Demographic, hemodynamic characteristics, and therapeutic trends of pulmonary hypertension patients: The Pulmonary Hypertension Mexican registry (REMEHIP).

Data on demographic characteristics and therapeutic approaches in Latin American pulmonary arterial hypertension (PAH) patients are scarce. Pulmonary Hypertension Mexican registry (REMEHIP) is a multicenter Mexican registry of adult and pediatric patients, including prevalent and incident cases. Objective: assess clinical characteristics, treatment trends, and in-hospital outcomes. Inclusion: age >2 years, diagnosis of pulmonary hypertension (PH) (groups 1 and 4), right heart catheterization with mPAP ≥25 mmHg, PWP ≤ 15 mmHg, and PVR > 3 Wood unit (WU). We included 875 PH patients, 619 adults, 133 pediatric idiopathic PAH (IPAH), and 123 chronic thromboembolic pulmonary hypertension (CTEPH) patients. We enrolled 48.4% of the incident and 51.6% of the prevalent adult and pediatric patients. PAH adults: age 43 ± 15, females 81.9%, functional class (FC) (I/II) 66.5%, 6-min walk distance (6MWD) 378 ± 112 m, mPAP 57.3 ± 19.0 mmHg, confidence interval (CI) 3.3 ± 1.5 L/min/m2, PVR 12.0 ± 8.1 WU. PAH pediatrics: age 9 ± 5, females 51.1%, FC (I/II) 85.5%, 6MWD 376 ± 103 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 16.4 ± 13.5 WU. CTEPH: age 44 ± 17, females 56.1%, FC (I/II) 65.5%, 6MWD 369 ± 126 m, mPAP 49.7 ± 13.4 mmHg, CI 2.6 ± 0.9 L/min/m2, PVR 10.5 + 6.5 WU. When we analyzed the IPAH group separately, it sustained a high functional class I/II incidence. REMEHIP shows better functional class in young females with severe PAH than in American and European patients. Also, PAH pediatric patients had a better functional class than other registries. However, our registry also shows that our population's access to specific pharmacologic treatments is still far from optimal.

Severe pulmonary hypertension in pulmonary alveolar microlithiasis: A comprehensive literature review.

This review focuses on Pulmonary Alveolar Microlithiasis (PAM), an autosomal recessive genetic disorder characterized by calcium crystal deposits (microliths) resulting from loss of function of the SLC34A2 gene. PAM is a rare disease with approximately 1100 reported cases globally. The historical context of its discovery and the genetic, epidemiological, and pathophysiological aspects are discussed. PAM falls under interstitial lung diseases and is associated with pulmonary hypertension (PH), primarily categorized as Group 3 PH. The clinical manifestations, diagnostic approaches, and challenging aspects of treatment are explored. A clinical case of PAM with severe pulmonary hypertension is presented, emphasizing the importance of comprehensive evaluation and the potential benefits of phosphodiesterase-5 inhibitors (PDE5i) therapy. Despite limited therapeutic options and challenging diagnosis, this review sheds light on recent developments and emerging treatments for PAM and associated pulmonary hypertension.

Systemic scleroderma: Review and updated approach and case description to addressing pulmonary arterial hypertension and idiopathic pulmonary fibrosis: A dual challenge in treatment.

Pulmonary arterial hypertension (PAH), idiopathic pulmonary fibrosis (IPF), and scleroderma (SSc) are three interrelated medical conditions that can result in significant morbidity and mortality. Pulmonary hypertension, a condition marked by high blood pressure in the lungs, can lead to heart failure and other complications. Idiopathic pulmonary fibrosis, a progressive lung disease characterised by scarring of lung tissue, can cause breathing difficulties and impaired oxygenation. Scleroderma, an autoimmune disease, can induce thickening and hardening of the skin and internal organs, including the lungs, leading to pulmonary fibrosis and hypertension. Currently, there is no cure for any of these conditions. However, early detection and proper management can improve the quality of life and prognosis of a patient. This review focusses on PH and IPF in patients with SSc, providing information on the causes, symptoms, and treatment of these conditions, together with illustrative images. It also provides an overview of interrelated medical conditions: PH, IPF, and SSc. It emphasises the importance of early detection and proper management to improve patient quality of life and prognosis.

