P3681Apparent sex differences in risk of life-threatening events in arrhythmogenic cardiomyopathy are related to exercise habits

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Rootwelt ◽  
Ø.H Lie ◽  
L A Dejgaard ◽  
T Edvardsen ◽  
K H Haugaa
Keyword(s):  
Sex Differences ◽  
Exercise Intolerance ◽  
Arrhythmogenic Cardiomyopathy ◽  
Physically Active ◽  
Exercise Habits ◽  
Life Threatening ◽  
Male Sex ◽  
Exercise Dose ◽  
Norwegian Research Council

Abstract Background Arrhythmogenic cardiomyopathy (AC) is an inheritable heart disease characterized by high risk of ventricular arrhythmias and exercise intolerance. Male sex is associated with higher risk of life-threatening events. However, this may be challenged by the fact that men exercise more than women. Purpose To assess whether the sex differences in arrhythmic outcome in AC-patients are driven by sex differences in exercise exposure. Methods We included AC-patients diagnosed between 1997 and 2017 in a longitudinal cohort study. We assessed exercise habits during the preceding 3 years before inclusion, and exercise dose was expressed as metabolic equivalent hours per week (MET-hours/week). Life-threatening events were defined as sustained ventricular tachycardia, aborted cardiac arrest or appropriate therapy from an implantable cardioverter-defibrillator at the time of diagnosis and prospectively during follow-up. Results We included 170 AC-patients (52% probands, 44% female, age 41±16 years). Males had higher exercise doses than female patients (36 [IQR, 14 to 54] vs. 12 [IQR, 8 to 22] MET-hours/week; P<0.001). Fifty-seven patients (34%) had previous life-threatening events, and 45 (26%) had life-threatening events during 5.7 (IQR, 2.8 to 9.4) years of follow-up. Male sex seemed to be a marker of previous life-threatening events (OR 2.0 [95% CI, 1.0 to 3.8]; P<0.05), but not when adjusted for exercise dose (Adjusted OR 1.3 [95% CI, 0.6 to 2.7]; P=0.44). Male sex also seemed to be a predictor of life-threatening events during follow-up (HR 2.0 [95% CI 1.0 to 3.9]; P=0.04) (Figure, left panel), but not when adjusted for exercise dose (Adjusted HR 1.5 [95% CI 0.8 to 3.0]; P=0.26) (Figure, right panel). Conclusions Sex differences in arrhythmic risk in AC were attributable to higher exercise doses in male AC-patients. This highlights the importance of exercise assessment in these patients, and challenges the current opinion of male sex as a risk factor in itself. Risk stratification based on sex may underestimate the risk of physically active female AC-patients. Acknowledgement/Funding This study was supported by Center for Cardiological Innovation funded by the Norwegian Research Council

1175Structural progression increases the risk of ventricular arrhythmias in patients with arrhythmogenic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Chivulescu ◽  
Ø.H Lie ◽  
H Skulstad ◽  
B A Popescu ◽  
R O Jurcut ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Arrhythmias ◽  
Arrhythmogenic Cardiomyopathy ◽  
Cardioverter Defibrillator ◽  
Structural Progression ◽  
Life Threatening ◽  
Arrhythmic Event ◽  
History Of ◽  
Structural Disease

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.

scholarly journals Left Ventricular Dysfunction in Arrhythmogenic Cardiomyopathy: Association With Exercise Exposure, Genetic Basis, and Prognosis

2021 ◽  
Author(s):  
Øyvind H. Lie ◽  
Monica Chivulescu ◽  
Christine Rootwelt‐Norberg ◽  
Margareth Ribe ◽  
Martin Prøven Bogsrud ◽  
...  
Keyword(s):  
Longitudinal Strain ◽  
Global Longitudinal Strain ◽  
Ventricular Tachyarrhythmia ◽  
Left Ventricular ◽  
Exercise Intolerance ◽  
Lv Function ◽  
Arrhythmogenic Cardiomyopathy ◽  
Lv Dysfunction ◽  
The Impact

