Septal Leaflet Of Tricuspid Valve Research Articles

PO-04-078 WHEN THE Q RUNS AWAY FROM P: A CASE OF EXTREME MOBITZ TYPE I

Advanced infranodal conduction disease usually presents with Mobitz 2 pattern and normal or mildly prolonged conducted PR intervals, whereas advanced AV node disease presents with Mobitz 1 pattern and more pronounced PR prolongation of conducted beats. AV node conduction properties can vary, with some patients demonstrating markedly prolonged PR intervals. We present a case of extreme PR prolongation and highly unusual Mobitz 1 patterns. To emphasize the variable presentation of AV node disease. N/A A 60 year old man with a history of Tetralogy of Fallot had serial repair surgeries over the years, including a Blalock Tausig shunt, Tetralogy of Fallot repair with pulmonary valve replacement, tricuspid valvuloplasty with chordal re-attachment, and closure of a cleft in the septal tricuspid valve leaflet. He had a dual chamber pacemaker for second degree AV block and RBBB. Due to recurrent tricuspid valve dysfunction, his pacemaker and leads were explanted at the time of repeat tricuspid valve surgery. Post-operatively, the surgical temporary epicardial pacing wires were not initially used, and various forms of asymptomatic Mobitz type 1 AV block were seen on serial ECGs. Careful scrutiny of the ECGs showed that the PR interval ranged from 640 ms to over 1300 ms. The markedly prolonged PR intervals led to highly unusual AV relationships, including overlapping PR intervals, as well as 1:1 and 2:1 AV conduction patterns that could only be properly deciphered in the context of other ECGs. When 2:1 AV conduction was seen, temporary pacing was implemented. He subsequently underwent bi-v pacemaker implantation to achieve proper AV and VV synchrony, in the context of concurrent LV dysfunction. AV conduction patterns can become complex in the setting of AV node disease, and more dramatically so in the context of congenital heart disease and valve surgery. Associations between specific P waves and QRS complexes should be ascertained to properly appreciate AV node disease severity. In the presented case, cursory ECG assessment led the ICU clinical team to conclude that AV conduction in the post-op setting followed a simple Mobitz 1 pattern with typical PR intervals. Therefore surgical temporary pacing wires were not utilized until the patient developed 2:1 AV conduction with more marked bradycardia. With careful ECG analysis, the severity of AV node dysfunction could be properly assessed and managed.

Laparotomy in a patient with Ebsteins anomaly- from OT to ICU.

Ebstein’s anomaly (EA) is a rare cyanotic congenital heart disease, affecting 1/200,000 live births and accounts for <1% of congenital heart diseases.1 First described by Wilhelm Ebstein in 1866 2 , the anomaly consists of downward displacement of the septal and posterior leaflet of tricuspid valve resulting in atrialization of the right ventricle and tricuspid regurgitation. The resulting functional impairment of right ventricle retard the forward flow of blood through right side of the heart, thereby causing a hypoplastic Right ventricle(RV), or RV outflow tract obstruction. This creates an accessory conduction pathways resulting in tachyarrythmias, pulmonary hypertension, intracardiac shunting. 3 Acute decompensated heart failure and sudden collapse are the most common cause of death.4

Laparotomy in a Patient with Ebsteins Anomaly- From OT to ICU

Ebstein’s anomaly (EA) is a rare cyanotic congenital heart disease, affecting 1/200,000 live births and accounts for <1% of congenital heart diseases [1]. First described by Wilhelm Ebstein in 1866 [2], the anomaly consists of downward displacement of the septal and posterior leaflet of tricuspid valve resulting in atrialization of the right ventricle and tricuspid regurgitation. The resulting functional impairment of right ventricle retard the forward flow of blood through right side of the heart, thereby causing a hypoplastic Right ventricle (RV), or RV outflow tract obstruction. This creates an accessory conduction pathways resulting in tachyarrythmias, pulmonary hypertension, intra-cardiac shunting [3].Acute decompensated heart failure and sudden collapse are the most common cause of death [4].

