Case report: the 'vanished' left pulmonary artery.

Background: We report a case of isolated ductal origin of pulmonary artery (DOPA) diagnosed in an asymptomatic newborn. The primary aim of this case is to highlight the need to investigate for DOPA in patients diagnosed with an 'absent branch pulmonary artery'. Case summary: Our patient was an asymptomatic newborn infant, with normal intracardiac anatomy. He was initially diagnosed post-natally with 'absent left pulmonary artery' (LPA), though the LPA was seen in antenatal scans. He underwent angiography and was re-diagnosed with bilateral arterial ducts, with ductal origin of the LPA from the left arterial duct. The LPA was salvaged by first stenting the left arterial duct on Day 11 of life, with subsequent surgery to connect the LPA to the main pulmonary artery at 4.5 months old. The patient had an uneventful recovery after the surgery. Discussion: Ductal origin of pulmonary artery is a rare vascular anomaly characterized by continuity of the left or right pulmonary artery (PA) with the distal end of the arterial duct, and discontinuity with the main PA. It is commonly misdiagnosed as pulmonary artery agenesis when the patent arterial duct constricts, with cessation of blood flow into the affected pulmonary artery. A high index of suspicion is necessary for diagnosis of DOPA. Once diagnosed, this lesion is clearly amenable to intervention, with benefits from unifocalization, to prevent late onset pulmonary hypertension or cardiac failure.

A Rare Case of Rectal Carcinoma With Pulmonary Artery Thrombosis.

Cancer is a well-recognized risk factor for thromboembolic events and thromboembolism. This case report presents the rare coexistence of rectal carcinoma and pulmonary artery thrombosis in a female patient. A 44-year-old female presented with complaints of abdominal pain, vomiting, and obstipation. She had recently been diagnosed with rectal carcinoma. She had not undergone any invasive procedures in the past. Given the possibility of intestinal obstruction, an exploratory laparotomy was performed, followed by retrocolic gastrojejunostomy with Roux-en-Y anastomosis with a feeding JT tube insertion. On postoperative day six, she experienced symptoms of shortness of breath, tachypnoea, and hypoxia. At that time, a two-dimensional echocardiography showed normal findings but a CT pulmonary angiogram (CTPA) revealed a thrombus in the right upper lobe pulmonary artery. There was no evidence of lung metastasis on CTPA. This report highlights the challenging scenario associated with rectal carcinoma and concomitant pulmonary artery thrombosis.

Multi-scaled temporal modeling of cardiovascular disease progression: An illustration of proximal arteries in pulmonary hypertension.

The progression of cardiovascular disease is intricately influenced by a complex interplay between physiological pathways, biochemical processes, and physical mechanisms. This study aimed to develop an in-silico physics-based approach to comprehensively model the multifaceted vascular pathophysiological adaptations. This approach focused on capturing the progression of proximal pulmonary arterial hypertension, which is significantly associated with the irreversible degradation of arterial walls and compensatory stress-induced growth and remodeling. This study incorporated critical characteristics related to the distinct time scales for the deformation, thus reflecting the impact of mean pressure on artery growth and tissue damage. The in-silico simulation of the progression of pulmonary hypertension was realized based on computational code combined with the finite element method (FEM) for the simulation of disease progression. The parametric studies further explored the consequences of these irreversible processes. This computational modeling approach may advance our understanding of pulmonary hypertension and its progression.

Single-cell sequencing analysis confirms the association of ANRIL with the increased smooth muscle cell proliferation and migration gene signatures in pulmonary artery hypertension in silico.

