Significant Pulmonary Hypertension Research Articles

Utility of Serum Matrix Metalloproteinase-7 as a Biomarker in Cholestatic Infants with Congenital Heart Disease.

Background: Matrix metalloproteinase 7 (MMP-7) is a novel biomarker for diagnosis of biliary atresia (BA), the most common cholestatic liver disease in infancy. There is a pressing need to determine the utility of MMP-7 levels in infants with congenital heart disease (CHD) to avoid unnecessary invasive diagnostic procedures in this high-risk population. We investigated the utility of MMP-7 in discriminating BA from non-BA cholestasis in infants with CHD and whether MMP-7 elevation was present in infants requiring treatment for clinically significant PH. Methods: This is a single center cross sectional study including infants <180 days of age with cholestasis and serum MMP-7 levels collected from 2019-2023. Demographic data and descriptive statistics were summarized with medians with interquartile ranges and frequencies with percentages. Median MMP-7 levels were assessed via Wilcoxon rank-sum test. Results: A total of 149 patients were included. Patients with CHD had significantly elevated MMP-7 levels relative to the non-CHD cohort (50 vs. 34 ng/mL, p=0.009). Sub-analysis comparing infants with and without PH revealed significantly elevated median MMP-7 levels in those with clinically significant PH (125 vs. 39 ng/mL, p=0.010). CHD patients with PH had greater median MMP-7 compared to CHD patients without PH (154 vs 43 ng/mL, p=0.028). Conclusions: Serum MMP-7 levels in infants with CHD-C were significantly elevated compared to those with cholestasis alone. MMP-7 may help identify non-BA cholestatic infants who have concurrent clinically significant pulmonary hypertension. Larger, prospective studies are needed to validate this finding and establish CHD-specific MMP-7 cutoffs.

Evaluation of Inhaled Nitric Oxide Generation Systems at Altitude.

Inhaled nitric oxide (INO) is a selective pulmonary vasodilator delivered from compressed gas cylinders filled to 2,200 psig (137.8 bar) with 800 ppm of NO in a balance of nitrogen. NO is currently FDA-approved for use in term or near-term infants with hypoxemia and signs of pulmonary hypertension in the absence of cardiac disease. INO has also been shown to improve oxygenation in adults with refractory hypoxemia. Current doctrine precludes the use of NO during military aeromedical transport owing to the requirement for large compressed gas cylinders. We performed a bench evaluation of 2 delivery systems that create NO from room air without the need for pressurized cylinders. We evaluated 2 portable nitric oxide INO generation systems (LungFit PH, Beyond Air Inc, Garden City, NJ and a prototype NO generator, Odic Inc, Littleton, MA) at ground level, 8,000, and 14,000 feet (2,437 and 4,267 meter) simulated altitude in an altitude chamber. The output from each device was injected into the inspiratory limb of the ventilator circuit that was attached to a test lung. A 731 ventilator (Zoll Medical, Chelmsford, MA) and T1 (Hamilton Medical, Reno, NV) were used employing 24 combinations of ventilator settings each repeated in duplicate. An INOmax DS IR was used to measure delivered INO and NO2 via a sampling line attached in the ventilator circuit inspiratory limb. A fast response oxygen analyzer (O2CAP, Oxigraf Inc, Sunnyvale, CA) was used to measure inspired FiO2. Target INO concentration was 20 ppm. Across all ventilator settings, the LungFit device delivered INO was 19.8 ± 1.6 ppm, 16.1 ± 1.9 ppm, and 11.6 ± 1.7 ppm at ground level, 8,000 ft (2,437 meter), and 14,000 ft (4,267 meter), respectively. The Odic device delivered INO dose was 20.6 ± 1.4 ppm, 21.3 ± 5.5 ppm, and 20.4 ± 9.1 ppm at ground level, 8,000 ft (2,437 meter), and 14,000 ft (4,267 meter), respectively. Both devices delivered a reliable INO dose at ground level. Altitude significantly affected INO delivery accuracy at 14,000 ft (4,267 meter) (P < 0.01) with both devices and at 8,000 ft (2,437 meter) (P < 0.01) with LungFit. Differences in INO dosage were not statistically significant with the Odic device at 8,000 ft (2,437 meter)(P > 0.05) although there were large variations with selected ventilator settings. With careful monitoring, devices creating INO from room air without cylinders could be used during aeromedical transport without the need for pressurized cylinders.

Cardiovascular disease assessment and management in liver transplantation.

