scholarly journals Myocardial Disease and Ventricular Arrhythmia in Marfan Syndrome – a prospective study

2020 ◽  
Author(s):  
Laura Muino Mosquera ◽  
Hans De Wilde ◽  
Daniel Devos ◽  
Danilo Babin ◽  
Luc Jordaens ◽  
...  

Abstract Background: Aortic root dilatation and -dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality.Methods: To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case-control study including 86 patients with MFS (55.8% women, mean age 36.3yr - range 13-70yr-) and 40 age- and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30±7months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N=55) and with previous surgery or valvular dysfunction (MFS-2; N=31).Results: Compared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles ((S)VES). Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT.Conclusion: Our study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Evaluation of myocardial function with echocardiography and AECG should be considered in all patients with MFS, especially in those with valvular disease and a history of cardiac surgery.

Author(s):  
Laura Muino Mosquera ◽  
Hans De Wilde ◽  
Daniel Devos ◽  
Danilo Babin ◽  
Luc Jordaens ◽  
...  

Abstract Background: Aortic root dilatation and -dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality. Methods: To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case-control study including 86 patients with MFS (55.8% women, mean age 36.3yr - range 13-70yr-) and 40 age- and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30±7months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N=55) and with previous surgery or valvular dysfunction (MFS-2; N=31). Results: Compared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles ((S)VES). Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT. Conclusion: Our study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Myocardial function should be evaluated in all patients with MFS, especially those with valvular disease and cardiac surgery.


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Laura Muiño-Mosquera ◽  
Hans De Wilde ◽  
Daniel Devos ◽  
Danilo Babin ◽  
Luc Jordaens ◽  
...  

Abstract Background Aortic root dilatation and—dissection and mitral valve prolapse are established cardiovascular manifestations in Marfan syndrome (MFS). Heart failure and arrhythmic sudden cardiac death have emerged as additional causes of morbidity and mortality. Methods To characterize myocardial dysfunction and arrhythmia in MFS we conducted a prospective longitudinal case–control study including 86 patients with MFS (55.8% women, mean age 36.3 yr—range 13–70 yr–) and 40 age—and sex-matched healthy controls. Cardiac ultrasound, resting and ambulatory ECG (AECG) and NT-proBNP measurements were performed in all subjects at baseline. Additionally, patients with MFS underwent 2 extra evaluations during 30 ± 7 months follow-up. To study primary versus secondary myocardial involvement, patients with MFS were divided in 2 groups: without previous surgery and normal/mild valvular function (MFS-1; N = 55) and with previous surgery or valvular dysfunction (MFS-2; N = 31). Results Compared to controls, patients in MFS-1 showed mild myocardial disease reflected in a larger left ventricular end-diastolic diameter (LVEDD), lower TAPSE and higher amount of (supra) ventricular extrasystoles [(S)VES]. Patients in MFS-2 were more severely affected. Seven patients (five in MFS-2) presented decreased LV ejection fraction. Twenty patients (twelve in MFS-2) had non-sustained ventricular tachycardia (NSVT) in at least one AECG. Larger LVEDD and higher amount of VES were independently associated with NSVT. Conclusion Our study shows mild but significant myocardial involvement in patients with MFS. Patients with previous surgery or valvular dysfunction are more severely affected. Evaluation of myocardial function with echocardiography and AECG should be considered in all patients with MFS, especially in those with valvular disease and a history of cardiac surgery.


