Pulmonary Venous Obstruction Research Articles

Transcatheter Correction of Superior Sinus Venosus Atrial Septal Defects as an Alternative to Surgical Treatment

BackgroundThe superior sinus venosus atrial septal defect (SVASD) is characterized by deficiency of the common wall between the superior vena cava (SVC) and the right upper pulmonary vein (RUPV), which is no longer committed to the left atrium. ObjectivesThis study sought to evaluate the potential for redirecting the SVC and RUPV flow to the right and left atria, respectively, by implantation of a covered stent in the SVC. MethodsReview of 48 consecutive adult SVASD patients undergoing assessment for correction. Pre-procedural evaluation included cross-sectional imaging and ex vivo simulation using printed or virtual 3-dimensional models. ResultsTranscatheter correction was performed in 25 patients, with a further 6 awaiting stent implantation. Only 8 patients were deemed technically unsuitable. The procedure involved balloon test inflation in the anticipated stent landing zone with simultaneous transesophageal echocardiography and pulmonary venography to confirm defect closure and unobstructed pulmonary venous drainage, followed by deployment of a 10-zig covered Cheatham platinum stent. Stents of lengths between 5 and 8 cm were implanted. A second, uncovered stent was used for anchoring in 9 patients. The RUPV was protected with a high-pressure balloon during stent implantation to prevent pulmonary venous obstruction in 4 patients. The median follow-up period was 1.4 (interquartile range: 0.8 to 1.7) years, with no mortality. Stent embolization occurred in 1 patient; another required drainage of hemopericardium. Cardiac computed tomography after 3 months confirmed unobstructed pulmonary venous return. At latest follow-up, a residual shunt was present in 1 patient. ConclusionsTranscatheter correction of SVASD may be considered as an alternative to surgery in a substantial proportion of patients.

Peculiarities of Histological Structure of Pulmonary Veins in Patients with Total Anomalous Pulmonary Venous Drainage as Morphological Substrates Relating to Formation of Postoperative Pulmonary Venous Obstruction.

The histological and immunohistochemical methods were employed to examine the peculiarities of histological structure of pulmonary veins and left atrium of the heart in norm and in various types of total anomalous drainage of pulmonary veins. In contrast to normal pulmonary vein covered with external multiple muscle layers (myocardial sleeve), such sleeve is absent in veins that have no connection with the left atrium irrespective of the type of the defect. In patients with total anomalous pulmonary venous drainage, the structure of left atrium was heterogeneous featuring either the presence or absence of inner angiomural lining in this atrium. The structural peculiarities are important for insight into etiology of the development of postoperative pulmonary venous obstruction in patients with total anomalous pulmonary venous drainage.

Impact of intrapulmonary-artery septation to pulmonary vein obstruction for two-lung Fontan

Patients with unbalanced pulmonary artery (PA) growth and decreased unilateral pulmonary circulation are considered unsuitable candidates for the Fontan procedure. Following our previous study on the utility of intrapulmonary-artery septation for patients with PA hypoplasia, we investigated its use in patients with pulmonary venous obstruction (PVO). We recruited 42 patients who underwent intrapulmonary-artery septation for unilateral PA hypoplasia and/or PVO between 1998 and 2018 and classified them into no PVO or PVO group. We analysed overall survival, success of the Fontan procedure and data from catheterization and echocardiography. In PVO, we evaluated the functional lung area before the Fontan procedure and the relevance of this parameter to operative outcomes. The PVO and no-PVO group included 24 and 18 patients, respectively. One patient in the no-PVO and 6 patients in the PVO group died during follow-up; this difference was statistically significant (log-rank P = 0.040). In the no-PVO group, 15 (83%) patients achieved two-lung Fontan circulation. In the PVO group, 12 (50%) patients achieved two-lung Fontan circulation. Multivariate analysis revealed that functional lung area and shunt size [significantly larger in patients with functional lung area ≥50% in affected lung (P = 0.040)] were significant factors for successful two-lung Fontan procedure (P = 0.030). Intrapulmonary-artery septation may contribute to increase functional lung area after PVO release to establish two-lung Fontan circulation in patients with PVO who have unbalanced PA growth and/or decreased unilateral pulmonary circulation.

