IgG4-Related Tumefactive Lesions at the Pulmonary Artery Causing Stenosis of Bilateral Primary Branches and Resultant Pulmonary Hypertension

Cardiology ◽  
2019 ◽  
Vol 143 (3-4) ◽  
pp. 136-144
Author(s):  
Mengwei Tan ◽  
Zhuodong Li ◽  
Hao Tang ◽  
Gaofeng Sun ◽  
Zhiyun Xu
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Multiple Organs ◽  
Igg4 Related Disease ◽  
Activity Assessment ◽  
Right Pulmonary Artery ◽  
Hemodynamic Disturbance ◽  
Left And Right ◽  
The Right

IgG4-related disease (IgG4-RD) is a newly recognized but rare entity involving multiple organs, with autoimmune pancreatitis, retroperitoneal mass, and the inflammation of glands being typical in most cases. IgG4-related perivascular lesions, although uncommon, have been increasingly reported in recent years. Diagnosis of IgG4-RD relies on comprehensive consideration of characteristic histopathological and immunostaining results, clinical and imaging findings, and serological results according to several widely recognized diagnostic criteria. This benign disorder frequently presenting tumefactive lesions should be distinguished from malignancy and other inflammatory mimics. Here we report a case of tumefactive mass at the bifurcation of the pulmonary trunk causing stenosis of the proximal left and right pulmonary artery (PA) and resultant pulmonary hypertension (PH). Bypass from the PA trunk to the right branch distal to stenosis was performed to resolve the obstructive hemodynamic disturbance and PH. Glucocorticoid monotherapy was performed after a diagnosis of definite IgG4-RD. Longitudinal disease activity assessment via imaging modalities, serological parameters, and IgG4-RD responder index verified no relapse during follow-up and the validity of the treatment strategy.

scholarly journals Pulmonary Artery Intimal Sarcoma: A Case Report

2016 ◽  
Vol 9 (1) ◽  
pp. 267-272 ◽  
Author(s):  
Joseph P. Kriz ◽  
Nabil A. Munfakh ◽  
Gregory S. King ◽  
Juan O. Carden
Keyword(s):  
Pulmonary Artery ◽  
Malignant Tumors ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Filling Defect ◽  
Intimal Sarcoma ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
Left And Right ◽  
The Right

Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung.

Anterior translocation of the right pulmonary artery for airway obstruction caused by pulmonary hypertension

2002 ◽  
Vol 74 (2) ◽  
pp. 569-571 ◽  
Author(s):  
Masaaki Yamagishi ◽  
Keisuke Shuntoh ◽  
Akiyuki Takahashi ◽  
Takeshi Shinkawa ◽  
Takako Miyazaki ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Airway Obstruction ◽  
Right Pulmonary Artery ◽  
The Right

Scimitar syndrome with absence of the right pulmonary artery: A case with volume-induced, reversible, left-sided pulmonary hypertension

1992 ◽  
Vol 13 (2) ◽  
pp. 119-121 ◽  
Author(s):  
Albrecht Beitzke ◽  
Gerfried Zobel ◽  
Bruno Rigler ◽  
J�rg Ingolf Stein ◽  
Christa Suppan
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Scimitar Syndrome ◽  
Right Pulmonary Artery ◽  
The Right

scholarly journals P1728 Pulmonary hypertension in a pregnant Women - a rare anatomical aetiology

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
J Grade Santos ◽  
F Ferreira ◽  
M Loureiro ◽  
A Almeida ◽  
A Pereira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Medical Therapy ◽  
Cardiac Mri ◽  
Left Pulmonary Artery ◽  
Severe Pulmonary Hypertension ◽  
Right Pulmonary Artery ◽  
History Of

Abstract A 32 year old female patient, with a medical history of an ill-characterized Pulmonary Arterial Hypertension associated with congenital heart disease, lost in the follow up with no medical therapy, attended an emergency department for a gynecological hemorrhage at 16 weeks of pregnancy. Due to high maternity mortality risk, informed consent was obtained, and termination of pregnancy was performed. She was then referred to our pulmonary hypertension center. At our center she had complains of fatigue with moderate intensity exertion, classified in a class II of the World Health Organization (WHO) classification, but was otherwise asymptomatic, with no history of dyspnea, angina or syncope. There was allusion to a self-limited episode of hemoptysis in the past. On physical examination she had an increased pulmonary component of the second heart sound, continuous heart murmur in left sternal border and no cyanosis (O2 peripheral saturation in the upper and lower limbs of 99% at room air). The performed echocardiograms (both transthoracic and transesophageal) showed an estimated systolic pulmonary artery pressure of 120 mmHg with severe right ventricular hypertrophy and systolic dysfunction. There was dilatation of the trunk and right pulmonary artery. The left pulmonary artery was not seen. Biochemical evaluation and viral serologies were unremarkable. The pulmonary function tests and the arterial blood gases were normal. Cardiac MRI demonstrated the presence of a right aortic arch and a right patent arterial duct. An anomalous origin of the left pulmonary artery from the ascending thoracic aorta could be noted. Associated congenital cardiac defects were excluded. A right heart catheterization confirmed the presence of severe pulmonary hypertension with mean pulmonary artery pressure of 86 mmHg and Pulmonary vascular resistance of 11 Wood Units. A large persistent arterial duct to the right pulmonary artery was confirmed with persistent left to right shunt. The left pulmonary artery was visualized when injection was performed in the aortic root. Coronary arteries were normally implanted. The patient was started on Sildenafil and Bosentan (later replaced by Macitentan due to hepatic toxicity). After 3 years of follow up, there was an improvement in symptoms and in the 6 minutes walking test, remaining in a low risk category and on a WHO class I. This case reports a very rare congenital abnormality identified in an adult patient. Despite the complex anatomy and severe pulmonary hypertension, the patient is reasonably well under medical therapy and close follow up. Abstract P1728 Figure. Cardiac MRI Cine Sequences

