Cardiovascular magnetic resonance (CMR) in restrictive cardiomyopathies.

The restrictive cardiomyopathies constitute a heterogeneous group of myocardial diseases with a different pathogenesis and overlapping clinical presentations. Diagnosing them frequently poses a challenge. Echocardiography, electrocardiograms and laboratory tests may show non-specific changes. In this context, cardiac magnetic resonance (CMR) may play a crucial role in defining the diagnosis and guiding treatments, by offering a robust myocardial characterization based on the inherent magnetic properties of abnormal tissues, thus limiting the use of endomyocardial biopsy. In this review article, we explore the role of CMR in the assessment of a wide range of myocardial diseases causing restrictive patterns, from iron overload to cardiac amyloidosis, endomyocardial fibrosis or radiation-induced heart disease. Here, we emphasize the incremental value of novel relaxometric techniques such as T1 and T2 mapping, which may recognize different storage diseases based on the intrinsic magnetic properties of the accumulating metabolites, with or without the use of gadolinium-based contrast agents. We illustrate the importance of these CMR techniques and their great support when contrast media administration is contraindicated. Finally, we describe the useful role of cardiac computed tomography for diagnosis and management of restrictive cardiomyopathies when CMR is contraindicated.

Idiopathic intramural hematoma of the right colon. A case report and review of the literature.

INTRODUCTION: Intestinal hematoma is usually observed after an abdominal trauma or in patients treated with anticoagulant therapy. Conversely, idiopathic bowel hematoma is very rare, being colon involvement sporadic with few reports in the Literature. PRESENTATION OF CASE: The Authors report a case of idiopathic spontaneous large bowel hematoma. A 48-year-old man was admitted for a thoraco-abdominal pain and signs of acute abdomen and fever. After ruling out heart diseases, a CT scan revealed a marked thickening of the ascending colon wall, obstructing the bowel lumen. Leukocytosis was observed. In an emergency setting, an explorative laparotomy was performed. Hemoperitoneum and a large hematoma involving the caecum and the ascending colon were detected, together with intramesenteric and retroperitoneal blood effusion. A right hemicolectomy was accomplished. Histopathology confirmed the diagnosis of large bowel hematoma. Post-operative molecular diagnostic testing for coagulative disorders failed to demonstrate any genetic variation associate with hemorrhagic predisposition. In the post-operative course, the patient experienced a left basal bronco-pneumonia with increased unilateral pleural effusion, successfully treated by a thoracic drain and antibiotic therapy. DISCUSSION: The reported case and Literature data show that diagnosis of idiopathic colon intramural hematoma is challenging, especially in the emergency setting. Although conservative therapy is the first line treatment, surgery still has an important role when the diagnosis is uncertain, medical treatment fails or a complication, such untreatable bleeding, perforation or occlusion occur. CONCLUSION: The Authors report a very rare case of spontaneous intramural hematoma of the right colon. Surgery still has a role in selected cases.

Octreotide-LAR in later-stage autosomal dominant polycystic kidney disease (ALADIN 2): A randomized, double-blind, placebo-controlled, multicenter trial.

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent genetically determined renal disease. In affected patients, renal function may progressively decline up to end-stage renal disease (ESRD), and approximately 10% of those with ESRD are affected by ADPKD. The somatostatin analog octreotide long-acting release (octreotide-LAR) slows renal function deterioration in patients in early stages of the disease. We evaluated the renoprotective effect of octreotide-LAR in ADPKD patients at high risk of ESRD because of later-stage ADPKD. METHODS AND FINDINGS: We did an internally funded, parallel-group, double-blind, placebo-controlled phase III trial to assess octreotide-LAR in adults with ADPKD with glomerular filtration rate (GFR) 15-40 ml/min/1.73 m2. Participants were randomized to receive 2 intramuscular injections of 20 mg octreotide-LAR (n = 51) or 0.9% sodium chloride solution (placebo; n = 49) every 28 days for 3 years. Central randomization was 1:1 using a computerized list stratified by center and presence or absence of diabetes or proteinuria. Co-primary short- and long-term outcomes were 1-year total kidney volume (TKV) (computed tomography scan) growth and 3-year GFR (iohexol plasma clearance) decline. Analyses were by modified intention-to-treat. Patients were recruited from 4 Italian nephrology units between October 11, 2011, and March 20, 2014, and followed up to April 14, 2017. Baseline characteristics were similar between groups. Compared to placebo, octreotide-LAR reduced median (95% CI) TKV growth from baseline by 96.8 (10.8 to 182.7) ml at 1 year (p = 0.027) and 422.6 (150.3 to 695.0) ml at 3 years (p = 0.002). Reduction in the median (95% CI) rate of GFR decline (0.56 [-0.63 to 1.75] ml/min/1.73 m2 per year) was not significant (p = 0.295). TKV analyses were adjusted for age, sex, and baseline TKV. Over a median (IQR) 36 (24 to 37) months of follow-up, 9 patients on octreotide-LAR and 21 patients on placebo progressed to a doubling of serum creatinine or ESRD (composite endpoint) (hazard ratio [HR] [95% CI] adjusted for age, sex, baseline serum creatinine, and baseline TKV: 0.307 [0.127 to 0.742], p = 0.009). One composite endpoint was prevented for every 4 treated patients. Among 63 patients with chronic kidney disease (CKD) stage 4, 3 on octreotide-LAR and 8 on placebo progressed to ESRD (adjusted HR [95% CI]: 0.121 [0.017 to 0.866], p = 0.036). Three patients on placebo had a serious renal cyst rupture/infection and 1 patient had a serious urinary tract infection/obstruction, versus 1 patient on octreotide-LAR with a serious renal cyst infection. The main study limitation was the small sample size. CONCLUSIONS: In this study we observed that in later-stage ADPKD, octreotide-LAR slowed kidney growth and delayed progression to ESRD, in particular in CKD stage 4. TRIAL REGISTRATION: ClinicalTrials.gov NCT01377246; EudraCT: 2011-000138-12.

