Severe Pulmonary Hypertension Research Articles

Sex-based differences in candidacy for transcatheter tricuspid valve intervention.

Women have a higher prevalence of tricuspid regurgitation (TR) and present at more advanced stages as compared with men. Given the high operative mortality associated with tricuspid valve (TV) surgery, transcatheter tricuspid valve interventions (TTVI) have emerged as a promising treatment option. We explored sex-based differences among patients with significant TR who would be expected to be eligible for TTVI. Between March 2021-2022, 12,677 unique adult patients underwent a transthoracic echocardiogram at our tertiary care institution. Clinical and echocardiographic data were collected for patients with more than moderate TR. The 2021 European Society of Cardiology valve guidelines were used to retrospectively define sub-populations who would have been eligible for TTVI, TV surgery, or medical therapy. Patients were grouped by sex and compared using t-tests, Wilcoxon rank-sum, Pearson chi-square, and Cox regression for survival analysis. Of 569 patients, 52% (296/569) were female. Men had a higher prevalence of left ventricular dysfunction (p < 0.001), mitral regurgitation (p = 0.023), and signs of heart failure (New York Heart Association stage III (p = 0.031)). Women had more isolated TR (p = 0.020) and TR due to severe pulmonary hypertension (p < 0.001). Most patients (74.6% of women, 76.9% of men) were precluded from both transcatheter and surgical intervention due to advanced disease. 10.8% of women and 9.2% of men would have qualified for TTVI (p = 0.511). The majority of patients with significant TR presenting to a tertiary care center are not eligible for TTVI. Sex is not a predictor of eligibility for TTVI among patients with significant TR.

Neuraxial anesthesia for patients with severe pulmonary arterial hypertension undergoing urgent open abdominal surgeries: two case reports

BackgroundThere is no consensus regarding the choice of anesthetic method for patients with pulmonary hypertension (PH). We report two cases in which neuraxial anesthesia was safely performed without general anesthesia during open abdominal surgery in patients with severe PH.Case presentationCase 1: A 59-year-old woman had an atrial septal defect and a huge abdominal tumor with a mean pulmonary arterial pressure (PAP) of 39 mmHg and pulmonary vascular resistance (PVR) of 3.5 Wood units. Case 2: A 23-year-old woman who had hereditary pulmonary artery hypertension (mean PAP, 65 mmHg; PVR, 16.45 Wood units). Both patients underwent open abdominal surgery under neuraxial anesthesia without circulatory collapse with intraoperative administration of vasoconstrictors.ConclusionAlthough anesthetic care must be personalized depending on the pathology and severity of PH, neuraxial anesthesia may be an option for patients with severe PH undergoing abdominal surgery.

An interdisciplinary consensus approach to pulmonary hypertension in developmental lung disease.

It is increasingly recognised that diverse genetic respiratory disorders present as severe pulmonary hypertension (PH) in the neonate and young infant, but many controversies and uncertainties persist regarding optimal strategies for diagnosis and management to maximise long-term outcomes. To better define the nature of PH in the setting of developmental lung disease (DEVLD), in addition to the common diagnoses of bronchopulmonary dysplasia and congenital diaphragmatic hernia, we established a multidisciplinary group of expert clinicians from stakeholder paediatric specialties to highlight current challenges and recommendations for clinical approaches, as well as counselling and support of families. In this review, we characterise clinical features of infants with DEVLD/DEVLD-PH and identify decision-making challenges including genetic evaluations, the role of lung biopsies, the use of imaging modalities and treatment approaches. The importance of working with team members from multiple disciplines, enhancing communication and providing sufficient counselling services for families is emphasised to create an interdisciplinary consensus.

Prevalence and risk factors for ventilator-associated pneumonia after cardiac surgery: a systematic review and meta-analysis.

