Pulmonary Venous Hypertension Research Articles

Editorial for “3D Vortex‐Energetics in the Left Pulmonary Artery for Differentiating Pulmonary Arterial Hypertension and Pulmonary Venous Hypertension Groups Using 4D Flow MRI”

Editorial for “<scp>3D</scp> Vortex‐Energetics in the Left Pulmonary Artery for Differentiating Pulmonary Arterial Hypertension and Pulmonary Venous Hypertension Groups Using <scp>4D</scp> Flow <scp>MRI</scp>”

3D Vortex-Energetics in the Left Pulmonary Artery for Differentiating Pulmonary Arterial Hypertension and Pulmonary Venous Hypertension Groups Using 4D Flow MRI.

Pulmonary hypertension (PH) is a life-threatening. Differentiation pulmonary arterial hypertension (PAH) from pulmonary venous hypertension (PVH) is important due to distinct treatment protocols. Invasive right heart catheterization (RHC) remains the reference standard but noninvasive alternatives are needed. To evaluate 4D Flow MRI-derived 3D vortex energetics in the left pulmonary artery (LPA) for distinguishing PAH from PVH. Prospective case-control. Fourteen PAH patients (11 female) and 18 PVH patients (9 female) diagnosed from RHC, 23 healthy controls (9 female). 1.5 T; gradient recalled echo 4D flow and balanced steady-state free precession (bSSFP) cardiac cine sequences. LPA 3D vortex cores were identified using the lambda2 method. Peak vortex-contained kinetic energy (vortex-KE) and viscous energy loss (vortex-EL) were computed from 4D flow MRI. Left and right ventricular (LV, RV) stroke volume (LVSV, RVSV) and ejection fraction (LVEF, RVEF) were computed from bSSFP. In PH patients, mean pulmonary artery pressure (mPAP), pulmonary capillary wedge pressure (PCWR) and pulmonary vascular resistance (PVR) were determined from RHC. Mann-Whitney U test for group comparisons, Spearman's rho for correlations, logistic regression for identifying predictors of PAH vs. PVH and develop models, area under the receiver operating characteristic curve (AUC) for model performance. Significance was set at P < 0.05. PAH patients showed significantly lower vortex-KE (37.14 [14.68-78.52] vs. 76.48 [51.07-120.51]) and vortex-EL (9.93 [5.69-25.70] vs. 24.22 [12.20-32.01]) than PVH patients. The combined vortex-KE and LVEF model achieved an AUC of 0.89 for differentiating PAH from PVH. Vortex-EL showed significant negative correlations with mPAP (rho = -0.43), PCWP (rho = 0.37), PVR (rho = -0.64). In the PAH group, PVR was significantly negatively correlated with LPA vortex-KE (rho = -0.73) and vortex-EL (rho = -0.71), and vortex-KE significantly correlated with RVEF (rho = 0.69), RVSV, (rho = 0.70). In the PVH group, vortex-KE (rho = 0.52), vortex-EL significantly correlated with RVSV (rho = 0.58). These preliminary findings suggest that 4D flow MRI-derived LPA vortex energetics have potential to noninvasively differentiate PAH from PVH and correlate with invasive hemodynamic parameters. 1 TECHNICAL EFFICACY: Stage 3.

Pulmonary hypertension and chronic kidney disease: prevalence, pathophysiology and outcomes.

