Pulmonary Venous Circulation Research Articles

Adult patients with univentricular physiology after Fontan palliation: prevalence and percutaneous closure of veno-venous collaterals

Abstract Background The Fontan operation resulted in improved survival of patients with congenital heart defects not equipped to sustain a biventricular circulation. Long-term complications are common. Veno-venous collaterals (VVC) can have both potentially beneficial and adverse physiological effects. The aim of this study was to evaluate the prevalence of VVC in adult Fontan patients and describe the percutaneous treatment strategy and the short term follow-up. Methods In this single-centre retrospective observational cohort study, consecutive adult Fontan patients under follow-up in the period between 2017-2023 were included. Results From a cohort of 51 Fontan patients, 14 (28%) underwent a percutaneous closure of VVC (71% female, mean age at intervention 24±4 years). Mean follow-up duration was 2.2±1.1 years. Indications were symptoms and/or significant exercise-related hypoxia. Ten patients (71%) had a systemic left ventricle, and 79% had an extracardiac tunnel (median conduit size 16[16-20]mm). Mean Fontan pressure was 10±4mmHg. Seven patients (50%) had Fontan-associated liver disease. A total of 24 VVC were occluded using coils and/or vascular plugs. No periprocedural complications occurred. The VVC originated from right-sided tributaries of the vena cava inferior (36%), superior vena cava (28%) or both (36%), with the majority draining directly into the pulmonary venous circulation. Eight patients (57%) reported a reduction of symptoms. Mean saturation in rest improved significantly, yet saturation during maximal exercise remained unchanged (94±3 to 97±3%, p=0.002 and 88±3 to 91±4%, p=0.103, respectively). Conclusions VVC are highly prevalent in the adult Fontan population and typically connect the right-sided tributaries of the vena cava inferior and/or superior with the pulmonary veins. Low Fontan pressures do not exclude the presence of VVC. Percutaneous closure of VVC is technically feasible and safe and is associated with reduction of symptoms and a significant rise in resting peripheral oxygen saturation. These results warrant further studies.

Pulmonary Vein Stenosis in an Infant with Bronchopulmonary Dysplasia and Partial Anomalous Pulmonary Venous Circulation: Is This Double Trouble?

Pulmonary Vein Stenosis in an Infant with Bronchopulmonary Dysplasia and Partial Anomalous Pulmonary Venous Circulation: Is This Double Trouble?

Venovenous Bridge Connecting Systemic and Pulmonary Venous Circulation: An Antecedent of the Sinus Venosus Defect.

Venovenous Bridge Connecting Systemic and Pulmonary Venous Circulation: An Antecedent of the Sinus Venosus Defect.

P160 RIGHT SISTEMIC VENTRICLE AND PREGNANCY

Abstract Patient with complex congenital heart disease: situs inversus with dextrocardia and transposition of the great vessels (D–TGA), right aortic arch and inferior cava agenesis with azygos continuation; at 4 months Mustard atrial switch (intratrial baffle for redirection of the systemic venous circulation in the left subpulmonary left ventricle and of the pulmonary venous circulation in the right subaortic systemic ventricle). At 22 years, 1st pregnancy without complications, caesarean section, birth weight 2.2 kg. Two pregnancies spontaneously terminated in the firts trimester. At 29 years echocardiography: right systemic ventricle hypertrophic, dilated and slightly hypokinetic with tricuspid (systemic) valve with mild to moderate insufficiency; Mustard circuit normally functioning on the systemic side, mild stenosis of the pulmonary buffle (Gmax 6mmHg). At 33 years monochorionic biamniotic twins pregnancy; the risk linked to pregnancy for the patient on the basis of her heart disease was included in a WHO class III worsened by twinning. Because of the high probability of maternal and fetal complications related to the continuation of pregnancy, it was necessary to recommend a selective interruption of the pregnancy of the fetus affected by severe selective underdevelopment with signs of twin to twin transfusion at the 20th week of gestation. Maternal clinical and haemodynamic conditions have been stable during pregnancy. At the 24th week of gestation echocardiography: worsening of the systemic right function ventricle, moderate tricuspid insufficiency and increased gradients in the Mustard circuit (systemic Gmax 13mmHg and pulmonary venous Gmed 8mmHg). After the pregnancy team‘s discussion, a delivery plan was proposed to transfer the patient to a third level cardiac surgery center with experience in congenital heart disease adult between the 35th and 36th week. This was not possible because the patient at 32th week presented preterm labor with caesarean section without maternal and newborn complications (birth weight 1,650 kg). In the puerperium ehocardiography: hypertrophic, dilated and moderately hypokinetic systemic right ventricle with moderate tricuspid insufficiency; Mustard circuit: systemic baffle max gradient 16mmHg and pulmonary venous medium gradient 9mmHg. 7 years after this pregnancy at the last visit: stable clinical and echocardiographic situation with persistence of moderate dysfunction of the systemic right ventricle.