Pulmonary venous obstruction after extracardiac total cavopulmonary connection in right atrial isomerism.

Patients with functional single ventricle and right atrial isomerism (RAI) often have multiform cardiac pulmonary venous (PV) connection, which could be a risk factor for pulmonary venous obstruction (PVO) after extracardiac total cavopulmonary connection (EC-TCPC) owing to compression of the conduit. To investigate the anatomical risk factors for PVO after EC-TCPC in RAI. Twenty-nine patients with RAI without extracardiac total anomalous pulmonary venous connection were enrolled. No patients had PVO before EC-TCPC. A total of 14 and 15 patients had PV orifices ipsilateral and contralateral to the extracardiac conduit, respectively. The former 14 patients were assigned to two groups based on development of PVO after EC-TCPC (groups O and N). The pre- and post-operative cardiac morphologies and their relationship with the conduit were compared. After the EC-TCPC, the pressure gradients between the atrium and the PV were 5.0 ± 2.5 and 0.44 ± 0.2mmHg in groups O and N, respectively (p < 0.01); however, the pressure gradients in the left and right PVs were not significantly different, suggesting stenosis of the common PV orifice. The ratio of the horizontal distance from the vertebrae to the PV orifice and to the lateral edge of the atrium was significantly higher (0.38 ± 0.2 vs. 0.17 ± 0.1; p = 0.04) and the orifice was smaller (8.9 ± 2.0 vs. 15 ± 4.7mm; p < 0.01) in group O than in group N. In cases with ipsilateral locations of the conduit and PV orifice, small size and more lateral location of the PV orifice may be preoperative risk factors for development of PVO.

Pulmonary Venous Flow After Lung Transplantation: Turbulence and High Velocities

Lung transplantation is the definitive treatment for end-stage lung disease. The pulmonary venous anastomosis has the potential for significant obstructive complications that can lead to considerable morbidity and mortality. The use of intraoperative transesophageal echocardiography, including color-flow and spectral Doppler, is instrumental in evaluating the pulmonary veins after lung transplantation. In this E-challenge, a case of intraoperative pulmonary venous obstruction after bilateral lung transplantation is described, the echocardiographic principles required to evaluate the pulmonary veins and screen for complications are reviewed, and when intervention may be required is discussed.

Transcatheter Angioplasty Using Drug Coating Balloons for Pulmonary Venous Obstruction in Children With Congenital Heart Disease

Transcatheter Angioplasty Using Drug Coating Balloons for Pulmonary Venous Obstruction in Children With Congenital Heart Disease

A new palliative surgical technique for high risk Total anomalous pulmonary venous connection (Sarmast-Takriti shunt)

BackgroundTotal Anomalous Pulmonary Venous Connection (TAPVC) is a rare heterogeneous condition That accounting for 1.5–3% of congenital heart diseases. It is characterized by failure of the Pulmonary Venous Confluence (PVC) to be directly connected to the left atrium in combination with a persistent splanchnic connection to the systemic venous circulation. The most critical status occurs when it is accompanied by pulmonary venous obstruction. Managing of this situation is very difficult and in fact, pulmonary venous obstruction is usually lethal. The real aim of this study is offering a new palliative surgical technique (Sarmast – Takriti Shunt) in order to alleviate the patient’s signs and symptoms until becomes ready for the main surgical correction.Case presentationThe study included a 4–day old, low birth weight boy who suffered from Critical Obstructive Total Anomalous Pulmonary Venous Connection. The decision was made to perform the new palliative technique using Gore - Tex (ePTFE). Anastomosis was established without Cardiopulmonary Bypass (CPB) between Pulmonary Venous Confluence (PVC) and the left atrial appendage. Therefore the Sarmast – Takriti Shunt (STS) was taken place.ConclusionAfter completion of the procedure, the pressure gradient across the venous confluence and the Left innominate vein became zero. Cyanosis, agitation and feeding Problem subsided. Three days later, when he was discharged, arterial oxygen saturation had reached as high as 91%. After 7 months we perfomed the main correction.

Predictors of In-Hospital Mortality in Isolated Total Anomalous Pulmonary Venous Connection.