Complications of Right Heart Catheterization in Patients ≥70 Years of Age With Suspected Pulmonary Hypertension: Experience From a Tertiary Care Center.

Right heart catheterization (RHC) represents the gold standard diagnostic approach for pulmonary hypertension (PH). Historically, the complication rates of RHC are known to be low. The study aimed to evaluate the indications for performing RHC and the occurrence of adverse events related to the procedure in patients > over 70 years of age in a Mexican Tertiary Care Center. We conducted a retrospective single-center registry from July 2017 to July 2022. A total of 517 patients with suspected PH underwent RHC. The cohort included patients <70 (n = 427) and ≥70 years of age (n = 90). Adverse events were classified as major (eg, death, pneumothorax, and carotid artery puncture) and minor (eg, atrial arrhythmia, superior vena cava dissection, incidental arterial puncture, and local hematoma). Appropriate hemodynamic parameters were recorded. No report of major adverse events in the entire cohort. In the <70 years age group, 9 minor events, and 3 minor events were in the ≥70-year-old patients (P < 0.0001). There was a significant difference in the measurement of mean pulmonary artery pressure (mPAP) between the <70 years old vs ≥70 years old (P < 0.001); there was a significant difference in right atrial pressures: 4.71 ± 3.14 mmHg in the <70-year-old vs 4.07 ± 1.94 mmHg for the ≥ 70-year-old group (P = 0.014). Our findings suggest that RHC can be safely performed in patients aged ≥70 years using different vascular access routes without significant major complications.

Prevalence and Prognosis of Hypothyroidism in Patients With Chronic Thromboembolic Pulmonary Hypertension: An Updated Review With Description of Case Series.

Hypothyroidism has been shown to have several effects on organs, including derangements in the coagulation system, impairing endothelial function, but data on the importance of hypothyroidism in the pathogenesis and development of chronic thromboembolic pulmonary hypertension (CTEPH) are limited. This report presents an updated review of the prevalence and prognosis of hypothyroidism in patients diagnosed with CTEPH, including a detailed retrospective description of the series. The descriptive case series included 34 adult patients diagnosed with CTEPH, of whom 11 patients were diagnosed with hypothyroidism. The prevalence of hypothyroidism in CTEPH was found to be 32.35%. All patients with hypothyroidism had NYHA functional Class II-III. Hemodynamic values obtained through right heart catheterization (RHC) showed that patients with hypothyroidism had significantly higher mean pulmonary arterial pressures (mPAP), with a mean of 56.91 mm Hg vs 43.93 mm Hg (p = 0.026), and the PVR in dynes/sec/cm5 was 932 vs 541 (p = 0.027). Significant differences in PVR were found in wood units (WU) 11.91 vs 7.11 (p = 0.042). The mean level of brain natriuretic peptide (BNP) between both groups was 797.3 pg/mL for patients with hypothyroidism vs 262.02 pg/mL in patients with euthyroidism (p = .032). Hypothyroidism may significantly affect patients' clinical and hemodynamic outcomes in patients with CTEPH. Hypothyroidism as a risk factor in the evaluation and treatment of these patients is vital to optimize outcomes in CTEPH; further research is warranted whether hypothyroidism therapies could alter such outcomes.

Venous air embolism: Case series of a complication of computed tomography pulmonary angiography (CTPA) in the Emergency Department of Medicine.

INTRODUCTION: Venous air embolism (VAE) consists of air entering vascular structures due to a pressure gradient generated during medical-surgical procedures. Most cases of VAE are iatrogenic. CASE REPORTS: Three hospitalised patients aged 23 to 86 years underwent venous air embolism (VAE) in the right heart system after performing CTPA. One of the patients died from a complication of venous thromboembolic disease (PE, coronary sinus thrombosis, mesenteric venous thrombosis). CONCLUSIONS: CTPA is a procedure that a priori seems innocuous, but it can be a potential cause of death or serious consequences for patients undergoing radiological procedures where the administration of contrast and the use of an injector could be counterproductive. Radiologists and physicians responsible for the patient should be aware of vascular gas embolism after contrast injection in patients undergoing CTPA.

Nonthrombotic Pulmonary Embolism Associated With Non-Hodgkin Lymphoma.

