Cardiac arrest in a patient with trichorhinophalangeal syndrome and dilated cardiomyopathy

2021 ◽  
Vol 14 (2) ◽  
pp. e237604
Author(s):  
Gautam Sen ◽  
Eleanor Barendt ◽  
Manas Sinha

A 44-year-old woman with known trichorhinophalangeal syndrome presented with an unheralded out of hospital cardiac arrest. Transthoracic echocardiography showed severe left ventricular systolic dysfunction with an ejection fraction <25% and cardiac MRI confirmed a diagnosis of congenital non-ischaemic dilated cardiomyopathy. The case highlights a very rare syndrome, it is previously unknown association with dilated cardiomyopathy and the possible benefit of cardiac screening for patients with known trichorhinophalangeal syndrome.

Author(s):  
N. P. Mitkovskaya ◽  
E. M. Balysh ◽  
T. V. Statkevich ◽  
N. A. Ladygina ◽  
E. B. Petrova ◽  
...  

The aim of the study was to investigate the features of clinically suspected myocarditis complicated by the left ventricular systolic dysfunction development. 93 patients with clinically suspected myocarditis were examined. The average age was 36.63 ± 1.15 years. In 43.01 % of patients the disease was accompanied by a decrease in left ventricular systolic function. In the group of patients with left ventricular systolic dysfunction in comparison with those with preserved left ventricular ejection fraction, a significantly lower proportion of men (75 % versus 81 %, respectively, χ2 = 9.3, p < 0,01) and a higher average group age (40.7 ± 1.87 versus 33.6 ± 1.3 years, respectively, p <  0,01) were revealed. The course of the disease in patients with left ventricular systolic dysfunction was characterized by a more frequent development of rhythm disturbances (65 % versus 43.3 %, respectively, χ2  = 4.3, p  < 0,05) and a higher heart rate at admission (94.5 (75‒100) and 85 (70‒89) beats per minute, respectively, p = 0.006). The structural and functional state of the heart according to echocardiography in patients with a reduced left ventricular ejection fraction versus comparison group was characterized by larger heart chambers sizes, more pronounced violations of local left ventricular contractility, more frequent involvement of the right ventricle in the pathological process (56.3  % versus 22.2  %, respectively, χ2   =  6.4, p  < 0,05). The relationships between the left ventricular ejection fraction Весці Нацыянальнай акадэміі навук Беларусі. Серыя медыцынскіх навук. 2020. Т. 17, № 4. C. 452–460 453 and the patient’s age (r = ‒0.36), the value of the heart rate at admission (r = ‒0.32), the severity of heart failure at admission, the degree of impaired local contractility of the left ventricle, the degree of right ventricular function (TAPSE, r  =  0.58), the severity of myocardial fibrosis according to cardiovascular magnetic resonance imaging (r = ‒0.32) were revealed.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Jacob C Jentzer ◽  
Hussein Abu-Daya ◽  
Asher Shafton ◽  
Meshe Chonde ◽  
Didier Chalhoub ◽  
...  

Introduction: Left ventricular systolic dysfunction (LVSD) is common after resuscitation from cardiac arrest (CA). The association of echocardiographic LVSD with cardiac rhythm during CA is not well described. Hypothesis: Patients with a shockable rhythm (VT/VF) will have a greater degree of LVSD by echocardiography after CA. Methods: Prospective registry of patients resuscitated from CA underwent transthoracic echocardiography (TTE) within 24 hours after CA. We determined 2D measurements, LVEF, spectral Doppler of mitral inflow and LV outflow, systolic and diastolic tissue Doppler of the mitral annulus velocity, and tricuspid plane annular excursion (TAPSE). We collected data on in-hospital mortality as well as vasopressor doses and troponin I levels. TTE parameters and clinical characteristics were compared between patients with a shockable (VT/VF) arrest rhythm and a non-shockable (asystole/PEA) arrest rhythm and between survivors and non-survivors using t-tests and ANOVA. Results: Of the 55 patients, the 23 (42%) with shockable CA rhythms had significantly higher LV end-systolic dimension (4.1cm vs. 3.3cm, p = 0.0073), lower LV fractional shortening (0.15 vs. 0.28, p <0.0001), and lower LVEF both by visual estimate (36.2% vs. 52.3%, p = 0.0012) and by Simpson’s biplane method (37.5% vs. 52.3%, p = 0.0506). Other measured TTE parameters did not differ between groups, including TAPSE (shockable 1.53 vs. non-shockable 1.82, p = 0.1731). Admission and peak 24 hour vasopressor requirements did not differ between groups. Peak troponin levels were higher (22.26 vs. 3.88, p = 0.0198) in patients with shockable CA rhythms, but admission troponin levels were no different (0.88 vs. 0.51, p = 0.1527). TTE parameters did not differ between survivors and non-survivors (visual LVEF 47.0% vs. 44.2%, p = 0.5968; LV fractional shortening 0.19 vs. 0.25, p = 0.0916). Conclusions: Patients with shockable CA rhythms have more severe LVSD on 24 hour echocardiography despite similar vasopressor requirements and admission troponin levels. Echocardiographic parameters at 24 hours did not predict in-hospital mortality. Early echocardiography after CA appears more useful for differentiating primary CA rhythm than for predicting mortality.


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