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Clinics in Perinatology Mar 2024
Topics: Infant, Newborn; Humans; Animals; Hypertension, Pulmonary; Animals, Newborn
PubMed: 38325951
DOI: 10.1016/j.clp.2023.12.004 -
Deutsches Arzteblatt International Dec 2023Up to 1% of the world population and 10% of all persons over age 65 suffer from pulmonary hypertension (PH). The latency from the first symptom to the diagnosis is more... (Review)
Review
BACKGROUND
Up to 1% of the world population and 10% of all persons over age 65 suffer from pulmonary hypertension (PH). The latency from the first symptom to the diagnosis is more than one year on average, and more than three years in 20% of patients. 40% seek help from more than four different physicians until their condition is finally diagnosed.
METHODS
This review is based on publications retrieved by a selective literature search on pulmonary hypertension.
RESULTS
The most common causes of pulmonary hypertension are left heart diseases and lung diseases. Its cardinal symptom is exertional dyspnea that worsens as the disease progresses. Additional symptoms of right heart failure are seen in advanced stages. Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are rare, difficult to diagnose, and of particular clinical relevance because specific treatments are available. For this reason, strategies for the early detection of PAH and CTEPH have been developed. The clinical suspicion of PH arises in a patient who has nonspecific symptoms, electrocardiographic changes, and an abnormal (NT-pro-)BNP concentration. Once the suspicion of PH has been confirmed by echocardiography and, if necessary, differential-diagnostic evaluation with a cardiopulmonary stress test, and after the exclusion of a primary left heart disease or lung disease, the patient should be referred to a PH center for further diagnostic assessment, classification, and treatment.
CONCLUSION
If both the (NT-pro-)BNP and the ECG are normal, PH is unlikely. Knowledge of the characteristic clinical manifestations and test results of PH is needed so that patients can be properly selected for referral to specialists and experts in PH.
Topics: Humans; Aged; Hypertension, Pulmonary; Exercise Test; Dyspnea; Echocardiography; Heart Diseases; Pulmonary Embolism; Chronic Disease
PubMed: 37882345
DOI: 10.3238/arztebl.m2023.0222 -
Seminars in Respiratory and Critical... Dec 2023The management of acute medical emergencies in patients with pulmonary hypertension (PH) can be challenging. Patients with preexisting PH can rapidly deteriorate due to... (Review)
Review
The management of acute medical emergencies in patients with pulmonary hypertension (PH) can be challenging. Patients with preexisting PH can rapidly deteriorate due to right ventricular decompensation when faced with acute physiological challenges that would usually be considered low-risk scenarios. This review considers the assessment and management of acute medical emergencies in patients with PH, encompassing both pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH), acknowledging these comprise the more severe groups of PH. Management protocols are described in a systems-based approach. Respiratory emergencies include pulmonary embolism, airways disease, and pneumonia; cardiac emergencies including arrhythmia and chest pain with acute myocardial infarction are discussed, alongside PH-specific emergencies such as pulmonary artery dissection and extrinsic coronary artery compression by a dilated proximal pulmonary artery. Other emergencies including sepsis, severe gastroenteritis with dehydration, syncope, and liver failure are also considered. We propose management recommendations for medical emergencies based on available evidence, international guidelines, and expert consensus. We aim to provide advice to the specialist alongside the generalist, and emergency doctors, nurses, and acute physicians in nonspecialist centers. A multidisciplinary team approach is essential in the management of patients with PH, and communication with local and specialist PH centers is paramount. Close hemodynamic monitoring during medical emergencies in patients with preexisting PH is vital, with early referral to critical care recommended given the frequent deterioration and high mortality in this setting.
Topics: Humans; Hypertension, Pulmonary; Emergencies; Lung; Pulmonary Embolism; Pulmonary Artery; Chronic Disease
PubMed: 37595615
DOI: 10.1055/s-0043-1770120 -
Chest Oct 2023Even though patients with pulmonary arterial hypertension have multiple therapeutic options, the disease can be refractory despite appropriate management. In patients... (Review)
Review
TOPIC IMPORTANCE
Even though patients with pulmonary arterial hypertension have multiple therapeutic options, the disease can be refractory despite appropriate management. In patients with end-stage pulmonary arterial hypertension, lung transplantation has the potential both to extend survival and improve health-related quality of life. Pulmonary arterial hypertension is the only major diagnostic indication for transplantation that is not a parenchymal pulmonary process, and thus the care of these patients is unique.
