Diagnosis and treatment of right ventricular failure secondary to acutely increased right ventricular afterload (acute cor pulmonale): a clinical consensus statement of the Association for Acute CardioVascular Care of the European Society of Cardiology.

Acute right ventricular failure secondary to acutely increased right ventricular afterload (acute cor pulmonale) is a life-threatening condition that may arise in different clinical settings. Patients at risk of developing or with manifest acute cor pulmonale usually present with an acute pulmonary disease (e.g. pulmonary embolism, pneumonia, and acute respiratory distress syndrome) and are managed initially in emergency departments and later in intensive care units. According to the clinical setting, other specialties are involved (cardiology, pneumology, internal medicine). As such, coordinated delivery of care is particularly challenging but, as shown during the COVID-19 pandemic, has a major impact on prognosis. A common framework for the management of acute cor pulmonale with inclusion of the perspectives of all involved disciplines is urgently needed.

Isolated unilateral pulmonary artery atresia in an adult presenting with cor pulmonale.

Unilateral pulmonary artery atresia (UPAA) is a rare embryonic vascular malformation, leading to general presentations of exertional dyspnoea, pneumonia and haemoptysis. Our patient, a man in his early 30s, presented with a history of progressive breathlessness over a period of 2 years. History showed multiple admissions for pneumonia over his childhood and adolescence. Physical examination revealed a loud P2, an ejection systolic murmur and coarse crepitations in multiple lung fields. Severe pulmonary artery hypertension was noted on two-dimensional echocardiography. CT studies confirmed right pulmonary artery atresia with aspergilloma and bronchiectasis. With this diagnosis of UPAA, this case report intends to raise awareness among clinicians to consider this as a rare cause of cor pulmonale in an adult.

Clinical challenges in the treatment of a patient with decompensated heart failure and glucose-6-phosphate dehydrogenase deficiency (G6PDd).

We present a case of a man in his 60s, known with glucose-6-phosphate dehydrogenase deficiency (G6PDd) and cor pulmonale, admitted to the department of cardiology due to cardiac decompensation and anaemia. The main complaint was dyspnoea. Echocardiography confirmed severe cor pulmonale with compression of the left ventricle. G6PDd has been linked with pulmonary hypertension which could contribute to aforementioned echocardiographic findings. Diuretics are the first line of treatment when it comes to cardiac decompensation, but sulfonamide diuretics can induce or exacerbate haemolysis in patients with G6PDd. Due to the respiratory distress of the patient, a treatment plan including sulfonamide diuretics was initiated in collaboration with the haematologists. Unfortunately, the patient died 2 days after admission. This case emphasises that not all cardiac patients can tolerate standard treatment with sulfonamide diuretics; despite this, they remain essential in the acute setting, and they are associated with foreseeable but only partly manageable complications in susceptible patients.

Etiology, characteristics and occurrence of heart diseases in rural Lesotho (ECHO-Lesotho): A retrospective echocardiography cohort study.

BACKGROUND: In 2019, 600'000 people in Africa died of heart failure and heart diseases will increase on the continent. It is crucial to understand the regional etiologies and risk factors for heart failure and underlying heart diseases. However, echocardiography data from rural Africa are scarce and from Lesotho non-existent. This study aims to examine the occurrence, characteristics and etiology of heart failure and heart diseases using echocardiography data from a referral hospital in rural Lesotho. METHODS: We conducted a retrospective cohort study at Seboche Mission Hospital, the only referral hospital in Butha-Buthe district (Lesotho) with an echocardiography department. We included data from all individuals referred to the department between January 2020 and May 2021. From non-hospitalized patients echocardiographic diagnosis, sex and age were available, from hospitalized patients additional sociodemographic and clinical data could be extracted. RESULTS: In the study period, a total of 352 echocardiograms were conducted; 213 had abnormal findings (among them 3 children). The majority of adult participants (130/210; 64%) were female and most frequent heart diseases were hypertensive (62/210, 30%), valvular (39/210, 19%) and chronic pulmonary (37/210, 18%). Heart failure represented 11% of hospitalizations in the same period. Among the 126 hospitalized heart failure patients, the most common etiology was chronic pulmonary heart disease (32/126; 25%). Former mine workers and people with a history of tuberculosis were more likely to have a chronic pulmonary heart disease. CONCLUSIONS: The leading cause of heart disease in this setting is hypertension. However, in contrast to other African epidemiological studies, chronic pulmonary heart disease is unexpectedly common. There is an urgent need to improve awareness and knowledge about lung diseases, make diagnostic and therapeutic options available and increase prevention.

