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1.
Clin Cardiol ; 41(4): 494-501, 2018 Apr.
Article in English | MEDLINE | ID: mdl-29663442

ABSTRACT

BACKGROUND: Late gadolinium enhancement (LGE) assessed with cardiovascular magnetic resonance (CMR) correlates with ventricular arrhythmias and survival in patients with structural heart disease. Whether some LGE characteristics may specifically improve prediction of arrhythmic outcomes is unknown. HYPOTHESIS: We sought to evaluate scar characteristics assessed with CMR to predict implantable cardioverter-defibrillator (ICD) interventions in dilated cardiomyopathy of different etiology. METHODS: 96 consecutive patients evaluated with CMR received an ICD. Biventricular volumes, ejection fraction, and myocardial LGE were evaluated. LGE was defined as "complex" (Cx-LGE) in presence of ≥1 of the following: ischemic pattern, involving ≥2 different coronary territories; epicardial pattern; global endocardial pattern; and presence of ≥2 different patterns. The primary endpoint was occurrence of any appropriate ICD intervention. A composite secondary endpoint of cardiovascular death, cardiac transplantation, or ventricular assist device implantation was also considered. RESULTS: During a median follow-up of 75 months, 30 and 25 patients reached the primary and secondary endpoints, respectively. Cx-LGE was correlated with a worse primary endpoint survival (log-rank P < 0.001). Cx-LGE and right ventricular end-diastolic volume were independently associated with the primary endpoint (HR: 3.22, 95% CI: 1.56-6.65, P = 0.002; and HR: 1.06, 95% CI: 1.00-1.12, P = 0.045, respectively), but not with the secondary endpoint. CONCLUSIONS: Cx-LGE identified at CMR imaging seems promising as an independent and specific prognostic factor of ventricular arrhythmias requiring ICD therapy in dilated cardiomyopathy of different etiologies.


Subject(s)
Arrhythmias, Cardiac/diagnostic imaging , Cardiomyopathy, Dilated/diagnostic imaging , Cicatrix/diagnostic imaging , Clinical Decision-Making , Defibrillators, Implantable , Electric Countershock/instrumentation , Magnetic Resonance Imaging, Cine , Adult , Aged , Aged, 80 and over , Arrhythmias, Cardiac/etiology , Arrhythmias, Cardiac/physiopathology , Arrhythmias, Cardiac/therapy , Cardiomyopathy, Dilated/etiology , Cardiomyopathy, Dilated/physiopathology , Cardiomyopathy, Dilated/therapy , Cicatrix/etiology , Cicatrix/physiopathology , Cicatrix/therapy , Contrast Media/administration & dosage , Death, Sudden, Cardiac/etiology , Death, Sudden, Cardiac/prevention & control , Disease Progression , Disease-Free Survival , Female , Gadolinium DTPA/administration & dosage , Humans , Male , Middle Aged , Patient Selection , Predictive Value of Tests , Recovery of Function , Registries , Retrospective Studies , Risk Factors , Time Factors , Treatment Outcome
2.
G Ital Cardiol (Rome) ; 19(2): 115-118, 2018 Feb.
Article in Italian | MEDLINE | ID: mdl-29531384

ABSTRACT

We report the case of a 56-year-old man showing numerous cardiovascular manifestations of Listeria infection (myo-pericarditis, coronary vasospasm, intracavitary vegetations/thrombus, sinoatrial blocks). Cardiac magnetic resonance and echocardiography images provided an unexpected picture of this infection, and were relevant for the appropriate clinical management. Transthoracic and transesophageal echocardiography were useful for assessing the presence of an endocavitary mass. Tissue characterization by cardiac magnetic resonance confirmed the presence of a thrombus and revealed signs of active inflammation in the myocardial wall, pericardium and vascular pedicle.


Subject(s)
Listeria monocytogenes/isolation & purification , Listeriosis/diagnostic imaging , Myocarditis/diagnostic imaging , Pericarditis/diagnostic imaging , Echocardiography, Transesophageal/methods , Humans , Listeriosis/pathology , Magnetic Resonance Imaging/methods , Male , Middle Aged , Myocarditis/microbiology , Pericarditis/microbiology , Thrombosis/diagnostic imaging
3.
PLoS One ; 13(2): e0192890, 2018.
Article in English | MEDLINE | ID: mdl-29466447

