Pulmonary Artery Research Articles

Anesthetic management of coronary artery reconstruction in a patient with myocardial ischemia caused by an anomalous origin of the right coronary artery running between the great vessels: a case report.

Coronary artery origin anomalies, though often incidentally detected, can lead to sudden death. Comprehensive perioperative management is essential. We report a case of an anomalous right coronary artery (RCA) arising from the left main coronary artery (LMCA) and coursing between the aorta and pulmonary artery, discovered after myocardial infarction, in which intraoperative management ensured successful coronary reconstruction. A 49-year-old woman presented with chest pain and ST segment elevation. Coronary angiography revealed an anomalous RCA demonstrating compressive ischemia by the aorta and pulmonary artery. Preoperatively, blood pressure was stabilized with an isosorbide dinitrate patch. Under cardiopulmonary bypass, the RCA was transected and reanastomosed to its physiological aortic position. Intraoperatively, nicorandil was administered to suppress vascular smooth muscle contraction, while five-lead ECG, transesophageal echocardiography, and operative ultrasound monitoring enabled early detection of ischemia and prevented hypertension. Postoperative ventricular premature contractions resolved by the next day, with uneventful recovery. Targeted pharmacologic blood pressure control and multimodal monitoring are vital for safe perioperative outcomes in anomalous coronary artery origin cases.

An Alternative Technique of Performing the Bidirectional Superior Cavopulmonary Connection without Cardiopulmonary Bypass.

There is an increasing trend of performing the bidirectional superior cavopulmonary anastomosis or bidirectional Glenn (BDG) without cardiopulmonary bypass (CPB). However, there are concerns of neurological dysfunction due to cerebral congestion and systemic oxygen desaturation due to clamping the superior vena cava and pulmonary artery, respectively, required for the off-pump anastomosis. In this report we present our alternative technique of performing the procedure without CPB in 13 patients. This technique may be a useful alternative in a select anatomic group of patients.

Exploring the Economic Burden of Pulmonary Arterial Hypertension and Its Relation to Disease Severity and Treatment Escalation: A Systematic Literature Review.

Pulmonary arterial hypertension (PAH) is a highly progressive disease characterized by luminal narrowing of the pulmonary arteries, leading to progressive dyspnoea and restricted functional capacity, which can ultimately result in right ventricular failure and death. Treatment goals include improving functional class and walk distance, recovering right ventricular function, halting disease progression, and improving survival. PAH carries a high mortality rate, and treatment escalation is a common feature of disease management. Due to the substantial impact of PAH, a high economic burden has been observed. A systematic literature review (SLR) was carried out to assess the contemporary economic burden of PAH, including the impact of disease severity and treatment escalation. An electronic database search was conducted and supplemented with a hand search of health technology assessments and conference materials. Studies were included from 2012 to 2024, with no restrictions on geographical location. The inclusion criteria specified that adult patients with PAH (≥ 18 years) and only English language studies were captured. The review included 148 studies and evaluations, 110 of which were observational studies, 14 were economic evaluations, and 24 were health technology assessments. The studies identified reported on several healthcare resource utilization (HCRU) outcomes including hospitalization, PAH-related hospitalization, inpatient visits, emergency department (ED) visits, intensive care unit (ICU) visits, and outpatient visits. Cost data were also reported, including total costs and costs for each of the above-mentioned types of HCRU, as well as specific costs such as pharmacy and drug costs. The results provide an overview of the high economic burden caused by PAH, indicating that the economic burden increases with increasing severity; reported mean monthly costs were as high as US $14,614 (cost converted to USD 2024) for the highest severity group. These data also demonstrated the impact of PAH-specific therapies in reducing HCRU, with efficacious treatment shifting management from an inpatient to outpatient setting (i.e., reduced inpatient admissions and length of stay). Further, while treatment escalation resulted in increased pharmacy costs, this was offset by a reduction in HCRU, including hospitalizations and ED visits. Timely diagnosis was also associated with reduced economic burden, as patients with a longer delay prior to diagnosis reported a higher mean number of monthly hospitalizations, ICU stays, and ED visits. Functional limitation is a common feature of PAH disease progression and can severely impact a patient's ability to work. This SLR identified few studies that investigated such outcomes as well as broader indirect costs, such as out-of-pocket costs and productivity loss. This study highlights the considerable economic burden associated with PAH, which is particularly evident for HCRU, and the importance of effective disease management in reducing this burden. Additionally, these findings demonstrate the economic value of treatment escalation and suggest higher drug costs can potentially be offset through improved patient outcomes and associated reductions in HCRU.

Lanreotide protects against LPS-induced inflammation in endothelial cells and mouse lungs.