2020 ◽  
Vol 4 (FI1) ◽  
pp. 1-6 ◽  
Author(s):  
Gianfranco Mitacchione ◽  
Marco Schiavone ◽  
Alessio Gasperetti ◽  
Giovanni B Forleo

Abstract Background Coronavirus disease 2019 (COVID-19) has been associated with myocardial involvement. Among cardiovascular manifestations, cardiac arrhythmias seem to be fairly common, although no specifics are reported in the literature. An increased risk of malignant ventricular arrhythmias and electrical storm (ES) has to be considered. Case summary We describe a 68-year-old patient with a previous history of coronary artery disease and severe left ventricular systolic disfunction, who presented to our emergency department describing cough, dizziness, fever, and shortness of breath. She was diagnosed with COVID-19 pneumonia, confirmed after three nasopharyngeal swabs. Ventricular tachycardia (VT) storm with multiple implantable cardioverter defibrillator (ICD) shocks was the presenting manifestation of cardiac involvement during the COVID-19 clinical course. A substrate-based VT catheter ablation procedure was successfully accomplished using a remote navigation system. The patient recovered from COVID-19 and did not experience further ICD interventions. Discussion To date, COVID-19 pneumonia associated with a VT storm as the main manifestation of cardiac involvement has never been reported. This case highlights the role of COVID-19 in precipitating ventricular arrhythmias in patients with ischaemic cardiomyopathy who were previously stable.


Heart ◽  
2021 ◽  
pp. heartjnl-2021-319418
Author(s):  
Upasana Tayal ◽  
John Gregson ◽  
Rachel Buchan ◽  
Nicola Whiffin ◽  
Brian P Halliday ◽  
...  

ObjectiveThe effect of moderate excess alcohol consumption is widely debated and has not been well defined in dilated cardiomyopathy (DCM). There is need for a greater evidence base to help advise patients. We sought to evaluate the effect of moderate excess alcohol consumption on cardiovascular structure, function and outcomes in DCM.MethodsProspective longitudinal observational cohort study. Patients with DCM (n=604) were evaluated for a history of moderate excess alcohol consumption (UK government guidelines; >14 units/week for women, >21 units/week for men) at cohort enrolment, had cardiovascular magnetic resonance and were followed up for the composite endpoint of cardiovascular death, heart failure and arrhythmic events. Patients meeting criteria for alcoholic cardiomyopathy were not recruited.ResultsDCM patients with a history of moderate excess alcohol consumption (n=98, 16%) had lower biventricular function and increased chamber dilatation of the left ventricle, right ventricle and left atrium, as well as increased left ventricular hypertrophy compared with patients without moderate alcohol consumption. They were more likely to be male (alcohol excess group: n=92, 94% vs n=306, 61%, p=<0.001). After adjustment for biological sex, moderate excess alcohol was not associated with adverse cardiac structure. There was no difference in midwall myocardial fibrosis between groups. Prior moderate excess alcohol consumption did not affect prognosis (HR 1.29, 95% CI 0.73 to 2.26, p=0.38) during median follow-up of 3.9 years.ConclusionDCM patients with moderate excess alcohol consumption have adverse cardiac structure and function at presentation, but this is largely due to biological sex. Alcohol may contribute to sex-specific phenotypic differences in DCM. These findings help to inform lifestyle discussions for patients with DCM.


2018 ◽  
Vol 22 (2) ◽  
Author(s):  
Małgorzata Ludzia ◽  
Radosław Pietrzak ◽  
Bożena Werner

Marfan syndrome is a systemic, autosomal dominant connective tissue disorder with variable expressivity. An early diagnosis is challenging but important, because Marfan syndrome is associated with premature death in untreated patients. The authors present a case of a 7-year-old girl with Marfan syndrome. The child’s father was diagnosed with Marfan syndrome confirmed by genetic tests. The first symptoms of Marfan syndrome in the presented patient occurred at the age of 2 years, when she presented with mitral and tricuspid valve prolapse, scoliosis, joint hypermobility and body height above 97 percentile. In regular check-ups, aortic root dilatation and the enlargement of the left ventricle were first described one year later. It was decided to introduce beta blocker therapy. Due to the further progression of left ventricular enlargement the girl was given additionally angiotensin II receptor antagonist. In echocardiography follow up no increasing of the aortic root dilatation and the left ventricular enlargement is observed.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1114.3-1115
Author(s):  
R. Dhahri ◽  
W. Lahmar ◽  
Y. Ben Abderrazek ◽  
M. Slouma ◽  
B. Louzir ◽  
...  

