Postoperative Pulmonary Vascular Resistance Research Articles

Riociguat as bridging therapy to pulmonary endarterectomy in patients with chronic thromboembolic pulmonary hypertension: a retrospective cohort study.

In operable chronic thromboembolic pulmonary hypertension (CTEPH) patients, the utilization of bridging therapy with targeted medications prior to pulmonary endarterectomy (PEA) remains a topic of controversy, despite being common in cases of severe hemodynamic impairment. This study aims to assess the impact of riociguat as a bridging therapy on postoperative hemodynamics and outcomes. We conducted a retrospective study involving patients undergoing PEA from December 2016 to November 2023. Patients were categorized into two groups based on the use of riociguat before PEA. Pulmonary vascular resistance (PVR) following riociguat administration was assessed pre-PEA. Postoperative outcomes, including mortality, complications, and hemodynamics, were compared, employing propensity score matching analysis. Among the patients, 41.8% (n=56) received riociguat as bridging therapy. In patients with PVR ≥800 dynes·sec·cm-5, riociguat resulted in a reduction in PVR {1,207 [974-1,698] vs. 1,125 [928-1,486] dynes·sec·cm-5, P<0.01}, while no significant difference was observed in patients with PVR <800 dynes·sec·cm-5 {641 [474-740] vs. 600 [480-768] dynes·sec·cm-5, P=0.46}. After propensity score matching, each group included 26 patients. The overall perioperative mortality rate was 2.6%. Postoperative PVR {326 [254-398] vs. 361 [290-445] dynes·sec·cm-5, P=0.35} was similar in the riociguat group compared to the control group. The incidence of residual pulmonary hypertension (PH) and other postoperative outcomes were also comparable. The use of riociguat as bridging therapy demonstrated hemodynamic improvement before PEA in patients with high preoperative PVR. However, no additional benefits in postoperative mortality or hemodynamics were observed.

A Novel Dual Energy Computed Tomography Score Correlates With Postoperative Outcomes in Chronic Thromboembolic Pulmonary Hypertension.

To compare dual-energy computed tomography (DECT) based qualitative and quantitative parameters in chronic thromboembolic pulmonary hypertension with various postoperative primary and secondary endpoints. This was a retrospective analysis of 64 patients with chronic thromboembolic pulmonary hypertension who underwent DECT. First, a clot score was calculated by assigning the following score: pulmonary trunk-5, each main pulmonary artery-4, each lobar-3, each segmental-2, and subsegmental-1 per lobe; the sum total was then calculated. The perfusion defect (PD) score was calculated by assigning 1 point to each segmental PD. The combined score was calculated by adding clot and PD scores. For quantitative evaluation, we calculated perfused blood volume (PBV) (%) of each lung and the sum of both lungs. Primary endpoints included testing association between combined score and total PBV with change in mean pulmonary arterial pressure ([mPAP], change calculated as preop minus postop values). Secondary endpoints included explorative analysis of the correlation between combined score and PBV with change in preoperative and postoperative pulmonary vascular resistance, change in preoperative 6-minute walk distance (6MWD), and immediate postoperative complications such as reperfusion edema, ECMO placement, stroke, death and mechanical ventilation for more than 48 hours, all within 1 month of surgery. Higher combined scores were associated with larger decreases in mPAP ( =0.27, P =0.036). On average, the decrease in mPAP (pre mPAP-post mPAP) increased by 2.2mmHg (95% CI: -0.6, 5.0) with each 10 unit increase in combined score. The correlation between total PBV and change in mPAP was small and not statistically significant. During an exploratory analysis, higher combined scores were associated with larger increases in 6MWD at 6 months postprocedure ( =0.55, P =0.002). Calculation of DECT-based combined score offers potential in the evaluation of hemodynamic response to surgery. This response can also be objectively quantified.

Impact of sex on outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension

The impact of sex on long-term outcomes after pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (PH) remains unclear. We therefore examined the early and long-term outcome after PEA to determine whether sex had an impact on the risk of residual PH and need for targeted PH medical therapy. Retrospective study of 401 consecutive patients undergoing PEA at our institution between August2005 and March2020 was performed. Primary outcome was the need for targeted PH medical therapy postoperatively. Secondary outcomes included survival and measures of hemodynamic improvement. Females (N =203, 51%) were more likely to have preoperative home oxygen therapy (29.6% vs 11.6%, p<0.01), and to present with segmental and subsegmental disease compared to males (49.2% vs 21.2%, p<0.01). Despite similar preoperative values, females had higher postoperative pulmonary vascular resistance (final total pulmonary vascular resistance after PEA, 437Dynes∙s∙cm-5 vs 324 Dynes∙s∙cm-5 in males, p<0.01). Although survival at 10 years was not significantly different between sexes (73% in females vs 84% in males, p=0.08), freedom from targeted PH medical therapy was lower in females (72.9% vs 89.9% in males at 5 years, p<0.001). Female sex remained an independent factor affecting the need for targeted PH medical therapy after PEA in multivariate analysis (HR 2.03, 95%CI 1.03-3.98, p=0.04). Although outcomes are excellent for both sexes, females had greater need for targeted PH medical therapy in the long-term. Early reassessment and long-term follow-up of these patients are important. Further investigations into possible mechanisms to explain the differences are warranted.

