Postoperative Pulmonary Hypertension Research Articles

Surgical repair of neonatal total anomalous pulmonary venous connection: A single institutional experience with 241 cases

ObjectiveChallenges persist in surgery for neonatal total anomalous pulmonary venous connection (neoTAPVC), with the high mortality risk not mitigated over time. MethodsA prospectively collected single-center database containing all neonates with TAPVC undergoing biventricular repair in 2012 to 2020 was retrospectively reviewed. The primary outcome was death or postoperative pulmonary venous obstruction (PPVO). Based on the preoperative admission location in our hospital, patients were classified into those being admitted to cardiac intensive care unit versus neonatal intensive care unit or general pediatric intensive care unit. Access to dedicated presurgical care (DPC) was defined as patients who were preoperatively admitted to the cardiac intensive care unit. ResultsOverall, 241 patients with a median age at surgery of 14 days (interquartile range [IQR], 9-21 days) were included. Anomalous return was supracardiac in 38.6%, cardiac in 26.1%, infracardiac in 28.6%, and mixed in 6.6%. Patients receiving DPC had better survival (96.3% vs 84.3%; P = .0028) and lower incidence of PPVO (15.2% vs 28.6%; P = .011) compared with those without DPC. Patients in the DPC group were less likely to undergo operation within 24 hours on presentation (27.1% vs 40.3%; P = .041), had improved lactate clearance (1.5 [IQR, 1.0-2.2] vs 2.8 [IQR, 1.8-4.1]; P < .001), and had lower incidence of postoperative pulmonary hypertension crisis (2.8% vs 18.7%; P < .001) compared with those in no-DPC group. After matching, no difference in PPVO could be observed in patients undergoing conventional versus sutureless repair (22.6% vs 12.9%; P = .29). ConclusionsAccess to DPC potentially improves outcomes in the neoTAPVC setting; freedom from PPVO were similar using conventional versus sutureless repair.

Chronic thromboembolic pulmonary hypertension and the post-pulmonary embolism (PE) syndrome.

Over a third of patients surviving acute pulmonary embolism (PE) will experience long-term cardiopulmonary limitations. Persistent thrombi, impaired gas exchange, and altered hemodynamics account for aspects of the postpulmonary embolism syndrome that spans mild functional limitations to debilitating chronic thromboembolic pulmonary hypertension (CTEPH), the most worrisome long-term consequence. Though pulmonary endarterectomy is potentially curative for the latter, less is understood surrounding chronic thromboembolic disease (CTED) and post-PE dyspnea. Advances in pulmonary vasodilator therapies and growing expertise in balloon pulmonary angioplasty provide options for a large group of patients ineligible for surgery, or those with persistent postoperative pulmonary hypertension. In this clinical review, we discuss epidemiology and pathophysiology as well as advances in diagnostics and therapeutics surrounding the spectrum of disease that may follow months after acute PE.

Respiratory viruses and postoperative hemodynamics in patients with unrestrictive congenital cardiac communications: a prospective cohort study

BackgroundPulmonary vascular abnormalities pose a risk for severe life-threatening hemodynamic disturbances following surgical repair of congenital cardiac communications (CCCs). In the distal lung, small airways and vessels share a common microenvironment, where biological crosstalks take place. Because respiratory cells infected by viruses express a number of molecules with potential impact on airway and vascular remodeling, we decided to test the hypothesis that CCC patients carrying viral genomes in the airways might be at a higher risk for pulmonary (and systemic) hemodynamic disturbances postoperatively.MethodsSixty patients were prospectively enrolled (age 11 [7–16] months, median with interquartile range). Preoperative pulmonary/systemic mean arterial pressure ratio (PAP/SAP) was 0.78 (0.63–0.88). The presence or absence of genetic material for respiratory viruses in nasopharyngeal and tracheal aspirates was investigated preoperatively in the absence of respiratory symptoms using real-time polymerase chain reaction (kit for detection of 19 pathogens). Post-cardiopulmonary bypass (CPB) inflammatory reaction was analyzed by measuring serum levels of 36 inflammatory proteins (immunoblotting) 4 h after its termination. Postoperative hemodynamics was assessed using continuous recording of PAP and SAP with calculation of PAP/SAP ratio.ResultsViral genomes were detected in nasopharynx and the trachea in 64% and 38% of patients, respectively. Rhinovirus was the most prevalent agent. The presence of viral genomes in the trachea was associated with an upward shift of postoperative PAP curve (p = 0.011) with a PAP/SAP of 0.44 (0.36–0.50) in patients who were positive versus 0.34 (0.30–0.45) in those who were negative (p = 0.008). The presence or absence of viral genomes in nasopharynx did not help predict postoperative hemodynamics. Postoperative PAP/SAP was positively correlated with post-CPB levels of interleukin-1 receptor antagonist (p = 0.026), macrophage migration inhibitory factor (p = 0.019) and monocyte chemoattractant protein-1 (p = 0.031), particularly in patients with virus-positive tracheal aspirates.ConclusionsPatients with CCCs carrying respiratory viral genomes in lower airways are at a higher risk for postoperative pulmonary hypertension, thus deserving special attention and care. Preoperative exposure to respiratory viruses and post-CPB inflammatory reaction seem to play a combined role in determining the postoperative behavior of the pulmonary circulation.

