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The Journal of Thoracic and... Oct 2021
Topics: Fontan Procedure; Hemodynamics; Homeostasis; Humans; Models, Cardiovascular; Phosphodiesterase 5 Inhibitors; Postoperative Complications; Pulmonary Circulation; Treatment Outcome; Univentricular Heart; Vascular Resistance
PubMed: 33618865
DOI: 10.1016/j.jtcvs.2020.08.114 -
Giornale Italiano Di Cardiologia (2006) Aug 2020The new coronavirus disease 2019 (COVID-19), which is causing hundreds of thousands of deaths worldwide, is complex and can present with a multi-organ localization. One... (Review)
Review
The new coronavirus disease 2019 (COVID-19), which is causing hundreds of thousands of deaths worldwide, is complex and can present with a multi-organ localization. One of its worst complications is an interstitial pneumonia with acute respiratory failure also known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which requires non-invasive or invasive ventilation. A severe coagulopathy with poor prognosis is found in 5-10% of cases. SARS-CoV-2 is manifesting as a multi-dimensional disease and, recently, unique co-existing pathophysiological and clinical aspects are being defined: (i) an increased immune and inflammatory response with the activation of a cytokine storm and consequent coagulopathy, which promote both venous thromboembolic events and in situ thrombosis localized in small arterioles and pulmonary alveolar capillaries; (ii) a high intrapulmonary shunt, which often accounts for the severity of respiratory failure, due to reduced hypoxic pulmonary vasoconstriction with pulmonary neo-angiogenetic phenomena. Furthermore, the high incidence of venous thromboembolism in COVID-19 patients admitted to the intensive care unit and the autoptic findings of in situ micro-thrombosis at the pulmonary vascular level, suggest that in this disease coagulopathy, unlike septic disseminated intravascular coagulation, is driven towards a hyper-thrombogenic state, giving rise to a debate (with ongoing studies) about the preventive use of anticoagulant doses of heparin to reduce mortality. The aim of this position paper from the Italian Association of Hospital Cardiologists (ANMCO) is to highlight the main implications that COVID-19 infection has on the pulmonary circulation from a pathophysiological, clinical and management point of view.
Topics: COVID-19; Cardiology; Cause of Death; Communicable Diseases, Emerging; Coronavirus Infections; Disease Progression; Female; Humans; Incidence; Italy; Lung Diseases, Interstitial; Male; Pandemics; Pneumonia, Viral; Pulmonary Circulation; Risk Assessment; Severe Acute Respiratory Syndrome; Societies, Medical; Survival Analysis; Venous Thromboembolism
PubMed: 32686781
DOI: 10.1714/3405.33888 -
Current Opinion in Pulmonary Medicine Sep 2021The coronavirus disease 2019 (COVID-19) pandemic has led to almost 3,000,000 deaths across 139 million people infected worldwide. Involvement of the pulmonary... (Review)
Review
PURPOSE OF REVIEW
The coronavirus disease 2019 (COVID-19) pandemic has led to almost 3,000,000 deaths across 139 million people infected worldwide. Involvement of the pulmonary vasculature is considered a major driving force for morbidity and mortality. We set out to summarize current knowledge on the acute manifestations of pulmonary vascular disease (PVD) resulting from COVID-19 and prioritize long-term complications that may result in pulmonary hypertension (PH).
RECENT FINDINGS
Acute COVID-19 infection can result in widespread involvement of the pulmonary vasculature, myocardial injury, evidence of persistent lung disease, and venous thromboembolism. Post COVID-19 survivors frequently report ongoing symptoms and may be at risk for the spectrum of PH, including group 1 pulmonary arterial hypertension, group 2 PH due to left heart disease, group 3 PH due to lung disease and/or hypoxia, and group 4 chronic thromboembolic PH.
SUMMARY
The impact of COVID-19 on the pulmonary vasculature is central to determining disease severity. Although the long-term PVD manifestations of COVID-19 are currently uncertain, optimizing the care of risk factors for PH and monitoring for the development of PVD will be critical to reducing long-term morbidity and improving the health of survivors.
