650The arrhythmic mitral valve prolapse: presentation and outcome

EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
A Sabbag ◽  
B Essayagh ◽  
C Antoine ◽  
G Benfari ◽  
J Malouf ◽  
...  

Abstract Background The majority of patients with of Mitral-valve-prolapse (MVP) have a excellent prognosis. Until recently most cases of mortality were thought to be related to mitral regurgitation and left ventricular dysfunction. The concept of the arrhythmic MVP emerged to describe cases of sudden cardiac death (SCD) in the presence of isolated MVP yet it’s phonotype remains incompletely and inconsistently defined. Purpose To analyze the prevalence, severity and characteristics of ventricular-arrhythmia (VA), to determine it’s phenotypical context and independent impact on outcome in patients with MVP. Methods A cohort of 595 (65 ± 16 years, 278 female) consecutive patients with MVP and comprehensive clinical, arrhythmia (24hour-Holter) and Doppler-echocardiographic characterization, was identified and long-term outcome analyzed. Results VA was frequent, present in 43% of patients with at least ventricular ectopy≥5%, but was most often moderate (ventricular-tachycardia—VT 120-179bpm) in 27% and rarely severe (VT≥180/min) in 8.6%.  Presence of VA was associated with older age, male sex, bileaflet-prolapse, marked leaflet redundancy, mitral-annulus-disjunction (MAD), larger left-atrium and left ventricular end-systolic diameter, and T-wave-inversion/ST-depression (all P ≤ 0.001).  Severe VA was independently associated with presence of MAD, leaflet-redundancy and T-wave-inversion/ST-depression (all P < 0.0001) but not with mitral regurgitation severity or ejection-fraction.  Outcome primary endpoint of overall survival after arrhythmia diagnosis (8-year 87 ± 2%) was strongly associated with arrhythmia-severity (8-year 90 ± 2% for no/trivial arrhythmia, 85 ± 3% for mild/moderate and 76 ± 7% for severe arrhythmia. P = 0.02, Figure). Excess-mortality was substantial for severe-arrhythmia (univariate-hazard-ratio 2.70[1.27-5.77], P = 0.01 vs. no/trivial arrhythmia), even adjusted comprehensively including for MVP-characteristics (adjusted-hazard-ratio 2.94[1.36-6.36], P = 0.006) ).  Conclusions This large cohort of isolated consecutive MVP characterized with 24-hour-Holter monitoring, clinical and Echocardiographic assessment, demonstrates that VA are frequent with MVP but rarely severe. The arrhythmic MVP was independently and strongly associated with specific ECG and morphologic patterns, particularly ST-T changes, MAD presence and marked leaflet redundancy, suggestive of a specific arrhythmic MVP phenotype, independently of MR-severity. Arrhythmia, particularly severe, is associated with long-term excess-mortality, independently of any other characteristics, including MR severity and LVEF. These findings lay the foundation for novel risk-stratification of MVP for the conduct of prospective controlled studies evaluating the management of MVP high-risk patients. Figure – Impact on survival of ventricular arrhythmia Overall survival of MVP stratified by ventricular arrhythmia (Panel A) or ventricular arrhythmia severity (Panel B) throughout follow-up. Abstract Figure.

Perfusion ◽  
2017 ◽  
Vol 33 (2) ◽  
pp. 115-122
Author(s):  
Thach Nguyen ◽  
Hoang Do ◽  
Tri Pham ◽  
Loc T Vu ◽  
Marco Zuin ◽  
...  

Background: New onset of heart failure (HF) is an indication for the assessment of coronary artery disease. The aim of this study was to clarify the mechanistic causes of new onset HF associated with ischemic electrocardiograph (EKG) changes and chest pain in patients with patent or minimally diseased coronary arteries. Methods: Twenty consecutive patients (Group A) were retrospectively reviewed if they had an history of new onset of HF, chest pain, electrocardiographic changes indicating ischemia (ST depression or T wave inversion in at least two consecutive leads and a negative coronary angiogram [CA]) and did not require percutaneous coronary intervention or coronary artery bypass grafting. A 1:1 matched cohort (Group B) was adopted to validate the results. Results: All patients had a negative CA. The majority of subjects in Group A had a higher left ventricular end diastolic pressure (LVEDP) when compared to the control group (p<0.05). Similarly, the aortic diastolic (AOD) pressure was lower in Group A than in Group B (p<0.05). In patients with elevated LVEDP and low AOD, with a coronary perfusion pressure (CPP) <20 mmHg, deep T wave inversion in two consecutive leads were more frequently observed. When the CPP was between 20-30 mmHg, a mild ST depression were more frequently recorded (p<0.05). Conversely, when the CPP was >30 mmHg, only mild non-specific ST-T changes or normal EKG were observed. Conclusions: In patients with HF and EKG changes suggestive of ischemia in at least two consecutive leads, a lower AOD could aggravate ischemia in patients with elevated left ventricular end diastolic pressure.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
B Essayagh ◽  
A Sabbag ◽  
C Antoine ◽  
G Benfari ◽  
J Maalouf ◽  
...  