Nonthrombotic pulmonary embolism (NTPE) challenges the medical community with its diverse etiologies and potential life-threatening implications. The classification section delves into the multifaceted nature of NTPE, which includes various embolic agents that traverse the vascular system. From air and fat emboli to tumor and amniotic fluid emboli, this exploration of diverse etiologies sheds light on the complexity of NTPE. Diagnostic methods play a crucial role in the effective management of NTPE. This article describes a range of traditional and cutting-edge diagnostic techniques, from computed tomography angiography to novel biomarkers, enabling the accurate and timely identification of NTPE. NTPE treatment options are diverse and patient-specific, requiring customized approaches to address varying embolic sources. Anticoagulation, embolus removal, and emerging interventions under study are discussed, providing clinicians with a comprehensive understanding of management strategies. This article uncovers the rare but captivating association between NTPE and non-Hodgkin lymphoma. Although rare, documented cases have sparked curiosity among researchers and medical practitioners. We explore potential pathophysiological connections, discussing challenges and considerations when encountering this unique scenario. In conclusion, this captivating review encapsulates the multifaceted realm of NTPE, covering its classification, diagnostics, and treatment modalities. Moreover, it presents a fascinating connection with non-Hodgkin lymphoma. This article offers a comprehensive and concise review of NTPE, guiding readers through its intricate classification, diagnostic approaches, and therapeutic interventions.

Pulmonary Embolism During Pregnancy: An Updated Review With Case Series Description.

Pulmonary embolism (PE) is a potentially life-threatening condition that can occur during pregnancy and pose a significant risk to the mother and the developing fetus. It is a major contributor to pregnancy-related morbidity and mortality in any trimester. It is estimated that the incidence of PE during pregnancy is approximately 1 in 1000 pregnancies. The mortality rate for pregnant women with PE is about 3%, significantly higher than that for nonpregnant women with PE. Overall, the topic of PE and pregnancy is essential for healthcare professionals to be aware of the risks, signs, and treatment options to improve outcomes and ensure the best possible care for both the mother and the developing fetus. To prevent the fatal condition, the physician is encouraged when there is a suspicion of the pathology. This report presents an updated comprehensive review of PE during pregnancy, discussing critical aspects of the clinical and imaging diagnosis, use of heparin, thrombolysis, and prevention. We believe this article will be helpful for cardiologists, obstetricians, and other health-related professionals.

Association Between the Degree of Severity of Pulmonary Hypertension With the Presence of Pulmonary Artery Aneurysm: A Brief Updated Review for Clinicians.

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary arterial pressure (mPAP) ≥20 mm Hg at rest as assessed by right cardiac catheterization. It has a median survival nowadays of 6 years, compared to 2.8 years in the 1980s. A pulmonary artery aneurysm (PAA) is the focal dilation of a blood vessel involving all 3 layers of the vessel wall; they have a diameter greater than 4 cm measured in the trunk of the pulmonary artery. PAAs can be classified into proximal (or central) and peripheral. The clinical manifestations of PAA are primarily nonspecific, and most patients remain undiagnosed, even those with large PAA, due to its silent course; however, clinical manifestations occur unless when there are complications such as bronchial or tracheal compression (leading to cough and dyspnea), dissection, or rupture (leading to hemoptysis). PAH is observed in 66% of patients with PAA. PA dissections are usually associated with PAH; 80% of dissections occur in the main pulmonary trunk. Although there is no clear guideline for the best treatment of PAA, surgery is indicated in patients with a pulmonary trunk aneurysm >5.5 cm. It has been observed that patients in the PAH group associated with congenital heart disease tend to develop PAA more commonly. Those with PAH associated with connective tissue disease have a smaller diameter of PA dilation. This report presents a comprehensive review of PAA, discussing critical aspects of the clinical and imaging diagnosis, hemodynamics, and treatment. A comprehensive updated literature review is included; we believe this article will interest cardiopulmonologists.

Review of the Myelodysplastic Syndrome as a Cause of Group 5 Pulmonary Arterial Hypertension: An Orphan Disease in an Orphan Pulmonary Hypertension Group.