Pulmonary hypertension (PH) is common in patients with chronic kidney disease (CKD) or kidney failure, with an estimated prevalence of up to 78% in those referred for right-heart catheterization. PH is independently associated with adverse outcomes in CKD, raising the possibility that early detection and appropriate management of PH might improve outcomes in at-risk patients. Among patients with PH, the prevalence of CKD stages 3 and 4 is estimated to be as high as 36%, and CKD is also independently associated with adverse outcomes. However, the complex, heterogenous pathophysiology and clinical profile of CKD-PH requires further characterization. CKD is often associated with elevated left ventricular filling pressure and volume overload, which presumably leads to pulmonary vascular stiffening and post-capillary PH. By contrast, a distinct subgroup of patientsat high risk is characterized by elevated pulmonary vascular resistance and right ventricular dysfunction in the absence of pulmonary venous hypertension, which may represent a right-sided cardiorenal syndrome defined in principle by hypervolaemia, salt avidity, low cardiac output and normal left ventricular function. Current understanding of CKD-PH is limited, despite its potentially important ramifications for clinical decision making. In particular, whether PH should be considered when determining the suitability and timing of kidney replacement therapy or kidney transplantation is unclear. More research is urgently needed to address these knowledge gaps and improve the outcomes of patients with or at risk of CKD-PH.

Immature Surfactant Protein B Increases in the Serum of Patients with Calcific Severe Aortic Stenosis.

Valvular disease is a complex pathological condition that impacts countless individuals around the globe. Due to limited treatments, it is crucial to understand its mechanisms to identify new targets. Valve disease may result in pulmonary venous hypertension, which is linked to compromised functioning of the alveolar and capillary membranes and hindered gas exchange. Nonetheless, the correlation between surfactant proteins (SPs) and valve disease remains unexplored. A total of 44 patients were enrolled in this study, with 36 undergoing aortic valve replacement and 8 needing a second aortic valve substitution due to bioprosthetic valve degeneration. Ten healthy subjects were also included. The results showed that patients who underwent both the first valve replacement and the second surgery had significantly higher levels of immature SP-B (proSP-B) compared to control subjects. The levels of the extra-lung collectin SP-D were higher in patients who needed a second surgery due to bioprosthetic valve degeneration, while SP-A levels remained unchanged. The research also showed that there was no reciprocal relationship between inflammation and SP-D as the levels of inflammatory mediators did not differ between groups. The present study demonstrates that circulating proSP-B serves as a reliable marker of alveolar-capillary membrane damage in patients with valvular heart disease.

Diagnosis, management and long term cardiovascular outcomes of phenotypic profiles in pulmonary hypertension associated with congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm resulting in herniation of viscera into the chest. This condition is characterized by pulmonary hypoplasia, pulmonary hypertension (PH) and cardiac ventricular dysfunction. PH is a key component of the pathophysiology of CDH in neonates and contributes to morbidity and mortality. Traditionally, PH associated with CDH (CDH-PH) is thought to be secondary to increased pulmonary arterial resistance and vasoreactivity resulting from pulmonary hypoplasia. Additionally, there is increasing recognition of associated left ventricular hypoplasia, dysfunction and elevated end diastolic pressure resulting in pulmonary venous hypertension in infants with CDH. Thus, hemodynamic management of these infants is complex and cautious use of pulmonary vasodilators such as inhaled nitric oxide (iNO) is warranted. We aim to provide an overview of different phenotypic profiles of CDH associated PH and potential management options based on current evidence and pathophysiology.

Pulmonary Hypertension Secondary to Myxomatous Mitral Valve Disease in Dogs: Current Insights into the Histological Manifestation and Its Determining Factors

Pulmonary venous hypertension (PVH) is caused by deteriorating left ventricular function. The most common cause of PVH in dogs is myxomatous mitral valve degeneration (MMVD). It causes left ventricular volume overload and an increase in left atrial and pulmonary venous pressure (PVH), which leads to pulmonary vascular wall remodeling and contributes to the perpetuation and worsening of PVH. Pulmonary vascular wall remodeling is also characteristic of pulmonary arterial hypertension (PAH). However, the changes in PVH arise secondary to heart failure and vascular remodeling progresses as the disease progresses. On the other hand, PAH is a primary disease that can be triggered, for example, by the use of certain drugs. Similar structural changes may suggest the influence of similar pathophysiological mechanisms or the intermediation of similar mediators. Therefore, this article discusses recent and hitherto uncommented findings elucidating the pathophysiology of the processes and influences on the pattern of histological changes observed in pulmonary hypertension secondary to degenerative mitral valve disease. In particular, we focus on the activity of factors such as endothelin, serotonin, and nitric oxide, which are involved in pulmonary vascular wall remodeling in both PVH and PAH.