Somatostatin is expressed in various tissues - including the hypothalamus - and strongly suppresses Growth Hormone levels to maintain homeostasis. Synthetic somatostatin analogs are currently used in clinics to treat neuroendocrine tumors and acromegaly. An emerging body of evidence suggests that those synthetic peptides exert anti-inflammatory activities. The present study examines the effect of Lanreotide (LAN) on Lipopolysaccharide (LPS)-triggered injury in endothelial cells and mice. Our findings indicate that LAN effectively mitigates LPS-induced endothelial hyperpermeability, inflammation, and reactive oxygen species (ROS) generation in bovine pulmonary artery endothelial cells (BPAEC) and human lung microvascular endothelial cells (HULEC-5a). A murine model of LPS-induced acute lung injury was also utilized, to examine the effects of LAN in lung edema and inflammation. Our observations suggest that LAN suppresses LPS-induced myosin light chain 2 (MLC2), Cofilin, extracellular signal-regulated kinase 1/2 (ERK1/2), STAT1, STAT3, P38 activation; and lung edema. In conclusion, and based on the aforementioned observations, it is suggested that LAN counteracts experimental LPS-induced injury in endothelial cells and mice.

Balloon Pulmonary Angioplasty in Heterozygous RNF213 p.Arg4810Lys Variant Carriers Diagnosed With Chronic Thromboembolic Pulmonary Hypertension.

The heterozygous ring finger protein 213 (RNF213) p.Arg4810Lys variant has been identified in patients with chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed to clarify the influence of the RNF213 p.Arg4810Lys variant on the response to balloon pulmonary angioplasty (BPA) in patients with CTEPH. We retrospectively analyzed 93 patients with CTEPH who underwent BPA by analyzing the RNF213 p.Arg4810Lys variant. Clinical parameters and hemodynamics following BPA were compared between RNF213 p.Arg4810Lys variant carriers and noncarriers, along with BPA-related complications. Among 93 patients, 7 (8%) were heterozygous RNF213 p.Arg4810Lys variant carriers and 86 (92%) were noncarriers. Both groups showed significant improvements in mean pulmonary artery pressure and pulmonary vascular resistance following BPA. However, the 6-minute walk distance, symptoms, cardiac index, and right ventricular function did not significantly improve in heterozygous RNF213 variant carriers, whereas noncarriers showed notable improvements. Group differences in mean change from baseline to follow-up were significant in cardiac index (0.4 L/min per m2 [95% CI, 0.1-0.8]; P=0.019), 6-minute walk distance (70 m [95% CI, 6-135]; P=0.036), and right ventricular ejection fraction (9% [95% CI, 5-12]; P<0.001), all favoring noncarriers. Of the 515 BPA procedures, complications were significantly higher in variant carriers than in noncarriers (25% versus 8%; P<0.001). Multivariate logistic regression analysis indicated a significant association between the RNF213 p.Arg4810Lys variant and BPA complications (adjusted odds ratio, 7.0 [95% CI, 1.1-44.4]; P=0.038). Heterozygous RNF213 p.Arg4810Lys variant carriers exhibited a poor response to BPA, suggesting that the RNF213 p.Arg4810Lys variant could be a risk factor for BPA complications.

Clinical analysis and follow-up study of transcatheter closure of secundum atrial septal defect in infants

Objective: To evaluate the clinical characteristics and prognosis of transcatheter closure of secundum atrial septal defect (ASD) in infants. Methods: A retrospective analysis was conducted on 83 cases with secundum ASD who underwent transcatheter closure at the Women and Children's Hospital of Qingdao University from January 2010 to December 2021. The clinical data included general information, pre- and post-operative echocardiography and electrocardiography, surgery records, and follow-up outcomes. The children who underwent successful occlusion were divided into two groups based on the presence or absence of pulmonary hypertension: the normal group and the pulmonary hypertension group. Differences between the two groups in terms of age, weight, right ventricular end-diastolic diameter, maximum ASD diameter, occluder size, and pulmonary-to-systemic flow ratio (Qp/Qs) were compared. Intergroup comparisons were performed using the Wilcoxon test. Results: Among the 83 infants with secundum ASD, 12 were males and 71 were females, with the age of (10.8±1.6) years. There were 29 cases (35%) with weight-for-age below the median level (less than the 25th percentile), 19 cases (23%) with recurrent or refractory pulmonary infections, and 40 cases (48%) with pulmonary hypertension. In this study, 81 cases (97%) successfully completed the procedure, while 2 cases failed due to soft defect margins. Postoperative arrhythmias occurred in 12 cases, of which 11 were transient arrhythmia; one case of atrioventricular block returned to normal rhythm after surgical removal of the occluder. The 81 infants who underwent successful occlusion were divided into normal group (41 cases) and pulmonary hypertension group (40 cases). The pulmonary artery systolic pressure, maximum diameter of ASD, the size of the occluder, and the cardiothoracic ratio were higher in the pulmonary hypertension group compared to those in the normal group, while the Qp/Qs value was lower (all P<0.05). Follow-up results indicated that infants with ASD who underwent transcatheter closure had no new arrhythmias, residual shunts, or intervention-related mortality. Within one year postoperatively, all infants exhibited normalization of right ventricular dimensions and pulmonary artery pressure. During a 3-year follow-up, all 29 cases that originally had a weight-for-age below the median level exhibited catch-up growth, reaching above the median level. Conclusions: Transcatheter closure for secundum ASD in infants is safe and feasible. Follow-up indicates that early transcatheter closure may be necessary for larger ASD diameters combined with pulmonary hypertension, with significant clinical improvement postoperatively.