Long-term outcomes of combined pulmonary endarterectomy and additional balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension.

The early and long-term outcomes after pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH) have been established by several high-volume centers, but the impact of postoperative residual pulmonary hypertension affecting postoperative clinical parameters remains unclear. This study aims to investigate the institutional surgical results of PEA and to evaluate the efficacy of additional balloon pulmonary angioplasty (BPA) for residual pulmonary hypertension. We retrospectively reviewed 222 patients (57.7 ± 12.9years old, 141 female) who underwent PEA for CTEPH at the National Cerebral and Cardiovascular Center between 2000 and 2020. The preoperative mean pulmonary artery pressure (mPAP) was 45.6 ± 9.7mmHg and pulmonary vascular resistance (PVR) was 1062 ± 451 dyne*sec/cm-5. Postoperative mPAP (23.4 ± 11mmHg, 204 patients, P < 0.001) and PVR (419 ± 291 dyne*sec/cm-5, 199 patients, P < 0.001) significantly improved after PEA. Since 2011, 62 patients (28%) underwent BPA after PEA for "catecholamine dependent" residual PH 1month after PEA in 14, "scheduled" BPA with residual PH 1year after PEA in 32, and 16 "symptomatic" patients without residual PH. Their mPAP had significantly improved by PEA (48.1 ± 7.7 to 32.0 ± 10.2mmHg, P < 0.001), and further improved (33.8 ± 11.1 to 26.5 ± 9.1mmHg, P < 0.001) after BPA. PEA provided immediate and substantial improvements in pulmonary hemodynamics and favorable long-term survival. In addition, postoperative BPA improved postoperative clinical parameters for eligible patients regardless of the presence of residual PH.

Management of failing bidirectional cavopulmonary shunt: Influence of additional systemic-to-pulmonary-artery shunt with classic Glenn physiology

ObjectivesSevere hypoxemia in the early postoperative period after bidirectional cavopulmonary shunt (BCPS) is a critical complication. We aimed to evaluate patients who underwent additional systemic to pulmonary shunt and septation of central pulmonary artery (partial takedown) after BCPS. MethodsThe medical records of all patients who underwent BCPS between 2007 and 2020 were reviewed. Patients who underwent partial takedown were extracted and their outcomes were analyzed. ResultsOf 441 BCPS patients, 27 patients (6%) required partial takedown. Most frequent diagnosis was hypoplastic left heart syndrome (n = 14; 52%). Additional complicating factors included pulmonary artery hypoplasia (n = 12) and pulmonary venous obstruction (n = 3). Thirteen patients (48%) underwent partial takedown on the same day of BCPS, and all of them survived the procedure. The remaining 14 patients (52%) underwent partial takedown between postoperative 1 to 64 days. The reasons for partial takedown were: postoperative high pulmonary vascular resistance (n = 4), early BCPS (<90 days) with PA hypoplasia (n = 3), mediastinitis/pneumonia (n = 3), pulmonary venous obstruction (n = 2), ventricular dysfunction (n = 1), and recurrent pneumothorax (n = 1). Four patients experienced hospital deaths. Six patients died after discharge, 10 achieved Fontan completion, and 6 were alive and waiting for Fontan. Overall survival after partial takedown was 54% at 3 years. The pulmonary venous obstruction (P = .041) and genetic/extracardiac anomalies (P = .085) were identified as risks for mortality after partial takedown. ConclusionsThe partial takedown resulted in a 3-year survival rate of more than 50%. Of these patients, a significant number underwent successful Fontan completion who would exhibit potential early death with conservative treatment.

Impact of Sex on Outcome After Pulmonary Endarterectomy (PEA) for Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