Background Arrhythmogenic cardiomyopathy (AC) is characterized by biventricular dysfunction, exercise intolerance, and high risk of ventricular tachyarrhythmias and sudden death. Predisposing factors for left ventricular (LV) disease manifestation and its prognostic implication in AC are poorly described. We aimed to assess the associations of exercise exposure and genotype with LV dysfunction in AC, and to explore the impact of LV disease progression on adverse arrhythmic outcome. Methods and Results We included 168 patients with AC (50% probands, 45% women, 40±16 years old) with 715 echocardiographic exams (4.1±1.7 exams/patient, follow‐up 7.6 [interquartile range (IQR), 5.4–10.9] years) and complete exercise and genetic data in a longitudinal study. LV function by global longitudinal strain was −18.8% [IQR, −19.2% to −18.3%] at presentation and was worse in patients with greater exercise exposure (global longitudinal strain worsening, 0.09% [IQR, 0.01%–0.17%] per 5 MET‐hours/week, P =0.02). LV function by global longitudinal strain worsened, with 0.08% [IQR, 0.05%–0.12%] per year; ( P <0.001), and progression was most evident in patients with desmoplakin genotype ( P for interaction <0.001). Deterioration of LV function predicted incident ventricular tachyarrhythmia (aborted cardiac arrest, sustained ventricular tachycardia, or implantable cardioverter defibrillator shock) (adjusted odds ratio, 1.1 [IQR, 1.0–1.3] per 1% worsening by global longitudinal strain; P =0.02, adjusted for time and previous arrhythmic events). Conclusions Greater exercise exposure was associated with worse LV function at first visit of patients with AC but did not significantly affect the rate of LV progression during follow‐up. Progression of LV dysfunction was most pronounced in patients with desmoplakin genotypes. Deterioration of LV function during follow‐up predicted subsequent ventricular tachyarrhythmia and should be considered in risk stratification.

scholarly journals Anemia, sarcopenia, physical activity, and the risk of tuberculosis in the older population: a nationwide cohort study

2021 ◽  
Vol 12 ◽  
pp. 204062232110159
Author(s):  
Jung Eun Yoo ◽  
Dahye Kim ◽  
Hayoung Choi ◽  
Young Ae Kang ◽  
Kyungdo Han ◽  
...  
Keyword(s):  
Physical Activity ◽  
Screening Program ◽  
Chronic Obstructive ◽  
Older Population ◽  
Timed Up And Go ◽  
Obstructive Pulmonary Disease ◽  
Mild Anemia ◽  
Increased Risk ◽  
Male Sex

Background: The aim of this study was to investigate whether physical activity, sarcopenia, and anemia are associated an with increased risk of tuberculosis (TB) among the older population. Methods: We included 1,245,640 66-year-old subjects who participated in the National Screening Program for Transitional Ages for Koreans from 2009 to 2014. At baseline, we assessed common health problems in the older population, including anemia and sarcopenia. The subjects’ performance in the timed up-and-go (TUG) test was used to predict sarcopenia. The incidence of TB was determined using claims data from the National Health Insurance Service database. Results: The median follow-up duration was 6.4 years. There was a significant association between the severity of anemia and TB incidence, with an adjusted hazard ratio (aHR) of 1.28 [95% confidence interval (CI), 1.20–1.36] for mild anemia and 1.69 (95% CI, 1.51–1.88) for moderate to severe anemia. Compared with those who had normal TUG times, participants with slow TUG times (⩾15 s) had a significantly increased risk of TB (aHR 1.19, 95% CI, 1.07–1.33). On the other hand, both irregular (aHR 0.88, 95% CI 0.83–0.93) and regular (aHR 0.84, 95% CI, 0.78–0.92) physical activity reduced the risk of TB. Male sex, lower income, alcohol consumption, smoking, diabetes, and asthma/chronic obstructive pulmonary disease increased the risk of TB. Conclusion: The risk of TB among older adults increased with worsening anemia, sarcopenia, and physical inactivity. Physicians should be aware of those modifiable predictors for TB among the older population.

scholarly journals Massive haemorrhagic complications of ruptured pulmonary arteriovenous malformations: outcomes from a 12 years’ retrospective study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xu Ma ◽  
Bing Jie ◽  
Dong Yu ◽  
Ling-Ling Li ◽  
Sen Jiang
Keyword(s):  
Arteriovenous Malformations ◽  
Arterial Oxygen Saturation ◽  
Arterial Oxygen ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Pulmonary Arteriovenous Malformations ◽  
Imaging Data ◽  
Peripheral Lung ◽  
Life Threatening ◽  
The Mean