Lung transplantation in primary pulmonary arterial hypertension and pulmonary venous hypertension.

End-stage lung disease from primary pulmonary hypertension (PPHTN) and pulmonary venous-occlusive disease (PVOD) may require lung transplantation (LT). While medical therapies exist for the palliation of PPHTN, no therapies exist for PVOD. The study's objective is to compare outcomes of LT in these patients. Patients with PPHTN and PVOD who had undergone LT were identified in the UNOS database (2005-2022). Univariable analyses compared differences between groups in demographic, clinical, and post-transplant outcomes. Multivariable logistic regression examined the association between the diagnosis group and survival. Overall survival time between groups was compared using the Kaplan-Meier method. Six hundred and ninety-six PPHTN and 78 PVOD patients underwent LT during the study period. Patients with PVOD had lower pulmonary artery mean pressure (47vs. 53mmHg, p<.001), but higher cardiac output (4.51vs. 4.31 L/min, p=.04). PVOD patients were more likely to receive lungs from donation after cardiac death donors (7.7vs. 2.9%, p=.04). There were no differences in postoperative complications or length of stay. PVOD was associated with superior survival at 30-day (100vs. 93%, p=.02) and 90-day post-transplant (93vs. 83%, p=.03), but not at later time points. In multivariable analyses, PVOD and brain death donor use were associated with better survival up to 90-day mark. Patients undergoing LT for PVOD had better initial survival, which disappeared after 1 year of transplantation. Donation after circulatory death donor use had a short-term survival disadvantage.

Connective tissue disease-associated pulmonary hypertension: A comprehensive review.

Connective tissue diseases (CTDs) can be associated with various forms of pulmonary hypertension, including pulmonary arterial hypertension (PAH), pulmonary veno-occlusive disease, pulmonary venous hypertension, interstitial lung disease-associated pulmonary hypertension, chronic thromboembolic pulmonary hypertension, and sometimes a combination of several processes. The prevalence of PAH varies among the different CTDs, with systemic sclerosis (SSc) having the highest at 8%-12%. The most recent European Society of Cardiology/European Respiratory Societyguidelines recommend routine annual screening for PAH in SSc and CTDs with SSc features. As CTDs can be associated with a myriad of presentations of pulmonary hypertension, a thorough evaluation to include a right heart catheterization to clearly delineate the hemodynamic profile is essential in developing an appropriate treatment plan. Treatment strategies will depend on the predominant phenotype of pulmonary vasculopathy. In general, management approach to CTD-PAH mirrors that of idiopathic PAH. Despite this, outcomes of CTD-PAH are inferior to those of idiopathic PAH, with those of SSc-PAH being particularly poor. Reasons for this may include extrapulmonary manifestations of CTDs, including renal disease and gastrointestinal involvement, concurrent interstitial lung disease, and differences in the innate response of the right ventricle to increased pulmonary vascular resistance. Early referral for lung transplant evaluation of patients with CTD-PAH, particularly SSc-PAH, is recommended. It is hoped that in the near future, additional therapies may be added to the armamentarium of effective treatments for CTD-PAH. Ultimately, a better understanding of the pathogenesis of CTD-PAH will be required to develop targeted therapies for this morbid condition.

