Artificial intelligence-based model for predicting pulmonary arterial hypertension on chest x-ray images.

BACKGROUND: Pulmonary arterial hypertension is a serious medical condition. However, the condition is often misdiagnosed or a rather long delay occurs from symptom onset to diagnosis, associated with decreased 5-year survival. In this study, we developed and tested a deep-learning algorithm to detect pulmonary arterial hypertension using chest X-ray (CXR) images. METHODS: From the image archive of Chiba University Hospital, 259 CXR images from 145 patients with pulmonary arterial hypertension and 260 CXR images from 260 control patients were identified; of which 418 were used for training and 101 were used for testing. Using the testing dataset for each image, the algorithm outputted a numerical value from 0 to 1 (the probability of the pulmonary arterial hypertension score). The training process employed a binary cross-entropy loss function with stochastic gradient descent optimization (learning rate parameter, α = 0.01). In addition, using the same testing dataset, the algorithm's ability to identify pulmonary arterial hypertension was compared with that of experienced doctors. RESULTS: The area under the curve (AUC) of the receiver operating characteristic curve for the detection ability of the algorithm was 0.988. Using an AUC threshold of 0.69, the sensitivity and specificity of the algorithm were 0.933 and 0.982, respectively. The AUC of the algorithm's detection ability was superior to that of the doctors. CONCLUSION: The CXR image-derived deep-learning algorithm had superior pulmonary arterial hypertension detection capability compared with that of experienced doctors.

Peripheral microvascular function is linked to cardiac involvement on cardiovascular magnetic resonance in systemic sclerosis-related pulmonary arterial hypertension.

AIMS: Systemic sclerosis (SSc) is characterized by vasculopathy, inflammation, and fibrosis, and carries one of the worst prognoses if patients also develop pulmonary arterial hypertension (PAH). Although PAH is a known prognosticator, patients with SSc-PAH demonstrate disproportionately high mortality, presumably due to cardiac involvement. In this cross-sectional study, the relationship between cardiac involvement revealed by cardiovascular magnetic resonance (CMR) and systemic microvascular disease severity measured with nailfold capillaromicroscopy (NCM) in patients with SSc-PAH is evaluated and compared with patients with idiopathic PAH (IPAH). METHODS AND RESULTS: Patients with SSc-PAH and IPAH underwent CMR, echocardiography, and NCM with post-occlusive reactivity hyperaemia (PORH) testing on the same day. CMR imaging included T2 (oedema), native, and post-contrast T1 mapping to measure the extracellular volume fraction (ECV, fibrosis) and adenosine-stress-perfusion imaging measuring the relative myocardial upslope (microvascular coronary perfusion). Measures of peripheral microvascular function were related to CMR indices of oedema, fibrosis, and myocardial perfusion. SSc-PAH patients (n = 20) had higher T2 values and a trend towards a higher ECV, compared with IPAH patients (n = 5), and a lower nailfold capillary density (NCD) and reduced capillary recruitment after PORH. NCD correlated with ECV and T2 (r = -0.443 and -0.464, respectively, P < 0.05 for both) and with markers of diastolic dysfunction on echocardiography. PORH testing, but not NCD, correlated with the relative myocardial upslope (r = 0.421, P < 0.05). CONCLUSION: SSc-PAH patients showed higher markers of cardiac fibrosis and inflammation, compared with IPAH patients. These markers correlated well with peripheral microvascular dysfunction, suggesting that SSc-driven inflammation and vasculopathy concurrently affect peripheral microcirculation and the heart. This may contribute to the disproportionate high mortality in SSc-PAH.

Toward the Implementation of Optimal Cardiac Magnetic Resonance Risk Stratification in Pulmonary Arterial Hypertension.

