Surgical treatment of Ebstein's malformation: state of the art in 2006

2006 ◽  
Vol 16 (S3) ◽  
pp. 12-20 ◽  
Author(s):  
Joseph A. Dearani ◽  
Patrick W. O'Leary ◽  
Gordon K. Danielson
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Ventricular Arrhythmias ◽  
Myocardial Dysfunction ◽  
Right Atrial ◽  
Anterior Leaflet ◽  
Ventricular Dilation ◽  
Rotational Displacement ◽  
The Right ◽  
Right Ventricular Dilation

Ebstein's malformation is a congenital anomaly of the tricuspid valve and right ventricle that is characterized by several features, each of which can exhibit a spectrum of malformation. The first is adherence of the leaflets of the tricuspid valve to the underlying myocardium, this representing failure of delamination during development. This feature involves the septal and inferior leaflets, but rarely the anterior leaflet (Fig. 1). The second feature is anterior and apical rotational displacement of the functional annulus (Fig. 2). The third abnormality is dilation of the “atrialized” portion of the right ventricle, with variable degrees of hypertrophy and thinning of the wall. The fourth finding is redundancy, fenestrations, and tethering of the anterior leaflet. A fifth abnormality is dilation of the right atrioventricular junction, this being the true tricuspid valvar annulus. The final feature is variable ventricular myocardial dysfunction. Each heart with Ebstein's malformation is different, and there is an infinite variability that can occur with the above mentioned characteristics. These anatomical and functional abnormalities cause important tricuspid regurgitation, which results in right atrial and right ventricular dilation, and atrial and ventricular arrhythmias.

scholarly journals Successful One and One-half Ventricular Circulation with Bioprosthetic Tricuspid Valve Replacement and Total Chordal Preservation in a Patient with Ebstein’s Anomaly: A Video Presentation

2021 ◽  
Vol 5 (02) ◽  
pp. 147-150
Author(s):  
Ujjwal K. Chowdhury ◽  
Sukhjeet Singh ◽  
Niwin George ◽  
Lakshmi Kumari Sankhyan ◽  
Sandeep Sharan ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Ebstein’S Anomaly ◽  
Anterior Leaflet ◽  
Tricuspid Annulus ◽  
Tricuspid Valve Replacement ◽  
Rotational Displacement ◽  
Atrioventricular Junction ◽  
Ebstein's Anomaly ◽  
The Right

AbstractEbstein’s anomaly is a rare congenital malformation of the right ventricle and tricuspid valve which is characterized by several features that can exhibit an infinite spectrum of malformation.The abnormalities include: i) adherence of the tricuspid leaflets to the underlying myocardium (failure of delamination); ii) anterior and apical rotational displacement of the functional tricuspid annulus (septal > posterior > anterior leaflet); iii) dilatation of the right atrioventricular junction (the true tricuspid annulus) with variable degrees of hypertrophy and thinning of the wall; iv) dilatation of the “atrialized” portion of the right ventricle; and v) redundancy, fenestration, and tethering of the anterior leaflet.

scholarly journals A probable right atrial myxoma prolapsing through the tricuspid valve into the right ventricle: a case report

Cases Journal ◽  
2008 ◽  
Vol 1 (1) ◽  
Author(s):  
Dike B Ojji ◽  
Stella S Ajiduku ◽  
Omonuyi O Omonua ◽  
Lukman L Abdulkareem ◽  
Will Parsonage
Keyword(s):  
Case Report ◽  
Right Ventricle ◽  
Tricuspid Valve ◽  
Atrial Myxoma ◽  
Right Atrial ◽  
Right Atrial Myxoma ◽  
The Right

Right Ventricle Mass Removal from Tricuspid Valve Apparatus: An Unusual Thromboembolic Complication of Severe Ketoacidosis

2016 ◽  
Vol 19 (2) ◽  
pp. 077
Author(s):  
Ireneusz Haponiuk ◽  
Maciej Chojnicki ◽  
Konrad Paczkowski ◽  
Wojciech Kosiak ◽  
Radosław Jaworski ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Mild Hypothermia ◽  
Heart Function ◽  
Thromboembolic Complication ◽  
Surgical Removal ◽  
Unusual Complication ◽  
Type I ◽  
Definitive Therapy ◽  
The Right