Echocardiography Assessment of Left Ventricular Function in Extremely Preterm Infants, Born at Less Than 28 Weeks’ Gestation, With Bronchopulmonary Dysplasia and Systemic Hypertension

The survival of smaller and more immature premature infants has been associated with lifelong cardiorespiratory comorbidities. Infants with bronchopulmonary dysplasia (BPD) undergo routine screening echocardiography to evaluate for development of chronic pulmonary hypertension, a late manifestation of pulmonary vascular disease. Our aim was to evaluate left ventricular (LV) performance in infants with BPD and pulmonary vascular disease who developed systemic hypertension. We hypothesized that infants with hypertension were more likely to have impaired LV performance. We present a single-center cross-sectional study of premature infants born at less than 28 0/7weeks'gestational age with a clinical diagnosis of BPD. Infants were categorized by the systolic arterial pressure (SAP) at time of echocardiography as hypertensive (SAP ≥90mm Hg) or normotensive (SAP <90mm Hg). Sixty-four patients were included. Infants with hypertension showed altered LV diastolic function with prolonged tissue Doppler imaging-derived isovolumic relaxation time (54.2±5.1 vs 42.9±8.2, P<.001), lower E:A, and higher E:e'. Indices of left heart volume/pressure loading (left atrium:aorta and LV end-diastolic volume [6.1±2 vs 4.2±1.2, P<.001]) were also higher in the hypertensive group. Finally, infants in the hypertensive group had higher pulmonary vascular resistance index (4.42±1.1 vs 3.69±0.8, P=.004). We conclude that extremely preterm infants with BPD who develop systemic hypertension are at risk of abnormal LV diastolic dysfunction. Increased pulmonary vascular resistance index in the hypertensive group may relate to pulmonary venous hypertension secondary to LV dysfunction. This is an important consideration in this cohort when selecting the physiologically most appropriate treatment.

Pulmonary Hypertension, an Echo Assessment: Is it Arterial or Venous?

Pulmonary hypertension (PH) is characterized by pulmonary vascular remodeling, right heart failure, and reduced survival. PH can be PH without left ventricular (LV) dysfunction - pulmonary arterial hypertension (PAH) - (Dana point Class I) and PH with LV dysfunction - pulmonary venous hypertension (PVH) - (Dana point Class II). Whatever the underlying cardiac disease, the presence of PH in patients with heart failure is associated with poor prognosis. Right ventricular dysfunction by ventricular interdependence can cause LV dysfunction. We aim to provide a distinction between PAH and PVH by echocardiography. Retrospective cross-sectional single-center data of 1075 subjects having PH as defined by echocardiography was collected. These were segregated into mild, moderate, and severe categories. The same cohort of PH subjects was also segregated by E/e' derived pulmonary capillary wedge pressure (PCWP) values. Echocardiographic measurements and effort tolerance in Mets were analyzed. Data for 707 normal subjects were taken from an earlier published study on normative echocardiographic measurements of healthy Indians. Our findings show that PAH and PVH can be distinguished using PCWP value >15 mmHg obtained by applying Nagueh's formulaon E/e'. We recommend that PCWP derived from E/e' should be reported with pulmonary artery systolic pressure measurement to distinguish between PAH and PVH.

The Role of HSP90 Inhibitors in the Treatment of Cardiovascular Diseases.

Cardiovascular disease is the result of complicated pathophysiological processes in the tissues that make up the blood vessels and heart. Heat shock protein 90 (HSP90) can interact with 10% of the proteome and is the most widely studied molecular chaperone in recent years. HSP90 is extensively involved in the regulation of protein folding and intracellular protein stability, making HSP90 a hopeful target for the treatment of multiple cardiovascular diseases. Numerous client proteins of HSP90 have been identified in known cardiac disease pathways, including MAPK signaling, PI3K/AKT (PKB)/mTOR, and TNF-α signaling. Therefore, these pathways can be controlled by regulating HSP90. Among them, the activity of HSP90 can be regulated via numerous inhibitors. In this review, first, we will discuss the function of HSP90 and its role in pathological pathways. In addition, HSP90 plays a significant role in most cardiovascular diseases, including hypertension, pulmonary venous hypertension, atherosclerosis, and heart failure; next we will focus on this part. Finally, we will summarize the currently known HSP90 inhibitors and their potential in the treatment of heart disease.

