Pulmonary Lymphatic Vessels Research Articles

ILC2-derived LIF licences progress from tissue to systemic immunity

Migration and homing of immune cells are critical for immune surveillance. Trafficking is mediated by combinations of adhesion and chemokine receptors that guide immune cells, in response to chemokine signals, to specific locations within tissues and the lymphatic system to support tissue-localized immune reactions and systemic immunity1,2. Here we show that disruption of leukaemia inhibitory factor (LIF) production from group 2 innate lymphoid cells (ILC2s) prevents immune cells leaving the lungs to migrate to the lymph nodes (LNs). In the absence of LIF, viral infection leads to plasmacytoid dendritic cells (pDCs) becoming retained in the lungs where they improve tissue-localized, antiviral immunity, whereas chronic pulmonary allergen challenge leads to marked immune cell accumulation and the formation of tertiary lymphoid structures in the lung. In both cases immune cells fail to migrate to the lymphatics, leading to highly compromised LN reactions. Mechanistically, ILC2-derived LIF induces the production of the chemokine CCL21 from lymphatic endothelial cells lining the pulmonary lymphatic vessels, thus licensing the homing of CCR7+ immune cells (including dendritic cells) to LNs. Consequently, ILC2-derived LIF dictates the egress of immune cells from the lungs to regulate tissue-localized versus systemic immunity and the balance between allergen and viral responsiveness in the lungs.

Efficient pulmonary lymphatic drainage is necessary for inflammation resolution in ARDS.

The lymphatic vasculature is the natural pathway for the resolution of inflammation, yet the role of pulmonary lymphatic drainage function in sepsis-induced acute respiratory distress syndrome (ARDS) remains poorly characterized. In this study, indocyanine green-near infrared lymphatic living imaging was performed to examine pulmonary lymphatic drainage function in septic mouse models. We found that the pulmonary lymphatic drainage was impaired owing to the damaged lymphatic structure in sepsis-induced ARDS. Moreover, prior lymphatic defects by blocking vascular endothelial growth factor receptor-3 (VEGFR-3) worsened sepsis-induced lymphatic dysfunction and inflammation. Posttreatment with vascular endothelial growth factor-C (Cys156Ser) (VEGF-C156S), a ligand of VEGFR-3, ameliorated lymphatic drainage by rejuvenating lymphatics to reduce the pulmonary edema and promote draining of pulmonary macrophages and neutrophils to pretracheal lymph nodes. Meanwhile, VEGF-C156S posttreatment reversed sepsis-inhibited CC chemokine ligand 21 (CCL21), which colocalizes with pulmonary lymphatic vessels. Furthermore, the advantages of VEGF-C156S on the drainage of inflammatory cells and edema fluid were abolished by blocking VEGFR-3 or CCL21. These results suggest that efficient pulmonary lymphatic drainage is necessary for inflammation resolution in ARDS. Our findings offer a therapeutic approach to sepsis-induced ARDS by promoting lymphatic drainage function.

Systemic lymphangitic carcinomatosis from unknown primary cancer mimicking lung disease in a new coronavirus infection (COVID-19): a case report

lymphangitic carcinomatosis is characterized by the spread of cancer cells through pulmonary lymphatic vessels, which results in secondary interstitial inflammation. The diagnosis of lymphangitic carcinomatosis is challenging due to nonspecific clinical symptoms and radiological findings that similar to those of interstitial lung disease.The purpose of the study was to demonstrate difficulties in the diagnosis of lymphangitic carcinomatosis with a systemic dissemination from unknown primary site. Case presentation. We report on a case of lymphangitic carcinomatosis from cancer of unknown primary origin with systemic involvement of internal organs imitating pulmonary disease caused by coVid-19. patient K., 58 years old, was treated for 12 bed-days in therapy departments with the diagnosis of bilateral viral coVid-19 pneumonia. clinical, laboratory and instrumental data were nonspecific. despite treatment, heart failure and kidney disease progressed to terminal stages, leading to the death of the patient. Autopsy revealed systemic lymphangitic carcinomatosis from cancer of unknown primary origin with involvement of the lungs, heart, liver, pancreas, spleen, kidneys and adrenal glands. lymphangitic carcinomatosis mimicked interstitial lung disease in a novel coronavirus infection coVid-19. metastasis to the heart mimicked coronary disease, and metastases to the kidneys led to acute kidney failure, which along with acute respiratory and heart failure caused the death of the patient.Conclusion. lymphangitic carcinomatosis, which does not have specific clinical manifestations, is able to mimic interstitial lung diseases, including a new coronavirus infection. cancer of unknown primary and multiple visceral metastases in this case confirm the concept of the independence of different types of tumor progression.