ABSTRACT

BACKGROUND: Iron overload-related heart failure is the principal cause of death in transfusion dependent patients, including those with Thalassemia Major. Linking cardiac siderosis measured by T2* to therapy improves outcomes. T1 mapping can also measure iron; preliminary data suggests it may have higher sensitivity for iron, particularly for early overload (the conventional cut-point for no iron by T2* is 20ms, but this is believed insensitive). We compared T1 mapping to T2* in cardiac iron overload. METHODS: In a prospectively large single centre study of 138 Thalassemia Major patients and 32 healthy controls, we compared T1 mapping to dark blood and bright blood T2* acquired at 1.5T. Linear regression analysis was used to assess the association of T2* and T1. A "moving window" approach was taken to understand the strength of the association at different levels of iron overload. RESULTS: The relationship between T2* (here dark blood) and T1 is described by a log-log linear regression, which can be split in three different slopes: 1) T2* low, <20ms, r2 = 0.92; 2) T2* = 20-30ms, r2 = 0.48; 3) T2*>30ms, weak relationship. All subjects with T2*<20ms had low T1; among those with T2*>20ms, 38% had low T1 with most of the subjects in the T2* range 20-30ms having a low T1. CONCLUSIONS: In established cardiac iron overload, T1 and T2* are concordant. However, in the 20-30ms T2* range, T1 mapping appears to detect iron. These data support previous suggestions that T1 detects missed iron in 1 out of 3 subjects with normal T2*, and that T1 mapping is complementary to T2*. The clinical significance of a low T1 with normal T2* should be further investigated.


Subject(s)
Blood/diagnostic imaging , Cardiac Imaging Techniques/methods , Iron Overload/diagnostic imaging , Magnetic Resonance Imaging/methods , beta-Thalassemia/diagnostic imaging , Adult , Female , Humans , Iron Overload/physiopathology , Linear Models , Male , Prospective Studies , beta-Thalassemia/physiopathology
4.
Eur Heart J Cardiovasc Imaging ; 19(6): 601-610, 2018 06 01.
Article in English | MEDLINE | ID: mdl-29309572

ABSTRACT

In patients with suspected or established hypertrophic cardiomyopathy (HCM), cardiovascular magnetic resonance (CMR) is widely employed for clinical management, given its multimodality approach capable of providing unique information on cardiac morphology, function, and tissue characterization. Guidance regarding all aspects of HCM diagnosis and management is provided by the comprehensive 2014 European Society of Cardiology (ESC) guidelines on HCM. CMR should be performed in centres with recognized expertise in heart muscle diseases, by physicians who are familiar with the whole HCM disease spectrum, differential diagnoses, and pitfalls. Because CMR is usually performed and interpreted by physicians not directly involved in patient care, detailed, bidirectional, and standardized communication becomes essential to obtain best results and avoid misinterpretation. In order to maximize the potential of CMR, it is of paramount importance that reporting physicians are provided with the essential clinical information and that, in turn, referring physicians are given a core set of CMR morphological, functional, and tissue characterization results following the test. This article aims to summarize the current knowledge on the role of CMR in managing HCM and, in addition, to review the importance of the clinical context in which the report is provided, in both adult and paediatric population, highlighting implications for clinical research.


Subject(s)
Cardiomyopathy, Hypertrophic/diagnostic imaging , Magnetic Resonance Imaging, Cine/methods , Myocardium/pathology , Radiographic Image Enhancement , Adult , Cardiomyopathy, Hypertrophic/pathology , Contrast Media , Echocardiography, Doppler/methods , Female , Gadolinium , Humans , Male , Middle Aged , Sensitivity and Specificity , Severity of Illness Index
5.
Circulation ; 136(6): 529-545, 2017 Aug 08.
Article in English | MEDLINE | ID: mdl-28576783

ABSTRACT

BACKGROUND: Previous reports have suggested that despite their dramatic presentation, patients with fulminant myocarditis (FM) might have better outcome than those with acute nonfulminant myocarditis (NFM). In this retrospective study, we report outcome and changes in left ventricular ejection fraction (LVEF) in a large cohort of patients with FM compared with patients with NFM. METHODS: The study population consists of 187 consecutive patients admitted between May 2001 and November 2016 with a diagnosis of acute myocarditis (onset of symptoms <1 month) of whom 55 required inotropes and/or mechanical circulatory support (FM) and the remaining 132 were hemodynamically stable (NFM). We also performed a subanalysis in 130 adult patients with acute viral myocarditis and viral prodrome within 2 weeks from the onset, which includes 34 with FM and 96 with NFM. Patients with giant-cell myocarditis, eosinophilic myocarditis, or cardiac sarcoidosis and those <15 years of age were excluded from the subanalysis. RESULTS: In the whole population (n=187), the rate of in-hospital death or heart transplantation was 25.5% versus 0% in FM versus NFM, respectively (P<0.0001). Long-term heart transplantation-free survival at 9 years was lower in FM than NFM (64.5% versus 100%, log-rank P<0.0001). Despite greater improvement in LVEF during hospitalization in FM versus NFM forms (median, 32% [interquartile range, 20%-40%] versus 3% [0%-10%], respectively; P<0.0001), the proportion of patients with LVEF <55% at last follow-up was higher in FM versus NFM (29% versus 9%; relative risk, 3.32; 95% confidence interval, 1.45-7.64, P=0.003). Similar results for survival and changes in LVEF in FM versus NFM were observed in the subgroup (n=130) with viral myocarditis. None of the patients with NFM and LVEF ≥55% at discharge had a significant decrease in LVEF at follow-up. CONCLUSIONS: Patients with FM have an increased mortality and need for heart transplantation compared with those with NFM. From a functional viewpoint, patients with FM have a more severely impaired LVEF at admission that, despite steep improvement during hospitalization, remains lower than that in patients with NFM at long-term follow-up. These findings also hold true when only the viral forms are considered and are different from previous studies showing better prognosis in FM.


