Branch Pulmonary Artery Research Articles

Anomalous bronchial artery origin - canary in the coal mine - for diagnosing chronic thromboembolic pulmonary hypertension

Anomalous bronchial artery origins may have clinical implications beyond their anatomical curiosity. In this case, the identification of such an anomaly led to the diagnosis of chronic thromboembolic pulmonary hypertension (CTEPH). A 49-year-old male with a history of recurrent deep vein thrombosis (DVT) and pulmonary embolism (PE) on anticoagulation presented with chest pain and shortness of breath. Laboratory analysis was remarkable for a troponin peak of 14.74 ng/ml, a brain natriuretic peptide level of 602 pg/ml and a D-dimer level of 0.62 µg/ml. Electrocardiogram showed non-specific ST elevation in the anterolateral and inferior leads. Computed tomography angiography (CTA) of the chest was positive for PE involving the right lower lobe pulmonary arterial tree. Echocardiogram showed reduced left ventricular function (ejection fraction 38%) and akinesis of the antero-apical and infero-apical segments. Cardiac catheterization revealed non-obstructive coronary arteries, and an anomalous origin of a right bronchial artery from the right coronary artery. The right bronchial hypertrophied as it supplied collateral flow to the occluded right pulmonary artery. This anomaly and the patient’s history of multiple DVT/PEs while on therapeutic levels of warfarin with near normal D-dimer levels raised suspicion for a false positive PE. Pulmonary angiogram revealed chronic occlusion in branches of the right pulmonary artery, mean pulmonary artery pressure of 36 mmHg and no acute thrombus. Ventilation-perfusion scan confirmed the diagnosis of CTEPH. The patient underwent successful pulmonary thromboendarterectomy and subsequently had normalization of mean pulmonary artery pressure. This case underscores the importance of a comprehensive diagnostic approach, and consideration of alternative explanations for imaging findings, that unveiled the diagnosis of a complex and life-threatening condition such as CTEPH.

Outcomes of Neonates Born with Symptomatic Tetralogy of Fallot and Absent Ductus Arteriosus

ObjectiveTo test the hypothesis that neonates with symptomatic tetralogy of Fallot (TOF) and absent ductus arteriosus (ADA) have worse clinical outcomes compared with those with a ductus arteriosus (DA), and that this difference is driven by those born with ADA and with critically deficient pulmonary blood flow (CDPBF). Study designWe performed a retrospective, multicenter cohort study of neonates who underwent intervention for symptomatic TOF comparing death and reintervention between subjects with and without a DA identified on fetal echocardiogram or on echocardiogram performed in the first postnatal day. Exclusion criteria were: inability to define DA status, collaterals supplying pulmonary blood flow, atrioventricular septal defect, and absent pulmonary valve. We defined CDPBF as undergoing a procedure to augment pulmonary blood flow on the date of birth or extracorporeal membrane oxygenation prior to such a procedure. ResultsThe study cohort included 519 patients, among whom 11% had ADA. Patients with ADA were more likely to have a genetic syndrome and had smaller branch pulmonary artery size. In analyses adjusting for center, interventional treatment strategy, genetic syndrome, and minimum branch pulmonary artery size, ADA was associated with higher mortality risk (adjusted hazard ratio of 2.37 (95% CI:1.07,5.27; p=0.034). Seven patients had CDPBF (1.3% of the entire cohort and 12% of patients with ADA). ConclusionsA minority of symptomatic TOF neonates have ADA, which is associated with higher adjusted mortality risk compared with those with a DA. CDPBF appears to be a rare but important entity in this population.

Is it safe to discontinue anticoagulant after surgical treatment of varicose vein disease, which caused pulmonary embolism?