<h3>Purpose</h3> The impact of sex on long term outcomes after PEA for CTEPH remains unclear. We therefore examined phenotypical and genotypical differences between males and females to determine whether sex had an impact on outcomes after PEA. <h3>Methods</h3> A retrospective study of 401 consecutive patients undergoing PEA at our institution between 08/2005 and 03/2020 was performed. Outcomes included survival, functional class and need for postoperative targeted PH therapy. PEA specimens were analyzed in 3 males and 2 females using single cell RNA sequencing. <h3>Results</h3> Females (n=203, 51%) were more likely to have preoperative home oxygen therapy (29.6% vs 11.6%, p<0.01), and to present with segmental and subsegmental disease compared to males (49.2% vs 21.2%, p<0.01). Despite similar preoperative values, females had less improvement in postoperative total pulmonary vascular resistance (final TPR after PEA, 437 Dynes•s•cm⁻⁵ vs 324 Dynes•s•cm⁻⁵ in males, p<0.01). Survival at 10 years reached 73% in females and 84% in males, but females had worse functional class and higher requirement for PH therapy (Figure). Female sex remained an independent factor affecting the need for targeted PH therapy after PEA in a multivariate analysis (HR 2.03, 95%CI 1.03-3.98, p=0.04). Both sexes expressed TGF-β1 in endothelial cells from the PEA specimen, but the receptor TGFβR1 was predominantly expressed in females (Figure). <h3>Conclusion</h3> In our cohort, we found significant differences between males and females undergoing PEA. Although outcomes are excellent for both sexes, females had greater need for targeted PH therapy in the long term. Greater TGFβR1 expression in females PEA specimen suggests biological differences in CTEPH pathology.

Evaluation of Asymmetric Dimethylarginine Levels in Patients With Chronic Thromboembolic Pulmonary Hypertension Undergoing Pulmonary Endarterectomy

Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary embolism, and pulmonary endarterectomy (PEA) is the surgical treatment. Asymmetric dimethylarginine (ADMA) levels are increased in pulmonary hypertension. This study aimed to investigate serum ADMA levels in patients with CTEPH, the effect of PEA on ADMA, and its prognostic value in long-term mortality. Eighty (80) patients with CTEPH and 32 healthy controls were included. Preoperative serum ADMA levels, determined using an enzyme-linked immunosorbent assay, were compared between patients with CTEPH and controls. Of 80 patients, 64 had PEA. Pre- and 6-month postoperative serum ADMA levels, 6-minute walk distance (6MWD), and haemodynamic parameters were collected from patients undergoing PEA. Patients were followed-up for survival analysis. Mean ± standard deviation serum ADMA levels were significantly higher in patients with CTEPH compared with controls (0.79±0.32 μmol/L vs 0.52±0.12 μmol/L; p=0.0001). Statistically significant differences were observed between preoperative and postoperative serum ADMA levels (0.78±0.30 μmol/L vs 0.62±0.22 μmol/L; p=0.0001), 6MWD (p=0.0001), and pulmonary vascular resistance (p=0.0001) in 60 patients who underwent and survived PEA. The decrease in serum ADMA levels and increase in 6MWD were significantly correlated (r=-0.286, p=0.027). No other correlation was found. Perioperative mortality was 6.3%, and the survival rate with a mean follow-up of 34.57±8.20 months was 93.3%. Patients with serum ADMA levels >0.8 μmol/L had a significantly lower survival rate (logrank: 5.86; p=0.015). Levels of circulating ADMA might add diagnostic and prognostic information in CTEPH. Pulmonary endarterectomy is associated with an improvement in serum ADMA levels. Preoperative serum ADMA levels may be useful for estimating the outcome of PEA.

Understanding Longitudinal Changes in Pulmonary Vascular Resistance After Left Ventricular Assist Device Implantation

BackgroundElevated pulmonary vascular resistance (PVR) is common in patients with advanced heart failure. PVR generally improves after left ventricular assist device (LVAD) implantation, but the rate of decrease has not been quantified and the patient characteristics most strongly associated with this improvement are unknown. Methods and ResultsWe analyzed 1581 patients from the Interagency Registry for Mechanically Assisted Circulatory Support registry who received a primary continuous-flow LVAD, had a baseline PVR of ≥3 Wood units (WU), and had PVR measured at least once postoperatively. Multivariable linear mixed effects modeling was used to evaluate independent associations between postoperative PVR and patient characteristics. PVR decreased by 1.53 WU (95% confidence interval [CI] 1.27–1.79 WU) per month in the first 3 months postoperatively, and by 0.066 WU (95% CI 0.060–0.070 WU) per month thereafter. Severe mitral regurgitation at any time during follow-up was associated with a 1.29 WU (95% CI 1.05–1.52 WU) higher PVR relative to absence of mitral regurgitation at that time. In a cross-sectional analysis, 15%–25% of patients had persistently elevated PVR of ≥3 WU at any given time within 36 months after LVAD implantation. ConclusionThe PVR tends to decrease rapidly early after implantation, and only more gradually thereafter. Residual mitral regurgitation may be an important contributor to elevated postoperative PVR. Future research is needed to understand the implications of elevated PVR after LVAD implantation and the optimal strategies for prevention and treatment.