We assessed risk factors for early mortality in isolated total anomalous pulmonary venous connection over a modern era excluding emergent cases to eliminate the influence of associated factors on surgical outcome. Methods: 70 patients with isolated total anomalous pulmonary venous connection who were repaired electively between January 2013 and February 2018 were included. In-hospital mortality was encountered in 4 patients (5.7%). Upon univariate analysis, low age (P = .003) and weight (P = .001) at surgery, preoperative pulmonary venous obstruction (P = .010), preoperative low oxygen saturation (P = .031), long cardiopulmonary bypass (P = .001) and aortic cross clamp (P = .003) times, long duration of mechanical ventilation (P = .001), chest infection (P = 0.041), postoperative low CO syndrome (P < .001) and long postoperative inotropic support (P = .015) were significant predictors of in-hospital mortality. In multivariate analysis postoperative low cardiac output syndrome (OR: 1.060; 95% CI: 1.008-1.116) and prolonged postoperative mechanical ventilation (OR: 1.772; 95% CI: 1.141-2.751) were independent factors of in-hospital mortality. Surgical repair of TAPVC is now performed with acceptable results. According to our study, postoperative low cardiac output syndrome and prolonged postoperative mechanical ventilation were the most significant predictors for early mortality.

Hemodynamic aspects of pulmonary and systemic bloodflow in patients with total anomalous pulmonary venous connection

This review is focused on the problem of the hemodynamics in patients with total anomalous pulmonary venous connection. The article describes a structural features of the pulmonary venous return depending on the anatomical type, pulmonary and systemic hemodynamics, origin and effect of the pulmonary venous return obstruction on the hemodynamics. We presented basic diagnostic problems and principles, including prenatal level, and discussed trends, results and unsolved questions of the surgical treatment of the patients with total anomalous pulmonary venous connection.

Outcomes of Reinterventions for Children with Postoperative Pulmonary Venous Restenosis.

This report aims to summarizeandevaluate the efficacy andexperienceof reintervention for children with postoperative pulmonary venous restenosis. Clinical data from 61 patients with postoperative pulmonary venous restenosis who underwent reintervention at Shanghai Children's Medical Center (SCMC) from September 2009 to June 2018 were retrospectively analyzed. The patients comprised 38 boys and 23 girls, with a mean age of 15.4 ± 12.6months (2-83months) and a mean weight of 8.1 ± 3.4kg (3.7-18.5kg). The mean pulmonary venousvelocity was 2.31 ± 0.47m/s (1.86-3.22m/s). Primary disease included 48 cases of total anomalous pulmonary venous drainage, nine cases of partial anomalous pulmonary venous drainage, and four cases of primary pulmonary venous stenosis. The reintervention procedures included 34 cases using the sutureless technique, ten cases using bovine pericardium enlargement, three cases using blunt enlargement, four cases of balloon dilatation, one case using stent implantation and nine cases involving more than two surgical methods. The early postoperative pulmonary venousvelocity was 1.16 ± 0.20m/s. There were five in-hospital deaths, resulting in a mortality rate of 8.2%. Fifty-six survivors were followed for 52.8 ± 46.5months (6-103months) with no delayed deaths. Echocardiography showed pulmonary venous anastomosis and diameter growth after reintervention, exhibiting a mean growth speed of 0.026 ± 0.013cm/month (p < 0.05) and a mean velocity of 1.24 ± 0.26m/s; five patients experienced varying degrees of pulmonary venous obstruction (> 1.6m/s), but did not require reoperation. Postoperative pulmonary venous restenosis is a common complication after surgery for pulmonary venous malformations. Reintervention should be performed in the early period of pulmonary venous obstruction. Growth of pulmonary venous anastomoses was observed after performing the sutureless technique, bovine pericardium enlargement and blunt enlargement. Although balloon dilatation has a good effect in the early postoperative period, its restenosis rate is high, and strict mid- to long-term follow-up is needed.

