Carney complex: Clinical and genetic 2010 update.

First described in the mid 1980s, Carney complex is a rare dominantly heritable multiple endocrine neoplasia syndrome that affects endocrine glands as the adrenal cortex, the pituitary and the thyroid. It is associated with many other nonendocrine tumors, including cardiac myxomas, testicular tumors, melanotic schwannoma, breast myxomatosis, and abnormal pigmentation or myxomas of the skin. The Carney complex gene 1 was identified 10 years ago as the regulatory subunit 1A of protein kinase A (PRKAR1A) located at 17q22-24. An inactivating heterozygous germ line mutation of PRKAR1A is observed in about two-thirds of Carney complex patients. This last decade many progresses have been done in the knowledge of this rare disease and its genetics. This review outlines the current state of this knowledge on Carney complex.

[Cardiac MRI: technical considerations]. / 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.

Impact of cardiac magnetic resonance imaging for assessment of Churg-Strauss syndrome: a cross-sectional study in 20 patients.

OBJECTIVE: To examine the diagnostic contributions of cardiac magnetic resonance imaging (CMRI) with delayed-enhancement (DE) in patients with Churg-Strauss syndrome (CSS). METHODS: We consecutively recruited 14 men and 6 women (mean age: 50+/-14 years) with CSS (mean disease duration: 4.5+/-3.6 years) and investigated them independently of the presence/absence of cardiac manifestations. Cardiac manifestations included heart failure in 6 patients, angina pectoris in 1, isolated ECG abnormality in 1, and isolated echocardiography and ECG abnormalities in 1. T1-weighted sequences were recorded after gadolinium injection to study myocardial DE. RESULTS: CMRI abnormalities were found in 13/20 patients, including all 9 patients with myocardial manifestations, and 4 of the 11 asymptomatic patients. DE was centromyocardial in 6 patients, subepicardial in 4, and subendocardial in 3. Most enhanced lesions were in the anteroseptal or lateral walls. Patients with myocardial symptoms and DE had higher transmyocardial wall DE scores (mean: 9.4 vs. 3.7, respectively; p=0.01) and lower left ventricular ejection fractions (mean: 42% vs. 59%; p=0.001) than asymptomatic patients with DE. CONCLUSION: CMRI with DE enabled the detection of myocardial involvement in CSS patients with or without clinical symptoms. The clinical relevance of CMRI abnormalities in patients without clinical, echocardiographic and ECG signs of cardiac involvement remains unknown and needs to be evaluated in future studies. It seems premature to intensify treatment or to prescribe systematically steroids and cytotoxic agents based on the presence of isolated CMRI anomalies.

Delayed-enhanced cardiac magnetic resonance imaging features in peripartum cardiomyopathy.

Peripartum cardiomyopathy (PPCM) is a rare disorder in which left ventricular systolic dysfunction and symptoms of heart failure occur in the peripartum period. Although cardiac magnetic resonance (CMR) is largely used for diagnosis and prognosis assessment in cardiomyopathies, its interest in PPCM is unknown. We reported two cases of patients with PPCM who underwent CMR. One patient had no CMR abnormality, while the second patient had several areas of myocardial delayed enhancement (MDE) on CMR images. During follow up, the patient with normal CMR was asymptomatic and had full recovery of cardiac function, whereas the patient with MDE was still symptomatic with persistence of a left ventricular dysfunction. CMR could have prognosis value in PPCM as demonstrated in other cardiomyopathies.

[The contribution of computed tomography coronary angiography in Kawasaki disease in adult patients. Report of two cases]. / Apport du scanner coronaire dans la prise en charge de la maladie de Kawasaki chez l'adulte. A propos de deux cas.