The prevalence and mortality related to end-stage liver disease (ESLD) continue to rise globally. Liver transplant (LT) recipients continue to be older and have inherently more comorbidities. Among these, cardiac disease is one of the three main causes of morbidity and mortality after LT. Several reasons exist including the high prevalence of associated risk factors, which can also be attributed to the rise in the proportion of patients undergoing LT for metabolic dysfunction-associated steatohepatitis (MASH). Additionally, as people age, the prevalence of now treatable cardiac conditions, including coronary artery disease (CAD), cardiomyopathies, significant valvular heart disease, pulmonary hypertension, and arrhythmias rises, making the need to treat these conditions critical to optimize outcomes. There is an emerging body of literature regarding CAD screening in patients with ESLD, however, there is a paucity of strong evidence to support the guidance regarding the management of cardiac conditions in the pre-LT and perioperative settings. This has resulted in significant variations in assessment strategies and clinical management of cardiac disease in LT candidates between transplant centres, which impacts LT candidacy based on a transplant centre's risk tolerance and comfort level for caring for patients with concomitant cardiac disease. Performing a comprehensive assessment and understanding the potential approaches to the management of ESLD patients with cardiac conditions may increase the acceptance of patients, who appear too complex, but rather require extra evaluation and may be reasonable candidates for LT. The unique physiology of ESLD can profoundly influence preoperative assessment, perioperative management, and outcomes associated with underlying cardiac pathology, and requires a thoughtful multidisciplinary approach. The strategies proposed in this manuscript attempt to review the latest expert experience and opinions and provide guidance to practicing clinicians who assess and treat patients being considered for LT. These topics also highlight the gaps that exist in the comprehensive care of LT patients and the need for future investigations in this field.

Intraoperative Assessment of Noninvasive Left Ventricular Myocardial Work Indices in Patients Undergoing Aortic Valve Replacement

ObjectivesEvaluation of noninvasive left ventricular (LV) myocardial work (MW) enables insights into cardiac contractility and efficacy beyond conventional echocardiography. However, there is limited intraoperative data on patients undergoing surgical aortic valve replacement (AVR). The aim of this study was to describe the feasibility and the intraoperative course of this technique of ventricular function assessment in these patients and compare it to conventional two- and three-dimensional echocardiographic measurements and strain analysis. DesignProspective observational study SettingAt single university hospital Participants25 patients scheduled for isolated AVR with preoperative preserved left and right ventricular function, sinus rhythm, without significant other heart valve disease or pulmonary hypertension, and an uneventful intraoperative course. InterventionsTransesophageal echocardiography (TEE) was performed after induction of anesthesia (T1), after termination of cardiopulmonary bypass (T2), and after sternal closure (T3). Evaluation was performed in stable hemodynamics, in sinus rhythm or atrial pacing and vasopressor support with norepinephrine ≤ 0.1 µg/kg/min. Measurements and Main ResultsEchoPAC v206 software (GE Vingmed Ultrasound AS, Norway) was used for analysis of 2D- and 3D LV ejection fraction (EF), LV global longitudinal strain (GLS), LV global work index (GWI), LV global constructive work (GCW), LV global wasted work (GWW), and LV global work efficiency (GWE). Estimation of myocardial work was feasible in all patients. Although there was no significant difference in the values of 2D- and 3D-EF, GWI and GCW decreased significantly after AVR [T1 v T2, 1647 ± 380 mmHg% v 1021 ± 233 mmHg%, p<0.001; T1 v T2, 2095 ± 433 mmHg% v 1402 ± 242 mmHg%, p<0.001, respectively], while GWW remained unchanged [T1 v T2, 296 mmHg% (IQR 178-452) v 309 mmHg% (IQR 255-438), p=0.97]. This resulted in a decreased GWE directly after bypass [T1 v T2, 84 ± 6 % v 78 ± 5 %, p<0.001], but GWE already improved at the end of surgery [T2 v T3, 78 ± 5 % v 81 ± 5 %, p=0.003]. There was no significant change in the values of GWI, GCW, 2D- and 3D-LVEF before and after sternal closure [T2 v T3]. ConclusionLV MW analysis showed a reduction of LV work load after bypass in our group of patients, which was not detected by conventional echocardiographic measures. This evolving technique provides deeper insights into cardiac energetics and efficiency in the perioperative course of aortic valve replacement surgery.

