scholarly journals Prenatal Diagnosis of Ebstein Anomaly in a Centre with Limited Resources: A Potential Pitfall

2021 ◽  
Vol 9 (C) ◽  
pp. 189-192
Author(s):  
Ramush Bejiqi ◽  
Ragip Retkoceri ◽  
Hana Bejiqi ◽  
Aferdita Mustafa ◽  
Arlinda Maloku ◽  
...  

BACKGROUND: Ebstein anomaly is a rare congenital cardiac malformation characterized by varying degrees of downward displacement of the tricuspid valve leaflets into the cavity of the right ventricle. The pathophysiology varies with the degree of valvular malfunction, the size, and contractility of the right ventricle, and the presence of obstruction within the right ventricular outflow tract. An early diagnosis makes worse the often prognosis. Fetal echocardiography is a useful tool for the diagnosis of congenital heart disease including the Ebstein anomaly which shows a wide spectrum of ultrasonographic manifestations and, sometimes it can be difficult to make a true prenatal diagnosis. CASE REPORT: Here, we describe a unique case of an Ebstein anomaly in 26 weeks of gestation fetus through prenatal echocardiographic evaluation. Fetus was suspected as a severe form of the Ebstein anomaly, with a severe form of right atrium enlargement, huge tricuspid insufficiency, and hypoplastic pulmonary artery. The atrial septum, dimensions of cardiac structures, left and right cardiac output, and Doppler interrogation of cardiac flows were examined. CONCLUSION: This case study presents a well-documented case of Ebstein’s anomaly type D that was diagnosed prenatally using fetal echocardiography.

2017 ◽  
Vol 136 (3) ◽  
pp. 262-265 ◽  
Author(s):  
Turgut Karabag ◽  
Caner Arslan ◽  
Turab Yakisan ◽  
Aziz Vatan ◽  
Duygu Sak

ABSTRACT CONTEXT: Obstruction of the right ventricular outflow tract due to metastatic disease is rare. Clinical recognition of cardiac metastatic tumors is rare and continues to present a diagnostic and therapeutic challenge. CASE REPORT: We present the case of a patient who had severe respiratory insufficiency and whose clinical examinations revealed a giant tumor mass extending from the right ventricle to the pulmonary artery. We discuss the diagnostic and therapeutic options. CONCLUSION: In patients presenting with acute right heart failure, right ventricular masses should be kept in mind. Transthoracic echocardiography appears to be the most easily available, noninvasive, cost-effective and useful technique in making the differential diagnosis.


2019 ◽  
Vol 21 (1) ◽  
pp. 65-66
Author(s):  
Sang-Hoon Seol

Double-chambered right ventricle (DCRV) is a cardiac disease of the right ventricular outflow tract obstruction characterized by anomalous muscle bundles that divide the right ventricle into two chambers. It may be also develop over time as an acquired lesion in patients with an abnormally short distance between the moderator band and the pulmonary valve. This report highlights the case of a man with double-chambered right ventricle after ventricular septal defect operation, who presented with syncope J MEDICINE JAN 2020; 21 (1) : 65-66


2005 ◽  
Vol 15 (S1) ◽  
pp. 17-26 ◽  
Author(s):  
Richard M. Martinez ◽  
Robert H. Anderson

In the normal heart, the morphologically right ventricle supports the pulmonary trunk. The key to echocardiographic evaluation of the junction between these structures is to understand not only the arrangement of the pulmonary valve, but also the complete muscular infundibulum that supports the valvar leaflets, lifting the valvar complex away from the base of the ventricular mass. As explained in the previous review,1 it is the presence of this free-standing muscular infundibular sleeve that makes it possible for the surgeon to remove the pulmonary valve for use as an allograft in the Ross procedure.2 In this review, we will address the echocardiographic features of the junction, considering primarily the situation in which the morphologically right ventricle supports the pulmonary trunk, but making comparisons with the abnormal arrangements in which either the aorta, or both arterial trunks, arise from the right ventricle. As we will see, the basic arrangement of the free-standing complete muscular infundibulum, or conus, is preserved with these abnormal arrangements, but there can be variation depending on the precise arrangement of the inner heart curvature, or ventriculo-infundibular fold, and the morphology can be further changed depending on the relationship of the arterial trunks themselves. The key to analysis, therefore, and also to accurate description, is to analyse separately the way in which the arterial trunks are joined to the ventricular mass, the relationships of the trunks one to the other, and the precise structure of the supporting right ventricular outflow tract, or outflow tracts in the setting of double outlet connection. If each of these features is then described in its own right, avoiding the temptation to make inferences regarding one feature from knowledge of another, then it is possible to avoid many of the persisting controversies relating to nomenclature.


1995 ◽  
Vol 5 (1) ◽  
pp. 78-81
Author(s):  
Eric Rosenthal ◽  
Shakeel A. Qureshi ◽  
Michael Tynan

SummaryA 33-year-old woman known to have tetralogy of Fallot and a straddling tricuspid valve had bilateral systemic-to-pulmonary arterial shunts constructed at the age of six months. She subsequently developed infundibular atresia. Antegrade flow of blood from the right ventricle to pulmonary trunk was re-established by percutaneous laser perforation of the outflow tract, later followed by implantation of a stent.