Three-dimensional visualization of the lymphatic, vascular and neural network in rat lung by confocal microscopy.

In order to demonstrate the intricate interconnection of pulmonary lymphatic vessels, blood vessels, and nerve fibers, the rat lung was selected as the target and sliced at the thickness of 100μm for multiply immunofluorescence staining with lymphatic vessel endothelial hyaluronan receptor 1(LYVE-1), alpha smooth muscle actin (α-SMA), phalloidin, cluster of differentiation 31 (CD31), and protein gene product 9.5 (PGP9.5)antibodies. Taking the advantages of the thicker tissue section and confocal microscopy, the labeled pulmonary lymphatic vessels, blood vessels, and nerve fibers were demonstrated in rather longer distance, which was more convenient to reconstruct a three-dimensional (3D) view for analyzing their spatial correlation in detail. It was clear that LYVE-1+ lymphatic vessels were widely distributed in pulmonary lobules and closely to the lobar bronchus. Through 3D reconstruction, it was also demonstrated that LYVE-1+ lymphatic vessels ran parallel to or around the α-SMA+ venules, phalloidin+ arterioles and CD31+ capillaries, with PGP9.5+ nerve fibers traversing alongside or wrapping around them, forming a lymphatic, vascular and neural network in the lung. By this study, we provide a detailed histological view to highlight the spatial correlation of pulmonary lymphatic, vascular and neural network, which may help us for insight into the functional role of this network under the physiological and pathological conditions.

Lymphatic coagulation and neutrophil extracellular traps in lung-draining lymph nodes of COVID-19 decedents

AbstractClinical manifestations of severe COVID-19 include coagulopathies that are exacerbated by the formation of neutrophil extracellular traps (NETs). Here, we report that pulmonary lymphatic vessels, which traffic neutrophils and other immune cells to the lung-draining lymph node (LDLN), can also be blocked by fibrin clots in severe COVID-19. Immunostained tissue sections from COVID-19 decedents revealed widespread lymphatic clotting not only in the lung but also in the LDLN, where the extent of clotting correlated with the presence of abnormal, regressed, or missing germinal centers (GCs). It strongly correlated with the presence of intralymphatic NETs. In mice, tumor necrosis factor α induced intralymphatic fibrin clots; this could be inhibited by DNase I, which degrades NETs. In vitro, TNF-α induced lymphatic endothelial cell upregulation of ICAM-1 and CXCL8, among other neutrophil-recruiting factors, as well as thrombomodulin downregulation; in decedents, lymphatic clotting in LDLNs. In a separate cohort of hospitalized patients, serum levels of Myeloperoxidase-DNA (MPO-DNA, a NET marker) inversely correlated with antiviral antibody titers, but D-dimer levels, indicative of blood thrombosis, did not correlate with either. Patients with high MPO-DNA but low D-dimer levels generated poor antiviral antibody titers. This study introduces lymphatic coagulation in lungs and LDLNs as a clinical manifestation of severe COVID-19 and suggests the involvement of NETosis of lymphatic-trafficking neutrophils. It further suggests that lymphatic clotting may correlate with impaired formation or maintenance of GCs necessary for robust antiviral antibody responses, although further studies are needed to determine whether and how lymphatic coagulation affects adaptive immune responses.

The regulatory effect of pulmonary lymphatic drainage on silicosis fibrosis

Silicosis is a fibrotic disease caused by long-term inhalation of SiO2 particles that currently has no effective treatment. Earlier studies have suggested that pulmonary lymphatic vessels play a key role in the transport of silica but have not address the long-term effects of altered pulmonary lymphatic drainage on silicosis. Here, we investigated the impact of impaired pulmonary lymphatic drainage on silicosis. In the past, lymphatic drainage disorders were established mainly through the use of VEGF inhibitors. For the first time, we established a model of pulmonary lymphatic drainage disorder by ligating the thoracic duct in rats. Impaired pulmonary lymphatic drainage was found to aggravate inflammation and oxidative damage in silicosis rats and accelerate silicosis progression. Next, we investigated the effect of pulmonary lymphatic drainage on silicosis. We have demonstrated the effect of sodium tanshinone IIA sulfonate(STS) on lymphangiogenesis, which revealed that STS promotes lymphangiogenesis and can delay inflammation, oxidative damage, and fibrosis progression in silicosis rats by promoting the pulmonary lymphatic drainage response, and this effect is mediated by the VEGFR-3/PI3K/AKT signaling pathway. These findings suggest that pulmonary lymphogenesis plays an important role in silicosis pathogenesis, and targeted intervention in pulmonary lymphangiogenesis may be a potential strategy for treating of silicosis in the future.

