Transcatheter closure of atrial septal defect associated with arrhythmogenic right ventricular cardiomyopathy: a case report and literature review

2014 ◽  
Vol 25 (3) ◽  
pp. 403-407
Author(s):  
Haiyan Wang ◽  
Hongyu Ding ◽  
Lei Lei ◽  
Xiaohong Zhang ◽  
Yuling Gong ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Atrial Septal Defect ◽  
Ventricular Arrhythmias ◽  
Septal Defect ◽  
Medical Literature ◽  
Transcatheter Closure ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Interventional Treatment ◽  
Ventricular Cardiomyopathy

AbstractArrhythmogenic right ventricular cardiomyopathy is characterised by progressive, fibrofatty replacement of myocardium, and ventricular arrhythmias, and its prognosis is usually poor. Arrhythmogenic right ventricular cardiomyopathy associated with atrial septal defect is very rare, and this combination may make the diagnosis, treatment, and prognosis difficult. We present a case of a patient with this association who underwent interventional treatment with a septal defect occluder. Transcatheter closure of atrial septal defect in a patient with arrhythmogenic right ventricular cardiomyopathy is hitherto unreported. During a 3-year follow-up he remained relatively stable. We also review the cases reported in the medical literature describing this uncommon association between arrhythmogenic right ventricular cardiomyopathy and atrial septal defect or patent foramen ovale.

scholarly journals Large atrial septal defect misdiagnosed as arrhythmogenic right ventricular cardiomyopathy

2019 ◽  
Vol 7 (30) ◽  
pp. 58-62
Author(s):  
Lisa Marie Moore ◽  
Cihan Cevik
Keyword(s):  
Congenital Heart Disease ◽  
Right Ventricular ◽  
Atrial Septal Defect ◽  
Congenital Heart ◽  
Septal Defect ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Secundum Atrial Septal Defect ◽  
Ventricular Cardiomyopathy ◽  
Initial Work ◽  
Work Up

We report on a case of a large ostium secundum atrial septal defect (ASD) that was initially diagnosed as arrhythmogenic right ventricular cardiomyopathy (ARVC). We discuss the initial work up, diagnosis, and treatment of this patient’s ASD and explore how the pathophysiology of ARVC may mimic congenital heart disease where there is a large left-to-right shunt.

Abstract 2935: Clinical-Instrumental Characteristics and Prognosis in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy: 30 Years of Experience in a Referral Center

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Andrea Di Lenarda ◽  
Bruno Pinamonti ◽  
Marco Merlo ◽  
Alberto Pivetta ◽  
Stylianos Pyxaras ◽  
...  
Keyword(s):  
Heart Transplantation ◽  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Stress Test ◽  
Systolic Dysfunction ◽  
Left Ventricular ◽  
Muscle Disease ◽  
Ventricular Cardiomyopathy

There are few studies regarding the clinical and instrumental presentation of Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC); all available data derive from few specialized centers. We sought to describe clinical characteristics, instrumental findings and prognosis in a large cohort of patients (pts) with an ARVC diagnosis. From 1976 to 2006 all pts matching ARVC diagnostic criteria were enrolled in the Heart Muscle Disease Registry of Trieste and underwent a structured diagnostic protocol and follow-up. 104 pts were studied, 66% males, mean age 33±13 years; 84% were symptomatic at enrolment (mean duration 44±67 months); 75% had symptomatic arrhythmias (43% with previous sustained ventricular tachycardia or ventricular fibrillation (SVT/VF)), 36% syncope, 20% heart failure (12% in NYHA class III-IV). Familial ARVC were present in 46% of pts. ECG was abnormal in 72% (right bundle branch block 25%; epsilon potentials 15%; anterior negative T waves 51%; QRS dispersion >40ms 11%). At signal averaged ECG late potentials were present in 58% of pts. At 2D echo, mean right ventricle (RV) end-diastolic area was 30± 8 cm2 and RV fractional area contraction (FAC) 30±13%; RV aneurysms were present in 67%. RV systolic dysfunction (defined as FAC<40%) was present in 78%, characterized severe (FAC <30%) in 48%. Left ventricular systolic dysfunction was present in 27% of pts. Stress test was interrupted in 11% of cases for ventricular arrhythmias (55% SVT/VF). RV fibrofatty tissue was present in 52% of Nuclear Magnetic Resonance and in 59% of RV endomyocardial biopsy (performed respectively in 24 and 27% of pts). During a mean follow-up of 132±89 months, 3 pts were lost; 20 out of the remaining 101 pts (20%) experienced death/heart transplantation, 6 (3%) died for refractory HF; 4 pts (2%) underwent heart transplantation (HT); 6 pts (3%) died suddenly; one (0.5%) for extracardiac disease and 3 (1.6%) for unknown reason. Ten-year cumulative survival free from HT was 57%. In our population ARVC presentation was characterized by symptomatic, frequently sustained ventricular arrhythmias. Notably, LV involvement was present in approximately one third of pts at enrolment. In the long term, the overall prognosis was severe.

