scholarly journals Ventricular Tachycardia in Congenital Adrenal Hyperplasia

2002 ◽  
Vol 30 (3) ◽  
pp. 380-381 ◽  
Author(s):  
V. S. Virdi ◽  
B. Bharti ◽  
B. Poddar ◽  
S. Basu ◽  
V. R. Parmar

Severe hyperkalaemia in patients with congenital adrenal hyperplasia in association with aggravating factors such as acidosis and hypocalcaemia can cause life-threatening ventricular arrhythmias. Treatment of the underlying cause may be the only modality required in such cases. We report a 20-day-old male presenting with ventricular tachycardia due to electrolyte abnormalities in salt-losing congenital adrenal hyperplasia. Sudden cardiac deaths reported earlier in such cases thus gain credence.

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Chivulescu ◽  
Ø.H Lie ◽  
H Skulstad ◽  
B A Popescu ◽  
R O Jurcut ◽  
...  

Abstract Background Arrhythmogenic cardiomyopathy (AC) is an inheritable cardiomyopathy with incomplete penetrance, variable phenotype severity and poorly described disease progression. It is characterized by high risk of life-threatening ventricular arrhythmias and sudden cardiac death in young individuals. Risk stratification and selection of patients presenting without history of life-threatening arrhythmic events for cardioverter-defibrillator implantation in primary prevention remains challenging. Purpose We aimed to assess the impact of disease progression on arrhythmic outcomes in AC patients. Methods We included consecutive AC probands and mutation-positive family members with at least one complete follow-up evaluation. Echocardiographic and electrical parameters were defined according to the 2010 Revised Task Force criteria at inclusion and at last follow-up. Structural progression was defined as development of new echocardiographic diagnostic criteria. Electrical progression was defined as the development of new diagnostic depolarization, repolarization and/or premature ventricular complex count criteria during follow-up. Non-sustained ventricular tachycardia or ventricular tachycardia occurring during follow-up defined incident ventricular arrhythmic events. Results We included a total of 144 patients (48% female, 47% probands, 40±16 years old). At inclusion, 54 patients (37%) had a history of arrhythmic events, 30 patients (21%) had overt structural disease and 114 (79%) had no or minor structural disease. During 7.0 (IQR: 4.5 to 9.4) years of follow-up, 49 patients (43%) with no or minor structural disease at inclusion developed new structural criteria being defined as progressors. Among 80 participants with no or minor structural disease and no arrhythmic history at inclusion, a first arrhythmic event occurred in 14 (17%). The incidence of arrhythmic events was higher in progressors (11/27, 41%) than in non-progressors (3/53, 6%) (p<0.001) (Figure). Structural progression was associated with higher risk of first arrhythmic events during follow-up when adjusted for sex, age at inclusion and follow-up duration, independent of electrical progression (7.6, 95% CI [1.5, 37.2], P=0.01). Incident arrhythmic events distribution Conclusion Almost half of patients without overt structural cardiac disease at genetic diagnosis develop new structural criteria during 7 years follow-up and 17% experienced their first ventricular arrhythmic event. Structural progression was independently associated with ventricular arrhythmic events during follow-up. These findings highlight the increased risk of arrhythmias when structural abnormalities are detected. Their finding may initiate the evaluation for primary prevention cardioverter-defibrillator implantation.


2020 ◽  
Vol 26 (4) ◽  
pp. 59-63
Author(s):  
Yu. V. Shubik

The differences between polymorphic spindle-shaped (such as “pirouette”, “torsade de pointes”) and polymorphic bidirectional ventricular tachycardia are discussed, examples of these life-threatening ventricular arrhythmias are given.


Author(s):  
Gurbinder Singh ◽  
Natalia S. Ivascu

This chapter assesses ventricular arrhythmias. The most common ventricular arrhythmias are premature ventricular complexes (PVCs). Occasional PVCs are rarely harmful and may be related to tissue reperfusion or electrolyte abnormalities. Other types of ventricular arrhythmias are non-sustained ventricular tachycardia and ventricular tachyarrhythmias, which include ventricular tachycardia and ventricular fibrillation. Ventricular tachycardia is defined as a rate >100 bpm with 3 or more ventricular complexes in a row, which is sustained. Meanwhile, ventricular fibrillation is a form of complex ventricular arrhythmias and usually indicates a left ventricular problem. The possible causes of ventricular arrhythmias in the perioperative period include ischemia; electrolyte abnormalities such as hypokalemia, hyperkalemia, hypomagnesemia, and hypocalcemia; pulmonary artery catheters; hypothermia and metabolic acidosis; antiarrhythmic medications; adrenergic medications and inotropes; and pacing wires. The treatment of ventricular arrhythmias includes cardioversion and defibrillation; identification and treatment of ischemia, including coronary artery bypass grafting, valvular surgery, and aortic root surgery; and the administration of lidocaine and amiodarone.