VEGFR3-driven pulmonary lymphangiogenesis exacerbates induction of bronchus-associated lymphoid tissue in allergic airway disease

Abstract Asthmatic lung samples present with both a higher density of pulmonary lymphatic vessels and a higher incidence of bronchus associated lymphoid tissue (BALT). Here, we asked whether lymphangiogenesis, stimulated by the VEGF-C/VEGFR-3 signaling axis in lymphatic endothelial cells (LECs), plays a role in promoting BALT in mouse models of allergy. First, we determined that chronic intratracheal instillation of house dust mite (HDM), a clinically relevant allergen, recapitulates both lymphangiogenesis and BALT induction. Intratracheal stimulation of VEGFR-3 in LECs exacerbated BALT, while blocking VEGFR-3 signaling reduced BALT. Furthermore, in transgenic mice with an expanded pulmonary lymphatic network (induced prior to allergen challenge), we found an exacerbated BALT response upon chronic HDM inhalation. Recent studies have determined that LEC-derived CXCL13 plays an important role in secondary lymphoid structure organogenesis, and thus we pondered whether LEC-derived CXCL13 could play a role in the development of tertiary lymphoid structures. Indeed, we observed an increase in lung infiltration by CXCR5+ cells when we used VEGF-C to modulate the chronic allergic response. Finally, we found that the VEGF-C exacerbated BALT phenomenon was indeed CXCL13 dependent. Altogether, these results suggest a causative role for pulmonary lymphatics in mediating BALT induction in chronic allergic airway inflammation.

Pulmonary lymphangiectasia a rare cause of chylothorax: A case report

Background: Pulmonary lymphangiectasia (PL) is a rare disease characterized by dilation of the pulmonary lymphatic vessels. PL should be considered in the differential diagnosis in infants with pleural effusion, especially in the presence of chylothorax. Case Presentation: A 3-week-old neonate presented with tachypnea, cough, and reluctance to feed. Work-up found bilateral chylous pleural effusion. The patient was initially managed conservatively with bilateral tube thoracostomies and octreotide, however output from chest tubes did not subside. A left thoracotomy was performed with wedge biopsy of the apical segment of the left inferior lung lobe and multiple titanium clips were used to control thoracic duct. Postoperatively the patient continued to have high chest tube output, despite placement of clips. Histology of the biopsy confirmed the diagnosis of congenital pulmonary lymphangiectasia. Conclusion: Pulmonary lymphangiectasia is a rare cause of chylothorax. The treatment is often challenging as found in the index case.

Reduced Prenatal Pulmonary Lymphatic Function Is Observed in Clp1 K/K Embryos With Impaired Motor Functions Including Fetal Breathing Movements in Preparation of the Developing Lung for Inflation at Birth.

Embryonic lungs must be inflated immediately after birth to establish respiration. In addition to pulmonary surfactant, recently, we have revealed lymphatic function as a previously unknown regulator of prenatal lung compliance that prepares the embryonic lung for inflation at birth. It is well-documented that the late gestation embryo performs episodic breathing-like movements called as fetal breathing movements (FBMs), but the physiological importance of these events is not clear. Here we aimed to study the physiological role of FBMs in preparation for air inflation at birth. Clp1K/K late gestation embryos develop a progressive loss of spinal motor neurons associated with axonal degeneration and denervation of neuromuscular junctions serving as an ideal genetic model to test the possible role of FBMs. We demonstrated that Clp1K/K newborns show impaired motor function resulting in fatal respiratory failure after birth. Next, we showed that the alveolar septa are thicker, and the alveolar area is reduced in Clp1K/K late gestation embryos, while the expression of molecular markers of lung development are not affected. Importantly, pulmonary lymphatic vessels are dilated and the prenatal pulmonary lymphatic function is reduced in Clp1K/K late gestation embryos. Our results have revealed that Clp1K/K mice show impaired motor functions including FBMs, and late gestation Clp1K/K embryos display reduced prenatal lymphatic function and impaired lung expansion represented as thickened alveolar septa and reduced alveolar area in preparation of the developing lung for inflation at birth. These findings suggest a possible mechanism that FBMs, similarly to breathing movements after birth, stimulate prenatal lymphatic function in pulmonary collecting lymphatics lacking smooth muscle coverage to prepare the developing lung for inflation and gas exchange at birth. Moreover, these results raise the possibility that stimulating FBMs during late gestation might be an effective way to reduce the risk of the development of neonatal respiratory failure.