PURPOSE: Smooth muscle cell (SMC) dysregulation is part of the pathological basis of pulmonary artery hypertension (PAH). We aimed to explore the heterogeneity of SMCs in PAH. METHODS: The profile GSE210248 was obtained from NCBI Gene Expression Omnibus, containing the scRNA-seq data of pulmonary arteries (PA) from three patients with PAH and three healthy donors. After quality control, normalization, and dimension reduction, cell clustering analysis was performed. Differential expression analysis and functional enrichment analysis were carried out successively in smooth muscle cells (SMCs). The enrichment scores of cell cycle and cell migration gene sets in SMCs were calculated. Then, the Spearman correlation coefficients between antisense non-coding RNA in the INK4 locus (ANRIL) expression and two gene sets were computed. RESULTS: Eight cell clusters were identified in PA from samples. The proportion of SMCs was increased in PAH samples. SMCs were divided into five subclusters with diverse biological functions. Muscle contraction-related SMC1 was decreased, while extracellular matrix organization-related SMC2, immune and inflammatory response-related SMC4 and SMC5 were increased in PAH samples compared with healthy donors. The enrichment scores of cell cycle and cell migration gene sets in SMCs were higher in PAH samples than in donors. ANRIL was down-regulated significantly in PAH samples and was negatively related to the scores of two gene sets. CONCLUSION: SMCs exhibited significant heterogeneity in PAH. The altered abilities of SMC proliferation and migration in PAH were associated with ANRIL expression.

Transient interruption of contrast on CT pulmonary angiography: effect of mid-inspiratory vs. end-inspiratory respiration command.

PURPOSE: To investigate the effects of mid-inspiratory respiration commands and other factors on transient interruption of contrast (TIC) incidence on CT pulmonary angiography. METHODS: In this retrospective study, 824 patients (mean age, 66.1 ± 15.3 years; 342 males) who had undergone CT pulmonary angiography between January 2021 and February 2023 were included. Among them, 545 and 279 patients were scanned at end- and mid-inspiratory levels, respectively. By placing a circular region of interest, CT attenuation of the main pulmonary artery (CTMPA) was recorded. Associations between several factors, including patient age, body weight, sex, respiratory command vs. TIC and severe TIC incidence (defined as CTMPA < 200 and 150 HU, respectively), were assessed using logistic regression analyses with stepwise regression selection based on Akaike's information criterion. RESULTS: Mid-inspiratory respiration command, in addition to patient age and lighter body weight, had negative association with the incidence of TIC. Only patient age, lighter body weight, female sex, and larger cardiothoracic ratio were negatively associated with severe TIC incidence. Mid-inspiratory respiration commands helped reduce TIC incidence among patients aged < 65 years (p = 0.039) and those with body weight ≥ 75 kg (p = 0.005) who were at high TIC risk. CONCLUSION: Changing the respiratory command from end- to mid-inspiratory levels, as well as patient age and body weight, was significantly associated with TIC incidence.

Non-canonical IKB kinases regulate YAP/TAZ and pathological vascular remodeling behaviors in pulmonary artery smooth muscle cells.

Pulmonary arterial hypertension (PAH) causes pulmonary vascular remodeling, increasing pulmonary vascular resistance (PVR) and leading to right heart failure and death. Matrix stiffening early in the disease promotes remodeling in pulmonary artery smooth muscle cells (PASMCs), contributing to PAH pathogenesis. Our research identified YAP and TAZ as key drivers of the mechanobiological feedback loop in PASMCs, suggesting targeting them could mitigate remodeling. However, YAP/TAZ are ubiquitously expressed and carry out diverse functions, necessitating a cell-specific approach. Our previous work demonstrated that targeting non-canonical IKB kinase TBK1 reduced YAP/TAZ activation in human lung fibroblasts. Here, we investigate non-canonical IKB kinases TBK1 and IKKε in pulmonary hypertension (PH) and their potential to modulate PASMC pathogenic remodeling by regulating YAP/TAZ. We show that TBK1 and IKKε are activated in PASMCs in a rat PH model. Inflammatory cytokines, elevated in PAH, activate these kinases in human PASMCs. Inhibiting TBK1/IKKε expression/activity significantly reduces PAH-associated PASMC remodeling, with longer-lasting effects on YAP/TAZ than treprostinil, an approved PAH therapy. These results show that non-canonical IKB kinases regulate YAP/TAZ in PASMCs and may offer a novel approach for reducing vascular remodeling in PAH.

Impact of Age-Related Change in Caval Flow Ratio on Hepatic Flow Distribution in the Fontan Circulation.