Subject(s)
Myocarditis/diagnosis , Ventricular Function, Left/physiology , Acute Disease , Adolescent , Adult , Echocardiography , Female , Follow-Up Studies , Heart/diagnostic imaging , Heart Transplantation , Heart-Assist Devices , Hemodynamics , Hospital Mortality , Humans , Immunosuppressive Agents/therapeutic use , Kaplan-Meier Estimate , Magnetic Resonance Imaging, Cine , Male , Myocarditis/mortality , Myocarditis/therapy , Myocardium/pathology , Young Adult
6.
Intern Emerg Med ; 12(6): 799-809, 2017 Sep.
Article in English | MEDLINE | ID: mdl-28456904

ABSTRACT

Traditional echocardiography is unable to detect neither the early stages of iron overload cardiomyopathy nor myocardial iron deposition. The aim of the study is to determine myocardial systolic strain indices in thalassemia major (TM), and assess their relationship with T2*, a cardiac magnetic resonance index of the severity of cardiac iron overload. 55 TM cases with recent cardiac magnetic resonance (CMR-T2*) underwent speckle tracking analysis to assess regional myocardial strains and rotation. The results were compared with a normal control group (n = 20), and were subsequently analyzed on the basis of the CMR-T2* values. Two TM groups were studied: TM with significant cardiac iron overload ("low" T2*, ≤20 ms; n = 21), and TM with normal T2* values ("normal" T2*, >20 ms; n = 34). TM patients show significant, uniform decrease in circumferential and radial strain (P < 0.05), and a remarkable reduction in end-systolic rotation, both global, and for all segments (P < 0.001). No significant differences were found between the low- and the normal T2* group either in regional strains and rotation or in standard echocardiographic and CMR parameters. Spearman's correlation coefficient shows no significant correlation between myocardial strains, rotation and cardiac T2* values. In conclusion, our results are in accordance with recent evidence that myocardial iron overload is not the only mechanism underlying iron cardiomyopathy in TM. Strain imaging can predict subclinical myocardial dysfunction irrespective of CMR-T2* values, although it cannot replace CMR-T2* in assessing cardiac iron overload. Finally, it might be useful to appropriately time cardioactive treatment.


Subject(s)
Cardiomyopathies/diagnosis , Echocardiography/methods , Iron Overload/diagnosis , Population Surveillance/methods , Adult , Female , Humans , Magnetic Resonance Imaging/methods , beta-Thalassemia/diagnosis , beta-Thalassemia/epidemiology
7.
G Ital Cardiol (Rome) ; 18(2): 164-168, 2017 Feb.
Article in Italian | MEDLINE | ID: mdl-28398370

ABSTRACT

Pheochromocytoma is a rare tumor, usually benign, potentially lethal in case of crisis with acute release of catecholamines. The heart is a target and the clinical presentation can mimic various cardiac conditions, thus rendering diagnosis elusive. Cardiac magnetic resonance is a valuable non-invasive diagnostic tool for the evaluation of cardiomyopathies; it allows the identification of catecholamine-induced myocarditis pattern and, in some cases, it can detect the primary tumor. The definitive treatment of pheochromocytoma is surgical, while the acute crisis may require mechanical support to circulation. We here report a case of pheochromocytoma in a 25-year-old man complicated by catecholamine-induced myocarditis and heart failure.


