Search Results (195)

Distributive shock and acute respiratory distress syndrome (ARDS) are syndromes of profound pathophysiological complexity, each independently associated with high morbidity and mortality. When coexistent, they create a state of synergistic cardiopulmonary failure where conventional, protocolized management approaches are often insufficient. This review synthesizes current mechanistic insights into heart–lung interactions in distributive shock with ARDS, highlighting the central role of right ventricular–pulmonary arterial coupling and the dual impact of altered lung mechanics and vascular dysregulation. We examine the distinct hemodynamic implications of pulmonary versus extrapulmonary ARDS phenotypes, including their divergent effects on transpulmonary pressure, venous return, and right ventricular afterload, and emphasize the clinical relevance of mixed phenotypes. Advanced monitoring modalities—esophageal manometry, echocardiography, and, in select cases, pulmonary artery catheterization—are presented as essential tools for dynamic phenotyping and individualized titration of ventilatory and hemodynamic strategies. Building on these principles, we outline phenotype-directed approaches to ventilation, fluid and vasoactive therapy, and adjunctive interventions such as proning and extracorporeal support. Finally, we discuss knowledge gaps and future directions, underscoring the need for integrative technologies and phenotype-stratified trials to refine precision management. The nuanced integration of cardiopulmonary physiology into bedside decision-making represents a paradigm shift toward individualized, physiology-guided care for this high-risk population. Full article

Background: Arteriovenous fistula (AVF) in patients with end-stage renal disease (ESRD) can lead to high-output heart failure (HOHF). There is limited data on the incidence and outcomes of HOHF in patients with AVF. Objective: The main goal of our study was to determine the incidence and prevalence of HOHF [diagnosed via right heart catheterization (RHC)] in chronic kidney disease (CKD)/ESRD patients with AVF. We also aimed to evaluate the clinical determinants of the development of HOHF in this group. Methods: We conducted a retrospective cohort study at the University of Kansas Medical Center from January 2011 to December 2023. Patients with CKD/ESRD with AVF who underwent RHC after AVF creation were included in the study. HOHF was defined as a cardiac index (CI) ≥ 4.0 L/min/m², measured either with the Fick or the Thermodilution method. Bivariate and multivariable regression analyses were performed to identify independent predictors of HOHF in this population. Results: Out of 84 patients with AVF, 34 patients met established inclusion and exclusion criteria. Ten out of thirty-four patients (29.4%) developed HOHF. Hemoglobin (Hb) was significantly lower in the HOHF group than the non-HOHF group (10.16 vs. 11.52 g/dL; p = 0.02). Patients with HOHF had significantly elevated CI when compared with the non-HOHF group (CI Fick: 4.54 vs. 2.91 L/min/m², p < 0.001). Similar mortality was observed in the HOHF and non-HOHF groups. After multivariant regression analysis, Hb was an independent predictor of HOHF (HR 0.86, 95% CI: 0.76–0.98, p =< 0.01). Conclusions: HOHF is common in patients with AVF; nearly 1/3 (29.4%) of the patients developed HOHF, confirmed with RHC. Low Hb was found to be an independent predictor of HOHF. Further larger studies are needed to confirm these findings and establish an early detection protocol to detect and treat this condition. Full article