Topics: COVID-19; Humans; Lung Diseases; Pandemics; Pulmonary Circulation; SARS-CoV-2; Vascular Diseases
PubMed: 34127622
DOI: 10.1097/MCP.0000000000000792 -
Pediatrics Mar 2021The transition from intrauterine life to extrauterine existence encompasses significant cardiorespiratory adaptations. These include rapid lung aeration and increase in... (Review)
Review
The transition from intrauterine life to extrauterine existence encompasses significant cardiorespiratory adaptations. These include rapid lung aeration and increase in pulmonary blood flow (PBF). Perinatal asphyxia and fetal growth restriction can severely hamper this transition. Hypoxia is the common denominator in these 2 disease states, with the former characterized by acute insult and the latter by utero-placental insufficiency and a chronic hypoxemic state. Both may manifest as hemodynamic instability. In this review, we emphasize the role of physiologic-based cord clamping in supplementing PBF during transition. The critical role of lung aeration in initiating pulmonary gas exchange and increasing PBF is discussed. Physiologic studies in animal models have enabled greater understanding of the mechanisms and effects of various therapies on transitional circulation. With data from sheep models, we elaborate instrumentation for monitoring of cardiovascular and pulmonary physiology and discuss the combined effect of chest compressions and adrenaline in improving transition at birth. Lastly, physiologic adaptation influencing management in human neonatal cohorts with respect to cardiac and vascular impairments in hypoxic-ischemic encephalopathy and growth restriction is discussed. Impairments in right ventricular function and vascular mechanics hold the key to prognostication and understanding of therapeutic rationale in these critically ill cohorts. The right ventricle and pulmonary circulation seem to be especially affected and may be explored as therapeutic targets. The role of comprehensive assessments using targeted neonatal echocardiography as a longitudinal, reliable, and easily accessible tool, enabling precision medicine facilitating physiologically appropriate treatment choices, is discussed.
Topics: Acute Disease; Adaptation, Physiological; Animals; Cardiac Output; Cardiac Output, Low; Chronic Disease; Constriction; Female; Fetal Development; Fetal Growth Retardation; Fetus; Humans; Hypoxia-Ischemia, Brain; Infant, Newborn; Models, Animal; Parturition; Placental Insufficiency; Precision Medicine; Pregnancy; Pulmonary Circulation; Pulmonary Gas Exchange; Respiration; Respiratory Therapy; Sheep; Ventricular Dysfunction, Right
PubMed: 33622795
DOI: 10.1542/peds.2020-016972 -
European Heart Journal. Cardiovascular... May 2021To explore the effects of age and sex in adolescents vs. young or middle-aged adults on pulmonary vascular function and right ventricular-arterial (RV-PA) coupling as...
AIMS
To explore the effects of age and sex in adolescents vs. young or middle-aged adults on pulmonary vascular function and right ventricular-arterial (RV-PA) coupling as assessed by exercise stress echocardiography.
METHODS AND RESULTS
Forty healthy adolescents aged 12-15 years were compared with 40 young adults aged 17-22 years and 40 middle-aged adults aged 30-50 years. Sex distribution was equal in the three groups. All the subjects underwent an exercise stress echocardiography. A pulmonary vascular distensibility coefficient α was determined from multipoint pulmonary vascular pressure-flow relationships. RV-PA coupling was assessed by the tricuspid annular plane systolic excursion (TAPSE) to systolic pulmonary artery pressure (PASP) ratio, who has been previously validated by invasive study. While cardiac index and mean PAP were not different, adolescents compared to young and middle-aged adults, respectively had higher pulmonary vascular distensibility coefficients α (1.60 ± 0.31%/mmHg vs. 1.39 ± 0.29%/mmHg vs. 1.20 ± 0.35%/mmHg, P < 0.00001). Adolescents and young adults compared to middle-aged adults, respectively had higher TAPSE/PASP ratios at rest (1.24 ± 0.18 mm/mmHg and 1.22 ± 0.17 mm/mmHg vs. 1.07 ± 0.18 mm/mmHg, P < 0.008) and during exercise (0.86 ± 0.24, 0.80 ± 0.15 and 0.72 ± 0.15 mm/mmHg, P < 0.04). The TAPSE/PASP ratio decreased with exercise. There were no sex differences in α or TAPSE/PASP.
CONCLUSION
Compared to adults, adolescents present with a sex-independent more distensible pulmonary circulation. Resting and exercise RV-PA coupling is decreased in middle-aged adults.