Superficial vein thrombosis, which is mostly caused by lower extremity varicose vein disease, can be complicated by pulmonary embolism. At the same time, the optimal duration of anticoagulant therapy for pulmonary embolism originated from varicose vein thrombosis is still under debate. On the one hand, the presence of varicose veins is considered a small risk factor for the development of venous thromboembolic events, which persistence determines an increased risk of relapse and requires prolonged anticoagulant therapy. On the other hand, elimination of varicose veins is associated with reduced risk of subsequent venous thromboembolic events. The article describes a clinical case of recurrent pulmonary embolism after surgical treatment of varicose vein disease, which caused primary pulmonary embolism. A 45-year-old patient suffering from left lower extremity varicose vein disease for 5 years was admitted to the intensive care unit with suspected pulmonary embolism. The further examination revealed signs of thrombotic occlusion of the segmental and subsegmental branches of the pulmonary arteries bilaterally and the middle lobe branch of the right pulmonary artery, as well as signs of the right-sided heart overload. In this case, pulmonary embolism was found to originate from thrombosis of the trunk of the great saphenous vein ofthe left lower extremity with a proximal border at the level of the lower leg. No signs of deep vein involvement were detected. The parenteral anticoagulant therapy initiated in the hospital was followed by switching to therapeutic doses of rivaroxaban. The signs of recanalization of involved veins were identified after 6 months of treatment, and it was decided to perform endovenous laser coagulation of the trunk of the great saphenous vein combined with mini-phlebectomy of varicose tributaries on continuous oral anticoagulant therapy. The rivaroxaban therapy was completed a month after intervention. However, 7 days later the patient was diagnosed with repeated symptomatic pulmonary embolism, which originated from thrombosis of the left popliteal vein. It was recommended to resume anticoagulant therapy of indefinite duration. The article discusses the issues of optimal duration of treatment for pulmonary embolism originated from thrombosis of superficial varicose veins, as well as the possible timing of completion of anticoagulant therapy after the intervention.

Machine Learning Quantification of Pulmonary Regurgitation Fraction from Echocardiography.

Assessment of pulmonary regurgitation (PR) guides treatment for patients with congenital heart disease. Quantitative assessment of PR fraction (PRF) by echocardiography is limited. Cardiac MRI (cMRI) is the reference-standard for PRF quantification. We created an algorithm to predict cMRI-quantified PRF from echocardiography using machine learning (ML). We retrospectively performed echocardiographic measurements paired to cMRI within 3months in patients with ≥ mild PR from 2009 to 2022. Model inputs were vena contracta ratio, PR index, PR pressure half-time, main and branch pulmonary artery diastolic flow reversal (BPAFR), and transannular patch repair. A gradient boosted trees ML algorithm was trained using k-fold cross-validation to predict cMRI PRF by phase contrast imaging as a continuous number and at > mild (PRF ≥ 20%) and severe (PRF ≥ 40%) thresholds. Regression performance was evaluated with mean absolute error (MAE), and at clinical thresholds with area-under-the-receiver-operating-characteristic curve (AUROC). Prediction accuracy was compared to historical clinician accuracy. We externally validated prior reported studies for comparison. We included 243 subjects (median age 21years, 58% repaired tetralogy of Fallot). The regression MAE = 7.0%. For prediction of > mild PR, AUROC = 0.96, but BPAFR alone outperformed the ML model (sensitivity 94%, specificity 97%). The ML model detection of severe PR had AUROC = 0.86, but in the subgroup with BPAFR, performance dropped (AUROC = 0.73). Accuracy between clinicians and the ML model was similar (70% vs. 69%). There was decrement in performance of prior reported algorithms on external validation in our dataset. A novel ML model for echocardiographic quantification of PRF outperforms prior studies and has comparable overall accuracy to clinicians. BPAFR is an excellent marker for > mild PRF, and has moderate capacity to detect severe PR, but more work is required to distinguish moderate from severe PR. Poor external validation of prior works highlights reproducibility challenges.