Background and Clinical Significance: Idiopathic pulmonary arterial hypertension is a rare disorder, often linked to genetic predisposition. Canonical pulmonary arterial hypertension genes such as BMPR2, KCNK3, and TBX4 are well described, but novel associations continue to emerge. Glomulin (GLMN) encodes a protein essential for vascular smooth-muscle biology, classically implicated in glomuvenous malformations, yet not previously associated with pulmonary arterial hypertension. Case Presentation: We present a 49-year-old woman with progressive dyspnea, edema, and persistent hypercapnic respiratory failure. Right-heart catheterization confirmed precapillary pulmonary hypertension. Comprehensive evaluation, including ventilation/perfusion scanning, autoimmune panel, polysomnography, and high-resolution computed tomography, excluded secondary causes. Respiratory assessment revealed diaphragmatic weakness and reduced respiratory muscle pressures, consistent with primary myopathy and explaining the unusual hypercapnic profile. Whole-genome sequencing identified a heterozygous pathogenic GLMN nonsense variant, while canonical pulmonary arterial hypertension genes were negative. No cutaneous or mucosal glomuvenous malformations were found. The patient was treated with oxygen therapy, diuretics, non-invasive ventilation, and dual oral pulmonary arterial hypertension therapy (ambrisentan and tadalafil), with stabilization but persistent hypercapnia. Conclusions: To our knowledge, this is the first reported co-occurrence of idiopathic pulmonary arterial hypertension and a pathogenic GLMN variant. While causality cannot be inferred, glomulin’s role in vascular smooth-muscle maturation provides a plausible link to pulmonary vascular remodeling. This case underscores the importance of assessing respiratory muscle function in idiopathic pulmonary arterial hypertension patients with hypercapnia and highlights the potential relevance of extended genetic testing in rare pulmonary vascular disease. Full article

Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a life-threatening vascular complication of SSc, marked by high morbidity and mortality. Early diagnosis remains a major challenge due to nonspecific symptoms and the limitations of conventional tools such as echocardiography (ECHO), pulmonary function tests (PFTs), and serum biomarkers. This review evaluates the emerging role of artificial intelligence (AI), particularly machine learning (ML) and deep learning (DL), in improving the diagnostic landscape of SSc-PAH. A comprehensive literature search was conducted across PubMed, Scopus, IEEE Xplore, Embase and Google Scholar to identify studies involving AI applications in SSc, pulmonary arterial hypertension (PAH), and their intersection. Evidence indicates that AI models can assist interpretation across modalities, including heart sounds, ECGs, chest X-rays (CXRs), ECHOs, CT pulmonary angiography (CTPA), and omics-based biomarkers. While several models show encouraging diagnostic performance, their accuracy varies by dataset and modality, and most require external validation against right heart catheterization (RHC)-confirmed cohorts. Integrating multimodal data through AI frameworks may enhance early recognition and individualized risk stratification; however, these tools remain exploratory. Future work should emphasize harmonized hemodynamic definitions, transparent validation protocols, and SSc-specific datasets to ensure clinical applicability and reproducibility. Full article

Background: Pulmonary hypertension (PH) in newborns is a rare but serious condition and potentially life-threatening disorder, often initially confused with congenital heart disease due to overlapping echocardiographic findings in the late third trimester. Evidence on prenatal predictors of postnatal PH is limited. We aimed to describe detailed third-trimester echocardiographic findings associated with postnatal PH in infants with prenatally suspected CoA based on a retrospective case series. Methods: We reviewed 18 years of fetal echocardiography (2004–2022) in a tertiary maternal–fetal–neonatal center. We identified fetuses with suspected coarctation of the aorta (CoA) in late gestation who were delivered at term (≥37 weeks) and had prolonged neonatal hospitalization (>10 days) without cardiac surgery or catheterization. Z-scores for cardiac dimensions were calculated. All examinations were performed by experienced fetal cardiologists. Postnatal evaluations confirmed PH based on echocardiographic and clinical findings. Results: Among 19,836 fetuses examined, 138 were prenatally suspected of CoA. In 70 cases, this diagnosis was not confirmed postnatally (false positives). Of these, eight infants (0.04% of the total cohort) developed postnatal PH. Postnatally, all eight neonates required intensive care. Prenatal features included ventricular/atrial disproportion (7/8), cardiomegaly (8/8), main pulmonary artery dilatation (10.2 ± 2.2 mm; Z-score +2.7 ± 1.3), tricuspid regurgitation (8/8), pulmonary regurgitation (4/8), and interventricular septal hypertrophy (>4.5 mm in 5/8). Postnatal evaluations confirmed PH based on echocardiographic criteria (elevated right ventricular pressure, septal flattening/bowing, right ventricular dilation or dysfunction, and abnormal shunt direction) combined with clinical compromise. All infants received prostaglandin E1 (PGE1) initially; none required extracorporeal membrane oxygenation-ECMO. Three died, while five survived with medical management (oxygen, inhaled nitric oxide, sildenafil). Conclusions: Specific functional abnormalities on late third-trimester echocardiography may indicate impaired pulmonary vascular adaptation and predict postnatal PH, particularly in cases initially suspected of CoA. Recognition and awareness of these findings can guide delivery planning, neonatal surveillance, and timely intervention. Prospective multicenter studies are needed to validate these associations and refine prenatal screening protocols. Full article