Cardiopulmonary bypass and cardiac surgery associated pulmonary hypertension: The need for proactivity and reactivity.

Pulmonary hypertension remains an important postcardiac surgical problem requiring prompt identification and management. In this edition of the Journal of Cardiac Surgery, Fayad and colleagues provide a detailed review of this topic, including epidemiology, mechanistic underpinnings, and available treatment options. In addition to responding to postoperative pulmonary hypertension, however, proactive measures including optimal timing of intervention are paramount to preventing the development of pulmonary hypertension and its associated complications.

Outcomes and occurrence of post-operative pulmonary hypertension crisis after late referral truncus arteriosus repair.

BackgroundTruncus arteriosus (TA) is a rare congenital heart disease with a high rate of early mortality. The occurrence of post-operative pulmonary hypertension crisis (PHC), known to be a common and life-threatening complication, increases due to the irreversible development of pulmonary vascular resistance with age. We sought to figure out the risk factors for PHC and describe the surgical outcomes of TA patients with late referral (repair <1 month excluded).Materials and methodsWe retrospectively reviewed patients after TA repair between 2009 and 2021 at Shanghai Children’s Medical Center. The occurrence of PHC was defined according to post-operative Pp/Ps ≥ 1 and clinical manifestations. Risk factors for PHC and mortality were conducted by multivariable analysis.ResultsA total of 98 patients were treated, including 55 males and 43 females. The median age at repair was 121 (69, 245) days. Post-operative PHC occurred in 22 (22.4%) patients with a median age of 186 (122, 293) days. By multivariable analysis, patients with the sum of Z-score of pre-operative bilateral pulmonary artery (PA) diameters (OR: 1.6, 95% CI: 1.2–2.3, P = 0.01) was more likely to experience PHC. Longer CPB duration contributed to early death (OR: 1.0, 95% CI: 1.0–1.0, P = 0.01). Total survival at 10 years was 81.4%. In 4.5 (2.9, 7.5) years of follow-up, twenty-six patients received 30 reinterventions. Valved reconstruction of RVOT most predicted reinterventions (OR: 4.2, 95% CI: 1.4–13.0, P = 0.01).ConclusionSurgical repair of TA patients with late referral has resulted in comparatively favorable early and mid-term outcomes. PHC occurred more commonly in patients with overextended bilateral PA pre-operatively. Meanwhile, valved reconstruction of RVOT would more likely lead to early reintervention.

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.

A systematic review of clinical study evidence for pulmonary vasodilator therapy following surgery with cardiopulmonary bypass in children with CHD.

Complications from pulmonary hypertension are one of the leading contributors to morbidity and mortality post-cardiopulmonary bypass surgery in children with CHD. Pulmonary vasodilator therapies are commonly used post-operatively, but the optimal target patient population, therapy choice, timing of therapy initiation, and duration of therapy are not well defined. We used PubMed and EMBASE to identify studies from 2000 to 2020 investigating the use of pulmonary vasodilator therapy post-cardiopulmonary bypass in children aged 0-18 years. To ensure eligibility criteria, studies were systematically reviewed by two independent reviewers. We identified 26 studies of 42,971 children across four medication classes; 23 were single centre, 14 were prospective, and 11 involved randomisation (four of which employed a placebo-control arm). A disproportionate number of children were from a single retrospective study of 41,872 patients. Definitions varied, but change in pulmonary haemodynamics was the most common primary outcome, used in 14 studies. Six studies had clinical endpoints, with mortality the primary endpoint for two studies. Treatment with inhaled nitric oxide, iloprost, and sildenafil all resulted in improved haemodynamics in specific cohorts of children with post-operative pulmonary hypertension, although improved outcomes were not consistently demonstrated across all treated children. Iloprost may be a cheaper alternative to inhaled nitric oxide with similar haemodynamic response. Studies were predominantly single-centre, a control arm was rarely used in randomised studies, and haemodynamic endpoints varied significantly. Further research is needed to reduce post-operative morbidity and mortality from pulmonary hypertension in children with CHD.