Cardiac arrhythmias in patients with type 2 diabetes mellitus

Introduction. The contribution of type 2 diabetes mellitus (type 2 diabetes) and cardiovascular diseases (CVD) to the increase in disability and mortality rates worldwide continues to grow steadily. Currently, the main focus is on the problem of atherosclerotic cardiovascular diseases (ACCD) in DM, while the manifestations of cardiac arrhythmias (LDC) in conditions of carbohydrate metabolism disorders have not been sufficiently studied.The purpose of the study. To analyze and compare laboratory, instrumental and objective indicators in patients with type 2 diabetes mellitus (type 2 diabetes mellitus) and without, who have a history of cardiac arrhythmias (LDC).Materials and methods. 103 patients with LDC (52 men and 51 women) were examined. 2 groups were formed: the main group – patients with LDC and type 2 diabetes (n=51) (group 1), the control group – patients with LDC without diabetes (n=52) (group 2). A comparative analysis of laboratory-instrumental and clinical-anamnestic data in the groups was performed. Statistical data processing was performed using the statistical software package "Excel" ("Microsoft"), the program "Statistica 10" ("Statsoft Inc"). The value of p &lt;0.05 was taken as the significance level. The reliability of the differences between the groups was determined using the Mann-Whitney U-test, and the correlation analysis was carried out using the Spearman criterion.Results. The average age of the general group was 67[18;97] years, patients with type 2 diabetes 69 [53;93] years, patients without diabetes 65 [18;97] years. According to the performed regression analysis, it was found that DM in patients was associated with the detection of atrial fibrillation (OR 2.69 (95% CI 1.076;6.71), p=0.034), in particular, its constant form (p=0.025, coefficient. Kramer's V = 0.33), as well as with the presence of supraventricular extrasystole (NJES) (OR 0.235 (95%CI 0.09;0.59), p=0.002, coefficient. Kramer's V = 0.311). At the same time, there was no significant association of DM with the presence of paroxysmal and persistent forms of atrial fibrillation, atrial flutter (TP), BLNPG, AV block, CA block, ventricular extrasystole, supraventricular tachycardia (NVT) and SSSI.When evaluating the functional parameters of the myocardium according to Echo-K G data, it was found that in the main group, compared with the control group, significant (34% vs. 27%) and pronounced (18% vs. 6%) pulmonary hypertension were more common, but the difference was not statistically significant (p&gt;0.05).When assessing the structural and geometric parameters of the myocardium, it was found that concentric myocardial hypertrophy was more common in the main group (22% vs. 15%), and in the control group – concentric myocardial remodeling (14% vs. 25%), (p&gt;0.05).Conclusions. DM is a significant risk factor for a more severe course of LDC, in particular atrial fibrillation and supraventricular ectopias. In this regard, it is of fundamental importance to control carbohydrate metabolism and prevent risk factors for cardiovascular diseases in order to preserve the basic functions of the myocardium and improve the quality of life of patients with diabetes mellitus.

Clinical case of pulmonary alveolar proteinosis in a newborn

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of protein-lipid complexes in the alveoli due to impaired utilization of surfactant by alveolar macrophages; it is most often idiopathic and occurs in healthy men and women aged 30–50 years. The congenital form of PAP is extremely rare, its diagnosis is complicated by the fact that it is impossible to conduct functional lung tests in newborns, and lung biopsy is used extremely rarely. Against this background, data obtained by computed tomography (CT) of the lungs of newborns are of particular importance. The article presents a clinical case of treatment of a newborn with respiratory failure (RF) and signs of pulmonary hypertension, who was admitted to the neonatal intensive care unit of the Children’s City Multidisciplinary Clinical Specialized Center for High Medical Technologies at the age of the first day of life. The article reflects the role of computed tomography in diagnosing the causes of respiratory failure in newborns, as well as the importance of anamnesis and a thorough clinical examination of patients. The presented clinical case demonstrates the need for a multidisciplinary approach to the diagnosis of newborns with pulmonary alveolar proteinosis.

(1123) - Temporary Mechanical Circulatory Support to Heart Transplant in Patients with Significant Pulmonary Hypertension: A Bridge Not Too Far?

(1123) - Temporary Mechanical Circulatory Support to Heart Transplant in Patients with Significant Pulmonary Hypertension: A Bridge Not Too Far?

Pulmonary Artery Aneurysm Associated with Sarcoidosis in a 75-year-old with Heart Failure: The Challenges of Diagnosis and Management.

Pulmonary artery aneurysm (PAA) is a rare abnormality of pulmonary vasculature. It can be idiopathic or secondary to various pathologies, frequently with multiple factors leading to its formation. We report the case of a man with concomitant sarcoidosis and PAA. A 75-year-old male with a diagnosis of pulmonary sarcoidosis was referred to the Cardiology department due to heart failure with reduced left ventricular ejection fraction (LVEF). The transthoracic echocardiogram revealed mildly reduced LVEF, aortic root and pulmonary artery (PA) dilatation, and no signs of pulmonary hypertension (PH). Cardiac magnetic resonance imaging was performed, revealing mild left ventricular dilation, LVEF of 40%, main PA dilation (43 mm) and a pattern of late gadolinium enhancement suggestive of cardiac sarcoidosis. At follow-up, a thoracic computed tomography (CT) angiography scan revealed ascending aorta ectasia and giant main PA aneurysm (60 mm). A right heart catheterisation was performed, and a mean PA pressure of 34 mmHg was obtained. Given the clinical context, the patient was considered to have PH due to lung disease and left heart disease, and PAA was possible due to vascular granulomatous involvement by sarcoidosis. PAA is a rare finding and mostly occurs in the setting of PH. Sarcoidosis is a granulomatous disease that mostly affects the lungs, but the sarcoid involvement of great vessels has been described. In this clinical case, the probable cause for the PA fragility leading to aneurysm formation remains sarcoid vascular infiltration, regarding the discrepancy between the PA dimensions and mildly elevated PA pressure. Pulmonary artery aneurysm is a rare abnormality of pulmonary vasculature that can be idiopathic or a consequence of pulmonary hypertension, congenital heart disease, infection, vasculitis or collagenopathies.Due to the low incidence of this disease, there are no guidelines for its diagnosis, management or follow-up, and treatment is based on the underlying aetiology, aneurysm dimensions and occurrence of symptoms.Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas that mostly involve the lungs, but can also affect skin, eyes, and lymph nodes. Sarcoid involvement of great vessels has been rarely described.