Background: Schistosomiasis, HIV, and tuberculosis frequently lead to pulmonary hypertension in low- and middle-income countries. Lack of specific testing and limited access to right heart catheterization hamper confirmation of the etiology of pulmonary hypertension due to schistosomiasis. In addition, low health literacy and poor socioeconomic status further compromise prevention, early diagnosis, and treatment. Clinical algorithms for early screening, including hand-held echocardiography and point-of-care testing performed by non-specialists, are needed in rural Sub-Saharan Africa to decentralize care and improve outcomes. Methods: We describe a case of pulmonary hypertension diagnosed in a child living in Mozambique, to discuss the challenges for the diagnosis of infectious pulmonary arterial hypertension in rural settings in Africa, based on a short literature review. Full article

(1) Objective: The objective was to test the hypothesis that cine MRI-derived radiomic features can detect functional tricuspid regurgitation (FTR) in the context of pulmonary hypertension (PH). (2) Materials and methods: In total, 53 PH patients were retrospectively enrolled. Thirty-three patients had echocardiography-defined mild-to-severe FTR, while the other twenty patients had no or trivial regurgitation. For all participants, 93 radiomic features were extracted from four-chamber cine MRI using a fixed-size region of interest (ROI) located in the right atrium (RA), 0.5–1 cm above the tricuspid valve. The levels of radiomic features were averaged over the ventricular systole and compared between patients with and without FTR using t tests. In patients with FTR, radiomic features were related to hemodynamic parameters in the right heart using the Pearson correlation coefficient (r). (3) Results: There were no significant differences in demographic information, right heart catheterization (RHC) results, and most cine MRI-derived cardiac function indices between the two subject groups. Eight of ninety-three radiomic features were significantly different between PH patients with and without FTR. Radiomic features can be used to discriminate two subject groups (AUC = 0.77). In patients with FTR, multiple radiomic features are related to the pressure in the RA, right ventricle (RV), and pressure difference between RA and RV (r: 0.4 to 0.55), p values < 0.05. (4) Conclusion: Cine MRI-derived radiomic features of the cardiac blood pool differ between PH patients with and without FTR. Cine MRI shows promise as a method for assessing FTR in the context of complex cardiovascular diseases (CVDs). Full article