1995 ◽  
Vol 268 (6) ◽  
pp. H2569-H2573 ◽  
Author(s):  
N. A. McHugh ◽  
S. M. Cook ◽  
J. L. Schairer ◽  
M. M. Bidgoli ◽  
G. F. Merrill

The purpose of this investigation was to determine if exogenous estrogen could attenuate the ventricular arrhythmias caused by myocardial ischemia and reperfusion. Conjugated equine estrogen, administered as an intravenous bolus injection (100 micrograms) to anesthetized, instrumented beagles of both genders, significantly attenuated the incidence of ventricular arrhythmias during a 20-min period of ischemia (2 +/- 1 vs. 19 +/- 16% ectopy) and in the first 5 min of reperfusion (15 +/- 9 vs. 69 +/- 20% ectopy). By 15-20 min of ischemia, ventricular salvos and nonsustained ventricular tachycardia were frequently observed in nontreated dogs. One dog in this group fibrillated during ischemia. In contrast, estrogen-treated dogs exhibited only an occasional ventricular premature beat during the same period of ischemia. When compared with baseline values, the percent ectopy during ischemia in estrogen-treated dogs was insignificant. During reperfusion, nontreated dogs displayed severe, life-threatening arrhythmias such as sustained ventricular tachycardia. In two of these dogs ventricular tachycardia deteriorated to ventricular fibrillation. In comparison, estrogen-treated dogs displayed only innocuous ventricular arrhythmias during reperfusion, i.e., ventricular premature beats, ventricular salvos, and ventricular bigeminy. In addition to the effect of estrogen on arrhythmias, there was a gradual increase in coronary blood flow on reperfusion in estrogen-treated dogs. This effect of estrogen was preceded by a significantly higher coronary perfusion pressure during ischemia (31 +/- 2 vs. 18 +/- 4 mmHg, P < 0.05). In conclusion, our findings suggest that antiarrhythmic effects of estrogen treatment might stabilize ventricular rhythmicity during ischemia and reperfusion.


2021 ◽  
Vol 28 (06) ◽  
pp. 799-803
Author(s):  
Waqas Imran Khan ◽  
Abdur Rehman ◽  
Erum Afzal ◽  
Asim Khurshid ◽  
Sidra Anjum ◽  
...  

Objective: To determine the etiology of ambiguous genitalia in newborn females with XX karyotype. Study Design: Cross Sectional Observational study. Setting: Children Hospital and Institute of Child Health Multan (CH& ICH). Period: July 2018 to December 2019. Material & Methods: Fifty two patients were enrolled after taking informed consent from parents/guardians. Various causes of ambiguous genitalia like CAH, maternal intake of virilizing drugs during pregnancy and isolated local genital defect were noted. Results: Out of 52 patients, mean gestational age was 38.23 ± 2.36 weeks and 10 (20.2 %) had gestational age up to 36 weeks while 42 (80.8%) had gestational age more than 36 weeks. Mean age was 12.37 ± 4.21 days. Mean weight was 2965.43 ± 412.23 grams while 24 (46.1%) had weight up to 2500 grams and 28 (53.9%) had weight more than 2500 grams. Congenital adrenal hyperplasia in our study cases was noted in 45 (86.5%). Congenital local genital defects in our study cases were noted in 6 (11.5%) while maternal ingestion of virilizing drugs during pregnancy was noted in 1 (2%). CAH was not associated with gestational age or weight of newborn with p value 0.680 and 0.595 respectively. Conclusion: Congenital adrenal hyperplasia in our study cases was the commonest reason of ambiguous genitalia in newborn females with XX karyotype followed by congenital local defects and maternal use of virilizing drugs. High index of suspicion is required for the diagnosis of potential life threatening disorder like CAH.


2021 ◽  
Vol 8 ◽  
Author(s):  
Ehud Chorin ◽  
Aviram Hochstadt ◽  
Arie Lorin Schwartz ◽  
Gil Matz ◽  
Sami Viskin ◽  
...  