Subject(s)
Adrenal Gland Neoplasms/metabolism , Catecholamines/adverse effects , Catecholamines/biosynthesis , Heart Failure/chemically induced , Myocarditis/chemically induced , Pheochromocytoma/metabolism , Adrenal Gland Neoplasms/complications , Adult , Humans , Male , Pheochromocytoma/complications
8.
J Cardiovasc Med (Hagerstown) ; 18(4): 244-248, 2017 Apr.
Article in English | MEDLINE | ID: mdl-27136700

ABSTRACT

BACKGROUND: Type 1 Gaucher disease (GD1) is the most common lysosomal disorder, characterized by the accumulation of beta-glucocerebroside into the macrophages of several organs. Cardiac involvement is rare and referred to as restrictive cardiomyopathy, pulmonary hypertension, and calcifications of the valves and the aortic arch. AIM: To assess the cardiovascular status by cardiac magnetic resonance, including evaluation of tissue characterization, in GD1 patients. METHODS: Nine GD1 patients were recruited at the Tertiary Care Centre for Rare Diseases at Ca' Granda Foundation IRCCS Hospital, Milan. The patients' records were available for a mean time of 6 ±â€Š3 years. Medical history of cardiac disease and cardiovascular risk factors were surveyed by direct interview. Patients were scanned with a 1.5 Avanto Siemens using a comprehensive cardiovascular evaluation protocol, including morphologic and functional sequences with gadolinium contrast media, to assess early and late enhancement (late gadolinium enhancement). Echocardiography was performed to study the cardiac morphology and function, including the measurement of pulmonary pressure. RESULTS: Three patients showed left atrial enlargement, one patient showed moderate aortic stenosis in bicuspid valve with mild aortic dilatation, and one patient showed moderate mitral regurgitation. No evidence of myocardial late gadolinium enhancement was detected after gadolinium contrast media. Seven patients received enzyme replacement therapy for a median of 1 year, and two patients were evaluated at diagnosis. CONCLUSION: Although cardiac disease in Gaucher disease is considered rare and associated with particular genotypes, we have found two valvular diseases and mild left atrial enlargement in three out of nine patients. Further studies to evaluate the prognostic value of these findings are warranted.


Subject(s)
Aortic Valve Stenosis/diagnostic imaging , Cardiomegaly/diagnostic imaging , Gaucher Disease/complications , Magnetic Resonance Imaging, Cine , Mitral Valve Insufficiency/diagnostic imaging , Adult , Aortic Valve/diagnostic imaging , Aortic Valve/physiopathology , Aortic Valve Stenosis/etiology , Aortic Valve Stenosis/physiopathology , Cardiomegaly/etiology , Cardiomegaly/physiopathology , Contrast Media/administration & dosage , Echocardiography , Enzyme Replacement Therapy , Female , Gaucher Disease/diagnosis , Gaucher Disease/drug therapy , Glucosylceramidase/therapeutic use , Humans , Italy , Male , Middle Aged , Mitral Valve/diagnostic imaging , Mitral Valve/physiopathology , Mitral Valve Insufficiency/etiology , Mitral Valve Insufficiency/physiopathology , Organometallic Compounds/administration & dosage , Predictive Value of Tests , Stroke Volume , Tertiary Care Centers , Time Factors , Treatment Outcome , Ventricular Function, Left , Ventricular Function, Right
9.
Eur Heart J Cardiovasc Imaging ; 18(2): 130-137, 2017 Feb.
Article in English | MEDLINE | ID: mdl-27625368

ABSTRACT

AIMS: The aim of the present study was to assess the association of the presence and amount of late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) with cardiovascular adverse events in patients with orthotopic heart transplantation (HTx). METHODS AND RESULTS: We enrolled 48 patients (mean age, 54.7 ± 14.6 years; 37 men) at various stages after HTx. All patients underwent standard CMR at 1.5 T, to characterize both cardiac anatomy and LGE. Late gadolinium enhancement was detected in 26 patients (54%). All-cause and cardiovascular mortalities, and a composite of major adverse cardiovascular events (MACE) recurrence were evaluated during the follow-up period for a median of 5.16 years. Ten patients (21%) died and 26 (54%) were readmitted because of MACE. Multivariate Cox analysis identified as independent predictors of MACE a diagnosis of cardiac allograft vasculopathy (CAV) (HR 3.63; 1.5-8.7 95% CI; P = 0.0039), left ventricular end systolic volume index (HR 1.04; 95% CI 1.01-1.079; P = 0.008), LGE mass (HR 1.04; 1.01-1.06 95% CI; P = 0.0007), LGE % of left ventricular mass (HR 1.083; 1.03-1.13 95% CI; P = 0.0002). Independent predictors of all-cause death were CAV (HR 6.33; 95% CI 1.33-30.03; P = 0.0201), LGE mass (HR 1.04; 1.01-1.07 95% CI; P = 0.005), LGE % of left ventricular mass (HR 1.075; 1.02-1.13 95% CI; P = 0.007). Patients with CAV had a risk of MACE by 5 years of 67% (95% CI 0.309-0.851%); the addition of 7.9 LGE % to the risk model increased the predicted risk to 88% (95% CI 0.572-0.967%). CONCLUSIONS: The current study demonstrated that the presence of CAV and the total amount of LGE have a significant independent association with MACE and mortality in HTx patients.