Low-dose and low-contrast computed tomography pulmonary angiography in pediatric with pulmonary embolism: a prospective study

ObjectiveWe evaluated the feasibility of reducing contrast agent and radiation dose in pediatric computed tomography pulmonary angiography (CTPA) while ensuring image quality.Materials and methodsIn this prospective study, two readers assessed the computed tomography (CT) image quality (using a 5-point scale (1: undiagnosable and 5: excellent) and objective evaluation criteria (measuring CT and noise values of the left atrium and pulmonary trunk) of 116 patients who underwent pulmonary artery computed tomography angiography (CTA) from January 2023 to April 2024. independent sample t-test and Chi-square test were used to analyze and evaluate group differences.ResultFifty-eight participants were enrolled in the study group (mean age, 6.86 years ± 2.74, 30 males) and fifty-eight participants were enrolled in the control group (mean age, 6.71 years ± 2.59, 22 males). The radiation dose was significantly decreased in the study group (study group, 3.01 ± 0.24 mGy, control group 3.77 ± 1.06 mGy, p < 0.001). Overall quality was higher in control group, but displaying ability of pulmonary artery trunk and branch was higher in study group (p < 0.001).ConclusionThis study proved that a low-dose, low-contrast CTPA strategy could reduce radiation dosage by 50% and contrast agent by 20% while maintaining a satisfying image quality.

The transcription factor PITX1 cooperates with super-enhancers to regulate the expression of DUSP4 and inhibit pyroptosis in pulmonary artery smooth muscle cells

BackgroundPulmonary hypertension (PH) is a highly fatal pathophysiological syndrome. The group 1 pulmonary arterial hypertension (PAH) is characterized by acute pulmonary vasoconstriction and chronic vascular remodeling caused by hyperplasia and hypertrophy of pulmonary artery smooth muscle cells (PASMCs) and chronic inflammation. Pyroptosis is an inflammatory mode of cell death that is regulated by super-enhancers (SEs) and occurs in the setting of tumors and cardiovascular diseases. However, whether SEs are involved in the pathological process of pyroptosis in PAH and the specific mechanism involved remain unclear.MethodsHere, we identified the SE target gene DUSP4 via ChIP-seq with an anti-H3K27ac antibody, and bioinformatics predictions revealed that the transcription factor PITX1 can bind to the promoter and SE sequences of DUSP4. The AAV5 vector was used to deliver shRNAs targeting PITX1 and DUSP4 to PASMCs.ResultsPITX1 overexpression reversed the increase in right ventricular systolic pressure and pulmonary vascular remodeling, restored the PAAT/PAVTI ratio in hypoxic pulmonary hypertension (HPH, Group 3 PH) and SuHx PAH (Group 1 PAH) mice, and suppressed pyroptosis in pulmonary vascular cells. However, knockdown of DUSP4 counteracted the effects of PITX1 overexpression. Similar results were obtained in cultured PASMCs. In addition, treatment with the SE inhibitors JQ1 and iBET decreased the transcription of DUSP4 and increased the expression of hypoxia-induced pyroptosis proteins in PASMCs.ConclusionWe confirmed that PITX1 can promote DUSP4 expression by binding to the DUSP4 promoter and SE to reduce pyroptosis in hypoxic PASMCs, providing new insights into the role of SEs and pyroptosis in pulmonary vascular remodeling and a theoretical basis for the treatment of PAH and related diseases.