CASE-TA-04 - A Rare Cause of Dyspnea in a Young Adult: Isolated Unilateral Absence of the Right Pulmonary Artery Associated with Signs of Pulmonary Hypertension

CASE-TA-04 - A Rare Cause of Dyspnea in a Young Adult: Isolated Unilateral Absence of the Right Pulmonary Artery Associated with Signs of Pulmonary Hypertension

Evaluation of Intraoperative Left-Ventricular Diastolic Function by Myocardial Strain in On-Pump Coronary Artery Bypass Surgery

Left ventricular (LV) diastolic function strongly predicts outcomes after cardiac surgery, but there is no consensus about appropriate intraoperative assessment. Recently, intraoperative diastolic strain-based measurements assessed by transesophageal echocardiography (TEE) have shown a strong correlation with LV relaxation, compliance, and filling, but there are no reports about evaluation through the entire perioperative period. Therefore, the authors describe the intraoperative course of this novel assessment technique in patients who underwent coronary artery bypass grafting, and compare it with conventional echocardiographic measures and common grading algorithms of LV diastolic dysfunction (LVDD). Prospectively obtained data. A single university hospital. Thirty adult patients scheduled for isolated on-pump coronary artery bypass grafting surgery with preoperative preserved left and right ventricular systolic function, without significant heart valve disease and pulmonary hypertension, and an uneventful intraoperative course were included. Transesophageal echocardiography was performed after induction of anesthesia (T1), after termination of cardiopulmonary bypass (T2), and after sternal closure (T3). Echocardiographic evaluation was performed in stable hemodynamic conditions, in sinus rhythm or atrial pacing, and vasopressor support with norepinephrine ≤0.1 µg/kg/min. Strain-based measurements of peak longitudinal strain rate during isovolumetric relaxation (SR-IVR) and during early (SR-E) and late (SR-A) LV filling were assessed using EchoPAC v204 software (GE Vingmed Ultrasound AS, Norway). Evaluation of conventional echocardiographic parameters included transmitral Doppler measures of early (E) and late (A) LV filling, as well as lateral-tissue Doppler velocity assessed during early (e´) and late (a´) LV filling, tricuspid regurgitation, and left atrial dilatation. Evaluation and grading of LV diastolic function by myocardial strain was feasible in all included patients at all time points of assessment. Using conventional grading algorithms, however, a substantial number of patients could not be sufficiently graded, falling into an indeterminate zone and not reliably estimating LVDD (T1, 40%; T2, 33%; T3, 36%). There was significant impairment of LV diastolic function after bypass, as measured by SR-IVR (T1 v T2, 0.28 s-1 [IQR 0.23; 0.31) v 0.18 s-1 [IQR 0.14; 0.22]; p < 0.001), SR-E (T1 v T2, 0.95 ± 0.34 s-1v 1.28 ± 0.36 s-1; p < 0.001), and E/SR-IVR (T1 v T2, 2.3 ± 1.0 m v 4.5 ± 2.1 m; p < 0.001]. Conventional echocardiographic measures remained unchanged during the same period (E/A T1 v T2, 1.27 [IQR 0.94; 1.59] v 1.21 [IQR 1.03; 1.47] [p = 1] and E/e´ T1 v T2, 7.0 [IQR 5.3; 9.6] v 6.35 [IQR 5.7; 9.9] [p = 0.9]). There were no significant changes in the values of SR-IVR, SR-E, SR-A, E/SR-IVR, E/A, and E/e´ before and after sternal closure (T2 v T3). Intraoperative assessment of strain-based measurements of LV diastolic function and strain-based LVDD grading was feasible in this group of selected patients, whereas conventional parameters failed to describe LVDD sufficiently in a substantial number of patients. Diastolic strain-based measurements showed impairment of LV relaxation and compliance after bypass, which was not detected by conventional echocardiographic parameters. Therefore, diastolic myocardial strain analysis might be more sensitive in detecting myocardial diastolic dysfunction by TEE in the perioperative setting, with its dynamic changes of loading conditions, and might provide valuable and additional information on the perioperative changes of LV diastolic function.

Impact of left ventricular end-diastolic pressure as a marker for diastolic dysfunction on long-term outcomes in patients undergoing transcatheter aortic valve replacement

