Septal Leaflet Of Tricuspid Valve Research Articles

GW24-e3091 The perioperative role of the echocardiography in left ventricular-right atrium shunt (Gerbode defect)

ObjectivesLeft ventricular-right atrium (LV-RA) shunt (Gerbode defect), is a rare type of ventricular septal defect. The purpose of this study was to discuss the role of the echocardiography in LV-RA...

Cardiac Magnetic Resonance Imaging Findings in a Patient with Ebstein’s Anomaly and Left Ventricular Dysfunction

Ebstein anomaly is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle and could be diagnosed in a routine transthoracic echocardiography exam. We reported a young man with right sided heart failure symptoms who was found to have biventricular failure with suspected apical displacement of septal leaflet of tricuspid valve. For better evaluation, cardiac magnetic resonance imaging (CMR) was performed and nicely proved the diagnosis of Ebstein's anomaly with late intramyocardial enhancement of both left and right ventricles. CMR has the potential for providing information while echocardiography cannot, especially regarding the right ventricle and related myocardial problems, and Ebstein’s anomaly with decreased left ventricular (LV) systolic function.

Ebstein Anomaly

Ebstein anomaly (EA) is a rare congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, with atrialization of the right ventricle and variable degree of malformation and displacement of the anterior leaflet. Patients can have a variety of symptoms related to the anatomic abnormalities of EA and their hemodynamic effects or associated structural and conduction system disease. Most frequently cyanosis, palpitations, fatigue and dyspnea can be observed. In severe forms edema and ascites. Treatment of EA is complex and depends of the severity of the disease itself and the effect of accompanying congenital structural and electrical abnormalities. Options of treatment include medical therapy, radiofrequency ablation, and surgical therapy. Main aim of the surgical therapy is to correct the underlying tricuspid valve, right ventricular abnormalities and any associated intracardiac defects if exist. Palliative procedures and cardiac transplantation can be considered in most sever EA patients. JRCD 2013; 1 (4): 5–9

Recurrent Transient Ischemic Attacks in a 48-Year-Old Patient With an Undiagnosed Ebstein's Anomaly: A Case Report

Ebstein ’ s anomaly is a rare congenital malformation of the heart characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialisation of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. Its presentation depends on the severity of the anomaly. Mild forms are often undiagnosed until adulthood due to its benign course. Importantly, interatrial communications are present in 80% of the patients with EA through which a paradoxical embolism may occur whenever a right-to-left shunt exists. The authors describe a case of a 47-year-old white man that presented with acute recurrent transitory ischemic brain attacks and progressive exertional dyspnea. A brain CT scan revealed infarcts in multiple vascular territories. Echocardiographic studies revealed an Ebstein ’ s anomaly with a right-to-left shunt through an interatrial communication and severe right heart failure. Doppler study of the cervical and extremities ’ vessels was negative for a possible thrombotic source. A diagnosis of transient ischemic attacks presumably secondary to paradoxical embolism with unknown source was established. The patient was successfully submitted to heart surgery and improvement of symptoms and heart function was observed. At follow-up he remains well and without further neurological events. doi:10.4021/jmc439w

Recurrent Transient Ischemic Attacks in a 48-Year-Old Patient With an Undiagnosed Ebstein’s Anomaly: A Case Report

Ebstein’s anomaly is a rare congenital malformation of the heart characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialisation of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. Its presentation depends on the severity of the anomaly. Mild forms are often undiagnosed until adulthood due to its benign course. Importantly, interatrial communications are present in 80% of the patients with EA through which a paradoxical embolism may occur whenever a right-to-left shunt exists. The authors describe a case of a 47-year-old white man that presented with acute recurrent transitory ischemic brain attacks and progressive exertional dyspnea. A brain CT scan revealed infarcts in multiple vascular territories. Echocardiographic studies revealed an Ebstein’s anomaly with a right-to-left shunt through an interatrial communication and severe right heart failure. Doppler study of the cervical and extremities’ vessels was negative for a possible thrombotic source. A diagnosis of transient ischemic attacks presumably secondary to paradoxical embolism with unknown source was established. The patient was successfully submitted to heart surgery and improvement of symptoms and heart function was observed. At follow-up he remains well and without further neurological events. J Med Cases. 2012;3(1):54-59 doi: https://doi.org/10.4021/jmc439w

Prospective comparison of echocardiography versus cardiac magnetic resonance imaging in patients with Ebstein’s anomaly