BACKGROUND: The Fontan operation is a palliative technique for patients born with single ventricle heart disease. The superior vena cava (SVC), inferior vena cava (IVC), and hepatic veins are connected to the pulmonary arteries in a total cavopulmonary connection by an extracardiac conduit or a lateral tunnel connection. A balanced hepatic flow distribution (HFD) to both lungs is essential to prevent pulmonary arteriovenous malformations and cyanosis. HFD is highly dependent on the local hemodynamics. The effect of age-related changes in caval inflows on HFD was evaluated using cardiac magnetic resonance data and patient-specific computational fluid dynamics modeling. METHODS: SVC and IVC flow from 414 patients with Fontan were collected to establish a relationship between SVC:IVC flow ratio and age. Computational fluid dynamics modeling was performed in 60 (30 extracardiac and 30 lateral tunnel) patient models to quantify the HFD that corresponded to patient ages of 3, 8, and 15 years, respectively. RESULTS: SVC:IVC flow ratio inverted at ≈8 years of age, indicating a clear shift to lower body flow predominance. Our data showed that variation of HFD in response to age-related changes in caval inflows (SVC:IVC, 2, 1, and 0.5 corresponded to ages, 3, 8, and 15+, respectively) was not significant for extracardiac but statistically significant for lateral tunnel cohorts. For all 3 caval inflow ratios, a positive correlation existed between the IVC flow distribution to both the lungs and the HFD. However, as the SVC:IVC ratio changed from 2 to 0.5 (age, 3-15+) years, the correlation's strength decreased from 0.87 to 0.64, due to potential flow perturbation as IVC flow momentum increased. CONCLUSIONS: Our analysis provided quantitative insights into the impact of the changing caval inflows on Fontan's long-term HFD, highlighting the importance of SVC:IVC variations over time on Fontan's long-term hemodynamics. These findings broaden our understanding of Fontan hemodynamics and patient outcomes.

Transcatheter recanalisation for absent intra-pericardial pulmonary arteries.

Unilateral absence of intra-pericardial pulmonary artery is a rare congenital malformation. If untreated, it can lead to morbidity and mortality in adulthood. Early intervention and restoration of physiologic pulmonary blood flow is necessary. Transcatheter stenting as initial intervention has been rarely reported. We present transcatheter recanalisation and stenting of the obliterated ductus in two newborns with unilateral absence of intra-pericardial pulmonary artery with cross-sectional imaging, procedural details, angiography, and follow up to surgical repair. We believe that such procedure promotes ipsilateral pulmonary vasculature growth to facilitate unifocalization surgery at a later age.

Cost-effectiveness of remote haemodynamic monitoring by an implantable pulmonary artery pressure monitoring sensor (CardioMEMS-HF system) in chronic heart failure in the Netherlands.

AIMS: Remote haemodynamic monitoring with an implantable pulmonary artery (PA) sensor has been shown to reduce heart failure (HF) hospitalizations and improve quality of life. Cost-effectiveness analyses studying the value of remote haemodynamic monitoring in a European healthcare system with a contemporary standard care group are lacking. METHODS AND RESULTS: A Markov model was developed to estimate the cost-effectiveness of PA-guided therapy compared to the standard of care based upon patient-level data of the MONITOR-HF trial performed in the Netherlands in patients with chronic HF (New York Heart Association class III and at least one previous HF hospitalization). Cost-effectiveness was measured as the incremental cost per quality-adjusted life year (QALY) gained from the Dutch societal perspective with a lifetime horizon which encompasses a wide variety of costs including costs of hospitalizations, monitoring time, telephone contacts, laboratory assessments, and drug changes in both treatment groups. In the base-case analysis, PA-guided therapy increased costs compared to standard of care by €12 121. The QALYs per patient for PA-guided therapy and standard of care was 4.07 and 3.481, respectively, reflecting a gain of 0.58 QALYs. The resulting incremental cost-effectiveness ratio was €20 753 per QALY, which is below the Dutch willingness-to-pay threshold of €50 000 per QALY gained for HF. CONCLUSIONS: The current cost-effectiveness study suggests that remote haemodynamic monitoring with PA-guided therapy on top of standard care is likely to be cost-effective for patients with symptomatic moderate-to-severe HF in the Netherlands.

