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.

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.

Pulmonary Hypertension or Pulmonary Arterial Hypertension in Idiopathic Pleuroparenchymal Fibroelastosis: An Updated Comprehensive Review.

Idiopathic pleuroparenchymal fibroelastosis (iPPFE) is a little-known entity with unique clinical, radiological, and pathological features. iPPFE is chronic interstitial pneumonia characterized by the thickening of elastic fibers in the pleura and subpleural parenchyma involving the upper lobes. Computed tomography pulmonary angiography (CTPA) usually depicts bilateral pleural thickening, with a left scalloped appearance that conditions retraction of the structures of the superior mediastinum and both pulmonary hila, associated with pulmonary consolidations with bronchogram air and thickening of the peribronchovascular interstitium, in addition to areas of left apical air trapping. When severe enough, the disease leads to progressive loss of volume of the upper lobes, decreased body mass, and platythorax. Some patients with iPPFE follow an inexorably progressive course culminating in irreversible respiratory failure and premature death. Up to 20% of patients might develop pulmonary hypertension (PH); transthoracic echocardiography is used as a screening test for PH; right heart catheterization performed in a tertiary-care hospital will confirm the diagnosis. Because iPPFE can be easily confused and misdiagnosed with infectious pathologies, such as pulmonary tuberculosis, and easily confuse physicians with little expertise in diffuse interstitial lung diseases, knowing the differential diagnoses, clinical presentation, imaging, and complications of the iPPFE allows for an early diagnosis and gives patients who suffer from it a better quality of life. This report presents a comprehensive review of PPFEi, discussing severe precapillary pulmonary hypertension and the associated findings demonstrated by right heart catheterization (RHC), which be of interest for cardiopulmonologists.

A Brief Review on Failed Hybrid Treatment for Massive Pulmonary Embolism: Catheter-Directed Thrombolysis (CDT) and Pharmaco-Mechanical Thrombolysis (PMT).

Acute massive or high-risk pulmonary embolism (PE), described as a lung arteries occlusion by an embolus, causes a significant compromise of hemodynamic stability and could lead to a lethal event. Systemic fibrinolytic therapy has been accepted as the standard reperfusion therapy in massive PE, except when there is an increased risk of bleeding. Catheter-based mechanical strategies (thrombofragmentation, thromboaspiration with catheter-guided thrombolysis) are described as options when there are absolute contraindications to systemic thrombolysis. We briefly reviewed clinical situations when patients with severe pneumonia due to COVID-19 are complicated by a high-risk saddle pulmonary embolism and underwent repeated pharmacomechanical thrombolysis and high-flow oxygen therapy. There are scarce reports of failed catheter-guided pharmacomechanical thrombolysis in patients with PE secondary to COVID-19. Re-administration of systemic thrombolysis and alteplase (15 mg dose) can show favorable results.

Pulmonary Thromboendarterectomy in Klinefelter Syndrome. Literature Review.

Klinefelter syndrome  is a form of male hypogonadism due to testicular sclerohyalinosis with atrophy and azoospermia, which is the most common cause of male infertility. The syndrome is usually accompanied by metabolic, morphological, and neurobehavioral manifestations; Venous thromboembolic diseases such as deep vein thrombosis and pulmonary embolism. The existence of chronic thromboembolic pulmonary hypertension in patients with Klinefelter syndrome   is scarce in the literature. We present the imaging and genetic analysis of a 37 -year-old male with a history of deep vein thrombosis who was admitted for exertional dyspnea.