Ebstein's anomaly (EA) is primarily diagnosed by echocardiography. The purpose of this study was to compare echocardiography and magnetic resonance imaging (MRI) in EA. Data from cardiac MRI and echocardiography were prospectively collected from 16 patients with EA. Imaging data also were compared with intraoperative findings. Information provided by MRI and echocardiography were comparable for left ventricular size and function, tricuspid valve repairability, qualitative assessment of right-sided cavities, and visibility of septal and anterior tricuspid valve leaflets. The posterior tricuspid valve leaflet and tricuspid valve fenestrations were better visualized with MRI; associated heart defects were equally recognized, apart from small shunts that tended to be more readily diagnosed with echocardiography. Quantification of right-cavity size and right ventricular ejection fraction was possible only with cardiac MRI. The degree of tricuspid valve regurgitation was underestimated by echocardiography (2 patients) and by MRI (4 patients) when compared with intraoperative assessment. When evaluating EA, echocardiography and MRI provide complementary data. For visualization of the posterior tricuspid valve leaflet and quantitative assessment of right ventricular size and function, MRI is preferable. For appropriate risk stratification in EA, both MRI and echocardiography should be performed before cardiac surgery.

Sudden cardiac death in Ebstein's malformation due to a cardiac haemangioma

A previously well 22-year-old man suddenly collapsed during a football game. Prolonged resuscitation of 50 min was unsuccessful. Pathological examination demonstrated Ebstein's malformation with apical displacement by 30 mm of the septal and posterior tricuspid valve leaflets ( Panel A ), and rotation of the septal leaflet into the right ventricular outflow tract (RVOT). The anterior …

A Rare Type of Gerbode Defect

A Gerbode defect is a left ventricle to right atrial communication. The type I defect (direct, acquired) results in a direct shunt through the atrioventricular part of membranous septum, while a type II (indirect, congenital) defect results in an indirect shunt through a perimembranous ventricular septal defect (VSD) and a defect in the septal tricuspid valve leaflet. We report a rare type of Gerbode defect wherein a small perimembranous VSD is completely covered by an elongated sail-like anterior tricuspid leaflet forming an aneurysm and directing the shunt into right atrium.

A Case of Septic Pulmonary Embolism Associated with MRSA Infective Endocarditis

Septic pulmonary embolism is the process in which an infected thrombus becomes detached from its site of origin and lodges in a pulmonary artery, and is usually associated with infective endocarditis, especially right-sided, or infection-associated with indwelling catheters, peripheral septic thrombophlebitis, and periodontal diseases, etc. Here, we report a case of septic pulmonary embolism associated with tricuspid valve infective endocarditis. A 23-year-old female was admitted to our hospital, due to fever, sore throat, and myalgia. In her past medical history, she had undergone a surgical operation for closure of a ventricular septal defect, but was informed that the operation resulted in an incomplete closure. The initial chest radiograph demonstrated multiple rounded, parenchymal nodules in various sizes; several nodules had central lucency suggesting cavitations. Echocardiography demonstrated a large vegetation attached to the septal tricuspid valve leaflet, extending from right ventricular inflow tract to outflow tract. Computed tomography of thorax revealed bilateral peripheral nodules and wedge-shaped consolidation at various sizes, mostly accompanied by cavitations.

Ebstein Anomaly Associated With Left Ventricular Noncompaction

A 56-year-old woman with gradually progressing exertional dyspnea was referred to our hospital. She had a known diagnosis of Ebstein anomaly and patent foramen ovale since childhood. One year previously, she had presented with a transient ischemic attack that was thought to be secondary to a paradoxal embolism through the patent foramen ovale, so an Amplatzer occluder had been implanted. Clinical examination revealed a normal sinus rhythm at 56 bpm and a New York Heart Association class II dyspnea without cyanosis. ECG (Figure 1) showed a first- degree atrioventricular block with a pattern of complete right bundle-branch block. Chest radiography (Figure 2) demonstrated mild cardiomegaly and clear lung fields. Two-dimensional echocardiography showed malposition of the anterior and septal tricuspid valve leaflets with grade 2 tricuspid regurgitation. The atrial septal occluder was in correct position without residual shunt. In …

P04.05: Atypical inferior vena cava and ductus venous blood flow velocity pattern in the fetus with Ebstein's anomaly: a case report