The Effects of Sequential Ventilation Therapy on Blood Gas Indexes, Pulmonary Function Indexes, Clinical Efficacy, and Safety in Patients with Severe Cor Pulmonale.

As a heart disease caused by pulmonary arterial hypertension, pulmonary heart disease has a high incidence and poor clinical prognosis in the elderly. Including mechanical ventilation in the current clinical treatment of cor pulmonale, with the development of medical technology, traditional invasive mechanical ventilation has been pointed out to have certain clinical limitations, so new invasive and noninvasive sequential ventilation treatments are gradually being used. Applied in clinical practice, this article selected 96 patients with severe cor pulmonale in our hospital from January 2020 to May 2021 as the research object and conducted a randomized prospective study. The experimental results show that the two general methods can improve the blood gas index and pulmonary function index of patients with heart disease and pulmonary dysfunction, but the improvement of the above indexes by the sequence of action of gas therapy is better than that of conventional invasive mechanical ventilation.

Cardiac function in critically ill patients with severe COVID: A prospective cross-sectional study in mechanically ventilated patients.

PURPOSE: To evaluate cardiac function in mechanically ventilated patients with COVID-19. MATERIALS AND METHODS: Prospective, cross-sectional multicenter study in four university-affiliated hospitals in Chile. All consecutive patients with COVID-19 ARDS requiring mechanical ventilation admitted between April and July 2020 were included. We performed systematic transthoracic echocardiography assessing right and left ventricular function within 24 h of intubation. RESULTS: 140 patients aged 57 ± 11, 29% female were included. Cardiac output was 5.1 L/min [IQR 4.5-6.2] and 86% of the patients required norepinephrine. ICU mortality was 29% (40 patients). Fifty-four patients (39%) exhibited right ventricle dilation out of whom 20 patients (14%) exhibited acute cor pulmonale (ACP). Eight out of the twenty patients with ACP exhibited pulmonary embolism (40%). Thirteen patients (9%) exhibited left ventricular systolic dysfunction (ejection fraction <45%). In the multivariate analysis acute cor pulmonale and PaO2/FiO2 ratio were independent predictors of ICU mortality. CONCLUSIONS: Right ventricular dilation is highly prevalent in mechanically ventilated patients with COVID-19 ARDS. Acute cor pulmonale was associated with reduced pulmonary function and, in only 40% of patients, with co-existing pulmonary embolism. Acute cor pulmonale is an independent risk factor for ICU mortality.

Predictors of Prolonged Hospital Stay in Patients with Acute Pulmonary Thromboembolism- A Hospital Based Cohort Study.

AIMS AND OBJECTIVES: Patients with pulmonary thromboembolism (PTE) are commonly admitted to hospital and generally have a prolonged hospital stay in this part of the world. We aimed to determine different clinical and laboratory parameters that are associated with prolonged hospital stay in our set-up and to analyse effectiveness of Pulmonary Embolism Severity Index (PESI) score as a predictor of prolonged hospital stay in patients with PTE. MATERIALS AND METHODS: It was a hospital based observational prospective study. Confirmed cases of PTE defined as patients with evidence of thrombus on CT pulmonary angiogram (CTPA) were included in this study. Depending on the length of hospital stay, patients were divided into two cohorts: Shorter Hospital stay (less than mean i.e., < 10 Days) and Prolonged Hospital stay (longer than mean i.e., ≥ 10 Days). Logistic regression analysis was done to identify predictors of prolonged hospital stay. RESULTS: 150 patients were included in the study with 67 patients (44.67%) having shorter hospital stay (<10 days) and 83 patients (55.33%) having prolonged hospital stay (≥10 days). On multivariate regression analysis, parameters that were found to be statistically significant were hypotension at presentation, decreased level of consciousness, pco2 < 30 mmHg, presence of S1Q3T3 pattern on electrocardiogram (ECG) and high risk PESI (class III-V). CONCLUSION: PESI class can be effectively used to predict prolonged hospital stay in patients with pulmonary embolism. Patients with hypotension at presentation, decreased level of consciousness, pco2 less than 30 mmHg, and S1Q3T3 on ECG are more likely to have prolonged hospital stay in our healthcare setup.