Case report: NSCLC with pulmonary embolism after treatment with almonertinib

Rationale: Almonertinib is the first independently developed third generation epidermal growth factor receptor tyrosine kinase inhibitors in China. It can be used not only for epidermal growth factor receptor (EGFR) sensitive mutation patients but also for T790M (+) resistant patients, and has better safety and survivability compared to the first and second generation EGFR-TKIs. The previous literature and case studies have reported significant treatment-related adverse reactions in non-small cell lung cancers patients treated with almonertinib, including elevated blood creatine phosphatase, prolonged cardiac QT interval, altered myocardial contractility, interstitial lung disease, eye disease, and hematological events. But there are few individual reports of pulmonary embolism events. Patient concerns: A 55-year-old female with no history of smoking visited a local hospital with mild cough and lower sacrococcygeal pain without obvious cause. Diagnosis: She was diagnosed with right lung adenocarcinoma with multiple bone metastases (T2bN1M1c, stage IVB) after image and histological examination, with EGFR 21 exon L858R sensitive mutation. Interventions: She initially underwent surgical treatment due to the discovery of a sacral tumor, and the pathology of the surgery indicated metastatic lung adenocarcinoma. Genetic testing results showed EGFR 21 exon L858R sensitive mutation. Combined with positron emission tomography/computed tomography imaging results and sacral tumor surgical pathology results, it was considered that the patient had lung adenocarcinoma with multiple bone metastases. Treatment plan: targeted treatment with almonertinib which is the third-generation EGFR-TKIs. After only 1 month of targeted treatment, chest enhanced computed tomography revealed pulmonary embolism imaging, combined with coagulation indicators, considering pulmonary embolism. As the thromboembolic site is located at the end of the pulmonary artery branch and imaging shows that tumor lesions are stable. Consider continuing targeted treatment with almonertinib and adjuvant oral anticoagulant therapy with rivaroxaban for treatment. Outcomes: Two months later, the patient’s condition was followed up and it was found that the imaging manifestations of pulmonary embolism disappeared, but the coagulation indicators remained in a hypercoagulable state. The patient’s treatment plan remained unchanged, and the patient was hospitalized for follow-up every 6 to 8 weeks to closely observe changes in the condition. Lessons: There are many reasons for the occurrence of hypercoagulable blood in tumor patients, and there are very few reports of treatment-related pulmonary embolism events in patients who are treated with almonertinib targeted therapy. It is particularly important to determine whether the hypercoagulable blood in such patients is related to targeted therapy drugs in clinical practice, as this will affect the clinical benefits of patients in epidermal growth factor receptor tyrosine kinase inhibitors treatment and the clinical indicators which need to pay more attention in follow-up observation, to avoid serious pulmonary embolism events and thus affect the patient’s survival status and quality of life.

Assessment of Pulmonary Arterial Vascular Impedance in Fetuses With Tetralogy of Fallot.

To evaluate the vascular impedance of the pulmonary arteries in fetuses with tetralogy of Fallot (TOF) by Doppler echocardiography. A total of 42 fetuses with TOF (TOF group) and 84 gestational age-matched normal fetuses (control group) were prospectively collected from the Second Xiangya Hospital of Central South University from August 2022 to January 2023. The severity of TOF was classified into mild TOF (z score ≥-2), moderate TOF (-4 < z score < -2), or severe TOF (z score ≤-4) according to the z score value of the pulmonary annulus diameter. The pulsatility index (PI) of the main pulmonary artery (MPA), distal left pulmonary artery (DLPA), and distal right pulmonary artery (DRPA) were measured by pulsed-wave Doppler. The differences in clinical data and echocardiographic parameters between TOF group, control group, and TOF subgroups were compared. Compared with the control group, MPA-PI increased significantly, whereas DLPA-PI and DRPA-PI decreased in TOF group (all P < .001). There were no significant differences in MPA-PI and DRPA-PI among mild TOF, moderate TOF, and severe TOF (all P > .05). However, DLPA-PI decreased significantly in severe TOF compared with mild TOF (P < .05). Fetuses with TOF presented increased vascular impedance in the pulmonary trunk and decreased impedance in distal pulmonary artery branches. Further large and follow-up studies are needed to demonstrate the associations between those changed vascular impedances and the development of PA in patients with TOF.