A Clinical Prediction Model for Normalization of Pulmonary Vascular Resistance After Left Ventricular Assist Device Implantation

Background Patients with heart failure with reduced ejection fraction (HFrEF) and elevated pulmonary vascular resistance (PVR) are at risk increased for poor outcomes and may be ineligible for heart transplantation (HT). Left ventricular assist device (LVAD) therapy can lower PVR for certain patients, but it is difficult to identify these patients preoperatively. Methods Using the INTERMACS registry, we developed a clinical prediction model to predict the likelihood of PVR normalization (≤ 3 WU) or HT within 1 year of LVAD implant among patients with baseline PVR > 3 WU. Backwards stepwise selection was used to select variables from 14 candidate predictors and logistic regression was used to estimate model coefficients. Model performance was summarized using measures of discrimination (c-statistic) and calibration (Harrel's EAVG). Internal validation was performed using bootstrap resampling over 200 iterations to generate an average calibration slope, which was used to uniformly shrink all model coefficients to generate the final model. Results There were 2209 patients available for analysis. Median preoperative PVR was 4.26 WU (IQR 3.54-5.54 WU) and the median time from LVAD implant to postoperative PVR assessment was 3 months. 1596 patients (72%) experienced PVR normalization or were transplanted within 1 year. 412 (19%) patients died and 201 (9%) were alive with persistently elevated PVR at 1 year. Final model coefficients are shown in the Table. Important predictors included older age (OR 0.72 per 10 years), severe baseline mitral regurgitation (MR) (OR 2.49 vs no MR), and increased baseline pulmonary capillary wedge pressure (PCWP) (OR 1.24 per 5 mmHg increase). The model c-statistic was 0.66 and EAVG was 0.004. Conclusion Among patients with elevated PVR, preoperative patient characteristics such as severe MR and elevated PCWP are able to predict the likelihood of PVR normalization or HT within 1 year of LVAD implantation. These findings can help to inform decision making and frame patient expectations regarding anticipated improvements in pulmonary vascular disease following LVAD surgery. Future studies are needed to investigate potential management strategies to increase the likelihood of PVR normalization with LVAD support.

Characteristics Associated with Improved Pulmonary Vascular Resistance Following LVAD Surgery

LVAD therapy improves pulmonary vascular resistance (PVR) in patients with heart failure, but the patient characteristics associated with PVR reduction are unknown. Primary continuous-flow LVAD recipients in the INTERMACS registry with implant PVR ≥3 Wood units (WU) and at least one reported postoperative PVR were included. Post-implant PVR was modeled as a function of 29 covariates using multivariable linear mixed effects modeling. 3741 patients had pre- and post-implant PVR available, of which 1581 (42%) had implant PVR ≥3 WU. Median follow up time was 6 mo (range 1 wk to 102 mo). While median time to PVR < 3 WU was 12 mo, 272 (17%) patients did not experience decrease in PVR to < 3 WU. Characteristics independently associated with PVR throughout follow up are in the Table. LVAD support was associated with a 0.06 WU per month reduction in PVR. More severe mitral regurgitation (MR), both at baseline and across follow-up, was associated with higher PVR-by as much as 1.6 WU-compared with no MR. More severe baseline tricuspid regurgitation was associated with lower PVR. While LVAD therapy is associated with PVR reduction, about 1 in 6 LVAD recipients with high baseline PVR do not experience PVR normalization. Residual MR is associated with higher postoperative PVR, highlighting the need for prospective studies of concomitant surgical repair of preoperative MR. These data may help inform decision making regarding LVAD implant strategies for patients with pulmonary hypertension.

Extracorporeal membrane oxygenation after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension.

Pulmonary endarterectomy (PEA) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH) but can result in respiratory and cardiac complications that may require extracorporeal membrane oxygenation (ECMO). We reviewed our experience with ECMO in patients undergoing PEA. Between January 2012 and August 2015, 35 patients underwent PEA for CTEPH. In all, four patients (11%) required veno-arterial (V-A) ECMO support due to severe cardiac and respiratory failure, including severe reperfusion pulmonary edema and persistent pulmonary hypertension. No significant differences in preoperative characteristics were found between patients who required ECMO and those who did not require ECMO. ECMO support was associated with a significantly higher incidence of postoperative respiratory complications, a longer intensive care unit stay, increased in-hospital mortality, residual pulmonary hypertension, and postoperative balloon pulmonary angioplasty (BPA). The postoperative mean pulmonary artery pressure and pulmonary vascular resistance were significantly higher in patients requiring ECMO. All patients requiring ECMO were successfully weaned off ECMO support (100%), and three of them were discharged from the hospital alive (75%). Patients with CTEPH may benefit from ECMO after PEA for cardiac and respiratory complications. A prompt decision to use V-A ECMO is critical for a successful outcome in these patients.

Pulmonary Thromboendarterectomy for Pulmonary Hypertension Before Considering Transplant.