Repair of Total Anomalous Pulmonary Venous Connection: Risk Factors for Postoperative Obstruction

Pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) results in substantial morbidity and mortality. Risk factors for postoperative obstruction remain ambiguous. In addition, the existing literature has no standard definition for preoperative obstruction, making patient counseling difficult. All patients undergoing repair of TAPVC at our institution from January 1, 2006, to October 23, 2017, were identified. The primary outcome was the development of postoperative obstruction, analyzed as a time-to-event outcome. Clinical information was extracted to assess risk factors. Degrees of preoperative obstruction were defined based on echocardiographic, catheterization, and clinical findings. Univariable and multivariable Cox proportional hazard regression methods were used to identify factors associated with the primary outcome. During the study interval, 119 patients underwent repair of TAPVC (40% single ventricle), and postoperative obstruction developed in 25 patients (21%). Risk factors associated with obstruction were heterotaxy syndrome, single-ventricle heart disease, additional procedures at the time of vein repair, mixed-type TAPVC, and preoperative obstruction. Having even mild preoperative obstruction (≥1.2 m/s by Doppler echocardiography) was predictive of postoperative obstruction. A multivariable model showed mixed-type TAPVC and the presence of preoperative obstruction were associated with a more than twofold greater hazard of obstruction. TAPVC in the setting of heterotaxy and a single ventricle remains challenging, with high rates of postoperative obstruction. Mixed-type TAPVC is an independent risk factor for postoperative obstruction, particularly in patients with isolated TAPVC. Even mild preoperative obstruction is a risk factor for postoperative obstruction. These results may help risk-stratify TAPVC patients.

Pulmonary venous obstruction in patients who underwent surgical repair of total anomalous pulmonary venous connection: A retrospective study from Saudi Arabia

Pulmonary venous obstruction in patients who underwent surgical repair of total anomalous pulmonary venous connection: A retrospective study from Saudi Arabia

Comparison of conventional and primary sutureless surgery for repairing supracardiac total anomalous pulmonary venous drainage

ObjectiveThe efficacy of using a sutureless approach in order to surgically manage postoperative pulmonary vein stenosis following total anomalous pulmonary venous drainage (TAPVD) has been reported, though outcomes of primary treatment of supracardiac TAPVD remain unclear. We retrospectively reviewed our cardiac center experience, and compared the differences in mid-term outcomes for those patients that received conventional surgery and those that underwent sutureless technique for the primary repair of supracardiac TAPVD.MethodsA total of 43 patients (median age, 199 days; range, 35 days to 1572 days) with supracardiac TAPVD underwent surgical treatment at our cardiac center from 2014 to 2018 were studied retrospectively. Primary sutureless repair was conducted in 20 cases (46.5%). The pulmonary vein scores, left ventricular ejection fraction (LVEF), baseline of the included patients, postoperative, and outcomes data were analyzed between the two groups.ResultsThe pulmonary vein scores, indicating the stenosis degree, of two groups were 0.1 ± 0.3 and 0.1 ± 0.3, left ventricular ejection fraction (LVEF) (%) were separately 66.2 ± 12.1 and 67.1 ± 13.6. The average cardiopulmonary bypass time of sutureless techniques group was much longer than conventional group (96.2 ± 32.6 min vs 75.6 ± 28.2 min, P < 0.05), but there was no difference in aortic cross-clamp time between the two groups. Followed up from 0.1 to 4 years, 3 cases died overall, with 1 (5.0%) individual dying from postoperative pulmonary venous obstruction (PVO) in sutureless group, and 2 (8.6%) dying in the conventional group respectively for postoperative infection and post-PVO. There were no differences in the length of stay in the ICU, grades of PVS after surgery, LVEF and reoperation rate between the two groups.ConclusionsThe mortality, post-PVO, follow up results of supracadiac TAPVD showed no differences between sutureless and conventional techniques. Post-PVO supposed to be the main reason for postoperative mortality.

Double-decker repair of partial anomalous pulmonary venous return into the superior vena cava

