scholarly journals P879 3D transoesophageal echocardiography to asses a case of a non-ebstein tricuspid valve congenital regurgitation

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
I Sanz Ortega ◽  
S Velasco Del Castillo ◽  
J J Onaindia Gandarias ◽  
I Rodriguez Sanchez ◽  
J Florido Perena ◽  
...  

Abstract Introduction Due to the complexity of congenital heart disease and limitations of transthorathic echocardiogram (TTE), especially in adult patients, it is not unusual to need other image techniques to assess cardiac anatomy and function. The most common primary anomaly of tricuspid valve (TV) is Ebstein anomaly, but there are other much rarer primary anomalies of this valve consisting in prolapse, cord retraction.... without downward displacement of the leaflet, generally causing tricuspid regurgitation (TR) that can be severe and sometimes intervention is needed, preferably reparation. Due to anatomical issues, it is difficult to assess anatomy of TV in TTE, so sometimes 3D-TTE must be performed to clarify the mechanism and to measure orifice, but when transthoracic view is not enough, 3D transoesophageal echocardiogram (TOE) can be useful for this purpose. Case We report the case of a 15-year-old boy that was referred to our clinic because of shortness of breath and a systolic tricuspid murmur. TTE was performed and an image compatible with tricuspid valve prolapse with no apical displacement of any leaflets (Figure, A) causing severe TR (Figure, B) was noticed, as well as severely dilated right chambers, with good ejection fraction of both ventricles. It was not clear the mechanism so 2D TOE was done, showing a prolapse of a leaflet (Figure, C) causing severe TR (Figure, D). The mechanism was finally clarified by 3D TOE (figure E). This was a prolapse of lateral portion of posterior leaflet (asterisk) with restrictive movement of anterior (triangle) and septal (arrow) ones, causing a huge coaptation defect in systole leading to a very severe tricuspid insufficiency with signs of volume overload of right ventricle. There was no atrial septal defect and pulmonary drainage anomalies were ruled out by cardiac magnetic resonance. Patient was referred to surgery due to symptoms and great dilatation of right chambers. Conclusión: Due to anatomical complexity and limitations of echography, cross and multimodality cardiac imaging is usually needed in assessing congenital heart disease. Apart from Ebstein anomaly, other congenital entities of tricuspid valve such as prolapse and/or retraction can lead to severe tricuspid regurgitation. Due to limitations of 2D TTE in assessing tricuspid valve anatomy, 3D TTE has to be performed, but if it is not enough, 3D TOE can be an option to evaluate mechanism and directly see the orifice of regurgitation in congenital disease of tricuspid valve. Abstract P879 Figure

Author(s):  
Zahra Khalaji ◽  
Nahid Rezaeian ◽  
Zahra Ansari

Ebstein anomaly, a rare congenital heart disease, is defined as displacement of hinge points of septal or posterior tricuspid leaflets but not anterior leaflet. Here we report a 35 year old lady with an extremely rare pattern of EA with all three tricuspid leaflets displaced downward to the apex.


Author(s):  
Gabriela Rangel Brandão ◽  
Amanda Thum Welter ◽  
Gabriel Dotta Abech ◽  
Carla Bastos da Costa Almeida ◽  
Caio Seiti Mestre Okabayashi ◽  
...  

AbstractTrisomy 21 is considered the most common chromosomal aneuploidy, and congenital heart disease (CHD) is highly prevalent and relevant to the morbidity and mortality of these patients. Ebstein anomaly (EA) is a rare CHD characterized by tricuspid valve dysplasia with inferior septal leaflet displacement. Herein, we describe a patient with trisomy 21 who presented with EA and discuss the association between the two conditions based on a literature review. We conclude that the concomitant occurrence of both conditions is considered to be rare. These individuals are most frequently diagnosed during birth and childhood, and they usually have a good prognosis, as observed with our patient and is typical for EA patients in general. However, it is important to be aware that electrophysiologic anomalies may also be present.


2019 ◽  
Vol 2019 ◽  
pp. 1-3 ◽  
Author(s):  
Amna Qasim ◽  
Soham Dasgupta ◽  
Ashraf M. Aly

Right ventricular (RV) hypoplasia may develop secondary to pulmonary or tricuspid valve atresia. These patients are usually symptomatic early in life and need prompt intervention. Isolated RV hypoplasia is a rare congenital heart disease. We report a case of 23-year-old twins who have been monitored for the last 14 years for isolated right ventricular hypoplasia. ECHO and MRI studies showed a small, heavily trabeculated, nonapex-forming RV and mild tricuspid valve insufficiency. The girl has a patent foramen ovale (PFO). Otherwise, the cardiac anatomy and function was normal. They have both been completely asymptomatic from the cardiac standpoint. The family history is remarkable for death of father at the age of 30 years with autopsy suggestive of a hypoplastic RV. The paternal uncle also died at the age of 46 years, and his son has an unidentified congenital heart disease. The family history appears to suggest an autosomal dominant pattern of inheritance with variable expressivity. However, the chromosome microarray analysis of the twins did not identify any variations of clinical significance.