2015 ◽  
Vol 2015 ◽  
pp. 1-3
Author(s):  
Bortolo Martini ◽  
Nicola Trevisi ◽  
Nicolò Martini ◽  
Li Zhang

A 43-year-old woman presented to the emergency room with a sustained ventricular tachycardia (VT). ECG showed a QRS in left bundle branch block morphology with inferior axis. Echocardiography, ventricular angiography, and cardiac magnetic resonance imaging (CMRI) revealed a normal right ventricle and a left ventricular diverticulum. Electrophysiology studies with epicardial voltage mapping identified a large fibrotic area in the inferolateral layer of the right ventricular wall and a small area of fibrotic tissue at the anterior right ventricular outflow tract. VT ablation was successfully performed with combined epicardial and endocardial approaches.


2021 ◽  
Vol 9 ◽  
Author(s):  
Wangping Chen ◽  
Chukwuemeka Daniel Iroegbu ◽  
Xia Xie ◽  
Wenwu Zhou ◽  
Ming Wu ◽  
...  

Introduction: The purpose of this study was to report our experience in the surgical reconstruction of the right ventricular outflow tract in double outlet right ventricle with a major coronary artery crossing the right ventricular outflow tract in the presence of mirror image-dextrocardia.Methods: From January 2005 to December 2019, 19 double outlet right ventricle patients (median age 4 years) with mirror image-dextrocardia and a major coronary artery crossing the right ventricular outflow tract received surgical repair. An autologous pericardial patch was used to enlarge the right ventricular outflow tract in four patients without pulmonary stenosis and three patients with mild pulmonary stenosis. A valved bovine jugular venous conduit was added to a hypoplastic native pathway in nine patients, among which six patients with moderate pulmonary stenosis received small-sized bovine jugular venous conduit implantation (diameter ≤ 16 mm). In comparison, a large-sized bovine jugular venous conduit (diameter >16 mm) was adopted in a total of three patients with severe pulmonary stenosis. Finally, three patients with preoperative pulmonary hypertension (mean pulmonary artery pressure ≥40 mmHg) did not undergo further intervention of right ventricular outflow tract due to the adequate outflow tract blood flow.Results: There was no hospital mortality. One patient with sub-pulmonary ventricular septal defect and concomitant severe pulmonary hypertension died from respiratory failure 11 months after the operation. Kaplan-Meier survival was 94% at 5, 10 years. Within a mean echocardiographic follow-up of 6.9 ± 3.6 years, a total of two patients received reintervention due to valvular stenosis of the bovine jugular venous conduit (pressure gradient > 50 mmHg at 4 and 9 years) after surgical operation. Actuarial freedom from reoperation was 90 and 72% at 5 and 10 years, respectively. During the last echocardiographic follow-up phase, all the survivors were in NYHA class I.Conclusions: Double outlet right ventricle with mirror image-dextrocardia is a rare and complicated congenital cardiac malformation. Surgical reconstruction of the right ventricular outflow tract should be individualized based on the degree of pulmonary stenosis and the specific anatomical features of each patient. Reconstructing the pulmonary artery using the various sizes of valved bovine jugular venous conduit is a safe and effective surgical method.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Wojtkowska ◽  
R Zarczuk ◽  
W Brzozowski ◽  
S Lukasik ◽  
M Tomaszewski ◽  
...  

Abstract Hypertrophy of the right ventricle (RV) in the course of hypertrophic cardiomyopathy (HCM) is found in 30-60% of cases, with the possibility of a right ventricular outflow tract obstruction (RVOTO), obstruction in the apex or the middle part of the right ventricle. A patient, aged 41, admitted due to limitation of exercise tolerance, effort dyspnea, presyncope. In an echocardiogram, interventricular septum (IVS) hypertrophy was observed up to 2.0 cm; normal size of the heart cavities; normal left ventricular systolic function (EF-70%). A color doppler mapping detected the zone of flow acceleraction and turbulent flow in right ventricular outflow tract (RVOT), next a spectral doppler examination showed the RVOT obstruction with a maximal gradient of 64 mmHg. Because of the suboptimal echocardiographic imaging, a heart CT scan was performed, revealing the features of left ventricular hypertrophy, most severe at the base and the medium part of IVS (up to 25 mm). Asymmetric hypertrophy of the middle portion of the right ventricle and right ventricular outflow tract obstruction was also observed. A hemodynamic study confirmed the presence of gradient in RVOT, up to 40 mmHg. Holter electrocardiogram recorded an episode of non-sustained ventricular tachycardia. Taking into account the clinical picture, the family history of the disease, and calculated HCM Risk SCD (7.55%), the decision was made to implant a dual chamber cardioverter defibrillator. The defibrillator electrode was fixed at the apex of the right ventricle. A short AV delay was programmed for prevalent right ventricular stimulation (AV delay 100 ms), resulting in 99.6% ventricular stimulation. The control echocardiogram showed a reduction in the maximum gradient in RVOT to 24 mmHg. In addition, the patient was treated with a beta-blocker. To sum up, in the case of HCM we should always examine the RV with color and spectral doppler to exclude potential narrowing in RV. Constant AV sequential stimulation with a short AV delay is a recognized method that can be considered in symptomatic adult HCM patients with a left ventricular outflow tract obstruction. In the case described here, the above mentioned method proved effective in the significant reduction of the gradient in the right ventricular outflow tract. Abstract 113 Figure. gradient in RVOT


Sign in / Sign up

Export Citation Format

Share Document