Background: Treprostinil has demonstrated effectiveness in treating Pulmonary Arterial Hypertension (PAH) and Pulmonary Hypertension associated with Interstitial Lung Disease (PH-ILD). However, tolerability remains a clinical challenge. Identifying factors influencing tolerability is important, given the adverse outcomes of PAH and PH-ILD and the potential of treprostinil to slow disease progression. Objective: This study was undertaken to identify tolerance factors and develop a predictive scoring system. Methods: A retrospective analysis of 65 patients (37 PAH, 28 PH-ILD) was conducted using patient history, pulmonary function tests (PFTs), transthoracic echocardiograms (TTEs), and right heart catheterizations (RHCs). Of these, 67.7% (n = 44) tolerated treprostinil, while 32.3% (n = 21) were intolerant. Results: Patients who tolerated treprostinil had better pulmonary function, with a higher forced expiratory volume in one second/forced vital capacity (FEV₁/FVC) ratio (82.27 ± 16.06 vs. 72.86 ± 17.76, p = 0.037) and superior right ventricular function, as indicated by higher tricuspid annular plane systolic excursion (TAPSE: 2.05 ± 0.37 vs. 1.64 ± 0.42, p < 0.001), higher cardiac index (CI: 2.51 ± 0.67 vs. 2.03 ± 0.53, p = 0.003), and improved functional status (p < 0.001). The Inhaled Treprostinil Intolerance Score (ITIS), incorporating TAPSE < 1.6, CI < 2, FEV₁/FVC < 70%, and WHO functional class (FC) 3 or 4, demonstrated strong predictive accuracy (cutoff ≥ 2, AUC = 0.884 ± 0.048, p < 0.001). Predictive performance was stronger in PAH patients (AUC = 0.921 ± 0.053) than PH-ILD (AUC = 0.833 ± 0.093, p < 0.001). Conclusions: These findings demonstrate the importance of clinical parameters in predicting treprostinil tolerance. Further investigation is warranted to refine the scoring system, particularly for PH-ILD patients. Full article

Background: Pulmonary hypertension (PH) disproportionately affects those residing in low- and middle-income countries (LMICs). Given that these countries also have a high prevalence of infectious diseases, many cases of PH are either directly or indirectly related to infectious etiologies. Despite this correlation, the precise burden of infectious disease-associated PH is largely underappreciated due to a lack of diagnostic resources, a shortage of clinical expertise to carry out right heart catheterization and poor access to healthcare facilities in many low- and middle-income settings. Methods: In this narrative review, we highlight the significant burden of infectious disease-associated PH in LMICs, outline the technical challenges faced by LMICs when diagnosing PH, and propose possible solutions for diagnosing PH in resource-constrained settings. Conclusions: Low-cost and sustainable solutions for infectious disease-associated PH in LMICs should be prioritized. Meaningful solutions require collaborative efforts and capacity building in LMICs. Full article

Background: Left ventricular myocardial work (MW) derived from non-invasive pressure–strain loops has emerged as a load-adjusted index of contractile performance. Its value for risk stratification in advanced heart failure (HF) remains uncertain. Methods: We retrospectively studied 151 consecutive patients with advanced HF undergoing comprehensive evaluation at our tertiary centre between January 2016 and December 2022. MW parameters—left ventricular global work index (LVGWI), global constructive work (LVGCW), global wasted work (LVGWW) and global work efficiency (LVGWE)—were derived from speckle-tracking echocardiography integrated with brachial blood pressure. Cardiopulmonary exercise testing (CPET), right heart catheterisation (RHC) and biochemical markers were obtained. Patients were stratified according to an LVGWI threshold of 600 mmHg%, identified by receiver operating characteristic (ROC) analysis for predicting the combined end point of cardiovascular mortality or HF hospitalisation. Correlations between MW and traditional indices were assessed, and event-free survival was analysed by Kaplan–Meier curves. Results: LVGWI correlated modestly with pVO₂ (r = 0.35, p = 0.01) and left ventricular ejection fraction (r = 0.42, p < 0.001) and inversely with NT-proBNP (r = −0.30, p = 0.03). LVGWI displayed the largest area under the curve (AUC 0.76 [95% confidence interval 0.65–0.85]) for predicting the combined end point compared with pVO₂ (AUC 0.73) and LVEF (AUC 0.67). Dichotomisation by LVGWI ≤ 600 mmHg% identified a high-risk group (Group A) with worse NYHA class, lower systolic blood pressure and reduced exercise capacity. After a median follow-up of 24 months, Group A exhibited significantly lower event-free survival (log-rank p = 0.02). Multivariable analysis was not performed owing to the limited sample size; therefore, findings should be interpreted with caution. Conclusions: In patients with advanced HF, left ventricular myocardial work, particularly LVGWI, provides incremental prognostic information beyond conventional markers. An LVGWI cut-off of 600 mmHg% derived from ROC analysis identified patients at increased risk of cardiovascular events and may inform timely referral for mechanical circulatory support or transplantation. Larger prospective studies are warranted to confirm these observations and to establish standardised thresholds across vendors. Full article

