scholarly journals Retrospective review: congenital pulmonary artery stenosis in dogs (prevalence forms)

2021 ◽  
Vol 2021 (4) ◽  
pp. 15-20
Author(s):  
Vladislava Illarionova ◽  
Adelya Kadyrova
Keyword(s):  
Pulmonary Artery ◽  
Right Atrium ◽  
Congenital Heart ◽  
Myocardial Hypertrophy ◽  
Pulmonary Artery Stenosis ◽  
Axis Deviation ◽  
Severe Stenosis ◽  
The Right ◽  
Direct Dependency ◽  
Yorkshire Terrier

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.

scholarly journals Identification and characterization of a novel ELN mutation in congenital heart disease with pulmonary artery stenosis

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Cuilan Hou ◽  
Junmin Zheng ◽  
Wei liu ◽  
Lijian Xie ◽  
Xiaomin Sun ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Heart Disease ◽  
Pulmonary Artery ◽  
Molecular Mechanism ◽  
Protein Level ◽  
Congenital Heart ◽  
Artery Stenosis ◽  
Pulmonary Artery Stenosis ◽  
Functional Component ◽  
Functional Changes

AbstractCongenital heart defects, one of the most common birth defects, affect approximately 1% of live birth globally and remain the leading cause of infant mortality in developed countries. Utilizing the pathogenicity score and inheritance mode from whole exome sequencing results, a heterozygous mutation (NM_001278939.1: c.1939G>T, p.Gly647Ter) in elastin (ELN) was identified among 6,440 variants in a female proband born with an atrial septal defect accompanied by pulmonary artery stenosis. Results of RT-PCR showed that the mutation (NM_001278939.1: c.1939G>T, p.Gly647Ter) did not affect the expression levels of ELN mRNA but increased protein level. The content of ELN truncate (functional component) was significantly lower in both the intracellular and extracellular compartments after mutation. These results indicate that the ELN mutation (NM_001278939.1: c.1939G>T, p.Gly647Ter) affected the protein truncate, which may be a functional component of ELN and play crucial roles for this pedigree. Here we report of an ELN heterozygous variant associated with congenital heart disease accompanied with pulmonary artery stenosis, which is less common. Based on our results, we speculate that this may be the main molecular mechanism underlying the mutation-led functional changes, and propose that the decrease of ELN protein level may cause this pedigree vascular abnormality, especially pulmonary artery stenosis, and reinforce the view that ELN insufficiency is the primary cause of these vascular lesions. This may be the main molecular mechanism underlying the mutation-led functional changes. Thus, systematic analysis not only enables us to better understand the etiology of this disease but also contributes to clinical and prenatal diagnosis.

scholarly journals A Rare Case of Adult Aortopulmonary Window Combined with Anomalous Origin of the Right Pulmonary Artery from the Aorta Leading to Eisenmenger Syndrome

2021 ◽  
Vol 49 (1) ◽  
pp. 030006052098465
Author(s):  
Mingyue Cui ◽  
Binfeng Xia ◽  
Heru Wang ◽  
Haihui Liu ◽  
Xia Yin
Keyword(s):  
Pulmonary Artery ◽  
Rare Case ◽  
Congenital Heart ◽  
Respiratory Tract Infections ◽  
Anomalous Origin ◽  
Aortopulmonary Window ◽  
Eisenmenger Syndrome ◽  
Right Pulmonary Artery ◽  
The Right ◽  
Tract Infections

Aortopulmonary window is a rare congenital heart disease that can increase pulmonary vascular resistance, exacerbate left-to-right shunt and lead to heart failure and respiratory tract infections. Most patients die during childhood. We report a 53-year-old male patient with a large aortopulmonary window combined with anomalous origin of the right pulmonary artery from the aorta, with Eisenmenger syndrome and without surgery.

scholarly journals P686 Right atrial, right ventricular and pulmonary cement embolism: a rare complication of percutaneous vertebroplasty

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Akhunova ◽  
R Khayrullin ◽  
N Stekolshchikova ◽  
M Samigullin ◽  
V Padiryakov
Keyword(s):  
Pulmonary Artery ◽  
Right Ventricle ◽  
Right Atrium ◽  
Percutaneous Vertebroplasty ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Stable Condition ◽  
Right Atrial ◽  
Cement Embolism ◽  
The Right