OBJECTIVES: During the past few years, multislice computed tomography coronary angiography has made great progress in terms of spatial and temporal resolution. Results on detection and quantification of stenoses are excellent. We found interesting reporting its achievements in aneurismal coronaropathies such as the Kawasaki disease. METHODS: We searched for young adults with Kawasaki disease who had a multislice computed tomography coronary angiography available. Several hospitals in the Paris area have been contacted and only two observations have been kept. RESULTS: Computed tomography provided higher performance than coronarography for the measurement of the real diameter of an aneurism taking into account the mural thrombus, evaluation of its links with the collateral branches and the other aneurisms, assessment of the development of recanalized vessels and the degree of development of collateral vessels, and visualization of non-circulating aneurisms which were not detected with coronary angiography. In addition, the evaluation of the location and the degree of the stenoses by the computed tomography matched the coronary angiography data. It was not possible to conclude with this observations that the computed tomography is better for the diagnostic of Kawasaki disease. CONCLUSION: Multislice computed tomography coronary angiography will be likely more and more used to detect and follow coronary anomalies in case of Kawasaki disease among teenagers and young adults. It is recommended for monitoring medium or large aneurisms in order to evaluate their progression to stenosis.

[Coronary artery implantation and course abnormalities: the contribution of multislice CT]. / Anomalies d'implantation et de trajet des artères coronaires: apport du scanner multicoupe.

Abnormalities in coronary artery origin and course are rare and often asymptomatic. However, visualizing them is of great interest because they can be responsible for iatrogenic trauma during cardiac surgery, and, for some of them, for myocardial infarction, which can lead to sudden death. We show the contribution of multislice CT in the positive diagnosis of these anatomic variations and in the differential diagnosis between benign and malignant forms, potentially responsible for myocardial ischemia.

[Radiologically-guided hysterosalpingography and tubal catheterization]. / Hystérosalpingograhie et cathétérisme tubaire sous contrôle radiologique.

Radiological exploration of hypofertility by means of hysterosalpingography is a method still valid but no longer sufficient. Tubal catheterization has thus become a necessary complementary exam in order to eliminate the pseudo-obturations of hysterosalpingography and, in case of proximal tubal obstructions, to obtain pregnancies. The conditions for the realization of hysteroalpingography and tubal catheterization are hereby described, with emphasis on the rigorous technique that is required together with careful listening to the patients.

[Large coronary aneurysm diagnosed during an acute coronary syndrome. A case report and review of the literature]. / Volumineux anévrisme coronaire découvert a l'occasion d'un syndrome coronaire aigu. A propos d'un cas et revue de la littérature.

Coronary aneurysm is an uncommon variant of coronary atherosclerosis. It usually involves the right coronary artery and is often associated with significant coronary stenosis. It may be revealed by an acute coronary syndrome (ACS). We report the case of a 49 year-old woman in whom a large coronary aneurysm of the left anterior descending artery was revealed by an ACS. Coronary angiography remains the gold standard diagnosis procedure, but spiral computed tomography may be of interest to specify the location and nature of the aneurysm, and thus guide surgical or percutaneous treatment.

Effects of corticosteroids and immunosuppressors on idiopathic inflammatory myopathy related myocarditis evaluated by magnetic resonance imaging.

BACKGROUND: Cardiac involvement in idiopathic inflammatory myopathy has been recognised as an important prognostic factor, but treatment remains empirical. OBJECTIVE: To investigate the effects of corticosteroids and immunosuppressors on myocarditis in patients with inflammatory myopathies. METHODS: Patients with inflammatory myositis of recent onset who had not received treatment were evaluated for associated myocarditis by magnetic resonance imaging (MRI) and reinvestigated after treatment with high dose corticosteroids and immunosuppressors. RESULTS: Four patients with histologically proven myositis were included. Two patients with polymyositis had cardiac clinical symptoms. Two other patients with dermatomyositis and diffuse cutaneous systemic sclerosis-polymyositis overlap syndrome were asymptomatic. In three cases the usual conventional screening tests were normal. For all patients an area of contrast enhancement and hypokinesia detected by cardiac MRI was markedly reduced after treatment with corticosteroids and immunosuppressors for 6 months. CONCLUSION: Treatment with intravenous methylprednisolone followed by prednisone and immunosuppressive therapy seems to be effective for treating myocardial involvement in patients with idiopathic inflammatory myopathies, either alone or presenting as overlap syndromes. Cardiovascular MRI is a non-invasive technique that may be a powerful tool for diagnosis and monitoring of myocardial inflammation in this setting.