The presence of a pathologic mass in the right ventricle (RV) may lead to hemodynamic consequences and to a life-threatening incident of pulmonary embolism. The diagnosis of an unstable thrombus in the right heart chamber usually necessitates intensive treatment to dissolve or remove the pathology. We present a report of an unusual complication of severe ketoacidosis: thrombus in the right ventricle, removed from the tricuspid valve (TV) apparatus. A four-year-old boy was diagnosed with diabetes mellitus (DM) type I de novo. During hospitalization, a 13.9 × 8.4 mm tumor in the RV was found in a routine cardiac ultrasound. The patient was referred for surgical removal of the floating lesion from the RV. The procedure was performed via midline sternotomy with extracorporeal circulation (ECC) and mild hypothermia. Control echocardiography showed complete tumor excision with normal atrioventricular valves and heart function. Surgical removal of the thrombus from the tricuspid valve apparatus was effective, safe, and a definitive therapy for thromboembolic complication of pediatric severe ketoacidosis.<br /><br />

scholarly journals P686 Right atrial, right ventricular and pulmonary cement embolism: a rare complication of percutaneous vertebroplasty

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Akhunova ◽  
R Khayrullin ◽  
N Stekolshchikova ◽  
M Samigullin ◽  
V Padiryakov
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Atrium ◽  
Percutaneous Vertebroplasty ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Stable Condition ◽  
Right Atrial ◽  
Cement Embolism ◽  
The Right

Abstract A 68-year-old man was admitted to the hospital with complaints of pain in the lumbar spine. He had L5 disc herniation, Spinal stenosis of the L5 root canal - S1 on the right in the past medical history. Percutaneous vertebroplasty at the level of L3 and Th8 vertebral bodies was performed six months ago due to painful vertebral hemangioma. The man is suffering from arterial hypertension, receives antihypertensive therapy. During routine transthoracic echocardiography, a hyperechoic structure with a size of 9.5 x 0.9 cm was found in the right atrium and right ventricle. Chest computed tomography with contrast enhancement revealed signs of bone cement in the right atrium and right ventricle, in the right upper lobe artery, in the branches of the upper lobe artery, in the paravertebral venous plexuses. Considering the duration of the disease, the stable condition, the absence of clinical manifestations and disorders of intracardiac hemodynamics, it was decided to refrain from surgical treatment. Antiplatelet therapy and dynamic observation were recommended. Conclusion Percutaneous vertebroplasty is a modern minimally invasive surgical procedure for the treatment of degenerative-dystrophic diseases of the spine. However, the cement can penetrate into the paravertebral veins and migrate to the right chambers of the heart and the pulmonary artery. This clinical case demonstrates asymptomatic cement embolism of the right chambers of the heart and pulmonary artery after percutaneous vertebroplasty, detected incidentally during routine echocardiography. Abstract P686 Figure.

scholarly journals Pulmonary atresia with intact septum: Growth of the right ventricle and tricuspid valve following primary procedure

1996 ◽  
Vol 27 (2) ◽  
pp. 343-344
Author(s):  
P.E.F. Daubeney ◽  
Z. Slavìk ◽  
B.R. Keeton ◽  
R.H. Anderson ◽  
S.A. Webber
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Pulmonary Atresia ◽  
Primary Procedure ◽  
The Right

Development of the inlet portion of the right ventricle in the embryonic rat heart: The basis for tricuspid valve development

1994 ◽  
Vol 239 (2) ◽  
pp. 216-223 ◽  
Author(s):  
Arnold C. G. Wenink ◽  
Bert J. Wisse ◽  
Pieter M. Groenendijk
Keyword(s):  
Right Ventricle ◽  
Tricuspid Valve ◽  
Rat Heart ◽  
Valve Development ◽  
The Right

scholarly journals Tricuspid Valve Dysplasia in a Dog

2019 ◽  
Vol 47 ◽  
Author(s):  
Gabriela De Carvalho Cid ◽  
Luciano Da Silva Alonso ◽  
Ana Paula De Castro Pires ◽  
Mariana Siqueira d'Avila Taïna Gonçalves ◽  
Taïna Gonçalves ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Tricuspid Valve ◽  
Right Atrial ◽  
Pulmonary Insufficiency ◽  
Papillary Muscles ◽  
Ventricular Enlargement ◽  
Pathological Findings ◽  
Chordae Tendineae ◽  
Clinicopathological Findings ◽  
The Right