There is currently significant variation in the reported incidence of ventilator-associated pneumonia (VAP) among postoperative cardiac patients. Moreover, the risk factors for VAP in postoperative cardiac patients remain controversial. This study aims to assess the incidence and risk factors of VAP in postoperative cardiac patients to provide a basis for further prevention and treatment of VAP. We systematically reviewed PubMed, EMBASE, and Cochrane Library databases to select studies that met the inclusion criteria until November 2023. Fifteen studies involving 10,478 patients who underwent cardiac surgery were selected for meta-analysis. The incidence of VAP in postoperative cardiac patients was 10%. The preoperative risk factors for VAP after cardiac surgery included age >70 years, chronic obstructive pulmonary disease (COPD), peripheral vascular disease, renal disease, and severe pulmonary hypertension. Furthermore, the perioperative risk factors for VAP after cardiac surgery included emergency surgery, redo surgery, airway instrumentation, gastric aspiration, reintubation, mechanical ventilation duration >3 days, intra-aortic balloon, New York Heart Association >3, American Society of Anesthesiologists >3, need for transfusion during surgery, and ascending aortic surgery. The incidence of VAP after cardiac surgery was found to be 10%, and the comprehensive risk factors for VAP were identified, emphasizing the critical need for targeted interventions, including optimization of preoperative health and refined surgical protocols, to effectively reduce the occurrence of VAP in postoperative cardiac patients.

Predictive Value of Esophageal Deviation Index for Clinical Outcomes of Patients With Left-Sided Congenital Diaphragmatic Hernia

PurposeThis study aimed to evaluate the prognostic value of postnatal esophageal deviation index (EDI) measured within the first 24 h of life for predicting mortality and morbidity in neonates with left-sided congenital diaphragmatic hernia (L-CDH). MethodThis retrospective study analyzed clinical data from 133 neonates with L-CDH admitted to Guangzhou Women and Children's Medical Center between January 2016 and January 2024. Patients were categorized into two groups based on outcomes: survivors (n = 108) and non-survivors (n = 27). Risk factors for mortality were identified using both univariate and multivariate analyses. A receiver operating characteristic (ROC) curve was utilized to evaluate the predictive value of EDI for mortality in L-CDH patients. Subsequently, patients were divided into two groups: those with an EDI> 16.1% and those with an EDI≤16.1%. The relationship between EDI and both mortality and morbidity was analyzed using Kaplan–Meier analysis, chi-square test, Fisher's exact test, and multivariate analysis. ResultsEDI (adjusted OR: 0.822, 95% CI 0.723–0.935; P = 0.003) was identified as the independent predictor of mortality through both univariate and multivariate logistic regression analysis. The ROC curve demonstrated that the area under the curve (AUC) for predicting the mortality was 0.854 (95%CI: 0.782–0.930) for EDI, with an optimal cut-off value of 16.125%. The cumulative mortality rate through Day 200 was higher in patients with an EDI>16.1% (P＜0.001). Among the 133 neonates with L-CDH, 24.8% had an EDI>16.1%. This was associated with significantly worse CDH characteristics, including a high incidence of intrathoracic stomach and a high occurrence of high-risk defect sizes (type C/D), (P＜0.001), as well as more severe pulmonary hypertension (P＜0.001). An EDI>16.1% was associated with higher mortality and a greater need for ECMO support compared to an EDI≤16.1% (P＜0.001). ConclusionEDI within the first 24 h of life in patients with L-CDH is associated with increased mortality and the need for ECMO, particularly when EDI exceeds 16.1%. Level of EvidenceIII.

Prognostic Relevance of Tricuspid Annular Plane Systolic Excursion to Systolic Pulmonary Arterial Pressure Ratio and Its Association With Exercise Hemodynamics in Patients With Normal or Mildly Elevated Resting Pulmonary Arterial Pressure