Clinical analysis of 15 cases of severe Mycoplasma pneumoniae pneumonia complicated by cardiac thrombosis in children

Objective: To investigate the clinical manifestations, therapeutic strategies and prognostic outcomes in pediatric patients with severe Mycoplasma pneumoniae pneumonia (SMPP) complicated by cardiac thrombosis. Methods: This case series study retrospectively analyzed 15 pediatric patients with SMPP complicated by cardiac thrombosis. The patients was recruited from the Department of Pediatric Respiratory Medicine at Shandong Provincial Hospital Affiliated to Shandong First Medical University between July 2018 and January 2025. Comprehensive clinical data and follow-up information were collected. Results: Among the 15 children, 10 were male and 5 were female, and the age of onset was 8.0 (6.3, 10.0) years. All 15 children presented with fever and cough, while additional symptoms included dyspnea in 7 cases, chest pain in 6 cases, hemoptysis in 3 cases, and chest tightness in 1 case. The white blood cell count was 11.7 (9.5, 15.9)×109/L, C-reactive protein was 31.6 (17.5, 64.8) mg/L and lactate dehydrogenase was 548.2 (410.4, 768.3) U/L. A total of 14 children underwent testing for the Mycoplasma pneumoniae drug resistance genes 2063A>G and 2064A>G, of which 13 tested positive. The plasma D-dimer levels of 15 children were 8.77 (7.23, 12.50) mg/L, all of which were higher than normal. Among the 15 children, 5 had decreased activity of anticoagulant proteins (protein C, protein S, antithrombin Ⅲ), and 8 tested positive for antiphospholipid antibodies. Chest CT scans of all 15 children showed pulmonary consolidation and (or) atelectasis, with pleural effusion present in 12 cases. In the 15 children, thrombosis was detected at 14.0 (11.0, 18.0) days after the onset of illness. The locations of cardiac thrombosis included the right ventricle in 9 cases, the right atrium in 5 cases, and the left atrium in 1 case. Additionally, 10 cases had pulmonary vascular embolism, comprising 9 cases of pulmonary artery thrombosis and 1 case of pulmonary vein thrombosis. After anticoagulant treatment, cardiac thrombi disappeared in 10 children. Five children who did not show improvement with anticoagulation underwent surgical thrombectomy. In the follow-up of 15 children, lung imaging basically returned to normal, with no major hemorrhagic events or other adverse events. Conclusions: In children with Mycoplasma pneumoniae pneumonia, the presence of clinical symptoms such as shortness of breath, chest pain and hemoptysis, along with elevated plasma D-dimer levels, should raise suspicion for the possibility of cardiac thrombosis. SMPP complicated by cardiac thrombosis, prognosis is good following anticoagulation or surgical treatment.

Outcomes of systemic thrombolysis with reteplase in high-risk acute pulmonary embolism.

High-risk pulmonary embolism (PE) is known as the presence of hemodynamic instability and has a high mortality rate, which necessitates immediate reperfusion treatment. According to current PE treatment guidelines, alteplase is the approved and preferred agent for systemic thrombolysis. Herein, we present the efficacy and safety of systemic thrombolysis with reteplase in patients with high-riskPE. The present retrospective observational study includes 30 patients with high-risk acute PE undergoing systemic thrombolysis with reteplase. All patients were in obstructive shock. The thrombolytic regimen consisted of two bolus doses of 10 U reteplase, given 30 min apart, followed by intravenous heparin. All patients had elevated cardiac troponin levels. D-dimer levels were elevated in 28 (93.3 %) patients. Systolic blood pressure increased after thrombolysis with reteplase, and the dyspnea, tachypnea, and coughing improved in all patients. Mean SPO2 was 83.6±6.96 % before treatment, which had risen to 95.7±1.29 % after thrombolysis (p=0.059). Right ventricular dilatation was seen in all patients at presentation, which improved by 96.6 % after treatment and systolic pulmonary artery pressure decreased significantly in all patients at discharge. None of the patients needed to repeat thrombolytic therapy. There were no major hemorrhagic complications or death after thrombolysis, while 5 (16.7 %) patients experienced minor self-limiting bleeding. Reteplase, as two bolus injections of 10 U given 30 min apart, is an effective thrombolytic regimen in treating acute high-risk PE with a low risk of major hemorrhagic complications.

Right ventricular strain predicts outcome in patients receiving sacubitril/valsartan: A sub-analysis of DISCOVER-ARNI.