Background:Patients with rheumatoid arthritis are at increased risk for cardiovascular disease (CVD).The early myocardial dysfunction in RA patients may be detectable sooner using speckle-tracking echocariodgraphy to evaluate ventricular strain especially the global longitudinal strain (GLS), this has provided more comprehensive information on ventricular dysfunction in these patients.Objectives:In the present study, we evaluated comorbidities that interfered the most with the GLS in rheumatoid arthritis patients.Methods:The study population was comprised of a case group (36 patients with rheumatoid arthritis with no history of CVD and normal LVEF in the outpatient population of the Rheumatology department in the military hospital of Tunis) and a matched control group (individuals without a history of rheumatoid arthritis or cardiac abnormalities referred for clinical check-ups). In both groups, 2D and 3D echocardiographic examinations were performed by a single cardiologist to assess cardiac functional parameters.Results:Anemia (36%) was found to be the most common comorbidity followed by diabetes mellitus (25%), arterial hypertension (17%) and dyslipidemia (17%).Myocardial deformation study revealed that rheumatoid arthritis patients had a significantly worse global longitudinal strain than healthy controls (18.99±2.81% vs 20.42±1.33%; P=.015). Moreover, a third of the rheumatoid arthritis patients (and no healthy controls) exhibited subclinical left ventricular systolic dysfunction (GLS<18%).Anemia (r=−0.368, P=.027), Age (r=−0.365, P=.029), Diabetes mellitus (r=−0.540, P=.001) and E/A (r=0.351, P=.036) were significantly correlated with GLS in our univariate study. Receiver operating characteristic curve analysis revealed hemoglobin as the best predictor for subclinical LVSD (AUC=0.752, 95% CI: 0.577-0.927, P=.02) when compared to Age and E/A.Conclusion:This prospective comparative study highlighted the diabetes mellitus and anemia burden on myocardial dysfunction in RA patients.Disclosure of Interests:None declared


2021 ◽  
Author(s):  
Luiz Darcy Cortez Caiado ◽  
Nathalia Caiado de Azevedo ◽  
Rafael R. C. Azevedo ◽  
Brasil R. Caiado

Abstract Coronavirus disease-19 (COVID-19) has been associated with subclinical myocardial dysfunction during its acute phase and a recurring pattern of reduced basal left ventricular longitudinal strain on speckle-tracking echocardiography (STE) in hospitalized patients. But a question still remains unanswered: speckle tracking echocardiography might also be suitable to detect residual myocardial involvement after acute stage of COVID-19? Methods and results: We studied 100 patients recovered from COVID-19 with STE to evaluate global (GLS) and segmentar longitudinal strain (LS) and compared with a control group of 100 healthy individuals. STE was performed at a median of 130.35 +/- 76.06 days after COVID-19 diagnostic. Demographic and echocardiographic parameters are similar in both groups. Left ventricular ejection faction (LVEF) and GLS were normal in COVID-19 patients (66.20 +/- 1.98% and -19.51 +/- 2.87%, respectively). A reduction in mean LS for the basal segments was found in COVID-19 (16.48 +/- 5.41%) when compared to control group (19.09 +/- 4.31%) (p<0.001). Conclusion: The present study suggests that COVID-19 induced cardiac involvement could persist after recovery of the disease and may be detected by deformation abnormalities using STE. COVID-19 induced myocardial involvement often shows specific LV deformation patterns due to pronounced edema and/or myocardial damage in basal LV segments.


2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Ichrak Ben Abdallah ◽  
Sonia Ben Nasr ◽  
Chadia Chourabi ◽  
Marouane Boukhris ◽  
Israa Ben Abdallah ◽  
...  