scholarly journals Right Pulmonary Artery Absence or Discontinuity in Ethiopian Toddler

2020 ◽  
Vol 30 (6) ◽  
Author(s):  
Henok Tadele ◽  
Etsegenet Gedlu
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Chronic Care ◽  
Severe Pulmonary Hypertension ◽  
High Grade Fever ◽  
Rare Congenital Abnormality ◽  
Right Pulmonary Artery ◽  
Diagnostic Work ◽  
Work Up

BACKGROUND፡ Unilateral pulmonary artery absence or discontinuity is a rare congenital abnormality. Its reported prevalence is 1 in 150,000 adults.CASE PRESENTATION: A 22-month-toddler girl was referred from a general hospital for cardiac follow-up to our hospital after diagnosis of severe pulmonary hypertension was made. She presented with generalized body swelling, cough, fast breathing and high-grade fever of four days duration. She had repeated severe chest infections. Echocardiography and chest computed tomography revealed right pulmonary artery discontinuity. Acute care was given and chronic care was established.CONCLUSION:Pulmonary artery discontinuity is a rare anomaly. It should be considered in any child presenting with recurrent chest infections/pulmonary hypertension. Appropriate and timely diagnostic work up will avoid misdiagnosis.

Surgical treatment of isolated unilateral absence of pulmonary artery with pulmonary hypertension

2021 ◽  
pp. 1-2
Author(s):  
Moyu Hasegawa ◽  
Shigemitsu Iwai ◽  
Kazuhiko Ishimaru
Keyword(s):  
Pulmonary Hypertension ◽  
Surgical Treatment ◽  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Left Pulmonary Artery ◽  
Single Stage ◽  
Artery Pressure ◽  
Medication Therapy ◽  
Right Pulmonary Artery ◽  
The Right

Abstract A one-and-a-half-month-old patient with isolated unilateral absence of the right proximal pulmonary artery with prominent left pulmonary hypertension was diagnosed using CT. Medication therapy was initiated first. Left pulmonary artery pressure decreased after the initiation of medication therapy, and single-stage reconstruction of the right pulmonary artery was performed. The patient is asymptomatic 14 months postoperatively.

Diverse experience with the CardioSEAL®and STARFlex®septal occluders

2004 ◽  
Vol 14 (4) ◽  
pp. 367-372 ◽  
Author(s):  
Sotiria C. Apostolopoulou ◽  
Cleo V. Laskari ◽  
Maria Kiaffas ◽  
John Papagiannis ◽  
Spyridon Rammos
Keyword(s):  
Pulmonary Artery ◽  
Arteriovenous Malformation ◽  
Initial Experience ◽  
Septal Defects ◽  
Atrial Septal Defects ◽  
Right Pulmonary Artery ◽  
The Right ◽  
Extracardiac Fontan ◽  
Ventricular Dimensions

Apart from closure of atrial septal defects, there is little information concerning the use of the CardioSEAL family of occluders in congenitally malformed hearts. We review here our initial experience using the CardioSEAL and STARFlex occluders in 12 patients aged 17.3 ± 11.2 years, with a range from 4 to 34 years. Of the patients, 5 had fenestrated extracardiac Fontan procedures, 5 had persistent patency of the arterial duct, 1 had a leak across a Mustard baffle, and the final patient had a huge pulmonary arteriovenous malformation. We implanted successfully 9 CardioSEAL, and 3 STARFlex occluders, with sizes from 17 to 40 mm. In one patient, the occluder embolized to the right pulmonary artery, from where it was retrieved through the catheter. In two patients, there was a trivial residual leak immediately after implantation, but no patient had a residual leak after 6 months of follow-up. We noted improved ventricular dimensions, without any fractures of the arms of the occluders, perforations, or disturbances of flow after 2.4 ± 0.9 years of follow-up. We have demonstrated, therefore, the versatility of the CardioSEAL and STARFlex occluders, which have been used safely and effectively to close a variety of intra and extracardiac communications other than atrial septal defects.

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