The coexistence of MDS and pulmonary hypertension (PH) is not a common finding and often goes unnoticed because symptoms such as dyspnea can be confused with the underlying pathology. The annual incidence of idiopathic pulmonary arterial hypertension (PAH) is only around 0.2 cases per 100,000 inhabitants, while MDS is 1 to 8 cases per 100,000 inhabitants. This review summarizes the clinical manifestations, functional respiratory tests, hemodynamic parameters using right heart catheterization, and imaging findings using echocardiography and tomography of pulmonary hypertension in myelodysplastic syndrome. We centered our discussion on the diagnosis of these patients within the hematologic disorders, especially in patients with the detriment of the functional class, as we were not used to looking for this diagnosis as a first choice. Several specialties dealing with patients with hematologic disorders (internists, hematologists, family physicians, geriatrics, oncologists) will find helpful the contents of this review.

A Review of the Usefulness of Catheter-Directed Thrombolysis for Pulmonary Embolism.

Venous thromboembolic disease (VTE) is a health problem; around 10 million cases occur yearly with substantial morbidity and mortality. Those who survive may be left with long-term sequelae. Those sequelae might include chronic thromboembolic pulmonary hypertension, persistent right ventricular dysfunction, exercise intolerance, and reduced quality of life. Current PE management consists of anticoagulation alone, systemic thrombolysis, catheter-directed thrombolysis, and surgical embolectomy. The severity of patients with pulmonary embolism (PE) depends on the clinic and not exclusively on the extent of radiological or anatomical involvement. In this review, we present the main clinical and functional characteristics of patients in whom thrombotic fragmentation plus catheter-guided thrombolysis is used to manage acute PE of intermediate-high risk and torpid evolution within the first hours of admission.

Air and Thrombotic Venous Embolism in a Department of Emergency Medicine. A Literature Review.

Among the different types of central venous access, 2 are mainly recognized, the internal jugular and the subclavian. The most common complications of these invasive procedures are hematoma, vascular injury, pneumothorax, and hemothorax. This review presents 2 atypical complications in the central vascular approach in a patient seen in the emergency department. Complications were air and thrombotic embolisms. Physicians dealing with central venous catheter placement (emergency department, intensive care unit, surgical room, interventionist procedures) should be aware that vascular air embolism is an iatrogenic able to be detected with imaging methods (computed tomography pulmonary angiography, magnetic resonance imaging) as strategies to minimize the risks, which even in expert hands is not exempt from the complications it can have.

Chronic Thromboembolic Pulmonary Hypertension (CTEPH): A Review of Another Sequel of Severe Post-Covid-19 Pneumonia.

The spectrum of pulmonary parenchymal and vascular pathologies related to the COVID-19 have emerged. There is evidence of a specific susceptibility related to thrombotic microangiopathy in situ and a complex immune-inflammatory cascade, especially in the pulmonary vascular bed. The potential to lead to transient or self-correcting sequelae of pulmonary vascular injury will only become apparent with longer-term follow-up. In this review, we aimed to present the findings in a group of patients with severe pneumonia due to covid-19 complicated by acute pe documented by chest angiography, who during a follow-up of more than 3 months with oral anticoagulant met clinical, hemodynamic, and imaging criteria of chronic thromboembolic pulmonary hypertension. We present a brief review of the epidemiology, pathophysiology, clinical findings, comorbidities, treatment, and imaging findings of chronic thromboembolic pulmonary hypertension as a sequel of severe post-covid-19 pneumonia; and compared and discussed these findings with similar reports from the medical literature.

Review of Functional Status and Hemodynamic Parameters in Patients Diagnosed with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) With and Without Antiphospholipid Syndrome (APLS).