ObjectiveThe aim of this study was to investigate the proportion of elevated left ventricular end-diastolic pressure (LVEDP) as an indicator of diastolic function after transcatheter aortic valve replacement (TAVR) and its implication in predicting long-term mortality. MethodsWe analyzed retrospectively collected data on 3328 patients with severe aortic stenosis undergoing TAVR in our institution between July 2009 and June 2021. Patients were stratified into two groups based on invasive post-procedural LVEDP measurements: normal (<15 mmHg) vs. elevated (≥15 mmHg) LVEDP. ResultsMean age of the patients was 81.6 years, and 53.3% were female. Elevated post-procedural LVEDP was identified in 2408 (72.3%) patients. The 5-year mortality rates were higher in the group with elevated LVEDP compared with the group with normal LVEDP (27.4% vs. 8.3%, p = 0.01; hazard ratio [HR] 1.22, 95% CI 1.05-1.41). A multivariate model revealed the following independent predictors of mortality after TAVR: post-procedural elevated LVEDP (HR 1.24, 95% CI 1.01-1.53), pre-procedural significant tricuspid regurgitation (HR 1.24, 95% CI 1.02-1.52) and pulmonary hypertension (PH) (HR 1.53, 95% CI 1.26-1.86). In the present study, a significant paravalvular leak after TAVR was not associated with higher mortality (HR 1.45, 95% CI-0.95-2.19, p = 0.75). ConclusionElevated post-procedural LVEDP in patients who undergo TAVR is an independent predictor of all-cause mortality. Furthermore, PH and tricuspid regurgitation were also identified as predictors of mortality. These data confirm that diastolic dysfunction is an important predictor of mortality in TAVR and should be considered to guide procedure timing, favoring an early interventional approach and management in aortic stenosis patients.

Cardiac abnormalities in stable chronic obstructive pulmonary disease: correlations and predictors

Abstract Background Patients with stable chronic obstructive pulmonary disease (COPD) are more likely to have cardiac abnormalities, which increase morbidity and mortality. The aim of the study is to explore correlations and probable predictors for these abnormalities in stable COPD patients. Patients and methods We conducted a prospective cohort study including 91 stable COPD cases, who were admitted to a tertiary health-care center during the period from August 2021 to January 2022. Spirometry, arterial blood gas, and ECG were performed for all participants. They were categorized using GOLD guidelines (2021) and assessed by two-dimensional Doppler echocardiography using the American and European Association of Echocardiography ASE recommendations. Results The study included 91 COPD patients; their mean age was 60.5 years with male predominance (62.6%). Of the patients, 76.9% were smokers and 49.5% of patients have respiratory failure type II. The most common abnormal ECG findings were P pulmonale, ventricular ectopic, and ischemic changes, which were more detected in severe COPD patients (94.29%). Echocardiographic assessment showed abnormal findings in almost 65%, more in the severe group. The most frequent findings were signs of pulmonary hypertension (PH) (72.5%), right ventricular dilatation (49.5%), and hypokinesia (21.98%). Risk factors of cardiac abnormalities were respiratory failure, COPD duration, comorbidities, and COPD stage. Conclusion Right ventricle dilatation, hypokinesia, and PH showed a positive correlation with echocardiographic findings and COPD severity. Respiratory failure and COPD duration were independent predictors of arrhythmia; COPD stage and the number of comorbidities were predictors of cardiac ischemia, while respiratory failure and comorbidities were predictors of PH.

Perioperative extracorporeal membrane oxygenation support for pulmonary endarterectomy: A 17-year experience from the UK national cohort

Pulmonary endarterectomy (PEA) is the guideline-recommended treatment for patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, some patients develop severe cardiopulmonary compromise before surgery, intraoperatively, or early postoperatively. This may result from advanced CTEPH, reperfusion pulmonary edema, massive endobronchial bleeding, or right ventricular (RV) failure secondary to residual pulmonary hypertension. Conventional cardiorespiratory support is ineffective when these complications are severe. Since 2005, we used extracorporeal membrane oxygenation (ECMO) as a rescue therapy for this group. We review our experience with ECMO support in these patients. This study was a retrospective analysis of patients who received perioperative ECMO for PEA from a single national center from August 2005 to July 2022. Data were prospectively collected. One hundred and ten patients (4.7%) had extreme cardiorespiratory compromise requiring perioperative ECMO. Nine were established on ECMO before PEA. Of those who received ECMO postoperatively, 39 were for refractory reperfusion lung injury, 20 for RV failure, 31 for endobronchial bleeding, and the remaining 11 were for "other" reasons, such as cardiopulmonary resuscitation following late tamponade and aspiration pneumonitis. Sixty-two (56.4%) were successfully weaned from ECMO. Fifty-seven patients left the hospital alive, giving a salvage rate of 51.8%. Distal disease (Jamieson Type III) and significant residual pulmonary hypertension were also predictors of mortality on ECMO support. Overall, 5- and 10-year survival in patients who were discharged alive following ECMO support was 73.9% (SE: 6.1%) and 58.2% (SE: 9.5%), respectively. Perioperative ECMO support has an appropriate role as rescue therapy for this group. Over 50% survived to hospital discharge. These patients had satisfactory longer-term survival.