We present a case of Ebstein’s anomaly with tri-phasic inferior vena cava (IVC) and ductus venosus (DV) forward flow pattern. Pulsed tissue Doppler imaging (TDI) were performed to elucidate the relationship of the atypical venous flow and the ventricular wall motions. A 32 year-old nulliparous woman conceived a monochorionic-diamniotic twin pregnancy by IVF. On a routine fetal sonography at 14 weeks of gestation, Twin A showed cardiomegaly (cardio-thoracic ratio: 53%) and severe tricuspid regurgitation (TR). Another twin was sonographically unremarkable. With advance of gestation, fetal echocardiography on Twin A revealed right atrial enlargement, severe holosystolic TR (1.3m/sec), the enlarged sail-like anterior leaflet of tricuspid valve (TV), and retrograde pulmonary artery and ductal flow in the absence of pulmonary regurgitation. Posterior and septal leaflets of TV were not detected. For sonographic findings stated above, Ebstein’s anomaly and pulmonary atresia was prenatally diagnosed. Pulsed Doppler examination showed tri-phasic IVC and DV forward flow pattern. Pulsed TDI recording of the right atrioventricular valve ring showed abnormal shortening after S wave from late systolic phase to isovolumic relaxation phase of the cardiac cycle. This abnormal shortening synchronized with the second wave of IVC and DV forward flow, and the left ventricle showed the same shortening. There were no sign of fetal arrhythmia or hydrops fetalis. A Cesarean section was performed at 36 weeks of gestations. Twin A was a female infant weighing 2290g with Apgar scores of 2 and 6 at 1 and 5 minutes, respectively. Postnatal diagnosis was the same as prenatal assessment. Starnes operation was performed at 6th day after birth. But she died at the 7th day because of cardiac failure. In conclusion, we detected tri-phasic IVC and DV forward flow pattern in the fetus with Ebstein’s anomaly. We speculate that the abnormal ventricular motion caused the atypical venous flow pattern.

Multiple accessory pathways, dual AV nodal physiology, non-compacted myocardium and patent foramen ovale in a patient with Ebstein's anomaly: Report of a case

Ebstein's anomaly is a rare congenital cardiac disease initially described by Wilhelm Ebstein in 1866. The primary pathology involves significant apical displacement of the septal tricuspid valve leaflet and the presence of a redundant, elongated, anterior tricuspid valve leaflet. This congenital malformation has multiple known associated morphological and electrophysiological cardiac pathologies. In the present article, we report, to the best of our knowledge for the first time in the literature, a case of Ebstein's anomaly with multiple accessory bypass tracts, dual AV nodal physiology, non-compacted left ventricular myocardium and a patent foramen ovale. Indeed, complete evaluation of the left heart is required in cases of Ebstein's anomaly to accurately assess left ventricular myocardial structure.

Ventricular Septal Defect With Secondary Left Ventricular–to–Right Atrial Shunt Is Associated With a Higher Risk for Infective Endocarditis and a Lower Late Chance of Closure

Although ventricular septal aneurysm may diminish or even close the shunt through the ventricular septal defect (VSD), developing a left ventricular-to-right atrial (LV-RA) shunt may be unfavorable. This study sought to clarify this issue on the basis of an extended observation of such patients. Sixty-eight patients (1201 patient-years) who had small perimembranous VSD and LV-RA shunt and were not operated on before 6 years of age were studied. The onset age of LV-RA shunt was 5.8 +/- 3.3 years, with clinical improvement later observed in 23 (34%). The murmur disappeared and showed spontaneous closure in 5 (7%). Seven episodes of infective endocarditis occurred in 6 (8.7%, or 58 per 10000 patient-years), with 2 receiving surgery. Another 4 received surgery during follow-up. With echocardiography, aneurysmal transformation involving the anterior and septal leaflets of tricuspid valve (double sac) was found in 56 (85%), whereas only the septal leaflet (single sac) was involved in 10. Patients with double sac were less likely to show improvement, whereas patients who had superior QRS axis and were female showed clinical improvement more frequently. VSD with secondary LV-RA shunt is associated with a higher risk for infective endocarditis but still has a low chance for late improvement and even closure.

Tricuspid Endocarditis in an Adult Patient with Ebstein’s Anomaly who has a Residual Pacemaker Lead

Ebstein's anomaly is defined as an apical displacement of the attachment of the septal tricuspid valve leaflet from the right atrioventricular annulus that exceeded 1.2 cm in length. Patients with Ebstein's anomaly are known to have a high potential for developing arrhythmia, in the vast majority, of the tachycardia type. Infective endocarditis is characterized by ulcerovegetational lesions that result from the graft of a microorganism, usually bacterial, on the valvuler endocardium (native valve endocarditis) or on a prosthesis (prosthetic valve endocarditis). Ebstein's anomaly with tricuspid regurgitation is also thought to be a predisposing condition for infective endocarditis. In this case, we report a patient who presented with atrial flutter and infective endocarditis due to residual pacemaker lead and Ebstein's anomaly.