In cases of chronic thromboembolic pulmonary hypertension (CTEPH), referral for possible surgical intervention is important because surgery can be curative. Surgery necessitates cardiopulmonary bypass and deep circulatory arrest with pulmonary thrombectomy and bilateral endarterectomy (PTE). If surgery fails, lung transplant is the next best surgical option. Medical treatment is also an important adjunct. A 35-year-old female presented 3 months after a pulmonary embolus was found to be completely occluding her left pulmonary artery. She was found to have pulmonary hypertension with a pulmonary artery pressure of 81/33 mmHg, with a mean pressure of 52 mmHg. The right atrial pressure was also severely elevated at 29 mmHg, and her echocardiogram revealed severe tricuspid regurgitation and severe right ventricular dysfunction. She underwent PTE and postoperatively was followed by the heart failure team. Her 6-minute walk distance improved from 396 meters at 1 month to 670 meters at 7 months, and her pulmonary artery pressure improved significantly to 55/17 mmHg with a mean pressure of 31 mmHg. The patient's right atrial pressure also improved significantly from 29 mmHg to 13 mmHg. CTEPH is likely underrecognized, and patients with pulmonary hypertension or a history of pulmonary embolism should be screened for CTEPH. This case illustrates the surgical treatment for CTEPH and discusses alternative and adjunctive treatments. Residual pulmonary hypertension after PTE occurs in approximately 35% of patients. Overall, 4-year mortality rates after surgery appear to be approximately 15%, and mortality rates correlate with the postoperative pulmonary vascular resistance. Recognition of chronic pulmonary thromboembolic disease as the etiology of pulmonary hypertension warrants evaluation for surgery.

The role of preoperative sildenafil therapy in controlling of postoperative pulmonary hypertension in children with ventricular septal defects.

Introduction: Most of the ventricular septal defects (VSD) are complicated with pulmonary arterial hypertension (PAH) which is the major cause of pulmonary hypertensive crisis and right ventricular failure. Methods: We reviewed clinical outcomes of 63 infants who underwent cardiac surgery and were divided into three groups. Control group (n=20) did not received sildenafil while group A (n = 22) received drug (0.3 mg/kg) before and after surgery. Group B (n=21) received drug at the initiation of surgery. Demographic data, preoperative and postoperative variables were compared among the patients. Results: Patients in the group A had lower preoperative pulmonary arterial pressure (PAP) compared to other groups (P < 0.001). Also, patients in control group had longer cardiopulmonary bypass time (P < 0.05). Postoperative PAP in patients of group A and B decreased significantly compared to control group (P < 0.001). Also, pre- and postoperative PVR (pulmonary vascular resistance) showed a significant decrease in group A compared with control and group B (P < 0.001). The intubation time in patients of the control group was significantly more prolonged compared with patients of group A and B (P < 0.001). Moreover, the length of ICU stay was significantly longer in patients of control group compared with group A and B (P < 0.001). Conclusion: Preoperative sildenafil therapy seems to be effective and safe to prevent postoperative PAH and pulmonary hypertensive crisis in children with ventricular septal defects and has a positive impact on postoperative care.

Prognostic and pathophysiological marker for patients with chronic thromboembolic pulmonary hypertension: Usefulness of diffusing capacity for carbon monoxide at diagnosis.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease in some patients, despite improved treatments. Microvasculopathy has been implicated in the poor outcomes of patients with CTEPH. A reduction in the diffusing capacity for carbon monoxide (DLCO ) was previously suggested to indicate microvasculopathy in CTEPH patients; therefore, we assessed DLCO /alveolar ventilation (DLCO /VA ) as a prognostic and pathophysiological marker in CTEPH. We performed a retrospective cohort study of 214 CTEPH patients consecutively diagnosed between 1986 and 2011. After exclusion of 24 patients because of missing DLCO data or severe obstructive impairment, the mortality rates of medically treated patients classified with normal or decreased DLCO values were compared, and prognostic factors were determined. The relationship between long-term surgical outcomes and DLCO /VA was also investigated. Ninety-one inoperable patients were treated medically, two of whom underwent balloon pulmonary angioplasty. Ninety-nine underwent pulmonary endarterectomy. The 5-year survival rate of medically treated patients was significantly lower in patients with decreased DLCO /VA than in those with normal values (61.4% vs 90.4%, P = 0.017). Decreased preoperative DLCO /VA was associated with a smaller percent decrease in post-operative pulmonary vascular resistance, but not with the extent of proximal thrombi; these results may support our hypothesis that DLCO reflects microvascular involvement. Decreased DLCO /VA was associated with poor outcomes of medically treated CTEPH patients; and may be useful for identifying high-risk patients, potentially leading to earlier and more appropriate interventions.