How to diagnose and treat acute pulmonary hypertension when you have no cardiology support

Acute pulmonary hypertension (aPH) is a complex, physiology-driven disorder that causes critical illness in newborns, the hallmark of which is elevated pressure in the pulmonary vascular bed. Several underlying hemodynamic phenotypes exist, including classic arterial aPH with resistance-driven elevations in pulmonary arterial pressure (PAP), alongside flow-driven aPH from left-to-right shunt lesions, and primary left ventricular dysfunction with pulmonary venous hypertension and elevated left atrial pressure. Targeted neonatal echocardiography (TnECHO) is an important tool for evaluation of hemodynamics in aPH and is highly useful for evaluating modulators of disease and targeting cardiovascular therapy. The diagnostic approach to aPH includes confirmation of elevation of PAP, evaluation of the cause and exclusion of structural cardiac disease, assessment of the response of the myocardium to adverse loading conditions, and appraisal of the adequacy of systemic blood flow. Therapeutic goals include support of right ventricular (RV) function, RV afterload reduction, and selection of cardiotropic agents that support underlying pathophysiology without adverse effects on heart rate or pulmonary vascular resistance in addition to routine supportive intensive care. Training programs for TnECHO exist across multiple jurisdictions and strong correlation with pediatric cardiology assessment has been demonstrated. Future directions include adapting TnECHO training with a greater focus on achieving competency, and further research into the role of the modality in providing individualized cardiovascular care for patients with heterogenous underlying physiology, and its effect on key neonatal outcomes.

Distinguishing exercise intolerance in early-stage pulmonary hypertension with invasive exercise hemodynamics: Rest VE /VCO2 and ETCO2 identify pulmonary vascular disease.

BackgroundAmong subjects with exercise intolerance and suspected early‐stage pulmonary hypertension (PH), early identification of pulmonary vascular disease (PVD) with noninvasive methods is essential for prompt PH management.HypothesisRest gas exchange parameters (minute ventilation to carbon dioxide production ratio: V E/VCO2 and end‐tidal carbon dioxide: ETCO2) can identify PVD in early‐stage PH.MethodsWe conducted a retrospective review of 55 subjects with early‐stage PH (per echocardiogram), undergoing invasive exercise hemodynamics with cardiopulmonary exercise test to distinguish exercise intolerance mechanisms. Based on the rest and exercise hemodynamics, three distinct phenotypes were defined: (1) PVD, (2) pulmonary venous hypertension, and (3) noncardiac dyspnea (no rest or exercise PH). For all tests, *p < .05 was considered statistically significant.ResultsThe mean age was 63.3 ± 13.4 years (53% female). In the overall cohort, higher rest V E/VCO2 and lower rest ETCO2 (mm Hg) correlated with high rest and exercise pulmonary vascular resistance (PVR) (r ~ 0.5–0.6*). On receiver‐operating characteristic analysis to predict PVD (vs. non‐PVD) subjects with noninvasive metrics, area under the curve for pulmonary artery systolic pressure (echocardiogram) = 0.53, rest V E/VCO2 = 0.70* and ETCO2 = 0.73*. Based on this, optimal thresholds of rest V E/VCO2 > 40 mm Hg and rest ETCO2 < 30 mm Hg were applied to the overall cohort. Subjects with both abnormal gas exchange parameters (n = 12, vs. both normal parameters, n = 19) had an exercise PVR 5.2 ± 2.6* (vs. 1.9 ± 1.2), mPAP/CO slope with exercise 10.2 ± 6.0* (vs. 2.9 ± 2.0), and none included subjects from the noncardiac dyspnea group.ConclusionsIn a broad cohort of subjects with suspected early‐stage PH, referred for invasive exercise testing to distinguish mechanisms of exercise intolerance, rest gas exchange parameters (V E/VCO2 > 40 mm Hg and ETCO2 < 30 mm Hg) identify PVD.

Histologic and proteomic remodeling of the pulmonary veins and arteries in a porcine model of chronic pulmonary venous hypertension.