A Rare Tricuspid Anomaly

54-year-old Mexican American woman was admitted to the Erie County Medical Center, Buffalo, NY, with the general symptoms of tiredness, weight loss with loss of appetite, leg weakness, and low blood pressure (80/60 mm Hg). The patient denied any paroxysmal nocturnal dyspnea, orthopnea, or leg edema. She did have some facial swelling, a skin rash on both hands, and joint swelling, which was consistent with a previous diagnosis of polymyositis and dermatomyositis. The patient also had evidence of septic arthritis of the left elbow. Physical examination revealed extensive wheezing and a grade 2/3 systolic ejection murmur, heard predominantly over the tricuspid valve area. Laboratory workup revealed pancytopenia and an elevation in liver function tests. All the markers for autoimmune disease were negative. Biopsies and computerized tomographic scans performed to search for visceral malignancy were negative. Blood cultures grew methicillin-sensitive Staphylococcus aureus. Electrocardiography revealed normal sinus rhythm with evidence of right atrial enlarge

Surgical treatment on residual shunt after repair of ventricular septal defect

To summarize the experience in surgical treatment of residual shunt after repair of ventricular septal defect and investigate the position of the residual shunts. Between January 1979 and May 2003, re-operations on residual shunt after repair of ventricular septal defect were performed in 37 patients with congenital heart disease including ventricular septal defect, tetralogy of Fallot, double outlet right ventricle in 19, 17 and 1 patients, respectively. It accounted for 0.21% (37/18000) of open heart operations performed during these years. The patients included 26 males and 11 females with age from 3 months to 53 years (mean 16 +/- 12 years). The residual shunt was diagnosed by postoperative murmur and echocardiography. Twenty-six cases were repaired with patch and 11 cases were closed directly with mattresses sutures. Two patients (2/37, 5%) died within 48 hrs postoperatively. The results in other 35 patients followed up after surgery from 3 months to 15 years were satisfactory. Most of the residual shunts occurred in base of septal leaflet of tricuspid valve, the second and the first transfer suture respectively. Effects of reoperations on residual shunts were satisfactory.

Tetralogy of Fallot: Total Correction

he events that give rise to the c1evelol)iiieiit of the T constellation of lesions that we refer to as tetralogj of Fallot are still micertain. Regardless of the true teleological cause, it is hel1)ful for the surgeon to approach this defect as resulting from anterocephalad deviation of the ventricular outlet septum. "monology with sequellae." The resulting malalignment ventricular septal defect (VSD), aortic override, ancl right ventricular outflow tract ol~struction are thus easily understood. The right ventricular hypertrophy can he appreciated as secondary to the VSD and right ventricular outflow tract ohstructioii. Right ventricular outflow obstruction is increased by hypertrophy of the anterior limb of the septal ]land, anomalous anterior muscular bands, and outlet septum as well as pulmonary valve and arterial anomalies (Fig I). Classically the VSD is circular, juxtaaortic, and nonrestrictive and results from the malalignment of the parietal extension of the infundibular and trabecular septae. As such, the defect lies between the infundibular septum and is cradled by the anterior and posterior limbs of the septal band (Fig 11).

Morphological Aspects of Ebstein's Anomaly in Adults

The essence of the Ebstein's malformation is that the tricuspid valve leaflets do not attach normally to the valve annulus, and the effective orifice is displaced downward into the right ventricular cavity at the junction of the inlet and trabecular components of the right ventricle. Only the septal and posterior leaflets are displaced and divide the right ventricle into two portions. The inlet portion is usually integrated functionally with the right atrium ("atrialized portion"), while the other, including the trabecular and outlet portions, constitutes the functional right ventricle. The proximal atrialized right ventricle often has a wall thinner than the distal functional right ventricle, due to partial congenital absence of myocardium. An atrial septal defect is present in more than one-third of hearts, and the majority of the remainder has a patent foramen ovale resulting in a right-to-left shunt. The downward displacement of the septal tricuspid valve leaflet is associated with discontinuity of the central fibrous body and septal atrioventricular ring, thus creating a potential substrate for accessory atrioventricular connections and ventricular pre-excitation making the patient at risk of sudden death. Angiography has demonstrated that a significant number of patients with Ebstein's anomaly also have morphofunctional abnormalities of the left ventricle, which may be explained by increased fibrosis in the left ventricular wall and ventricular septum as demonstrated by histological studies. Regarding embryology, the leaflets and tensile apparatus of the tricuspid valve are believed to be formed mostly by a process of delamination of the inner layers of the inlet zone of the right ventricle. The downward displacement of the leaflets in Ebstein's anomaly suggests that delamination from the inlet portion failed to occur.

