scholarly journals P1323 From a suspicion of pulmonary arterial hypertension to a rare form of Takayasu disease

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
K Wrona ◽  
M Polak ◽  
K Mizia-Stec
Keyword(s):  
Right Ventricle ◽  
Laboratory Tests ◽  
Continuous Wave ◽  
Retroperitoneal Fibrosis ◽  
Nyha Class ◽  
Pulmonary Stenosis ◽  
Arterial Blood ◽  
Serum Igg4 ◽  
Angio Ct ◽  
The Right

Abstract A 27-year-old female with a history of glomerulonephritis,with diagnosed systemic lupus and retroperitoneal fibrosis was admitted due to fast worsening dyspnoea(NYHA class IV) over the prior four weeks with suspicion for PA hypertension. In 2015, retroperitoneal fibrosis was diagnosed based on MR examination. With the methylprednisolone treatment a regression of changes was obtained. The patient was chronically treated with prednisone 7.5mg/day and mycophenolate mofetil 2g/d. When admitted to the ward, the patient was in a severe condition, with symptoms of resting dyspnea and blood oxygen saturation up to 80%. A loud systolic murmur was audible over the entire chest. In laboratory tests, the NT-proBNP values (11,000 pg/ml) were significantly elevated, arterial blood gases showed tendency to alkalosis, hypoxemia (PO2 45mmHg)with hypocapnia. Parameters of inflammation were moderately elevated (CRP 25mg/l, WBC 12,000/ul), with normal serum IgG4 concentrations. In TTE, significant dilatation of the right ventricle with left chamber compression were observed. Tricuspid ring dilation with no leaflets coaptation and a severe tricuspid regurgitation (Vmax 5m/s, RVSP 100mmHg) were found. In addition, the critical proximal pulmonary artery (PA) stenosis was revealed (left PA Vmax 3.3m/s, Pmax 42mmHg, right PA Vmax 5.2 m/s, Pmax 110mmHg). Angio-CT confirmed critical proximal constriction of both PAs with a flow channelup to 4 mm. Based on angio-CT and cardiac MR study the inflammatory etiology of stenoses was suspected and an empiric diagnosis of the Takayahu disease of pulmonary arteries was made. The patient was qualified for conservative treatment using prednisone and cyclophosphamide. Over the course of two months,2.8 g of cyclophosphamide were administered intravenously in three cycles with good response to the treatment. It resulted in regression of dyspnea and a improvement in exercise tolerance - to the NYHA class II, normalization of saturation. Laboratory tests showed reduction of NT-proBNP (1300 pg/ml). Control TTE revealed reduction in the size of the right ventricle and regression of PAs stenoses (left PA 11mm, right PA 7mm), also reduction of systolic flow gradients (left PA Vmax 3.1 m / s, Pmax 39 mmHg, right PA 4.1 m/s, Pmax 68 mmHg). Because of the significant improvement in patient’s condition,it was decided to continue the treatment. Legend for the Fig.1 A angio-CT, critical proximal constriction of both PA B TTE, parasternal short axis view, imflammatory infiltration C TTE, continuous wave doppler, flow velocity pattern in the PA stenosis D TTE PLAX, dilatation of the right ventricle with left chamber compression fig. 1 Abstract P1323 Figure. Fig.1 artety pulmonary stenosis

scholarly journals Repair of double-chambered right ventricle using right ventricular outflow chamber ventriculotomy via left intercostal thoracotomy under beating heart in two dogs

2014 ◽  
Vol 4 (1) ◽  
Author(s):  
Keiichi Sato ◽  
Isamu Kanemoto ◽  
Kippei Mihara ◽  
Koudai Kawase ◽  
Takuya Mori ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Continuous Wave ◽  
Clinical Signs ◽  
Major Complication ◽  
Beating Heart ◽  
Moderate Hypothermia ◽  
Continuous Wave Doppler ◽  
Novel Approach ◽  
The Right

Double-chambered right ventricle was diagnosed in two dogs, one of them a pup and the other full grown. Both dogs underwent surgery using the novel approach of right ventricular outflow chamber ventriculotomy via left intercostal thoracotomy with moderate hypothermia and moderate pump flow cardiopulmonary bypass under beating heart. No major complication occurred during and after the operation. On continuous wave Doppler echocardiography, the pressure gradient across the stenosis in the right ventricle decreased from 130 mmHg pre-operatively to 40 mmHg post-operatively at 1 year 5 months in the adult dog, and from 209 mmHg pre-operatively to 47 mmHg post-operatively at 1 year in the pup. Both dogs are active without clinical signs.

scholarly journals Immune Thromboses of the Pulmonary Vessels

1977 ◽  
Author(s):  
S.V. Andreev ◽  
A.A. Kubatiev
Keyword(s):  
Right Ventricle ◽  
Vascular Wall ◽  
True Nature ◽  
Pulmonary Vessels ◽  
Arterial Blood ◽  
Compensatory Hyperfunction ◽  
Antigenic Complex ◽  
Pulmonary Arterial ◽  
The Right

According to current concepts, pathogenesis of intravascular trombus formation is underlaid by three crucial factors: lesion of the vascular wall, impairment of hemodynamics and hemostatic properties of the blood. While admitting the important role of each of these factors, one should acknowledge that the true nature of thrombosis is much more complicated and does not always fit the framework of this triad. In our experiments on rabbits, it was demonstrated that the thrombotic process in the basin of the pulmonary vessels could be successfully reproduced even in intact animals under conditions of disturbed immune homeostasis. A distinctive feature of immune thrombosis of the pulmonary vessels was a generalized lesion of the microcirculatory bed, gradual increase in the thrombotic masses and involvement of the major branches of the pulmonary vessels (PV). Morphologically, a picture of lymphoid-cellular infiltration and localization of the antigenic complex in the affected PV was revealed. As a result of progressive decrease in the pulmonary arterial blood circulation and increasing resistance in the system of the lesser circulation there were noted, already during the first hours after the onset of the capillary thrombosis, a compensatory hyperfunction of the right ventricle of the heart which was manifested in its highly increased contractility, higher levels of cyclic AMP and phosphorylation potential. At a later period, however, the compensatory possibilities of the right ventricle of the heart failed to overcome this resistance with resulting incompetence of the organ starting to develop within 2-3 days.