Nexplanonectomy—the surgical removal of an embolized implanted contraceptive device: a case report and review of the literature

BackgroundNexplanon implants are a common hormonal contraceptive modality. Though rare, these devices can embolize into the injured wall of the basilic vein, through the right heart, and finally wedge itself into a pulmonary artery. With adherence to the arterial wall over time, it becomes less amenable to endovascular retrieval. Patients may present with symptoms mimicking a pulmonary embolism, or without any symptoms at all. In asymptomatic cases, endovascular retrieval and/or surgery is required when patients wish to begin having children prior to biological inactivity. The current literature showed as little as nine case reports detailing lung tissue removal in the aim of reversing a patient’s implanted contraceptive device.Case presentationA 22-year-old asymptomatic active-duty Caucasian female presented for elective outpatient Nexplanon removal. The suspicion of possible implant migration arose when it was discovered to be non-palpable in her left arm. After plain film x-rays failed to localize the implant, a chest x-ray and follow-up Computed Tomography (CT) scan revealed that the Nexplanon had migrated to a distal branch of the left pulmonary artery. Due to the patient’s strong desires to begin having children, the decision was made for removal. Initial endovascular retrieval failed due to Nexplanon encapsulation within the arterial wall. Ultimately, the patient underwent a left video-assisted thoracoscopic surgery (VATS) for exploration and left lower lobe basilar S7–9 segmentectomy, which successfully removed the Nexplanon.ConclusionsImplanted contraceptive devices can rarely result in migration to the pulmonary vasculature. These radiopaque devices are detectable on imaging studies if patients and clinicians are unable to palpate them. An endovascular approach should be considered first to spare lung tissue and avoid chest-wall incisions, but can be complicated by encapsulation and adherence to adjacent tissue. A VATS procedure with single-lung ventilation via a double-lumen endotracheal tube allows surgeons to safely operate on an immobilized lung while anesthesiologists facilitate single-lung ventilation. This patient’s case details the uncommon phenomenon of Nexplanon migration, and the exceedingly rare treatment resolution of lung resection to remove an embolized device.

Infected right ventricular outflow tract pseudoaneurysm in an infant with tetralogy of Fallot: Successful palliation using a Gore VBX® covered stent

ABSTRACT Pseudoaneurysm formation within the right ventricular outflow tract (RVOT) is a rare but serious complication following cardiac surgeries involving the RVOT. This report presents the case of a 3-month-old, 4 kg infant with tetralogy of Fallot and pulmonary atresia, previously treated with ventricular septal defect closure and right ventricle (RV)–pulmonary artery homograft placement. The patient presented critically ill with septic shock and suprasystemic RV pressure. A high-risk surgical approach was averted through the endovascular exclusion of the pseudoaneurysm using a Gore VBX® balloon expandable covered stent, in addition to stenting of bilateral proximal branch pulmonary arteries to alleviate RV hypertension. This case underscores the multifactorial nature of RVOT pseudoaneurysm formation and the importance of a high index of suspicion for diagnosis. Management options, including surgical and transcatheter strategies, are discussed, focusing on the successful use of a covered stent for stabilization in a critically ill patient.

Managing Bilateral Discontinuous Pulmonary Arteries of Ductal Origin in Single-Ventricle Anatomy

BackgroundWe describe outcomes and management strategies for single-ventricle and bilaterally discontinuous pulmonary arteries (PAs) originating from bilateral ductus arteriosus. MethodsWe reviewed 22 patients with aforementioned anatomy and PA centralization from 1995 to 2023, excluding those with biventricular repair. ResultsMedian age at centralization was 9 days (minimum-maximum, 0 days-2 years). Centralization was performed with systemic-to-pulmonary shunt (n =20 [91%]; 2 after bilateral ductal stents) or bidirectional cavopulmonary connection (n = 2 [9%]) using pericardial roll (n = 14 [64%]), patch-augmented direct anastomosis (n = 7 [32%]), and interposition graft (n = 1 [5%]) techniques. Concurrent total anomalous pulmonary venous connection (TAPVC, n = 11 [50%]) was associated with significantly inferior survival (P = .01). Five patients (23%) died at a median of 59 days (minimum-maximum, 6-257 days) after centralization, all with noncardiac TAPVC. At the latest follow-up for 17 survivors (median, 13.5 years; minimum-maximum, 0.5-25.1 years after centralization), 12 completed Fontan, 4 completed second-stage palliation, and 1 received a transplant before second-stage palliation. PA reintervention was required in 14 patients (64%), including 3 with reoperations independent of staged palliation. Echocardiography from baseline to before the second stage demonstrated branch PA growth with significantly increased diameters (left, P = .0006; right, P = .0002); z-scores significantly increased for right (P = .004) but not left (P = .11). ConclusionsSuccessful single-ventricle palliation is possible, although high risk, for patients with bilateral discontinuous ductal PAs. Early postcentralization mortality remains substantial, particularly with associated noncardiac TAPVC. Many require reintervention to maintain PA growth, typically concurrently with staged palliation.