Background: Congenital cardiac diseases are a common cause of death in puppies. Tricuspid valve dysplasia is characterized by thickening and displacement of the leaflets of the tricuspid valve, agenesis of the valves, and incomplete separation of valve components. Papillary muscles may fuse and display shortened or absent chordae tendineae that contribute to tricuspid regurgitation. Diagnostic features of tricuspid valve dysplasia include cardiomegaly with massive right atrium enlargement on thoracic radiography and tricuspid insufficiency on an ultrasound. We aimed to describe clinicopathological findings in a dog (Canis familiaris) with tricuspid dysplasia.Case: We aimed to describe tricuspid valve dysplasia in a dog referred for necropsy at the Anatomical Pathology Sector of The Rural Federal University of Rio de Janeiro, Brazil, with a clinical history of abdominal swelling, dyspnea, cyanosis, ascites, and prostration. Echocardiography and abdominal ultrasound revealed right ventricular enlargement, hepatomegaly, and splenomegaly. Examination of the heart showed prominent enlargement, thickening and dilation of the right chambers, thickening of the tricuspid leaflets, and moderately shortened chordae tendineae. The liver was enlarged, with a nutmeg pattern, and foci of clotting and fibrin adhesions in the lateral right lobule.Discussion: Epidemiological, clinical, and pathological findings were consistent with tricuspid valve dysplasia. Although structural abnormalities of the tricuspid and mitral valves are well known in fetuses and neonates, congenital and secondary tricuspid malformations are rare in dogs. The survival rate is associated with the severity of heart lesions. Tricuspid valve dysplasia is mostly observed in large-breed dogs (>20 kg), particularly in Labrador Retrievers, Boxers, and German Shepherds. Regardless, most dogs with tricuspid valve dysplasia are of a pure-breed, which differs from our findings because our dog was a mongrel. Our dog displayed signs of dyspnea, cyanosis, abdominal swelling, prostration, and enlarged liver and spleen on ultrasound examination. Tricuspid valve dysplasia led to heart enlargement and right congestive heart failure, with consequent ascites, abdominal swelling, weakness, lethargy, jugular venous distension, and hepatomegaly. Overall, the heart showed prominent enlargement, thickening and dilation of the right chambers, thickening of the tricuspid leaflets, and moderately shortened chordae tendineae. The liver had a nutmeg pattern. Tricuspid valve dysplasia is characterized by malformation of the tricuspid valve leaflets, chord tendineae, or papillary muscles. Malformed tricuspid valves are known to result in variable degrees of regurgitation, leading to right atrial overflow and ventricular eccentric hypertrophy. Differential diagnosis includes myocarditis, tricuspid valve endocarditis, tricuspid endocardiosis, tricuspid valve prolapse and right ventricular dysplasia, right ventricular enlargement with tricuspidal regurgitation due to pulmonary insufficiency, and arrhythmogenic right ventricular cardiomyopathy. Signs of heart murmurs (irregular sounds of the heart) on clinical examination may indicate an irregular blood flow pattern, and imaging tests may be necessary for assessing the presence and severity of any lesions. The epidemiologic, clinical, and pathological findings were consistent with those of tricuspid valve dysplasia. 

