scholarly journals Echocardiography in a young adult with congenital heart disease presenting with isolated right-sided heart failure – Ebstein’s anomaly.

2015 ◽  
Vol 10 (3-4) ◽  
pp. 71-71
Author(s):  
Jasmina Catic ◽  
Sandra Jaksic Jurinjak ◽  
Robert Blazekovic
2020 ◽  
Vol 4 (2) ◽  
pp. 222-224
Author(s):  
Ashley Rider ◽  
Andrea Dreyfuss ◽  
Roberto Inga

Ebstein’s anomaly is a congenital heart defect that when left untreated can lead to unique physical exam and ultrasound findings. This case describes a patient who presented with dyspnea and was found to have cyanosis, clubbing, and dilation of right-sided chambers on point-of-care ultrasound. The series of images highlights findings in late-stage Ebstein’s anomaly and serves as a springboard for the discussion of the pathophysiology, diagnosis, and treatment of this rare congenital heart disease.


2021 ◽  
Author(s):  
Sato Kengo ◽  
Watanabe Ryosuke ◽  
Okada Tsuyoshi ◽  
Nishiyori Yasushi ◽  
Kobayashi Toshiyuki ◽  
...  

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.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
H S A Abdelgawad ◽  
N Hisham ◽  
M Shehata ◽  
M A Abdelhay

Abstract Introduction Ebstein’s anomaly is characterized by displacement of the proximal attachments of the tricuspid valve from the atrioventricular ring into the right ventricle. This structural abnormality divides the right ventricle into an ‘atrialized’ portion and a distal ‘ventricularized’ portion. The severity is variable and accounts for the broad clinical spectrum, from severe disease causing fetal or neonatal death to mild disease compatible with natural survival as late as the eighth decade of life. Ebstein’s anomaly is an uncommon defect occurring in less than 1% of patients with congenital heart disease, but it is disproportionately represented in the adult congenital heart disease population because of its favourable natural history Case report A 55 year old man with no previous cardiac history .He presented to our medical facility complaining of acute retrosternal squeezing retrosternal chest pain few hours before admission. On clinical examination, he had a pansystolic murmur over the tricuspid area .ECG showed right bundle branch block. Laboratory results were unremarkable except elevated cardiac enzymes. 2D Transthoracic Echocardiography revealed a small well-functioning right ventricle, 15 mm/m2 apical displacement of the tricuspid septal leaflet with severe tricuspid regurgitation . 2D and 3D transesophageal echocardiography with intravenous agitated saline injection revealed patent foramen ovale with right to left shunt with immediate crossing of bubbles.Coronary angiography revealed normal coronaries. The patient refused intervention and he was maintained on warfarin treatment . Conclusion The clinical presentation of Ebstein’s anomaly is highly variable—depending on anatomic severity, haemodynamics ,and degree of interatrial shunting. The majority of patients have shunting through a secundum ASD or patent-foramen ovale. Paradoxical embolism maybe an indication for PFO/ ASD closure Abstract P843 Figure.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Veerappan ◽  
S Moharem-Elgamal ◽  
J Luszczak ◽  
N Manghat ◽  
S Lyen ◽  
...  

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


2021 ◽  
Vol 5 (8) ◽  
Author(s):  
Mario García Gómez ◽  
Aitor Uribarri ◽  
José Alberto San Román Calvar ◽  
Alexander Stepanenko

Abstract Background Due to improvement in the management of patients with congenital heart disease (CHD), the likelihood of their survival to adulthood is increasing. A relevant population suffers end-stage right ventricular failure (RVF) in their 3rd–4th decade of life. Hence, heart transplantation is still gold standard of treatment of end-stage heart failure, mechanical circulatory assistance has become a valuable tool in the bridging to heart transplant or definitive therapy. Use of implantable short-term or long-term devices is reported by others. However, within this clinical context, presence of significant tricuspid regurgitation (TR) or CHD is used as exclusion criteria for insertion of a percutaneous right ventricular circulatory support. Case summary We described a 36-year-old patient diagnosed with Ebstein's anomaly and severe TR who is admitted to hospital due to RVF refractory to standard medical treatment. After case presentation to the heart team, an Impella RP device insertion was scheduled, in spite of the presence of TR or CHD after evaluation of pulmonary valve competency and 3D reconstruction with virtual device implantation. During support, the patient improved clinically and haemodynamically. Due to device displacement to the right ventricle, it was bedside explanted after 30 days of support. After mechanical unloading during 30 days patients’ right ventricle recovered partially, permitting patient to improve his functional class. Discussion Although TR and CHD are exclusion criteria for the implantation of the Impella RP device, we report clinical experience in patient with Ebstein's anomaly and severe TR supported with percutaneous device as bridge to heart transplantation during 30 days.


PEDIATRICS ◽  
1954 ◽  
Vol 14 (2) ◽  
pp. 117-121
Author(s):  
ROBERT W. WINTERS ◽  
SAUL J. ROBINSON ◽  
GEORGE BATES

A case of multiple hemangiomata of the liver is reported in an infant who presented signs and symptoms strongly suggesting congenital heart disease. The post mortem examination revealed no gross anomalies of the heart or great vessels, but did show a heart with cardiac hypertrophy. A mechanism to explain the cardiac failure in this case is discussed.


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