860 TRICUSPID REGURGITATION RELATED TO CARDIAC IMPLANTABLE DEVICES: A NEW ENTITY?

Abstract Background. The incidence of cardiac implantable electronic device (CIED) implantation is currently growing. Patients who develop a moderate to severe CIED-induced tricuspid regurgitation (TR) are at higher risk of pulmonary hypertension, right heart failure (HR) and have higher mortality. Transthoracic echocardiography (TTE) is the most common method for TR grading, while transesophageal echocardiography (TOE) with three-dimensional (3D) imaging is nowadays gaining importance to reconstruct valve morphology and to determine the TR pathophysiology. Case Summary. We present the case of an 83-year-old man with multiple comorbidities, affected by non-ischemic dilated cardiomyopathy, for which a biventricular defibrillator (ICD-CRT) was implanted in 2007 in primary prevention. Over the years, the patient has been a CRT-responder, with partial recovery of the ejection fraction (40% in 2022 versus 25%). In 2018 he had an episode of acute pulmonary oedema and ventricular fibrillation with ICD intervention; during that hospitalization, the right ventricular catheter (EC) was replaced due to dysfunction. In 2022, the patient presented to our hospital for anasarca due to right HF. TTE and TOE showed new onset of right heart dilation and dysfunction with severe TR, which was predominantly caused from lead impingement of the septal tricuspid valve (TV) leaflet and later from secondary dilation of the TV annulus. The patient responded well to medical therapy and was discharged. Discussion. CIED-related TR is getting more relevant since its growing prevalence and its prognostic impact, even if little is known about why some patients develop moderate-to-severe CIED-related TR. Clinicians should be aware of this entity and follow the patients with TR over the time with periodic echocardiograms. TOE and 3D imaging should always be performed to understand the mechanism of the TR and to select the optimal treatment. Currently, no guidelines nor randomized data are available in order to guide treatment in these patients. Figure 1:4-chamber views with and without color Doppler showing the ventricular EC passing the TV through with the presence of peri-catheter TR.Figure 2:trans-gastric TOE xPlane views showing how the ventricular EC determines a restrictive movement of the septal TV leaflet, causing the severe TR to form.

482 A CASE OF RIGHT SIDE INFECTIVE ENDOCARDITIS IN PATIENT WITH VENTRICULAR SEPTAL DEFECT

Abstract A 25-years old white female was admitted to our emergency department presenting with dyspnea, fever, cough and nausea. Her medical background included a small ventricular septal defect (VSD) (congenital) with a left to right shunt, micropolicistic ovary syndrome, Sars Cov 2 infection on January 2022, history of cutaneous infection after sternal piercing in the last three years. Patient showed onset of fever, headache and nausea since 20 days and had a history of ampicillin and cephalosporin usage for 15 days for comunitary pneumonia. Upon arrival in the emergency room, physical examination revealed temperature 38°C, crackles on down right lung fields, regular but tachycardic rhythm, 3/6 holosystolic murmur in the third left intercostal space, also skin redness around the piercing zone. Laboratory test showed increasing of WB (white blood cells), C-reactive protein (CPR) and procalcitonin. Because of worsening of respiratory conditions, CT chest was performed, showing tree in bud sign, with pulmonary pattern suggestive of staphylococcal “emboligenous-like” infectious state. According to patient's clinical history and CT results, she was referred to transthoracic echocardiogram (TTE) demonstrating the presence, on the right side of the small VSD, of a isoechoic large mass (20x 13 mm) with irregular margins attached to the right ventricular wall near the ostium of the VSD, compatible with vegetation; septal tricuspid valve leaflet involvement could not be ruled out. Methicillin-susceptible Staphylococcus aureus (MSSA) was detected six times from blood cultures; therapy with oxacillin 2 gr every 4 hours combined with daptomycin 750 mg daily was started. Cardiac MRI performed after few days, documented the infective involvement of the ventricular and atrial side of the septal leaflet with moderate tricuspid regurgitation (TR). Congenital heart disease (especially Tetralogy of Fallot, bicuspid aortic valve, aortic coarctation, ventricular septal defect) is a lifelong risk factor for infective endocarditis (IE). Size of VSD is generally not correlated with IE that is directly correlated with turbulent flow; tricuspid valve involvement is mostly seen in VSD, often complicated by pulmonary embolism. In this predisposing situation, skin infection of the piercing zone could have caused transient bacteremia which led to the formation of vegetations in the highest turbulence flow zone.