Unusual cause of dyspnea and hemoptysis in a healthy female

Rare congenital malformation can lead to the aberrant communication between systemic arterial circulation with pulmonary venous circulation which can range from sequestration to arteriovenous fistulas. We describe a 24 year old female with no significant medical history presenting with dyspnea and hemoptysis. On Computerized Tomography (CT) and angiogram found to have an aberrant vessel branching from celiac axis supplying the right lower lobe and draining into the inferior pulmonary vein with evidence of vascular steal. Due to the size of the vessel, decision was made to forego coil embolization and underwent Video-Assisted Thoracoscopic Surgery (VATS) ligation of the aberrant vessel. She had an uncomplicated operative course and post operatively had resolution of her symptoms. This report demonstrates a rare anatomical congenital vascular malformation causing hemoptysis and resolution of symptoms after ligation of the aberrant blood vessel. Keywords: pulmonary arteriovenous fistula; vats; hemoptysis.

Systems analysis of the mechanisms governing the cardiovascular response to changes in posture and in peripheral demand during exercise

Blood flows and pressures throughout the human cardiovascular system are regulated in response to various dynamic perturbations, such as changes to peripheral demands in exercise, rapid changes in posture, or loss of blood from hemorrhage, via the coordinated action of the heart, the vasculature, and autonomic reflexes. To assess how the systemic and pulmonary arterial and venous circulation, the heart, and the baroreflex work together to effect the whole-body responses to these perturbations, we integrated an anatomically-based large-vessel arterial tree model with the TriSeg heart model, models capturing nonlinear characteristics of the large and small veins, and baroreflex-mediated regulation of vascular tone and cardiac chronotropy and inotropy. The model was identified by matching data from the Valsalva maneuver (VM), exercise, and head-up tilt (HUT). Thirty-one parameters were optimized using a custom parameter-fitting tool chain, resulting in an unique, high-fidelity whole-body human cardiovascular systems model. Because the model captures the effects of exercise and posture changes, it can be used to simulate numerous clinical assessments, such as HUT, the VM, and cardiopulmonary exercise stress testing. The model can also be applied as a framework for representing and simulating individual patients and pathologies. Moreover, it can serve as a framework for integrating multi-scale organ-level models, such as for the heart or the kidneys, into a whole-body model. Here, the model is used to analyze the relative importance of chronotropic, inotropic, and peripheral vascular contributions to the whole-body cardiovascular response to exercise. It is predicted that in normal physiological conditions chronotropy and inotropy make roughly equal contributions to increasing cardiac output and cardiac power output during exercise. Under upright exercise conditions, the nonlinear pressure-volume relationship of the large veins and sympathetic-mediated venous vasoconstriction are both required to maintain preload to achieve physiological exercise levels. The developed modeling framework is built using the open Modelica modeling language and is freely distributed.

A Rare Pulmonary Arterio-venous Malformation with Circular Shunting Due to Inferior Phrenic Artery Supply with Pulmonary Venous Drainage: a Case Report

A rare case of a pulmonary arterio-venous malformation not requiring treatment is reported. An incidental peripheral right lower lobe intrapulmonary pulmonary arterio-venous malformation was identified on computed tomography. On catheter angiography, no pulmonary artery supply was identified. Systemic artery catheter arteriography identified systemic arterial supply from a conjoined inferior phrenic artery with pulmonary venous drainage to the left atrium. Due to this lung abnormality being a systemic arterial circulation to pulmonary venous circulation (left to left or circular) shunt, no treatment was indicated.

