High Probability Of Pulmonary Hypertension Research Articles

Inhaled Nitric Oxide in Fibrotic Lung Disease: A Randomized, Double-Blind, Placebo-Controlled Trial.

Inhaled nitric oxide (iNO) has been shown to result in benefits in moderate to vigorous physical activity (MVPA) in patients with fibrotic interstitial lung disease (if-ILD) on supplemental oxygen in two independent trials. This phase 3 randomized double-blind placebo-controlled study sought to validate the benefit of ambulatory iNO in patients with f-ILD requiring supplemental oxygen. Patients with f-ILD on supplemental long-term oxygen were randomized in a 1:1 fashion to inhaled nitric oxide at 45 µg/kg ideal body weight/hour or placebo for 16 weeks. The primary outcome was the change from baseline to week 16 in MVPA assessed by accelerometry. Secondary outcomes included overall activity, six-minute walk distance and patient reported outcomes. 145 patients were enrolled; 75 were assigned to receive iNO and 70 to placebo. The change from baseline in MVPA at 16 weeks was -9.2 minute/day (SE 3.51) in the iNO45 group versus -3.7 (3.76) minute/day in the placebo group (difference, 5.5; P=0.265). No statistically significant differences between the two treatment arms were found for any of the secondary outcomes. A subgroup analysis of patients with intermediate or high probability of pulmonary hypertension on echocardiography did not demonstrate any benefit. The most common adverse events reported were respiratory tract infections, but the therapy was generally very well tolerated. There was no demonstrable benefit to iNO in patients with f-ILD on supplemental oxygen in daily physical activity assessed by actigraphy, a potential novel clinical trial endpoint. Clinical trial registration available at www. gov, ID: NCT03267108.

Myxomatous mitral valve disease in Labrador Retrievers and Golden Retrievers.

To characterize myxomatous mitral valve disease (MMVD) in Labrador Retrievers (LRs) and Golden Retrievers (GRs). 52 LRs and 20 GRs diagnosed with MMVD composed a retrospective study sample (February 1, 2010, to July 31, 2021). Stored echocardiograms were remeasured. Dogs were staged by 2019 MMVD consensus guidelines. Mean age was 9.9 years in LRs and 9.5 years in GRs, with 31 of 52 LRs (59.6%) and 13 of 20 (65.0%) GRs being male. Forty-six LRs were stage B1 (88.5%), 3 were B2 (5.8%), and 3 were C (5.8%). Fourteen GRs were stage B1 (70.0%), 2 were B2 (10.0%), and 4 were C (20.0%). Of LRs and GRs in stage B2/C, 50% had systolic dysfunction. Atrial fibrillation (AF) and ventricular arrhythmias were identified in 2 of 52 (3.8%) and 10 of 52 (19.2%) LRs at initial diagnosis versus 3 of 20 (15.0%) and 3 of 20 (15.0%) GRs, respectively. All 5 AF dogs were stage C, with intermediate to high probability of pulmonary hypertension. Two additional GRs developed AF during follow-up; thus 5 of 6 (83.3%) stage B2/C GRs ultimately experienced AF. Subjective mitral valve thickening was frequent in both breeds (41/52 LRs [78.8%]; 18/20 GRs [90.0%]), while mitral valve prolapse was more common in LRs (22/52 [42.3%]) than GRs (5/20 [25.0%]). In LRs and GRs, MMVD was relatively late onset, with males overrepresented. Both breeds exhibited mitral valve thickening in association with MMVD, while LRs more commonly exhibited mitral valve prolapse. While most LRs and GRs with MMVD were stage B1, those in stage B2/C had increased prevalence of systolic dysfunction and AF.

THE SIGN OF THE HORNS: A RARE CASE OF DOUBLE PERI–COMMISSURAL MITRAL FLAIL

Abstract A 80–year–old man with history of hypertension and dyslipidemia was admitted to our Cardiology Unit for exertional dyspnea (NYHA III) occurred during the last few days. The physical examination showed a systolic heart murmur (4/6 on Levine scale) and signs of pulmonary congestion. Transthoracic echocardiography revealed a massive mitral regurgitation (Fig.1) together with severe dilation of the left atrium, mild–to–moderate aortic regurgitation, high probability of pulmonary hypertension and normal biventricular systolic function. A transesophageal echocardiography (TEE) was performed to clarify mitral valve anatomy. A double flail of the posterior leaflet was present involving the peri–commissural portions due to a single chordal rupture on P1 scallop and a double chordal rupture on P3 scallop (Fig.2). P2 scallop was prolapsing and there was a billowing of the anterior leaflet. In addiction mitral annulus was dilated (antero–posterior diameter of 43 mm and intercommissural diameter of 45 mm). We named this finding “sign of the horns” for his 3D echocardiographic appearance (Fig.3) given the double flail resembling two fingers. A surgical correction was performed with 3 GoreTex neochords implantation on the posterior leaflet and an annuloplasty ring (Physio II n. 34) implantation during extracorporeal circulation through right anterior minithoracotomy. Surgical and echocardiographic results were optimal. The patient was discharged after 7 days of hospitalization. Conclusion Coexistence of a double peri–commissural flail in the same leaflet is a very rare echocardiographic finding and we named it “double horn sign”. TEE is an essential examination in this scenario in order to understand the exact mechanism of the regurgitation for an optimal preoperative planning.