Pulmonary Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension Due to Subclavian Vein Thrombosis From Bilateral First Rib Anomaly

SESSION TITLE: Pulmonary Vascular Disease Cases ISESSION TYPE: Affiliate Case Report SlidePRESENTED ON: Monday, October 26, 2015 at 11:00 AM - 12:00 PMINTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) results from unresolved pulmonary emboli originating from a site of venous thrombosis. Development of CTEPH from subclavian vein (SCV) thrombosis due to thoracic outlet syndrome has been rarely reported and none yet associated with bilateral first rib fractures.CASE PRESENTATION: A 19-year-old previously healthy female noted to have acute dyspnea and hemoptysis was diagnosed with acute pulmonary embolism. She was treated with low molecular weight heparin then warfarin at therapeutic levels. However, developed recurrent pulmonary embolism with severe right heart failure 4 months after her initial embolic event. A large left SCV thrombosis associated with thoracic outlet syndrome was identified. She was also noted to have symmetric bilateral healed first rib fractures on chest x-ray without any relatable trauma history. This was deemed to be the likely underlying cause of her thoracic outlet syndrome. She underwent successful directed thrombolysis and angioplasty of her left SCV. Right heart catheterization (RHC) was performed due to persistent dyspnea, which showed mean pulmonary artery pressure (mPAP) of 37mmHg. A computed tomography angiogram revealed abnormalities consistent with CTEPH. Patient was referred to a pulmonary thromboendarterectomy (PTE) specialty center. At the time of referral, her functional status was WHO class III with repeated presyncopal episodes and exertional dyspnea. A ventilation/perfusion (V/Q) scan showed multiple mismatched perfusion defects consistent with CTEPH. A hypercoagulability evaluation was unremarkable. A RHC preoperatively showed mPAP of 47mmHg and a pulmonary vascular resistance (PVR) of 775 dyne-second/cm5. Pulmonary angiography confirmed the presence of surgically-accessible chronic thromboembolic disease. She underwent a successful bilateral PTE with postoperative PVR of 227 dyne-second/cm5 and mPAP of 20mmHg. Postoperative V/Q scan showed improvement in the areas of preoperative perfusion defects. She was discharged home after an uneventful postoperative course.DISCUSSION: This illustrates an unusual case of CTEPH from upper extremity thrombosis associated with first rib anomaly. Also, hemodynamic and clinical improvement can be achieved with PTE for CTEPH due to this condition.CONCLUSIONS: Consideration for first rib anomaly should be included as part of evaluation of CTEPH secondary to SCV thrombosis.Reference #1: Kim N et al. Chronic Thromboembolic Pulmonary Hypertension. J Am Coll Cardiol. 2013;62:S92-9.DISCLOSURE: The following authors have nothing to disclose: Stephanie Shin, Peter Fedullo, Theodore Frank, William AugerNo Product/Research Disclosure Information SESSION TITLE: Pulmonary Vascular Disease Cases I SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Monday, October 26, 2015 at 11:00 AM - 12:00 PM INTRODUCTION: Chronic thromboembolic pulmonary hypertension (CTEPH) results from unresolved pulmonary emboli originating from a site of venous thrombosis. Development of CTEPH from subclavian vein (SCV) thrombosis due to thoracic outlet syndrome has been rarely reported and none yet associated with bilateral first rib fractures. CASE PRESENTATION: A 19-year-old previously healthy female noted to have acute dyspnea and hemoptysis was diagnosed with acute pulmonary embolism. She was treated with low molecular weight heparin then warfarin at therapeutic levels. However, developed recurrent pulmonary embolism with severe right heart failure 4 months after her initial embolic event. A large left SCV thrombosis associated with thoracic outlet syndrome was identified. She was also noted to have symmetric bilateral healed first rib fractures on chest x-ray without any relatable trauma history. This was deemed to be the likely underlying cause of her thoracic outlet syndrome. She underwent successful directed thrombolysis and angioplasty of her left SCV. Right heart catheterization (RHC) was performed due to persistent dyspnea, which showed mean pulmonary artery pressure (mPAP) of 37mmHg. A computed tomography angiogram revealed abnormalities consistent with CTEPH. Patient was referred to a pulmonary thromboendarterectomy (PTE) specialty center. At the time of referral, her functional status was WHO class III with repeated presyncopal episodes and exertional dyspnea. A ventilation/perfusion (V/Q) scan showed multiple mismatched perfusion defects consistent with CTEPH. A hypercoagulability evaluation was unremarkable. A RHC preoperatively showed mPAP of 47mmHg and a pulmonary vascular resistance (PVR) of 775 dyne-second/cm5. Pulmonary angiography confirmed the presence of surgically-accessible chronic thromboembolic disease. She underwent a successful bilateral PTE with postoperative PVR of 227 dyne-second/cm5 and mPAP of 20mmHg. Postoperative V/Q scan showed improvement in the areas of preoperative perfusion defects. She was discharged home after an uneventful postoperative course. DISCUSSION: This illustrates an unusual case of CTEPH from upper extremity thrombosis associated with first rib anomaly. Also, hemodynamic and clinical improvement can be achieved with PTE for CTEPH due to this condition. CONCLUSIONS: Consideration for first rib anomaly should be included as part of evaluation of CTEPH secondary to SCV thrombosis. Reference #1: Kim N et al. Chronic Thromboembolic Pulmonary Hypertension. J Am Coll Cardiol. 2013;62:S92-9. DISCLOSURE: The following authors have nothing to disclose: Stephanie Shin, Peter Fedullo, Theodore Frank, William Auger No Product/Research Disclosure Information

Evaluation of the Microcirculation in Chronic Thromboembolic Pulmonary Hypertension Patients: The Impact of Pulmonary Arterial Remodeling on Postoperative and Follow-Up Pulmonary Arterial Pressure and Vascular Resistance.