Subject(s)
Gadolinium DTPA , Heart Failure/mortality , Heart Failure/surgery , Heart Transplantation/methods , Magnetic Resonance Imaging, Cine/methods , Radiographic Image Enhancement/methods , Adult , Analysis of Variance , Cause of Death , Cohort Studies , Female , Graft Rejection , Graft Survival , Heart Failure/diagnostic imaging , Heart Transplantation/adverse effects , Humans , Kaplan-Meier Estimate , Magnetic Resonance Angiography/methods , Male , Middle Aged , Multivariate Analysis , Predictive Value of Tests , Prognosis , Proportional Hazards Models , Prospective Studies , Risk Adjustment , Statistics, Nonparametric , Survival Analysis , Time Factors
10.
Eur J Intern Med ; 39: 51-56, 2017 Apr.
Article in English | MEDLINE | ID: mdl-27727077

ABSTRACT

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis characterized by hypereosinophilia. EGPA typically develops in three clinical phases, beginning with asthma, followed by tissue eosinophilia and finally systemic vasculitis. Cardiac involvement is the most important predictor of mortality; it occurs in approximately 15-60% of EGPA patients, a significant proportion of whom are asymptomatic and have normal electrocardiogram (ECG) and echocardiogram. Early detection and management of cardiac disease could positevely affect prognosis. Cardiovascular magnetic resonance (CMR) has emerged as the gold standard cardiac imaging technique in the evaluation of cardiomyopathies, due to its ability to reliably assess anatomy, function, and tissue characterization. AIM: Purpose of this study was to assess the role of CMR in detecting cardiac disease in patients with EGPA in clinical remission. METHODS: A dedicated CMR protocol including functional analysis, and pre and post-contrast tissue characterization was performed in 11 patients with EGPA and the results were compared with 11 healthy subjects. RESULTS: EGPA patients had lower left ventricular ejection fraction compared to controls (56±19 vs 68.7±5.2, p value 0.02). Late gadolinium enhancement (LGE), representing replacement fibrosis, was positive in 9/11 (82%) patients, mainly with a non-ischemic pattern. In 3/11 (27%) patients a left ventricular thrombus was detected; in 3/11 (27%) patients myocardial edema was detected. CMR parameters of interstitial fibrosis were significantly more elevated in EGPA patients compared to controls. CONCLUSIONS: Patients with EGPA in clinical remission showed a high cardiovascular burden as demonstrated by lower EF, signs of active inflammation, presence of interstitial and replacement fibrosis and intraventricular thrombosis. Further studies on wider populations are warranted to better understand how these findings could impact on prognosis and eventually guide therapy.


Subject(s)
Churg-Strauss Syndrome/diagnostic imaging , Granulomatosis with Polyangiitis/diagnostic imaging , Heart/diagnostic imaging , Heart/physiopathology , Magnetic Resonance Imaging , Adult , Asthma/complications , Case-Control Studies , Eosinophilia/complications , Female , Humans , Male , Middle Aged , Prognosis , Ventricular Function, Left
11.
Int J Cardiol ; 231: 216-221, 2017 Mar 15.
Article in English | MEDLINE | ID: mdl-27913009

ABSTRACT

BACKGROUND: The presence of late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) has diagnostic and prognostic value in patients with acute myocarditis (AM). Aim of our study was to quantify the changes in LGE extension (LGE%) early after AM and evaluate its relations with biventricular function and morphology. METHODS: We investigated 76 consecutive patients with AM (acute onset of chest pain/heart failure/ventricular arrhythmias not explained by other causes, and raised troponin) that met CMR criteria based on myocardial oedema at T2-weighted images and LGE on post-contrast images at median time of 6days from onset of symptoms. We quantified LGE% at baseline and after 148days in 49 patients. RESULTS: Median left ventricular (LV)-ejection fraction (EF) was 64% (interquartile range [Q1-Q3]: 56-67%), and LGE% 9.4% (Q1-Q3: 7.5-13.2%). LGE% was correlated with LV end-systolic volume index (LV-ESVi; r=+0.34; p=0.003). LGE% was inversely correlated with LV-EF (r=-0.31; p=0.009) and time to CMR scan (r=-0.25; p=0.028). In the 49 patients with a second CMR scan, despite no significant variations in LV-EF, a significant decrease of LGE% was observed (p<0.0001) with a relative reduction of 42% compared with baseline. Patients showing increased LV-ESVi at follow up had a lower decrease of LGE% (p=0.038). CONCLUSIONS: In the acute phase of AM the LGE extension is a dynamic process that reflects impairment of LV function and is time dependent. LGE% appears one of the CMR parameters with the largest relative variations in the first months after AM.