Isolated rheumatic tricuspid valve regurgitation: it is only rare not just a myth: rare case report

BackgroundIsolated rheumatic tricuspid regurgitation (IRTR) is a rare condition that can manifest as right heart failure (RHF) and pulmonary hypertension (PH) symptoms. Diagnosing and treating IRTR in cases of latent RHD can be a challenge and crucial for future research to establish new guidelines for echocardiography in RHD that focus not only on the mitral and aorta but also the tricuspid valve.Case presentationA young female patient with clinical symptoms of RHF suspected IRTR due to latent RHD from echocardiography. Echocardiography revealed significant thickening and calcification of all tricuspid valve (TV) leaflets, with partial prolapse posterior leaflet and severe tricuspid regurgitation (TR) with a high probability of PH, no significant anatomical and functional abnormality pulmonary valve (PV), mitral valve (MV), and aortic valve (AV). She was administered daily doses of Ramipril, bisoprolol, spironolactone, and furosemide. Although she received therapy, she persisted in suffering dyspnea when doing mild physical activity (NYHA functional class III). She was admitted to the surgical conference, due to our center’s limitation of percutaneous intervention for valve replacement, and she was approved to undergo tricuspid valve replacement (TVR) surgery.ConclusionsEchocardiography plays a crucial role in identifying latent RHD. Isolated rheumatic TR shows echocardiographic results similar to rheumatic mitral regurgitation, except for the presence of a high-velocity jet. Diuretics temporarily slow symptoms, but disease progression remains uncertain. TV surgery is effective for severe symptoms, but isolated TVR is rare and has a poor prognosis.

“SEEK, AND YOU WILL FIND” - IMPLICATIONS OF PULMONARY HYPERTENSION DIAGNOSIS AND TREATMENT OF PATIENT WITH CHRONIC OBSTRUCTIVE PULMONARY DISEASE AND LEFT HEART FAILURE

Objective: The case report presents a patient with multimorbidity. A diagnosis of pulmonary hypertension was made during multiple hospitalizations due to bad compliance. The objective of this case report is to describe the difficulties involved in diagnosing pulmonary hypertension in a patient with chronic obstructive pulmonary disease and left heart failure. Design and method: Case report of patient with pulmonary hypertension, chronic obstructive pulmonary disease, left heart failure and multiple comorbidities. Observation of the patient's health condition during many hospitalizations led to further diagnosis of pulmonary hypertension and forced the modification of current treatment. Results: Patient went through many hospitalizations because of bad compliance to chronic obstructive pulmonary disease and left heart failure pharmacotherapy. The patient was hospitalized in our ward for chest pain. He also presented combination of nonspecific symptoms such as dyspnoea (including resting dyspnoea), exercise intolerance and peripheral oedema. Chest x-ray showed signs of pneumonia. Therefore, the diagnosis of pulmonary hypertension was not made during the first hospitalisations. Echocardiography was planned to exclude chronic coronary syndrome. It revealed patent ductus arteriosus, right ventriculum and pulmonary trunk dilation, tricuspid regurgitation and high probability of pulmonary hypertension (mean pulmonary artery pressure 46,5mmHg), without regional cardiac wall dysfunction. The next hospitalization was scheduled, during which cardiac catheterization was performed (pulmonary capillary wedge pressure 14,5 mmHg) and precapillary pulmonary hypertension was confirmed. After many consultations, excluding other causes, such as thromboembolic changes, chronic obstructive pulmonary disease was considered the most likely cause of precapillary pulmonary hypertension. Treatment of basal diseases has been modified, off-label treatment with sildenafil was started, and then due to ineffective treatment (deterioration of the patient's condition resulted in a transition from WHO functional class II to III) the patient was qualified for the bosentan clinical drug treatment program. Conclusions: Diagnosis of pulmonary hypertension can be difficult when symptoms of diseases from other systems are overlapping. This case shows the necessity of watchful observation of patients with multimorbidity, but also difficulties in maintaining good medical compliance. Pulmonary hypertension is still a rare diagnosis, but we should stay alerted about it.

Performance of handheld ultrasound devices in diagnosis of pulmonary hypertension and right heart dysfunction in ICU patients.