Successful ECMO treatment in patients with cerebral hemorrhage and PROC gene mutation associated with VTE: a case report

In this report, we report a case of a middle-aged male, admitted to the ICU with cerebral hemorrhage resulting from a severe high-altitude fall. The patient encountered significant challenges in oxygenation index correction, attributed to extensive embolism in both the primary and branch pulmonary arteries. Consequently, the patient underwent an immediate initiation of veno-arterial extracorporeal membrane oxygenation (VA-ECMO) therapy, persisting for 20 days. During this treatment period, a mutation in the protein C (PROC) gene was identified. The medical team meticulously navigated the delicate balance between anticoagulation and bleeding risks. Eventually, the patient was successfully weaned off VA-ECMO and subsequently discharged. This report aims to delve into the etiology and therapeutic approaches of this uncommon case, with the intention of offering insightful reference for managing similar clinical scenarios in the future.

Pulmonary artery thrombosis prophylaxis and treatment in clinical practice and experiment

The effectiveness of anticoagulant therapy in the prevention and treatment of pulmonary artery thrombosis and the possibility of anti-inflammatory therapy in preventing this complication in clinical practice and experiments were assessed. Data from patients with a new coronavirus infection and those suffering from urgent noninfectious pathology with confirmed pulmonary artery thrombosis were retrospectively analyzed. The outcomes of anticoagulant therapy and anticoagulant therapy combined with glucocorticoid and/or anticytokine drugs were assessed. Histological preparations of the lung vessels of patients were examined. Using an experimental model of rats with induced thrombosis of the posterior vena cava, changes in the pulmonary artery branches were assessed in the main group administered with edible mussel (Mytilus edulis) hydrolyzate and the control group given a physiological solution. No statistically significant relationship was found between the therapeutic, intermediate, and preventive anticoagulant therapy regimens and mortality, changes in lung dynamics, and D-dimer levels in 313 patients with new coronavirus infection. No predominance of any anticoagulant therapy regimen used was found among deceased patients. Thirty-nine patients were treated with glucocorticoid and/or anticytokine drugs in the presence of anticoagulant therapy. No statistically significant relationship in the onset of thrombotic complications was found between the groups receiving therapy with glucocorticoid and anticytokine drugs. No differences were noted in the drug-induced pathomorphosis of the wall of the pulmonary artery branches in the group receiving anticoagulant therapy or in the group receiving a combination of anticoagulant therapy and glucocorticoid and/or anticytokine drugs. Pulmonary artery thrombosis developed in all 19 patients suffering from urgent noninfectious pathology, 11 of whom were under anticoagulant therapy. In 12 of 15 rats in the control group with thrombosis of the posterior vena cava, blood clots were found in the lumen of the pulmonary artery branches. In 14 rats of the main group administered M. edulis hydrolyzate, no blood clots were found in the pulmonary artery branches. Thus, the systemic effects of anticoagulant therapy were offset by the local prothrombotic effects of the vascular wall caused by inflammation. Glucocorticoid and anticytokine drugs did not affect inflammatory changes in the vascular wall and did not prevent pulmonary artery thrombosis. The introduction of M. edulis in the experiment prevented pulmonary artery thrombosis in the presence of posterior vena cava thrombosis, which indicates a promising direction in the search for pathogenetic prevention of this complication.

Autologous Reconstruction of Anomalous Origin of a Right Pulmonary Artery From the Aorta.

Anomalous origin of a pulmonary artery branch from the aorta is a rare congenital anomaly in which one of the pulmonary arteries arises from the aorta. These patients require early surgery to prevent development of severe irreversible pulmonary arterial hypertension. Multiple techniques have been described for repair of this condition. In this report, we describe a different technique compared with previously described procedures and discuss its advantages.

