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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Right Heart Catheterization (RHC): A Comprehensive Review of Provocation Tests and Hepatic Hemodynamics in Patients With Pulmonary Hypertension (PH).

The classic definition of pulmonary arterial hypertension (PAH) is a mean pulmonary artery pressure (mPAP) of 20 mmHg. The gold standard for assessing pulmonary hemodynamics is right heart catheterization (RHC), which is necessary to confirm the diagnosis of PH. In some instances, RHC evaluates the degree of hemodynamic dysfunction and performs vasoreactivity tests. Measurement of the hepatic venous pressure gradient remains the gold standard diagnostic for identifying portal hypertension. This review aims to describe the procedure of RHC and the hemodynamic measurement in patients with PAH and Portopulmonary hypertension (PoPH). The RHC remains the gold standard for diagnosing PAH and PoPH.

A Brief Review on Gender Differences in Mexican-Mestizo Patients with Pulmonary Arterial Hypertension (PAH) at a Tertiary-Level Hospital.

Pulmonary hypertension (PH) is a hemodynamic condition with different etiological groups but common pathophysiology. Gender differences have been studied in group 1 of the PH classification, the pulmonary arterial hypertension (PAH) group. PAH has an etiopathogenic basis in sex hormones and directly affects the pulmonary vasculature and the heart. Gender differences are observed before and after the age of 45 when women lose the cardioprotective effect of estrogen. A retrospective cohort study in adult patients ≤45 years and >45 years. We compared hemodynamic, echocardiographic, and imaging variables that demonstrated gender differences in adult patients with PAH below and above 45 years. Gender differences in adults ≤45 years were significant for the pronounced pulmonic component of the second heart sound (P2) and the right atrium pressure, on the other hand, more significant sex differences were observed in patients over 45 years of age including the pronounced pulmonic component of P2 (greater in women), the brain natriuretic peptide had a higher median in men, the same happened in the echocardiographic data referring to the area of the right atrium and tricuspid annular plane systolic excursion, abnormal values predominate in men. Although PAH has greater incidence and prevalence in women, the lesions corresponding to cardiac remodeling that subsequently led to right ventricular failure are more remarkable in men, raising their mortality. These findings help recognize its clinical usefulness and propose new research studies aimed at mortality and new pharmacological therapies that might unveil the pathophysiological mechanisms to treat PAH.

Evaluation of Hepatic Hemodynamics (Hepatic Venous Pressure Gradient) During Right Heart Catheterization: A Comprehensive Review.

Centers for the research of patients with pulmonary hypertension (PH) usually perform right cardiac catheterization (RHC) to document this hemodynamic condition; traditionally, the procedure is performed by the interventional cardiologist, while the interventional radiologist generally conducts the study of hepatic hemodynamics. In our center, where the leading cause of catheterization of the hepatic veins is orthotopic liver transplantation, the cardio-pulmonologist performs the procedure to diagnose the possibility of porto-pulmonary hypertension and its implications. Routine measurement of the hepatic venous pressure gradient (HVPG) during RHC is not recommended but is performed to confirm the diagnosis of portal hypertension (PoH). Our objective in this review was to graphically describe the technique of hemodynamic recording of suprahepatic veins in patients with chronic liver disease and PoH who are in liver transplant protocol. The concepts included in this manuscript are measuring portal pressure, the definition of the hepatic venous pressure gradient (HVPG), procedures for a correct measurement of the HVPG, techniques associated with a suprahepatic vein catheterization, contraindications, and complications of HVPG, and clinical applications of HVPG. Clinically significant PoH is defined as an increase in GPVH ≥10 mmHg. HVPG measurement is currently the best available method for assessing the presence and severity of PoH. The RHC is the standard gold method for diagnosing PoPH that confirms its existence and provides additional data to exclude other causes of PAH in liver transplant candidates.