The use of handheld ultrasonography devices (HHUD) has increased recently but there are limited data about their performance in the evaluation of right heart dysfunction (RHD) and pulmonary hypertension (PHT). The aim of the study is to compare the performance of a HHUD with a conventional ultrasound device (CUD) in assessing PHT and RHD. This single-center prospective study was performed in a seven-bed teaching hospital intensive care unit (ICU). PHT and RHD criteria were compared by HHUD and CUD. Additional PHT criteria and right ventricle (RV) systolic and diastolic dysfunction criteria were also measured. Forty-six patients were included in the study. There was no significant difference between the imaging rates and mean values of the parameters measured by both devices. When the positivity rates for additional PHT parameters and RHD criteria were compared, there were no significant differences between the devices. In Bland-Altman's analysis, there was good agreement and there was no bias between the measurements of the two devices but left ventricular end-systolic eccentricity index (LVSEI), right atrium area (RAA), and pulmonary artery diameter (PAD). Ninety percent of the patients had PHT probability, of whom 43% had a low, 37% had intermediate and 10% had a high probability of PHT. Ninety-two percent of the patients had RHD and there was no significant difference between the devices in the diagnosis of RHD (p=.212). When RV systolic and diastolic dysfunction evaluations of the devices were compared according to the British Society of Echocardiography (BSE) criteria there was no significant difference between the devices' measurements in the evaluation of systolic and diastolic function. The imaging and measurement capabilities of the HHUDs for PHT and RHD parameters were similar to CUDs, and considering the inconsistent parameters, the HHUD can be useful in diagnosing these problems.

ECG patterns to predict pulmonary arterial hypertension in patients with severe tricuspid regurgitation

IntroductionEchocardiographic evaluation of tricuspid regurgitation (TR) velocity is a key measure in screening for pulmonary hypertension. Based on its value and additional features of right ventricle overload patients are classified into low, intermediate or high probability of pulmonary hypertension which transfers into decisions of further invasive evaluation. However, in the presence of severe TR echocardiography underestimates pulmonary artery pressure and therefore pulmonary hypertension may be overlooked in some patients. Accordingly, in the present study we aimed to assess the role of electrocardiography in predicting the presence of pulmonary arterial hypertension (PAH) in patients with severe TR. ResultsWe analysed 83 consecutive patients with severe TR who were diagnosed in our centre between February 2008 and 2021 and who underwent right heart catheterization. Of them 58 had PAH while 25 had isolated TR (iTR).We found that the following ECG criteria supported the diagnosis of PAH as opposed to the diagnosis of iTR: R:SV1 > 1.0, max RV1 or 2 + max S I or aVL –SV1 > 6 mm, SI/RI > 1 in I. For these parameters using ROC analysis we found that the optimal thresholds suggesting the presence of pulmonary hypertension were: R:SV1 > 1.5 (AUC = 0.74, p = 0.0004, sensitivity 57.1%,specificity of 85%), max RV1 or 2 + max S I or aVL – SV1 > 3 mm (AUC = 0.76, p < 0.0001, sensitivity 91.4%, specificity of 60%) and for SI:RI > 0.71 (AUC = 0.79, p < 0.0001, sensitivity 82.5%,specificity of 70.8%). Presence of atrial fibrillation predicted iTR with 76% sensitivity and 81% specificity. ConclusionsECG analysis can improve the diagnostic process for patients with severe TR. The presence of atrial fibrillation facilitates the diagnosis of isolated tricuspid regurgitation (iTR), while increased values of R:SV1, R:SI, and increased max RV1 or 2 + max SI or aVL – SV1 favor the diagnosis of TR secondary to PAH.

Correlation analysis between lung ultrasound scores and pulmonary arterial systolic pressure in patients with acute heart failure admitted to the emergency intensive care unit

BackgroundNo convenient, inexpensive, and non-invasive screening tools exist to identify pulmonary hypertension (PH) - left heart disease (LHD) patients during the early stages of the disease course. This study investigated whether different methods of lung ultrasound (LUS) could be used for the initial investigation of PH-LHD. MethodsThis was a single-center prospective observational study which was performed in the Zigong Fourth People's Hospital. We consecutively enrolled patients with heart failure (HF) admitted to the emergency intensive care unit from January 2018 to May 2020. Transthoracic echocardiography and LUS were performed within 24 h before discharge. We used the Spearman coefficient for correlation analysis between ultrasound scores and pulmonary arterial systolic pressure (PASP). Bland-Altman plots were generated to inspect possible bias, and receiver operating characteristic (ROC) curves were calculated to assess the relationship between ultrasound scores and an intermediate and high echocardiographic probability of PH-LHD. ResultsSeventy-one patients were enrolled in this study, with an overall median age of 79 (interquartile range: 71.5–84.0) years. Among the 71 patients, 36 (50.7%) cases were male, and 26 (36.6%) had an intermediate and high echocardiographic probability of PH. All four LUS scores in patients with an intermediate and high probability of PH were significantly higher than in patients with a low probability of PH (P <0.05). The correlation coefficient (r) between different LUS scoring methods and PASP was moderate for the 6-zone (r=0.455, P <0.001), 8-zone (r=0.385, P=0.001), 12-zone (r=0.587, P <0.001), and 28-zone (r=0.535, P <0.001) methods. In Bland-Altman plots, each of the four LUS scoring methods had a good agreement with PASP (P <0.001). The 8-zone and 12-zone methods showed moderately accurate discriminative values in differentiating patients with an intermediate and high echocardiographic probability of PH (P <0.05). ConclusionsLUS is a readily available, inexpensive, and risk-free method that moderately correlates with PASP. LUS is a potential screening tool used for the initial investigation of PH-LHD, especially in emergencies or critical care settings.