Significant increases in the density and number of lymphatic vessels in pleuroparenchymal fibroelastosis.

Some investigators have detected fibrinous exudate or immature organisation in the alveolar spaces prior to the development of subpleural elastofibrosis in patients with pleuroparenchymal fibroelastosis (PPFE). We hypothesised that PPFE progress is associated with an impaired lymphatic drainage system, resulting in the failed resolution of intra-alveolar exudate. The aim of this study is to investigate the pulmonary lymphatic vessels in PPFE, histologically. We retrospectively reviewed our medical records from 1995 to 2017, and selected autopsied or surgically biopsied patients with PPFE (n=18), pulmonary apical cap (n=18), and IPF (n=26). We detected lymphatic endothelial cells by using immunostained specimens, calculating the percentage of lymphatic vessel area in the non-aerated area (lymphatic vessel density) and the number of lymphatic vessels per non-aerated area (per mm2 ) (lymphatic vessel number). These parameters in PPFE were compared with those in apical cap, IPF, and normal lung tissue. The lymphatic vessel density in PPFE patients [2.97%; interquartile range (IQR) 2.61-3.86] was significantly higher than that in normal lung (0.91%; IQR 0.84-1.07), pulmonary apical cap (0.67%; IQR 0.58-0.83), and IPF (0.91%; IQR 0.68-1.25) (P<0.01 in any comparison). The lymphatic vessel number in PPFE was also significantly higher than that in normal lung, pulmonary apical cap, and IPF. Among PPFE patients, the increase in lymphatic vessel density was found to be correlated with the characteristic physiology of PPFE, such as a flattened chest cage on computed tomography and high residual volume/total lung capacity ratio on spirometry. Significant increase in the density and number of lymphatic vessels is a supportive characteristic that enables the differentiation of PPFE from IPF and apical cap.

Congenital pulmonary lymphangiectasis mimicking congenital pulmonary interstitial emphysema in a patient with congenital diaphragmatic hernia

Congenital pulmonary lymphangiectasis is a rare abnormality of the lung characterized by dilated pulmonary lymphatic vessels. The diagnosis of congenital pulmonary lymphangiectasis is challenging due to its unspecific presentation, as well as possible radiological imitation of interstitial emphysema. We present a patient where diagnosis was hampered by the extremely rare combination of pulmonary lymphangiectasis and congenital diaphragmatic hernia.

P3.04-001 Near-Infrared Fluorescent Identification of Lymphatic Flow in Non-Small Cell Lung Cancer: Topic: Miscellaneous I

None of the methods of intraoperative determining the sentinel lymph nodes is used in lung cancer. We tried to evaluate the features of lymphatic flow and sentinel lymph nodes (SLN) mapping in patients with NSCLC using near-infrared (NIR) fluorescence imaging. 50 patients with NSCLC (squamous cell – 34, adenocarcinoma – 15, large cell – 1) who underwent curative resections (pneumonectomy – 19, lobectomy – 31) were prospectively divided into two groups – with preoperative chemotherapy (CT+S group, 15 patients) and without (S group, 35 patients). Immediately after entering the pleural cavity 2 ml of indocyanin green (ICG) solution as an NIR fluorescent lymphatic tracer was injected in 3-4 points around the tumor. Lymphatic flow and SLN were real-time identified by fluorescence imaging system intraoperatively every 15 minutes after injection and postoperatively ex vivo. Ipsilateral hilar and mediastinal lymphadenectomy was done. The fluorescent identification rate of pulmonary lymphatic vessels were 97% (34 of 35 patients) in S group and 40% (6 of 15 patients) in CT+S group, p=0,001. The interval between injection and visualization of lymphatic channels was 15 min in 9 patients (18%), 30 min in 28 patients (56%) and 45 minutes in 3 patients (6%). In 40 patients with positive NIR visualization, lymphatic vessels were presented in the form of thin glowing fluorescent lines in 36 patients (90%), and in the form of diffuse fluorescent glow throughout the affected lobe in 4 patients (10%). At least one SLN was detected in 46 of 50 patients (97% in S group and 80% in CT+S group, p=0,10) with a number of SLNs identified of 1 to 4 per patient (an average, 2,7). Metastatic nodal disease was never identified in patients with a histologically negative SLN (overall accuracy rate 100%). No adverse reactions were noted. In 4 patients nor SLN neither lymphatic vessels were detected: 3 of them had complete responses after preoperative treatment. Application of NIR fluorescence allows studying features of lymphatic vessels and SLN in NSCLC. Absence of metastatic disease in the SLN directly correlates with final nodal status of the lymphadenectomy specimen.