Postoperative Pulmonary Hemodynamics and Systemic Inflammatory Response in Pediatric Patients Undergoing Surgery for Congenital Heart Defects.

There is scarce information about the relationships between postoperative pulmonary hemodynamics, inflammation, and outcomes in pediatric patients with congenital cardiac communications undergoing surgery. We prospectively studied 40 patients aged 11 (8–17) months (median with interquartile range) with a preoperative mean pulmonary arterial pressure of 48 (34–54) mmHg who were considered to be at risk for postoperative pulmonary hypertension. The immediate postoperative pulmonary/systemic mean arterial pressure ratio (PAP/SAPIPO, mean of first 4 values obtained in the intensive care unit, readings at 2-hour intervals) was correlated directly with PAP/SAP registered in the surgical room just after cardiopulmonary bypass (r = 0.68, p < 0.001). For the entire cohort, circulating levels of 15 inflammatory markers changed after surgery. Compared with patients with PAP/SAPIPO ≤ 0.40 (n = 22), those above this level (n = 18) had increased pre- and postoperative serum levels of granulocyte colony-stimulating factor (p = 0.040), interleukin-1 receptor antagonist (p = 0.020), interleukin-6 (p = 0.003), and interleukin-21 (p = 0.047) (panel for 36 human cytokines) and increased mean platelet volume (p = 0.018). Using logistic regression analysis, a PAP/SAPIPO > 0.40 and a heightened immediate postoperative serum level of macrophage migration inhibitory factor (quartile analysis) were shown to be predictive of significant postoperative cardiopulmonary events (respective hazard ratios with 95% CIs, 5.07 (1.10–23.45), and 3.29 (1.38–7.88)). Thus, the early postoperative behavior of the pulmonary circulation and systemic inflammatory response are closely related and can be used to predict outcomes in this population.

Prediction of Pulmonary Arterial Pressure Level after Repair of Congenital Cardiac Communications and Discharge from the Hospital: Role of Down Syndrome and Early Postoperative Hemodynamics

Background: Postoperative pulmonary hypertension limits the success of surgical treatment in some patients with unrestrictive congenital cardiac communications. Identifying patients at risk of developing postoperative pulmonary hypertension is important to individualize follow-up strategies. Methods: We analyzed a prospective cohort of 52 pediatric patients (age 3 to 35 months) looking for perioperative predictors of mildly elevated pulmonary arterial pressure 6 months after surgery, defined as a systolic pressure greater than 30 mmHg by transthoracic echocardiography. This corresponds to a mean pulmonary arterial pressure of >20 mmHg. Clinical, echocardiographic and hemodynamic parameters were investigated. Perioperative hemodynamics was assessed by directly measuring pulmonary and systemic arterial pressures using indwelling catheters. Early postoperative pulmonary hemodynamics was defined as the mean pulmonary/systemic mean arterial pressure ratio (PAP/SAP) obtained per patient during the first 6 h of postoperative care. Results: Among the factors that were investigated as possible predictors, perioperative hemodynamics and the presence of Down syndrome were initially selected using univariate analysis (p < 0.030). Early postoperative PAP/SAP was correlated with PAP/SAP obtained in the operating room just after cardiopulmonary bypass (r = 0.70, p < 0.001), and it was higher in subjects with Down syndrome than in nonsyndromic individuals (p = 0.003). Early postoperative PAP/SAP was the only predictor selected using multivariate analysis. It was characterized as an independent predictor after adjustments for possible confounders. An early postoperative PAP/SAP of >0.35 was 76% sensitive and 74% specific at predicting a systolic pulmonary arterial pressure of >30 mmHg 6 months after surgery (hazard ratio with 95% CI 8.972 [2.428–33.158], p = 0.002). Conclusion: The hypertensive early postoperative behavior of the pulmonary circulation was strongly but not exclusively associated with Down syndrome, and it was characterized as an independent predictor of altered pulmonary arterial pressure after discharge from the hospital.