Pulmonary Hypertension in Pregnancy: A Case Report

Background: Pulmonary Hypertension (PH) is a disease characterized by distressing symptoms and decreased life expectancy due to the narrowing of the blood vessels of the lungs, which often leads to right heart failure. The prevalence of PH in women is 97 cases per million, with 64% of the main causes of PH in pregnancy congenital heart defects, resulting in a very high maternal and fetal mortality rate. Case Report: A 38-year-old G4P1A2 31-week gestational age complained of shortness of breath for 5 days. Physical examination revealed blood pressure was 107/62 mmHg, Heart Rate was 98 beats per minute, respiration 40x per minute, and SpO2 88% with NRM 10 Lpm. The heart examination obtained heart sound I -II regular and a systolic murmur was heard between the left 2nd ribs. Abdominal examination was single fetus, intrauterine, breech presentation, His (+), fetal heart rate 160 beats per minute. vaginal toucher 2 cm in labour. ultrasound examination singles fetal, transverse lies, with an estimated fetal weight of 1600 grams. Echocardiography finding: ASD II L to R shunt with LV EF 60%(T), 62% (S), dilated RA-RV, TR severe, MR mild, High Probability of Pulmonary Hypertension. The patient was diagnosed with Dyspnea, Pulmonary edema caused by cardiogenic, ASD II, High probability of PH, NYHA IV, and Breech presentation in labor. Decided to perform a caesarian section and sterilization. The Male baby was born with 1570 grams Apgar Score 3-5-7. post operation patient was admitted to ICU. Twelve hours after the operation the patient had decreased control and became a PH crisis then the patient was declared dead. Results: The death of the patient, in this case, was caused by cardiogenic shock due to Pulmonary Hypertension Crisis. Conclusion: Early diagnosis along with collaborative and comprehensive management of pulmonary hypertension is needed for good maternal and fetal outcomes. Keywords: pulmonary hypertension, pregnancy, heart disease. Correspondence: Trisulo Wasyanto. Department of Cardiology and Vascular Medicine, Faculty of Medicine, Sebelas Maret University / Dr. Moewardi Hospital. Jl. Kol. Sutarto 132, Surakarta 57126, Central Java, Indonesia. Email: trisulo.wasyanto@staff.uns.ac.id. Mobile: +62811294225.

Is relevant to add stress echocardiography with semi-supine bicycle at the screening of pulmonary hypertension in systemic sclerosis patients?

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Pulmonary hypertension (PH) is the leading cause of death in systemic sclerosis, with a 50% mortality rate within three years of PH diagnosis. The 3-year survival of patients with SSc and PH is estimated to be 56% compared with 94% for those without PH.(1,2). Patients with SSc are at risk of developing group 1 to 3 PH. Detection of PH with echocardiography at rest remains the essential clinical tool; several studies suggested that in patients with SSc, an even earlier change would be the abnormal increase in pulmonary pressures with exercise. (3–6) Purpose Exercise-induced pulmonary hypertension may represent an early but clinically relevant phase in the spectrum of pulmonary vascular disease. On the other hand, increases in mean pulmonary artery pressure on exercise echocardiography can be followed over time, indicating the progression of the pulmonary vascular disease before PH. Methods Observational, prospective, cross-sectional, and analytical study. A basal transthoracic echocardiogram (TTE) and physical stress echocardiogram test were performed, and a TTE follow-up at four years. Results 40 patients. A follow-up was 48 months ED 6 months. Women (93%). Mean age of 49.8 ED 11.4 years. 9.8% of the patients had systemic arterial hypertension; no patient had diabetes mellitus or other chronic-degenerative diseases. 28 (68.3%) patients had the limited cutaneous variety, and 13 (31.7%) patients had the diffuse type. In the basal TTE at the first visit, all had a low probability of presenting pulmonary hypertension; in the follow-up echocardiogram, 11 (26.8%) patients showed a high likelihood of pulmonary hypertension. TTE with a cycle ergometer at first visit: 19 (46.3%) patients presented PSAP &amp;gt;50 mmHg, 16 (39%) patients gave DeltaPSAP &amp;gt;24 mmHg, 19 (46.3%) patients showed a relationship mPAP &amp;gt;30 mmHg + PVR &amp;gt; 3 mmHg/L/min The correlation between stress test variables and the high probability of pulmonary hypertension at follow-up was PSAP&amp;gt;50 mmHg exercise (r 0.58, p&amp;lt;0.001). Delta PSAP &amp;gt;24 mmHg exercise(r 0.40, p 0.011). mPAP&amp;gt;30 mmHg + PVR&amp;gt;3 mmHg/L/min exercises (r 0.45, p 0.003). PVR and &amp;gt;3 mmHg/L/min (r 0.15, p 0.310). Conclusion(s) Exercise-induced pulmonary hypertension is a promising tool in screening patients with systemic sclerosis for the detection of pulmonary hypertension at earlier preclinical stages. Identifying patients with closer follow-up for TTE at rest every 3 to 6 months and not every year as suggested in this disease.