Topics: Adolescent; Echocardiography, Doppler; Echocardiography, Stress; Humans; Middle Aged; Prospective Studies; Pulmonary Artery; Pulmonary Circulation; Ventricular Dysfunction, Right; Ventricular Function, Right; Young Adult
PubMed: 32380528
DOI: 10.1093/ehjci/jeaa085 -
Journal of the American College of... Aug 2022PVDOMICS (Pulmonary Vascular Disease Phenomics) is a precision medicine initiative to characterize pulmonary vascular disease (PVD) using deep phenotyping. PVDOMICS...
BACKGROUND
PVDOMICS (Pulmonary Vascular Disease Phenomics) is a precision medicine initiative to characterize pulmonary vascular disease (PVD) using deep phenotyping. PVDOMICS tests the hypothesis that integration of clinical metrics with omic measures will enhance understanding of PVD and facilitate an updated PVD classification.
OBJECTIVES
The purpose of this study was to describe clinical characteristics and transplant-free survival in the PVDOMICS cohort.
METHODS
Subjects with World Symposium Pulmonary Hypertension (WSPH) group 1-5 PH, disease comparators with similar underlying diseases and mild or no PH and healthy control subjects enrolled in a cross-sectional study. PH groups, comparators were compared using standard statistical tests including log-rank tests for comparing time to transplant or death.
RESULTS
A total of 1,193 subjects were included. Multiple WSPH groups were identified in 38.9% of PH subjects. Nocturnal desaturation was more frequently observed in groups 1, 3, and 4 PH vs comparators. A total of 50.2% of group 1 PH subjects had ground glass opacities on chest computed tomography. Diffusing capacity for carbon monoxide was significantly lower in groups 1-3 PH than their respective comparators. Right atrial volume index was higher in WSPH groups 1-4 than comparators. A total of 110 participants had a mean pulmonary artery pressure of 21-24 mm Hg. Transplant-free survival was poorest in group 3 PH.
CONCLUSIONS
PVDOMICS enrolled subjects across the spectrum of PVD, including mild and mixed etiology PH. Novel findings include low diffusing capacity for carbon monoxide and enlarged right atrial volume index as shared features of groups 1-3 and 1-4 PH, respectively; unexpected, frequent presence of ground glass opacities on computed tomography; and sleep alterations in group 1 PH, and poorest survival in group 3 PH. PVDOMICS will facilitate a new understanding of PVD and refine the current PVD classification. (Pulmonary Vascular Disease Phenomics Program PVDOMICS [PVDOMICS]; NCT02980887).
Topics: Carbon Monoxide; Cross-Sectional Studies; Humans; Hypertension, Pulmonary; Pulmonary Circulation; Vascular Diseases
PubMed: 35953136
DOI: 10.1016/j.jacc.2022.05.038 -
PloS One 2022Percutaneous patent ductus arteriosus (PDA) stenting is a therapeutic modality in patients with duct-dependent pulmonary circulation with reported success rates from...
OBJECTIVES
Percutaneous patent ductus arteriosus (PDA) stenting is a therapeutic modality in patients with duct-dependent pulmonary circulation with reported success rates from 80-100%. The current study aims to assess the outcome and the indicators of success for PDA stenting in different ductal morphologies using various approaches.
METHODS
A prospective cohort study from a single tertiary center presented from January 2018 to December 2019 that included 96 consecutive infants with ductal-dependent pulmonary circulation and palliated with PDA stenting. Patients were divided according to PDA origin into 4 groups: Group 1: PDA from proximal descending aorta, Group 2: from undersurface of aortic arch, Group 3: opposite the subclavian artery, Group 4: opposite the innominate/brachiocephalic artery.
RESULTS
The median age of patients was 22 days and median weight was 3 kg. The procedure was successful in 78 patients (81.25%). PDA was tortuous in 70 out of 96 patients. Femoral artery was the preferred approach in Group 1 (63/67), while axillary artery access was preferred in the other groups (6/11 in Group 2, 11/17 in Group 3, 1/1 in Group 4, P <0.0001). The main cause of procedural failure was inadequate parked coronary wire inside one of the branch of pulmonary arteries (14 cases; 77.7%), while 2 cases (11.1%) were complicated by acute stent thrombosis, and another 2 cases with stent dislodgment. Other procedural complications comprised femoral artery thrombosis in 7 cases (7.2%). Patients with straight PDA, younger age at procedure and who had larger PDA at pulmonary end had higher odds for success (OR = 8.01, 2.94, 7.40, CI = 1.011-63.68, 0.960-0.99, 1.172-7.40,respectively, P = 0.048, 0.031,0.022 respectively).