Long-Term Follow-Up of Pediatric Patients with Severe Postoperative Pulmonary Hypertension After Correction of Congenital Heart Defects

The surgical repair of congenital heart defects in children with preoperative pulmonary hypertension (PH) is to varying degree associated with the occurrence of postoperative PH. The objective of this study was to follow up children with severe postoperative PH (pulmonary arterial/aortic pressure ratio ≥ 1.0) to evaluate if pulmonary arterial pressure spontaneously normalized or needed PH-targeting therapy and to identify potential high-risk diagnoses for bad outcome. Twenty-five children who developed clinically significant severe PH on at least three occasions postoperatively were included in the follow-up (20–24 years). Data from chart reviews, echocardiographic investigations, and questionnaires were obtained. Three children died within the first year after surgery. Three children were lost to follow-up. The remaining 17 children normalized their pulmonary arterial pressure without the use of PH-targeting drugs at any time during the follow-up. Two children had a remaining mild PH with moderate mitral valve insufficiency. All three children with bad outcome had combined cardiac lesions causing post-capillary pulmonary hypertension. Normalization of the pulmonary arterial pressure occurred in almost all children with severe postoperative PH, without any need of supplemental PH-targeting therapies. All children with bad outcome had diagnoses conformable with post-capillary PH making the use of PH-targeting therapies relatively contraindicated. These data emphasize the need to perform randomized, blinded trials on the use of PH-targeting drugs in children with postoperative PH before accepting it as an indication for routine treatment.

Predictors of Failure after DeVega Repair for Functional Tricuspid Regurgitation

Background: Untreated tricuspid regurgitation during mitral valve surgery may progress to severe symptomatic tricuspid regurgitation. Concomitant repair may increase the operative risk; however, re-operative tricuspid valve surgery is a high-risk procedure. This study's objective was to identify the predictors of DeVega repair failure in patients with functional tricuspid regurgitation and concomitant mitral valve surgery.&#x0D; Methods: This research is a retrospective comparative study that included 140 patients who underwent tricuspid valve repair concomitant with mitral valve replacement. We divided the patients into two groups; the first group (n=106) included patients with no DeVega failure at six-months follow-up (The sustained repair group). The second group included 34 patients who developed moderate or higher TR after the DeVega and was named the failed repair group.&#x0D; Results: The demographic data and comorbidities were not statistically different between both groups. The preoperative atrial fibrillation (73 (69%) vs. 30 (88%)’ p= 0.027) pulmonary artery pressure (64.8±3.6 vs. 81±6.5 mmHg; p= 0.02), right ventricular dimension (4.85±0.24 vs. 5.23±0.37 cm; p= 0.03), and time between the indication of surgery and operation (8.3 ± 3.1 vs. 14.7 ± 5.4 months; p = 0.003) were higher in patients with failed DeVega repair. There was no statistically significant difference regarding the mean bypass time, cross-clamp time, ICU and hospital stay, and postoperative complications between both groups. Predictors of failure after six months were preoperative heart failure (OR: 15.4 (95% CI: 3- 92.3); p= 0.003), long time between diagnosis and surgery (OR: 12.3 (95% CI: 2.1- 84.7); p= 0.007), and postoperative severe pulmonary hypertension (OR: 24.7 (95% CI: 3.1- 199.6); p= 0.003).&#x0D; Conclusions: DeVega repair is associated with a high failure rate after six months. The study of predictors of failure could change our management plans to reach the best results for repair.

Inadequate Dosage May Lead to the Recurrence of Postoperative Pulmonary Hypertension in Patients With Congenital Heart Disease

Background: Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) occurs predominantly among patients with uncorrected congenital heart disease (CHD). It is an intractable problem to control PAH continuously and stably after an operation. Methods: 1) OPLS-DA combined with S-plot was used to retrospectively analyze the results of preoperative and postoperative PAH and 39 biochemical indicators of 235 patients admitted to Fuwai Yunnan Cardiovascular Hospital from January 2019 to December 2020. 2) Combined with Meta-analysis, the recurrence in postoperative PAH was analyzed in terms of operation factors, doses administered, and age factors. Results: 1) 4 indicators (PAH, RBC, HGB, and CO2) that reflect the prognosis of patients had been found by OPLS-DA combined with S-plot. 2) The recurrence rate of postoperative PAH was 37.02%. The comprehensive therapeutic effect of interventional closure was better than that of surgical operation. PAH was not significantly higher again in patients who received either the instruction dose or the literature dose. Postoperative combination therapy (bosentan and sildenafil) was more effective than bosentan alone. Recovery after treatment was better in infants than in the other four age groups. Conclusion: OPLS-DA combined with S-plot was used for the first time to analyze clinical examination data. In this study, this method proved to be a feasible method for analyzing clinical data We recommend interventional closure as the first choice for patients with PAH-CHD. For postoperative oral therapy, we recommend the combination therapy (bosentan with sildenafil). To prevent the recurrence, the dose should be strictly prescribed according to the instructions, literature, or body surface area converted. Moreover, we recommend treatment at a young age in these patients.