BackgroundEchocardiographic TAPSE/sPAP ratio is a non-invasive surrogate for right ventricular-pulmonary arterial (RV-PA) coupling. It has been related to outcome in patients with moderate to severe pulmonary hypertension (PH). Research QuestionIs RV-PA coupling of prognostic relevance in patients with suspected PH but only normal or mildly elevated mean pulmonary arterial pressure (mPAP) and is it associated with impaired exercise capacity and exercise hemodynamics? Study Design and MethodsPatients with mPAP<25mmHg who underwent echocardiography and exercise right heart catheterization in our PH-clinic, were retrospectively analyzed. Mild PH was defined by mPAP 21-24mmHg and exercise PH (EPH) by a mPAP/cardiac-output (CO) slope >3mmHg/L/min. Multivariate analysis was performed to identify independent predictors for clinical worsening, defined by disease-related hospitalization, transplantation, or death. Results237 patients (female: N=155; median age: 64 (IQR 54-73) years, no PH N=147; mild PH N=90; EPH N=202) were included. During the observation time of 63 (IQR:29-104) months, 36 patients died and 126 events of clinical worsening occurred. TAPSE/sPAP ratio turned out as age- and sex independent predictor of mortality (HR 0.09 95% CI: (0.01 – 0.62) p=0.014) and clinical worsening (HR 0.05 95% CI: (0.35-0.78); p=0.002). TAPSE/sPAP was also significantly correlated to 6-minute walk distance (r= 0.33; p<0.001) and exercise hemodynamics (mPAP/CO slope: rρ=-0.56, p<0.001). The best multivariate predictive model for clinical worsening in this patient collective consisted of TAPSE/sPAP ratio (HR: 0.71 (0.53-0.95), p=0.021), NT-proBNP (HR 1.15 (0.99-1.34), p=0.065) and 6MWD (HR: 0.998 (0.995-1.00), p=0.042). InterpretationIn patients with suspected PH but normal or only mildly elevated resting mPAP, TAPSE/sPAP ratio is an independent predictor of outcome. In addition, it is significantly associated with exercise capacity and exercise hemodynamics and may be a helpful tool in the prediction of future clinical worsening of this patient population.

Scattered finding of Acute Severe aortic regurgitation in a child with Ventricular septal defect: floating non coronary cusp due to rupture fibrous strands

A 14 months kid was admitted for routine ventricular septal defect (VSD) closure surgery due to severe pulmonary hypertension (PHT) but day before surgery developed acute severe aortic regurgitation (AR). Per-operative findings highly suggested that this AR due to ruptures fibrous strands of aortic valve. He had no cardiac catheterization before surgery, so its due to turbulent VSD jet affected noncoronary cusp free floating. Refixation with multiple interrupted sutures make near normal anatomy with trival AR at 6 months follow up Echocardiography. Bangladesh Heart Journal 2024; 39(2): 177-178

Severe pulmonary arterial hypertension in congenital sideroblastic anemia from PUS1 mutation – a case report

BackgroundMyopathy, lactic acidosis and inherited sideroblastic anemia (MLASA) are a group of rare intriguing disorders with wider pathophysiological implications. One of the causes of MLASA is the mutation in PUS1 gene that encodes for pseudouridine synthase. This PUS1 mutation results in MLASA in which anemia and myopathy predominate. Severe pulmonary arterial hypertension has not been previously reported in patients with PUS1 gene mutation.Case reportA 17 year old girl with congenital sideroblastic anemia presented with worsening of breathlessness. Severe pulmonary artery hypertension was documented on investigations. A homozygous variant in exon 3 of gene PUS1,( chromosome 12:g.131932301 C > T c.430 C > T) was found on sanger sequencing.ConclusionWe document severe pulmonary arterial hypertension in a patient of congenital sideroblastic anemia from PUS1 gene. We hypothesis that cross talk with TGFb pathways might occur in PUS1 mutation, and that might cause severe PAH. This observation might have therapeutic implications.