Abstract Background Mitral-annulus-disjunction (MAD), often described in mitral-valve-prolapse (MVP) patients, remains incompletely defined and its clinical outcome consequences are unknown. Objectives To assess MAD prevalence, clinical and echocardiographic characteristics, and independent impact on outcome in patients with isolated-MVP. Methods A cohort of 595 (65±16 years, 278 female) consecutive patients with MVP and comprehensive clinical, rhythmic and Doppler-echocardiographic characterization, was identified. Primary-endpoint was overall-survival, or occurrence of ventricular-arrhythmia. To eliminate clinical differences, we matched MAD-patients 1:1 with no-MAD based on propensity score for overall survival analysis using a greedy 5-to-1 digit-matching algorithm. Results MAD was frequent, present in 186 (31%) of MVP-patients and independently associated to bileaflet-MVP, leaflet-redundancy, younger-age, female-sex, enlarged left-ventricle-endsystolic-diameter and higher left-ventricle-ejection-fraction (all P≤0.04). Outcome primary-endpoint of survival after MVP diagnosis in matched-cohort (10-year 95±1%) was not associated with MAD presence (10-year 93±2% for no-MAD and 97±1% for MAD, P=0.4). Mortality was comparable for MAD (univariate-hazard-ratio 0.64 [0.25–1.65], P=0.4 vs. no-MAD), even adjusted-comprehensively including for MVP-characteristics (adjusted-hazard-ratio 1.17 [0.39–3.50], P=0.8). However, 220 (51%) MVP-patients were diagnosed ventricular-arrhythmia (161 with ventricular-tachycardia only) and long-term, MAD was associated with lower arrhythmia- and ventricular-tachycardia- overall freedom (adjusted-hazard-ratio for MAD 2.35 [1.75–3.15], P&lt;0.0001 vs. no-MAD and 2.71 [1.93–3.82], P&lt;0.0001 respectively). Hence, 10-year freedom of significant ventricular-arrhythmia in overall-cohort was severely reduced with MAD (41±4% vs. 62±3% for no-MAD). Conclusion In this unique MVP cohort, with arrhythmia and MAD comprehensively-characterized, MAD presence was frequent and independently associated to bileaflet-MVP and leaflet-redundancy. Long-term, MAD compared to no-MAD is not independently associated with higher-mortality but to reduced arrhythmia-freedom occurrence. Survival overall and by V arrhythmia Funding Acknowledgement Type of funding source: Foundation. Main funding source(s): Mayo Foundation


2010 ◽  
Vol 31 (6) ◽  
pp. 881-883 ◽  
Author(s):  
Wei Wang ◽  
Weihua Zhu ◽  
Yujia Wang ◽  
Jianhua Li ◽  
Fangqi Gong

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Anastasios Athanasiadis ◽  
Birke Schneider ◽  
Johannes Schwab ◽  
Uta Gottwald ◽  
Ellen Hoffmann ◽  
...  

Background : The German tako-tsubo cardiomyopathy (TTC) registry has been initiated to further evaluate this syndrome in a western population. We aimed to assess different patterns of left ventricular involvement in TTC. Methods : Inclusion criteria were: 1) acute chest symptoms, 2) reversible ECG changes (ST-segment elevation±T-wave inversion), 3) reversible left ventricular dysfunction with a wall motion abnormality not corresponding to a single coronary artery territory, 4) no significant coronary artery stenoses. Results : A total of 258 patients (pts) from 33 centers were included with a mean age of 68±12 years. Left ventriculography revealed the typical pattern of apical ballooning in 170 pts (66%) and an atypical mid-ventricular ballooning with normal wall motion of the apical and basal segments in 88 pts (34%). Mean age (68±11 vs 67±13 years) and gender distribution (150 women/20 men vs 80 women/8 men) were similar in both groups. Triggering events were present in 78% of the pts with apical ballooning (35% emotional, 34 physical and 9% combination) and in 75% of the pts with mid-ventricular ballooning (39% emotional, 25% physical and 11% combination). As assessed by left ventriculography, ejection fraction was significantly lower in pts with mid-ventricular ballooning (50±15% vs 45±13%, p=0.006). There was no difference in right ventricular involvement. Creatine kinase and troponin I were comparable in both groups. The ECG on admission showed ST-segment elevation in 87% of pts with apical ballooning and in 78% of pts with mid-ventricular ballooning. T-wave inversion was seen in 70% of the pts irrespective of the TTC variant. A Q-wave was significantly less present in pts with mid-ventricular ballooning (30% vs 16%, p=0.04). The QTc interval during the first 3 days was not different among both groups. Conclusion : A variant form with mid-ventricular ballooning was observed in one third of the pts with TTC. Left ventricular ejection fraction was significantly lower in these pts, although they revealed significantly less Q-waves on the admission ECG. All other parameters were similar and confirm the concept that apical and mid-ventricular ballooning represent two different manifestations of the same syndrome.


Author(s):  
Anne-Laure Constant Dit Beaufils ◽  
Olivier Huttin ◽  
Antoine Jobbe-Duval ◽  
Thomas Senage ◽  
Laura Filippetti ◽  
...  