ECHOGENIC MASS ATTACHED TO TRICUSPID VALVE

SUMMARY
 A 5-day old female child with complaints of tachypnea, had TTE which revealed an echogenic mass on septal leaflet of Tricuspid valve. Based on echocardiographic features, it was diagnosed as Papillary fibroelastoma.
 CASE DESCRIPTION
 A 5-day old female child was referred for transthoracic echocardiographic study due to respiratory difficulty. The child was afebrile and had a respiratory rate of 60/min, heart rate 130 bpm, and blood pressure 80/50 mmHg. No murmur or added sound heard on CVS examination. TTE revealed a dense mass (Figure 1) attached to right atrial side of septal leaflet of Tricuspid valve by a small pedicle. The mass was mobile, prolapsing into right ventricle during diastole (Clips) without causing obstruction with size of 0.8 X 0.74 cm. Intracardiac masses attached to valves are usually Papillary fibroelastomas. Other possible differential diagnoses could be myxoma, vegetation, Lambl’s excrescence or secondary tumors etc. Based on the location and echo features of the mass (mobility, attachment to downstream side of valve, and frond like speckled appearance) the diagnosis of Papillary fibroelastoma was made.
 LEARNING POINTS
 
 Cardiac tumors are rare and mostly benign.
 The three most common primary tumors of heart, in order of frequency, are myxomas, lipomas and papillary fibroelastomas respectively. PFE constitute 7% - 9% of all.1
 PFE usually arise from valves of the heart and are mostly left-sided.2
 They are mobile, range from 0.5 – 2 cm in size and have a frond-like appearance. On echo, they are dense with a speckled interior and shimmering surface.3
 Being prone to embolization and thrombosis, their clinical course may not be benign, especially left sided.
 
 Clips
 
 Subcostal Clip: https://youtu.be/_xTZmx--WcA
 PS SAX View: https://youtu.be/lLh6D9Vn4Fw
 A4C View: https://youtu.be/_xZZvTIh0S8
 A4C RV Directed View: https://youtu.be/vJRyHrevftA
 
 QUESTIONS WITH ANSWERS
 
 Question 1: What is the most common complication of papillary fibroelastoma?
 Question 2: The behavior of right and left sided papillary fibroelastomas could be markedly different, Y/N?
 Question 3: What is the most important feature of papillary fibroelastoma for embolization?
 Question 4: PFE preferentially affects females, Y/N?
 
 Answers
 
 Question 1: Cerebral embolism
 Question 2: Yes
 Question 3: Mobility
 Question 4: No
 
 References
 
 Bouhzam N, Kurtz B, Doguet F, Eltchaninoff H, Bauer F. Incidental papillary fibroelastoma multimodal: imaging and surgical decisions in 2 patients. Tex Heart Inst J. 2012;39(5):731-5.
 Eidem BW, Cetta F, O’ Leary PW. Echocardiography in pediatric and adult congenital heart disease. Lippincott, Williams and Wilkins, Wolters Kluwer. 2010 p 355-361.
 Klarich KW, Enriquez-Sarano M, Gura GM, Edwards WD, Tajik AJ, Seward JB. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation. J Am Coll Cardiol. 1997;30(3):784-90.