Subject(s)
Heart Ventricles/diagnostic imaging , Magnetic Resonance Imaging, Cine/methods , Myocarditis/diagnosis , Organometallic Compounds/pharmacology , Acute Disease , Adult , Contrast Media/pharmacology , Disease Progression , Female , Follow-Up Studies , Gadolinium , Heart Ventricles/physiopathology , Humans , Male , Myocarditis/physiopathology , Myocardium/pathology , Prognosis , Reproducibility of Results , Retrospective Studies , Stroke Volume , Time Factors , Ventricular Function, Left/physiology
12.
Br J Haematol ; 176(2): 309-314, 2017 Jan.
Article in English | MEDLINE | ID: mdl-27766631

ABSTRACT

Although pulmonary function abnormalities in thalassaemia major (TM) were described in 1980, the pathogenetic mechanism is not clear and data are contradictory, probably because of study heterogeneity and the multifactorial nature of the pathogenesis. We retrospectively analysed 73 adult TM patients to evaluate the prevalence of pulmonary dysfunction in adult TM and investigate relationships with iron load. All patients underwent body plethysmography and carbon monoxide diffusion (DLCO) was assessed in 63, in addition to blood tests, echocardiogram and T2* myocardial and liver magnetic resonance imaging. Restrictive lung disease was present in 26 (35·6%) patients. Serum ferritin levels were higher in patients with restrictive pattern (1526 µg/l vs. 975 µg/l, P = 0·05). Restrictive lung disease did not correlate with cardiac or liver iron overload. However, considering only patients with serum ferritin >2500 µg/l, those with restrictive pattern also had heart (T2* 14·28 ± 9·99 ms vs. 31·59 ± 7·43 ms) and liver iron overload (LIC 16·02 ± 8·44 mg vs. 5·02 ± 2·69 mg Fe/g dry weight) compared to those without restrictive pattern. Twenty-five patients (39·7%) had decreased DLCO. No correlation was observed with iron parameters. In our data restrictive pattern was predominant; we observed a relationship with serum ferritin levels suggesting that iron, particularly its chronic effect, could play a role in the pathogenesis of pulmonary disease.


Subject(s)
Iron/metabolism , Lung Diseases/etiology , beta-Thalassemia/complications , Adult , Carbon Monoxide/blood , Female , Ferritins/blood , Humans , Iron Overload/complications , Male , Plethysmography, Whole Body , Prevalence , Retrospective Studies , beta-Thalassemia/diagnosis , beta-Thalassemia/epidemiology
13.
J Am Coll Cardiol ; 68(20): 2166-2181, 2016 11 15.
Article in English | MEDLINE | ID: mdl-27855806

ABSTRACT

BACKGROUND: Cardiac magnetic resonance (CMR) is useful for the diagnosis of left ventricular noncompaction (LVNC). However, there are limited data regarding its prognostic value. OBJECTIVES: The goal of this study was to evaluate the prognostic relevance of CMR findings in patients with LVNC. METHODS: A total of 113 patients with an echocardiographic diagnosis of LVNC underwent CMR at 5 referral centers. CMR diagnostic criterion of LVNC (noncompacted/compacted ratio >2.3 in end-diastole) was confirmed in all patients. We performed left ventricular (LV) and right ventricular quantitative analysis and late gadolinium enhancement (LGE) assessments and analyzed the following LVNC diagnostic criteria: left ventricular noncompacted myocardial mass (LV-ncMM) >20% and >25%, total LV-ncMM index >15 g/m2, noncompacted/compacted ratio ≥3:1 ≥1 of segments 1 to 3 and 7 to 16 or ≥2:1 in at least 1 of segments 4 to 6 of the American Heart Association model. Outcome was a composite of thromboembolic events, heart failure hospitalizations, ventricular arrhythmias, and cardiac death. RESULTS: At a mean follow-up of 48 ± 24 months, cardiac events (CEs) occurred in 36 patients (16 heart failure hospitalizations, 10 ventricular arrhythmias, 5 cardiac deaths, and 5 thromboembolic events). LV dilation, impaired LV ejection fraction, and LV-ncMM >20% was significantly more frequent in patients with CEs. LV fibrosis was detected by using LGE in 11 cases. CMR predictors of CEs were LV dilation and LGE. LGE was associated with improved prediction of CEs, compared with clinical data and CMR functional parameters in all 3 models. No CEs occurred in patients without dilated cardiomyopathy and/or LGE. CONCLUSIONS: In patients with LVNC evaluated by using CMR, the degree of LV trabeculation seems to have no prognostic impact over and above LV dilation, LV systolic dysfunction, and presence of LGE.