INSTRUMENTAL MARKERS OF THE RIGHT VENTRICLE FUNCTIONAL CONDITION WITH TISSUE DOPPLER IN CHILDREN WITH TETRALOGY OF FALLOT AFTER SURGICAL CORRECTION AND THEIR CONNECTION WITH VALVULAR FUNCTION OF PULMONARY ARTERY

2020 ◽  
Vol 73 (11) ◽  
pp. 2364-2369
Author(s):  
Veronika M. Dudnyk ◽  
Olha O. Zborovska ◽  
Yuilia V. Vyzhga ◽  
Vladymyr P. Popov ◽  
Valentyn S. Bakhnivskyi
Keyword(s):  
Right Ventricle ◽  
Tetralogy Of Fallot ◽  
Myocardial Dysfunction ◽  
Systolic Dysfunction ◽  
Pulmonary Regurgitation ◽  
Functional Condition ◽  
Tissue Doppler ◽  
Surgical Correction ◽  
Heart Cycle ◽  
The Right

The aim: To improve efficacy of the right ventricle functional condition evaluation in children with tetralogy of Fallot after surgical correction by estimation of instrumental markers of myocardial dysfunction. Materials and methods: We completely examined 35 children with tetralogy of Fallot after their surgical correction at the age of 3 – 17 years. For all the patients was presented tissue doppler. We evaluated peak myocardial velocities of right ventrical in different phases of the heart cycle (S, E`, A`), tricuspid annular plane systolic excursion (TAPSE), diastolic myocardial velocities ratio (E/E`), peak myocardial velocity during isovolumic contraction (IVV), isovolumic relaxation time (IVRT). Results: All children of the study group had pulmonary insufficiency of different severity with main predominance of mild pulmonary regurgitation (20 patients, 57,14±8,36 %). Children with tetralogy of Fallot after surgical correction were admitted with: decreased TAPSE up to 1,39±0,28 cm, decreased S` up to 8,00±1,90 cm/s, and decreased IVV up to 5,69±0,95 cm/s that is significantly lower results of the healthy children. Severe pulmonary regurgitation usually followed by high chances of the right ventricle systolic dysfunction, exactly with: decresed TAPSE<1,5 cm (OR=0,500; 95% CI 0,323 – 0,775), S`<8,1 cm/s (OR=0,600; 95% CI 0,420 – 0,858) and IVV<5,9 cm/s (OR=0,250; 95% CI 0,117 – 0,534). As well we admitted significant decline of the velocities in earl and end diastole periods to compare with the results of the control group (E`= 12,11±1,22, A`= 4,56±0,92 cm/s (Р=0,009 and P=0.0002)), boost of the E/E` ratio – 7,96±2,33 (P=0.01) and decline of the RV IVRT up to 43,49±6,04 ms (P=0.017). Severe pulmonary regurgitation followed by high chances of the right ventricle systolic dysfunction development with TAPSE <1,5 cm (OR=0,500; 95% CI 0,323 – 0,775), S`<8,1 cm/s (OR=0,600; 95% CI 0,420 – 0,858) and IVV<5,9 cm/s (OR=0,250; 95% CI 0,117 – 0,534). As well we noticed high chances of the E/E`ratio > 6,0 in 1,5 times (95% CI 1,072 – 1,903) and decreased E` <12,2 cm/s (OR=0,200; 95% CI 0,083 – 0,481). Conclusions: Apart of clinical symptoms of the heart failure in children with tetralogy of Fallot after surgical correction markers of the right ventricle myocardial dysfunction are presented by indices of myocardial velocities, received during tissue doppler in different phases of the heart cycle.

Cardiovascular Physical Examination

2016 ◽  
pp. 53-58
Author(s):  
Kyle W. Klarich ◽  
Lori A. Blauwet ◽  
Sabrina D. Phillips
Keyword(s):  
Physical Examination ◽  
Right Ventricle ◽  
Wave Amplitude ◽  
Venous Pressure ◽  
Atrial Pressure ◽  
Right Atrial ◽  
Jugular Venous Pressure ◽  
Structural Disease ◽  
The Right ◽  
The Relationship

Jugular venous pressure reflects right atrial pressure and the relationship between right atrial filling and emptying into the right ventricle. Changes in wave amplitude may indicate structural disease and rhythm changes. Normal jugular venous pressure is 6 to 8 cm H2O. It is best evaluated with the patient supine at an angle of at least 45°. The right atrium lies 5 cm below the sternal angle, and thus the estimated jugular venous pressure equals the height of the jugular venous pressure above the sternal angle + 5 cm.

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