2016 ◽  
Vol 27 (3) ◽  
pp. 592-596 ◽  
Author(s):  
Amee M. Bigelow ◽  
Brandon S. Arnold ◽  
Gregory C. Padrutt ◽  
John M. Clark

AbstractIn current practice, children with anatomically normal hearts routinely undergo fluoroscopy-free ablations. Infants and children with congenital heart disease (CHD) represent the most difficult population to perform catheter ablation without fluoroscopy. We report two neonatal patients with CHD in whom cardiac ablations were performed without fluoroscopy. The first infant had pulmonary atresia with intact ventricular septum with refractory supraventricular tachycardia, and the second infant presented with Ebstein’s anomaly of the tricuspid valve along with persistent supraventricular tachycardia. Both patients underwent uncomplicated, successful ablation without recurrence of arrhythmias. These cases suggest that current approaches to minimising fluoroscopy may be useful even in challenging patients such as neonates with CHD.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
E Cambronero Cortinas ◽  
P Moratalla-Haro ◽  
A E Gonzalez-Garcia ◽  
P Avila-Alonso ◽  
M Bret-Zurita ◽  
...  

Abstract Introduction and objectives Atrial tachycardia (AT) is a major late complication in congenital heart disease (CHD) after surgery. These arrhythmias arise between areas of anatomical barriers or in fibrotic tissue/scars. In our study we examined the clinical predictors of AT. Methods A retrospective study of cases and controls in adult patients with CHD was performed. We have included consecutively 71 patients with AT and 71 controls matched by gender, age and type of CHD located in the same database. Medical records, electrocardiograms and echocardiograms were reviewed. Multivariate analysis of the risk factor (logistic regression) and analysis of adverse cardiovascular events (Kaplan-Meier) were performed. Results Systolic pressure of subpulmonary ventricle ≥40mmHg (HR:7, 95%CI:2.4-18, p = 0.001), right atrium (RA) dilatation≥21cm2 (HR:3, 95%CI:1.2-7.6, p = 0.005), significant tricuspid regurgitation (HR:4, 95%CI:1.3-10, p = 0.018) were identified as main risk factors for AT. Patients with AT had worse outcomes with more major adverse cardiovascular events (86% vs 14%, P = 0.006), and a 58% free survival events compared to 98% of patients without AT at 8 years of follow-up (p = 0.01). The treatment of the first episode of AT was electrical cardioversion (38%), pharmacological cardioversion (13%), heart rate control (20%). Antiarrhythmic treatment was not been given in 30% of the patients 73% of them had percutaneous ablation as first choice treatment. Ablation was performed in 40 pacientes and 6 patients have recurrences. In total, 24 patients have recurrences of AT. Previous Maze (OR 9 (IC al 95% 1.5, 50), p = 0.016) and surgical paliative shunt (OR 21 (IC al 95% 2.3,192), p = 0.007) were identified as main risk factor for AT recurrences. Conclusions The main risk factors identified for AT were RA dilatation, raised systolic pressure of subpulmonary ventricle and significant tricuspid regurgitation. The main risk factors for AT recurrences were previous Maze and surgical paliative shunt. Early treatment of these arrhythmic problems is mandatory in order to improve the prognosis in this group of patients. Abstract P1582 Figure. Central illustration of AT in CHD


1985 ◽  
Vol 5 (1) ◽  
pp. 70S-76S ◽  
Author(s):  
Lawrence J. Sinak ◽  
Yun-He Liu ◽  
Michael Block ◽  
Douglas D. Mair ◽  
Paul R. Julsrud ◽  
...  

2020 ◽  
Vol 22 (12) ◽  
Author(s):  
Benjamin Kelly ◽  
Sheyanth Mohanakumar ◽  
Vibeke Elisabeth Hjortdal

Abstract Purpose of Review Lymphatic disorders have received an increasing amount of attention over the last decade. Sparked primarily by improved imaging modalities and the dawn of lymphatic interventions, understanding, diagnostics, and treatment of lymphatic complications have undergone considerable improvements. Thus, the current review aims to summarize understanding, diagnostics, and treatment of lymphatic complications in individuals with congenital heart disease. Recent Findings The altered hemodynamics of individuals with congenital heart disease has been found to profoundly affect morphology and function of the lymphatic system, rendering this population especially prone to the development of lymphatic complications such as chylous and serous effusions, protein-losing enteropathy and plastic bronchitis. Summary Although improved, a full understanding of the pathophysiology and targeted treatment for lymphatic complications is still wanting. Future research into pharmacological improvement of lymphatic function and continued implementation of lymphatic imaging and interventions may improve knowledge, treatment options, and outcome for affected individuals.


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