Óxido nítrico plasmático em cães com hipertensão pulmonar secundária ou não à doença cardíaca esquerda

ABSTRACT Nitric oxide (NO) is an important mediator responsible for vasodilation in pulmonary hypertension (PH) in humans. Based on human literature, it is suggested that in dogs there is also NO production decrease in lung tissue in the presence of PH with hypoxia. Therefore, the aim of this research was to determine the indirect plasmatic NO concentration in dogs with PH secondary or not to the left-side heart disease (LHD) and also with low, intermediate and high probability of PH to characterize the NO involvement on PH in dogs. Blood samples were collected from 35 dogs with probability of PH to NO measurement. NO concentration was estimated by the nitrite/nitrate concentration, and it was significantly different (p=0.002) in dogs with PH secondary to LHD (median=14 µM, range 11.19-16.59) and not secondary to LHD (median=25.88µM, range 15.08-36.71). However, this was not significant for the probability of low, intermediate, and high PH, although there was a tendency for NO concentration to be higher in dogs with high PH. The results of this study demonstrate that there is release of NO in dogs with PH, as well as that its dosage could differentiate dogs with PH secondary to LHD from dogs with non-secondary PH.

Risk stratification for systemic lupus erythematosus associated pulmonary hypertension by simplified ESC/ERS guidelines: data from SOPHIE registry

Abstract Background The ESC/ERS guideline has established a diagnostic algorithm for pulmonary hypertension (PH) based on the peak velocity of tricuspid regurgitation, clinical history of left heart disease, chest radiography, and other chest radiological techniques. Unfortunately, due to the medical resource limitations, not all patients can receive the comprehensive inspections listed above. Purpose The purpose of the study was to sort out the practical predicting factors and evaluate the efficacy of the simplified diagnostic algorithm derived from the 2015 ESC/ERS clinical guidelines. Methods According to the ESC/ERS guideline, patients in the intermediate or high echocardiographic probability of PH group and further satisfied the following risk stratification criteria (anti-RNP (+), Raynaud phenomenon (+), 6MWD &amp;lt;440 meters, and SVR &amp;gt;1500 wood) were considered to have the intermediate (IR), and high risk (HR) of PH. Patients with a low echocardiographic probability of PH and those with intermediate or high probability of PH without satisfied the risk stratification algorithm were categorized into the low risk (LR) of the PH group. Results 1005 patients were categorized into the LR group, 30 patients in the IR group, and 19 in the HR group. Precisely, patients in the LR group were relatively younger (49.9±0.4) than that of the IR group (57.8±2.3, p=0.001) and the HR group (56.0±3.0, p=0.035). Raynaud phenomenon was occurred more frequently in the IR group (50.0%) or HR group (47.4%) compared to that of the LR group (24.1%). The occurrence of anti-RNP positive was higher in the HR group, taken up for 57.9% compared to 31.5% in the LR group (p=0.013). The incidence of right axis deviation (RAD) was increasing in the IR group (17.2%) and HR group (16.7%) compared to the LR group (3.6%). In addition, patients in IR and HR group have reduced lung volume parameters, including forced vital capacity (FVC%), forced expiratory volume at the first second (FEV1%), FEV1/FVC ratio, and forced expiratory flow at 50% of forced vital capacity (FEF50%) when compared to the LR group. Multivariate analysis was performed in four different modes to sort out the independent predictors of PH. Anti-RNP (OR=10.5, CI: 1.9, 56.5) and RAD (OR=21.9, CI: 5.2, 92.2) in mode 1, RVH (OR=10.4, CI: 1.9, 56.3) in mode 2, RAD (OR=10.3, CI: 2.0, 50.7) and FEF50% (OR=1.0, CI: 0.90, 0.97) in mode 3, and RVH (OR=56.8, CI: 6.9, 467.5) and FEF50% (OR=1.0, CI: 0.90, 0.97) in mode 4 were identified as the independent predictors of PH for SLE patients. Conclusions This registry has provided main clinical characteristics and phenotypes of Hong Kong Chinese patients with SLE and has demonstrated that Anti-RNP, RAD, RVH, or FEF50% was independent predictors of pulmonary hypertension in patients with SLE. Funding Acknowledgement Type of funding sources: None.