Pulmonary hypertension is a hemodynamic state defined by a mean pulmonary arterial pressure >20 mmHg and a pulmonary vascular resistance ≥3 WU, subdivided into 5 groups. Chronic Thromboembolic Pulmonary Hypertension (CTEPH) corresponds to group 4. The antiphospholipid syndrome is one of the most associated thrombophilia, with a prevalence of CTEPH of 2%-50%. A case-control study was conducted where data from the Right Cardiac Catheterization Registry of the PH Clinic were collected, with a diagnosis of CTEPH in patients aged 18-60 years and any sex. Antiphospholipid Syndrome (APLS) patients were separated from those with only CTEPH. It was developed in a statistical analysis based on frequencies, means, and standard deviation. The variables were evaluated using the Kolmogorov-Smirnov, Student's T, Mann-Whitney U, and Chi-Square tests with a 95% confidence interval. A total of 12 patients with APLS diagnosis and 30 without it were identified. The comparison between both groups shows that the patients with APLS were younger (38 ± 14.35 vs 51.63 ± 15.02 years, P 0.010) and had a significant association with autoimmune diseases (25% vs 0%, P 0.003). The patients diagnosed with APLS were primarily men (7 vs 5), and no statistically significant difference was found between laboratory and hemodynamic parameters. Patients diagnosed with CTEPH and APLS are mainly male, younger mean age, and have a greater significant association with autoimmune diseases than patients with CTEPH.

Review of Acute Leukemia as a New Cause of Dual Thrombosis (Pulmonary Vein Thrombosis and Pulmonary Embolism).

Pulmonary vein thrombosis is rare in surgical situations and medical pathologies. The coexistence of pulmonary thrombosis with pulmonary vein thrombosis has been called dual thrombosis and has been reported in a scarce number of post-surgical patients undergoing lung transplantation. We describe a patient with acute leukemia who attended an evaluation for dyspnea. CT angiography of the chest reveals the presence of bilateral pulmonary thromboembolism, as well as pulmonary vein thrombosis. The review of the medical literature in the English language does not report similar cases in medical pathology. It would be the first case of dual thrombosis secondary to acute leukemia.

Pulmonary Embolism (PE) Prevalence in Mexican-Mestizo Patients With Severe SARS-COV-2 (COVID-19) Pneumonia At A Tertiary-Level Hospital: A Review.

Since the report of the first case of COVID-19 in Wuhan, China, on December 31, 2019, several associated thrombotic complications have been reported, mainly venous thromboembolic events, and myocardial infarctions, in addition to peripheral arterial thrombosis and cerebral vascular events, which have been attributed to a hypercoagulable state. We aimed to know the prevalence and prognostic biomarkers in patients with pulmonary thromboembolism (PE) and SARS Cov-2 pneumonia. Hospitalized patients with SARS Cov-2 pneumonia who have had clinical, biomarker, and imaging data (chest angiography) of pulmonary thromboembolism were included. Descriptive statistics and prevalence rates were calculated. For the analysis between the groups, the paired Student's t and the Wilcoxon test were performed. CT angiography was performed on 26 patients at our institution, with a diagnosis of severe pneumonia secondary to SARS-CoV2. 9 of the patients (34.6%) had a venous thromboembolic disease. Type 2 DM was the most frequent comorbidity up to 55.5% of the total; it was followed by obesity and overweight in 55.5%, and in third place, by systemic arterial hypertension in 33.3% of the cases, 1 (11.1%) patient had chronic kidney disease and 1 (11.1%) patient with a history of cancer, only 1 patient met criteria and was treated with thrombolysis. 6 (66.6%) of the patients had segmental PE, 3 (33.3%) patients had subsegmental PE, and 4 (44.4%) patients presented pulmonary infarction.

Acute Pulmonary Embolism Code and Rapid Response Teams are Necessary: A Review of Global and Mexico's Teams (MGH PERT).

Pulmonary embolism (PE) worldwide is an underdiagnosed disease; at the moment, there are no statistical data to make inferences regarding the thrombotic problem in Mexico. Although, in general, small emboli (subsegmental) are well tolerated in the pulmonary circulation, difficulties frequently occur for medium to large emboli that occlude more than 30% of the pulmonary circulation. In the United States, it is estimated that up to 100,000 PE-related deaths occur each year. A PE code consists of activating a group of specialists in PE for the consensual making of therapeutic decisions; it is beneficial for the clinical evolution of these patients and reduces their mortality; a PE response team (PERT) codes in reference hospitals to manage this disease. This report presents an updated summary of the PERT status globally and in Mexico, the explanation of why a PE code is necessary, and the effects of PERT teams in the detection (chronic thromboembolic pulmonary hypertension, chronic thromboembolic disease, and venous thromboembolism); therapeutic procedures (catheter-directed thrombolysis, systemic thrombolysis or surgical thrombectomy); selection of patients from low to high risk of PE; and future directions for PERT teams.