Transcatheter ablation of the atrioventricular junction in refractory atrial fibrillation: A clinicopathological study

BackgroundCatheter ablation of the specialized atrioventricular junction (AVJ) with a right-side approach is an effective therapy for refractory atrial fibrillation with fast ventricular rate. Our aim is to assess the efficacy of the procedure in a single center experience and investigate the histologic findings of AVJ after catheter ablation. MethodsA) Analysis of AVJ ablation efficacy in a consecutive series of patients with refractory atrial fibrillation; B) Histopathologic study of the conduction system by serial section technique and clinical-electrophysiologic correlation in four patients who underwent AVJ ablation. ResultsA) Right-sided AVJ ablation was successful in all 87 consecutive patients (mean procedural time 19.2±17.9 min). Energy applications ranged from 1 to 27 (mean 5.8±5.1) with eight patients (9%) requiring > 15 applications. B) Fibrotic disruption of atrioventricular (AV) node and/or His bundle interruption was found in three cases with previous AVJ ablation. In the case requiring a left side approach, the compact AV node and common His bundle appeared undamaged whereas extensive fibrosis of the summit of the ventricular septum, branching His bundle and proximal bundle branches was found. Noteworthy, a continuity between the septal and anterior tricuspid valve leaflets was present. ConclusionOur data confirm that the ideal site for ablation of the specialized AVJ is the AV node. In selected cases with unsuccessful AV node ablation, a shift towards the His bundle is needed. A continuity between the septal and anterior leaflets of the tricuspid valve may protect the His bundle as to require multiple shocks and prolong the procedure.

The echocardiographic exam for the diagnosis of cardiac involvement in non-Hodgkin lymphoma in childhood

Clinical signs and symptoms of cardiac(Cd) involvement in non-Hodgkin lymphoma(NHL), is frequently undetected and diagnosis could be missed unless a routine echocardiographical investigation(echo) might be performed. To reveal the value and utility of echo for diagnose and follow up the Cd involvement in Nnhl n child, other than that determined by the specific treatment. Authors were studied 38 children: 21 males and 17 females, aged between 3month and 17 years with NHL), hospitalised. In all cases were performed: clinical exam, ECG, chest X-ray and echo. Cd involvement was proved in 6 cases (15, 8%); 5 cases (83%) T-cell and 1 case (17%) B-cell lymphoma; clinical signs on onset like astenia, dyspnea, cough, superior vena cava sdr have been assigned to the base disease. ECG: low voltage of QRS complexes and T waves. Chest X-ray: mediastinal involvement (5 pts) and massive right-side pleural effusion (1 case). Eco aspects: pericardial effusion (4) to cardiac tamponade (2); pericardial tumor (1 case). In 2 cases at first eco exam intra Cd masses: case 1 with a tumoral mass filling the right atrium(RA) with implantation base towards superior vena cava- confirmed later in the autopsy; case 2-tumoral mass extending from the free wall of the RA to the septal tricuspid valve leaflet, associated with an massive pericardial effusion. In the case 2 and in all cases with pericardial effusion on onset:at 1 year the echo turned to normality after chemotherapy. Because of high incidence of Cd involvement in NHL in child and antemortem diagnosis is often difficult, echo is necessary in all cases at the first exam, even if Cd involvement signs are missing, in order to prevent the evolution to Cd tamponade or other Cd emergency. Echo is the most important investigation to diagnose and follow-up the Cd involvement in NHL in child.