Surgical Repair of Atrial Septal Defect with Severe Pulmonary Hypertension: A Case Report

This case report details the successful surgical repair of an atrial septal defect (ASD) in a 28-year-old female with severe pulmonary arterial hypertension (PAH), who presented with dyspnea, fatigue, and palpitations. The patient had a large secundum ASD (26 mm size) with bi-directional shunt and severe PAH, with a Pulmonary Arterial Systolic Pressure (PASP) of 107 mmHg and Mean Pulmonary Arterial Pressure (mPAP) of 57 mmHg, as revealed by echocardiography. The patient was optimized medically with diuretics, oxygen, and pulmonary vasodilators (Sildenafil &amp; Ambrisentan), and was deemed eligible for surgical closure of the ASD. A pericardial patch was used to close the defect, and the patient's postoperative recovery was uneventful. Follow-up echocardiography showed a significant reduction in mPAP and right ventricular size. Although surgical closure of an ASD with severe PAH is a high-risk procedure, it can be safely performed in carefully selected patients with appropriate preoperative evaluation, medical optimization, and careful surgical technique. Timely closure of the defect can result in a considerable improvement in the patient's hemodynamic status and quality of life. Cardiovasc j 2024; 16(2): 110-113

Clinical features of pulmonary hypertension in lymphangioleiomyomatosis

Objective: To describe the prevalence of lymphangioleiomyomatosis-pulmonary hypertension (LAM-PH), to explore the clinical features of patients with LAM-PH, and to evaluate the role of pulmonary artery optical coherence tomography (OCT) in the diagnosis of LAM-PH. Methods: Among 234 patients diagnosed with LAM in our center from June 2017 to August 2023, echocardiography was performed in 167 patients, 15 patients with PH indicated by echocardiography were selected as the LAM-PH group. From the remaining 152 patients, 32 patients were randomly selected as the control group. We compared the demographic data, clinical manifestations, pulmonary function, blood gas analysis, and serum vascular endothelial growth factor D (VEGF-D) levels between the two groups. We also evaluated the data from right heart catheterization in five patients and the images from optical coherence tomography of pulmonary arteries in two patients. Results: Echocardiography showed pulmonary hypertension in 15 patients (8.98%). Compared with the control group, LAM-PH group had a higher proportion of patients receiving oxygen therapy. Patients with PH had lower FEV1, FVC, FEV1/FVC, DLCO and higher serum VEGF-D levels compared with those without PH. Right heart catheterization was performed in five patients. Five patients had a pre-capillary pattern and three of these patients had severe pulmonary hypertension. Optical coherence tomography showed that there were no obvious abnormalities in the intima and lumen of the blood vessels in the two patients. Conclusions: The prevalence of PH in LAM is low. Patients in the LAM-PH group had more severe hypoxemia, worse lung function and higher serum VEGF-D levels than those in the control group. PH in LAM is mainly pre-capillary. Optical coherence tomography can evaluate the pulmonary artery vascular condition and help to identify the cause of pulmonary hypertension in LAM patients.

Double Intervention in A Patient with Large Patent Ductus Arteriosus and Moderate Upper Muscular Ventricular Septal Defect with Bidirectional Shunt with Severe Pulmonary Hypertension

Patent ductus arteriosus (PDA) and Ventricular septal defect (VSD) are commonly encountered problems and constitute about 40% of all congenital heart lesions. The association of these two conditions in a single patient is not very uncommon. Both these conditions can be treated by placing intracardiac devices. Double interventional closure of PDA and VSD was performed in a gravely ill infant at her 3 months and 5 months of age respectively, who was suffering from recurrent pneumonia and heart failure since birth. These were lifesaving procedure for that patient which led to writing this report. Cardiovasc j 2024; 16(2): 105-109

A Clinical Study on the Treatment of Adult Atrial Septal Defect Using Thoracoscopic-Assisted Right Vertical Infra-Axillary Thoracotomy, Total Thoracoscopic, and Median Sternotomy Approaches