Evaluation of the effect of nifedipine upon myocardial perfusion and contractility using cardiac magnetic resonance imaging and tissue Doppler echocardiography in systemic sclerosis.

BACKGROUND: Primary myocardial involvement due to microcirculation impairment is common in systemic sclerosis (SSc). Cardiovascular magnetic resonance imaging (MRI) and tissue Doppler echocardiography (TDE) were recently shown to be more sensitive than conventional methods for the respective assessment of myocardial perfusion and contractility. Previous studies have suggested that dihydropyridine-type calcium channel blockers mitigate both myocardial perfusion and function abnormalities. OBJECTIVE: To investigate the effects of nifedipine on myocardial perfusion by MRI and on contractility by TDE, in patients with SSc. PATIENTS AND METHODS: 18 patients with SSc without clinical heart failure and with normal pulmonary arterial pressure (14 women, 4 men; mean (SD) age 59 (9) years; mean (SD) disease duration 7 (4) years, 10 with diffuse and 8 with limited cutaneous forms) were prospectively evaluated. The MRI perfusion index, determined from time-intensity curves, and systolic and diastolic strain rate determined by TDE were assessed at baseline, after a 72 hour vasodilator washout period, and after 14 days of oral treatment with nifedipine 60 mg/day. RESULTS: Nifedipine treatment led to a significant increase in the MRI perfusion index (mean (SD) 0.26 (0.07) v 0.19 (0.05) at baseline, p = 0.0003) and in systolic and diastolic strain rate (2.3 (0.6) v 1.5 (0.4) s(-1) at baseline, p = 0.0002, and 4.2 (1.6) v 3.0 (1.2) at baseline, p = 0.0003, respectively). CONCLUSION: Fourteen days of treatment with nifedipine simultaneously improves myocardial perfusion and function, as evaluated by highly sensitive and quantitative methods.

Cardiac gas embolism after central venous catheter removal.

Clinical images reporting intracardiac gas level are sparce and, to our knowledge, the presence of gas embolism into the coronary arteries has never been reported. We describe the case of a young man who experiences life-threatening gas embolism with the presence of gas bubbles into cardiac cavities and coronary arteries.

[Multislice CT and MRI of coronary artery disease: current and future role]. / Maladie coronaire: les questions cliniques et les réponses potentielles en scanner multicoupe et en IRM.

If the role of multislice CT and MRI is not clearly defined in ischemic heart disease, these new cardiac imaging techniques are very promising due to recent technological advances. The detection of coronary artery plaque and stenosis, as well as coronary bypass graft and stenting patency, the evaluation of left ventricular remodeling after infarction, are available in routine with a 16-slices multislice CT. MRI is the technique of choice to appreciate the impact of a coronary artery lesion on myocardium: detection of ischemia by first-pass perfusion imaging at rest and under pharmacological stress, detection and extension of infarcted myocardium and assessment of myocardial viability. The potential indications will be discussed for each technique in the light of the main clinical questions asked by the cardiologist in daily practice.

[Value of MRI in the evaluation of congenital anomalies of the heart and great vessels]. / Apport de l'IRM dans l'exploration des anomalies cardiaques congénitales et des gros vaisseaux.

Management of congenital heart diseases (CHD) frequently is a diagnostic challenge. MRI, as a complement to echocardiography, plays an important role in the non-invasive evaluation of these anomalies. MRI allows high resolution anatomical evaluation of these structures in multiple planes as well as functional evaluation. These features are helpful to further characterize extra-cardiac anomalies that may be difficult to assess at US and even angiography. MRI is thus a valuable imaging tool in the evaluation of CHD.

[Cardiomyopathies]. / Cardiomyopathies.

Cardiomyopathies include a wide variety of cardiac diseases. The value of MR imaging is not only to provide information about cardiac function, but also to detect the underlying cause of the disease.