Background: Mitral valve prolapse (MVP) is a frequent disease that can be complicated by mitral regurgitation (MR), heart failure, arterial embolism, rhythm disorders and death. Left ventricular (LV) replacement myocardial fibrosis, a marker of maladaptive remodeling, has been described in patients with MVP, but the implications of this finding remain scarcely explored. We aimed at assessing the prevalence, pathophysiological and prognostic significance of LV replacement myocardial fibrosis through late gadolinium enhancement (LGE) by cardiac magnetic resonance (CMR) in patients with MVP. Methods: Four hundred patients (53±15 years, 55% male) with MVP (trace to severe MR by echocardiography) from 2 centers, who underwent a comprehensive echocardiography and LGE CMR, were included. Correlates of replacement myocardial fibrosis (LGE+), influence of MR degree, and ventricular arrhythmia were assessed. The primary outcome was a composite of cardiovascular events (cardiac death, heart failure, new-onset atrial fibrillation, arterial embolism, and life-threatening ventricular arrhythmia). Results: Replacement myocardial fibrosis (LGE+) was observed in 110 patients (28%; 91 myocardial wall including 71 basal inferolateral wall, 29 papillary muscle). LGE+ prevalence was 13% in trace-mild MR, 28% in moderate and 37% in severe MR, and was associated with specific features of mitral valve apparatus, more dilated LV and more frequent ventricular arrhythmias (45 vs 26%, P<0.0001). In trace-mild MR, despite the absence of significant volume overload, abnormal LV dilatation was observed in 16% of patients and ventricular arrhythmia in 25%. Correlates of LGE+ in multivariable analysis were LV mass (OR 1.01, 95% CI [1.002-1.017], P=0.009) and moderate-severe MR (OR: 2.28, 95% CI [1.21-4.31], P=0.011). LGE+ was associated with worse 4-year cardiovascular event-free survival (49.6±11.7 in LGE+ vs 73.3±6.5% in LGE-, P<0.0001). In a stepwise multivariable Cox model, MR volume and LGE+ (HR: 2.6 [1.4-4.9], P=0.002) were associated with poor outcome. Conclusions: LV replacement myocardial fibrosis is frequent in patients with MVP, is associated with mitral valve apparatus alteration, more dilated LV, MR grade, ventricular arrhythmia, and is independently associated with cardiovascular events. These findings suggest a MVP-related myocardial disease. Finally, CMR provides additional information to echocardiography in MVP.


Kardiologiia ◽  
2020 ◽  
Vol 60 (5) ◽  
pp. 136-145
Author(s):  
I. R. Tsoy ◽  
I. P. Kolos

The T-wave inversion (TWI) is a common electrocardiographic finding. Causes for TWI are numerous and sometimes TWI may appear in life-threatening conditions. The aim of this review was to provide an up-to-date analysis of TWI, including i) definition and prevalence; ii) causes, and iii) differential diagnosis in benign TWI, reversible myocardial ischemia of the left ventricular anterior wall; takotsubo cardiomyopathy; subarachnoid hemorrhage; pulmonary embolism; right ventricular arrhythmogenic cardiomyopathy; and “cardiac memory”. The review presents practical electrophysiological criteria, which allow suspecting in time a life-threatening condition to choose an up-to-date treatment.


2021 ◽  
Vol 14 (1) ◽  
pp. e239297
Author(s):  
H Ravi Ramamurthy ◽  
Onkar Auti ◽  
Vimal Raj ◽  
Kiran Viralam

A 16-month-old, healthy, asymptomatic male child presented with a diagnosis of dilated cardiomyopathy. Cardiovascular examination and chest radiograph were normal. ECG revealed sinus rhythm, and the augmented vector left lead showed raised ST segment, T wave inversion and q waves. Echocardiography showed a globular left ventricle with notched cardiac apex, abnormal echogenicity in the left ventricular apical myocardium, single papillary muscle and normal biventricular function. Cardiac MRI scan revealed a globular left ventricle with fibrofatty changes and retraction of the apex, the papillary muscles closely approximated, and the right ventricle wrapping around the apex of the left ventricle. This is described as isolated left ventricular apical hypoplasia. Diagnosis of this rare entity can be made by MRI, and it has been diagnosed largely in adults. The pathophysiology and long-term outcomes are unknown. We characterise the echocardiography findings of this rare anomaly in a child for the first time in the literature.


ESC CardioMed ◽  
2018 ◽  
pp. 1298-1301
Author(s):  
Federico Migliore ◽  
Sebastiano Gili ◽  
Domenico Corrado

Takotsubo syndrome (TTS) is typically characterized by dynamic electrocardiographic (ECG) repolarization changes, which consist of mild ST-segment elevation on presentation (acute phase) followed by T-wave inversion with QT interval prolongation within 24–48 h after presentation (subacute phase). It is noteworthy that subacute ECG repolarization abnormalities of TTS resemble those of the so-called Wellens’ ECG pattern, which is characterized by transient T-wave inversion in the anterior precordial leads as a result of either myocardial ischaemia or other non-ischaemic conditions, all characterized by a reversible left ventricular dysfunction (‘stunned myocardium’).


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