Morphologic spectrum of ebstein's malformation: Revisitation relative to surgical repair

Objective: Our aim was to elucidate the morphologic spectrum of Ebstein's malformation of the tricuspid valve with regard to diagnosis and the feasibility of surgical repair, in the light of the currently favored reconstructive techniques. Methods: We examined 23 autopsied hearts. Taking the displacement of the septal and mural leaflets of the abnormal tricuspid valve as our diagnostic criterion, we focused subsequently on the location of the distorted valvular orifice and the attachment and formation of the anterosuperior leaflet. We also assessed the dimensions of the components of the right ventricle relative to the plane of the displaced valvular orifice. Results: In all hearts, the septal and mural leaflets were hinged at various points within the inlet of the right ventricle. In many cases, however, these leaflets were virtually absent. The plane of the effective tricuspid valvular orifice was displaced anterosuperiorly to varying degrees. In the most severe forms, the valvular mechanism took the form of a 1-leaflet valve. The length of the functional right ventricle when compared with the left ventricle ranged proportionally from 0.6 to 1.1 (mean, 0.9). Conclusions: Ebstein's malformation is much more than simple “downward displacement” of the leaflets. In essence, the valvular orifice is formed within the ventricular cavity at the junction of the atrialized inlet and functional ventricular components. When surgical intervention becomes necessary, it is essential to make a detailed assessment of both valvular and ventricular abnormalities. (J Thorac Cardiovasc Surg 1999;117:148-55)

Mimics of Ebstein’s anomaly

The purpose of this study was to determine the most discriminating clinical and echocardiographic features that are most helpful in correctly identifying Ebstein’s anomaly of the tricuspid valve from other causes of tricuspid regurgitation. Ebstein’s anomaly is an uncommon malformation of the tricuspid valve with diagnostic echocardiographic features. Other cardiac disorders associated with tricuspid valve regurgitation and predominate right-sided heart chamber enlargement can be misdiagnosed as Ebstein’s anomaly. All patients who were referred to our institution between 1982 and 1995 with the diagnosis of Ebstein’s anomaly but were found to have other abnormalities of the tricuspid valve or right ventricle were identified. Their clinical, echocardiographic, and surgical records were reviewed retrospectively. Twenty-two patients (12 males and 10 females), aged 7 to 68 years (mean 33 years), were referred to our institution with the diagnosis of Ebstein’s anomaly but were found to have another abnormality that mimicked clinical and diagnostic features of Ebstein’s anomaly. The most common initial symptom was exercise intolerance (13 [59%] patients) followed by atrial arrhythmia (seven [32%] patients). Two patients had cyanosis. Three patients had paroxysmal and six had chronic atrial fibrillation/flutter. Cardiomegaly on chest x-ray film was noted in 18 (82%) patients. Referral diagnosis of Ebstein’s anomaly had been made by echocardiography (12 patients), cardiac catheterization (four patients), both techniques (five patients), and echocardiography and magnetic resonance imaging (one patient). All 22 patients had predominate right atrial and right ventricular enlargement, and 18 (82%) of 22 patients also had right ventricular dysfunction. However, Ebstein’s anomaly was confidently ruled out with repeat comprehensive echocardiography at our institution by establishing (1) absence of significant apical displacement of the septal tricuspid valve leaflet (≥8 mm/m 2 ) and (2) lack of a redundant, elongated, anterior tricuspid valve leaflet in all 22 patients (100%). All had significant tricuspid regurgitation caused by tricuspid valve dysplasia (nine patients), tricuspid valve prolapse (four patients), trauma (four patients), right ventricular dysplasia (three patients), endocarditis (one patient), and annular dilation caused by free pulmonary regurgitation (one patient). In all 15 patients who subsequently underwent surgery (tricuspid valve repair [seven patients] or replacement [eight patients]), the absence of Ebstein’s anomaly was confirmed. Echocardiographic absence of the characteristic degree of displacement of the septal leaflet of the tricuspid valve (≥8 mm/m 2 ) and the presence of a nonelongated, nonredundant anterior tricuspid valve leaflet consistently excluded the diagnosis of Ebstein’s anomaly. Under such circumstances, other anomalies of the tricuspid valve or right ventricle were consistently identified. Recognition of the mimics of Ebstein’s anomaly had important surgical implications. (Am Heart J 1997;134:508-13.)