Invasive haemodynamics predict outcomes in paediatric pulmonary artery hypertension.

BACKGROUND: Invasive haemodynamics are often performed for initiating and guiding pulmonary artery hypertension therapy. Little is known about the predictive value of invasive haemodynamic indices for long-term outcomes in children with pulmonary artery hypertension. We aimed to evaluate invasive haemodynamic data to help predict outcomes in paediatric pulmonary artery hypertension. METHODS: Patients with pulmonary artery hypertension who underwent cardiac catheterisation (2006-2019) at a single centre were included. Invasive haemodynamic data from the first cardiac catheterisation and clinical outcomes were reviewed. The combined adverse outcome was defined as pericardial effusion (due to right ventricle failure), creation of a shunt for pulmonary artery hypertension (atrial septal defect or reverse Pott's shunt), lung transplant, or death. RESULTS: Among 46 patients with a median [interquartile range (IQR)] age of 13.2 [4.1-44.7] months, 76% had CHD. Median mean pulmonary artery pressure was 37 [28-52] mmHg and indexed pulmonary vascular resistance was 6.2 [3.6-10] Woods units × m2. Median pulmonary artery pulsatility index was 4.0 [3.0-4.7] and right ventricular stroke work index was 915 [715-1734] mmHg mL/m2. After a median follow-up of 2.4 years, nine patients had a combined adverse outcome (two had a pericardial effusion, one underwent atrial level shunt, one underwent reverse Pott's shunt, and six died). Patients with an adverse outcome had higher systolic and mean pulmonary artery pressures, higher diastolic and transpulmonary pressure gradients, higher indexed pulmonary vascular resistance, higher pulmonary artery elastance, and higher right ventricular stroke work index (p < 0.05 each). CONCLUSION: Invasive haemodynamics (especially mean pulmonary artery pressure and diastolic pressure gradient) obtained at first cardiac catheterisation in children with pulmonary artery hypertension predicts outcomes.

Frequency of Pulmonary Hypertension and Its Associated Risk Factors in End-Stage Renal Disease (ESRD) Patients on Maintenance Hemodialysis.

Introduction Pulmonary hypertension (PH) is a recognized complication in patients with end-stage renal disease (ESRD undergoing maintenance hemodialysis (MHD). PH is commonly found in patients with chronic kidney disease (CKD) and ESRD. PH is associated with increased morbidity and mortality in patients with CKD. Methodology This cross-sectional study aimed to assess the prevalence of PH and its associated risk factors in MHD patients. A total of 220 ESRD patients on MHD patients at The Kidney Center, Karachi, Pakistan, aged 18-70 were included. Patients with chronic obstructive lung disease, valvular heart disease, and obstructive sleep apnea were excluded, as these conditions can be responsible for PH. PH was evaluated by echocardiography (ECHO), which was performed by a cardiologist. Results The mean age was 50.65 ± 14.4 years, with 131 (59.5%) males and 89 (40.5%) females. The average duration on hemodialysis was 5.3 ± 2.8 years. Hypertension (89.5%) and ischemic heart disease (24.1%) were prominent comorbidities. Hypertensive nephropathy (42.7%) was the leading cause of ESRD. Left ventricular hypertrophy was mild in most cases (85.5%), whereas regional wall motion abnormality (RWMA) was common (67.3%). The average pulmonary artery pressure was 35.2 ± 15.3 mmHg. Out of 220 patients, 109 patients (49.8%) of them had mild PH, nine patients (4.1%) had severe PH, and 72 patients (32.7%) had moderate PH. Associations between PH and various factors were examined. RWMA, left ventricular hypertrophy, and left ventricular ejection fraction were significantly associated with PH (p < 0.001). Serum calcium and albumin levels were also associated with PH severity (p < 0.05). Other demographic and laboratory parameters did not show a significant association. Conclusion This study highlights the prevalence of PH in MHD patients and identifies associated risk factors. Understanding these associations can aid in better managing PH in ESRD patients.