In heart failure (HF), pulmonary venous hypertension (PVH) produces pulmonary hypertension (PH) with remodeling of pulmonary veins (PV) and arteries (PA). In a porcine PVH model, we performed proteomic-based bioinformatics to investigate unique pathophysiologic mechanisms mediating PA and PV remodeling. Large PV were banded (PVH, n = 10) or not (Sham, n = 9) in piglets. At sacrifice, PV and PA were perfusion labelled for vessel-specific histology and proteomics. The PA and PV were separately sampled with laser-capture micro-dissection for mass spectrometry. Pulmonary vascular resistance [Wood Units; 8.6 (95% confidence interval: 6.3, 12.3) vs. 2.0 (1.7, 2.3)] and PA [19.9 (standard error of mean, 1.1) vs. 10.3 (1.1)] and PV [14.2 (1.2) vs. 7.6 (1.1)] wall thickness/external diameter (%) were increased in PVH (P < 0.05 for all). Similar numbers of proteins were identified in PA (2093) and PV (2085) with 94% overlap, but biological processes differed. There were more differentially expressed proteins (287 vs. 161), altered canonical pathways (17 vs. 3), and predicted upstream regulators (PUSR; 22 vs. 6) in PV than PA. In PA and PV, bioinformatics indicated activation of the integrated stress response and mammalian target of rapamycin signalling with dysregulated growth. In PV, there was also activation of Rho/Rho-kinase signalling with decreased actin cytoskeletal signalling and altered tight and adherens junctions, ephrin B, and caveolae-mediated endocytosis signalling; all indicating disrupted endothelial barrier function. Indeed, protein biomarkers and the top PUSR in PV (transforming growth factor-beta) suggested endothelial to mesenchymal transition in PV. Findings were similar in human autopsy specimens. These findings provide new therapeutic targets to oppose pulmonary vascular remodeling in HF-related PH.

Ultrasound diagnosis of congestion in the pulmonary and systemic circulations in patients with atrial fibrillation and chronic heart failure

Rationale — Fluid retention is among the most common causes of heart failure decompensation. The goal of our study was to evaluate the sensitivity and specificity of the ultrasound method for evaluating congestive phenomena in both systemic and pulmonary circulatory systems in patients with atrial fibrillation (AF) and chronic heart failure (CHF). Material and Methods — The study included 28 patients with paroxysmal AF, with or without CHF, who were scheduled for radiofrequency pulmonary vein isolation. The maximum and minimum diameters of the right superior pulmonary vein (RSPV) and inferior vena cava (IVC) were measured via echocardiography on expiration. Mean blood pressure in both right and left atria was measured intraoperatively. We calculated the correlations between maximum and minimum diameters of the RSPV and a mean pressure in the left atrium, as well as between IVC on expiration and a mean pressure in the right atrium, and evaluated the sensitivity and specificity of ultrasound diagnostics for evaluating congestive phenomena in both systemic and pulmonary circulation. Results — We established positive correlation between the minimum diameter of RSPV and invasively measured mean left atrial pressure (R=0.65, P&lt;0.05), and between invasively measured mean right atrial pressure and IVC diameter on expiration (R=0.49, P&lt;0.05). The proposed method sensitivity for detecting pulmonary venous hypertension (PVH) on the basis of the maximum RSPV diameter ≥21.7 mm was 75%, and the specificity was 86%. The sensitivity and specificity for detecting PVH on the basis of the minimum RSPV diameter ≥10.5 mm constituted 85% and 86%, respectively. When using the IVC diameter on expiration ≥18.5 mm as an indicator, the sensitivity and specificity for detecting PVH were evaluated at the levels of 100% and 92%, correspondingly. Conclusion — The proposed ultrasound method of diagnosing congestion in pulmonary circulation via maximum and/or minimum diameter of the RSPV can be effectively employed in clinical practice in the same way as conventional technique of congestion diagnostics in systemic circulation via the diameter of IVC in patients with atrial fibrillation and chronic heart failure.