Postoperative Pulmonary Hypertension and Right Ventricular Dysfunction are Independently Associated with Poor Outcomes after LVAD Implantation

<h3>Purpose</h3> While preoperative hemodynamic risk factors associated with right ventricular (RV) dysfunction after LVAD are well-established, the relationship between postoperative hemodynamics and subsequent outcomes is poorly defined. <h3>Methods</h3> We analyzed adult patients from the STS-INTERMACS registry with hemodynamic data available at 1 or 3 months after LVAD implant. Hemodynamic parameters analyzed included measures of RV afterload (mean PA pressure [mPAP] and pulmonary vascular resistance [PVR]) and RV function (right atrial pressure [RAP], RAP:PCWP, and pulmonary artery pulsatility index [PAPi]). The primary outcome was the composite of death or late right heart failure (LRHF), and the secondary outcome was gastrointestinal bleeding (GIB), both conditional on survival to 1 month. Associations were assessed using Cox proportional hazards modeling, adjusted for 10 relevant clinical covariates. <h3>Results</h3> Among 1328 patients meeting inclusion criteria, postoperative mean RAP was 9.6 ± 6.0 mmHg and mean PCWP was 13.1 ± 6.9 mmHg. Post-LVAD pulmonary hypertension was common, with 775 (58.4%) patients having mPAP ≥ 20 mmHg and 227 (17.1%) having PVR ≥ 3 WU. mPAP (HR 1.1 per 5 mmHg increase), PVR (HR 1.1 per 1 WU increase), PAPi (HR 1.26 per log-decrease), and RAP:PCWP (HR 1.37 per log-increase) were independently associated with death or LRHF (p < 0.05 for all). When stratified by tertiles of mPAP and either PAPi or RAP:PCWP, risk varied 4-5 fold between patients with the most vs least favorable hemodynamic profiles (Figure). RAP (HR 1.21 per 5 mmHg increase), PAPi (HR 1.3 per log-decrease) and RAP:PCWP (HR 1.39 per log-increase) were associated with GIB (p < 0.01 for all). <h3>Conclusion</h3> Postoperative RV dysfunction and elevated RV afterload are associated with mortality, LRHF, and GIB after LVAD implantation. Whether strategies aimed at optimizing RV function and afterload can reduce the burden of adverse events requires prospective study.

Increased Mortality in Patients With Preoperative and Persistent Postoperative Pulmonary Hypertension Undergoing Mitral Valve Surgery for Mitral Regurgitation: A Cohort Study.

BackgroundPreoperative pulmonary hypertension (PH) is associated with excess mortality among patients with severe mitral regurgitation undergoing mitral valve surgery (MVS). However, the links between PH phenotype, pulmonary vascular remodeling, and persistent postoperative PH are not well understood. We aimed to describe the associations between components of pulmonary hemodynamics as well as postoperative residual PH with longitudinal mortality in patients with severe mitral regurgitation who received MVS.Methods and ResultsPatients undergoing MVS for severe mitral regurgitation from 2011 to 2016 were retrospectively identified within our health system (n=488). Mean pulmonary artery pressure and other hemodynamic variables were determined by presurgical right‐heart catheterization. Postoperative pulmonary artery systolic pressure was assessed on echocardiogram 42 to 365 days post‐MVS. Longitudinal survival over a mean 3.9 years of follow‐up was evaluated using Cox proportional hazards modeling to compare survival after adjustment for demographics, surgical characteristics, and comorbidities. Pre‐MVS prevalence of PH was high at 85%. After adjustment, each 10‐mm Hg increase in preoperative mean pulmonary artery pressure was associated with a 1.38‐fold increase in risk of death (95% CI, 1.13–1.68). Elevated preoperative pulmonary vascular resistance, transpulmonary gradient, and right atrial pressure were similarly associated with increased mortality. Among 231 patients with postoperative echocardiogram, evidence of PH on echocardiogram (pulmonary artery systolic pressure ≥35 mm Hg) was associated with increased risk of death (hazard ratio [HR], 2.02 [95% CI, 1.17–3.47]); however, this was no longer statistically significant after adjustment (HR, 1.55 [95% CI, 0.85–2.85]).ConclusionsIn patients undergoing MVS for mitral regurgitation, preoperative PH, and postoperative PH were associated with increased mortality.