Introduction. Although epirubicin has significantly improved outcome in breast cancer (BC) patients, it is responsible for myocardial dysfunction that affects patients’ quality of life. The use of 2D global longitudinal strain (GLS) has been reported to detect early myocardial dysfunction. The aim of this study was to evaluate how GLS changes can predict cardiotoxicity. Methods. We conducted a prospective study from March 2018 to March 2020 on 66 patients with no cardiovascular risk factors, who presented with BC and received epirubicin. We measured left ventricular ejection fraction (LVEF) and GLS before chemotherapy, at three months (T3), and at 12 months (T12) from the last epirubicin infusion. Chemotherapy-Related-Cardiac-Dysfunction (CTRCD) was defined as a decrease of 10% in LVEF to a value below 53% according to ASE and EACI 2014 expert consensus. Results. The mean age at diagnosis was 47 ± 9 years old. At baseline, median LVEF was 70% and median GLS was −21%. Shortly after chemotherapy completion, two patients presented with symptomatic heart failure while asymptomatic CTRCD was revealed in three other patients at T12. Three months after the last epirubicin infusion, median LVEF was 65%, median GLS was −19%, and median GLS variation was 5%. However, in patients who presented with subsequent CTRCD, median GLS at T3 was −16% and median GLS variation was 19% ( p = 0.002 and p < 0.001 , respectively, when compared to patients who did not develop cardiotoxicity). Persistent GLS decrease at T3 was an independent predictor of CTRCD at T12. Age and left-sided thoracic irradiation did not increase the risk of cardiotoxicity in our study while the cumulative dose of epirubicin significantly affected cardiologic findings ( p = 0.001 ). Conclusion. This was the first North African study that assesses the value of measuring GLS to early detect cardiotoxicity. Patients whose GLS remained decreased after 3 months from anthracyclines-base chemotherapy had an increased risk for developing subsequent CTRCD. Further studies with larger sample size are warranted to identify the best cardioprotective molecules to be initiated in these patients before LVEF declines.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C Kapelios ◽  
M Bonou ◽  
D Basoulis ◽  
C Masoura ◽  
E Athanasiadi ◽  
...  

Abstract Background Cardiomyopathy presenting in people living with HIV (PLWHIV) has been attributed to the effect of inflammation, opportunistic infections, myocyte invasion and cardiac steatosis, while peripheral artery disease (PAD) is linked to immune activation, abnormalities in lipid metabolism, and traditional risk factors. The diagnosis of subclinical myocardial dysfunction and PAD could enable prompt implementation of therapeutic measures. However, data available to date on the specific topic are limited. Μethods:We investigated the association between global longitudinal strain (GLS) and a) patient history, b) baseline characteristics, c) carotid intima-media thickness (IMT) and presence of carotid atheromatic plaque(s) d) temperature difference (ΔT) along each carotid artery, measured by microwave radiometry (MWR) and e) basic blood panel measurements, including high-sensitivity troponin-T(hsTnT) and NT-proBNP in PLWHIV and no history of cardiovascular disease. Results We prospectively enrolled forty asymptomatic PLWHIV on long-term highly active antiretroviral therapy. Thirty-seven (93%) were men, while mean age was 52 ± 13 years. Subclinical left ventricular systolic dysfunction(SLVSD), defined as a value of GLS&gt; -18.7%, was present in 35% of patients. GLS value was significantly associated with age (r = 0.410,P = 0.013), history of hyperlipidemia (r = 0.370,P = 0.026), body mass index (r = 0.462,P = 0.005), waist circumference (r = 0.471,P = 0.007) and right bulb IMT (r = 0.390,P = 0.036). hs-TnT levels were significantly associated with age (r = 0.513, P = 0.001), CD4 count (r=-0.357,P = 0.025), serum creatinine (r = 0.338,P = 0.035) and the presence of carotid plaque (r = 0.374,P = 0.038). NT-proBNP levels were significantly associated with history of diabetes (r = 0.336,P = 0.048) and serum creatinine (r = 0.548,P = 0.001). No significant associations were demonstrated between carotid ΔΤ and other parameters. Conclusion Our results indicate that apart from age, a dysmetabolic component, expressed by higher BMI and history of hyperlipidemia, may be implicated in the pathogenesis of SLVSD, which may lead to cardiomyopathy, in PLWHIV.


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