Abstract Background The life-threatening haemorrhagic complications of pulmonary arteriovenous malformations (PAVMs) are extremely rare, and only described in isolated cases. This study was designed to comprehensively investigate management of ruptured PAVMs. Methods We retrospectively assessed clinical and imaging data of ruptured PAVMs to summarize incidence, clinical characteristics, and outcomes following embolisation between January 2008 and January 2021. Results Eighteen of 406 (4.4%) patients with PAVMs developed haemorrhagic complications. Twelve of 18 patients were clinically diagnosed with hereditary haemorrhagic telangiectasia (HHT). Haemorrhagic complications occurred with no clear trigger in all cases. Eight of 18 patients (44.4%) were initially misdiagnosed or had undergone early ineffective treatment. 28 lesions were detected, with 89.3% of them located in peripheral lung. Computed tomography angiography (CTA) showed indirect signs to indicate ruptured PAVMs in all cases. Lower haemoglobin concentrations were associated with the diameter of afferent arteries in the ruptured lesions. Successful embolotherapy was achieved in all cases. After embolotherapy, arterial oxygen saturation improved and bleeding was controlled (P < 0.05). The mean follow-up time was 3.2 ± 2.5 years (range, 7 months to 10 years). Conclusions Life threatening haemorrhagic complications of PAVMs are rare, they usually occur without a trigger and can be easily misdiagnosed. HHT and larger size of afferent arteries are major risk factors of these complications. CTA is a useful tool for diagnosis and therapeutic guidance for ruptured PAVMs. Embolotherapy is an effective therapy for this life-threatening complication.

scholarly journals Open Capsulorrhaphy for Recurrent Multidirectional Instability of the Shoulder

2021 ◽  
Vol 1 (3) ◽  
pp. 263502542110071
Author(s):  
Ioanna K. Bolia ◽  
Rebecca Griffith ◽  
Nickolas Fretes ◽  
Frank A. Petrigliano
Keyword(s):  
Shoulder Instability ◽  
Return To Sport ◽  
Young Female ◽  
Subscapularis Tendon ◽  
Shoulder Stabilization ◽  
Ehlers Danlos Syndrome ◽  
Physically Active ◽  
Recurrent Shoulder Instability ◽  
Multidirectional Instability

Background: The management of multidirectional instability (MDI) of the shoulder remains challenging, especially in athletes who participate in sports and may require multiple surgical procedures to achieve shoulder stabilization. Open or arthroscopic procedures can be performed to address shoulder MDI. Indications: Open capsulorrhaphy is preferred in patients with underlying tissue hyperlaxity and who had 1 or more, previously failed, arthroscopic shoulder stabilization procedures. Technique Description: With the patient in the beach-chair position (45°), tissue dissection is performed to the level of subscapularis tendon via the deltopectoral approach. The subscapularis tenotomy is performed in an L-shaped fashion, and the subscapularis tendon is tagged with multiple sutures and mobilized. Careful separation of the subscapularis tendon from the underlying capsular tissue is critical. Capsulotomy is performed, consisting of a vertical limb and an inferior limb that extends to the 5 o’clock position on the humeral neck (right shoulder). After evaluating the integrity of the labrum, the capsule is shifted superiorly and laterally, and repaired using 4 to 5 suture anchors. The redundant capsule is excised, and the subscapularis tendon is repaired in a side-to-side fashion, augmented by transosseous equivalent repair using the capsular sutures. Results: Adequate shoulder stabilization was achieved following open capsulorrhaphy in a young female athlete with tissue hyperlaxity and history of a previously failed arthroscopic soft tissue stabilization surgery of the shoulder. The athlete returned to sport at 6 months postoperatively and did not experience recurrent shoulder instability episodes at midterm follow-up. Discussion/Conclusion: Based on the existing literature, 82% to 97% of patients who underwent open capsulorrhaphy for MDI had no recurrent shoulder instability episodes at midterm follow-up. One study reported 64% return-to-sport rate following open capsulorrhaphy in 15 adolescent athletes with Ehlers-Danlos syndrome, but more research is necessary to better define the indications and outcomes of this procedure in physically active patients.

scholarly journals A life-threatening spontaneous ascending aortic rupture due to a small penetrating aortic ulcer

2021 ◽  
Vol 9 ◽  
pp. 2050313X2110377
Author(s):  
Yasuhito Nakamura ◽  
Kiyoshi Doi ◽  
Syojiro Yamaguchi ◽  
Etsuji Umeda ◽  
Osamu Sakai ◽  
...  
Keyword(s):  
Rare Case ◽  
Aortic Wall ◽  
Aortic Rupture ◽  
Ascending Aorta ◽  
Bleeding Site ◽  
Penetrating Aortic Ulcer ◽  
Postoperative Course ◽  
Life Threatening ◽  
Aortic Dissections