BACKGROUND: The 2022 European Society of Cardiology/European Respiratory Society pulmonary hypertension (PH) guidelines incorporate cardiac magnetic resonance (CMR) imaging metrics in the risk stratification of patients with pulmonary arterial hypertension (PAH). Thresholds to identify patients at estimated 1-year mortality risks of < 5%, 5% to 20%, and > 20% are introduced. However, these cutoff values are mostly single center-based and require external validation. RESEARCH QUESTION: What are the discriminative prognostic properties of the current CMR risk thresholds stratifying patients with PAH? STUDY DESIGN AND METHODS: We analyzed data from incident, treatment-naïve patients with PAH from the Amsterdam University Medical Centres, Vrije Universiteit, The Netherlands. The discriminative properties of the proposed CMR three risk strata were tested at baseline and first reassessment, using the following PH guideline variables: right ventricular ejection fraction, indexed right ventricular end-systolic volume, and indexed left ventricular stroke volume. RESULTS: A total of 258 patients with PAH diagnosed between 2001 and 2022 fulfilled the study criteria and were included in this study. Of these, 172 had follow-up CMR imaging after 3 months to 1.5 years. According to the CMR three risk strata, most patients were classified at intermediate risk (n = 115 [45%]) upon diagnosis. Only 29 (11%) of patients with PAH were classified at low risk, and 114 (44%) were classified at high risk. Poor survival discrimination was seen between risk groups. Appropriate survival discrimination was seen at first reassessment. INTERPRETATION: Risk stratifying patients with PAH with the recent proposed CMR cutoffs from the European Society of Cardiology/European Respiratory Society 2022 PH guidelines requires adjustment because post-processing consensus is lacking and general applicability is limited. Risk assessment at follow-up yielded better survival discrimination, emphasizing the importance of the individual treatment response.

[Applying of magnetic resonance tomography for assessment of cardiac remodeling and risk stratification in patients with pulmonary arterial hypertension].

Magnetic resonance imaging - is high precision method for diagnosing cardiovascular diseases. Simultaneously with the anatomy and function of the right ventricle, magnetic resonance imaging allows to assess the pulmonary circulation, which leads to the widespread use of this method in the diagnosis and dynamic monitoring of patients with pulmonary arterial hypertension. The article is devoted to the assessment of cardiac remodeling and risk stratification of this group of patients. Special attention is given to new prognostic parameters included in the scale for risk stratification of patients with pulmonary hypertension of the European Society of Cardiology/European Respiratory Society 2022.

Establishing minimally important differences for cardiac MRI end-points in pulmonary arterial hypertension.

BACKGROUND: Cardiac magnetic resonance (CMR) is the gold standard technique to assess biventricular volumes and function, and is increasingly being considered as an end-point in clinical studies. Currently, with the exception of right ventricular (RV) stroke volume and RV end-diastolic volume, there is only limited data on minimally important differences (MIDs) reported for CMR metrics. Our study aimed to identify MIDs for CMR metrics based on US Food and Drug Administration recommendations for a clinical outcome measure that should reflect how a patient "feels, functions or survives". METHODS: Consecutive treatment-naïve patients with pulmonary arterial hypertension (PAH) between 2010 and 2022 who had two CMR scans (at baseline prior to treatment and 12 months following treatment) were identified from the ASPIRE registry. All patients were followed up for 1 additional year after the second scan. For both scans, cardiac measurements were obtained from a validated fully automated segmentation tool. The MID in CMR metrics was determined using two distribution-based (0.5sd and minimal detectable change) and two anchor-based (change difference and generalised linear model regression) methods benchmarked to how a patient "feels" (emPHasis-10 quality of life questionnaire), "functions" (incremental shuttle walk test) or "survives" for 1-year mortality to changes in CMR measurements. RESULTS: 254 patients with PAH were included (mean±sd age 53±16 years, 79% female and 66% categorised as intermediate risk based on the 2022 European Society of Cardiology/European Respiratory Society risk score). We identified a 5% absolute increase in RV ejection fraction and a 17 mL decrease in RV end-diastolic or end-systolic volumes as the MIDs for improvement. Conversely, a 5% decrease in RV ejection fraction and a 10 mL increase in RV volumes were associated with worsening. CONCLUSIONS: This study establishes clinically relevant CMR MIDs for how a patient "feels, functions or survives" in response to PAH treatment. These findings provide further support for the use of CMR as a clinically relevant clinical outcome measure and will aid trial size calculations for studies using CMR.