Angiotensin receptor/neprilysin inhibitors (ARNI) have emerged as a pivotal medical treatment considerably improving the clinical outcome of patients with heart failure and reduced ejection fraction (HFrEF). Identifying individuals who stand to benefit the most from ARNI could markedly enhance patient management strategies. The aim of this sub-analysis of DISCOVER-ARNI register was to evaluate the prospective prognostic significance of speckle tracking echocardiography (STE) parameters in patients undergoing ARNI therapy. DISCOVER-ARNI multicentre Italian register retrospectively enrolled 341 patients with HFrEF referred for treatment with ARNI. These patients underwent clinical, biohumuoral, and echocardiographic assessment at baseline. Subsequently, among those with available right ventricular STE data, a prospective long-term follow-up was conducted by telephone interview or on-site visits. The primary endpoint encompassed a composite of outcomes, including all-cause or cardiovascular mortality, heart failure hospitalization, heart transplantation, and left ventricular assist device (LVAD) implantation. Overall, 136 HFrEF patients were included in this sub-analysis (mean age 65±10years, 82% male). The mean follow-up was 40±18months, during which 32 patients reached the primary endpoint (14 deaths of which 10 due to cardiovascular reasons, 22 hospitalization, 3 heart transplantation, 1 LVAD implantation). Baseline assessment revealed that patients with events had higher LV volumes and EF (LV end-diastolic volume 212±65 vs.174±57mL, P=0.002; LV end-systolic volume 156±52 vs. 122±49mL, P=0.001; LV EF=26±5 vs. 29±5mL, P=0.006, respectively), lower but preserved tricuspid annular plane systolic excursion (TAPSE, 17±3 vs.19±3, P=0.008), and higher systolic pulmonary artery pressures (38±11 vs. 31±8mmHg, P=0.001) compared to those who did not experience events. LV, left atrial (LA), and free wall right ventricular longitudinal strain (fwRVLS) were reduced in patients with events (-7±2 vs. -8±2%, P=0.002; 11±3 vs. 15±7%, P=0.001 and -15±5 vs. -22±5%, P=0.007, respectively). Employing Cox proportional hazard model including LVEF, TAPSE, RVFAC, LV strain, LA strain, and fwRVLS, the latest emerged as the sole independent predictor of the combined endpoint (hazard ratio=1.15 [1.05;1.26], P=0.002). Receiver operating characteristic (ROC) curves determined that fwRVLS=-20% was the optimal cut-off for predicting the combined endpoint (area under curve [AUC]=0.70). This threshold was used for constructing Kaplan-Meier survival curves, demonstrating effective risk stratification of fwRVLS over long-term follow-up for the primary endpoint. fwRVLS by STE holds promise as a valuable parameter to assess response to ARNI therapy in terms of overall survival, heart failure hospitalizations, heart transplantation, or LVAD implantation.

Identification of serum C4BPA as a potential diagnostic marker of right ventricular remodelling via proteomic analysis.

Right ventricular (RV) remodelling, a progressive condition characterized by maladaptive cardiac structural and functional changes, primarily results from prolonged pressure overload in patients with pulmonary hypertension (PH). Accurate, universal and easy-to-use biomarkers for assessing the severity of RV remodelling are lacking. This study aimed to identify serum proteins as potential biomarkers of RV remodelling using high-throughput proteomic analysis-based screening. Sprague-Dawley rats were subjected to sham surgery (control group) or pulmonary artery banding for 4weeks with 2.3-mm diameter and 1.8-mm diameter rubber rings to induce mild and severe RV modelling, respectively. Serum proteomic profiling revealed 170 differentially expressed serum proteins (DEPs) among the three groups, and three DEPs gradually increased with worsening RV remodelling. Among the three DEPs, C4b-binding protein alpha chain (C4BPA) exhibited the highest upregulation in the severe group (6.93 vs. 16.5ng/mL, P<0.001), and linear regression analysis revealed a negative correlation between serum C4BPA levels and tricuspid annular plane systolic excursion (TAPSE) in rats [beta=-0.78, 95% confidence interval (CI) -14.5 to -7.11, P<0.001]. The diagnostic power of C4BPA was further validated in 127 patients (34 with adaptive RV pressure overload, 36 with maladaptive RV pressure overload, 32 with left ventricular hypertrophy and 25 controls). Control and left ventricular hypertrophy patients exhibited lower serum C4BPA levels than the two RV groups, and serum C4BPA levels were higher in patients with maladaptive RV than in those with adaptive RV (754 vs. 524pg/mL, P<0.001). Linear regression analysis revealed a negative correlation between serum C4BPA levels and TAPSE in PH patients. The predictive power of C4BPA for maladaptive RV function in PH patients, indicated by receiver operating characteristic analysis (cut-off value 573pg/mL, area under the curve 0.792), was as good as that of B-type natriuretic peptide (BNP). High serum C4BPA levels (≥573pg/mL) were associated with lower TAPSE/pulmonary arterial systolic pressure ratios (P<0.001) and higher BNP levels (P<0.001). Serum C4BPA may represent a novel diagnostic biomarker for RV pathological remodelling associated with RV maladaptation in PH patients.