Abstract A 68-year-old man was admitted to the hospital with complaints of pain in the lumbar spine. He had L5 disc herniation, Spinal stenosis of the L5 root canal - S1 on the right in the past medical history. Percutaneous vertebroplasty at the level of L3 and Th8 vertebral bodies was performed six months ago due to painful vertebral hemangioma. The man is suffering from arterial hypertension, receives antihypertensive therapy. During routine transthoracic echocardiography, a hyperechoic structure with a size of 9.5 x 0.9 cm was found in the right atrium and right ventricle. Chest computed tomography with contrast enhancement revealed signs of bone cement in the right atrium and right ventricle, in the right upper lobe artery, in the branches of the upper lobe artery, in the paravertebral venous plexuses. Considering the duration of the disease, the stable condition, the absence of clinical manifestations and disorders of intracardiac hemodynamics, it was decided to refrain from surgical treatment. Antiplatelet therapy and dynamic observation were recommended. Conclusion Percutaneous vertebroplasty is a modern minimally invasive surgical procedure for the treatment of degenerative-dystrophic diseases of the spine. However, the cement can penetrate into the paravertebral veins and migrate to the right chambers of the heart and the pulmonary artery. This clinical case demonstrates asymptomatic cement embolism of the right chambers of the heart and pulmonary artery after percutaneous vertebroplasty, detected incidentally during routine echocardiography. Abstract P686 Figure.

scholarly journals Novel technique of repairing right partial anomalous pulmonary venous connection with intact atrial septum using in situ interatrial septum as a flap in a 68-year-old-woman: a case report

2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Atsushi Morishita ◽  
Ikuo Hagino ◽  
Hideyuki Tomioka ◽  
Seiichiro Katahira ◽  
Takeshi Hoshino ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Right Atrium ◽  
Septal Defect ◽  
Surgical Repair ◽  
Interatrial Septum ◽  
Pulmonary Artery Hypertension ◽  
Right Heart ◽  
Intact Atrial Septum ◽  
The Right ◽  
Anomalous Pulmonary Venous Connection

Abstract Background Partial anomalous pulmonary venous connection draining into the right atrium with an intact atrial septum is a very rare clinical entity in the adult population. Partial anomalous pulmonary venous connection must be suspected as a differential diagnosis when the cause of right heart enlargement and pulmonary artery hypertension is unknown. Case presentation This study describes the surgical case of an isolated right partial anomalous pulmonary venous connection to the right atrium in a 68-year-old woman, who underwent tricuspid ring annuloplasty and right-sided maze procedure simultaneously. She had complaints of gradually progressing dyspnea on exertion. However, a diagnosis could not be established despite consultations at multiple hospitals for over a year. Right heart catheterization revealed severe pulmonary artery hypertension with a mean pulmonary artery pressure of 46 mmHg, step-up phenomenon of oxygen saturation at the mid-level of the right atrium with a pulmonary-to-systemic blood flow ratio of 2.4, and a pulmonary vascular resistance of 3.1 Wood Units. As medical treatment with pulmonary artery vasodilator therapy did not improve her symptoms, she underwent surgical repair. An atrial septal defect was created surgically with a curvilinear tongue-shaped cut. The right anomalous pulmonary veins were rerouted through the surgically created atrial septal defect into the left atrium with a baffle comprised of the interatrial septum flap, kept in continuity with the anterior margin and sutured while mobilizing the enlarged right atrium. The patient had an uneventful postoperative course and remains asymptomatic. Conclusions The described surgical technique could be considered an effective alternative for patients undergoing surgical repair for a partial anomalous pulmonary venous connection isolated to the right atrium. The indication for surgery must be judged on a case-by-case basis in these patients with prevalent systemic-to-pulmonary shunting.

scholarly journals P189 Duplication of the tricuspid valve (DOTV): case report of a rare congenital anomaly

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
S Moscatelli ◽  
G Trocchio ◽  
N Stagnaro ◽  
A Siboldi ◽  
M Derchi ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Atrial Flutter ◽  
Tricuspid Valve ◽  
Tricuspid Regurgitation ◽  
Right Atrium ◽  
Rare Condition ◽  
Systolic Pulmonary Artery Pressure ◽  
Severe Tricuspid Regurgitation ◽  
One Year ◽  
The Right