Background: To compare the clinical outcomes of three surgical approaches for treating adult atrial septal defects (ASD): Thoracoscopic-assisted right vertical infra-axillary thoracotomy (TARVIAT) under central cardiopulmonary bypass (CPB), totally thoracoscopic (TT), and median sternotomy (MS) approaches, and to assess the feasibility and safety of the TARIAVT approach. Methods: This study reviewed 62 cases patients of repairing atrial septal defects via a TARVIAT and central extracorporeal circulation from 2019 to 2023. The patients included 22 males, aged between 18 and 59 years, with a mean age of 33.35 ± 10.97 years. The surgical indications were adult patients with moderate to severe tricuspid regurgitation and who were unsuitable for interventional closure of atrial septal defects. Exclusion criteria included patients diagnosed with severe pulmonary hypertension or Eisenmenger syndrome. Additionally, 67 patients who underwent TT approach repair of ASD and 72 patients who underwent MS approach repair of ASD were selected as the control groups. Operative time, CPB time, aortic clamping time, postoperative Intensive care unit (ICU) stay, postoperative mechanical ventilation time, 24-hour postoperative chest drainage, incision length, postoperative hospital stay, hospital costs, and postoperative complications were compared to assess statistical differences. Results: There were no deaths or major complications observed in any of the three groups. Statistically significant differences were found among the three groups in terms of extracorporeal circulation time, aortic clamping time, surgical time, 24-hour postoperative chest drainage, postoperative mechanical ventilation time, postoperative ICU stay, incision length, and postoperative hospital stay (p &lt; 0.05). The TT group exhibited longer CPB and aortic clamping times compared to the TARVIAT and MS groups, while demonstrating lower 24-hour postoperative chest drainage volumes compared to the TARIAVT and MS groups. Both the TARVIAT and TT groups showed shorter surgical times, postoperative mechanical ventilation times, postoperative ICU stay, incision lengths, and postoperative hospital. However, there were no statistically significant differences among the three groups regarding hospital costs, postoperative left ventricular ejection fraction (LVEF) values, and the incidence of postoperative complications (p &gt; 0.05). Conclusions: TARVIAT under central CPB is feasible and safe for adult patients. This approach presents lower invasiveness, shorter operative duration, and faster recovery compared to other methods. In conclusion, the TARVIAT approach for repairing adult ASD is both safe and effective, offering a viable surgical option for this condition.

Sildenafil-induced priapism in a dog : an unusual case report

BackgroundPriapism is defined as erection that lasts for more than 4 h without sexual stimulation. There are various causes of priapism, but there are no reports of sildenafil-induced priapism in dogs. In human medicine, there were no pre-marketing reports of priapism caused by sildenafil, but post-marketing surveillance has shown that it is rare. In cases of pulmonary hypertension in dogs, sildenafil is the first-line drug of choice for symptomatic relief.Case presentationAn 11-year-old neutered male Maltese dog that presented with tachypnea and cough was diagnosed with myxomatous mitral valve disease, American College of Veterinary Internal Medicine (ACVIM) stage C, and was treated medically. Eighteen months after the diagnosis, severe pulmonary hypertension occurred due to left heart disease. At 20 months postdiagnosis, pleural effusion occurred, and sildenafil (2 mg/kg twice daily) was added to the existing treatment. Two weeks later, the dyspnea recurred, confirming pleural fluid recurrence, and sildenafil was increased to 2 mg/kg thrice daily. One day later, the patient developed persistent erections and penile pain. Penile amputation and urethrostomy were recommended but were refused; therefore, analgesia and palliative care were provided. The patient died of acute dyspnea 22 months after the first presentation, with no specific priapism recurrence at the time of death.ConclusionTo the best of our knowledge, this is the first report of sildenafil-induced priapism in a dog with pulmonary hypertension.

Case Report: Anesthetic Management in Pregnant Woman with Severe Pulmonary Hypertension

Case Report: Anesthetic Management in Pregnant Woman with Severe Pulmonary Hypertension

Insulin-like Growth Factor-Binding Protein 2 in Severe Aortic Valve Stenosis and Pulmonary Hypertension: A Gender-Based Perspective.

