BACKGROUND: Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare but serious complication in patients with malignancy; its main manifestation includes acute pulmonary hypertension with severe respiratory distress. More than 200 cases have been reported since it was first identified in 1990. PTTM accounts for approximately 0.9% to 3.3% of deaths due to malignancy, but only a minority of patients are diagnosed ante-mortem, with most patients having a definitive diagnosis after autopsy.
METHODS: Two middle-aged women both died within a short period of time due to progressive dyspnea and severe pulmonary hypertension.
METHODS: One patient was definitively confirmed as a gastrointestinal malignant tumor by liver puncture biopsy pathology. Ultimately, the clinical diagnosis was pulmonary tumor thrombotic microangiopathy.
METHODS: The patient was treated symptomatically with oxygen, diuresis, and anticoagulation, while a liver puncture was perfected to clarify the cause.
RESULTS: Two cases of middle-aged female patients with rapidly progressive pulmonary hypertension and respiratory failure resulted in death with malignant neoplasm.
CONCLUSIONS: PTTM has a rapid onset and a high morbidity and mortality rate. Our clinicians need to be more aware of the need for timely diagnosis through a targeted clinical approach, leading to more targeted treatment and a better prognosis.

UNASSIGNED: To estimate the predictors, prevalence and prognostic role of pulmonary hypertension (PH) in patients with chronic kidney disease (CKD) using meta-analysis.
UNASSIGNED: The PubMed, EmBase, and the Cochrane library were systematically searched for eligible studies from inception till May 2024. All of pooled analyses were performed using the random-effects model.
UNASSIGNED: Fifty observational studies involving 17,558 CKD patients were selected. The prevalence of PH in CKD patients was 38% (95% confidence interval [CI]: 33%-43%), and the prevalence according to CKD status were 31% (95% CI: 20%-42%) for CKD (I-V), 39% (95% CI: 25%-54%) for end stage kidney disease (ESKD) (predialysis), 42% (95% CI: 35%-50%) for ESKD (hemodialysis), and 26% (95% CI: 19%-34%) for renal transplant. We noted the risk factors for PH in CKD included Black individuals (relative risk [RR]: 1.39; 95% CI: 1.18-1.63; p < 0.001), chronic obstructive pulmonary disease (RR: 1.48; 95% CI: 1.21-1.82; p < 0.001), cardiovascular disease history (RR: 1.62; 95% CI: 1.05-2.51; p = 0.030), longer dialysis (RR: 1.70; 95% CI: 1.18-2.46; p = 0.005), diastolic dysfunction (RR: 1.88; 95% CI: 1.38-2.55; p < 0.001), systolic dysfunction (RR: 3.75; 95% CI: 2.88-4.87; p < 0.001), and grade 5 CKD (RR: 5.64; 95% CI: 3.18-9.98; p < 0.001). Moreover, PH in CKD patients is also associated with poor prognosis, including all-cause mortality, major cardiovascular events, and cardiac death.
UNASSIGNED: This study systematically identified risk factors for PH in CKD patients, and PH were associated with poor prognosis. Therefore, patients with high prevalence of PH should be identified for treatment.

As the pediatric patient with right pulmonary artery agenesis (PAA) matured, she progressively presented symptoms of pulmonary hypertension and hemoptysis. There is limited clinical literature on this condition, and currently, there is no consensus regarding its diagnosis and treatment. This article presents a case study of a 16-year-old female patient with right pulmonary artery hypoplasia, providing a comprehensive summary and analysis of her developmental progression, pathology, diagnosis, and treatment.

