Management of Pregnancy in 31-Year-Old Woman with Persistent Atrial Flutter After Cone Reconstruction for Ebstein’s Anomaly

There is a growing number of mothers suffering innate heart disease which could potentially risk their gestation period. Ebstein’s anomaly refers to a rare innate disease in the heart which accounts for less than 1% of most cases of innate heart disease, and only 5% of the patients surviving beyond 50 years of age. Cone reconstruction (CR) is an option for the repairment of tricuspid valve in patients with Ebstein anomaly. However, persistent arrhytmia, such as AFL, is possible to occur after CR. This case report aims to opt suitable pregnancy management of patients with persistent atrial flutter (AFL) after Cone reconstruction for Ebstein’s Anomaly. We report a 31 year-old woman who consulted for pregnancy planning due to history of Ebstein’s Anomaly, and had undergone successful cone reconstruction. No recorded history of AFL is reported. Physical examination finding was tricuspid regurgitation murmur without signs and symptoms of right ventricular failure. Electrocardiography showed counter-clockwise typical AFL. Echocardiography finding demonstrated post Cone reconstruction (CR), mild tricuspid regurgitation, and EF of 76%.

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Surgical Treatment of Ebstein's Anomaly

The Thoracic and Cardiovascular Surgeon ◽

10.1055/s-0037-1604469 ◽

2017 ◽

Vol 65 (08) ◽

pp. 639-648 ◽

Cited By ~ 4

Author(s):

Rüdiger Lange ◽

Melchior Burri

Keyword(s):

Surgical Treatment ◽

Right Ventricular ◽

Surgical Repair ◽

Surgical Techniques ◽

Right Ventricular Failure ◽

Ebstein’S Anomaly ◽

Ventricular Failure ◽

Ebstein's Anomaly ◽

History Of ◽

Surgical Options

AbstractSurgical repair of the tricuspid valve is a milestone in the medical history of patients with Ebstein's anomaly. The timely alleviation of the insufficiency has an important impact on the prognosis. In this review, we describe features of the disease relevant to surgical correction and the evolution of surgical techniques over six decades. We compare the results of different repair and replacement techniques. Additionally, we discuss concomitant antiarrhythmic surgery and bailout strategies for postoperative right ventricular failure. Finally, we review the surgical options in symptomatic neonates with Ebstein's disease.

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Anorexia Nervosa in a Postoperative Patient With Ebstein Anomaly

10.21203/rs.3.rs-616147/v1 ◽

2021 ◽

Author(s):

Sato Kengo ◽

Watanabe Ryosuke ◽

Okada Tsuyoshi ◽

Nishiyori Yasushi ◽

Kobayashi Toshiyuki ◽

...

Keyword(s):

Eating Disorders ◽

Congenital Heart Disease ◽

Anorexia Nervosa ◽

Heart Disease ◽

Congenital Heart ◽

Heart Diseases ◽

Ebstein’S Anomaly ◽

Ebstein Anomaly ◽

Ebstein's Anomaly ◽

Fontan Surgery

Abstract Background: Along with the improved life prognosis of patients with congenital heart disease, their diverse complications have come under scrutiny. Due to the various medical restrictions attendant on their upbringing, patients with congenital heart disease often have coexisting mental disorders. However, reports of patients with congenital heart disease and coexisting eating disorders are very rare. In this paper the authors report the case of a patient who developed anorexia nervosa following surgery for Ebstein’s anomaly.Case Presentation: A 21-year-old female with Ebstein’s anomaly who underwent a Fontan surgery was transferred to our institution with suspected anorexia nervosa after a more than 2-year intermittent stay in a medical hospital for decreased appetite. Initially, she did not express a desire to lose weight or a fear of obesity, and we suspected that she was suffering from appetite loss due to a physical condition associated with the Fontan circulation. But gradually the pathology of the eating disorder became apparent.Conclusions: Patients with congenital heart disease are more likely to have a psychological background as well as physical problems that might contribute to eating disorders. Indeed, it is unclear why there are not more cases of eating disorders in association with congenital heart diseases.

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Right ventricular exclusion procedure for right ventricular failure of Ebstein's anomaly

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2008.09.029 ◽

2009 ◽

Vol 138 (5) ◽

pp. 1246-1248 ◽

Cited By ~ 4

Author(s):

Sungkyu Cho ◽

Woong-Han Kim ◽

Jae Gun Kwak ◽

Chun Su Park

Keyword(s):

Right Ventricular ◽

Right Ventricular Failure ◽

Ebstein’S Anomaly ◽

Ventricular Failure ◽

Ebstein's Anomaly

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Preoperative predictability of right ventricular failure following surgery for Ebstein’s anomaly

European Journal of Cardio-Thoracic Surgery ◽

10.1093/ejcts/ezy425 ◽

2018 ◽

Vol 55 (6) ◽

pp. 1187-1193 ◽

Cited By ~ 2

Author(s):

Kariem Mrad Agua ◽

Melchior Burri ◽

Julie Cleuziou ◽

Elisabeth Beran ◽

Christian Meierhofer ◽

...