Severe aortic valve stenosis (AS) and pulmonary hypertension (PH) are life-threatening cardiovascular conditions, necessitating early detection and intervention. Recent studies have explored the role of Insulin-like Growth Factor-Binding Protein 2 (IGF-BP2) in cardiovascular pathophysiology. Understanding its involvement may offer novel insights into disease mechanisms and therapeutic targets for these conditions. A total of 102 patients (46 female, 56 male) with severe AS undergoing a transcatheter aortic valve replacement (TAVR) in a single-center study were classified using echocardiography tests to determine systolic pulmonary artery pressure (sPAP) and the presence (sPAP ≥ 40 mmHg) or absence (sPAP < 40 mmHg) of PH. Additionally, serial laboratory determinations of IGF-BP2 before, and at 24 h, 96 h, and 3 months after intervention were conducted in all study participants. Considering the entire cohort, patients with PH had significant and continuously higher serum IGF-BP2 concentrations over time than patients without PH. After subdivision by sex, it could be demonstrated that the above-mentioned results were only verifiable in males, but not in females. In the male patients, baseline IGF-BP2 levels before the TAVR was an isolated risk factor for premature death after intervention and at 1, 3, and 5 years post-intervention. The same was valid for the combination of male and echocardiographically established PH patients. The predictive role of IGF-BP2 in severe AS and concurrent PH remains unknown. A more profound comprehension of IGF-BP2 mechanisms, particularly in males, could facilitate the earlier consideration of the TAVR as a more effective and successful treatment strategy.

A case of the effective inhalation of nitric oxide therapy for caused severe pulmonary hypertension with protamine neutralization of systemic heparinization during totally endoscopic minimally invasive cardiac surgery

A case of the effective inhalation of nitric oxide therapy for caused severe pulmonary hypertension with protamine neutralization of systemic heparinization during totally endoscopic minimally invasive cardiac surgery

Right atrial and right ventricular strain: prognostic value depends on the severity of tricuspid regurgitation.

Assessing right heart function is challenging, particularly when significant tricuspid regurgitation (TR) is present. Among available echocardiographic techniques for assessment, literatures suggests that strain imaging may be more reliable and less susceptible to loading conditions. Thus, we aimed to assess the validity of RA and RV strain relative to conventional metrics as well as their utility in predicting patient outcomes in TR. We studied 262 consecutive patients (mean age 74 ± 11.2 years, 53% male) who underwent same-day echocardiography and right heart catheterization between 2018 and 2023. We compared right heart strain to traditional metrics of RV function and subsequently correlated RA and RV strain to heart failure (HF)-related death or hospitalization, whichever came first. Over a mean follow-up of 34 ± 15 months, there were 103 deaths and HF hospitalizations. Both RA and RV strain were correlated with echocardiographic and invasive measures of right heart function. Across all patients, preserved RA strain was associated with lower risk of adverse outcomes (HR 0.763, 95% CI 0.618-0.943). Similarly, preserved RV strain was correlated with better outcomes, though this was only statistically significant in patients without severe TR or pulmonary hypertension (HR 2.450, 95% CI 1.244-4.825). Moreover, abnormal ratios of RV strain to pulmonary pressures and RV size were significantly correlated with adverse outcomes (p < 0.05 each). RA and RV strain are independently correlated with echocardiographic and invasive measures of cardiac function. Moreover, preserved RA and RV strain are likely associated with better clinical outcomes.

Pregnancy conditions and outcomes of Chinese women with mild, moderate and severe pulmonary arterial hypertension.