Prognostic Implication of Pulmonary Arterial Pressure in Surgical Repair of Predominantly Congenital Mitral Valve Regurgitation-Based Intracardiac Abnormalities

Introduction: Knowledge is limited regarding the significance of pulmonary arterial pressure (PAP) in predominantly congenital mitral valve regurgitation (MR)-based intracardiac abnormalities. Methods: From a prospective cohort, we included 200 patients with congenital MR regardless of other associated intracardiac abnormalities (mean age 60.4 months, 67% female, systolic PAP (sPAP) 54.2 mm Hg) surgically repaired in 2012–2019 and followed up to 2020 (median 30.0 months). Significant pulmonary hypertension (PH) was defined as sPAP >50 mm Hg at rest or mean PAP >25 mm Hg on right heart catheterization. By perioperative sPAP changes, patients were stratified as group I (pre-normotension to post-normotension), group II (pre-hypertension to post-normotension), or group III (pre-hypertension to post-hypertension). Primary outcomes were the recurrence of MR (defined as the regurgitation grade of moderate or greater) and the progression of MR (defined as any increase in the magnitude of regurgitation grade after surgery). Cox proportional hazard and Kaplan-Meier curve were performed. Results: There was no association between preoperative PH and the recurrent MR (adjusted hazard ratios [aHR]: 1.146 [95% CI: 0.453–2.899]) and progressive MR (aHR: 1.753 [95% CI: 0.807–3.804]), respectively. There were no significant differences among group I, group II, and group III in the recurrent MR but in the progressive MR. A dose dependency was identified for preoperative sPAP with recurrent MR (aHR: 1.050 [95% CI: 1.029–1.071]) and progressive MR risks (aHR: 1.037 [95% CI: 1.019–1.055]), respectively. Conclusions: Preoperative higher sPAP is associated with worse outcomes, warranting heightened attention to the identification of perioperative sPAP.

Timing of the narrowest neoLVOT with trans-catheter mitral valve implantation, a cardiac computed tomography study

Abstract Funding Acknowledgements Type of funding sources: None. Background Trans-catheter mitral valve replacement (TMVR) procedures had emerged as an alternative solution for patients who have surgical high risk for mitral valve surgery. One of the main challenges faced during the percutaneous approach is the degree of left ventricular outflow tract (LVOT) narrowing after implanting the new percutaneous mitral prosthesis. The remaining LVOT area (NeoLVOT) can be estimated by a commercially available CT analysis software that allows the insertion of a virtual valve within the CT dataset in the native annulus and/or the degenerated bioproshesis and hence allows measurement of the NeoLVOT using the multi-planar reconstruction (MPR) mode. The systolic phase that shows the narrowest NeoLVOT is not fully understood, and hence there is a considerable variability between operators in choosing the best phase in systole at which they should insert the virtual valve within the mitral annulus in the CT dataset to measure the narrowest NeoLVOT. Purpose To detect the systolic phase that shows the narrowest NeoLVOT and hence standardize the way to use the software to decrease variability among operators. Methods A retrospective observational single center study, including 20 patients (age 62± 4 years, 70% men), who had undergone a clinically indicated cardiac computed tomography (CT). All these patients were in sinus rhythm, had no significant coronary artery disease bases on CT report, and had normal left ventricular systolic function with no regional wall motion abnormalities, we excluded patients who had previous cardiac surgeries, hypertrophic cardiomyopathy, significant pulmonary hypertension or congenital heart disease that may affect septal motion. CTs were performed using a 128 slice dual-source (Somatom Definition Flash, Siemens Healthineers, Erlangen, Germany) with a retrospective ECG-gated acquisition CT analysis was done by a single operator using the commercially available software (3mensio Structural Heart 10.3, Pie Medical Imaging, The Netherlands). Multiphase scans were used and mid-diastole (80%), early-systole (10%), mid-systole (20%), late-systole (30–40%) were identified and analysed. Measurements done were; mitral annulus (MA) antero-posterior (AP) diameter, inter-commissural (IC) diameter, annulus area (Area), annulus perimeter (Peri), aorto-mitral angel, and the NeoLVOT area. (Figure 1). Results All measurements were feasible in all phases for all patients. The neoLVOT area was significantly narrower at end-systole (224 ± 62mm²), compared to early-systole (299 ± 70mm²) &amp; mid-systole (261 ± 75 mm²), (p = 0.005). All other measurement did not show statistically significant changes despite taking certain trends. (Figure 2). Conclusions End-systole is the cardiac phase that shows the narrowest predicted Neo-LVOT area and hence it should be the standard phase to be used during CT analysis before TMVR to predict the narrowest NeoLVOT.

0980 A Child with Achondroplasia, Cervicomedullary Junction Compression, Severe Obstructive Sleep Apnea and Pulmonary Hypertension