479 When 2 rights make a wrong

Abstract Background The presence of an intra-cardiac mass is always a cause for concern, with regards to not only aetiology, but also treatment of complications. We describe a case series of 2 right sided cardiac myxomas, where the first case described an unusual location for tumour occurrence, while the second case provided insights into complications of a cardiac myxoma. Methods The first case involved a 70 year old asymptomatic lady who was referred for an additional heart sound. A transthoracic echocardiogram (TTE) showed a large, mobile 1.5 X 1.2 cm mass, attached to the atrial surface of septal tricuspid valve leaflet, prolapsing in and out of the right sided chambers. This was confirmed on transoesophageal echocardiogram (TEE). Cardiac MRI (magnetic resonance imaging) showed a similar mass attached to the septal tricuspid leaflet with features consistent with a myxoma. She was referred to cardiothoracic surgery, and 2 lobulated tumours arising the septal tricuspid valve and adjacent posterior leaflet were seen. The tumours were resected and a bio-prosthetic tricuspid valve replacement implanted. Histology of the tumours showed myxomatous degeneration of tricuspid valve, consistent with cardiac myxoma. The second case was a 56 year old lady who had dyspnoea, pedal oedema and an elevated jugular venous pulse on examination. A TTE done showed a large 7 X 4 cm mass extending from the right atrium (RA) into the right ventricle (RV). The left ventricular ejection fraction was 35%. Cardiac MRI confirmed the presence of a large mass in the right ventricle that exerted pressure effects on the ventricular septum and RV anterior free wall. Intra-operatively, a large RA mass attached by a stalk to the fossa ovalis was seen. The mass was excised and histology was consistent with cardiac myxoma. A repeat transthoracic echocardiogram done 2 weeks later showed normalisation of the LVEF. See images below for more information. Conclusion While myxomas are the most common benign cardiac tumours, they occur less commonly in the right atrium, and much less so on the tricuspid valve. Clinical manifestations range from being completely asymptomatic, as in the 1st case, to non-specific constitutional symptoms such as fever or general malaise, and to life-threatening complications. These include embolism to the pulmonary circulation, causing sudden death, or to the systemic circulation through an intra-cardiac shunt, causing strokes. This risk is increased if the tumour is large, polypoidal and friable. Large tumours can also cause obstructive symptoms and heart failure. Thus timely diagnosis with multi-modality imaging tools, and definitive treatment with complete resection of the tumour are essential. Continued monitoring for recurrences of the tumour, which can occur in 1-5% of all cases, should be performed as well. Abstract 479 Figure. Right sided cardiac masses

Defining a New Normal: Apical Displacement of Septal Leaflet of Tricuspid Valve is Far Less than Reported in Literature

Defining a New Normal: Apical Displacement of Septal Leaflet of Tricuspid Valve is Far Less than Reported in Literature

A rare case of intracardiac fibrosarcoma with myxoid features inducing venous occlusion in a dog \u2013 a case report

BackgroundIn both humans and animals, cardiac fibrosarcoma is rare among primary cardiac malignant neoplasia. The overall prevalence of cardiac neoplasia in dogs is low, reported to be between 0.17% and 0.19% of hospital admissions. The aim of this report is to describe the clinical and pathological findings of a dog presenting signs of right sided congestive heart failure due to an intracardiac and venous obstructing mass, diagnosed by histopathology as cardiac fibrosarcoma with myxoid features.Case presentationA 7 years old male mix breed Husky weighing 23 kg was presented to our Veterinary Teaching Hospital the owner reporting weight loss, inappetence and exercise intolerance and on presentation exhibited breathlessness and an enlarged abdomen. A 5 minutes six leads electrocardiogram and cardiac ultrasonography were performed using standard, established techniques. Complete blood count, serum liver enzyme activities and renal parameters were assessed. Shortly after the cardiologic examination, the dog died and necropsy examination of the cardiovascular system revealed an elongated and branched mass attached dorsally to the endocardial insertion of the septal tricuspid valve leaflet. This mass extended retrogradely into the lumen of the cervical veins, obstructing the venous flow. Histological diagnosis of the mass was cardiac fibrosarcoma with myxoid features. Multiple metastases were found inside the lungs only.ConclusionThis is the first report describing a right cardiac fibrosarcoma with myxoid features and venous obstruction in a dog. Cardiac fibrosarcoma is a rare finding, however should be considered when an intracardiac mass is diagnosed.