Altered maturation and activation state of circulating monocytes is associated with their enhanced recruitment in pulmonary arterial hypertension

BackgroundIt is well-established that patients with pulmonary arterial hypertension (PAH) exhibit increased recruitment of circulating monocytes to their pulmonary arteries. However, it remains unclear whether these monocytes have intrinsic abnormalities that contribute to their recruitment and to PAH pathogenesis. This study aimed to characterize the gene expression profiles of circulating classical, intermediate, and non-classical monocytes and assess their maturation trajectory in patients with idiopathic (I) PAH compared to control subjects. Additionally, it sought to explore the relationship between the observed IPAH abnormalities and deficiencies in bone morphogenetic receptor 2 (BMPR2), the most frequently mutated gene in PAH, and to assess adhesion and transendothelial migration, key processes in monocyte infiltration of pulmonary arteries.MethodsDifferentially expressed genes and maturation trajectories of circulating monocytes from patients with IPAH vs. control subjects were compared using single cell RNA sequencing (scRNAseq), followed by FACS analysis. Observations from IPAH and control cells were related to reduced BMPR2 using a THP1 monocyte cell line with BMPR2 reduced by siRNA as well as induced pluripotent stem cell (iPSC) derived monocytes (iMono) from hereditary (H) PAH patients with a BMPR2 mutation and monocytes from mice with Bmpr2 deleted (MON-Bmpr2−/−).ResultsClassical IPAH monocytes have decreased CD14 mRNA leading to a deviation in their maturation trajectory and early terminal fate, which is not rescued by cytokine treatment. Monocytes that evade early cell death show elevated STAT1, PPDPF and HLA-B, and an interferon (IFN) signature indicative of an altered activation state. A strong link between decreased BMPR2 and CD14 was observed in THP1 cells and in HPAH iMono with a BMPR2 mutation associated with STAT1 and IFN related genes, and in monocytes from MON-Bmpr2−/− mice. Increased adhesion to iPSC-derived endothelial cells (iECs) in HPAH-BMPR2 mutant iMono was associated with elevated ICAM1 expression. Enhanced transendothelial migration of these cells was associated with the reduction in endothelial VE-cadherin (CDH5).ConclusionsIPAH monocytes exhibit an altered activation state associated with reduced BMPR2 and CD14, along with elevated STAT1-IFN expression. These changes are linked to intrinsic functional abnormalities that contribute to the monocytes’ increased propensity to invade the pulmonary circulation.

Double Valve Replacement in the Pregnant Patient: A Successful Case and Brief Literature Review

Cardiovascular disease is the leading cause of maternal mortality accounting for &gt;25% of maternal deaths. The risk of cardiac surgery is very high with poor neonatal outcomes. The authors would like to report a patient who was a 19 year old lady with 27 weeks gestation who had severe mitral stenosis (mitral valve area-0.9cm2, pulmonary artery systolic pressure (PaSP-90mm Hg)), moderate mitral regurgitation, moderate aortic stenosis with severe aortic regurgitation, (modified world health organization class (m WHO) class IV) who successfully underwent double valve replacement under cardiopulmonary bypass (CPB) and went on to deliver a healthy but premature male baby. Cardiac surgery especially cardiopulmonary bypass is high risk procedure when needed to be performed must be done in second trimester of prgnancy. The authors have divided the discussion into 4 parts for ease of discussion i.e Anesthetic, surgical, intensivist and perfusion related considerations. Anesthetic considerations included maintaining uteroplacental perfusion, higher cardiac index other considerations of pregnancy including low systemic vascular resistance, difficult airway, lateral tilt. Surgical considerations include minimizing cross clamp and bypass time to avoid fetal complications. Post operatively intensivist considerations include hemodynamic monitoring with regular point of care ultrasound (POCUS) for fluid management. Vasopressor and fluid management in our case was tailored to the Flo Trac (cardiac output) monitoring system and POCUS assessments. The patient had a successful outcome however delivered a premature baby at 33 weeks gestation. The incidence of premature delivery is about 50% as per literature review. The literature around cardiac bypass in pregnancy is limited and we hope that our case adds to further research on this topic.

The influence of the interaction of right ventricle and pulmonary artery system on the development of adverse outcomes in acute decompensation of chronic heart failure

The influence of the interaction of right ventricle and pulmonary artery system on the development of adverse outcomes in acute decompensation of chronic heart failure

Unexpected Finding of an Inflammatory Myofibroblastic Tumor of the Heart Causing Pulmonary Artery Obstruction in an Asymptomatic Toddler.

Inflammatory myofibroblastic tumor (IMT) of the heart is a very rare tumor, constituting less than 5% of primary heart tumors. This case report describes the unexpected finding of IMT of the heart causing pulmonary artery obstruction in an asymptomatic toddler.

Relationship Between Remote, Ambulatory Pulmonary Artery Pressures, and All-Cause Mortality in Patients With Chronic Heart Failure.