BackgroundChronic thromboembolic pulmonary hypertension (CTEPH) is generally recognized to be caused by persistent organized thrombi that occlude the pulmonary arteries. The aim of this study was to investigate the characteristics of small vessel remodeling and its impact on the hemodynamics in CTEPH patients.Methods and ResultsHemodynamic data were obtained from right heart catheterization in 17 CTEPH patients before pulmonary endarterectomy (PEA). Lung tissue specimens were obtained at the time of PEA. Pathological observations and evaluation of quantitative changes in pulmonary muscular arteries and veins were performed using light microscopy on 423 slides in 17 patients. The relationship between the results and the hemodynamics of CTEPH was investigated. Pulmonary arteriopathy and venopathy were recognized in most cases, although no plexiform lesions and no capillary-hemangiomatosis-like lesions were detected in any of the specimens. The severity of pulmonary arteriopathy was correlated with pulmonary vascular resistance (PVR) in the postoperative and follow-up periods. The PVR and mean pulmonary arterial pressure were significantly higher in the high-obstruction group than in the low-obstruction group. The findings in pulmonary venopathy were similar to the findings seen in pulmonary veno-occlusive disease in some cases, although severe venopathy was only observed in a portion of the pulmonary veins. There was a significant correlation between the extent of pulmonary arteriopathy and venopathy, although an effect of pulmonary venopathy to hemodynamics, including pulmonary arterial wedged pressure (PAWP), could not be identified.ConclusionThe vascular remodeling of the pulmonary muscular arteries was closely associated with the hemodynamics of CTEPH. Severe pulmonary arteriopathy might be related to residual pulmonary hypertension after PEA. Those altered pulmonary arteries might be a new target for the persistent PH after the operation.

Neutrophil/Lymphocyte Ratio Can Predict Postoperative Mortality in Patients with Chronic Thromboembolic Pulmonary Hypertension.

The aim of our study was to investigate clinical importance of neutrophil/ lymphocyte ratio in patients with Chronic thromboembolic pulmonary hypertension. 125 consecutive patients with a diagnosis of Chronic thromboembolic pulmonary hypertension were operated pulmonary thromboendarterectomy in our center between February 2011 and August 2013. 106 patients included into the study due to limitations. The patients were classified into two groups as patients discharged alive (Group 1) and those dying in the hospital (Group 2). Baseline neutrophil/lymphocyte ratio level was measured by dividing neutrophil count to lymphocyte count. 84 patients (79%) were in Group 1, 22 patients (21%) were in Group 2. Patients with higher neutrophil/lymphocyte ratio in admission have a significantly higher mortality rate and postoperative pulmonary vascular resistance was found statistically significant variable to predict the mortality. Receiver operator characteristic (ROC) analysis revealed that using a cut-off point of 2.54, admission neutrophil/lymphocyte ratio predicts mortality. Also, correlation analysis showed a significant correlation between preoperative pulmonary vascular resistance and neutrophil/lymphocyte ratio. The neutrophil/lymphocyte ratio level may be a useful and noninvasive biomarker for operative risk stratification for mortality after pulmonary thromboendarterectomy.

Anaesthesia Management with Deep Hypothermia and Circulatory Arrest During Surgery for Chronic Thromboembolic Pulmonary Hypertension / Anestēzija Pie Plaušu Artēruas Endarterektomijas Dziļā Hipotermuā Ar Cirkulācijas Apiurēšanu

Abstract Chronic thromboembolic pulmonary hypertension (CTEPH) occurs in 1 to 4% after acute pulmonary embolism. CTEPH can be cured by pulmonary endarterectomy (PEA), which is approved golden standard in chronic condition. There were performed three cases of PEA in Latvian Cardiology Centre during 2013-2014. General anaesthesia under cardiopulmonary bypass (CPB) with deep hypothermic circulatory arrests was provided. The core issue is correct patient selection (in terms of central PA obstruction by thrombus) as well as pulmonary circulation recovery capacity. Neuroprotection was provided by deep hypothermia, topical cooling of the head, Trendelenburg position, mild hypocapnia, Hb 9-10 g/L and pharmacological agents. For screening postoperative cognitive function the mini mental state examination (MMSE) was used before and after the surgery. Postoperative pulmonary vascular resistance index decreased by 56.3% (right ventricular systolic pressure decreased from 93.3 ± 25.7 to 44.5 ± 11.2 mmHg). Before the surgery three patients had NYHA functional class III or IV, at the time of discharge - I or II. In one case moderate (MMSE 18) cognitive disorders was observed at discharge from the ICU. No one died neither in the hospital nor within 30 days of discharge. The surgery improved RV function and pulmonary perfusion with no considerable organ failure, except mild cognitive disorders.