Unusual Tissue on the Septal Leaflet of the Tricuspid Valve Identified as Intracardiac Bronchogenic Cyst in a New-born

Unusual Tissue on the Septal Leaflet of the Tricuspid Valve Identified as Intracardiac Bronchogenic Cyst in a New-born

Microscopic Topography of Atrioventricular Conduction System–A Feature of Potential Clinical Significance

ObjectivesProximity of atrioventricular (AV) conduction system to tricuspid, mitral and aortic valves renders it susceptible to injury leading to disturbances in patients undergoing valve operations and radiofrequency catheter ablations. Hence, the knowledge of topography of AV conduction system and its relations with surrounding cardiac structures is indispensible.MethodsIn 20 cases, a tissue block of 3×4 cm at AV septal junction was excised which included the borders of Koch's triangle. The block was divided into three equal segments by parallel vertical incisions made perpendicular to insertion of septal leaflet of tricuspid valve. Each segment was processed for Hematoxylin & Eosin and Gomori stain.ResultsThe upper pole of compact AV node was at the level of mitral valve leaflet in 50% cases. Lower pole of AV node was vertically above tricuspid septal leaflet and at the level of muscular IV septum in 100% and 66.7% cases respectively. The subendocardial depth of upper and lower pole of AV node was 1.45±0.6 mm and 0.92±0.3 mm respectively. Minimum subendocardial depth (0.68±0.2mm) was seen at the center of compact AV node. Penetrating bundle of His was seen at the level of tricuspid septal leaflet just deep to it, while Branching bundle of His was 1.98±1.5 mm below the tricuspid septal leaflet. AV nodal extensions began at the level of mitral valve leaflet while its inferior extension ended 2.92±1.3 mm above tricuspid septal leaflet.ConclusionPrior knowledge of distances of AV conduction system from the surrounding landmarks might help in surgical incision planning, safe suture placement and successful device insertion.Support or Funding InformationFrom Director PGIMER, Chandigarh, India.

Gross right heart dilatation secondary to Ebstein's anomaly.

Cardiac imaging is essential for the diagnosis and follow-up individuals with congenital heart disease (CHD). Currently echocardiography is the first-line investigation, although no single imaging technique is sufficient for complete evaluation of cardiac anatomy and physiology in these patients. Increasingly, adults with CHD are referred for cardiac magnetic resonance (CMR) imaging. The high spatial resolution, functional assessment capabilities and excellent reproducibility of CMR make it a desirable investigation for CHD [1]. Ebstein's anomaly is a rare (incidence ≈1 in 200 000 live births) congenital heart disorder characterized by malformations of the tricuspid valve (TV) and right ventricle (RV). The hallmark features are clearly demonstrated in this CMR study. They are: Malposition of the posterior and septal TV leaflets in the walls of the RV with enlargement, fenestration and/or tethering of the anterior leaflet. Dilatation of the true atrio-ventricular junction and downward displacement of the functional TV annulus. Atrialization of the RV [2]. In the four-chamber view (Fig. 1A), the grossly dilated right heart is clearly evident, with an atrialized RV and dilated tricuspid annulus. The enlarged anterior TV leaflet is well visualized (red arrow), as is the hypoplastic septal leaflet (yellow arrow). The LA and LV are small, owing to compression from the volume-overloaded right heart. Figure 1: (A) CMR four-chamber cine view. Seen here is the grossly dilated right heart, with an atrialized RV and dilated tricuspid annulus. The enlarged anterior TV leaflet is well visualized (red arrow), as is the mislaid and hypoplastic septal leaflet (yellow ... The massively dilated atrial and ventricular portions of right heart are visualized in the short-axis cine view (Fig. 1B). Again the tricuspid valve septal leaflet is seen (red arrow) and the pale jet of tricuspid regurgitation is notable during systole. The intraventricular septum flattened during diastole consistent with right-sided volume overload and there is slow filling of the LV consistent with ventricular–ventricular interaction.