Keyword(s):

Right Ventricular ◽

Right Ventricular Failure ◽

Ebstein’S Anomaly ◽

Ventricular Failure ◽

Ebstein's Anomaly

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P263 Paradoxical embolus causing myocardial infarction in a young adult with congenital heart disease

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jez319.123 ◽

2020 ◽

Vol 21 (Supplement_1) ◽

Author(s):

S Veerappan ◽

S Moharem-Elgamal ◽

J Luszczak ◽

N Manghat ◽

S Lyen ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Tricuspid Regurgitation ◽

Congenital Heart ◽

Multimodality Imaging ◽

Left Ventricular ◽

Ebstein’S Anomaly ◽

Gadolinium Enhancement ◽

Ebstein's Anomaly ◽

Paradoxical Embolus

Abstract Case Report A 17 year old girl presented to her local hospital 24 hours after the onset of severe central chest pain that lasted for 30 minutes and spontaneously resolved. Her congenital heart disease background was of Ebstein’s anomaly of the tricuspid valve, coarctation of the aorta and ventricular septal defect (VSD). She underwent surgical repair of the coarctation and PA band at two months of age, followed by VSD closure when she was a year old.. An atrial communication was deliberately left open at the time of this operation and she had a known residual VSD and mild tricuspid regurgitation. Her resting saturations were 97% in air . On admission, she had a significantly raised Troponin T of 24,000ng/L with no significant ST segment elevation. An echocardiogram demonstrated new left ventricular (LV) systolic impairment with an ejection fraction of 33%. She underwent a ‘triple rule out’ cardiac CT scan and subsequently a cardiac MRI. On retrospective review, the CT demonstrated a sizeable atrial communication with left to right shunt, focal tight mid left anterior descending (LAD) artery stenosis aftersecond diagonal branch with mid to apical anterior and anteroseptal hypoperfusion. The MRI demonstrated extensive full thickness late gadolinium enhancement in mid to apical septum extending to the apical cap and apical inferior segment with microvascular obstruction (MVO) in the mid septum with possible VSD patch breakdown. No thrombi was notes on the gadolinium enhancement images. She was transferred to our hospital and underwent an invasive coronary angiogram that demonstrated recanalization of her coronary artery. She remained haemodynamically well throughout the admission. Anticoagulation with a direct acting oral anticoagulant (DOAC) was started alongside ACE-I and Beta blocker therapy. Discharge transthoracic echocardiogram showed flow across the atrial septum, a small residual VSD and mild LV dysfunction with akinesia of mid-to-distal LAD territory and very good function of remaining LV segments. She was discharged home with a plan for elective outpatient admission for a closure of her interatrial communication in a few months once she has recovered. Figure 1 summarises the key aspects of the multimodality imaging in this patient. Discussion Coronary embolus is the underlying cause of 3% of acute coronary syndromes with a small proportion being from a paradoxical embolus from the venous circulation through a right to left shunt across an atrial communication. Ebstein’s anomaly is a rare disorder, accounting for <1% of all congenital heart defects. Right-to-left shunt across the atrial communication is common in this condition due to RA dilatation and increased RA pressures secondary to tricuspid regurgitation. Our case illustrates the importance of having a high index of suspicion in patients with congenital heart disease for this rare complication of paradoxical coronary embolus, especially given the significant morbidity. Abstract P263 Figure 1

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New onset severe right ventricular failure associated with COVID-19 in a young infant without previous heart disease

Cardiology in the Young ◽

10.1017/s1047951120001857 ◽

2020 ◽

Vol 30 (9) ◽

pp. 1346-1349 ◽

Cited By ~ 1

Author(s):

Moises Rodriguez-Gonzalez ◽

Patricia Rodríguez-Campoy ◽

Maria Sánchez-Códez ◽

Irene Gutiérrez-Rosa ◽

Ana Castellano-Martinez ◽

...

Keyword(s):

Heart Disease ◽

Right Ventricular ◽

Young Infant ◽

Right Ventricular Failure ◽

Future Research ◽

Recent Experience ◽

Ventricular Failure ◽

History Of ◽

Inflammatory Syndrome ◽

New Onset

AbstractWe present our recent experience with a 6-month-old infant with a personal history of short bowel syndrome that presented with fever, cyanosis, and cardiogenic shock secondary to severe pulmonary hypertension and right ventricular failure without pulmonary thromboembolism. He did not present signs of toxin-mediated disease or Kawasaki disease. He was finally diagnosed with SARS-CoV-2 infection. If this presentation is confirmed in future research, the severe cardiovascular impairment in children with COVID-19 could be also attributable to the primary pulmonary infection, not only to a multisystem inflammatory syndrome but also in children without heart disease.