Abstract Introduction Achondroplasia is a short stature autosomal dominant skeletal dysplasia. Children with Achondroplasia are at risk for multiple medical comorbidities including sleep disordered breathing due to midface hypoplasia and craniocervical junction compression. Recent guidelines from American Academy of Pediatrics on health supervision of children with achondroplasia recommend routine clinical screening for symptoms of obstructive sleep apnea (OSA) and craniocervical junction compression in which case a polysomnogram should be considered. Report of case(s) The patient is a now 15 month old male who was initially seen in sleep medicine clinic at age 4 months for snoring and respiratory distress while awake. Polysomnogram 2 days later demonstrated severe OSA. The study demonstrated an oAHI of 25.1/hour, O2 nadir of 77%, and TcCO2 with evidence of significant hypoventilation with TcCO2 max of 67mmHg. This was adequately treated with supplemental oxygen. The patient subsequently presented to the emergency department at age 7 months with hypoxemic respiratory failure and signs of right heart failure and was started on CPAP. Echocardiogram demonstrated significant pulmonary hypertension. Subsequent CPAP titration study during admission demonstrated adequate treatment of OSA and his pulmonary hypertension significantly improved with the patient requiring no pulmonary hypertension treatment at discharge. At age 9 months, the patient was again admitted with hypoxemic respiratory failure and subsequent brain MRI demonstrated foramen magnum stenosis requiring subsequent surgical decompression. He was discharged on CPAP, however, was not tolerating this at home. Therefore, repeat polysomnogram was obtained that demonstrated severe OSA that was not responsive to supplemental oxygen. The patient was then started on BPAP therapy which he has since been tolerating well. Conclusion Children with achondroplasia are at increased risk for sleep disordered breathing and craniocervical junction compression. Our patient experienced significant complications from OSA at a young age. His sleep apnea worsened significantly in a short period making it unresponsive to previous treatment recommendations. There is a need for further studies to determine better screening guidelines for craniocervical junction narrowing and OSA. This case highlights the dynamic nature of sleep disordered breathing in achondroplasia and need for close follow up and monitoring of symptoms at all clinical encounters. Support (if any)

Characteristics of patients with chronic heart failure in different age groups

There are indications that the severity of clinical manifestations of chronic heart failure (CHF) is related to the age of patients, with older age groups characterized by a more severe course of CHF and more significant pathological changes in instrumental and laboratory parameters. In view of the above, the aim of the present study was to compare NT-proBNP level, other basic parameters of left ventricular myocardial contractility and the results of 6-min walk test, in CHF patients of different age. We investigated these parameters by dividing the total number of subjects into subgroups of young (18-44 years), middle-aged (45-59 years) and elderly (60-74 years) age according to WHO guidelines. There were 111 patients with CHF (82 men and 29 women) aged 20 to 74 years (mean age 60.4±1.25 years). The patients were divided into three subgroups: 1st - young age (21 patients), 2nd - middle age (37 patients) and 3rd - old age (53 patients). Echocardiographic examination, 6-minute walking test, determination of NT-proBNP level in blood plasma were used during the examination. The number of patients with decreased EF (&lt;35%) was significantly lower among young patients, while those with preserved EF was significantly higher among elderly patients. There were more patients with signs of pulmonary hypertension (high systolic pulmonary artery pressure (SPAP)) among elderly patients. The average distance walked during a 6-minute walk was relatively shorter in elderly patients, as well as a greater number of patients with higher (III-IV functional classes (FC)). The number of patients with elevated blood NT-proBNP levels as well as mean NT-proBNP levels were also relatively higher in the older age groups. Thus, the clinical course of CHF is characterized by a more severe course in elderly patients, despite the prevalence of patients with preserved EF, which is confirmed by more frequent occurrence of FC III-IV, clinical-instrumental and laboratory parameters, and is associated, at least partly, with more frequent occurrence of comorbid pathology.

Normal range of intraoperative three-dimensionally derived right ventricular free-wall strain in coronary artery bypass surgery patients.

Data on intraoperative three-dimensionally derived right ventricular free-wall strain (3D-RV FWS) is sparse. We sought to evaluate the normal range of intraoperative 3D-RV FWS in patients scheduled for coronary artery bypass graft (CABG) surgery and compared to conventional echocardiographic parameters. Prospective observational study. A total of 150 patients with preserved left and right ventricular (RV) function and sinus rhythm, without significant heart valve disease or pulmonary hypertension undergoing isolated on-pump CABG surgery, with an uneventful, complication-free intraoperative course. 3D-RV FWS analysis and conventional echocardiographic assessment of RV function were performed intraoperatively in anesthetized and ventilated patients using transesophageal echocardiography (TEE). TomTec 4D RV-Function 2.0 software for assessment of 3D-RV FWS and three-dimensional right ventricular ejection fraction (3D-RV EF). Philips QLAB 10.8 was used to evaluate tissue velocity of the tricuspid annulus (RV S´), tricuspid annular systolic excursion (TAPSE), and RV fractional area change (FAC). All echocardiographic measurements were performed under stable hemodynamic conditions and predefined fluid management without any vasoactive support or pacing. The prospective observational study was performed in a single university hospital setting. Assessment of 3D-RV FWS was feasible in 95% of patients. No included patient experienced any serious perioperative complication. In our group of patients, median values with interquartile range (IQR) for 3D-RV FWS and 3D-RV EF were -25.2 (IQR -29.9 to -21.8) and 46.3% (IQR 41.0%-50.1%), respectively. RV FAC, RV S´, and TAPSE accounted for 39.7% (IQR 34.5%-44.4%), 14.8cm/s (IQR 11.8-19.0cm/s), and 22mm (IQR 20-25mm). The normal range (2.5% to 97.5% percentile) for 3D-RV FWS was -37.1 to -12.8. There was no relevant correlation of 3D-RV FWS to postoperative outcome in this group of CABG patients. We present distribution values for intraoperative 3D-RV FWS and conventional parameters of RV function assessment in a healthy on-pump CABG patient population without serious perioperative complications. We observed no correlations of these parameters with any of the outcome parameters considered. Therefore, we consider these values to be intraoperative TEE-assessed normal values, which can be expected in on-pump CABG patients.