ObjectiveConventional procedures for partial anomalous pulmonary venous return (PAPVR) to the superior vena cava (SVC) still have serious complications, such as late SVC and/or pulmonary venous obstruction and supraventricular arrhythmia. We aimed to introduce our newly developed surgical technique with minimum right atriotomy and double-barreled arrangement of systemic and pulmonary venous channels (double-decker technique). MethodsFrom 1998 to 2018, 21 consecutive patients with PAPVR to the SVC underwent this new procedure. The patients' median age and body weight were 4.4 years and 16.5 kg, respectively. Two female patients underwent lateral thoracotomy. Postoperative hemodynamics of both venous channels were assessed using time-resolved 3-dimensional magnetic resonance phase contrast imaging in 6 patients. ResultsThe median follow-up period was 11.0 years. There was no early mortality and late death. No patient required reoperation and/or intervention. All patients maintained normal sinus rhythm, and supraventricular arrhythmia did not occur. The median blood flow velocity of the neo-SVC and neopulmonary venous channel was 0.40 and 0.30 m/s, respectively. Using time-resolved 3-dimensional magnetic resonance phase contrast imaging, the straight and nonrestrictive flow and low wall shear stress were visualized in both venous channels. ConclusionsOur newly developed double-decker technique is a useful alternative surgical procedure for PAPVR to the SVC. Late complications can be completely avoided using this method. Growth potential of both channels is also maintained.

Unusual pulmonary venous return obstruction associated with atrio-ventricular septal defect

Unusual pulmonary venous return obstruction associated with atrio-ventricular septal defect

Development of acquired intrapulmonary venous anastomosis contributing to establishment of Fontan circulation

Pulmonary venous (PV) obstruction is associated with a poor prognosis, as well as a high risk of recurrence, following surgical treatment. It can also interfere with the successful completion of Fontan circulation in patients with complex congenital heart disease. A case of a patient who had right isomerism (also known as asplenia syndrome), total anomalous pulmonary venous connection (TAPVC), and a single right ventricle is presented. Although bilateral total occlusion of the inferior PVs was identified postoperatively, the formation of the anastomosis and collateral vessels into the superior and middle PVs enabled successful completion of Fontan circulation. Anastomoses and collateral flow of the PVs were found largely in the interlobar pleura and not in the lung parenchyma.

C albicans FH binding molecule Hgt1p, a low glucose induced membrane protein trafficked to the cell wall impairing phagocytosis

We present the case of a patient with symptomatic ventilation-perfusion mismatch due to mixed pulmonary arterial and venous obstruction after partial right lung resection for non-small cell lung cancer. Non-invasive imaging suggested complete occlusion of the right lower lobe pulmonary artery, but we were able to successfully percutaneously recanalize the vessel and improve his symptoms and quality of life.

Surgical results of total anomalous pulmonary venous connection repair in 256 patients.

This study was performed to analyse the surgical results of total anomalous pulmonary venous connection (TAPVC) repair at a single institution and to identify trends and variables associated with mortality and morbidity, particularly predictors of recurrent pulmonary venous obstruction (PVO). Our surgical database contained 256 patients with biventricular anatomy who underwent surgical repair for TAPVC from 1981 to 2016. The anatomic TAPVC subtypes in this study were as follows: 114 supracardiac (44.5%), 56 cardiac (21.9%), 64 infracardiac (25.0%) and 22 mixed (8.6%) types. The follow-up for the entire study ranged from 1.6 months to 28.2 years (median 10.4 years). Preoperative PVO was present in 128 patients (50.0%). All patients with TAPVC with single-ventricle anatomy were excluded from the analysis. Data were retrospectively reviewed. Seven (2.7%) early deaths and 26 (10.1%) late deaths occurred. The actuarial survival rate at 20 years postoperatively was 85.3%. The preoperative predictors of operative mortality were younger age and the era of TAPVC repair (before 1998). In addition to these variables, associated cardiac anomalies were predictors of late mortality. Those for postoperative PVO were younger age, lower weight and being an emergency case. The actuarial survival rate at 20 years was 38.6% for patients with postoperative PVO and 92.2% for patients without postoperative PVO (P < 0.001). The long-term outcomes after TAPVC repair in patients with biventricular anatomy were satisfactory. Mortality was significantly associated with the rate of progression of postoperative PVO, and careful follow-up was required especially within 6 months after the primary operation.