Better Outcomes in Pulmonary Arterial Hypertension After Repair of Congenital Heart Disease, Compared With Idiopathic Pulmonary Arterial Hypertension

BackgroundPulmonary vascular changes in postoperative pulmonary artery hypertension (PAH) are similar to those seen in idiopathic PAH. Data are sparse on direct comparative midterm outcomes for these 2 high-risk populations. MethodsPatients with idiopathic or postoperative PAH referred to a large tertiary hospital between June 2005 and July 2019 were retrospectively evaluated. ResultsA total of 364 consecutive patients were studied, including 201 postoperative PAH patients and 163 patients with idiopathic PAH, with a median age of 18.7 (interquartile range 10.0, 31.5) and 7.3 (IQR: 2.9, 18.3) years, respectively. PAH-specific drugs were used in 77.7% of patients; 31.4% received combination therapy. Patients with idiopathic PAH had a shorter 6-mintue walk distance, lower percutaneous oxygen saturation, and higher B-type natriuretic peptide levels than those with postoperative PAH at diagnosis (all P < 0.001), During a median follow-up time of 3.4 (interquartile range: 2.1, 5.8) years, 56 patients (15.4%) died, and one underwent bilateral lung transplantation. Patients with postoperative PAH had better survival than those with idiopathic PAH, according to age (hazard ratio [HR] 0.128, 95% confidence interval [CI]: 0.07-0.22, P < 0.0001); Kaplan–Meier survival estimates at 5 years for idiopathic and postoperative PAH patients were 74.3% and 92.6%, respectively. Patients in New York Heart Association functional class III–IV had an over 4-fold increased risk of death (HR 4.85, 95% CI: 2.61-9.00, P < 0.0001). Patients < 18 years of age at idiopathic PAH diagnosis had a worse survival compared to adult patients (HR 6.90, 95% CI: 4.19-15.56, P = 0.040). ConclusionsPostoperative-PAH patients had better midterm survival compared to patients with idiopathic PAH. Mortality was significant in both PAH groups, reinforcing the need for early diagnosis and optimal individualized management to improve outcomes.

Preoperative right ventricular overwork is a major determinant of residual pulmonary arterial hypertension in patients with repaired arterial septal defect

Abstract Funding Acknowledgements Type of funding sources: None. Background The haemodynamic effect of atrial septal defect (ASD) is a chronic volume overload of the right heart and pulmonary vasculature. Pulmonary overcirculation is generally compensated for by the right ventricular (RV) and pulmonary arterial (PA) reserve. However, in a subset of patients, prolonged pulmonary overcirculation insidiously induces obstructive pulmonary vasculopathy, which results in postoperative residual pulmonary arterial hypertension (PAH) after ASD closure. Postoperative PAH is a major concern because it is closely associated with poor outcomes and impaired quality of life. However, to date, no clinically robust predictors of postoperative residual PAH have been clearly identified. Purpose This study sought to assess the haemodynamic characteristics of ASD patients in terms of mechano-energetic parameters and to identify the predictors of postoperative residual PAH in these patients. Methods A total of 120 ASD patients (age: 58 ± 17 years) and 46 normal controls were recruited. As previously reported, the simplified RV contraction pressure index (sRVCPI) was calculated as an index of RV external work by multiplying the tricuspid annular plane systolic excursion (TAPSE) by the pressure gradient between the RV and right atrium. RV- PA coupling was evaluated using TAPSE divided by PA systolic pressure as an index of the RV length-force relationship. These parameters were measured both at baseline and 6 months after ASD closure. Results As expected, baseline sRVCPI was significantly greater in patients with ASD than in controls (775 ± 298 vs. 335 ± 180 mm Hg • mm, P &amp;lt; 0.01), which indicated significant "RV overwork". As a result, RV-PA coupling in ASD patients was significantly impaired compared to that in controls (0.9 ± 0.8 vs. 3.5 ± 1.7 mm/mm Hg, P &amp;lt; 0.01). All 120 ASD patients underwent transcatheter or surgical shunt closure; 15 of them had residual PAH after closure. After 6 months, RV-PA coupling index significantly improved in patients without residual PAH, from 0.96 ± 0.81 to 1.27 ± 1.24 mm/mm Hg (P = 0.02). Furthermore, RV load was markedly reduced, with sRVCPI falling from 691 ± 258 to 434 ± 217 mm Hg • mm, P &amp;lt; 0.01). However, in patients with residual PAH, RV-PA coupling index deteriorated from 0.64 ± 0.23 to 0.53 ± 0.12 mm/mm Hg (P &amp;lt; 0.01). As a result, RV overload was not significantly relieved (sRVCPI; from 971 ± 382 to 783 ± 166 mm Hg • mm, P = 0.22). In a multivariate analysis, baseline pulmonary vascular resistance (hazard ratio 1.009; P &amp;lt; 0.01) and preoperative sRVPCI (hazard ratio 1.003; P &amp;lt; 0.01) revealed to be independent predictors of residual PAH. Conclusion In terms of mechano-energetic function, preoperative "RV overwork" can be used as a robust predictor of an impaired RV-PA relationship in ASD patients. Moreover, periodic assessment of sRVPCI may contribute to the better management for patients with unrepaired ASD. Abstract Figure.