P226 A STRANGE CASE OF ISOLATED RIGHT VENTRICULAR DYSFUNCTION

Abstract Introduction In Europe, approximately 29% of the population aged between 15 and 64 have used illicit drugs. Italy is at the top of this ranking. Description 45–year–old female, no previous cardiology events. She enters the Emergency Department for typical chest pain following alcohol and cocaine abuse. EKG (see figure 1) detects sinus tachycardia and mild non–specific changes in ventricular repolarization. Blood tests reveal an increase in Troponin T levels (305 ng/L, NV &amp;lt; 14 ng/L, then decreased with subsequent measurements) and D–dimer (902 mg/mL, NV &amp;lt; 500 mg/mL). Echocardiography reveals a non–dilated right ventricle with severe systolic dysfunction (TAPSE 11 mm). No signs of pulmonary hypertension but a dilated and hypocollapsing inferior vena cava is noted. Abnormal motion of the interventricular septum with systolic d–shape is present (see figure 2). Left ventricle appears normal. Pulmonary angio–Tc was negative for embolic phenomena and coronary angiography detects no lesions on coronary arteries. At discharge, right ventricular systolic dysfunction and septal d–shape were completely regressed (see Figure 3). Discussion Cocaine is a strong systemic adrenergic agonist, which can cause vasospasm of small arterioles of the cardiac microcirculation with possible formation of intracoronary thrombi. Peculiarity of this case is the selective and reversible involvement of the right ventricle, an extremely rare presentation as evidenced by the almost absence of scientific literature on the subject (there is only a similar case report published on the American Journal of Emergency Medicine in 2015). Reversible damage from vasospasm and/or intracoronary thrombosis localized to the marginal branch of the right coronary artery, induced by cocaine use, is conceivable. D–shape of the interventricular septum associated with right ventricular dilatation is likely caused by acute right ventricular failure causing volume overload in the right sections. Take home message Drugs abuse is a widespread problem among the population. Cocaine intake is associated with important adverse cardiovascular effects. A rare presentation can be isolated right ventricular ischemia associated with acute right heart failure.

Diseases associated with prematurity in correlation with N-terminal pro-brain natriuretic peptide levels during the early postnatal life

The aim of this observational study was to investigate the influence of different typical preterm diseases on NT-proBNP serum levels in the early postnatal period of life of a preterm infant. NT-proBNP levels of 118 preterm infants born ≤ 31 weeks GA were determined at the first week of life, after 4 ± 1 weeks of life, and at a corrected gestational age of 36 + 2 weeks. Relevant complications with a possible influence on NT-proBNP values in the first week of life such as early neonatal infection, hemodynamically significant PDA (hsPDA), early pulmonary hypertension (early PH), and intraventricular hemorrhage (IVH) were evaluated; at 4 ± 1 weeks of life, bronchopulmonary dysplasia (BPD), BPD-related pulmonary hypertension (BPD-associated PH), late infection, IVH, and intestinal complications were evaluated. At a corrected gestational age of 36 ± 2 weeks, we examined the effect of retinopathy of prematurity (ROP), BPD, BPD-associated PH, and late infection on NT-proBNP levels. In the first days of life, only the isolated occurrence of hsPDA resulted in significantly increased NT-proBNP levels. In multiple linear regression analysis, early infection remained independently associated with NT-proBNP levels. At 4 ± 1 weeks of age, the isolated presence of BPD and BPD-related PH resulted in increased levels, and the effect remained significant in the multiple regression analysis. At a corrected gestational age of 36 ± 2 weeks, infants with relevant complications at this final evaluation time tended to have lower NT-proBNP values than our exploratory reference values. Conlusion: NT-proBNP in the first week of life seems to be mainly influenced by an hsPDA and infection or inflammation. BPD and BPD-related PH are the most important factors influencing NT-proBNP serum levels in the first month of life. When preterm infants reach a corrected GA of 36 ± 2 weeks, chronological age rather than complications of prematurity must be considered when interpreting NT-proBNP levels.What is Known:• Several complications associated with prematurity, such as hemodynamically significant PDA, pulmonary hypertension, bronchopulmonary dysplasia, and retinopathy of prematurity, have been shown to influence NT-proBNP levels in preterm infants in their early postnatal life.What is New:• Hemodynamically relevant PDA is a major factor in the increase of NT-proBNP levels in the first week of life.• Bronchopulmonary dysplasia and pulmonary hypertension associated with bronchopulmonary dysplasia are important factors in the increase in NT-proBNP levels in preterm infants at approximately 1 month of age.