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Ebstein Anomaly Focusing on Pre-excited Atrial Fibrillation Management

Indonesian Medical and Life Science Journal ◽

10.53536/melisa.v1i2.11 ◽

2021 ◽

Vol 1 (2) ◽

pp. 41-46

Author(s):

Imelda Krisnasari ◽

Sasmojo Widito ◽

Ardian Rizal

Keyword(s):

Atrial Fibrillation ◽

Beta Blockers ◽

Accessory Pathway ◽

Ebstein’S Anomaly ◽

Ebstein Anomaly ◽

Ebstein's Anomaly ◽

Wpw Syndrome ◽

Downward Displacement ◽

Long Time ◽

History Of

Introduction: Ebstein’s anomaly is a rare abnormality of the heart associated with atrialization right ventricle and apical (downward) displacement of the tricuspid valve functional annulus. Twenty percents of patients with Ebstein’s anomaly accompanied with accessory pathway. The dilatation of atrium and aging process may develop atrial fibrillation (AF).Case Description: A 35 years old patient with recurrency palpitation, accompanied with dizziness and epigastric discomfort. He had history of taking propafenone 3 x 150 mg for long time while the palpitation recurrent. He was hospitalization due to propafenone could not suppress the palpitation. During monitor in hospital revealed haemodynamic stable with heart rate 160-180 beats/minute irregularly irregular. The electrocardiography showed atrial fibrillation with pre-excitation WPW syndrome. We performed electrical cardioversion 100 joule. Then the atrial fibrillation was convert to sinus rhythm with WPW pattern. The propafenone 3 x 150mg was continued. The patient was performed catheter radiofrequency ablation of the accessory pathway. Electrophysiology showed AV fusion at right anteroseptal pathway and preexcited atrial fibrillation with shortest RR interval 220 ms that converted by cardioversion. The ablation was successfully performed. Discussion: The accessory pathway is a complication of ebstein anomaly. Digoxin, beta-blockers, diltiazem, verapamil, and amiodarone are potentially harmful in pre-excited atrial fibrillation. Propafenone reduces fast inward potential by sodium channels, reduces spontaneous automaticity and prolongs the effective refractory periode so could be used in this case. Catheter ablation of accessory pathway in Ebstein anomaly with WPW syndrome was class I recommendation. In our case, the accessory pathway was successfully ablated.

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Two decades of experience on ablation in children with Ebstein’s anomaly

Cardiology in the Young ◽

10.1017/s1047951121002353 ◽

2021 ◽

pp. 1-7

Author(s):

Tevfik Karagöz ◽

İlker Ertuğrul ◽

Ebru Aypar ◽

Aydın Adıgüzel ◽

Hayrettin Hakan Aykan ◽

...

Keyword(s):

Atrial Flutter ◽

Atrial Tachycardia ◽

Ebstein’S Anomaly ◽

Atrioventricular Nodal Reentrant Tachycardia ◽

Success Rates ◽

Recurrence Rates ◽

Ebstein's Anomaly ◽

Accessory Pathways ◽

Rare Occasion ◽

The Right

Abstract Introduction: Accessory pathways are commonly seen due to delamination of tricuspid valve leaflets. In addition to accessory pathways, an enlarged right atrium due to tricuspid regurgitation and incisional scars creates substrates for atrial re-entries and ectopic tachycardia. We sought to describe our experience with catheter ablation in children with Ebstein’s anomaly. Methods and results: During the study period, of 89 patients diagnosed with Ebstein’s anomaly, 26 (30.9%) of them who underwent 33 ablation procedures were included in the study. Accessory pathways were observed in the majority of procedures (n = 27), whereas atrial flutter was observed in five, atrioventricular nodal reentrant tachycardia in five, and atrial tachycardia in two procedures. Accessory pathways were commonly localised in the right posteroseptal (n = 10 patients), right posterolateral (n = 14 patients), septal (n = two patients), and left posteroseptal (n = one patient) areas. Multiple accessory pathways and coexistent arrhythmia were observed in six procedures. All ablation attempts related to the accessory pathways were successful, but recurrence was observed in five (19%) of the ablations. Ablation for atrial flutter was performed in five patients; two of them were ablated successfully. One of the atrial tachycardia cases was ablated successfully. Conclusions: Ablation in patients with Ebstein’s anomaly is challenging, and due to nature of the disease, it is not a rare occasion in this group of patients. Ablation of accessory pathways has high success, but also relatively high recurrence rates, whereas ablation of atrial arrhythmias has lower success rates, especially in operated patients.

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