Plain language summaryPulmonary arterial hypertension (PAH) is a disease of the vessels of the lung that causes their narrowing and stiffening. As a result, the heart pumping blood into these diseased lung vessels has to work harder and eventually gets worn out. PAH can affect patients' ability to function in daily activities and impact their quality of life. It also reduces their life expectancy dramatically. Patients are, therefore, often monitored and undergo several investigations to adapt treatment according to their situation. These investigations include a survey of how a patient feels (the emPHasis-10 questionnaire), functions (walking test) and how well the heart is coping with the disease (MRI of the heart). Until now, it is unclear how changes on MRI of the heart reflect changes in how a patient feels and functions. Our study identified patients that had the emPHasis-10 questionnaire, walking test and MRI of the heart at both the time of PAH diagnosis and one year later. This allowed us to compare how the changes in the different tests relate to each other. And because previous research identified thresholds for important changes in the emPHasis-10 questionnaire and the walking tests, we were able to use these tests as a benchmark for changes in the MRI of the heart. Our study identified thresholds for change on heart MRI that might indicate whether a patient has improved or worsened. This finding might have implications for how patients are monitored in clinical practice and future research on PAH treatments.

Virtual Echocardiography Screening Tool Identifies Pulmonary Arterial Hypertension Significantly Earlier Than High-Risk Clinical Diagnosis.

Pulmonary arterial hypertension (PAH) is often a progressive, fatal disease. Because of nonspecificity of symptoms and limited awareness of PAH, patients are often diagnosed and referred late to accredited pulmonary hypertension (PH) centers, contributing to worsening survival and overall prognosis. The objective of the present study was to determine if the virtual echocardiography screening tool (VEST), a simple scoring system using routinely reported echocardiographic metrics, could capture earlier diagnoses of PAH before clinical recognition and referral to expert PH centers. This study is a retrospective analysis of 132 patients with PAH evaluated consecutively at 2 accredited referral PH centers. VEST scores and time to evaluation at PH center were quantified based on the first available echocardiogram before referral. Clinical risk assessment was calculated at initial evaluation by the PH center using the REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) 2.0 calculator. An overwhelming majority (93%) of the study participants had markedly abnormal VEST scores predictive of PAH before evaluation at a PH referral center. The median delay from VEST to evaluation was >6 months at 206 days (quartile 1, quartile 3: 55, 757). At initial evaluation, 72% were intermediate or high-risk based on REVEAL 2.0 risk assessment. In conclusion, we propose that VEST is a powerful yet simple scoring tool that can capture high-risk patients with PAH, prompting earlier diagnosis and referrals to accredited PH centers, and allowing for earlier expert implementation of PH medical therapies.

Pulmonary Artery Strain Predicts Prognosis in Pulmonary Arterial Hypertension.