Gigantic Pulmonary Arteriovenous Malformation Presenting as Recurrent Lung Infection

The abnormal connection between pulmonary arterial and venous circulation is known as Pulmonary Arteriovenous Malformation (PAVM). It was first described by Churton in 1897. The presentation varies from incidental findings to shortness of breath, haemoptysis, chest pain, syncope and cyanosis. Patients with hereditary haemorrhagic telangiectasia are prone to develop PAVM. PAVMs are quite rare, the frequency ranges from 2-3 per 1,00,000 population. Treatment options include surgical intervention and embolisation, the former is now used seldom. The surgical intervention is more invasive than the percutaneous intervention procedure also known as embolotherapy or embolisation. In the present case, authors encountered a rare case of a 30-year- old male presented with giant PAVM with 13 mm diameter (60.65×35.32 mm in dimensions), where patient presented with fever since two months and vomiting. Considering his symptoms, he was initially suspected as Coronavirus-2019 (COVID-19) positive. However, his Reverse Transcription-Polymerase Chain Reaction (RT-PCR) test was reported negative. It was decided to treat him with vascular plug embolisation. A 16 mm Ampletzer vascular plug II was deployed in such a way that it does not occlude the lower branches of Left Pulmonary Artery (LPA). After deploying the vascular plug, the patient was stable and tolerated the procedure well. He was discharged after two days of observation considering his haemodynamic stability.

A Rare Pulmonary-Systemic Connection: Levoatriocardinal Vein.

Levoatriocardinal vein is a rare persistent connection between pulmonary and systemic venous circulation that might result in bidirectional shunt and paradoxical embolism.

Incidental Bronchial Artery Aneurysm with Associated Bronchial Artery to Pulmonary Venous Fistula: Treatment By Embolization

Background Bronchial artery aneurysm (BAA) is a rare vascular phenomenon with potentially life-threatening complications, especially since most cases are asymptomatic. Symptoms may vary depending on the aneurysm’s size and position Case Presentation Here we present the case of a 40 year old healthy gentleman who developed right sided chest pain following an exercise stress test for insurance purposes. CT of the chest showed an aneurysmal accessory artery arising from the thoracic aorta. Focused CT of the thoracic aorta showed a 21mm arterial aneurysm posterior to the left main bronchus with a long, tortuous efferent forming a fistula with the pulmonary venous circulation. The patient underwent prophylactic embolization and coiling of the aneurysm. Complete occlusion was confirmed on angiography. Discussion Size of bronchial artery aneurysm does not appear to be related to risk of rupture. Many are asymptomatic or present with rupture and haemorrhagic shock. BAAs should be treated immediately regardless of size and symptoms. Embolization is an acceptable treatment modality as it is minimally invasive however treatment is complex when concomitant fistula is present, especially in those with fistulation to the pulmonary venous circulation, due to risk of emboli entering the systemic circulation.

Pulmonary arteriovenous malformations: what the interventional radiologist needs to know.

Pulmonary arteriovenous malformations (PAVMs) or fistulas are rare direct pathological connections between pulmonary arterial and venous circulation. Most of PAVMs are congenital and closely associated with hereditary hemorrhagic telangiectasia, but acquired PAVMs have also been described in the literature. Diagnosis of PAVMs is a priority for clinicians, in order to prevent potentially fatal events such as cerebrovascular stroke, systemic septic embolization, hemoptysis and hemothorax. In this scenario, the radiologist plays a key role in both diagnostic and therapeutic workups of PAVMs: Chest X-ray, computed tomography and magnetic resonance are effective tools for PAVMs identification and confirmation of the suspected diagnosis. Furthermore, imaging modalities provide most of the elements for PAVMs classification according to their angioarchitecture (simple and complex) and help the clinicians in establishing which lesion requires prompt treatment and which one will benefit of imaging follow-up alone. Endovascular management of PAVMs has grown up as the first-line treatment in respect of surgery during last decades, showing lower risk of intra- and post-procedural complications and offering a wide number of treatment options and materials, ensuring effective management in virtually any clinical situation; interventional treatment aims to exclude PAVMs from pulmonary circulation, and specific technique and embolic agents should be selected according to pre-treatment imaging, in order to obtain the best procedural outcome. This paper proposes a review of the clinical and radiological features that a radiologist needs to know for PAVMs diagnosis and proper management, also showing an overview of the most common endovascular treatment strategies and embolization materials.

Ex vivo lung perfusion as a human platform for preclinical small molecule testing.