Hemodynamically guided management of patients with chronic heart failure (HF), using a remote, ambulatory pulmonary artery (PA) pressure monitor, has been shown to reduce mortality and morbidity. These improved outcomes were associated with a reduction in PA pressure. However, several pivotal questions remain unanswered: do systolic, diastolic, or mean PA pressures each predict all-cause mortality? Do PA pressures predict mortality across the ejection fraction (EF) spectrum? Do increases or decreases in PA pressure over time predict increases or decreases in mortality? Retrospective analyses of data from CHAMPION (CardioMEMS Heart Sensor Allows Monitoring of Pressure to Improve Outcomes in NYHA Class III Heart Failure Patients; n=550), GUIDE-HF (Hemodynamic-GUIDEed management of Heart Failure; n=2358), US PAS (CardioMEMS HF System Post Approval Study; n=1200), and MEMS-HF (CardioMEMS Monitoring Study for Heart Failure; n=234) were performed, including all enrolled patients regardless of treatment assignments (Total N=4342). PA systolic, PA diastolic, and PA mean pressures were examined in patients with HF and reduced EF (<50%, n=2562) and preserved EF (≥50%, n=1454). Baseline pressure (averaged over 14 days after implantation) and change in pressure (increase/decrease/no change) from baseline to 6 months (averaged over 14 days just before the 6-month time point) were related to all-cause mortality over a 2-year follow-up period. Baseline PA diastolic, independent of other covariates, was a significant predictor of mortality (hazard ratio, 1.04 [95% CI, 1.03-1.05]; P<0.0001). Change in PA diastolic from baseline to 6 months (assessed as a continuous variable) was an independent predictor of mortality after 6 months (landmark analysis; hazard ratio, 1.03 [95% CI, 1.01-1.05]; P=0.0042). Change in PA diastolic from baseline to 6 months(assessed as a categorical variable) decrease or increase of >2 mm Hg compared with no change predicted a 14.7% decrease and 26.7% increase in mortality, respectively (P=0.0237). PA systolic and PA mean pressures in both HF with reduced EF and HF with preserved EF patients, for both baseline and change from baseline to 6 months, were also predictive of all-cause mortality. Baseline PAP (systolic, diastolic, and mean) and change in PAP (systolic, diastolic, and mean) from baseline to 6 months were independent predictors of 2-year mortality in patients with chronic HF in both preserved and reduced EF. URL: https://www.clinicaltrials.gov; Unique identifier: CHAMPION NCT00531661. URL: https://www.clinicaltrials.gov; Unique identifier: GUIDE-HF NCT03387813. URL: https://www.clinicaltrials.gov; Unique identifier: USPAS NCT02279888. URL: https://www.clinicaltrials.gov; Unique identifier: MEMS-HF NCT02693691.

Pulmonary arterial hypertension in disorders of porto-caval hemodynamics. The role of ultrasound diagnostics

Portal hypertension is a complex syndrome that effects the systemic circulation. Some authors name pulmonary hypertension with portal hypertension such as portopulmonary hypertension. According to foreign registries, the prognosis of patients with portopulmonary hypertension is one of the most unfavorable. Ultrasound diagnostic methods are ones of keis to confirm portopulmonary hypertension, while echocardiography detects high pressure in the pulmonary artery, liver ultrasound with vascular doppler reveals changes in portocaval blood flow. Aim. To analyze the clinical, functional and hemodynamic status of patients with pulmonary arterial hypertension with portocaval hemodynamic disorders. To demonstrate the significance of abdominal ultrasonic diagnostics in determining the genesis of pulmonary hypertension. Material and methods. The study enrolls 14 patients with portocaval disorders. There were 5 patients with cirrhotic portal hypertension, 6 patients with Abernethy malformation and 3 patients with surgical portocaval shunts among them. All patients had a complex examination that included laboratory tests, echocardiography, abdominal ultrasound with Doppler ultrasonography and the right heart catheterization. Results. Among 14 patients, portocaval hemodynamic abnormalities were detected more often in women (73.3%). The mean age was 53.2±10.9 years. We knew about 5 patients (10%) with diagnosis of liver cirrhosis, three patients (5%) had got surgical portocaval shunts, 6 (10%) patients had Abernethy malformation. According to echocardiography, right atrial area was 25.3±2.3 cm2, pulmonary artery systolic pressure was 96±10.8 mm Hg. According to right heart catheterization data the median mean pulmonary arterial pressure was 59±10 mm Hg, SpO2 95.7±2.22%, cardiac output 4.6±0.9 L/min, the median pulmonary vascular resistance was 943.5±87.3 din sec/cm-5. The median 6-minute walking distance was 429.5±70.4 meters, which was corresponding to WHO functional class II, the median Borg dyspnea index was 4±1.7. Conclusions. All patients with pulmonary hypertension are shown to exclude portocaval shunts. The study of clinical features, functional and hemodynamic status of these patients will help to find the optimal treatment and tactical solutions.

