scholarly journals Left sided arrhythmogenic ventricular dysplasia in siblings

Heart ◽  
2001 ◽  
Vol 86 (2) ◽  
pp. 128-130
Author(s):  
C G De Pasquale ◽  
W F Heddle

A 32 year old man with no previous medical history suffered a sudden cardiac death. Post mortem examination revealed circumferential fibro-fatty infiltration of the left ventricular myocardium. Histological appearance was characteristic of arrhythmogenic right ventricular dysplasia but unusual for its localisation only to the left ventricle. As a result of this sudden cardiac death the family of the deceased was screened for cardiac disease. A brother of the index case was 36 years old and free of cardiac history and symptoms. Cardiac investigations revealed a functionally and electrically abnormal left ventricle with apparent sparing of the right ventricle. The brothers may have a left sided form of arrhythmogenic ventricular dysplasia and illustrate the importance of screening family members of young victims of sudden cardiac death.

1996 ◽  
Vol 6 (4) ◽  
pp. 344-347 ◽  
Author(s):  
Angelika Lindinger ◽  
Yvonne Masur ◽  
Hans-Gerhard Limbach

SummaryAbsence of myocardial fibers in the right ventricle is the essence of so-called Uhl's anomaly, which should be distinguished from the fatty replacement producing arrhythmogenic right ventricular dysplasia of the adolescent and young adult. In this report, we describe a newborn with nearly complete absence of the myocardium of the left ventricle. The infant died on the seventh day because of myocardial incompetence of the left ventricle, which was unable to open the aortic valve.


1998 ◽  
Vol 275 (2) ◽  
pp. H378-H384 ◽  
Author(s):  
Joachim Weil ◽  
Thomas Eschenhagen ◽  
Gerrit Fleige ◽  
Clemens Mittmann ◽  
Ellen Orthey ◽  
...  

The enkephalins are derived from a common precursor protein known as preproenkephalin (ppENK). Enkephalins appear to be one of the endogenous ligands for the opiate receptors. In the rat the ventricular myocardium contains more ppENK mRNA than any other tissue. To gain further insight into the role of cardiac enkephalins, the regional and developmental distribution of ppENK mRNA was studied by Northern blotting and in situ hybridization. In the early postnatal period, ppENK mRNA is low in atrial and ventricular myocardium. With maturation, ppENK expression increases threefold in left and right ventricular tissue, but not in the atria or cardiac conductive system. Interestingly, ppENK mRNA levels are four times higher in the left than in the right chamber. Thus, to our knowledge, ppENK is the only gene exhibiting marked differences in expression between the adult right and left ventricle. Given the left-side preference of ppENK expression, the possibility is raised that the left ventricle is an endocrine organ that supplies the body with enkephalins.


2018 ◽  
Vol 58 (2) ◽  
pp. 93-96 ◽  
Author(s):  
Lydia Krexi ◽  
Mary N Sheppard

Background In forensic practice, a blow to the chest can lead to sudden cardiac death (SCD). Commotio cordis and contusio cordis are leading causes. Methods From a database of 4678 patients who suffered from SCD, we found three patients with commotio cordis and two patients with contusio cordis. All the patients were examined macroscopically and microscopically and had negative toxicology screen. Results The three patients who died due to commotio cordis were young males (16, 23 and 38 years old). The circumstances of death were: a blow to the chest by a football, by a friend during a party and during an assault. The hearts were completely normal at autopsy. The two patients who had contusio cordis were older males (42 and 63 years old). Both patients died during traffic accidents. At autopsy, one had significant contusion over the left ventricle, and the second had contusion over the right ventricle. Conclusion This study indicates that a blow to the chest is very important to document in the circumstances of death, and a detailed history is vital. It raises the left ventricular intra-cavitary pressure, leading to commotio cordis with immediate death with a normal heart. Blunt chest trauma can cause direct myocardial lesions, with acute changes leading to contusio cordis.


2020 ◽  
Vol 9 (1) ◽  
pp. 140-147
Author(s):  
M. K. Mazanov ◽  
N. I. Kharitonova ◽  
A. A. Baranov ◽  
S. Yu. Kambarov ◽  
N. M. Bikbova ◽  
...  

ABSTRACT. The rupture of the left ventricle free wall is one of the most dangerous complications of myocardial infarction. Due to the widespread availability of echocardiography method, the detection of this fatal complication and the number of lives saved after surgery grew. The survival of patients depends on early diagnosis, stabilization of the patient’s condition, promptness and tactics of surgical intervention. We report a case of successful closure of a rupture of the left ventricle free wall on the 15th day after myocardial infarction.


2021 ◽  
Vol 9 (11) ◽  
pp. 643-648
Author(s):  
Nava Udaya N.R ◽  
◽  
Ramiah Rajesh Kannan ◽  
Srikanth Moorthy ◽  
Resmi Sekhar ◽  
...  