Efficacy of citrulline supplementation to decrease the risk of pulmonary hypertension after congenital heart disease surgery. A local experience.

Pulmonary hypertension (PH) is a major cause of morbi-mortality among patients with congenital heart disease (CHD) and also a potentially severe complication after surgical repair. Oral citrulline, a precursor to NO synthesis, is safe and efficacious for decreasing the risk of postoperative PH. Objective: The aim of the present study was to investigate in pediatric patients the changes of plasma citrulline, arginine, homocysteine and nitric oxide (NO) metabolites and pulmonary artery pressures (PAP) pre-post cardiac surgery in order to describe our population status with regard to the risk of pulmonary hypertension and look for potential biomarkers for early detection and treatment. 16 Argentine pediatric patients with CHD undergoing cardiopulmonary bypass were randomized in two groups: (A) with and (B) without perioperative citrulline supplementation. We found that plasma citrulline median levels before surgery were lower in both groups respect to referential values, probably due to the poor nutritional status of our patients; only group A surpassed post-surgery the minimum recommended level to avoid PH. Furthermore, none of the patients in group A showed mean PAP higher than 20 mmHg, whereas in group B, 67% of the measurements were ≥ than the reference level. We reaffirm that citrulline supplementation it is effective in reducing postoperative pulmonary hypertension and biomarkers could evidence patient status as a translational medicine application.

Results of a three-year follow-up and quality of life dynamics after pulmonary thromboendarterectomy

Aim. To evaluate the results of a three-year follow-up of patients with chronic thromboembolic pulmonary hypertension (CTEPH) and quality of life (QOL) dynamics after pulmonary thromboendarterectomy (PTE).Material and methods. The study included 125 patients with CTEPH aged 49,7± 11,9 years. Three-year follow-up included the recording of adverse cardiovascular events, surgeries, death during the period from the end of hospitalization and up to three years. The SF-36 questionnaire was used to assess physical and mental wellbeing before and three years after PTE. Multivariate linear regression was used to assess the factors affecting QOL in the long-term postoperative period.Results. During a three-year follow-up, adverse cardiovascular events was recorded in 1,9% of patients. One patient underwent coronary artery bypass graft surgery and one patient — cholecystectomy. Reoperative PTE was not carried out. The overall three-year survival rate was 90,4%.At baseline, patients with CTEPH had a low level of physical and mental well-being (&lt;40 points). Three years after the operation, these parameters significantly increased (p&lt;0,05), but did not exceed 50 points. Multivariate linear regression revealed an unfavorable effect of early postoperative residual pulmonary hypertension on the physical health three years after PTE. Other factors (age, sex, body weight, comorbidity, hospital acquired complications) did not affect the physical and emotional aspects of QOL.Conclusion. Three-year survival rate in patients with CTEPH after PTE was 90,4%. During the follow-up period, no recurrent thromboembolic events were recorded. Adverse cardiovascular events were recorded in 1,9% of patients. Three years after surgery, the physical and mental health increased in comparison with preoperative values, but did not exceed 50 points on the SF-36 questionnaire. The physical aspect of QOL after surgery is affected by early postoperative residual pulmonary hypertension.