Abstract Introduction Tricuspid valve duplication is an extremely rare condition and in most of the cases it is associated with other congenital cardiac malformations. Because of its rarity, the clinical presentation and the management are not defined yet. Clinical Case We report the case of an 18 y/o caucasian male, who was admitted to our Hospital in February 2018 for rapid atrial flutter not responsive to medical therapy (propanolol and digossin). He had a pre-natal diagnose of ventricular septum defect (VSD) and tricuspid straddling. At 1 year of age he underwent pulmonary artery bandage and one year later VSD closure was performed. Blood test showed sub-clinic hypothyroidism, probably related to previous amiodaron therapy. A transthoracic echocardiogram was obtained. The right atrium (RA) was severely dilated and the atrial septum dislocated towards left ventricle (LV); two right atrioventricular valves (tricuspid valves) were detected: the ‘true’ tricuspid opening was inside the right ventricle, and an ‘accessory‘ opening was located inside the LV and severely regurgitant into the RA; the mitral valve was morphologically and functionally normal; both ventricles were dilated with preserved systolic function; systolic pulmonary artery pressure was not detectable. A Cardiac Magnetic Resonance clearly delineated the anomaly. Atrial flutter radio frequency transcatheter ablation was succesfully performed before corrective surgery. The regurgitant accessory tricuspid orifice was closed with an heterologous pericardial patch and a right reduction atrioplasty was also done. The post-operative course was uneventful and only a mild paraseptal tricuspid jet with LV to RA shunt was present at post op echocardiography. After one year follow-up the patient remained asymptomatic, without arrhythmia recurrence. Conclusion DOTV is an extremely rare condition that could be responsible of severe tricuspid regurgitation. At the moment, there are not sufficient data to establish the correct timing for surgical intervention. In our case, the presence of severe tricuspid regurgitation, right atrium dilatation, biventricular overload and atrial flutter guided the clinical management and suggested surgical correction. Abstract P189 Figure.

scholarly journals P689 Surgical treatment of a right heart thrombus-in-transit with massive bilateral pulmonary embolism

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C M Angelescu ◽  
I Hantulie ◽  
Z Galajda ◽  
I Mocanu ◽  
A T Paduraru ◽  
...  
Keyword(s):  
Pulmonary Embolism ◽  
Pulmonary Artery ◽  
Right Atrium ◽  
Intracardiac Thrombus ◽  
Severe Pulmonary Hypertension ◽  
Right Heart ◽  
Right Pulmonary Artery ◽  
The Right ◽  
In Transit ◽  
Right Heart Dysfunction

Abstract Introduction Right heart thrombi are rare, found in up to 20% of pulmonary emboli (PE), and associated with significantly increased mortality(1). A thrombus entrapped in a PFO is a rare form of right heart thromboembolism. Clinical Case A 73-year-old male patient who had dyspnea for 10 days, was transferred to our hospital for the surgical treatment of a cardiac tumor. We performed TTE which revealed a free floating, huge mass( measuring more than 8 cm long) in the right atrium, that protruded in the right ventricle, with high risk of embolization. Another smaller mass, attached to the interatrial septum. Severe right ventricular dysfunction and severe pulmonary hypertension were present. Contrast-enhanced computer tomography was performed, which revealed severe bilateral pulmonary artery emboli with complete occlusion of right pulmonary artery branch. Clinical and paraclinical data strongly suggested that the huge cardiac mass was a thrombus that originated from the lower extremity veins. TEE showed that the thrombus was entrapped through the PFO, with a smaller part in the left atrium and the biggest portion in the right atrium. The patient underwent an emergent on-pump surgical cardiac and right pulmonary artery embolectomy. The right atrium was opened and a huge intracardiac thrombus with a lengh of 14 cm extending from the coronary sinus, to PFO in the left atrium and also in the right ventricle was removed. The right branch of the pulmonary artery was opened and a large volume of clot- 9 cm long- was removed. The patient was removed from cardio-pulmonary by-pass (CPB) on high doses of norepinephrine and dobutamine and necessitated initiation of venous-arterial ECMO to support the severe right heart dysfunction. He was extubated after 10 days, with little improvement in the clinical status. Transthoracic echocardiography showed smaller right heart cavities), normal left ventricular function, but persistent severe RV dysfunction and severe pulmonary hypertension. In the thirteenth postoperative day, he installed cardio-respiratory arrest and he died. Discussion In this report we describe a case of a patient with a huge intracardiac thrombus, entrapped through a PFO, associated with massive pulmonary embolism, with late presentation in our hospital and severe refractory right heart dysfunction. He underwent successful embolectomy, which is a unique procedure in the treatment of an acute pulmonary embolism and entrapped thrombus in a PFO. Conclusion The treatment of choice for emboli-in-transit is controversial. In a recent review, surgical thromboembolectomy showed a trend toward improved survival and significantly reduced systemic emboli compared to anticoagulation. Thrombolysis in these patients may cause fragmentation of thrombus and systemic embolization, resulting in increased mortality. Management decisions should be made with multidisciplinary coordination and consideration of complicating factors such as PFO. Abstract P689 Figure. Extensive biatrial thrombus

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