calcification (LLAC) was determined by non-contrast enhanced helicoidal CT-scan. A calcification score (Ca-score) was computed for the femoral, popliteal and sub-popliteal artery segments of both legs. Forty patients with PXE had an ABI<0.90 and none had an ABI>1.40. LLAC increased with age, significantly more in PXE subjects than controls. A negative association was found between LLAC and ABI (r = −0.363, p = 0.002). The LLAC was independently associated with PXE and age, and ABI was not linked to cardiovascular risk factors. Conclusions The presence of AC was associated with PAD and PXE without affecting arterial compressibility. PAD in PXE patients is probably due to proximal obstructive lesions developing independently from cardiovascular risk factors.

Applications of phase-contrast velocimetry sequences in cardiovascular imaging

AIMS To describe and illustrate the main applications of phase-contrast flow quantification in cardiovascular imaging. CONCLUSION Phase-contrast velocimetry sequences provide an accurate, reliable, reproducible and non-invasive study of blood flow, information which is sometimes not available from other investigation methods. The haemodynamic information obtained from these complement MRI angiography images. They appear to have a range of clinical applications, firstly improving pathophysiological understanding but also contributing to the treatment and follow-up strategy after surgical or endovascular treatment.

Les conditions techniques d'utilisation de l'IRM cardiaque

Establishing a clinical cardiovascular magnetic resonance imaging (MRI) program needs a dedicated technical surroundings as well as a specific and expert staff. These guidelines based either on proofs or on expert consensus are stated in order to help the physicians to reach or maintain the competence required for clinical use of cardiovascular MRI. After the general safety statements, the guidelines are focused on hardware and software requirements, the MRI sequences and views, the post-acquisition analysis, and the staff. Specific safety concerns are then approached, more particularly stress testing MRI.

MRI and venographic aspects of pelvic venous insufficiency.

Pelvic venous insufficiency is a frequent pathology in multiparous women. Diagnosis can be made by chance or suspected in the case of symptoms suggesting pelvic congestion syndrome or atypical lower limb varicosity fed by pelvic leaks. After ultrasound confirmation, dynamic venography is the reference pretherapeutic imaging technique, searching for pelvic varicosity and possible leaks to the lower limbs. MRI is less invasive and allows a three-dimensional study of the varicosity and, with dynamic angiography, it can assess ovarian reflux. It also helps to plan or even sometimes avoid diagnostic venography.