UNASSIGNED: Pulmonary hypertension (PH) is a life-threatening disease, especially in paediatric population. Symptoms of paediatric PH are non-specific. Accurate detection of paediatric PH is helpful for early treatment and mortality reduction. Therefore, we assessed the overall performance of brain natriuretic peptide (BNP) and N-terminal brain natriuretic peptide (NT-proBNP) for diagnosing PH in paediatric population.
UNASSIGNED: PubMed, Web of Science, Cochrane Library and Embase databases were screened since their respective inceptions until August 2023. A bivariate random model and a hierarchical summary receiver operating characteristic model were used together to evaluate and summarize the overall performance of BNP and NT-proBNP for diagnosing paediatric PH.
UNASSIGNED: Eighteen studies using BNP/NT-proBNP were assessed, comprising 1127 samples. The pooled sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), diagnostic odds ratio (DOR) and area under the curve (AUROC) of BNP/NT-proBNP were separately as 0.81, 0.87, 6.33, 0.21, 29.50 and 0.91, suggesting a good diagnostic performance of BNP/NT-proBNP for detecting PH in paediatric population. For BNP, the pooled sensitivity, specificity, PLR, NLR, DOR and AUROC were 0.83, 0.89, 7.76, 0.19, 40.90 and 0.93, indicating the diagnostic accuracy of BNP for paediatric PH patients was good. For NT-proBNP, the pooled sensitivity, specificity, PLR, NLR, DOR and AUROC were 0.81, 0.86, 5.59, 0.22, 24.96 and 0.90, showing that NT-proBNP could provide a good value for detecting paediatric PH.
UNASSIGNED: Both BNP and NT-proBNP are good markers for differentiating paediatric PH patients from non-PH individuals.
Accurate detection of paediatric PH is helpful for early treatment and mortality reduction. This study shows that both BNP and NT-proBNP are good markers for detecting paediatric PH. In clinical practice, we recommend that BNP and NT-proBNP are auxiliary biomarkers in diagnosing paediatric PH.

Chronic thromboembolic pulmonary hypertension (CTEPH) presents as a progressive vascular condition arising from previous episodes of acute pulmonary embolism, contributing to the development of pulmonary hypertension (PH). Pulmonary thromboendarterectomy (PTE) is the gold-standard surgical treatment for CTEPH; however, it may be associated with postoperative sequelae, including atrial arrhythmias (AAs). This comprehensive literature review explores the potential mechanisms for PTE-induced AAs with emphasis on the role of PH-related atrial remodelling and the predisposing factors. The identified preoperative predictors for AAs include advanced age, male gender, elevated resting heart rate, previous AAs, and baseline elevated right atrial pressure. Furthermore, we explore the available data on the association between post-PTE pericardial effusions and the development of AAs. Lastly, we briefly discuss the emerging role of radiomic analysis of epicardial adipose tissue as an imaging biomarker for predicting AAs.

Despite the end of the pandemic, coronavirus disease 2019 (COVID-19) remains a major public health concern. The first waves of the virus led to a better understanding of its pathogenesis, highlighting the fact that there is a specific pulmonary vascular disorder. Indeed, COVID-19 may predispose patients to thrombotic disease in both venous and arterial circulation, and many cases of severe acute pulmonary embolism have been reported. The demonstrated presence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) within the endothelial cells suggests that direct viral effects, in addition to indirect effects of perivascular inflammation and coagulopathy, may contribute to pulmonary vasculopathy in COVID-19. In this review, we discuss the pathological mechanisms leading to pulmonary vascular damage during acute infection, which appear to be mainly related to thromboembolic events, an impaired coagulation cascade, micro- and macrovascular thrombosis, endotheliitis and hypoxic pulmonary vasoconstriction. As many patients develop post-COVID symptoms, including dyspnea, we also discuss the hypothesis of pulmonary vascular damage and pulmonary hypertension as a sequela of the infection, which may be involved in the pathophysiology of long COVID.

