pulmonary artery stenosis
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Qi-Xian Zeng ◽  
Tao Yang ◽  
Qun-Ying Xi ◽  
Zhi-Hui Zhao

Abstract Therapeutic effect evaluation is based on morphology performed by angiography conventionally. QFR is a new angiography-based tool for vascular assessment of functional severity. We report the first QFR examination in the field of Takayasu's arteritis patient with pulmonary artery stenosis and pulmonary arterial hypertension. It seems simple and practicable during off-line pulmonary artery intervention assessment.

2021 ◽  
Vol 2021 (4) ◽  
pp. 15-20
Vladislava Illarionova ◽  
Adelya Kadyrova

Objective: to determine the occurrence and clinical characteristics of different types of PS (pulmonary stenosis) in dogs Reseach tasks: to identify the breed predisposition to PS, to study the clinical, echocardiographic and electrocardiographic characteristics of various types of PS and to analyse their dependence on the severity of the heart defect. Materials and methods: a retrospective analysis of Biocontrol veterinary clinic medical records throughout six years (2014–2020). Cardiological examination of dogs presented to the primary ambulatory appointment included physical examination, echocardiography using the Philips HD15 ultrasound system, radiography and electrocardiography. Animals: 31 dogs with isolated pulmonary artery stenosis (17 males and 14 females). Breeds: French Bulldog, English Bulldog, Yorkshire Terrier, German Spitz, American Pit Bull Terrier, Staffordshire Terrier, Toy Terrier, Cane Corso, Whippet, Biewer Yorkshire Terrier, Chihuahua, Entlebucher, German Boxer, East European Shepherd, American Bully. Results and discussion: 43 dogs with a PS were examined from 1 January 2014 to 31 December 2020. Of these, 31 dogs (72 %) with isolated PS and 12 dogs (28 %) with a combination of PS with other congenital heart defects. The most common combinations were PS with aortic stenosis (25 %) and PS with ventricular septal defect (25 %). The most common form of isolated PS was type A valve stenosis (68 %). Severe stenosis prevailed (58 %). The most common breeds were French Bulldogs (22.6 %), English Bulldogs (16.1 %) and Yorkshire Terriers (9.7 %). Males were more prevalent (55 %). 61 % of animals from the group of dogs with severe PS visited clinic because of signalment, and syncope was the most frequently reported symptom (28 %). The symptoms associated with congenital heart defect were not identified in the group of animals with moderate and mild degrees of PS. ECG showed that 100 % of dogs were diagnosed with sinus rhythm. Electrical right axis deviation of the heart was detected in 45 % of dogs. Direct dependency was between the severity of the stenosis and the severity of electrical right axis deviation. According to echocardiographic studies — 100 % of animals with severe and moderate stenosis and 14% with mild stenosis were diagnosed with concentric, eccentric or mixed forms of right ventricle myocardial hypertrophy, enlargement of the right atrial. Direct dependency is founded between the severity of stenosis, the type of right ventricle hypertrophy and the size of the right atrium. The more severe stenosis, the more mixed form of hypertrophy; increase of the right atrium was recorded in dogs with mixed right ventricular hypertrophy. Post-stenotic dilatation of the trunk and branches of the pulmonary artery was determined in all dogs with single right coronary artery type R2A. Conclusions: PS is most commonly found in French Bulldogs. The most common type of PS is type A valvular stenosis in the form of an isolated defect. Severe PS is prevalent. There is direct dependency is between the severity of the defect and the severity of right ventricular myocardial hypertrophy, the large size of the right atrium and electrical right axis deviation of the heart.

2021 ◽  
pp. 1-6
Dan Ozaki ◽  
Hidenori Endo ◽  
Ryosuke Tashiro ◽  
Koichiro Sugimura ◽  
Shunsuke Tatebe ◽  