BACKGROUND: Current cardiac magnetic resonance (CMR) imaging in pulmonary arterial hypertension (PAH) focuses on measures of ventricular function and coupling. OBJECTIVES: The purpose of this study was to evaluate pulmonary artery (PA) global longitudinal strain (GLS) as a prognostic marker in patients with PAH. METHODS: The authors included 169 patients with PAH from the ASPIRE (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre) and INITIATE (Integrated computatioNal modelIng of righT heart mechanIcs and blood flow dynAmics in congeniTal hEart disease) registries, and 82 normal controls with similar age and gender distributions. PA GLS was derived from CMR feature tracking. Right ventricular measurements including volumes, ejection fraction, and right ventricular GLS were also derived from CMR. Patients were followed up a median of 34 months with all-cause mortality as the primary endpoint. Other known risk scores were collected, including the REVEAL (Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management) 2.0 and COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) 2.0 scores. RESULTS: Of 169 patients (mean age: 57 ± 15 years; 80% female), 45 (26.6%) died (median follow-up: 34 months). Mean PA GLS was 23% ± 6% in normal controls and 10% ± 5% in patients with PAH (P < 0.0001). Patients with PA GLS <9% had a higher risk of mortality than those with PA GLS ≥9% (P < 0.001), and this was an independent predictor of mortality in PAH on multivariable analysis after adjustment for known risk factors (HR: 2.93; P = 0.010). Finally, in patients with PAH, PA GLS provided incremental prognostic value over the REVEAL 2.0 (global chi-square; P = 0.001; C statistic comparison; P = 0.030) and COMPERA 2.0 (global chi-square; P = 0.001; C statistic comparison; P = 0.048). CONCLUSIONS: PA GLS confers incremental prognostic utility over the established risk scores for identifying patients with PAH at higher risk of death, who may be targeted for closer monitoring and/or intensified therapy.

Derivation of a Risk Score (REVEAL-ECHO) Based on Echocardiographic Parameters of Patients With Pulmonary Arterial Hypertension.

BACKGROUND: Multiparametric risk assessment tools determine mortality risk in patients with pulmonary arterial hypertension (PAH) by combining invasive and noninvasive variables so management strategies can be tailored to individuals. RESEARCH QUESTION: Can a risk score based on common echocardiographic parameters risk-stratify patients with PAH? STUDY DESIGN AND METHODS: A Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) echocardiographic risk score (REVEAL-ECHO) was derived using retrospective echocardiographic data from 2,400 adult patients with PAH enrolled in the REVEAL registry database. A stepwise Cox regression model identified echocardiographic parameters significantly predictive of survival. Values were assigned to each selected parameter based on survival at 12 months' follow-up (Kaplan-Meier estimates). The REVEAL-ECHO risk score was the sum of individual values. Patients were categorized as having low, intermediate, or high risk based on Kaplan-Meier-predicted 12-month survival. RESULTS: The risk score included four echocardiographic parameters-right ventricular (RV) chamber enlargement, reduced RV systolic function, tricuspid regurgitation severity, and pericardial effusion-and accounted for PAH etiology. Higher REVEAL-ECHO risk scores signaled lower probability of 12-month survival. Statistically significant separation of mortality risk was observed among the risk strata: intermediate vs low (hazard ratio [HR], 1.43; 95% CI, 1.17-1.75; P = .0004) and high vs low (HR, 2.60; 95% CI, 2.19-3.10; P < .0001). Augmentation of the REVEAL Lite 2 risk calculator with REVEAL-ECHO risk scores achieved separation of REVEAL Lite 2 into four risk groups and identified a subgroup of patients with a low REVEAL Lite 2 risk score who were at higher risk (intermediate-low risk) and a subgroup of patients with an intermediate REVEAL Lite 2 risk score who also were at higher risk (intermediate-high risk). INTERPRETATION: A REVEAL-ECHO risk score, derived using four echocardiographic parameters, may discriminate risk further when used as an adjunct to current risk assessment scores. Further validation is required.

Right ventricular energetic biomarkers from 4D Flow CMR are associated with exertional capacity in pulmonary arterial hypertension.

