scholarly journals P1700 Recurrent inflammatory myofibroblastic tumor of the right ventricle and pulmonary artery

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Akhunova ◽  
R Dzhordzhikiia ◽  
A Rafikov ◽  
E Paschenko ◽  
N Gizatullina

Abstract Introduction Inflammatory myofibroblastic tumor (IMT), also known as plasma granuloma, is a very rare tumor with varying biological behavior, from completely benign to malignant. IMT can occur in virtually every organ, but cardiac origin is extremely rare. IMT occurs mainly in children and young people. The mainstay of treatment is complete surgical resection of the tumor. We present a case of 28 year old white woman with no contributory medical and family history, who was admitted to hospital because of dyspnea and cough. The transthoracic echocardiography (TTE) showed elevated pulmonary artery systolic pressure up to 61 mm Hg and mild dilation of right chambers. Further assessment by computed tomography showed thrombosis of the main pulmonary artery (PA), narrowing its lumen by 90%, with spread to the lobar and segmental arteries of the left lung - signs of massive pulmonary embolism. During thromboendarterectomy revealed a mass, about 4,1 x 3,1 x 1,8 cm in size, with thrombotic overlays, locating at the right ventricular outflow tract (RVOT) and the pulmonary trunk. Histological examination and Immunohistochemistry studies was consistent with IMT with an intermediate potential of malignancy. A year after the operation, the patient became pregnant. Routine TTE showed low density mobile mass narrowing the pulmonary trunk. The transvalvular blood flow velocity was 2.4 m/s. Cardiac magnetic resonance (CMR) was consistent with echo findings. The pregnancy has been preserved, and after 36 weeks it was decided to perform the birth of a girl by means of a Cesarian section. After 2 months, due to an increase in the size of the tumor and the dyspnea, a reoperation was performed to remove the tumor lining the RVOT and intimately connected with the PA valve, excision of the PA valve. A bioprosthesis was installed. The tumor had an identical morphological picture. Conclusion This case report describes a rare recurrent cardiac inflammatory myofibroblastic tumor involving RVOT and PA. The prognosis after surgical treatment of IMT is usually favorable, but it is known that some tumors recur, and rarely they can even metastasize. The long-term outcome is not well established due to the rarity of these tumors. Abstract P1700 Figure.

1992 ◽  
Vol 2 (2) ◽  
pp. 179-183
Author(s):  
Deborah M. Friedman ◽  
John Fernandes ◽  
Monika Rutkowski ◽  
Delores Danilowicz

AbstractA common systolic ejection murmur of the neonate has been attributed to physiologic peripheral pulmonic stenosis. We investigated this auscultatory finding using duplex pulsed Doppler. Three groups of normal fuliterm neonates less than one week old were studied—10 without murmurs, 10 with grade 1/6 murmurs and nine with at least grade 2/6 murmurs. We measured the anatomical size and peak flow velocities in the main pulmonary artery and left and right branches, the peak velocity in the right ventricular outflow tract, and the bifurcation angle. Flow gradients were calculated as 4 (Vmax)2 Groups were compared by t-tests. A loud peripheral pulmonic stenosis murmur was associated with increased pulmonary artery velocities, with left pulmonary artery velocity the most discriminating variable (1.3 ± 0.29 vs 0.94 ± 0.19 m/s; p ≤ 0.05). Although the peak gradient never exceeded 12 mmHg, there was an increased gradient in the loud murmur group (8.7 ± 2.6 vs 5.7 ± 2.2 mmHg; p ≤ 0.05) which may even be underestimated by the lack of angle correction. The left pulmonary artery diameter was also larger in the loud murmur group, but there were no other anatomic or volumetric flow differences between groups. The soft murmur group could not be separated from normals. We conclude that Doppler techniques can confirm the physiologic basis of peripheral pulmonic stenosis murmurs.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Maria Drakopoulou ◽  
Konstantinos Stathogiannis ◽  
Konstantinos Toutouzas ◽  
George Latsios ◽  
Andreas Synetos ◽  
...  