An Alternative Endovascular Technique for Treatment of Pulmonary Arteriovenous Malformation: Microballoon-occluded Transcatheter Embolization Using n-butyl-2-cyanoacrylate.

PURPOSE: To investigate the safety and efficacy of microballoon-occluded transcatheter embolization using n-butyl-2-cyanoacrylate (NBCA) in patients with a single pulmonary arteriovenous malformation (PAVM). METHODS: From November 2017 to November 2020, this retrospective study included 38 previously untreated patients with a single PAVM who underwent microballoon-occluded transcatheter embolization using NBCA. All 38 patients had follow-up that included simple chest radiography and contrast-enhanced chest computed tomography (CT). RESULTS: A microballoon was successfully placed in a feeding artery of the PAVM to control the delivery of the NBCA cast in all 38 patients, with complete embolization of sacs and the feeding artery achieved in all cases. The mean diameters of the feeding artery, sac, and draining vein were 3.9 ± 0.9 mm, 7.5 ± 2.6 mm, and 4.6 ± 1.3 mm, respectively. A fixed 1:2 NBCA/Lipiodol ratio was used, and the mean amount of embolic mixture per patient was 1.4 mL (range 0.6-2.2 mL). There were no complications related to microballoon adhesion and non-target embolization of the systemic circulation. Follow-up CT in all 38 patients with a mean delay of 34.5 ± 8.8 months (range 20.7-56.5 months) showed no continued perfusion of the PAVM. CONCLUSION: In our hands, microballoon-occluded n-butyl-2-cyanoacrylate embolization seemed to be safe and appeared to be clinically effective in patients with simple and complex types of single PAVM. Therefore, the present technique has the potential to become a standard treatment for a single PAVM.

Transcatheter Pulmonary Valve Replacement With the Harmony Valve in Patients Who Do Not Meet Recommended Oversizing Criteria on the Screening Perimeter Plot.

BACKGROUND: Anatomic selection for Harmony valve implant is determined with the aid of a screening report and perimeter plot (PP) that depicts the perimeter-derived radius along the right ventricular outflow tract (RVOT) and projects device oversizing. The PP provides an estimation of suitability for implant, but its sensitivity as a screening method is unknown. This study was performed to describe anatomic features and outcomes in patients who underwent Harmony TPV25 implant despite a PP that predicted inadequate oversizing. METHODS: We reviewed RVOT anatomic features and measurements in patients who underwent transcatheter pulmonary valve replacement with the Harmony TPV25 device despite a PP that predicted inadequate oversizing. RESULTS: This study included 22 patients. There were no unsuccessful implants or adverse valve-related events. Anatomic features varied, but all patients fit into 1 of 3 anatomic types characterized by differences in RVOT dimensions. Type 1 patients (n=9) had a long RVOT with a choke point and a wide main pulmonary artery. Type 2 patients (n=6) had a short RVOT that was pyramidal in shape, with no choke point, and extensive main pulmonary artery lengthening/expansion during systole. Type 3 patients (n=7) had a short, bulbous main pulmonary artery with a choke point and an open pulmonary artery bifurcation. CONCLUSIONS: Transcatheter pulmonary valve replacement with the Harmony valve is feasible in some patients whose PP fit analysis predicts inadequate oversizing. All cases in this series fit into 1 of 3 anatomic patterns, which are not identified in the screening report. Implanters must review cases individually to assess the feasibility of the implant.

Pirfenidone and nintedanib attenuates pulmonary artery endothelial and smooth muscle cells transformations induced by IL-11.