The acute respiratory distress syndrome (ARDS) causes an estimated 70,000 US deaths annually. Multiple pharmacologic interventions for ARDS have been tested and failed. An unmet need is a suitable laboratory human model to predictively assess emerging therapeutics on organ function in ARDS. We previously demonstrated that the small molecule BC1215 blocks actions of a proinflammatory E3 ligase-associated protein, FBXO3, to suppress NF-κB signaling in animal models of lung injury. Ex vivo lung perfusion (EVLP) is a clinical technique that maintains lung function for possible transplant after organ donation. We used human lungs unacceptable for transplant to model endotoxemic injury with EVLP for 6 hours. LPS infusion induced inflammatory injury with impaired oxygenation of pulmonary venous circulation. BC1215 treatment after LPS rescued oxygenation and decreased inflammatory cytokines in bronchoalveolar lavage. RNA sequencing transcriptomics from biopsies taken during EVLP revealed robust inflammatory gene induction by LPS with a strong signal for NF-κB-associated transcripts. BC1215 treatment reduced the LPS induction of genes associated with inflammatory and host defense gene responses by Gene Ontology (GOterm) and pathways analysis. BC1215 also significantly antagonized LPS-mediated NF-κB activity. EVLP may provide a unique human platform for preclinical study of chemical entities such as FBXO3 inhibitors on tissue physiology.

Multislice Computed Tomography Imaging of Total Anomalous Pulmonary Venous Circulation: Three Rare Case Reports

Total anomalous pulmonary venous connection is a rare congenital cardiac anomaly in which the pulmonary veins have no connection with the left atrium and connect directly to the right atrium or to one of the systemic veins. In the diagnosis of total anomalous pulmonary venous connection, the presence and severity of the venous obstruction is important. Computed Tomography (CT) imaging is known to be a useful method for the evaluation of total anomalous pulmonary venous connection. Here we report 3 cases of total anomalous pulmonary venous circulation (TAPVC) where 32 slice CT scan played an important role in pre-operative diagnosis and management. Age of the patients ranged from seven months to sixteen years. Symptoms of the patients’ varied from fatigue, shortness of breath, mild cyanosis and reduced growth for their age. Echocardiography done revealed dilated right cardiac chambers and atrial septal defect (ASD) with suspicion of TAPVC. CT scan was performed and it provided detailed information about intra-cardiac anatomy, pulmonary veins and their confluence, route, drainage and most importantly their distance and relation with the left atrium. It also provided information about coronary sinus and its drainage. This information is very important for surgical mapping and planning.
 Ibrahim Card Med J 2016; 6 (1&2): 73-77

Contemporary Management of Pulmonary Arteriovenous Malformations.

Pulmonary arteriovenous malformations (PAVMs) are atypical vascular structures involving a direct connection between the pulmonary arterial and venous circulations. While PAVMs are a relatively uncommon disorder, unmanaged cases are at risk for the development of serious complications including embolization and infection. Since their first description in 1897, PAVMs have been identified and treated in a variety of ways. Advancements in diagnostic methods and operative techniques have allowed for more effective treatment of the disease. Most recently, the use of vascular plug transcatheter embolization has been described as an effective therapeutic procedure in the management of PAVMs. In this report, we present our experience with nine cases of PAVMs treated at the University of Kentucky, including special consideration of an exemplary case that illustrates the typical course of treatment for the disease.

Cyanotic Congenital Heart Defects – literature review

Congenital heart defects are one of the most interesting and important chapters regarding abnormal fetal growth pathology. The objective of this article is to present a literature review for the main cyanotic congenital heart defects. The ones presented in the article are: tetralogy of Fallot, transposition of the great vessels, double outlet right ventricle, truncus arteriosus, total anomalous pulmonary venous circulation and additional information regarding very rare malformations such as pentalogy of Cantrell and Uhl anomaly. An early and precise identification of congenital heart defects is an important step in an accurate follow-up of a potential problematic pregnancy. Knowing the sonographic aspect, associated pathology and the current available treatment procedures are vital for the fetal outcome and for the physician to adapt the right management in every situation that might appear during the pregnancy and in the neonatal period.

Control of a Pediatric Pulsatile Ventricular Assist Device: A Hybrid Cardiovascular Model Study.