Pregnancy is normally contraindicated in pulmonary arterial hypertension (PAH). Thanks to medical advances, the prognosis for pregnancy in patients with PAH has improved. The aim of our study was to investigate pregnancy conditions and outcomes in patients with mild, moderate and severe PAH. We searched PubMed, Embase, CNKI, Wanfang and Weipu databases for studies published before May 2024. Data from 29 included studies from 1898 references were pooled and analyzed. We calculated the rates for each group as well as the risk ratio (RR) and 95% confidence interval (CI) between pairwise. There was no statistical difference in maternal and neonatal survival between the mild and moderate groups. Maternal survival in the mild, moderate and severe groups was 100.0%, 99.7% and 88.8%, respectively, and neonatal survival was 100.0%, 99.7% and 96.0%, respectively. The incidence of NYHA class III-IV, pregnancy loss, intensive care unit (ICU) admission, fetal growth restriction, and neonatal asphyxia was lowest in patients with mild PAH and highest in patients with severe PAH (P < 0.001). The incidence of vaginal deliveries and term pregnancies was highest in the mild group and lowest in the severe group (P < 0.001). In conclusion, pregnant women with mild PAH can safely deliver a newborn. Given similar survival rates but greater economic and medical burdens, caution is advised in the moderate group. Pregnancy in the severe group is considered contraindicated.

Living-donor lobar lung transplantation for pulmonary Langerhans cell histiocytosis complicated by extensive thrombi in central pulmonary arteries

BackgroundPulmonary Langerhans cell histiocytosis (PLCH) is a rare disorder characterized by the proliferation of Langerhans cells along the small airways, which causes nodular and cystic changes in the lung parenchyma. Lung transplantation can be a life-saving option for patients with severe respiratory failure or pulmonary hypertension. Herein, we present a case of successful lung transplantation in a patient with PLCH who developed unusually large thrombi in the central pulmonary artery.Case presentationA 47-year-old woman with 16-year history of PLCH with rapidly developing respiratory failure was admitted to our hospital for the evaluation of a lung transplant. Enhanced computed tomography revealed large thrombi in dilated central pulmonary arteries. Right heart catheterization revealed severe pulmonary hypertension, with a mean pulmonary artery pressure of 48 mmHg. The thrombi shrank markedly after 3 months of anticoagulation therapy. However, the respiratory status of the patient did not improve. We performed bilateral living-donor lobar lung transplantation with thrombectomy under extracorporeal membrane oxygenation for the remaining thrombi in the main pulmonary arteries. The dilated main pulmonary arteries of the recipient required direct plication for size mismatch. The patient survived in good condition for more than 2 years with no recurrence of thrombosis.ConclusionPreoperative anticoagulation therapy for massive thrombi in the pulmonary arteries was effective and led to safe lung transplantation.

Pulmonary Hypertension Induced by Right Pulmonary Artery Occlusion: Hemodynamic Consequences of Bmpr2 Mutation.

The primary genetic risk factor for heritable pulmonary arterial hypertension is the presence of monoallelic mutations in the BMPR2 gene. The incomplete penetrance of BMPR2 mutations implies that additional triggers are necessary for pulmonary arterial hypertension occurrence. Pulmonary artery stenosis directly raises pulmonary artery pressure, and the redirection of blood flow to unobstructed arteries leads to endothelial dysfunction and vascular remodeling. We hypothesized that right pulmonary artery occlusion (RPAO) triggers pulmonary hypertension (PH) in rats with Bmpr2 mutations. Male and female rats with a 71 bp monoallelic deletion in exon 1 of Bmpr2 and their wild-type siblings underwent acute and chronic RPAO. They were subjected to full high-fidelity hemodynamic characterization. We also examined how chronic RPAO can mimic the pulmonary gene expression pattern associated with installed PH in unobstructed territories. RPAO induced precapillary PH in male and female rats, both acutely and chronically. Bmpr2 mutant and male rats manifested more severe PH compared with their counterparts. Although wild-type rats adapted to RPAO, Bmpr2 mutant rats experienced heightened mortality. RPAO induced a decline in cardiac contractility index, particularly pronounced in male Bmpr2 rats. Chronic RPAO resulted in elevated pulmonary IL-6 (interleukin-6) expression and decreased Gdf2 expression (corrected P value<0.05 and log2 fold change>1). In this context, male rats expressed higher pulmonary levels of endothelin-1 and IL-6 than females. Our novel 2-hit rat model presents a promising avenue to explore the adaptation of the right ventricle and pulmonary vasculature to PH, shedding light on pertinent sex- and gene-related effects.