Background: There are many causes of left ventricular hypertrophy which can result in arrhythmias and sudden cardiac death. Hypertrophic cardiomyopathy (HCM) is one of the commonly encountered cause of sudden cardiac death in young adults. Aim: Identifying the role of Cardiac MRI in characterising the diagnostic parameters of HCM. Materials and methods: 125 patients with clinical suspicion or genetic evidence of HCM referred for cardiac MRI between June 2013 to June 2021 were included under the study. Image interpretations were done by fellowship qualified cardiac imaging radiologist. Categorical variables were expressed using frequency and percentage. Numerical variables were presented using mean and standard deviation. Results: Out of the total population, 119 patients (95 %) had LV wall thickness > 13 mm, 48 patients (38.4%) had Left ventricle outflow tract obstruction (LVOTO) and 32 patients (25.6 %) had mid cavity obstruction, 39 patients (37.9 %) had myocardial scar > 15 % and asymmetric septal hypertrophy was the most frequently encountered left ventricle morphology Conclusion: Cardiac MRI detected HCM has a statistically significant association with the final diagnosis (histopathological and genetic correlation). CMRI hence serves as a reliable tool in identifying and characterising the various diagnostic and non- diagnostic parameters of HCM.


2021 ◽  
Vol 74 (5) ◽  
pp. 1158-1163
Author(s):  
Serhiy V. Popov ◽  
Oleksandr I. Smiyan ◽  
Andrii M. Loboda ◽  
Viktoriia O. Petrashenko ◽  
Olena K. Redko ◽  
...  

The aim: Studying the features of the structure and function of the heart in athletes and identifying the factors that influence the development of these changes. Materials and methods: The study included 54 athletes, 29 men and 25 women. The ultrasound study was performed according to standard methods with determining the size of the main structures of the heart, indicators normalized to body surface area, height. Results: The heart of dilatation and hypertrophy of the left ventricular myocardium were found in 25.93% of the athletes. When comparing the diameter of the left ventricle of individual athletes with the average values of the norm, their excess was found in 94.44% of athletes. The Odds ratio (OR) of the relationship between left ventricle diameter (LVd) and time of the exercise less than 10 y was 16.13, time of the exercise less than 5 y – 0.17 (p <0.05). OR of increase LVd to age less than 20 years was 3.56 units (p <0.05). The ejection fraction was above the normative mean in 75.93%, as well as the ratio of the periods of filling of the ventricles. Conclusions: The most common sign of an athlete’s heart development was left ventricular dilatation, which occurred at a rate of 25 percent. Age less than 20 years and the duration of sports activities from 5 to 10 years is associated with a higher frequency of the athlete’s heart.


2020 ◽  
Author(s):  
Marek Jastrzębski ◽  
Grzegorz Kiełbasa ◽  
Karol Curila ◽  
Paweł Moskal ◽  
Agnieszka Bednarek ◽  
...  

AbstractBackgroundDuring left bundle branch (LBB) area pacing, it is important to confirm that the capture of the LBB is achieved, not just the capture of only the adjacent left ventricular myocardium (LV septal capture). Our aim was to establish ECG criteria for LBB capture by analyzing ECGs with confirmed LBB capture and non-capture. We hypothesized that since LBB pacing results in physiologic depolarization of the left ventricle then the native QRS can serve as a reference for the diagnosis of LBB capture in the same patient.MethodsOnly patients with direct evidence of LBB capture (output-dependent or refractoriness-dependent QRS morphology transition) were included. Several QRS characteristics were compared between the native rhythm and different types of LBB area capture. Receiver-operator characteristics analysis was performed to determine the optimal V6 R-wave peak time (RWPT) cut-off for LBB diagnosis.ResultsA total of 357 ECG tracing (124 patients) were analyzed: 118 with native rhythm, 124 with non-selective LBB capture, 69 with selective LBB capture and 46 with LV septal capture. Our hypotheses that during LBB capture the paced V6 RWPT (measured from QRS onset) equals the native V6 RWPT and that the paced V6 RWPT (measured from the stimulus) equals the LBB potential to V6 R-wave peak interval were positively validated. Criteria based on these rules had sensitivity and specificity of 98.0–88.2% and 85.7–95.4%, respectively. The optimal and 100% specific V6 RWPT values for differentiation between LBB capture and LV septal capture in patients with narrow QRS / right bundle branch block were 83 ms and 74 ms, respectively; while in patients with left bundle branch block/asystole/ventricular escape the optimal and 100% specific V6 RWPT values were 101 ms and 80 ms, respectively.ConclusionsNovel criteria for LBB capture were developed and optimal V6 RWPT cut-offs were determined.What this study addsWe showed that LBB pacing truly reproduce the physiological depolarization of the left ventricle since the paced V6 RWPT equals the native conduction V6 RWPT.Individualized LBB capture criteria, that use the native QRS as a reference, were developed.The optimal V6 RWPT values for differentiation between LBB capture and LV septal capture were determined, separately for patients with healthy and diseased LBB.


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