Exercise-induced pulmonary hypertension assessed by echocardiography in patients with chronic thromboembolic pulmonary disease

Abstract Background Many patients with chronic thromboembolic pulmonary disease (CTEPD) without pulmonary hypertension (PH) at rest suffer from exercise intolerance. Exercise echocardiography, which is a noninvasive examination, may have potential to discover exercise-induced PH in these patients, however, its role is scarcely explored in this population. Purpose To determine the occurrence of abnormal pulmonary pressure during rest and by exercise echocardiography in patients with CTEPD. Methods In total, 24 patients with CTEPD, all diagnosed after pulmonary embolism, underwent exercise echocardiography with dynamic supine leg exercise using a cycle ergometer. In addition, all participants underwent a modified incremental shuttle walk test (mISWT), pulmonary function tests and measurement of NT pro-BNP. Systolic pulmonary arterial pressure (sPAP) &amp;gt;50 mmHg by echocardiography during exercise was chosen as cutoff to define exercise-induced pulmonary hypertension (EIPH). Left ventricular diastolic dysfunction during stress was defined according to the American Society of Cardiology guidelines from 2016. Mean pulmonary artery pressure was estimated by sPAP measurements using the Chemla formula (0.61 x sPAP + 2 mmHg), and pulmonary vascular resistance (PVR) by the Doppler method (5.19 x TRV2/TVI RVOT − 0.4) proposed by Abbas et al. [1]. Subjects with heart failure with reduced or preserved ejection fraction, significant valvular heart disease, chronic pulmonary disease and chronic thromboembolic pulmonary hypertension were excluded. Results 11 (46%) of the patients had EIPH at peak exercise (range 50 to 89 mmHg). PVR at peak exercise ranged from 2.6 to 5.9 WU, whereas 10 had PVR &amp;gt;3.0 WU. None had unmasked left ventricular diastolic dysfunction during exercise, resting tricuspid regurgitation peak velocity (TR V max) &amp;gt;3.4m/s, or a high probability of PH at rest. Three patients had TR V max between 2.9 and 3.4m/s or intermediate probability of PH. All patients had normal biventricular systolic function at rest and during exercise (Table 2). Conclusion Patients with CTEPD had normal pulmonary pressures at rest. However, approximately half of the patients showed abnormal rise in pulmonary pressure during exercise, which may explain or contribute to the exercise intolerance in these patients. Funding Acknowledgement Type of funding sources: Public hospital(s). Main funding source(s): Ostfold Hospital Trust

MISSED OPPORTUNITIES AND ECHOCARDIOGRAPHIC EVIDENCE OF PULMONARY HYPERTENSION: A QUALITY-IMPROVEMENT TASK FORCE TO TRIGGER EARLY REFERRAL

MISSED OPPORTUNITIES AND ECHOCARDIOGRAPHIC EVIDENCE OF PULMONARY HYPERTENSION: A QUALITY-IMPROVEMENT TASK FORCE TO TRIGGER EARLY REFERRAL

Clinical assessment for pulmonary hypertension in interstitial lung disease.

Pulmonary hypertension (PH) is an important complication of interstitial lung disease (ILD), as its development confers a poor prognosis. There are no specific recommendations for methods of assessment for PH in ILD populations. To determine current assessment practices for PH in an Australian ILD centre. In the Austin Health ILD database, 162 consecutive patients with idiopathic pulmonary fibrosis or connective tissue disease-associated ILD were identified and retrospectively evaluated for methods of PH assessment with transthoracic echocardiography (TTE), serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and right heart catheterisation (RHC) in relation to patient demographic and physiological parameters. The median follow-up was 30 (14.4-56.4) months. At baseline, vital capacity was 80.0 ± 18.4% predicted, and diffusing capacity for carbon monoxide was 59.6 ± 15.2% predicted. Evaluation for PH was performed in 147 (90.7%) patients, among whom 105 (64.8%) had TTE performed at least once. At the initial TTE, 33.7% patients had high probability of PH, defined as RVSP >40 mmHg + RAp and/or right ventricular dysfunction. At the time of the most recent TTE, these criteria were met in 45 (52.3%) patients. Elevated serum NT-proBNP levels during the first year were observed in 47 (38.8%) patients. Only 14 (8.6%) patients had RHC. Our institutional PH assessment practice in ILD demonstrates a substantial prevalence of probable PH at baseline. As new therapies emerge for the treatment of PH in ILD, well-defined screening practices are important in this population for early identification and optimal management.