The aim of this work is to study pediatric pneumatic ventricle (PVAD) performance, versus VAD rate (VADR) and native heart rate (HR) ratio Rr (VADR/HR). The study uses a hybrid model of the cardiovascular system (HCS). HCS consists of a computational part (a lumped parameter model including left and right ventricles, systemic and pulmonary arterial and venous circulation) interfaced to a physical part. This permits the connection of a VAD (15 mL PVAD). Echocardiographic and hemodynamic data of a pediatric patient (average weight 14.3 kg, HR 100 bpm, systemic pressure 75/44 mm Hg, CO 1.5 L/min) assisted apically with asynchronous PVAD were used to set up a basal condition in the model. After model tuning, the assistance was started, setting VAD parameters (ejection and filling pressures, systole duration) to completely fill and empty the PVAD. The study was conducted with constant HR and variable VADR (50-120, step 10, bpm). Experiments were repeated for two additional patients' HRs, 90 and 110 bpm and for two values of systemic arterial resistance (Ras ) and Emax . Experimental data were collected and stored on disk. Analyzed data include average left and right ventricular volumes (LVV, RVV), left ventricular flow (LVF), VAD flow (VADF), and total cardiac output (COt). Data were analyzed versus Rr. LVV and RVV are sensitive to Rr and a left ventricular unloading corresponds in general to a right ventricular loading. In the case of asynchronous assistance, frequency beats are always present and the beat rate is equal to the difference between HR and VADR. In the case of pulsatile asynchronous LVAD assistance, VADR should be chosen to minimize frequency beat effects and right ventricular loading and to maximize left ventricular unloading.

Portal Vein Thrombosis in the Setting of Newly Diagnosed Cushing's Syndrome.

The hallmark manifestations of Cushing’s syndrome (CS) are well known, but hypercoagulability is perhaps least recognized. Patients with CS are at increased risk of both spontaneous and postoperative thromboembolism, with the significant majority of events occurring in the lower extremity and pulmonary venous circulations. We present a case of portal vein thrombosis (PVT) occurring in the setting of newly diagnosed CS due to a left adrenal adenoma. Factor VIII activity was approximately 2.5-fold elevated, a known mechanism by which hypercortisolemia predisposes to venous thrombosis. Acute abdominal pain and fever responded well to unfractionated heparin and parenteral antibiotics, and CS was eventually cured by left adrenalectomy. No thromboembolic events have occurred since surgery. PVT is uncommon and usually occurs as a complication of primary or secondary hepatobiliary malignancies and cirrhosis. To the best of our knowledge, this is just the second reported case of PVT due to CS and the first published in the English language literature.

Contemporary diagnosis and management of pulmonary hypertension

Pulmonary hypertension (PH) encompasses a group of diseases associated with an elevated mean pulmonary artery pressure (mPAP) ≥25 mm Hg at rest. The symptoms of PH are non-specific, and it...

Year in review 2015: Interstitial lung disease, pulmonary vascular disease, pulmonary function, sleep and ventilation, cystic fibrosis and paediatric lung disease.

Keywords: interstitial lung disease; lung function; paediatric lung disease; pulmonary vascular disease; sleep

CT of the bronchopulmonary veins in patients with superior vena cava or left brachiocephalic vein obstruction.

The purpose of this study was to show the arrangement and connections of the bronchopulmonary veins (i.e., vessels draining the bronchi, bronchioles, and pleura in patients with chronic superior vena cava (SVC) or left brachiocephalic vein (LBCV) obstruction using CT. Contrast-enhanced CT scans of the chest of 16 patients with chronic SVC or LBCV obstruction were analyzed retrospectively. Scans were acquired using various standard protocols. The mean age of the patients (10 men and six women) was 63 years (range, 41-86 years). The causes of obstruction were malignancy (7/16, 44%), catheter-related thrombosis (7/16, 44%), chronic fibrosing mediastinitis (1/16, 6%), and unknown (1/16, 6%). The following sites were obstructed: SVC (9/16, 56%), SVC below the azygos vein (4/16, 25%), and lower LBCV (3/16, 19%). The bronchopulmonary veins were opacified via the brachiocephalic, azygos, or accessory hemiazygos veins or their branches. We observed long vessels that could be traced along the lateral mediastinum or alongside the trachea and central bronchi to their termination in the central pulmonary veins from the level of the ostia to segmental divisions. These vessels intercommunicated and gave rise to smaller veins contiguous with the walls of the bronchi and pulmonary arteries. The pulmonary venous connections of the bronchopulmonary veins were more frequent with the lower lobe pulmonary veins. Pericardial and esophageal veins were also opacified through the brachiocephalic or azygos veins and anastomosed commonly with the bronchopulmonary veins. The arrangement and connections of the bronchopulmonary veins in patients with chronic SVC or LBCV obstruction can be depicted by CT; these vessels form an intricate network connecting the systemic and pulmonary venous circulations and can act as systemic-pulmonary shunts.