<b><i>Background:</i></b> Moyamoya disease (MMD) and peripheral pulmonary artery stenosis (PPAS) are relatively rare and demonstrate steno-occlusive vascular lesions in different organs. Genetic studies identified <i>RNF213</i> polymorphism c.14576G&#x3e;A (rs112735431) as a susceptibility variant for East Asian MMD. <i>RNF213</i> polymorphism c.14576G&#x3e;A is further associated with various vascular lesions of other organs. In this study, we aimed to clarify the incidence and clinical manifestations of PPAS in MMD patients and analyze the correlation between <i>RNF213</i> genotype and PPAS. <b><i>Methods:</i></b> This retrospective case-control study investigated the association between <i>RNF213</i> polymorphism and PPAS in 306 MMD/quasi-MMD patients, reviewing the medical charts and imaging records of consecutive patients with MMD admitted from January 2015 to December 2020. <b><i>Results:</i></b> PPAS was observed in 3 MMD/quasi-MMD patients (0.98%, 3/306). <i>RNF213</i> polymorphism c.14576G&#x3e;A was determined for all 306 MMD/quasi-MMD patients. The incidence of PPAS in <i>RNF213</i>-wildtype, <i>RNF213</i>-heterozygote, and <i>RNF213</i>-homozygote MMD/quasi-MMD patients was 0% (0/101), 0.5% (1/200), and 40% (2/5), respectively. The association between PPAS and homozygote polymorphism of <i>RNF213</i> c.14576G&#x3e;A was statistically significant in MMD/quasi-MMD patients (<i>p</i> = 0.0018). In all cases, pulmonary artery hypertension due to PPAS was evident during their childhood and young adolescent stages. Surgical indications for MMD were discouraged in 1 case due to her severe cardiopulmonary dysfunction. <b><i>Conclusions:</i></b> The homozygote variant of <i>RNF213</i> polymorphism c.14576G&#x3e;A can be a potential predisposing factor for PPAS in MMD/quasi-MMD patients. Despite the relatively rare entity, PPAS should be noted to determine surgical indications for MMD/quasi-MMD patients.

2021 ◽  
Vol 79 (9) ◽  
pp. 1046-1047
Mateusz Polak ◽  
Marek Grabka ◽  
Wojciech Wróbel ◽  
Iwona Woźniak-Skowerska ◽  
Katarzyna Mizia-Stec

2021 ◽  
Vol 73 (1) ◽  
Saud Bahaidarah ◽  
Jameel Al-Ata ◽  
Naif Alkhushi ◽  
Ahmad Azhar ◽  
Zaher Zaher ◽  

Abstract Background Ductal stenting is the preferred method of securing adequate pulmonary blood flow in patients with duct-dependent pulmonary circulation. The main limitation in most centers is the difficult vertical tubular or convoluted ducts that represent real challenges to interventional pediatric cardiologists. We present our experience in patent ductus arteriosus (PDA) stenting with some technical tips to overcome difficulties, especially in stenting tortuous or long tubular ducts. This study was conducted on all patients with cyanotic congenital heart disease who underwent PDA stenting between January 2011 and December 2018. Results We attempted to stent the PDA in 43 patients, with a success rate of 93% (40 patients) and only one procedural mortality. There was also one stent migration that needed to be treated with urgent surgery. Three-fourths of the patients had difficult ductal morphology and origin. One stent was used to cover the PDA in 27 patients (62.8%), two stents were used in 13 (30.2%), and three stents were used in 2 patients (4.6%). In-stent stenosis rate was 12.5% (5 patients) and the development of progressive left pulmonary artery stenosis was seen in two patients (5%). Pulmonary artery growth was adequate in all patients. Conclusions PDA stenting is an effective method of palliation for patients with duct-dependent pulmonary circulation. It has low morbidity and mortality rates. Stenting difficult ducts have become more feasible with evolving materials and techniques.

2021 ◽  
pp. 097321792110367
Deepanjan Bhattacharya ◽  
Anit Kaur ◽  
Lesa Dawman ◽  
Karalanglin Tiewsoh

We report a 2-month-old girl, presenting with anasarca in the early infancy, and was diagnosed to have congenital nephrotic syndrome. In view of a systolic murmur, echocardiography was done which revealed ostium secundum atrial septal defect and branch pulmonary artery stenosis. Genetic analysis was suggestive of single base pair duplication, resulting into frame shift mutation of NPHS1 (nephrin) gene.

Qiang Zheng ◽  
Xiaoke Shang ◽  
Nianguo Dong ◽  
Jiawei Shi

Abstract Background COVID-19 continues to be a pandemic worldwide. Lung transplantation is the last option to increase life expectancy of end-stage COVID-19 patients. Branch pulmonary artery stenosis (PAS) is a rare complication after lung transplantation with an extremely poor prognosis. The current trend in the management of branch PAS is percutaneous balloon angioplasty and/or stent implantation, rather than high-risk reoperation with a lower success rate. Case summary The subject was a 54-year-old male with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection who underwent a double-lung transplantation. He suffered hypoxemia and right heart dysfunction following the operation. Right cardiac catheterization and pulmonary angiography examination revealed severe stenosis of the right branch pulmonary artery. Due to immunosuppression and reduced coagulation function, the patient underwent pulmonary artery balloon dilatation and stent implantation, and ultimately recovered well. Discussion The combination of balloon dilatation and stent implantation is a good alternative to reoperation for patients with COVID-19.

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