Subject(s)
Heart Ventricles/diagnostic imaging , Isolated Noncompaction of the Ventricular Myocardium/diagnosis , Magnetic Resonance Imaging, Cine/methods , Adult , Disease Progression , Echocardiography , Female , Follow-Up Studies , Humans , Male , Middle Aged , Prognosis , Prospective Studies , Reproducibility of Results , Time Factors
16.
Congenit Heart Dis ; 11(4): 365-8, 2016 Jul.
Article in English | MEDLINE | ID: mdl-27237845

ABSTRACT

INTRODUCTION: Partial anomalous pulmonary venous return (PAPVR) is an uncommon cause of right ventricular dilation. It may be difficult to identify and often remains undiagnosed. METHODS: We reviewed the database of the Cardiac Magnetic Resonance (CMR) Laboratory of Niguarda Hospital, in order to identify the cases of PAPVR between 2008 and 2014. RESULTS: On a total number of 7832 CMR scans, we identified 24 patients with PAPVR (14 male, age 41 ± 18 y) corresponding to 0.31% of the total population. Only 30% of patients had been referred for known or suspected PAPVR, 33% of patients had been referred for suspected right ventricular arrhythmogenic dysplasia and 37% had been referred for other cardiac disease. PAPVR involved mainly the right pulmonary veins (18 patients, 75%) and in 62% of our cases was associated with an atrial septal defect. Eight patients underwent corrective surgery in our institution, which confirmed and successfully repaired the anomalies. CONCLUSIONS: PAPVR is a rare congenital cardiac pathology which should be suspected in case of unexplained right chambers enlargement. CMR imaging allows an accurate anatomic and functional definition of this pathology and associated abnormalities. Early correction has an excellent prognosis and prevents long term complications like pulmonary hypertension, right ventricular failure and atrial fibrillation.


Subject(s)
Heart Defects, Congenital/complications , Hypertrophy, Right Ventricular/etiology , Pulmonary Veins/abnormalities , Adult , Cardiac Surgical Procedures , Databases, Factual , Female , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/physiopathology , Heart Defects, Congenital/surgery , Humans , Hypertrophy, Right Ventricular/diagnostic imaging , Hypertrophy, Right Ventricular/physiopathology , Italy , Magnetic Resonance Imaging , Male , Middle Aged , Predictive Value of Tests , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/physiopathology , Pulmonary Veins/surgery , Retrospective Studies , Treatment Outcome
17.
J Cardiovasc Med (Hagerstown) ; 17 Suppl 2: e109-e111, 2016 Dec.
Article in English | MEDLINE | ID: mdl-26556445

ABSTRACT

: Necrotizing eosinophilic myocarditis (NEM) is a life-threatening condition that needs rapid diagnosis by endomyocardial biopsy and hemodynamic support usually by mechanical circulatory systems. We present the case of a 25-year-old Caucasian man who developed a refractory cardiogenic shock due to a NEM that was supported with a peripheral veno-arterial extracorporeal membrane oxygenation associated with intravenous steroids and recovered after 2 weeks. Further instrumental investigations lead to the final diagnosis of NEM as first presentation of eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome), remarking the importance of identifying the systemic disorder that usually triggers the eosinophilic damage of the myocardium.


Subject(s)
Churg-Strauss Syndrome/complications , Granulomatosis with Polyangiitis/complications , Myocarditis/etiology , Shock, Cardiogenic/etiology , Administration, Intravenous , Adult , Biopsy , Churg-Strauss Syndrome/diagnosis , Churg-Strauss Syndrome/therapy , Extracorporeal Membrane Oxygenation , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/therapy , Humans , Immunosuppressive Agents/administration & dosage , Magnetic Resonance Imaging , Male , Myocarditis/diagnosis , Myocarditis/therapy , Myocardium/pathology , Necrosis , Shock, Cardiogenic/diagnosis , Shock, Cardiogenic/therapy , Steroids/administration & dosage , Time Factors , Treatment Outcome
18.
J Cardiovasc Med (Hagerstown) ; 17 Suppl 2: e151-e153, 2016 Dec.
Article in English | MEDLINE | ID: mdl-25643196

ABSTRACT

: A 31-year-old man presenting with cardiogenic shock and left ventricular ejection fraction of 10% received the diagnosis of giant cell myocarditis by endomyocardial biopsy. The patient was successfully treated with high-dose inotropes, intra-aortic balloon pump and venoarterial extracorporeal membrane oxygenation for 21 days associated with combined immunosuppression (thymoglobulin, steroids, cyclosporine). Immunosuppression including thymoglobulin is the regimen associated with the highest probability of recovery in case of giant cell myocarditis. Immunosuppression needs time to be effective; thus, hemodynamic support must be guaranteed. In the present case, we observed that full recovery can be obtained up to 21 days of support with extracorporeal membrane oxygenation and adequate immunosuppression.