Classification and prognosis of congenital lung anomalies in children

Congenital lung anomalies are rare lung diseases, which caused by the deformity of the anatomical structure of the respiratory system in the development during the embryonic development.At present, there is a lack of standardization of nomenclature and classification for children with congenital lung anomalies.Based on the most up-to-date research and treatment, this paper classifies congenital lung anomalies into 5 broad categories: bronchial ano-malies, vascular anomalies, pulmonary parenchyma anomalies, combined lung and vascular anomalies, pulmonary lymphatic vessels anomalies.Knowledge of these areas is essential for accurate, timely, diagnosis, which aids in optimizing patient outcomes. Key words: Nomenclature; Classification; Prognosis; Congenital lung anomalies; Child

Extrapulmonary lymphangioleiomyoma presenting as a mesenteric cystic mass

Lymphangioleiomyoma (LAM) is a rare benign tumor characterized by a disorganized smooth muscle cell proliferation within the walls of pulmonary, mediastinal, and retroperitoneal lymphatic vessels. The mesenteric LAM is a quite unusual presentation. A 47-year-old woman was admitted to our hospital with a 7-year history of abdominal mass. A computed tomography showed a well marginated mass with soft tissue attenuation. Laparoscopic excision was performed. The specimen revealed a well circumscribed dark cystic mass. The cut surface showed a thin walled cystic tumor with haphazardly distributed trabeculated septa. Microscopically, the tumor showed variable sized ectatic spaces lined by flattened endothelial cells and surrounded by epithelioid or spindle-shaped cells. The lining cells were positive for D2-40, CD31, and CD34. The surrounding cells were positive for alpha-smooth muscle actin and human melanoma black-45. The final diagnosis was a solitary LAM arising in the mesentery. The patient showed an uneventful postoperative course for 10 months.

Pulmonary Lymphangitic Carcinomatosis from Rectal Cancer: Report of a Case

Pulmonary lymphangitic carcinomatosis (LC) is defined as the presence of tumor cells in the pulmonary lymphatic vessels and lung interstitium. Pulmonary LC is a rare form of lung metastasis from rectal cancer. Here, we report the case of a 57- year-old woman who developed dyspnea after neoadjuvant concurrent chemoradiation therapy (CCRT) for rectal cancer. Chest radiographs revealed bilateral reticulonodular infiltrates and pleural effusion but no obvious nodules. The patient was treated as a case of pneumonia initially but the dyspnea worsened rapidly. For lack of bacterial growth from sputum culture, and on account of the cytological examination of the pleural effusion which revealed malignant cells. pulmonary LC was suspected and systemic chemotherapy was prescribed; however, the treatment was ineffective and the patient died of acute progressive respiratory failure. We suggest if a patient having cancer presents with respiratory compromise and increased bilateral infiltration and/or reticulonodular changes on chest radiographs, the possibility of pulmonary LC should be considered. If the patient treated as a case of pneumonia or other benign diseases, has a rapidly worsening clinical course, early diagnosis and aggressive chemotherapy are essential for the proper management of pulmonary LC.

Congenital pulmonary lymphangiectasis

Congenital pulmonary lymphangiectasis (CPL) characterized by dilatation of pulmonary lymphatic vessels occurs as a congenital anomaly. With poor prognosis, neonatal presentation of bilateral CPL is associated with the severe compromise of pulmonary gas exchange and high mortality. A male infant born at 39 weeks of gestation was found to have CPL. Cyanosis and cardiac arrest occurred a few minutes after birth, and the symptoms remained after artificial ventilation. The infant died of hypoxemic cardiac failure 45 minutes after birth. Autopsy showed neither pleural effusion nor valvular abnormalities. Microscopically dilated vessels with lymphatics were seen in the lung of the infant. Atelectasis, CPL, inhalation of amniotic fluid, partial hydropic degeneration of hepatic cells, and scrotal edema were diagnosed. With regard to treatment and prognosis, CPL must be distinguished from interstitial emphysema and other diseases.