Delayed enhancement imaging in a contemporary patient cohort following correction of tetralogy of Fallot

2014 ◽  
Vol 25 (7) ◽  
pp. 1268-1275 ◽  
Author(s):  
Uta Preim ◽  
Philipp Sommer ◽  
Janine Hoffmann ◽  
Jana Kehrmann ◽  
Lukas Lehmkuhl ◽  
...  
Keyword(s):  
Left Ventricle ◽  
Right Ventricle ◽  
Tetralogy Of Fallot ◽  
Nyha Class ◽  
Left Ventricular ◽  
Delayed Enhancement ◽  
Volume Index ◽  
Delayed Enhancement Imaging ◽  
The Right ◽  
Myocardial Scars

AbstractObjectiveTo test the hypothesis that myocardial scars after repair of tetralogy of Fallot are related to impaired cardiac function and adverse clinical outcome.MethodsA total of 53 patients were retrospectively analysed after repair of tetralogy of Fallot. The median patient age was 20 years (range 2–48).Cardiac MRI with a 1.5 T magnet included cine sequences to obtain volumes and function, phase-sensitive inversion recovery delayed enhancement imaging to detect myocardial scars, and flow measurements to determine pulmonary regurgitation fraction. In addition, clinical parameters were obtained.ResultsAn overall 83% of patients were in NYHA class I. All patients with the exception of 2 (96%) had pulmonary insufficiency. Mean ejection fraction and end-diastolic volume index were 46% and 128 ml/m2 for the right ventricle and 54% and 82 ml/m2 for the left ventricle, respectively. Excluding enhancement of the septal insertion and prosthetic patches, delayed enhancement was seen in 11/53 cases (21%). Delayed enhancement of the right ventricle was detected in 6/53 patients (11%) and of the left ventricle in 5/53 patients (9%). The patient group with delayed enhancement was significantly older (p=0.003), had later repair (p=0.007), and higher left ventricular myocardial mass index (p=0.009) compared with the group without delayed enhancement.ConclusionsThis study reveals that scarring is common in patients after surgical repair of tetralogy of Fallot and is associated with older age and late repair. However, there was no difference in right ventricular function, NYHA class, or occurrence of clinically relevant arrhythmias between patients with and those without myocardial scars.

The Impact of Chronic Pulmonary Regurgitation on the Right Ventricle: Pulmonary Stenosis vs. Tetralogy of Fallot

2008 ◽  
Vol 17 ◽  
pp. S198
Author(s):  
Rajesh Puranik ◽  
Phillip Lurz ◽  
Wendy Norman ◽  
Alessandra Frigiola ◽  
Phillip Bonhoeffer ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Tetralogy Of Fallot ◽  
Pulmonary Stenosis ◽  
Pulmonary Regurgitation ◽  
The Right ◽  
The Impact

scholarly journals Individualized Surgical Reconstruction of the Right Ventricle Outflow Tract in Double Outlet Right Ventricle With Mirror Image-Dextrocardia

2021 ◽  
Vol 9 ◽  
Author(s):  
Wangping Chen ◽  
Chukwuemeka Daniel Iroegbu ◽  
Xia Xie ◽  
Wenwu Zhou ◽  
Ming Wu ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Right Ventricular ◽  
Pulmonary Stenosis ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Double Outlet Right Ventricle ◽  
Mirror Image ◽  
Outflow Tract ◽  
Surgical Reconstruction ◽  
The Right

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.

Origin of Both Great Arteries from the Right Ventricle and Pulmonary Stenosis

1962 ◽  
Vol 42 (4) ◽  
pp. 372-378 ◽  
Author(s):  
MORRIS J. LEVY ◽  
RICHARD DEWALL ◽  
LARRY P. ELLIOTT ◽  
LEO CUELLO
Keyword(s):  
Right Ventricle ◽  
Pulmonary Stenosis ◽  
The Right

Incomplete isolation of the left innominate artery from the right aortic arch: a rare case of double-outlet right ventricle with pulmonary stenosis

2019 ◽  
Vol 29 (5) ◽  
pp. 717-719
Author(s):  
Jun Sato ◽  
Atsuko Kato ◽  
Naoki Ohashi
Keyword(s):  
Right Ventricle ◽  
Aortic Arch ◽  
Rare Case ◽  
Surgical Repair ◽  
Pulmonary Stenosis ◽  
Innominate Artery ◽  
Double Outlet Right Ventricle ◽  
Right Aortic Arch ◽  
Female Infant ◽  
The Right

AbstractIsolation of the left innominate artery from the right aortic arch is a rare anomaly. Herein, we present an even rarer case of incomplete isolation of the proximal left innominate artery with the right aortic arch in a 3-month-old female infant with a double-outlet right ventricle and pulmonary stenosis. Surgical repair at 6 months of age was successful, leading to the restoration of adequate flow in the left arm.

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