Supra-systemic pulmonary hypertension after complicated percutaneous mitral balloon valvuloplasty: a case report and review of literature

BackgroundThe World Symposium of Pulmonary Hypertension in 2018, updated the definition of pulmonary hypertension (PH) as mean pulmonary artery pressures (PAP) > 20 mmHg. Pulmonary venous hypertension secondary to left-heart disease, constitutes the most common cause of PH, and the determination of a co-existent pre-capillary (primary) PH becomes paramount, particularly at the moment of evaluating and managing patients with heart failure. Pulmonary artery pressures above the systemic pressures define supra-systemic PH and generally leads to frank right ventricular failure and high mortality.Case presentationWe present the perioperative management of a patient with rheumatic mitral valve disease, initially found to have severe PH due to pulmonary venous hypertension, who underwent percutaneous mitral balloon valvuloplasty complicated with mitral chordae rupture, severe mitral regurgitation and supra-systemic PH. Multiple medical therapies and an intra-aortic balloon pump were used as means of non-surgical management of this complication.ConclusionsThis case report illustrates the perioperative implications of combined pre- and post-capillary PH and supra-systemic PH, as this has not been widely discussed in previous literature. A thorough literature review of the clinical characteristics of PH, methods to determine co-existent pre- and post-capillary PH components, as well as concomitant right ventricular failure is presented. Severe PH has known detrimental effects on the hemodynamic status of patients, which can ultimately lead to a decrease in effective cardiac output and poor tissue perfusion.

Легочная гипертензия, ассоциированная с системными заболеваниями соединительной ткани: обзор литературы с акцентом на диагностические аспекты

Легочная гипертензия (ЛГ) является важной причиной заболеваемости и смертности при системных заболеваниях соединительной ткани (СЗСТ). СЗСТ могут способствовать возникновению ЛГ за счет нескольких механизмов: легочная артериальная гипертензия (ЛАГ), ассоциированное интерстициальное заболевание легких, нервно-мышечные заболевания и/или расстройства сна, приводящие к гипоксии, ассоциированная тромбоэмболическая ЛГ и легочная венозная гипертензия вследствие дисфункции левого желудочка. Согласно данным недавних исследований, ЛГ, связанная с заболеваниями соединительной ткани (СЗСТ-ЛАГ), имеет некоторые отличительные клинические аспекты от других видов ЛГ: широкая распространенность, вовлечение в патологический процесс венозного русла и сердца, менее благоприятный прогноз, наличие алгоритма обнаружения — DETECT, ответ на иммуносупрессивную терапию, частое наличие комбинированных форм. Давление в легочной артерии может быть измерено с помощью эхокардиографии, но золотой стандарт для диагностики — катетеризация правых отделов сердца. ЛАГ-специфическая терапия, кроме иммуносупрессивной терапии, является самым распространенным методом лечения СЗСТ-ЛАГ. В настоящее время существует много противоречивых мнений относительно начала и срока назначения этих препаратов. В данном обзоре мы обсуждаем эпидемиологию, патогенез, клиническую картину СЗСТ-ЛАГ, акцентируем внимание врачей на ранних методах диагностирования заболевания, использовании алгоритма раннего выявления осложнения и подходах к лечению пациентов.

Noninvasive Risk Score to Screen for Pulmonary Hypertension With Elevated Pulmonary Vascular Resistance in Diseases of Chronic Volume Overload