Role of human visceral pleura in pleural fluid turnover: Need for morphological evidence of lymphatic stomata

To the Editor: We read with great interest the article by Gao and his colleagues1 proposing a new schema and hypothesis regarding pleural fluid turnover. In the proposed hypothesis, the visceral pleura possesses a more active role in humans than what was believed. The authors support the notion that in humans the pulmonary lymphatic vessels originate directly from the mesothelium of the visceral pleura and that it has an effect on both infiltration and reabsorption of pleural fluid in human beings. However, there are some points that are worth commenting. Should the lymphatic vessels originate from the visceral mesothelium, they would form stoma-like projections on the pleura cavity, as implied by the authors. According to the study of Leak and Rahil2 and the study of Nakatani et al,3 the morphology of a typical orifice of a lymphatic stoma under the scanning electron microscopy (SEM) consists of two margins: an outer microvillous margin formed by a perimeter of cuboidal mesothelial cells and an inner, relatively microvillous-free margin, formed by lymphatic endothelial cells. A study on the existence of lymphatic stomata in humans failed to demonstrate any stomas in specimens of 30 patients.4 Peng et al5 found stomas only in 2 of 9 human specimens. Additionally, Li6 demonstrated stomas only in human diaphragmatic parietal pleura but none in both the thoracic parietal pleura and in the visceral pleura in this study. Another study on the sheep pleura, which is thick and morphologically resembles the human pleura,7 revealed that the pleura mesothelium was composed of two types of mesothelial cells: flattened cells with well developed tight junctions and cuboidal cells with lesser tight junctions. The visceral pleura consists mainly of flat cells, indicating a tighter mesothelium. The parietal pleura consists of both flattened and cuboidal mesothelial cells as mentioned above around the orifice of a stoma. The lower permeability of the visceral sheep pleura compared to the parietal was also verified by our research group by means of electrophysiology.8 The absence of many cuboidal cells in the visceral pleura and the finding of a tighter mesothelium other than the parietal pleura, do not favor the existence of visceral lymphatic stomata. It will be of great interest if the authors could provide any figures of SEM. Should these figures depict stomas, the theory proposed by Gao and colleagues will be strengthened. Nevertheless, further experimental evaluation of this interesting new theory is imperative. If it is proven to be valid, research into the pleural fluid turnover will be redirected.

Pulmonary Air Embolization Inhibits Lung Lymph Flow by Increasing Lymphatic Outflow Pressure

Air embolization of the pulmonary vascular tree increases pulmonary microvascular filtration and induces pulmonary edema formation. Flow from cannulated pulmonary lymphatic vessels increases significantly following air embolization. However, in the intact animal, lymph flows into the venous system and the magnitude of lymph flow is directly affected by systemic venous pressure. We hypothesized that pulmonary air embolization would lead to systemic venous hypertension and that this increase in lymphatic outflow pressure would prevent an increase in pulmonary lymph flow. Pulmonary air embolization was induced in dogs under general anesthesia. Flow from cannulated pulmonary lymphatic vessels was recorded for lymphatic outflow pressure set equal to atmospheric pressure (Q(LA)) and for outflow pressure set equal to systemic venous pressure (Q(LV)) both before and after embolization. Air embolization resulted in significant increases in systemic venous pressure from 6.4 +/- 0.3 to 12.4 +/- 1.2 mm Hg and in QLA from 48 +/- 9 to 175 +/- 29 microL . min(1). However, embolization did not increase Q(LV) (10 +/- 2 vs. 3 +/- 3 microl . min(1)). Pulmonary air embolization impedes pulmonary lymph flow by increasing systemic venous pressure and, thereby, contributes to pulmonary edema formation.

Hypoplastic left heart syndrome with intact atrial septum

In patients with hypoplastic left heart syndrome (HLHS) and intact atrial septum, the blood entering the left atrium cannot egress. Emergency treatment interventionally or surgically is mandatory immediately after birth. We describe a patient with HLHS and intact atrial septum who underwent successful transvenous atrial septostomy immediately after birth. When the interatrial communication became restrictive, stent implantation into the arterial duct and into the atrial septum was performed on the 7th day of life. Despite good hemodymanic response, the lung damage was severe and persistent, rendering staged surgical correction impossible. The child died on the 23rd day of life. Autopsy showed patent and correct placed stents in the duct and the atrial septum. There was severe dilatation of pulmonary lymphatic and venous vessels, suggestive of long-standing pulmonary venous hypertension. In conclusion, this form of HLHS has a poor prognosis despite early and aggressive interventional treatment.

Cervical venous anastomoses of pulmonary lymphatic vessels

Cervical venous anastomoses of pulmonary lymphatic vessels