Aims: Assessing the effectiveness of novel bio-sensing technology (CardiacSense), for accuracy and reliability of automatic detection of life-threatening arrhythmias.Methods and Results: This prospective study consisted of Eighteen patients (13 males and 5 females, mean age 59.4 ± 21.3 years) undergoing induction of ventricular tachycardia/fibrillation or provocation of transient ventricular asystole. We tested the detection of provoked ventricular arrhythmias by a wrist-worn watch-like device which uses photoplethysmography (PPG) technology to detect the cardiac rhythm. We used simultaneous electrocardiographic (ECG) recordings as gold standard for arrhythmia definition and confirmation of beat-to-beat detection. A total of 1,527 QRS complexes were recorded simultaneously by ECG and PPG. The overall correlation between the ECG (R-R intervals) and the PPG (G-G intervals) was high, with a correlation coefficient of R = 0.949 (p &lt; 0.001). The device accurately detected all events of mimicked life endangering arrhythmias, including five events of transient (adenosine-induced) ventricular asystole as well as seven episodes of monomorphic ventricular tachycardia and 6 events of ventricular fibrillation.Conclusion: This proof-of-concept study suggests that wearable devices using PPG technology, currently used to detect atrial fibrillation, may also have a role as automatic detectors of life-threatening arrhythmias.


2021 ◽  
Vol 25 (1-2) ◽  
pp. 72-78
Author(s):  
Т.В. Дубровінська ◽  
Ю.А. Остапчук ◽  
К.Е. Вакуленко ◽  
Б.Б. Кравчук ◽  
Н.О. Люлька ◽  
...  

In today's conditions, given the difficult economic situation in the country and the low adherence of patients to treatment, a difficult and relevant issue is the treatment of post-myocardial infarction patients, especially in the complicated course of the disease. The main reason for the occurrence of complex cardiac arrhythmias is the formation of a focus of ectopic activity in the myocardium or the appearance of a re-entry wave. In case of recurrence of life-threatening tachycardias, despite antiarrhythmic therapy, the choice must be between escalating drug therapy and radiofrequency catheter ablation (RFA). Purpose: a detailed description of the clinical case of the disease in a patient with low compliance to medical treatment, who suffered an acute myocardial infarction and was subsequently hospitalized several times in a specialized cardiac hospital for the development of complex ventricular arrhythmias. The article describes in detail modern approaches to the diagnosis and treatment of life-threatening tachycardias, including emergency care, comparison of escalation of antiarrhythmic therapy with RFA in a patient with post-infarction cardiosclerosis and frequent attacks of recurrent polymorphic ventricular tachycardia, treatment of modern defibrillator, electrophysiological study, RFA. Conclusions: Long-term follow-up of a patient with low compliance to the treatment of complex ventricular arrhythmias showed that ablation of the ventricular tachycardia substrate was more effective than escalation of antiarrhythmic therapy, which led to the remodulation of the heart cavities, improving the quality of life of the patient and preventing the progression of cardiovascular events.


2018 ◽  
Vol 103 (6) ◽  
pp. 2336-2345 ◽  
Author(s):  
Diala El-Maouche ◽  
Courtney J Hargreaves ◽  
Ninet Sinaii ◽  
Ashwini Mallappa ◽  
Padmasree Veeraraghavan ◽  
...  

Abstract Context Patients with congenital adrenal hyperplasia (CAH) are at risk for life-threatening adrenal crises. Management of illness episodes aims to prevent adrenal crises. Objective We evaluated rates of illnesses and associated factors in patients with CAH followed prospectively and receiving repeated glucocorticoid stress dosing education. Methods Longitudinal analysis of 156 patients with CAH followed at the National Institutes of Health Clinical Center over 23 years was performed. The rates of illnesses and stress-dose days, emergency room (ER) visits, hospitalizations, and adrenal crises were analyzed in relation to phenotype, age, sex, treatment, and hormonal evaluations. Results A total of 2298 visits were evaluated. Patients were followed for 9.3 ± 6.0 years. During childhood, there were more illness episodes and stress dosing than adulthood (P &lt; 0.001); however, more ER visits and hospitalizations occurred during adulthood (P ≤ 0.03). The most robust predictors of stress dosing were young age, low hydrocortisone and high fludrocortisone dose during childhood, and female sex during adulthood. Gastrointestinal and upper respiratory tract infections (URIs) were the two most common precipitating events for adrenal crises and hospitalizations across all ages. Adrenal crisis with probable hypoglycemia occurred in 11 pediatric patients (ages 1.1 to 11.3 years). Undetectable epinephrine was associated with ER visits during childhood (P = 0.03) and illness episodes during adulthood (P = 0.03). Conclusions Repeated stress-related glucocorticoid dosing teaching is essential, but revised age-appropriate guidelines for the management of infectious illnesses are needed for patients with adrenal insufficiency that aim to reduce adrenal crises and prevent hypoglycemia, particularly in children.


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