CONCLUSIONS
The approach for PDA stenting and hence the outcome is markedly determined by the PDA origin and morphology. Patients with straight PDA, younger age at procedure and those who had relatively larger PDA at the pulmonary end had better opportunity for successful procedure.
Topics: Cardiac Catheterization; Ductus Arteriosus, Patent; Humans; Infant; Prospective Studies; Pulmonary Circulation; Retrospective Studies; Stents; Treatment Outcome
PubMed: 35421117
DOI: 10.1371/journal.pone.0265031 -
Seminars in Respiratory and Critical... Oct 2023
Review
Topics: Humans; Hypertension, Pulmonary; Lung; Pulmonary Artery; Pulmonary Circulation
PubMed: 37816343
DOI: 10.1055/s-0043-1771162 -
Journal of the American College of... Apr 2021The National Heart, Lung, and Blood Institute and the Cardiovascular Medical Research and Education Fund held a workshop on the application of pulmonary vascular disease... (Review)
Review
The National Heart, Lung, and Blood Institute and the Cardiovascular Medical Research and Education Fund held a workshop on the application of pulmonary vascular disease omics data to the understanding, prevention, and treatment of pulmonary vascular disease. Experts in pulmonary vascular disease, omics, and data analytics met to identify knowledge gaps and formulate ideas for future research priorities in pulmonary vascular disease in line with National Heart, Lung, and Blood Institute Strategic Vision goals. The group identified opportunities to develop analytic approaches to multiomic datasets, to identify molecular pathways in pulmonary vascular disease pathobiology, and to link novel phenotypes to meaningful clinical outcomes. The committee suggested support for interdisciplinary research teams to develop and validate analytic methods, a national effort to coordinate biosamples and data, a consortium of preclinical investigators to expedite target evaluation and drug development, longitudinal assessment of molecular biomarkers in clinical trials, and a task force to develop a master clinical trials protocol for pulmonary vascular disease.
Topics: Biomedical Research; Cardiovascular Diseases; Computational Biology; Education; Humans; Lung Diseases; National Heart, Lung, and Blood Institute (U.S.); Pulmonary Circulation; Research Report; Review Literature as Topic; United States; Vascular Diseases
PubMed: 33888254
DOI: 10.1016/j.jacc.2021.02.056 -
European Respiratory Review : An... Mar 2022Pulmonary arterial pressure rises minimally during exercise. The pulmonary microcirculation accommodates increasing blood flow recruitment of pulmonary capillaries and,... (Review)
Review
Pulmonary arterial pressure rises minimally during exercise. The pulmonary microcirculation accommodates increasing blood flow recruitment of pulmonary capillaries and, at higher flows, by distention of already perfused capillaries. The flow transition range between recruitment and distention has not been studied or compared across mammalian species, including humans. We hypothesised that the range would be similar. Functional pulmonary capillary surface area (FCSA) can be estimated using validated metabolic techniques. We reviewed data from previous studies in three mammalian species (perfused rabbit lungs and dog lung lobes, and exercising humans) and generated blood flow-FCSA curves over a range of flows. We noted where the curves diverged from the theoretical line of pure recruitment (Recruitment) and determined the flow where the curve slope equalled 50% that of Recruitment, or equalled that of a theoretical curve representing full capillary distention (Distention). The three mammalian species have similar flow ranges for the transition from predominantly recruitment to predominantly distention, with dogs having the highest transition point. Within the physiological range of most daily activity, the species are similar and accommodate increasing blood flow mainly recruitment, with progressive distention at higher flows. This is highly relevant to pulmonary physiology during exercise.
Topics: Animals; Blood Pressure; Capillaries; Dogs; Hemodynamics; Humans; Lung; Pulmonary Circulation; Rabbits
PubMed: 35197268
DOI: 10.1183/16000617.0248-2021