BACKGROUND: Cardiovascular magnetic resonance (CMR) offers comprehensive right ventricular (RV) evaluation in pulmonary arterial hypertension (PAH). Emerging four-dimensional (4D) flow CMR allows visualization and quantification of intracardiac flow components and calculation of phasic blood kinetic energy (KE) parameters but it is unknown whether these parameters are associated with cardiopulmonary exercise test (CPET)-assessed exercise capacity, which is a surrogate measure of survival in PAH. We compared 4D flow CMR parameters in PAH with healthy controls, and investigated the association of these parameters with RV remodelling, RV functional and CPET outcomes. METHODS: PAH patients and healthy controls from two centers were prospectively enrolled to undergo on-site cine and 4D flow CMR, and CPET within one week. RV remodelling index was calculated as the ratio of RV to left ventricular (LV) end-diastolic volumes (EDV). Phasic (peak systolic, average systolic, and peak E-wave) LV and RV blood flow KE indexed to EDV (KEIEDV) and ventricular LV and RV flow components (direct flow, retained inflow, delayed ejection flow, and residual volume) were calculated. Oxygen uptake (VO2), carbon dioxide production (VCO2) and minute ventilation (VE) were measured and recorded. RESULTS: 45 PAH patients (46 ± 11 years; 7 M) and 51 healthy subjects (46 ± 14 years; 17 M) with no significant differences in age and gender were analyzed. Compared with healthy controls, PAH had significantly lower median RV direct flow, RV delayed ejection flow, RV peak E-wave KEIEDV, peak VO2, and percentage (%) predicted peak VO2, while significantly higher median RV residual volume and VE/VCO2 slope. RV direct flow and RV residual volume were significantly associated with RV remodelling, function, peak VO2, % predicted peak VO2 and VE/VCO2 slope (all P < 0.01). Multiple linear regression analyses showed RV direct flow to be an independent marker of RV function, remodelling and exercise capacity. CONCLUSION: In this 4D flow CMR and CPET study, RV direct flow provided incremental value over RVEF for discriminating adverse RV remodelling, impaired exercise capacity, and PAH with intermediate and high risk based on risk score. These data suggest that CMR with 4D flow CMR can provide comprehensive assessment of PAH severity, and may be used to monitor disease progression and therapeutic response. TRIAL REGISTRATION NUMBER: https://www. CLINICALTRIALS: gov . Unique identifier: NCT03217240.

Prognostic Value of Hepatic Native T1 and Extracellular Volume Fraction in Patients with Pulmonary Arterial Hypertension.

Background Right heart failure may lead to impaired liver perfusion and venous congestion, resulting in different extents of liver fibrosis. However, whether hepatic tissue deterioration determined by native T1 mapping and extracellular volume fraction using cardiac magnetic resonance imaging is associated with poor outcomes in patients with pulmonary arterial hypertension remains unclear. Methods and Results A total of 131 participants with pulmonary arterial hypertension (mean age, 36±13 years) and 64 healthy controls (mean age, 44±18) between October 2013 and December 2019 were prospectively enrolled. Hepatic native T1 and extracellular volume fraction values were measured using modified Look-Locker inversion recovery T1 mapping sequences. The primary end point was all-cause mortality; the secondary end point was all-cause mortality and repeat hospitalization attributable to heart failure. Cox regression models and Kaplan-Meier survival analysis were used to identify the association between variables and clinical outcome. During a median follow-up of 34.5 months (interquartile range: 25.3-50.8), hepatic native T1 (hazard ratio per 30-ms increase, 1.22 [95% CI, 1.07-1.39]; P=0.003) and extracellular volume fraction (hazard ratio per 3% increase, 1.18 [95% CI, 1.04-1.34]; P=0.010) values were associated with a higher risk of death. In the multivariate Cox model, hepatic native T1 value (hazard ratio per 30-ms increase, 1.15 [95% CI, 1.04-1.27]; P=0.009) remained as an independent prognostic factor for the secondary end point. Conclusions Hepatic T1 mapping values were predictors of adverse cardiovascular events in participants with pulmonary arterial hypertension and could be novel imaging biomarkers for poor prognosis recognition.

Right ventricular remodelling in pulmonary arterial hypertension predicts treatment response.