Objective: Severe aortic stenosis leads to increased pulmonary arterial systolic pressure. A controversy still remains regarding the impact of persistent pulmonary hypertension (PHT) on prognosis of patients undergoing transcatheter aortic valve implantation (TAVI). We sought to investigate the impact of persistent PHT on 2-year all-cause mortality of patients with severe aortic stenosis following TAVI. Methods: Patients with severe and symptomatic aortic stenosis (effective orifice area [EOA]≤1 cm 2 ) who were scheduled for TAVI with a self-expanding valve at our institution were prospectively enrolled. Prospectively collected echocardiographic data before and after TAVI were retrospectively analyzed in all patients. Pulmonary artery systolic pressure was estimated as the sum of the right ventricular to the right atrial gradient during systole and the right atrial pressure. PHT following TAVI was classified as absent if <35 mmHg and persistent if ≥35 mmHg. Primary clinical end-point was 2-year all-cause mortality defined according to the criteria proposed by the Valve Academic Research Consortium-2. Results: Hundred and forty patients (mean age: 82±9 years) were included in the study. The primary clinical end point occurred in 17 patients (12%) during a median follow-up period of 2 years. Mean pulmonary artery systolic pressure was reduced in all patients following TAVI (45±9 versus 41±6 mmHg, p<0.01). Mortality rate was higher in patients with persistent PHT compared to patients with normal pulmonary artery systolic pressure following TAVI (26% versus 14 %, p<0.01). Patients that reached the primary clinical end point had a higher post procedural mean systolic pulmonary pressure (43±9 versus 39±6 mmHg, p=0.02). In multivariate regression analysis, persistence of PHT (OR: 2.51, 95% CI: 1.109-7.224, p=0.01) was an independent predictor of long-term mortality. Conclusions: The persistence of pulmonary hypertension after TAVI is associated with long term mortality. Identifying the population that will clearly benefit from TAVI is still need to be validated by larger trials.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Atsuko Kato ◽  
Christian Drolet ◽  
Shi-Joon Yoo ◽  
Andrew Redington ◽  
Lars Grosse-Wortmann

Introduction: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well known. We hypothesized that unilaterally increased pulmonary vascular resistance (PVR), resulting from lung compression by the enlarged and levorotated heart leads to greater PR in the LPA. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: This is a single-center retrospective analysis of 50 magnetic resonance studies in patients after TOF repair. Patients with more than mild discrete branch pulmonary artery stenosis were excluded. Blood flow was measured by phase-contrast velocity encoding within the branch pulmonary arteries. On the axial image with the largest total cardiac surface area, cardiac angle (α) between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured (Figure). Results: There was no difference in LPA and RPA diameters. The LPA showed significantly less total forward flow (p=0.04), smaller net forward flow (p=<0.001), and greater RF (p=0.001) than the RPA. Left lung area was smaller than the right (p<0.001). RVEDVi correlated with LPA RF (R=0.48, p<0.001), but not with RPA RF. Larger RVEDVi correlated with a larger α angle (R=0.46, p<0.001), i.e. a more leftward cardiac axis and with smaller left lung area (R=-0.58, p<0.001). LPA RF, but not RPA RF, correlated inversely with left lung area indexed to the left hemithorax area (R=-0.34, p=0.02). Conclusions: An enlarged and levorotated heart - as a result of PR - is associated with smaller left lung size, and augments diastolic flow reversal in the LPA, presumably via increased left PVR. By imposing a further volume load on the RV, LPA regurgitation may thus close a positive feed-back loop of PR and RV dilatation.


1981 ◽  
Vol 50 (1) ◽  
pp. 102-106 ◽  
Author(s):  
P. S. Barie ◽  
T. S. Hakim ◽  
A. B. Malik

We determined the effect of pulmonary hypoperfusion on extravascular water accumulation in anesthetized dogs by occluding the left pulmonary artery for 3 h and then reperfusing it for 24 h. The lung was reperfused either at normal left atrial pressure (Pla) or during increased Pla induced by a left atrial balloon. In each case the extravascular water content-to-bloodless dry weight ratio (W/D) of the left lung was compared with that of the right lung. The W/D of the left lung of 3.26 +/- 0.49 ml/g was not significantly different from the value of 2.87 +/- 0.37 for the right lung after the reperfusion at normal Pla. However, the W/D of the left lung of 5.10 +/- 0.38 ml/g was greater (P less than 0.05) than the value of 4.42 +/- 0.34 for the right lung after reperfusion at Pla of 25 Torr. This difference could not be prevented by pretreatment with heparin, suggesting that the increase in lung water content was not due to activation of intravascular coagulation secondary to stasis occurring during the occlusion. Because the left lung was more edematous than the right one, even though both lungs had been subjected to the same increase in Pla, the results suggest that a period of pulmonary hypoperfusion causes an increase in the interstitial protein concentration.


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.


Author(s):  
Joseph R. Nellis ◽  
Charles M. Wojnarski ◽  
Zachary W. Fitch ◽  
Nicholas A. Andersen ◽  
Joseph W. Turek

Pulmonary fibroelastomas are a rare primary cardiac tumor with less than 50 cases reported in the literature to date. We performed a minimally invasive valve-sparing tumor resection through a left anterior mini-incision (LAMI). The procedure was performed without cardiac arrest or aortic cross clamp, expediting postoperative recovery and allowing for an uncomplicated discharge on postoperative day 5. LAMI is a safe and reliable alternative to median sternotomy for patients requiring interventions on the right ventricular outflow tract and main pulmonary artery, including pulmonary fibroelastoma resection and pulmonary valve replacement when needed.


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