Background: Pulmonary arterial pressure is a key parameter for diagnosing cardiovascular and pulmonary diseases. Its measurement through right heart catheterization is considered the gold standard, and it is an invasive procedure that entails significant risks for patients. This has motivated the development of non-invasive techniques based on patient-specific imaging, such as Physics-Informed Neural Networks (PINNs), which integrate clinical measurements with physical models, such as the 1D reduced Navier–Stokes model, enabling biologically plausible predictions with limited data. Methods: This work implements a PINN model that uses velocity and area measurements in the main bifurcation of the pulmonary artery, comprising the main artery and its secondary branches, to predict pressure, velocity, and area variations throughout the bifurcation. The model training includes penalties to satisfy the laws of flow and momentum conservation. Results: The results show that, using 4D Flow MRI images from a healthy patient as clinical data, the pressure estimates provided by the model are consistent with the expected ranges reported in the literature, reaching a mean arterial pressure of 21.5 mmHg. Conclusions: This model presents an innovative approach that avoids invasive methods, being the first study to apply PINNs to estimate pulmonary arterial pressure in bifurcations. In future work, we aim to validate the model in larger populations and confirm pulmonary hypertension cases diagnosed through catheterization. Full article

Background: Right heart catheterization (RHC) is the gold-standard for diagnosis of pulmonary hypertension (PH) but is a terminal procedure in neonatal mice. The objective was to validate echocardiographic measures of PH to establish the diagnostic capability against pulmonary vascular histology in neonatal mice. Methods: Adult mice, exposed to hypoxia or normoxia, were assessed by echocardiography and RHC to evaluate right ventricle (RV) morphometry and function. Echocardiographic measures identified in adult mice were then used to evaluate PH characteristics in hypoxia-exposed neonatal mice. Physiological parameters were compared to histopathology in all mice. Results: Hypoxia-challenged adult mice developed PH with RHC, demonstrating confirmed elevated RV systolic pressure (RVSP), RV hypertrophy, and increased cross-sectional area and neomuscularization of pulmonary vessels. Echocardiography-derived RV free wall (RVFW) thickness correlated with RV mass. Tricuspid valve annulus tissue Doppler imaging (TV TDI), tricuspid annular plane systolic excursion (TAPSE), pulmonary artery acceleration measures (PAAT), and TAPSE × PAAT (a measure of RV work) all correlated with RVSP determined by RHC. In neonatal mice exposed to hypoxia, PAAT, TV TDI, TAPSE, and TAPSE × PAAT were decreased and RVFW thickness was increased, correlating with the histologic phenotype of PH. Conclusions: Echocardiographic indices of RV morphology and function provide reliable estimates of invasive RV hemodynamics in hypoxia-induced PH. Full article

Background/Objectives:: Right ventricular (RV) pressure assessment is crucial in both the diagnosis and follow-up of patients with chronic thromboembolic pulmonary hypertension (CTEPH). While right heart catheterization (RHC) and pulmonary angiography (PA) are gold-standard invasive methods, transthoracic echocardiography (TTE) offers a safer and more accessible alternative. This study aimed to evaluate the reliability of echocardiographic RV pressure estimation compared to invasive techniques and to identify clinical predictors of response to balloon pulmonary angioplasty (BPA). Methods: In this prospective study, 17 patients with confirmed CTEPH underwent RV pressure assessment via TTE, RHC (Swan-Ganz catheterization), and PA within the same hospitalization period. BPA responders were defined based on clinical improvement and were compared to poor responders using pre- and post-treatment parameters. Results: A strong correlation was found between Swan-Ganz and PA-derived pressures (r = 0.96), with a slightly lower correlation between TTE and PA (r = 0.84), and the lowest between TTE and Swan-Ganz (r = 0.78). In the well-responding group, the 6 min walk distance (6MWD) increased by 60 ± 18 m, compared to 12 ± 10 m in poor responders (p < 0.01). NT-proBNP levels decreased by 40% in responders versus 10% in non-responders (p < 0.01). TAPSE improved significantly in responders (from 16.0 ± 2.0 mm to 19.5 ± 2.5 mm, p < 0.01), while RV basal diameter decreased by 15% (p < 0.05). No significant echocardiographic improvement was observed in poor responders. Conclusions: TTE provides a reliable estimate of RV pressure in CTEPH when standardized protocols are followed. NT-proBNP levels and RV size may serve as useful predictors of BPA response. Full article