OBJECTIVES: To determine the prognostic value of patterns of right ventricular adaptation in patients with pulmonary arterial hypertension (PAH), assessed using cardiac magnetic resonance (CMR) imaging at baseline and follow-up. METHODS: Patients attending the Sheffield Pulmonary Vascular Disease Unit with suspected pulmonary hypertension were recruited into the ASPIRE (Assessing the Spectrum of Pulmonary hypertension Identified at a REferral Centre) Registry. With exclusion of congenital heart disease, consecutive patients with PAH were followed up until the date of census or death. Right ventricular end-systolic volume index adjusted for age and sex and ventricular mass index were used to categorise patients into four different volume/mass groups: low-volume-low-mass, low-volume-high-mass, high-volume-low-mass and high-volume-high-mass. The prognostic value of the groups was assessed with one-way analysis of variance and Kaplan-Meier plots. Transition of the groups was studied. RESULTS: A total of 505 patients with PAH were identified, 239 (47.3%) of whom have died at follow-up (median 4.85 years, IQR 4.05). The mean age of the patients was 59±16 and 161 (32.7%) were male. Low-volume-low-mass was associated with CMR and right heart catheterisation metrics predictive of improved prognosis. There were 124 patients who underwent follow-up CMR (median 1.11 years, IQR 0.78). At both baseline and follow-up, the high-volume-low-mass group had worse prognosis than the low-volume-low-mass group (p<0.001). With PAH therapy, 73.5% of low-volume-low-mass patients remained in this group, whereas only 17.4% of high-volume-low-mass patients transitioned into low-volume-low-mass. CONCLUSIONS: Right ventricular adaptation assessed using CMR has prognostic value in patients with PAH. Patients with maladaptive remodelling (high-volume-low-mass) are at high risk of treatment failure.

The added value of right ventricular function normalized for afterload to improve risk stratification of patients with pulmonary arterial hypertension.

BACKGROUND: Risk stratification is central to the management of pulmonary arterial hypertension (PAH). For this purpose, multiparametric tools have been developed, including the ESC/ERS risk score and its simplified versions derived from large database analysis such as the COMPERA and the French Pulmonary Hypertension Network (FPHN) registries. However, the distinction between high and intermediate-risk profiles may be difficult as the latter lacks granularity. In addition, neither COMPERA or FPHN strategies included imaging-derived markers. We thus aimed at investigating whether surrogate echocardiographic markers of right ventricular (RV) to pulmonary artery (PA) coupling could improve risk stratification in patients at intermediate-risk. MATERIAL AND METHODS: A single-center retrospective analysis including 102 patients with a diagnosis of PAH was performed. COMPERA and FPHN strategies were applied to stratify clinical risk. The univariate linear regression was used to test the influence of the echo-derived parameters qualifying the right heart (right ventricle basal diameter, right atrial area, and pressure, tricuspid regurgitation velocity, tricuspid annular plane systolic excursion -TAPSE-). Among these, the TAPSE and tricuspid regurgitation velocity ratio (TAPSE/TRV) as well as the TAPSE and systolic pulmonary artery pressure ratio (TAPSE/sPAP) were considered as surrogate of RV-PA coupling. RESULTS: TAPSE/TRV and TAPSE/sPAP resulted the more powerful markers of prognosis. Once added to COMPERA, TAPSE/TRV or TAPSE/sPAP significantly dichotomized intermediate-risk group in intermediate-to-low-risk (TAPSE/TRV≥3.74 mm∙nm/s)-1 or TAPSE/sPAP≥0.24 mm/mmHg) and in intermediate-to-high-risk subgroups (TAPSE/TRV<3.74 mm∙(m/s)-1 or TAPSE/sPAP<0.24 mm/mmHg). In the same way, TAPSE/TRV or TAPSE/sPAP was able to select patients at lower risk among those with 2, 1, and 0 low-risk criteria of both invasive and non-invasive FPHN registries. CONCLUSIONS: Our results suggest that adopting functional-hemodynamic echo-derived parameters may provide a more accurate risk stratification in patients with PAH. In particular, TAPSE/TRV or TAPSE/sPAP improved risk stratification in patients at intermediate-risk, that otherwise would have remained less characterized.