Left ventricular reverse remodeling after transcatheter aortic valve implantation: a cardiovascular magnetic resonance study.

BACKGROUND: In patients with severe aortic stenosis, left ventricular hypertrophy is associated with increased myocardial stiffness and dysfunction linked to cardiac morbidity and mortality. We aimed at systematically investigating the degree of left ventricular mass regression and changes in left ventricular function six months after transcatheter aortic valve implantation (TAVI) by cardiovascular magnetic resonance (CMR). METHODS: Left ventricular mass indexed to body surface area (LVMi), end diastolic volume indexed to body surface area (LVEDVi), left ventricular ejection fraction (LVEF) and stroke volume (SV) were investigated by CMR before and six months after TAVI in patients with severe aortic stenosis and contraindications for surgical aortic valve replacement. RESULTS: Twenty-sevent patients had paired CMR at baseline and at 6-month follow-up (N=27), with a mean age of 80.7±5.2 years. LVMi decreased from 84.5±25.2 g/m2 at baseline to 69.4±18.4 g/m2 at six months follow-up (P<0.001). LVEDVi (87.2±30.1 ml /m2vs 86.4±22.3 ml/m2; P=0.84), LVEF (61.5±14.5% vs 65.1±7.2%, P=0.08) and SV (89.2±22 ml vs 94.7±26.5 ml; P=0.25) did not change significantly. CONCLUSIONS: Based on CMR, significant left ventricular reverse remodeling occurs six months after TAVI.

A complex renal cyst: it is time to call the oncologist?

Introduction. Hydatid disease is a cyclozoonotic parasitic infestation caused by the cestode Echinococcus granulosus. The cysts mainly arise in the liver (50 to 70%) or lung (20 to 30%), but any other organ can be involved, in abdominal and pelvic locations, as well as in other less common sites, which may make both diagnosis and treatment more complex. Isolated renal involvement is extremely rare. Case Presentation. We report a rare case of isolated renal hydatid disease in a 71-year-old man with a history of vague abdominal pain, anemia, fever, and microhematuria. Ultrasonographic examination revealed a complex cyst in the right kidney, including multiple smaller cysts with internal echoes. A magnetic resonance scan of the abdomen confirmed the findings, and hydatid cyst disease was diagnosed. Right nephrectomy was performed, and microscopic examination confirmed the diagnosis of hydatid cyst. Albendazole, 10 mg/kg per day, was given for 4 weeks (2 weeks preoperatively and 2 weeks postoperatively). Conclusion. Isolated primary hydatidosis of the kidney should always be considered in the differential diagnosis of any cystic renal mass, even in the absence of accompanying involvement of liver or other visceral organs.

Prevalence of "high-riding" superior pericardial recesses on thin-section 16-MDCT scans.

OBJECTIVE: The aim of this study was to evaluate the prevalence of "high-riding" superior pericardial recess (HRSPR) on thin-section (1 mm) 16-multidetector computed tomography (MDCT) scans. MATERIALS AND METHODS: Three hundred and fourteen consecutive chest CT scans obtained with a thin-section 16 MDCT were retrospectively evaluated. The prevalence and characteristic of HRSPR were analyzed. RESULTS: HRSPR was depicted in 21 patients (11 men and 10 women) (6.6%) who ranged in age from 28 to 72 years (mean age, 57 years). The extended recesses were rounded/oval shaped in five patients and triangular, spindle, half moon or irregular shaped in the other 16 patients. CONCLUSION: Our data suggest as HRSPRs are more frequently and better depicted on thinsection MDCT scans, and this improves the capability to distinguish this superior extension of the superior aortic recess from abnormal findings such as lymphadenopathy, cystic lesions, and aortic dissection.