Pulmonary complications of systemic scleroderma (SSc), such as interstitial lung disease and pulmonary hypertension (PH), are responsible for up to 60% of deaths among patients. For many years, most centers considered SSc a contraindication to lung transplantation (LTx); however, recent publications show that appropriately selected SSc candidates for LTx give results comparable to patients with idiopathic PH or idiopathic pulmonary fibrosis. This paper presents the cases of a 60-year-old male patient (patient 1) and a 42-year-old female patient (patient 2) diagnosed with SSc in 2019 and 2013, respectively. In both patients, interstitial-fibrotic changes in the lungs leading to respiratory failure were confirmed by high-resolution computed tomography as well as pulmonary hypertension (WHO group 3), which was also diagnosed during right heart catheterization. In both cases, despite pharmacotherapy, pulmonary fibrosis progressed, leading to severe respiratory failure. The patients were referred for LTx qualification. LTx was possible to consider in patients due to the lack of significant changes in other internal organs. Double LTx was successfully performed in both patients (patient 1-July 19, 2022; patient 2-September 14, 2022). They were discharged from the hospital in good condition on the 22nd and 20th postoperative day, respectively. LTx is a last-chance therapy that saves lives among patients with extreme respiratory failure in the course of SSc. It prolongs and improves the quality of life. The selection of appropriate patients is key to the success of the procedure.

The pathophysiological interplay between sleep-disordered breathing (SDB) and pulmonary hypertension (PH) is complex and can involve a variety of mechanisms by which SDB can worsen PH. These mechanistic pathways include wide swings in intrathoracic pressure while breathing against an occluded upper airway, intermittent and/or sustained hypoxemia, acute and/or chronic hypercapnia, and obesity. In this review, we discuss how the downstream consequences of SDB can adversely impact PH, the challenges in accurately diagnosing and classifying PH in the severely obese, and review the limited literature assessing the effect of treating obesity, obstructive sleep apnea, and obesity hypoventilation syndrome on PH.

Pulmonary hypertension (PH) with high pulmonary vascular resistance (PVR) is a very often diagnosed contraindication for orthotopic heart transplantation (OHT). It is a direct consequence of left ventricle failure characterized by high diastolic pressure obstructing the collection of blood from the pulmonary vessels. The occurrence of this situation grows with the increasing time of waiting for OHT, and with the progression of heart failure. Mechanical circulatory support (MCS) devices, particularly left ventricular assist devices (LVADs), have emerged as pivotal interventions for patients with fixed PH, offering a potential bridge to transplantation. The pathophysiological impact of PH in heart transplant candidates is profound, as it is associated with increased perioperative risk and heightened mortality post-transplantation. The selection of heart transplant candidates thus mandates a careful evaluation of PH, with an emphasis on distinguishing between reversible and fixed forms of the condition. Reversible PH can often be managed with medical therapies; however, fixed PH presents a more daunting challenge, necessitating more aggressive interventions like MCS. Patients are supported with LVADs until evidence of pulmonary afterload reversal is evident and then can be considered for heart transplantation. However, in those who are non-responders or have complications while being supported, their option for transplant is revoked. Despite these advancements, the heterogeneity of MCS devices and their mechanisms of action necessitates a nuanced understanding of their efficacy.

Tricuspid valve disease is an often underrecognized clinical problem that is associated with significant morbidity and mortality. Unfortunately, patients will often present late in their disease course with severe right-sided heart failure, pulmonary hypertension, and life-limiting symptoms that have few durable treatment options. Traditionally, the only treatment for tricuspid valve disease has been medical therapy or surgery; however, there have been increasing interest and success with the use of transcatheter tricuspid valve therapies over the past several years to treat patients with previously limited therapeutic options. The tricuspid valve is complex anatomically, lying adjacent to important anatomic structures such as the right coronary artery and the atrioventricular node, and is the passageway for permanent pacemaker leads into the right ventricle. In addition, the mechanism of tricuspid pathology varies widely between patients, which can be due to primary, secondary, or a combination of causes, meaning that it is not possible for 1 type of device to be suitable for treatment of all cases of tricuspid valve disease. To best visualize the pathology, several modalities of advanced cardiac imaging are often required, including transthoracic echocardiography, transesophageal echocardiography, cardiac computed tomography, and cardiac magnetic resonance imaging, to best visualize the pathology. This detailed imaging provides important information for choosing the ideal transcatheter treatment options for patients with tricuspid valve disease, taking into account the need for the lifetime management of the patient. This review highlights the important background, anatomic considerations, therapeutic options, and future directions with regard to treatment of tricuspid valve disease.