Saddle pulmonary embolism in a pediatric patient with nephrotic syndrome and recent COVID-19 pneumonia: A case report.

The pediatric population accounts for a small portion of those with severe disease related to COVID-19. There are few published reports of hypercoagulable states in children with COVID-19. We describe an 11-year-old male with nephrotic syndrome who required inpatient treatment for COVID-19 pneumonia eight weeks prior. He returned to the emergency department with vomiting, tachypnea and was found to have a pulmonary embolism. In this case report, we discuss the risk factors for, presentation and evaluation of hypercoagulable state and its relation to COVID-19 in a pediatric patient.

The relationship between serum uric acid level and uric acid/creatinine ratio with chronic obstructive pulmonary disease severity (stable or acute exacerbation) and the development of cor pulmonale.

BACKGROUND: There are studies reporting that uric acid elevation is a marker for hypoxemia and pulmonary hypertension secondary to some diseases. AIM: The aim of this study is to investigate the relationship between serum uric acid level and uric acid/creatinine ratio with chronic obstructive pulmonary disease (COPD) exacerbation, hypoxemia in exacerbation and development of cor pulmonale. METHODS: A total of 96 COPD patients who were admitted to Ondokuz Mayis University Faculty of Medicine emergency department and Chest Diseases outpatient clinic and whose written consent was obtained were included in our study. Forty-three of these patients were in the period of exacerbation (Group 1), and 53 were in the stable period (Group 2). Complete blood count, blood biochemistry (including serum uric acid level) and arterial blood gas analysis were performed in our patients. In addition, spirometry and echocardiography findings were examined. RESULTS: Serum uric acid level of patients in the period of exacerbation group (Group 1) was 6.97 ± 1.34 and in stable COPD group (Group 2) was 4.30 ± 1.01 (P < .05). Uric acid/creatinine ratios in Group 1 was 8.00 ± 2.06; in Group 2, it was 5.52 ± 1.57 (P < .05). In patients with hypoxemia, serum uric acid level and uric acid/creatinine ratio were significantly higher than nonhypoxemic patients (P < .05). Serum uric acid level and serum uric acid/creatinine ratio of Group 1 were significantly higher than Group 2 (P < .001). Serum uric acid level and serum uric acid/creatinine ratio of patients who developed cor pulmonale were significantly higher than patients without cor pulmonale (P < .05). CONCLUSION: Serum uric acid level and uric acid/creatinine ratio were found to be higher in patients with exacerbation of COPD and those developing cor pulmonale. Consequently, it suggests that serum uric acid level and serum uric acid/creatinine ratio may be a stimulating laboratory test for the severity of COPD and the development of COPD induced cor pulmonale.

Usefulness of Right Ventricular Longitudinal Shortening Fraction to Detect Right Ventricular Dysfunction in Acute Cor Pulmonale Related to COVID-19.

OBJECTIVE: To compare two-dimensional-speckle tracking echocardiographic parameters (2D-STE) and classic echocardiographic parameters of right ventricular (RV) systolic function in patients with coronavirus disease 2019 (COVID-19)-related acute respiratory distress syndrome (CARDS) complicated or not by acute cor pulmonale (ACP). DESIGN: Prospective, between March 1, 2020 and April 15, 2020. SETTING: Intensive care unit of Amiens University Hospital (France). PARTICIPANTS: Adult patients with moderate-to-severe CARDS under mechanical ventilation for fewer than 24 hours. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Tricuspid annular displacement (TAD) parameters (TAD-septal, TAD-lateral, and RV longitudinal shortening fraction [RV-LSF]), RV global longitudinal strain (RV-GLS), and RV free wall longitudinal strain (RVFWLS) were measured using transesophageal echocardiography with a dedicated software and compared with classic RV systolic parameters (RV-FAC, S' wave, and tricuspid annular plane systolic excursion [TAPSE]). RV systolic dysfunction was defined as RV-FAC <35%. Twenty-nine consecutive patients with moderate-to-severe CARDS were included. ACP was diagnosed in 12 patients (41%). 2D-STE parameters were markedly altered in the ACP group, and no significant difference was found between patients with and without ACP for classic RV parameters (RV-FAC, S' wave, and TAPSE). In the ACP group, RV-LSF (17% [14%-22%]) had the best correlation with RV-FAC (r = 0.79, p < 0.001 v r = 0.27, p = 0.39 for RVGLS and r = 0.28, p = 0.39 for RVFWLS). A RV-LSF cut-off value of 17% had a sensitivity of 80% and a specificity of 86% to identify RV systolic dysfunction. CONCLUSIONS: Classic RV function parameters were not altered by ACP in patients with CARDS, contrary to 2D-STE parameters. RV-LSF seems to be a valuable parameter to detect early RV systolic dysfunction in CARDS patients with ACP.