Evaluation of right ventricular myocardial strain in pulmonary arterial hypertension associated with atrial septal defect by cardiac magnetic resonance feature tracking.

We aimed to research the role of right ventricular strain parameters (RVSP) quantified by cardiac magnetic resonance feature tracking (CMR-FT) in the early assessment of right ventricular (RV) function in patients with pulmonary arterial hypertension associated with atrial septal defect (PAH-ASD). From September 2017 to May 2021, we retrospectively enrolled 41 patients with PAH-ASD and 20 healthy controls. All subjects underwent CMR-FT, and right heart catheterization was conducted in patients with PAH-ASD. The relationship between RVSP and RV functional parameters was subjected to correlation analysis, and intragroup correlation coefficient (ICC) and Bland-Altman plots were used to assess the consistency. The subjects were divided into three groups: Group A (controls; n = 20), Group B (PAH-ASD, RVEF ≥ 45%; n = 14), and Group C (PAH-ASD, RVEF < 45%; n = 27). Compared with healthy controls, the RV global longitudinal strain (GLS) in Group B was significantly decreased (- 19.68 ± 2.72% vs. - 25.21 ± 3.6%, P < 0.05). In RVEF-preserved PAH-ASD patients (Group B), compared with patients with GLS ≤ - 20%, patients with GLS > - 20% also had significantly elevated right ventricular end-diastolic pressure (RVEDP) [8 (6.5-8.25) mmHg vs. 4.5 ± 1.64 mmHg, P < 0.05]. RV GLS had a moderate to strong correlation with RVEF, RVESVi, RVEDVi, RVEDP, and NT-proBNP (P < 0.05). ICC and Bland-Altman plots showed good intragroup and intergroup consistency in radial, circumferential and longitudinal strains of RV. In conclusion, it is feasible to quantify RV strain in patients with PAH-ASD by CMR-FT, and GLS is valuable for the early assessment of RV dysfunction in patients with PAH-ASD.

In systemic sclerosis, the TAPSE/sPAP ratio can be used in addition to the DETECT algorithm for pulmonary arterial hypertension diagnosis.

OBJECTIVE: Early detection of pulmonary arterial hypertension (PAH) is crucial for improving patient outcomes. The aim of this study was to compare the positive predictive value (PPV) of the echocardiography-derived tricuspid annular plane systolic excursion/systolic pulmonary artery pressure (TAPSE/sPAP) ratio with that of the DETECT algorithm for PAH screening in a cohort of SSc patients. METHODS: Fifty-one SSc patients were screened for PAH using the DETECT algorithm and echocardiography. RESULTS: Echocardiography was recommended by the DETECT algorithm step 1 in 34 patients (66.7%). Right heart catheterization (RHC) was recommended by the DETECT algorithm step 2 in 16 patients (31.4%). PAH was confirmed by RHC in 5 patients. The DETECT algorithm PPV was 31.3%. The TAPSE/sPAP ratio was higher in SSc patients not referred for RHC than in SSc patients referred for RHC according to the DETECT algorithm step 2 [0.83 (0.35-1.40) mm/mmHg vs 0.74 (0.12-1.09) mm/mmHg, P < 0.05]. Using a cut-off of 0.60 mm/mmHg, 8 (15.7%) SSc patients had a TAPSE/sPAP ratio of ≤0.60 mm/mmHg. PAH was confirmed by RHC in 5 patients. The PPV of TAPSE/sPAP was 62.5%. In multiple regression analysis, TAPSE/sPAP was associated with age [ß coefficient = -0.348 (95% CI: -0.011, -0.003); P < 0.01], DETECT algorithm step 1 [ß coefficient = 1.023 (95% CI: 0.006, 0.024); P < 0.01] and DETECT algorithm step 2 (ß coefficient = -1.758 [95% CI: -0.059, -0.021]; P < 0.0001). CONCLUSION: In SSc patients with a DETECT algorithm step 2 total score of >35, the TAPSE/sPAP ratio can be used to further select patients requiring RHC to confirm PAH diagnosis.