P245 A RARE CAUSE OF OVERESTIMATION OF PULMONARY PRESSURE BY ECHOCARDIOGRAPHY: WHEN HEMODYNAMICS RELIEVES ECHOCARDIOGRAPHY AND ECHOCARDIOGRAPHY EXPLAINS HEMODYNAMICS

Abstract Echocardiography is the gatekeeper of the diagnostic workflow of pulmonary hypertension (PH). However, it lacks precision, and right heart catheterization (RHC) may be needed in selected cases. A morbidly obese 47–year–old woman (BMI= 58 Kg/m2) with dyspnea NYHA II was referred to our Center after a high probability of PH was found at echocardiography, this latter performed as a routine assessment before bariatric surgery. Despite a suboptimal acoustic window, the tricuspid regurgitant jet profile was well–defined, with an estimated systolic pulmonary artery pressure (PAP) of 100 mmHg. There were no overt echocardiographic signs of left heart disease. During RHC, mean PAP was just above normal values, with filling pressures at the upper limit of normal (Figure 1), high cardiac index (3.6 L/min/m2) and normal pulmonary vascular resistance (1.4 WU). The echocardiographic estimate of high systolic PAP was explained by an intraventricular RV pressure gradient of about 100 mmHg (Figure 2). Oximetry ruled out a significant intracardiac shunt. Since resting hemodynamics did not explain the patient’s symptoms, the patient pre–test probability of having heart failure with preserved ejection fraction (HFpEF) was intermediate–high, and PAWP was in a grey–zone. Accordingly, we performed an exercise RHC. The test eventually unmasked HFpEF, as witnessed by an abnormal increase of PAWP and LV end–diastolic pressure (Figure 1 panel C and D). Exercise capacity was mildly reduced (71% of predicted) due to peripheral limitation with normal cardiac and respiratory reserves. Transesophageal echocardiography was then performed. It showed a muscular obstruction below the infundibulum dividing the RV into a high–pressure apical portion and a low–pressure infundibulum (Figure 3). This rare congenital anomaly was also associated with a restrictive perimembranous ventricular septal defect. Double–chambered RV has been reported to present rarely in adults, but it might be a cause of false Doppler echocardiographic estimate of PAP. Based on a meticulous invasive hemodynamic characterization at rest and during exercise, integrated with cardiovascular imaging, we could perform such a diagnosis. This congenital anomaly did not fully explain the patient’s symptoms, which may better be attributed to cardiovascular complication of obesity (HFpEF). Accordingly, we referred the patient for bariatric surgery first, postponing the indication to cardiac surgery during the follow–up.

P430 TUMORAL THROMBOTIC MICROANGIOPATHY: A RARE CAUSE OF ACUTE PULMONARY HYPERTENSION

Abstract Introduction Among the causes of acute pulmonary hypertension, the occlusion of the little pulmonary vessels caused by tumoral microemboli, even from an unknown neoplasm, is an infrequent condition with a very poor prognosis. Clinical Case A 55–years old woman with no relevant past medical history presented to the Emergency Department with dyspnea at rest, desaturation, palpitations and a brief episode of chest pain, after several days of fatigue and exertional dyspnea. ECG showed sinus tachycardia and signs of right ventricle pressure overload with inverted T waves in V3–V4 and in the inferior leads (Fig. 1). On laboratory tests there were high D–dimer values and a slightly elevated TnI. Blood gas analysis showed hypoxemic hypocapnic respirstory alkalosis. An Echocardiogram revealed severe dilatation and dysfunction of the right ventricle and signs of high probability of pulmonary hypertension (Fig. 2). Pulmonary CT scan ruled out embolism and showed irregular thickening of the interstitium and mediastinal lymphoadenopathy. Then, a rapid clinical deterioration happened, refractory to inotropes infusion and larger volumes of oxygen. The programmed diagnostic pathway cannot be pursued. On day 4, there was respiratory distress and pulseless electrical activity cardiac arrest during endotracheal intubation. ECMO mechanical support was positioned but on day 7 the patient died. Autopsy, made to discover the cause of pulmonary hypertension, revealed the presence of a colonic invasive adenocarcinoma with micropapillary pattern and thoracic and pelvic lymph–nodal metastases. Microscopic evaluation of the lungs showed a widespread neoplastic vascular invasion with microthromboemboli (Fig. 3). Discussion Tumoral thrombotic microangiopathy is a cause of pulmonary hypertension of multifactorial origin and should be suspected in cases of pulmonary hypertension without pulmonary embolism or interstitial lung disease. The rarity of this condition and the absence of an oncological medical history make difficult the diagnostic hypothesis. Besides, the lacking of an effective therapy makes the prognosis poor.