Prenatal diagnosis of total and partial anomalous pulmonary venous connection: multicenter cohort study and meta‐analysis

The aims of this study were to review systematically literature on and describe the sonographic features and associated anomalies of total (TAPVC) and partial (PAPVC) anomalous pulmonary venous connection and scimitar syndrome (SS). A retrospective cohort study was carried out of cases of TAPVC, PAPVC and SS that underwent comprehensive ultrasound examination, seen over a 20-year period at two tertiary referral centers. Assessed variables included TAPVC subtype, gestational age at diagnosis, area behind the left atrium, ventricular disproportion, vertical vein, pulmonary venous obstruction, mode of diagnosis, association with cardiac and extracardiac conditions, and pregnancy and fetoneonatal outcomes. The outcome was considered favorable if the individual was alive and well (no functional impairment from surgery or cardiac or extracardiac conditions). Cases associated with right isomerism were excluded from the analysis, as TAPVC in these cases was only one of several major cardiac anomalies affecting sonographic signs. A systematic review was performed in order to obtain a synthesis of characteristics associated with TAPVC, PAPVC and SS. The literature search of PubMed and EMBASE (1970-2016) included reviews, case series and case reports. A meta-analysis was conducted only for TAPVC. Random-effects models were used to obtain pooled estimates of the frequencies of clinical characteristics and sonographic features. For TAPVC, a total of 15 studies involving 71 patients (including 13 from the current cohort study) were included in the systematic review and meta-analysis. The pooled estimate for the association of TAPVC with congenital heart disease was 28.3% (95% CI, 18.1-41.3%) and with extracardiac anomalies it was 18.5% (95% CI, 10.5-30.6%). Of TAPVC cases, obstructed venous return was observed in 34.1% (95% CI, 22.7-47.7%), a favorable outcome in 43.8% (95% CI, 24.0-65.8%), ventricular disproportion in 59.2% (95% CI, 45.1-72.0%), increased area behind the left atrium in 58.1% (95% CI, 41.1-73.5%) and a vertical vein in 59.3% (95% CI, 41.1-75.3%). Diagnosis was established by using color or power Doppler in 84.9% (95% CI, 67.3-93.9%) of cases. For SS, there were only three studies describing eight cases, to which the current study added another five. Ventricular disproportion was present in three out of nine SS cases for which data were available, but for two of these, there was a concurrent heart anomaly. Color Doppler was used for all SS diagnoses, and four-dimensional echocardiography was useful in two out of six cases in which it was used. Outcome for SS cases was generally good. For PAPVC, there were only five studies describing five cases, to which the current study added another two. Major cardiac anomalies were associated in four out of seven of these cases, and extracardiac anomalies in three out of six cases for which data were available. TAPVC can be associated with other cardiac and extracardiac anomalies in a significant percentage of cases. Leading sonographic signs are ventricular disproportion, increased area behind the left atrium and the finding of a vertical vein. Color/power Doppler is the key mode for diagnosis of TAPVC. Obstructed venous return can be expected in roughly one-third of cases of TAPVC and outcome is favorable in less than half of cases. Data for SS and PAPVC are too few to synthesize. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Cardiothoracic Area Ratio Predicts Lethal Pulmonary Venous Obstruction in Patients with Single Ventricle and Total Anomalous Pulmonary Venous Connection.

Background and Objectives When single ventricle (SV) is complicated with total anomalous pulmonary venous connection (TAPVC), the pulmonary vein obstruction (PVO) occurs at a high rate. There are some patients who died from the lethal PVO (l-PVO) which needed PVO release dead due to severe desaturation within 24 hours after birth. The purpose of this study was to find a predictive marker for l-PVO during the fetal period. Methods We enrolled 21 patients diagnosed with SV associated with TAPVC in the antenatal period. Ten patients had supracardiac, five had cardiac, five had infracardiac, and one had mixed TAPVC. We reviewed fetal echocardiography and measured cardiothoracic area ratio (CTAR) and total cardiac dimension (TCD). We divided 21 cases into l-PVO group (6) and non-l-PVO group (15) and compared the fetal echocardiography findings and postnatal prognoses between the groups. Results CTAR at the final fetal echocardiography was 16 to 29% (median: 21) in the l-PVO group and 22 to 38% (median: 28) in the non-l-PVO group ( p = 0.01). TCD/week at the final echocardiography was 0.67 to 1.0 (median: 0.77) in the l-PVO group and 0.78 to 1.2 (median: 0.96) in the non-l-PVO group ( p = 0.02). Conclusion Reduced CTAR in the antenatal period is a good predictor of l-PVO after birth.