Volume overload promotes pulmonary hypertension (PH) through pulmonary venous hypertension. However, PH with elevated pulmonary vascular resistance (hereafter PH-PVR) may develop in patients with diseases of volume overload, such as heart failure or chronic kidney disease (CKD). In such cases, volume management alone may be insufficient to slow PH progression. An accurate, noninvasive method to screen for PH-PVR in these diseases would facilitate early targeted therapy. We integrated invasive hemodynamic and echocardiography data collected from a single-center clinical cohort and identified patients with CKD or heart failure at the time of assessment. We applied penalized regression to derive a risk score of clinical parameters and echocardiography data associated with PH-PVR and categorized patients into low- (≤5 points), intermediate- (6-10 points), or high-risk (>10 points) groups. Using an internal validation strategy, we evaluated the ability of this risk score to predict PH-PVR and determined the association of this risk classification with 3-year all-cause mortality. Of 2422 patients, 42.4% had PH-PVR. In adjusted analyses, tricuspid regurgitant velocity, right ventricular function, BMI, heart rate, and hemoglobin most strongly associated with PH-PVR. The risk score significantly associated with PH-PVR (age-adjusted odds ratio 11.69 for the highest-risk group, 95% confidence interval [CI] 6.54-20.92). The high-risk group also associated with a significantly higher risk of 3-year all-cause mortality in adjusted analyses (hazard ratio 1.85, 95% CI 1.50-2.27). In conclusion, a noninvasive risk score derived from echocardiography and clinical parameters significantly associated with PH-PVR and all-cause mortality in a cohort of patients with CKD and heart failure.

Y-006. Predicting algorithm for risk of pre-eclampsia using 1st trimester circulating microRNAs

In potential liver transplant candidates, pulmonary vascular diseases, including portopulmonary hypertension (PoPH) and pulmonary venous hypertension (PVH), can be associated with high morbidity and mortality. Although there are clear guidelines regarding management and transplant listing criteria for patients with PoPH, the listing criteria for PVH are not well defined.The aim of this study was to describe and compare the perioperative and postoperative morbidity and mortality associated with PoPH and PVH in patients undergoing liver transplantation.We conducted a retrospective observational study of all patients referred for liver transplantation to our center between 2005 and 2015 who underwent a right heart catheterization (RHC) for screening for pulmonary hypertension as suggested by initial echocardiography. Based on the RHC data, the patients were grouped into no pulmonary hypertension (No PH), PoPH, and PVH categories. In patients who underwent liver transplantation, we recorded vital status intraoperatively and at 30 days and 1-year post-transplant, and we recorded the incidence of postoperative cardiopulmonary and renal complications.Of the 134 patients who underwent RHC as part of the initial transplant evaluation, 50 patients were successfully transplanted. There was 1 intraoperative death in the PoPH group. No significant difference in mortality was noted between the No PH, PoPH, and PVH groups intraoperatively and 30 days after liver transplantation. At 1 year, the survival rates were 100%, 69.2%, and 94.1% in the No PH, PoPH, and PVH groups, respectively. With respect to cardiopulmonary and renal complications, no statistically significant difference was noted among the groups, though there was a trend toward increased post-transplant reversible pulmonary complications in the PVH group.Our findings suggest that the post-transplant outcomes of patients with PoPH and PVH are similar. In light of the growing recognition of diastolic dysfunction and cirrhotic cardiomyopathy in decompensated cirrhotic patients at the time of transplant, the issue of pulmonary hypertension related to PVH will gain increasing importance as we assess these patients for transplantation. Therefore, future studies are needed to define evidence based guidelines to determine candidacy for liver transplantation in the context of PVH.

Left atrial myxoma swinging like a ball valve thrombus: “The wrecking ball effect”

Left atrial myxoma is the most common benign primary tumor of the heart, more common in females, and often presents with non-specific symptoms with incidental detection in echocardiography. The bimodal mode of presentation of the left atrial myxoma is as follows: one group of patient’s presents with symptoms mimicking infective endocarditis and the other group of patient’s presents with symptoms of typical mitral stenosis. Large left atrial myxoma acting as a ball valve thrombus causing complete obstruction of the mitral valve and causing pulmonary venous hypertension with acute pulmonary edema has not been described in the literature so far. We report a rare case of large left atrial myxoma swinging like a ball valve thrombus across the mitral valve in a 76-year-old female presenting with acute pulmonary edema and desaturation. The patient underwent emergent surgical excision of the left atrial myxoma which relieved the pulmonary edema and desaturation as well. Left atrial myxoma being completely occlusive led to passive pulmonary venous hypertension and acute pulmonary edema: a rare phenomenon to be observed in a clinical scenario.