Right ventricle in acute and chronic pulmonary embolism (2013 Grover Conference series).

Venous thromboembolism (VTE) encompasses deep-vein thrombosis and pulmonary embolism (PE). It is the third-most-frequent cardiovascular disease, with an overall annual incidence of 1-2 per 1,000 population. Chronic thromboembolic pulmonary hypertension (CTEPH) is regarded as a late sequela of PE, with a reported incidence varying between 0.1% and 9.1% of those surviving acute VTE. Right ventricular (RV) function is dependent on afterload. The most precise technique to describe RV function is invasive assessment of the RV-to-pulmonary vascular coupling. However, assessments of RV afterload (i.e., steady and pulsatile flow components and their product, the RC-time) may be useful hemodynamic surrogates of coupling. RV load is different in acute and chronic PE. In acute PE, more than 60% occlusion of the cross-sectional area of the pulmonary artery within a short period of time leads to abrupt hemodynamic collapse. If the time of occlusion is limited to ∼15 seconds, significant decreases in fractional area change, tricuspid annulus systolic excursion, and RV free-wall deformation (strain) occur, with the latter showing significant postsystolic shortening. These changes have similarities to ischemic stunning, and they recover within minutes. In CTEPH, studies of pulmonary vascular resistance (PVR) and pulmonary arterial compliance demonstrated low RC-times that were further lowered after pulmonary endarterectomy (PEA). Immediate postoperative PVR was the only predictor of long-term survival/freedom from lung transplantation, suggesting that the effect of PEA on opening vascular territories to flow outweighs its effect on proximal stiffness. This review summarizes the current knowledge on vascular and intrinsic RV adaptation to VTE, including CTEPH, and the role of imaging.

The change in pulmonary vascular resistance after left ventricular assist device implantation – the predictive role of platelets revisited

E-mail: marijan.pasalic@yahoo.com Purpose: While analyzing the group of patients implanted with a left ventricular assist device (LVAD) at our institution to verify which of the preand postoperative factors constitute the optimal survival outcome predictors, we determined a significant increase in postoperative pulmonary vascular resistance (PVR) values in the expired patients1. The aim of this study was to further analyze the data in order to determine which of the preoperative factors were related to the aforementioned increase in postoperative PVR values. Methods: For the 20 patients (18 M, 2 F; mean age 58.7±8.3 years) that have been implanted with an LVAD in our institution during the past 2 years, preimplantation echocardiography, right heart catheterization (RHC) and laboratory data were collected and compared according to the values of the postimplantation PVR. The groups were compared by using the adequate statistical test (t-test, Mann Whitney U test, statistical significance set at 0.05). Correlation analysis and linear regression were performed. Results: Among the 20 patients, 14 had postoperative RHC data and 4 of them were proven to have elevated PVR values (>2.4 WU). When comparing the pts. with elevated to those with normal PVR values, no significant difference was found neither in the RV function (FAC 33±7% vs 22±12%, TAPSE 1,0±0,7 cm vs 1.6±0.5 cm, NS), nor in the RV and LV dimensions (RVIDd 34±9 mm vs 35±12 mm, LVIDd 65±10 mm vs 73±9 mm). The borderline significance was found in the left ventricular EF (28±3% vs 19±8%, p=0.06) and the degree of the MR (median values 1 vs 2, p=0.05). The preoperative RHC parameters were not found to be predictive of changes in postoperative PVR (preoperative PVR 4,2±3,4 vs 3,4±1,5 WU, C.I. 1,8±0,7 vs 1,9±0,4 L/min/m, TPG 14±11 vs 13±4 mmHg and RVSWI 11,4±2,2 vs 8,9±2,1, NS). As for the laboratory values, only the platelet count significantly differed between the groups (128 ±73 vs 246±65 E3/mm, p<0.05). The correlation analysis showed a strong negative correlation between the platelet count and postoperative PVR values (r=-0,761, p<0.01). The linear regression verified the following relationship between the variables PVR=6,247-0,017xPLT, p<0.01). Conclusion: These preliminary data show that the platelet count is a significant predictor of the postoperative PVR values in patients with an LVAD (a previously shown survival predictor1). Further investigation will be conducted to explain the role of platelets in the etiology of PVR in our group of pts.