scholarly journals Right-to-left Shunt via Iatrogenic Atrial Septal Defect Requiring Emergency Surgical Repair following Cryoballoon Atrial Fibrillation Ablation in a Patient with Arrhythmogenic Right Ventricular Cardiomyopathy

2022 ◽  
Author(s):  
Ryohsuke Narui ◽  
Seigo Yamashita ◽  
Michio Yoshitake ◽  
Tomohisa Nagoshi ◽  
Takashi Kunihara ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
Right Ventricular ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Balloon Catheter ◽  
Atrial Fibrillation Ablation ◽  
Transseptal Puncture ◽  
Ventricular Cardiomyopathy ◽  
Iatrogenic Atrial Septal Defect

An 81-year-old woman with arrhythmogenic right ventricular cardiomyopathy underwent catheter ablation for atrial fibrillation and atrial flutter. Hypoxemia refractory to the administration of oxygen was seen after transseptal puncture. Transthoracic echocardiography revealed right to left shunt via an iatrogenic atrial septal defect (IASD) that was increased by tricuspid regurgitation flow. Her hypoxemia improved after IASD occlusion with the inflation of a venogram balloon catheter. Emergent surgical IASD closure was successfully performed. IASD after transseptal puncture for atrial fibrillation ablation infrequently causes severe complications that require emergent repair.

P2247Pregnancies and childbirth in women with arrhythmogenic right ventricular cardiomyopathy are associated with low risk of ventricular arrhythmias

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P G Platonov ◽  
J Carlson ◽  
I Castrini ◽  
A Svensson ◽  
M K Christiansen ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Free Survival ◽  
Child Birth ◽  
Mutation Carriers ◽  
Nulliparous Women ◽  
The Impact

Abstract Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is associated with a risk of ventricular arrhythmias (VA) and sudden cardiac death (SCD). Even though female patients with ARVC are considered to be at lower risk of VA, the impact of pregnancy and child birth on the arrhythmic risk and development of arrhythmic substrate in the context of ARVC remains insufficiently studied. Objective To assess the risk of VA in relation to childbirth in women with ARVC and the impact of multiple pregnancies on progression of arrhythmic manifestations of the disease. Methods The study included 186 females with definite ARVC (n=107, 70 probands) or unaffected mutation-carriers (n=79) with median age at the end of follow up of 48 (IQR 34–60) years. Seventeen women had 1, 59 had 2 and 29 had ≥3 child births by the age of 40 years. VA was defined as ventricular tachycardia, appropriate ICD therapy, aborted cardiac arrest or SCD. Proportions of patients who experienced VA by the age of 40 years were compared between nulliparous women (n=81) and those with reported child births (n=105). VA-free survival after accomplished pregnancies was assessed for women ≥40 years of age (n=119). Cumulative probability of VA for each pregnancy (n=230) was assessed from conception through 2 years after child birth and compared between those that occurred before ARVC diagnosis (Pre-Ds, n=164), after it (Post-Ds, n=11) and in unaffected mutation carriers (No-Ds, n=55). Results The nulliparous women had lower age at ARVC diagnosis (37 vs 44, p=0.023) and more often had VA before the age of 40 (31% vs 13%, p=0.003) while the number of child births was not related to the prevalence of VA (18% among women with 1 childbirth, 12% in those with 2 and 14% in those with 3 or more, ns). Three women suffered SCD before the age of 40. VA-free survival after 40 years did not differ between nulliparous and those who gave birth (Figure A). Only four pregnancy-related events were documented (Figure B): 1 in the Post-Ds group and three in the Pre-Ds group. No pregnancy-related events were reported in the unaffected mutation carriers. Conclusion In this Scandinavian cohort of women with ARVC we observed no indication of an increased VA risk either associated with pregnancies or during long-term follow up after the last child birth.

scholarly journals Transcatheter closure of atrial septal defect in the elderly: Early outcomes and mid-term follow-up

2020 ◽  
Vol 1 ◽  
pp. 100058
Author(s):  
Mario Giordano ◽  
Gianpiero Gaio ◽  
Michele D'Alto ◽  
Giuseppe Santoro ◽  
Giancarlo Scognamiglio ◽  
...  
Keyword(s):  
Atrial Septal Defect ◽  
Septal Defect ◽  
Transcatheter Closure ◽  
The Elderly
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