Tricuspid valve remodelling in functional tricuspid regurgitation: multidetector row computed tomography insights

Multidetector row computed tomography (MDCT) may help to understand the underlying mechanisms of functional tricuspid regurgitation (TR), a highly prevalent valve disease with novel transcatheter therapies under development. The purpose of the present study was to assess the geometrical changes of the tricuspid valve in patients with functional TR using MDCT and to correlate these changes with the TR grade assessed with echocardiography. In 114 patients undergoing transcatheter aortic valve implantation (47 men, age 81 ± 8 years), including 33 (28.9%) patients with TR ≥ 3+, the tricuspid valve and right ventricle (RV) were geometrically analysed with 320-slice MDCT. The antero-posterior and septal-lateral diameters, perimeter and area of the annulus, degree of tethering of the anterior, septal and posterior tricuspid valve leaflets, and RV volumes and ejection fraction were assessed and subsequently correlated with TR grade in multivariate models. Patients with pacemaker or implantable cardioverter defibrillator leads were excluded.Patients with TR ≥ 3+ had larger tricuspid annulus area (1539.7 ± 260.2 vs.1228.4 ± 243.5 mm(2), P < 0.001), larger septal and anterior leaflet angles, and larger RV end-systolic volumes (93.2 ± 29.8 vs. 64.2 ± 23.6 mL, P < 0.001) compared with patients with TR < 3+.The antero-posterior tricuspid annulus diameter was independently correlated with TR ≥ 3+ (odds ratio 1.35; 95% confidence interval 1.07-1.69, P = 0.010), after adjusting for estimated pulmonary pressure and RV end-systolic volume. In patients with TR ≥ 3+, MDCT demonstrated larger tricuspid annulus and RV dimensions and pronounced tethering of the anterior and septal tricuspid leaflet. The antero-posterior annulus diameter was independently correlated with the grade of functional TR.

Ebstein malformation associated with left atrium myxoma

A 62 year old lady, diagnosed with Ebstein malformation 12 years ago, presented to clinic with severe fatigue, breathlessness, chest pain, palpitations, and loss of weight. She had clear evidence for irregular rhythm, rales at the basal lung fields and clinical signs of severe right sided failure. A 12 lead ECG showed atrial fibrillation with wide QRS complex (130 ms) and right bundle-branch block. Echocardiography showed septal tricuspid valve leaflet displacement of 1.6 cm into the right ventricle (Figure 1&2). The right atrium was enlarged, 70 x 110 cm in diameter, with moderate tricuspid regurgitation and estimated pulmonary hypertension of 90 mmHg. In addition, there was an echo dense mass (26 x 30 mm) attached to the left atrial side of the interatrial septum, with morphologic appearance consistent with atrial myxoma. According to our knowledge, this case of such combination seems the first reported in the literature.

Anesthetic management of a patient with restrictive cardiomyopathy and ebstein anomaly for femoral hernia repair

Ebstein anomaly along with restrictive cardiomyopathy is a rare entity characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet along with the restriction of diastolic filling of ventricles. If patients with this anomaly present to us along atrial fibrillation and liver dysfunction due to right-sided heart failure, it would be a challenge for anesthesiologist to give anesthesia. Herein, we are providing a brief review of the clinical presentation and anesthetic consideration in these types of patients if they present for noncardiac surgeries.

Successful device closure in a congenital Gerbode defect.