Background/Objectives: Interstitial lung disease (ILD) is frequently complicated by pulmonary hypertension (PH), which is associated with reduced exercise capacity and poor prognosis. Early and accurate non-invasive detection of PH remains a clinical challenge. This study evaluated whether combining quantitative CT analysis of lung fibrosis with cardiac MRI-derived measures of right ventricular (RV) function improves the diagnostic accuracy of PH in patients with ILD. Methods: We retrospectively analyzed 72 ILD patients who underwent chest CT, cardiac MRI, and right heart catheterization (RHC). Lung fibrosis was quantified using a Gaussian Histogram Normalized Correlation (GHNC) software that computed the proportions of diseased lung, ground-glass opacity (GGO), honeycombing, reticulation, consolidation, and emphysema. MRI was used to assess RV end-systolic volume (RVESV), ejection fraction, and RV longitudinal strain. PH was defined as a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg and pulmonary vascular resistance ≥ 3 Wood units on RHC. Results: Compared to patients without PH, those with PH (n = 21) showed significantly reduced RV strain (−13.4 ± 5.1% vs. −16.4 ± 5.2%, p = 0.026) and elevated RVESV (74.2 ± 18.3 mL vs. 59.5 ± 14.2 mL, p = 0.003). CT-derived indices also differed significantly: diseased lung area (56.4 ± 17.2% vs. 38.4 ± 12.5%, p < 0.001), GGO (11.8 ± 3.6% vs. 8.65 ± 4.3%, p = 0.005), and honeycombing (17.7 ± 4.9% vs. 12.8 ± 6.4%, p = 0.0027) were all more prominent in the PH group. In receiver operating characteristic curve analysis, diseased lung area demonstrated an area under the curve of 0.778 for detecting PH. This increased to 0.847 with the addition of RVESV, and further to 0.854 when RV strain was included. Combined models showed significant improvement in risk reclassification: net reclassification improvement was 0.700 (p = 0.002) with RVESV and 0.684 (p = 0.004) with RV strain; corresponding IDI values were 0.0887 (p = 0.03) and 0.1222 (p = 0.01), respectively. Conclusions: Combining CT-based fibrosis quantification with cardiac MRI-derived RV functional assessment enhances the non-invasive diagnosis of PH in ILD patients. This integrated imaging approach significantly improves diagnostic precision and may facilitate earlier, more targeted interventions in the management of ILD-associated PH. Full article

The right heart–pulmonary circulation unit (RH-PCU) constitutes an integrated anatomo-functional system characterized by high-volume blood flow, low intravascular pressure, and minimal pulmonary vascular resistance. The RH-PCU dysfunction is a challenge for clinicians, as it can result from numerous pathological conditions, each with different clinical presentations. The pathophysiological changes underlying the hemodynamic alterations in the pressure and volume affecting the right ventricle can lead the patient to present with the primary symptom: dyspnea. We review the clinical presentation, the laboratory test, and the role of multimodality imaging in the evaluation of the disfunction of the RHPCU, including echocardiography, stress echocardiography, computed tomography, magnetic resonance imaging, nuclear imaging, and invasive pressure measurement through catheterization. We therefore aimed to describe the various diagnostic options available to clinicians, evaluating their effectiveness and limitations of use. Full article