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.

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.

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.

Portopulmonary Hypertension: Prevalence, Clinical and Hemodynamic Features.

Portopulmonary hypertension (PoPH) is a vascular complication of portal hypertension. This study aims to identify the prevalence and analyzing the clinical and hemodynamic features of patients with PoPH from a cohort of pulmonary arterial hypertension (PAH) patients. A retrospective transversal descriptive and analytical study. Patients with PoPH taken from a PAH cohort. We compare with those reported in the literature. We found prevalence of 6.1% of 244 consecutive patients with PAH, 11 females and 4 males. The mean age was 62 years and the main etiology of portal hypertension was primary biliary cirrhosis. Statistical differences were found in mean pulmonary arterial pressure, pulmonary vascular resistance, right atrial pressure; we found levels lower than reported. We found significant differences in clinical and hemodynamic characteristics such as older age and hemodynamic parameters of less severity in the group of patients analyzed compared with reported data.

Mucormicosis pulmonar en un paciente con trasplante renal y hemoptisis incoercible / Pulmonary mucormycosis in a patient with kidney transplant and uncontrolled haemoptysis

Antecedentes. La mucormicosis pulmonar es una infección oportunista rara con alta mortalidad causada por hongos Mucorales. Los más frecuentes son Rhizopus, Mucor, Lichtheimia y Rhizomucor. Caso clínico. Se presenta el caso de una mujer de 56 años con diabetes mellitus tipo 2 y enfermedad renal crónica, receptora de un trasplante de riñón cadavérico dos años antes de su ingreso. Por este motivo recibió tratamiento inmunomodulador con timoglobulina, micofenolato de mofetilo, tacrolimus y prednisona. La paciente ingresó por un cuadro neumónico con tos, expectoración y disnea; una tomografía computarizada mostró una lesión cavitada en el lóbulo superior derecho. Con la sospecha de una aspergilosis pulmonar invasiva se comenzó un tratamiento antifúngico con voriconazol, sin mejoría. Se realizó una biopsia por aspiración con aguja fina, y en el cultivo de la misma creció Rhizomucor pusillus. La identificación se confirmó por PCR. A pesar del tratamiento con anfotericina B, la paciente presentó hemoptisis masiva incoercible, que provocó su fallecimiento. Conclusiones. La mucormicosis pulmonar es una infección rara que suele ser fatal en receptores de trasplante renal con terapia antirrechazo. Los mucorales producen fenómenos trombóticos, necrosis y destrucción tisular, que provocaron en nuestra paciente una hemoptisis incoercible. Es importante un diagnóstico preciso que permita instaurar un tratamiento quirúrgico adecuado y la administración de anfotericina B (AU)

Background. Pulmonary mucormycosis is a rare opportunistic infection with high mortality that is caused by species of Mucorales. The most common species involved are Rhizopus, Mucor, Lichtheimia, and Rhizomucor. Case report. A 56 year-old woman presented with a clinical history of diabetes mellitus type 2 and chronic renal disease. She underwent a cadaveric kidney transplantation two years before her admission, for which immunomodulating therapy with thymoglobulin, tacrolimus, mofetil-microphenolate and prednisone was established. The patient suffered a pneumonic process with cough, expectoration, and dyspnoea. The computed tomography scan showed a cavitation in the right upper lobe. With all these findings an invasive broncopulmonary aspergillosis was suspected and the patient began an antifungal treatment with voriconazole without improvement. Rhizomucor pusillus was isolated from a clinical specimen obtained by fine needle aspiration, and its identification was confirmed by PCR. After this finding amphotericin B was administered, but the patient had an uncontrolled haemoptysis and died. Conclusions. Pulmonary mucormycosis is a rare infection, usually fatal in kidney transplant recipients with anti-rejection therapy. Mucorales species usually produce thrombotic phenomena, associated with necrosis and parenchymal destruction that caused a fatal uncontrolled haemoptysis in our patient. Early diagnosis is important in order to perform any surgical treatment and to administer amphotericin B (AU)

[Pulmonary mucormycosis in a patient with kidney transplant and uncontrolled haemoptysis]. / Mucormicosis pulmonar en un paciente con trasplante renal y hemoptisis incoercible.

BACKGROUND: Pulmonary mucormycosis is a rare opportunistic infection with high mortality that is caused by species of Mucorales. The most common species involved are Rhizopus, Mucor, Lichtheimia, and Rhizomucor. CASE REPORT: A 56 year-old woman presented with a clinical history of diabetes mellitus type 2 and chronic renal disease. She underwent a cadaveric kidney transplantation two years before her admission, for which immunomodulating therapy with thymoglobulin, tacrolimus, mofetil-microphenolate and prednisone was established. The patient suffered a pneumonic process with cough, expectoration, and dyspnoea. The computed tomography scan showed a cavitation in the right upper lobe. With all these findings an invasive broncopulmonary aspergillosis was suspected and the patient began an antifungal treatment with voriconazole without improvement. Rhizomucor pusillus was isolated from a clinical specimen obtained by fine needle aspiration, and its identification was confirmed by PCR. After this finding amphotericin B was administered, but the patient had an uncontrolled haemoptysis and died. CONCLUSIONS: Pulmonary mucormycosis is a rare infection, usually fatal in kidney transplant recipients with anti-rejection therapy. Mucorales species usually produce thrombotic phenomena, associated with necrosis and parenchymal destruction that caused a fatal uncontrolled haemoptysis in our patient. Early diagnosis is important in order to perform any surgical treatment and to administer amphotericin B.