A 22-year-old man was admitted to our department due to mild exertional dyspnea for 2 months. He had no other cardiac symptoms and his medical history was unremarkable. 4/6 systolic murmur was heard at the left sternal border and at the apex. Electrocardiogram revealed sinus rhythm with negative T waves in leads V1 to V4. Two-dimensional transthoracic echocardiography (2D TTE) revealed a clear systolic jet across a defect (7 mm) between the left ventricular outflow tract (LVOT) and right atrium (RA) consistent with a Gerbode type defect located above the tricuspid septal leaflet (Figure 1A). Qp/Qs ratio, maximal velocity (Vmax) and trans-shunt pressure gradient were 2.1, 5.1 m/s and 103 mm Hg, respectively. Right ventricular systolic pressure (RVSP) was estimated at 51 mm Hg assuming a right atrial pressure of 15 mm Hg. The direction of the Doppler signal confirmed our diagnosis (Figure 1B). Furthermore, three-dimensional transthoracic echocardiography (3D TTE) also demonstrated a supravalvular connection between these chambers in detail. Due to the proximity of the defect to the septal tricuspid valve leaflet, the Amplatzer duct occluder (AGA Medical Corporation; Golden Valley, USA) provided a reasonable fit because it has no right-sided disk that might interfere with tricuspid valve motion. The defect size and trans-shunt pressure gradient were compatible with echocardiographic evaluation. The patient refused the surgery; therefore we suggested device closure. After the patient's consent, the defect was successfully closed percutaneously with an Amplatzer Duct Occluder (size: 10-8 mm) (Figure 1C). After the successful implantation, no complications or residual shunt were found. Figure 1 A – 2D TTE revealed tunnel-like appearance of Gerbode defect between LVOT and RA in parasternal short-axis view (see arrow). B – Color Doppler echocardiography demonstrated clear systolic jet directed from the LVOT to the RA (see arrow). ... The Gerbode defect is a rare form of ventricular septal defect (VSD) that allows for communication between the left ventricle (LV) and RA. The congenital form of the LV-RA shunt is uncommon and the incidence is 0.08% of all catheterized congenital defects. Anatomically, two types of Gerbode defect are defined, supravalvular (less common) and subvalvular, depending on whether the defect in the membranous septum is above or below the tricuspid valve. Shunting from the LV to the RA mainly occurs in systole, resulting in a high-velocity jet by spectral Doppler between these chambers [1–3]. The LV-RA shunt is traditionally corrected surgically, but device closure is more comfortable new method. Six case reports of successful transcatheter closure in adults have been reported in the literature. Five patients were over 60 years old, and one patient was 22 years old. Five of these cases were performed after mitral valve surgery, and one was performed after VSD repair. Three communications were closed with Amplatzer duct occluders (ADO), one with VSD, and two with atrial septal defect occluders. Residual shunt of only trivial to mild degree was noted in four of those 6 cases. There were no major complications. All reported cases of catheter-based closure seemed to have 100% survival based on reviewing these case reports [4].

Insights gained from three-dimensional imaging modalities for closure of ventricular septal defects.

Ventricular septal defects (VSDs) are the commonest group of congenital heart lesions, accounting for ≈30% of congenital heart defects in liveborn infants.1 Defects in the ventricular septum are classified according to their position when the ventricular septum is viewed from the right ventricle and these may vary in their location, size, and number.2,3 VSDs can occur as isolated lesions or as part of more complex disease, for example, transposition of the great arteries or double outlet right ventricle, where the location and size of such defects assume great importance in planning the optimal mode of surgical repair.4 In most cases, surgical closure remains the mainstay treatment for VSDs, but selected lesions may be amenable to catheter closure.5,6 Cross-sectional echocardiography is the most commonly used noninvasive diagnostic method to delineate the position of a VSD before surgery.7 Multiple sonographic cuts and cross-sectional sweeps of the region of interest are used to build a virtual reconstruction of the three-dimensional (3D) anatomy in the mind of the echocardiographer. However, for the surgeon who deals solely with 3D images there can remain an element of uncertainty when called to operate on the patient. The number, size, location of VSDs, and their relationship to adjacent structures are central for selecting whether the defects might be best closed via the tricuspid valve, via the semilunar valves, or via a ventriculotomy or whether these might not be readily approachable and a palliative pulmonary artery banding is desirable.8,9 In this review, we present key 3D echocardiographic projections and examples of different types of VSDs where the images have a direct effect on the approach taken to VSD closure. Comparison of 3D echocardiography (3DE) with other 3D imaging modalities is also described. Images are presented in an …

Spontaneous Tricuspid Leaflet Partial Patch Closure of a Muscular Ventricular Septal Defect

Spontaneous closure of congenital ventricular septal defect occurs commonly in infants and young adults. Muscular VSDs usually undergo spontaneous closure as a result of muscular occlusion. We report first time a rare spontaneous partial patch closure of a muscular ventricular septal defect by septal leaflet of tricuspid valve in a 52 years old female diagnosed by cardiovascular magnetic resonance imaging.