Echocardiography-Based Pulmonary Artery Pulsatility Index Correlates with Outcomes in Patients with Acute Pulmonary Embolism

Objectives: The pulmonary artery pulsatility index (PAPI) is a novel hemodynamic parameter that reflects right ventricular (RV) function. PAPI was shown to be useful in predicting outcomes following left ventricular assist device (LVAD) implantation, acute RV infarction, and in patients with chronic RV failure. The standard method to estimate PAPI is during right heart catheterization (RHC); however, echocardiography-based PAPI was also shown to be accurate. In the current study, we evaluated the ability of echocardiography-based PAPI to predict outcomes of patients with acute pulmonary embolism (PE). Methods: A total of 177 patients (mean age 67 ± 15, 54.1% male) with acute PE were included in the study. PAPI was calculated based on measurements from standard transthoracic echocardiography. Results: 27% of patients needed oxygen support, 5.6% were on mechanical ventilation, and 7.3% were on inotropic support. The 30-day mortality rate in the whole cohort was 8.3%. Lower PAPI measurements were associated with increased 30-day mortality (p &lt; 0.05), a higher rate of RV failure (p &lt; 0.001), and the need for inotropic support (p &lt; 0.05). There was no association between PAPI and the need for oxygen support (p = 0.59), mechanical ventilation (0.06), or length of stay (LOS) (p = 0.414). PAPI was superior to tricuspid annular plane systolic excursion (TAPSE) in predicting mortality and RV failure. Conclusions: Echocardiography-derived PAPI is feasible and superior over TAPSE in predicting RV failure and mortality among patients with acute PE.

Prevalence and clinical significance of anti-neutrophil cytoplasmic antibodies in rheumatoid arthritis-associated interstitial lung disease

BackgroundPatients with rheumatoid arthritis (RA) or rheumatoid arthritis-associated interstitial lung disease (RA-ILD) are commonly positive for antineutrophil cytoplasmic antibodies (ANCAs). The causal relationship between RA-ILD and ANCAs and the role of ANCAs in RA-ILD remain unclear. The purpose of this study was to estimate the prevalence of ANCAs in RA-ILD patients and to investigate the clinical characteristics and outcomes of ANCA-positive RA-ILD patients.MethodsData from 104 RA-ILD patients with available ANCA results at our centre from March 2010 to June 2024 were retrospectively reviewed. ANCA positivity was defined as the presence of any one or a combination of perinuclear ANCAs (P-ANCAs), cytoplasmic ANCAs (C-ANCAs), anti-MPO or anti-PR-3. Clinical data from each patient’s initial diagnosis were collected and analysed. The clinical characteristics and survival of the ANCA-positive and ANCA-negative RA-ILD groups were compared.ResultsThirty-three out of the 104 (31.7%) RA-ILD patients were positive for ANCAs. The percentages of patients positive for P-ANCAs, MPO-ANCAs, C-ANCAs, and PR3-ANCAs were 27.9% (29/104), 1.9% (2/104), 1.9% (2/104), and 1.0% (1/104), respectively. Compared with ANCA-negative RA-ILD patients, a greater proportion of ANCA-positive RA-ILD patients had respiratory symptoms, serum autoantibody (ANA) positivity, poorer baseline pulmonary function, and acute exacerbations of ILD (AE-ILD). The usual interstitial pneumonia (UIP) pattern (57.6%) was the most common chest high-resolution computed tomography (HRCT) pattern observed. ANCA-positive RA-ILD patients were more likely to have traction bronchiectasis (P = 0.029), honeycombing (P < 0.001), and oddly shaped cysts (P = 0.020) than were ANCA-negative RA-ILD patients. Univariate Cox analysis revealed that P-ANCA positivity [hazard ratio (HR) = 2.24, 95% confidence interval (CI): 0.91–5.52; P = 0.046] is a trend of survival association in RA-ILD, but this was not confirmed in the multivariate analysis. Multivariate Cox analyses revealed that a history of smoking (HR = 2.53, 95% CI: 1.10–5.83; P = 0.030) and a systolic pulmonary artery pressure (SPAP) ≥ 37 mmHg (HR = 10.24, 95% CI: 4.07–25.77; P < 0.001) were independently associated with shorter survival in RA-ILD patients.ConclusionsThe prevalence of ANCAs in patients with RA-ILD is high, and ANCA testing could be considered in the diagnostic workup for RA-ILD. Oddly shaped cysts with or without a UIP pattern may be a characteristic chest imaging manifestation of ANCA-positive RA-ILD. More attention should be given to RA-ILD patients who have elevated SPAP.