Subject(s)
Antilymphocyte Serum/administration & dosage , Extracorporeal Membrane Oxygenation , Giant Cells/drug effects , Immunosuppressive Agents/administration & dosage , Myocarditis/therapy , Shock, Cardiogenic/therapy , Adult , Biopsy , Cardiotonic Agents/therapeutic use , Combined Modality Therapy , Giant Cells/immunology , Giant Cells/pathology , Humans , Intra-Aortic Balloon Pumping , Magnetic Resonance Imaging , Male , Myocarditis/diagnosis , Myocarditis/immunology , Myocarditis/physiopathology , Recovery of Function , Shock, Cardiogenic/diagnosis , Shock, Cardiogenic/immunology , Time Factors , Treatment Outcome
19.
G Ital Cardiol (Rome) ; 16(7-8): 437-41, 2015.
Article in Italian | MEDLINE | ID: mdl-26228614

ABSTRACT

Coronary artery disease is a rare entity in young patients and accurate assessment of its prevalence is difficult. Although coronary artery disease is frequently a silent process, it may also acutely present with myocardial infarction (MI). One of the most feared complications of MI is left ventricular thrombus formation. Transthoracic echocardiography is recommended for all patients with MI, and cardiac magnetic resonance should be considered because of its higher sensitivity if thrombus cannot clearly be demonstrated. The optimal treatment is based on anticoagulant therapy that should be started early and maintained for 3-4 months after the index event. We report the case of a 35-year-old male patient with anterior MI, complicated by left ventricular thrombus formation, extensive edema, microvascular obstruction and hemorrhagic core of the apical septum on cardiac magnetic resonance assessment.


Subject(s)
Anterior Wall Myocardial Infarction/therapy , Heart Ventricles/pathology , Thrombosis/pathology , Adult , Anterior Wall Myocardial Infarction/physiopathology , Anticoagulants/therapeutic use , Echocardiography , Edema/etiology , Edema/pathology , Humans , Magnetic Resonance Imaging , Male , Thrombosis/complications , Thrombosis/diagnosis
20.
Int J Cardiol ; 199: 326-32, 2015 Nov 15.
Article in English | MEDLINE | ID: mdl-26241638

ABSTRACT

BACKGROUND: Hereditary hemochromatosis, thalassemia and myelodysplastic syndromes represent disease models with evidence of iron-related heart failure. Non-Transferrin Bound Iron (NTBI) induces cardiac toxicity through the production of reactive oxygen species and lipid peroxidation. In ST-elevation acute myocardial infarction (STEMI) with evidence of microvascular obstruction (MVO) and hemorrhage (HEM), HEM may be a source of iron-related cardiac toxicity through NTBI and pro-inflammatory mediators. AIM OF THE STUDY: The study aims to assess NTBI in patients with STEMI and its possible relationship with MVO and HEM. METHODS AND RESULTS: NTBI, LPO-Malondialdehyde (MDA) and interleukin-6 (IL-6) were assessed in 15 patients with STEMI immediately before primary percutaneous coronary intervention (PPCI) and at 3, 6, 9, 12, and 24h post-PPCI. Cardiac Magnetic Resonance (CMR) was performed at 5days and 6months after STEMI. Myocardial edema and HEM were assessed by T2 and T2* mapping. MVO and necrotic area were assessed by early and late gadolinium enhancement (LGE). NTBI was detected in 13/15 patients with the highest values in 4 patients with evidence of MVO and HEM. NTBI levels were significantly related to CK-MB and troponin T values. NTBI kinetics appeared to be different in patients with MVO and HEM (7/15 patients), with a peak value at 6h after PCI, in comparison with those with no evidence of MVO and HEM, in whom NTBI values were lower and remained indeterminable after the first 24h. CONCLUSIONS: The detection of elevated NTBI values in patients with STEMI, MVO and HEM suggests a possible role of iron cardiotoxicity in myocardial damage.


Subject(s)
Iron/metabolism , Magnetic Resonance Imaging/methods , Myocardial Infarction/metabolism , Transferrins/metabolism , Creatine Kinase, MB Form/metabolism , Female , Hemorrhage/metabolism , Humans , Interleukin-6/metabolism , Iron/blood , Male , Malondialdehyde/metabolism , Microcirculation/physiology , Microvessels/pathology , Middle Aged , Myocardial Infarction/blood , Myocardial Infarction/pathology , Myocardial Infarction/therapy , Myocarditis/metabolism , Myocarditis/pathology , Myocardium/metabolism , Myocardium/pathology , Percutaneous Coronary Intervention , Prospective Studies , Reperfusion Injury/physiopathology , Transferrins/blood , Troponin T/blood , Troponin T/metabolism , Ventricular Remodeling
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