Idiopathic pulmonary fibrosis (IPF) associated to pulmonary hypertension (PH) portends a poor prognosis, characterized by lung parenchyma fibrosis and pulmonary artery remodeling. Serum and parenchyma levels of Interleukin 11 (IL-11) are elevated in IPF-PH patients and contributes to pulmonary artery remodeling and PH. However, the effect of current approved therapies against IPF in pulmonary artery remodeling induced by IL-11 is unknown. The aim of this study is to analyze the effects of nintedanib and pirfenidone on pulmonary artery endothelial and smooth muscle cell remodeling induced by IL-11 in vitro. Our results show that nintedanib (NTD) and pirfenidone (PFD) ameliorates endothelial to mesenchymal transition (EnMT), pulmonary artery smooth muscle cell to myofibroblast-like transformation and pulmonary remodeling in precision lung cut slices. This study provided also evidence of the inhibitory effect of PFD and NTD on IL-11-induced endothelial and muscle cells proliferation and senescence. The inhibitory effect of these drugs on monocyte arrest and angiogenesis was also studied. Finally, we observed that IL-11 induced canonical signal transducer and activator of transcription 3 (STAT3) and non-canonical mitogen-activated protein kinase 1/2 (ERK1/2) phosphorylation, but, PFD and NTD only inhibited ERK1/2 phosphorylation. Therefore, this study provided evidence of the inhibitory effect of NTD and PFD on markers of pulmonary artery remodeling induced by IL-11.

Massive left pulmonary artery aneurysm with a co-existing patent ductus arteriosus in a five-year-old female child: A case report.

Pulmonary Artery Aneur ysm (PAA), whether congenital or acquired, is a rare diagnostic find ing com pare d to aor tic aneur ysms. There have been fe w cases where PA As were documented as a complication of untreated Patent Ductus Ar teriosus (PDA) due to long-standing Pulmonary Arterial H ypertension (PAH). However, it is quite rare for a case of PAA to be reported with co-existing PDA without PAH. This report highlights a case of a five -year-old girl who was presented with palpitations, easy fatigability, fever, c yanos is, and vomiting. A Chest X-ray s howed mo derate cardiomega ly. A PDA of 6 mm was diagnosed on Transthoracic E chocardiog rap hy ( TTE ) and a large cavity con necte d with LPA raised suspicion of a possible LPA aneur ysm. A Chest CT scan confirm ed the diagnosis of a saccular aneurysm, originating from the distal part of the main Left Pulmonary Artery (LPA) just proximal to the point of bifurcation into lobar branches, measuring 7.5x6.5 cm. During surgery, the aneurysm was opened, emptied with suction and closed without resecting the aneur ysmal walls. The patient had an uneventful post-op course and is doing well during regular interval follow up visits.

Giant pulmonary artery aneurysm associated with SARS-CoV-2 infection and Actinomyces odontolyticus sepsis: A case report.

Giant septic pulmonary artery aneurysms (PAAs) are rare but important entities, with few cases having been reported worldwide. Early diagnosis and prompt treatment are crucial in the management of such cases. We report a 56-year-old female patient presenting with fatigue, nausea and vomiting who was first diagnosed with diabetic ketoacidosis (DKA) and developed life-threatening giant infectious PAA secondary to SARS-CoV-2 infection and Actinomyces odontolyticus sepsis. The patient did not develop any specific symptoms, and enhanced computed tomography (CT) revealed a massive PAA of 5.6 × 4.9 cm in size at the left pulmonary hilar with normal pulmonary artery (PA) pressures. After multidisciplinary discussion and after considering the critical condition accompanied by sepsis increased the risk of surgery, endovascular treatment was the first therapy of choice for the patient; nevertheless, the patient ultimately opted for hospice care. This case report aims to raise awareness of PAAs, which are rare but potentially fatal complications of infectious diseases such as COVID-19 pneumonia and Actinomyces odontolyticus sepsis.

Infectious Pulmonary Artery Pseudoaneurysm Secondary to a Lung Abscess Treated With Pulmonary Artery Coil Embolization: A Case Report.

Pulmonary artery pseudoaneurysms (PAPs) are uncommon, yet they frequently result in hemoptysis and are associated with a poor prognosis. We report a case of an 87-year-old male patient. Initially, he was admitted to a previous hospital, and diagnosed with a lung abscess in the left lower lobe. On the second hospital day, he developed hemoptysis. A contrast-enhanced chest computed tomography (CT) identified an infectious pulmonary artery pseudoaneurysm. On the ninth hospital day, pulmonary artery coil embolization was successfully performed, significantly improving the patient's condition.