scholarly journals Myocardial Disease and Ventricullar Arrhythmia in Marfan Syndrome – A Prospective Study

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 ◽  
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.


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 ◽  
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.


2021 ◽  
Vol 5 (2) ◽  
Author(s):  
Stephan Stöbe ◽  
Bhupendar Tayal ◽  
Adrienn Tünnemann-Tarr ◽  
Andreas Hagendorff

Abstract Background The diagnosis of acute myocarditis (AM) is often challenging and mainly performed by cardiac magnetic resonance (CMR). Case summary The present case describes echocardiographic findings of a 38-year-old male patient with exertional dyspnoea probably due to myocardial involvement of previously undiagnosed acute human immunodeficiency virus (HIV) infection. Myocardial deformation imaging might be helpful to detect early stages of myocardial dysfunction in patients with AM and/or systemic infectious diseases by documentation of patchy abnormalities of longitudinal, circumferential and rotational left ventricular (LV) deformation. Discussion CMR still represents the gold standard to diagnose AM, which has been confirmed by myocardial oedema and hyperaemia in the present case. However, speckle tracking echocardiography seems to be useful to detect myocardial involvement in HIV infection by dynamic alterations of different components of LV deformation. This was documented by comparing echocardiographic findings at the acute stage of HIV infection to findings at follow-ups during antiviral treatment. The diagnostic option to detect myocardial involvement by deformation imaging in a patient with HIV infection is described for the first time.


Animals ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 2011
Author(s):  
Mehmet Ege Ince ◽  
Kursad Turgut ◽  
Amir Naseri

The purpose of this study was to monitor left ventricular systolic dysfunction (LVSD) and diastolic dysfunction (LVDD) using transthoracic echocardiography (TTE) in dogs with severe sepsis and septic shock (SS/SS). A prospective longitudinal study using 23 dogs with SS/SS (experimental group) and 20 healthy dogs (control group) were carried out. All the clinical, laboratory and cardiologic examinations for the experimental dogs were performed at admission, 6 and 24 h after the start of treatment and on the day of discharge. LVSD was described as LV ejection fraction (LVEF) < 50%. LVDD was determined when the septal mitral annulus early diastolic velocity (LVEm) was <8 cm/s. LVSD and LVDD were present in 3 and 16 dogs with SS/SS, respectively, with both types of dysfunction present in 2 of the dogs. Although all the dogs with LVSD survived, 8 dogs with LVDD did not. The survival period was significantly shorter in dogs with an LVEm < 8 cm/s (1.3 ± 1.4 days). In conclusion, LVDD, rather than LVSD, was a common cardiovascular abnormality in the septic dogs, and this may be a negative prognostic factor. TTE is a useful tool for the identifying and monitoring of myocardial dysfunction in the dogs with SS/SS.


2005 ◽  
Vol 6 (2) ◽  
pp. 27
Author(s):  
Dimitrios Buklas ◽  
Massimo Massetti ◽  
Eric Saloux ◽  
Eugenio Neri ◽  
Olivier LePage ◽  
...  

Several techniques are currently in use for mitral valve reconstruction. We report a mitral repair case in which the use of a combination of different surgical techniques resulted in the necessary correction. A 47-year-old woman underwent surgical intervention to treat severe mitral valve insufficiency due to A1/A2/A3 and P2 prolapsed valve tissue. A combination of quadrangular resection, sliding leaflet, single chordal transposition, "flip-over" leaflet, and ring annuloplasty techniques were applied, and postsurgical correct valve function was documented by results of a left ventricular saline filling test and transesophageal echocardiography control. Complex mitral valve repairing techniques can be combined to reestablish valvular function.


2018 ◽  
Vol 2 (S1) ◽  
pp. e000127
Author(s):  
Kushali Tanna ◽  
K M Mehariya ◽  
Suchita Munsi ◽  
Charul Pujani

Aims and Objectives: To study an incidence of myocardial dysfunction in neonates admitted with perinatal asphyxia, to find out its correlation with severity of birth asphyxia and its outcome. Methods: This prospective study was conducted among 40 term neonates admitted in NICU of Civil Hospital Ahmedabad who had suffered with perinatal asphyxia (defined by WHO ), resuscitated as per NRP guidelines-2015 including both intramural and extramural admissions and who developed to hypoxic ischemic encephalopathy as defined by Levene staging. Neonates with congenital heart diseases, major central nervous system malformations and neonatal sepsis were excluded. Myocardial involvement was assessed by clinical evaluation, ECG, Creatinine Kinase Total (25-200IU/L), CK-MB (0-25IU/L) and Troponin I (0-0.03ug/L) measurements. Results: Among 40 cases, 10(25%) neonates had moderate birth asphyxia while 30(75%) had severe birth asphyxia. Respiratory distress was observed in 34(77.5%), poor spontaneous respiration 4(10%),shock in 14(35%),CCF 19(47.5%) while ECG was abnormal in 30(76.7%). Serum levels of CPK Total, CPK- MB and Troponin I were raised in 34(85%), 32(80%) and 28 (70%) neonates, respectively.  Conclusion: There was a direct correlation between ECG changes and enzymatic levels which showed increasing abnormalities with increasing with severity of HIE.  


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