Impact of prosthesis-patient mismatch on early and late outcomes after mitral valve replacement: a meta-analysis.

BackgroundPrognostic significance of prosthesis-patient mismatch (PPM) after mitral valve replacement (MVR) remains uncertain because of the limited studies reporting inconsistent or even contrary results. This meta-analysis pooled results of all available studies comparing early and late prognoses between patients with significant mitral PPM and those without.MethodsStudies were identified by searching Pubmed, Excerpta Medica Database, Cochrane Central Register of Controlled Trials, and ClinicalTrials.gov. Impact of PPM on postoperative hemodynamic results, thirty-day mortality, overall mortality, mortality of thirty-day survivors, and primary morbidity after MVR was evaluated via meta-analysis. Robustness of pooled estimates, source of heterogeneity, and publication bias were assessed via sensitivity analyses, meta-regression as well as subgroup analysis stratified according to methodological or clinical heterogeneity, or sequential omission method, and funnel plot or Begg's and Egger's tests, respectively.ResultsNineteen cohort studies involving 9302 individuals (PPM group: n = 5109, Control group: n = 4193) were included for meta-analysis. Total PPM and severe PPM prevalence were 3.8%–85.9% and 1%–27%, with a mean value of 54.9% and 14.1%, respectively. As compared with control group, mitral PPM group demonstrated a poorer postoperative hemodynamic status of higher mean and peak residual transprosthetic pressure gradients (TPG), higher postoperative systolic pulmonary artery pressure (SPAP) and less reduction, higher postoperative pulmonary hypertension (PH) prevalence and less PH regression, smaller net atrioventricular compliance, less NYHA class decrease, higher postoperative functional tricuspid regurgitation prevalence and less regression. The PPM group also revealed a higher thirty-day mortality, long-term overall mortality, mortality of thirty-day survivors, and postoperative congestive heart failure prevalence, which were positively correlated with the severity of PPM if it was classified into tri-level subgroups. Left ventricular end-diastolic diameter, postoperative atrial fibrillation (AF) prevalence, and the AF regression were analogous between groups. Most pooled estimates were robust according to sensitivity analyses. Male patients and bioprosthesis implantation proportion were prominent source of between-study heterogeneity on thirty-day mortality. Publication bias was not significant in tests for all the outcomes, except for SPAP and TPG.ConclusionsMitral PPM would result in poorer postoperative hemodynamics and worse early and late prognosis. Severe PPM must be avoided since deleterious impact of mitral PPM was severity dependent.

Association of Preoperative Mixed Venous Oxygen Saturation with Postoperative Segmental Pulmonary Hypertension in Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collaterals.

In patients with pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs), segmental pulmonary hypertension is common; however, its pathophysiology remains to be elucidated. This study aimed to identify preoperative hemodynamic factors associated with segmental pulmonary hypertension after intracardiac repair in patients with PA/VSD/MAPCAs. This study included 12 patients with PA/VSD/MAPCAs who underwent unifocalization and intracardiac repair from 2009 at our institution. Hemodynamic measurements of preoperative and postoperative cardiac catheterization were collected. Patients were considered to have pulmonary hypertension if the mean pulmonary pressure measured postoperatively at any peripheral pulmonary artery was ≥ 20mmHg. The data were compared between patients with and without postoperative segmental pulmonary hypertension; p value < 0.05 was considered statistically significant. Intracardiac repair was performed at the age of 2.1years (range 0.5-28.2years). Unifocalization was performed separately prior to intracardiac repair in two patients and concomitantly in 10 patients. Postoperative cardiac catheterization, performed 1.2years (range 0.2-8.7years) after intracardiac repair, revealed that five patients had segmental pulmonary hypertension. Patients with postoperative pulmonary hypertension had significantly lower preoperative mixed venous oxygen saturation than those without pulmonary hypertension (57% [55-63%] vs. 65% [53-75%], p < 0.05). No significant differences were found between the groups in terms of age at intracardiac repair, preoperative arterial oxygen saturation, and preoperative peripheral pulmonary arterial mean pressure. Lower preoperative mixed venous oxygen saturation was associated with segmental pulmonary hypertension after intracardiac repair in patients with PA/VSD/MAPCAs.