We reported a rare case of spontaneous frank rupture of a small (4 mm) penetrating aortic ulcer in the ascending aorta resulted in catastrophic bleeding. The ulcer only created a pinhole wound in the adventitia without saccular aneurysms, intramural hematomas, or aortic dissections. Notably, the wound could be directly closed because the aortic wall was intact only 5 mm away from the bleeding site. The postoperative course was uneventful, and the patient was discharged on the 11th postoperative day. After 8 months, follow-up computed tomography showed no abnormality of the aortic wall at the repair site.

scholarly journals Treatment and therapeutic strategies for pituitary apoplexy in pregnancy: a case series

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuya Kato ◽  
Yoshikazu Ogawa ◽  
Teiji Tominaga
Keyword(s):  
Pituitary Apoplexy ◽  
Clinical Symptoms ◽  
Case Series ◽  
Optimal Timing ◽  
Presenting Symptoms ◽  
Pathological Evaluation ◽  
Mother And Child ◽  
Life Threatening ◽  
In Pregnancy

Abstract Background Pregnancy is a known risk factor for pituitary apoplexy, which is life threatening for both mother and child. However, very few clinical interventions have been proposed for managing pituitary apoplexy in pregnancy. Case presentation We describe the management of three cases of pituitary apoplexy during pregnancy and review available literature. Presenting symptoms in our case series were headache and/or visual disturbances, and the etiology in all cases was hemorrhage. Conservative therapy was followed until 34 weeks of gestation, after which babies were delivered by cesarean section with prophylactic bolus hydrocortisone supplementation. Tumor removal was only electively performed after delivery using the transsphenoidal approach. All three patients and their babies had a good clinical course, and postoperative pathological evaluation revealed that all tumors were functional and that they secreted prolactin. Conclusions Although the mechanism of pituitary apoplexy occurrence remains unknown, the most important treatment strategy for pituitary apoplexy in pregnancy remains adequate hydrocortisone supplementation and frequent hormonal investigation. Radiological follow-up should be performed only if clinical symptoms deteriorate, and optimal timing for surgical resection should be discussed by a multidisciplinary team that includes obstetricians and neonatologists.

scholarly journals Determinants of therapeutic lag in multiple sclerosis

2021 ◽  
pp. 135245852098130
Author(s):  
Izanne Roos ◽  
Emmanuelle Leray ◽  
Federico Frascoli ◽  
Romain Casey ◽  
J William L Brown ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Expanded Disability Status Scale ◽  
Disability Progression ◽  
Delayed Onset ◽  
Disease Characteristics ◽  
Disability Status ◽  
Male Sex ◽  
Pre Treatment ◽  
Patient Subgroups

Background: A delayed onset of treatment effect, termed therapeutic lag, may influence the assessment of treatment response in some patient subgroups. Objectives: The objective of this study is to explore the associations of patient and disease characteristics with therapeutic lag on relapses and disability accumulation. Methods: Data from MSBase, a multinational multiple sclerosis (MS) registry, and OFSEP, the French MS registry, were used. Patients diagnosed with MS, minimum 1 year of exposure to MS treatment and 3 years of pre-treatment follow-up, were included in the analysis. Studied outcomes were incidence of relapses and disability accumulation. Therapeutic lag was calculated using an objective, validated method in subgroups stratified by patient and disease characteristics. Therapeutic lag under specific circumstances was then estimated in subgroups defined by combinations of clinical and demographic determinants. Results: High baseline disability scores, annualised relapse rate (ARR) ⩾ 1 and male sex were associated with longer therapeutic lag on disability progression in sufficiently populated groups: females with expanded disability status scale (EDSS) < 6 and ARR < 1 had mean lag of 26.6 weeks (95% CI = 18.2–34.9), males with EDSS < 6 and ARR < 1 31.0 weeks (95% CI = 25.3–36.8), females with EDSS < 6 and ARR ⩾ 1 44.8 weeks (95% CI = 24.5–65.1), and females with EDSS ⩾ 6 and ARR < 1 54.3 weeks (95% CI = 47.2–61.5). Conclusions: Pre-treatment EDSS and ARR are the most important determinants of therapeutic lag.

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