Novel Coronavirus-Induced Right Ventricular Failure and Point of Care Echocardiography: A Case Report.

Various cardiovascular complications have been reported in patients with coronavirus disease 2019. Common complications include acute myocardial injury, myocarditis, arrhythmia, pericarditis, heart failure, and shock. We present a case of cor pulmonale diagnosed with serial point of care ultrasound. Given the current shortage of personal protective equipment (PPE) and high infectivity of this virus, we acknowledge the utility of this tool in obtaining important clinical information while minimizing exposure and PPE consumption.

Endothelial-specific Loss of IFT88 Promotes Endothelial-to-Mesenchymal Transition and Exacerbates Bleomycin-induced Pulmonary Fibrosis.

Intraflagellar transport protein 88 (Ift88) is required for ciliogenesis and shear stress-induced dissolution of cilia in embryonic endothelial cells coincides with endothelial-to-mesenchymal transition (EndMT) in the developing heart. EndMT is also suggested to underlie heart and lung fibrosis, however, the mechanism linking endothelial Ift88, its effect on EndMT and organ fibrosis remains mainly unexplored. We silenced Ift88 in endothelial cells (ECs) in vitro and generated endothelial cell-specific Ift88-knockout mice (Ift88endo) in vivo to evaluate EndMT and its contribution towards organ fibrosis, respectively. Ift88-silencing in ECs led to mesenchymal cells-like changes in endothelial cells. The expression level of the endothelial markers (CD31, Tie-2 and VE-cadherin) were significantly reduced with a concomitant increase in the expression level of mesenchymal markers (αSMA, N-Cadherin and FSP-1) in Ift88-silenced ECs. Increased EndMT was associated with increased expression of profibrotic Collagen I expression and increased proliferation in Ift88-silenced ECs. Loss of Ift88 in ECs was further associated with increased expression of Sonic Hedgehog signaling effectors. In vivo, endothelial cells isolated from the heart and lung of Ift88endo mice demonstrated loss of Ift88 expression in the endothelium. The Ift88endo mice were born in expected Mendelian ratios without any adverse cardiac phenotypes at baseline. Cardiac and pulmonary endothelial cells isolated from the Ift88endo mice demonstrated signs of EndMT and bleomycin treatment exacerbated pulmonary fibrosis in Ift88endo mice. Pressure overload stress in the form of aortic banding did not reveal a significant difference in cardiac fibrosis between Ift88endo mice and control mice. Our findings demonstrate a novel association between endothelial cilia with EndMT and cell proliferation and also show that loss of endothelial cilia-associated increase in EndMT contributes specifically towards pulmonary fibrosis.

[Cor pulmonale]. / Cœur pulmonaire.

Cor pulmonale is a disease of the heart characterised by dilatation of the right ventricle and paradoxical movement of the interventricular septum. The diagnosis depends on echocardiography even if pulmonary artery catheterisation suggests it. It is secondary to pulmonary disease or a disorder of the pulmonary circulation. These two mechanisms, which are often connected, involve pulmonary hypertension as the origin of a systolic and diastolic overload of the right ventricle, which then leads to the alterations of its structure and performance. Acute cor pulmonale is usually secondary to an acute respiratory distress syndrome or to a pulmonary embolism but it can also be seen in primary lactic acidosis, a vaso-occlusive crisis in a patient with sickle cell anaemia, severe acute asthma, and entry of air or injected crushed tablets into the circulation. Chronic cor pulmonale is the terminal stage of pulmonary hypertension. Clinically these patients are dyspnoeic with signs of chronic right heart failure. They should have an echocardiogram confirming the cardiac involvement. Certain precipitating factors, such as infection of any origin, have been reported, leading to acute on chronic cor pulmonale that has a particularly high mortality.