Remodeling of Murine Branch Pulmonary Arteries Under Chronic Hypoxia and Short-Term Normoxic Recovery.

Chronic hypoxia plays a central role in diverse pulmonary pathologies, but its effects on longitudinal changes in the biomechanical behavior of proximal pulmonary arteries remain poorly understood. Similarly, effects of normoxic recovery have not been well studied. Here, we report hypoxia-induced changes in composition, vasoactivity, and passive biaxial mechanics in the main branch pulmonary artery of male C57BL/6J mice exposed to 10% FiO2 for 1, 2, or 3 weeks. We observed significant changes in extracellular matrix, and consequently wall mechanics, as early as 1 week of hypoxia. While circumferential stress and stiffness returned toward normal values by 2-3 weeks of hypoxia, area fractions of cytoplasm and thin collagen fibers did not return toward normal until after 1 week of normoxic recovery. By contrast, elastic energy storage and overall distensibility remained reduced after 3 weeks of hypoxia as well as following 1 week of normoxic recovery. While smooth muscle and endothelial cell responses were attenuated under hypoxia, smooth muscle but not endothelial cell responses recovered following 1 week of subsequent normoxia. Collectively, these data suggest that homeostatic processes were unable to preserve or restore overall function, at least over a brief period of normoxic recovery. Longitudinal changes are critical in understanding large pulmonary artery remodeling under hypoxia, and its reversal, and will inform predictive models of vascular adaptation.

Staged Norwood operation for patients with hypoplastic left heart syndrome and cor triatriatum: a report of two cases

Two patients with hypoplastic left heart syndrome (HLHS) concomitant with cor triatriatum underwent the staged Norwood procedure following hybrid palliation. Cor triatriatum was diagnosed after birth in both cases. Case 1 with aortic stenosis and mitral atresia underwent bilateral pulmonary artery banding at 2 days of age. Subsequently, balloon atrial septostomy was conducted at 21 days of age due to hypoxia and increased intra-atrial pressure gradient. At 2 months of age, concomitant Norwood operation, cor triatriatum repair, and tricuspid valve repair were successfully performed. She has suffered from branch pulmonary artery hypoplasia since then; thus, Fontan operation was out of indication. Case 2 with aortic stenosis and mitral stenosis underwent bilateral pulmonary artery banding at 6 days of age. After that, the patient’s hemodynamic condition remained stable. The Norwood operation concomitant with cor triatriatum repair was performed at 2 months of age. However, during the procedure, massive pulmonary edema and hemorrhage occurred right after the initiation of cardiopulmonary bypass. She could not be weaned from cardiopulmonary bypass.

Cardiac Catheterization Interventions in the Right Ventricular Outflow Tract and Branch Pulmonary Arteries Following the Arterial Switch Operation

Cardiac Catheterization Interventions in the Right Ventricular Outflow Tract and Branch Pulmonary Arteries Following the Arterial Switch Operation

Pulmonary artery thrombosis. Clinical cases

Accumulating evidence supports the need to consider pulmonary artery thrombosis the separate thrombotic complication, which requires its own treatment algorithms.&#x0D; The aim of the work is to demonstrate the pulmonary artery thrombosis clinical aspects and the effect of the standard thrombotic prophylactic algorithms with clinical examples.&#x0D; Clinical cases of patients with pulmonary artery thrombosis are presented. Pulmonary artery thrombosis is considered to be the thrombotic obstruction of the pulmonary artery branches in the absence of venous and cardiac chambers thrombosis.&#x0D; Patient S. with COVID-19 and 75% of lung involvement was treated in the accordance with the actual COVID-19 treatment recommendations. Enoxaparin sodium 6000 IE once a day followed by the increasing dosage of 8000 IE twice a day was used. To prevent exacerbation tocilizumab was infused, and systemic thrombolytic therapy was performed. On the 6th day after systemic thrombolytic therapy patient died. Examination revealed thrombi in the pulmonary artery branches.&#x0D; Patient P. with continued growth of cerebral left frontoparietal region glioblastoma was hospitalized. Surgical tumor removal was performed a year ago. Conservative therapy, dexamethasone injections and anticoagulant prophylaxis (enoxaparin sodium 4000 IE once a day) were prescribed. After the patient death thrombi were found in the pulmonary artery segmental branches.&#x0D; Outpatient D. had a history of four episodes of dyspnea during the acute respiratory diseases. Computed tomography performed at the time of the third and fourth episodes revealed thrombi in the pulmonary artery segmental branches. The fourth episode occured despite anticoagulant prophylaxis with rivaroxaban (10 mg once a day). Patient was recommended to switch to low molecular weight heparins in case of any disease. There have been no recurrent episodes over the past 8 months.&#x0D; Distinctive features of pulmonary artery thrombosis pathogenesis determine the development of the complication despite anticoagulant prophylaxis. Preventive effect of anti-inflammatory drugs on the pulmonary artery thrombosis development has not been demonstrated.