Pulmonary arterial hypertension after atrial septal defect closure: a case report

Most patients with congenital heart disease (CHD) who underwent successful shunts defect repair can be remained asymptomatic for years. It leads to a high number of losses to follow-up after patients discharge. After closure, pulmonary hypertension (PH) prevalence seems to be high and associated with increased morbidity and mortality. We reported a 55 y.o. female diagnosed with pulmonary arterial hypertension (PAH) and atrial fibrillation (AF) 31 years after atrial septal defect (ASD) closure by surgery, who never had routine follow-up evaluation because she remained asymptomatic for years. Physical examination revealed heart enlargement with irregular rhythm and pan systolic murmur in the fourth left sternal border. Electrocardiogram showed AF normal ventricular response, right axis deviation and suggestive for right ventricular hypertrophy. Laboratory testing found the NT pro-BNP level was 2,476 pg/mL. The chest X-ray showed enlargement of the heart and was representative of PH. From echocardiography study, transthoracic and transoesophageal echocardiography, we found no sign of residual shunt. There were right atrial and right ventricular dilatation, severe tricuspid regurgitation (TR) and a high probability of PH with TR velocity of 4.46 m/s. Right heart catheterization concluded mean pulmonary arterial pressure 46 mmHg, flow ratio 1.1, and pulmonary artery resistance index 15.5 Woods unit.m -2 . We highlight this case because of the high incidence of PH long after defect closure. The high number of lost to follow-up patients can lead to morbidity and mortality.

Assessment of Platelet and Plasma Serotonin in Canine Pulmonary Hypertension Secondary to Degenerative Mitral Valve Disease.

Background: Pulmonary hypertension (PH) is a common complication of degenerative mitral valve disease (DMVD), the most common cardiovascular disease in dogs. Serotonin has been suspected to play a role in the pathogenesis of PH, so this study aimed to investigate the differences in platelet and plasma serotonin between normal, DMVD and DMVD with PH (DMVD+PH) dogs.Materials and Methods: Sixty-two small-breed dogs were enrolled to the study and divided into the normal (n = 22), DMVD (n = 20), and DMVD+PH (n = 20) groups. The platelet and plasma serotonin concentrations were measured by the competitive ELISA.Results: The Kruskal–Wallis revealed the difference among the four groups of normal (179.73 [102.37–352.24] ng/109 platelets), DMVD (325.99 [96.84–407.66] ng/109 platelets), DMVD with intermediate probability of PH (291.11 [106.69–400.84] ng/109 platelets) and DMVD with high probability of PH (35.82 [2.69–126.35] ng/109 platelets) (p = 0.014). The Dunn's post-hoc test showed a decrease in the platelet serotonin concentration of the DMVD dogs with high probability of PH compared to the DMVD group (p = 0.008). The plasma serotonin concentration was not different between normal, DMVD, and DMVD+PH dogs.Conclusion: In conclusion, a decrease in platelet serotonin concentration, which is associated with a degree of PH probability was found in DMVD dogs with PH. Further studies investigating roles of platelet serotonin in PH secondary to DMVD should be performed.

Prevalence of Pulmonary Hypertension in an Unselected Community-Based Population: A Retrospective Echocardiographic Study-RES-PH Study.

Introduction. The actual prevalence of pulmonary hypertension (PH) in Italy is unknown. Echocardiography is useful in the screening of patients with suspected PH by estimation of the pulmonary artery systolic pressure (PASP) from the regurgitant tricuspid flow velocity evaluation, according to the simplified Bernoulli equation. Objectives. We aimed to evaluate the frequency of suspected PH among unselected patients. Methods. We conducted a retrospective cross-sectional database search of 7005 patients, who underwent echocardiography, to estimate the prevalence of PH, between January 2013 and December 2014. Medical and echocardiographic data were collected from a stratified etiological group of PH, using criteria of the European Society of Cardiology classifications. Results. The mean age of the study population was 57.1 ± 20.5 years, of which 55.3% were male. The prevalence of intermediate probability of PH was 8.6%, with nearly equal distribution between men and women (51.3 vs. 48.7%; p = 0.873). The prevalence of high probability of PH was 4.3%, with slightly but not significant higher prevalence in female patients (43.2 vs. 56.8%; p = 0.671). PH is predominant in patients with chronic obstructive pulmonary disease (COPD) or left ventricle (LV) systolic dysfunction and related with age. PASP was significantly linked with left atrial increase and left ventricular ejection fraction. In addition, an increased PASP was related to an enlargement of the right heart chamber. Conclusions. PH has a frequency of 4.3% in our unselected population, but the prevalence may be more relevant in specific subgroups. A larger epidemiological registry could be an adequate strategy to increase quality control and identify weak points in the evaluation and treatment of these patients.