Perinatal Management and Long-Term Follow-up of a Primipara With Severe Pulmonary Arterial Hypertension Associated With Systemic Lupus Erythematosus.

BACKGROUND: Systemic lupus erythematosus (SLE) accounts for the largest portion of connective tissue disease-associated pulmonary arterial hypertension (PAH) in Asian countries, especially in China, and SLE-PAH poses multiple challenges during pregnancy and delivery. Patients with SLE-PAH tend to have lower survival rates and worse quality of life than other subgroups of PAH. CASE PRESENTATION: Presented in this report is a 28-year-old primipara who suffered from SLE for 13 years and SLE-PAH for nine years. She had cardiac care throughout these years. She was admitted at 26 weeks of gestation for progressive dyspnea on exertion and her condition improved after a three-week PAH-targeted therapy consisting of prostacyclin and PDE-5 inhibitor. At 29 weeks of gestation, she was infected with influenza H1N1 and her clinical status deteriorated with increased dyspnea. After two weeks of influenza therapy and maximization of PAH therapy, a cesarean delivery was performed under epidural anesthesia at 31 weeks of gestation. She was discharged ten days after delivery. Although the targeted therapy for both PAH and SLE was readjusted after delivery and regular follow-up showed a gradual recovery and a stable condition, she still died suddenly at home 12 months after delivery. The child is healthy. CONCLUSIONS: Sequential combination therapy of PAH and SLE and the structured perinatal management might lead to optimal short-term outcomes in the mother and fetus. Long-term outcomes in women with PAH who become pregnant are poor, with high rates of morbidity and mortality. Delivery strategies remain an important challenge for modern Pregnancy Heart Teams.

Evaluation of pulmonary arterial pressure in patients with connective tissue disease-associated pulmonary arterial hypertension by myocardial perfusion imaging.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a complex and severe complication of connective tissue disease (CTD). We aimed to evaluate the application value of myocardial perfusion imaging (MPI) in evaluating CTD-associated PAH (CTD-PAH). METHODS: We retrospectively included 88 patients who were diagnosed with CTD between January 2018 and December 2020 at our hospital. Fifty-eight patients had PAH and were included into the CTD-PAH group. Thirty patients without PAH were included in the control group. All patients received routine physical examination, biochemical tests and cardiac function evaluation, right heart catheterization (RHC), and 99m Tc-MIBI MPI. PAH patients were divided into the mild, moderate, and severe PAH group according to their mean pulmonary artery pressures by RHC. Pearson correlation analysis was used to calculate the correlation between the right ventricle target/background (T/B) and right ventricle stroke volume (RV-SV), total pulmonary resistance (TPR), pulmonary vascular resistance (PVR), mean pulmonary arterial pressure (mPAP), 6-minute walk distance (6-MWD), and N-terminal B-type natriuretic peptide (NT-proBNP). The ROC curves of T/B and pulmonary artery pressure classification were plotted and the sensitivity and specificity of T/B in diagnosing PAH of different severities were analyzed. RESULTS: The analysis of correlation revealed that T/B correlated negatively with 6-MWD and positively with NT-proBNP and exhibited good positive correlation with mPAP, TPR, and PVR by RHC and negative correlation with RV-SV. T/B was of the most diagnostic value for severe PAH, and its correlation with severe PAH was stronger than that with mild PAH and moderate PAH. CONCLUSIONS: Target/background is a noninvasive method that can simultaneously evaluate pulmonary arterial pressure and myocardial perfusion of CTD-CHD patients and is particularly of relatively high value for severe PAH patients.