Williams Syndrome: A Rare Case Report

We report a rare case of Williams Syndrome diagnosed at an age of two months based on subtle facial dysmorphism (elfin facies) and a pulmonary systolic murmur at birth. Echocardiogram revealed mild supravalvular aortic stenosis and mild supravalvular pulmonary stenosis extending into branch pulmonary arteries. Mild developmental delay, hypotonia, feeding problems, constipation and failure to thrive were present. In view of distinctive facial phenotype in combination with cardiac abnormalities, Williams Syndrome was strongly suspected. It was confirmed by fluorescence in situ hybridisation test demonstrating a deletion of elastin gene on chromosome 7. This case highlights the importance of pediatricians awareness about malformation syndromes which can help in early diagnosis, timely genetic counselling to the family and better patient outcomes.

Morte súbita causada por embolismo tumoral em um cão

ABSTRACT: Tumor embolization is a well-described cause of unexpected death in human oncologic patients; however, it is poorly explored in veterinary medicine. We report a case of pulmonary tumor embolization in a dog with sudden death. During necropsy, a neoplastic mass was observed replacing the left adrenal gland and forming an intravascular thrombus within the central adrenal vein. A large embolus occluded the left pulmonary artery. The adrenal mass was histologically confirmed as a pheochromocytoma, and the embolus was constituted of neoplastic cells. Multiple smaller neoplastic emboli were observed within the pulmonary artery branches. Tumor embolization should be considered as a possible cause of sudden death in dogs, particularly in oncologic patients.

МНОГОЭТАПНОЕ ЛЕЧЕНИЕ ТЕТРАДЫ ФАЛЛО: ОТ ПАЛЛИАТИВНЫХ ВМЕШАТЕЛЬСТВ ДО ГИБРИДНОГО БИФУРКАЦИОННОГО Y-СТЕНТИРОВАНИЯ ЛЁГОЧНОЙ АРТЕРИИ И РАДИКАЛЬНОЙ КОРРЕКЦИИ (КЛИНИЧЕСКИЙ СЛУЧАЙ)

Primary radical correction of tetralogy of Fallot (ToF) is an optimal surgical option; however, in some conditions, in particular with concomitant extracardiac pathology and hypoplasia of the pulmonary artery (PA) branches, this intervention is not possible. Obstruction and/or hypoplasia of the PA is one of the risk factors for primary radical correction of this congenital heart defect (CHD). In world practice, there are several methods of palliative approach for PA growth with the possibility of further radical correction. However, in some cases, palliative approaches do not give the desired result. In this regard, we present a clinical case of a successful hybrid bifurcation Y-stenting of the PA and radical correction of ToF. Keywords: Tetralogy of Fallot, radical correction, branches of the pulmonary artery, hypoplasia, stenting of the branches of the pulmonary artery, bifurcation Y-stenting, clinical case.

Kiosk 3R-TB-01 - Improved Quantitative Flow Analysis of Main and Branch Pulmonary Arteries Distal to the Vortex in Repaired Tetralogy of Fallot- a Single Center 4D Flow Cardiovascular Magnetic Resonance Study

Kiosk 3R-TB-01 - Improved Quantitative Flow Analysis of Main and Branch Pulmonary Arteries Distal to the Vortex in Repaired Tetralogy of Fallot- a Single Center 4D Flow Cardiovascular Magnetic Resonance Study