REVIEW FINDINGS
This review focuses on the complexities of lung transplantation for patients with pulmonary arterial hypertension, presents the updated referral and listing criteria, and discusses the inequities in the organ allocation process that impact this disease group and the strategies to optimize outcomes for patients with pulmonary arterial hypertension who require lung transplantation.
SUMMARY
Lung transplantation is an effective and lifesaving therapy for patients with end-stage lung disease. Sadly, patients with pulmonary arterial hypertension face many challenges as it relates to transplantation including higher perioperative risks, inequities in the allocation system, and less favorable long-term outcomes. This review covers the complexities of transplantation in patients with pulmonary vascular disease.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Quality of Life; Lung Transplantation; Familial Primary Pulmonary Hypertension
PubMed: 37150504
DOI: 10.1016/j.chest.2023.04.047 -
American Journal of Respiratory and... Aug 2023
Topics: Humans; Hypertension, Pulmonary; Lung Diseases, Interstitial
PubMed: 37343302
DOI: 10.1164/rccm.202306-1005ED -
Methodist DeBakey Cardiovascular Journal 2024Pulmonary arterial hypertension (PAH) is a rare devastating disease characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance.... (Review)
Review
Pulmonary arterial hypertension (PAH) is a rare devastating disease characterized by elevated pulmonary artery pressure and increased pulmonary vascular resistance. Females have a higher incidence of PAH, which is reflected globally across registries in the United States, Europe, and Asia. However, despite female predominance, women had better outcomes compared with male patients, a finding that has been labeled the "estrogen paradox." Special considerations should be given to women with PAH regarding sexual health, contraception, family planning, and treatment before, during, and after pregnancy. Pregnant women with PAH should be referred to a pulmonary hypertension care center; a multidisciplinary team approach is recommended, and Cesarean section is the preferred mode of delivery. While pregnancy outcomes have improved over the years with PAH-specific therapy, pregnancy portends a high-risk for those with PAH. Continued research is needed to tailor PAH treatment for women.
Topics: Female; Pregnancy; Humans; Male; Hypertension, Pulmonary; Cesarean Section; Pregnancy Outcome; Familial Primary Pulmonary Hypertension; Hypertension
PubMed: 38495664
DOI: 10.14797/mdcvj.1308 -
Current Opinion in Pulmonary Medicine Sep 2023The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial... (Review)
Review
PURPOSE OF REVIEW
The purpose of this review is to provide an overview of assessment of right ventricular function in the context of pulmonary hypertension and pulmonary arterial hypertension (PAH). We will review unique features of right ventricular anatomy, delineation of cause of pulmonary hypertension through careful right ventricular assessment, echocardiographic and hemodynamic evaluation, and the importance of this assessment in prognosis.
RECENT FINDINGS
The importance of performance in prognosis and risk assessment in patients with pulmonary hypertension has been continually emphasized in ongoing research. Representative parameters of right ventricular function have been shown to be predictive of prognosis in patients with pulmonary hypertension. Further, the importance of serial right ventricular assessment in risk assessment and prognosis has remained an emerging theme.
SUMMARY
Careful evaluation of right ventricular function is paramount in assessing the cause of pulmonary hypertension and severity of disease. Further, it has prognostic significance, as many representative parameters of right ventricular function have been linked with mortality. In our opinion, right ventricular function should be assessed serially throughout the course of treatment in pulmonary hypertension, and baseline parameters in addition to dynamic changes should be incorporated into risk assessment. Achieving normal or near-normal right ventricular performance may serve as a principal goal in the treatment of pulmonary hypertension.
Topics: Humans; Hypertension, Pulmonary; Pulmonary Arterial Hypertension; Prognosis; Familial Primary Pulmonary Hypertension; Echocardiography; Ventricular Dysfunction, Right
PubMed: 37410491
DOI: 10.1097/MCP.0000000000000980 -
Cardiology Clinics May 2024Pulmonary hypertension is a challenging disease entity with various underlying etiologies. The management of patients with pulmonary arterial hypertension (WHO Group 1)... (Review)
Review
Pulmonary hypertension is a challenging disease entity with various underlying etiologies. The management of patients with pulmonary arterial hypertension (WHO Group 1) remains challenging especially in the critical care setting. With risk of high morbidity and mortality, these patients require a multidisciplinary team approach at a speciality care facility for pulmonary hypertension for comprehensive evaluation and rapid initiation of treatment. For acute decompensated right heart failure, management should concentrate on optimizing preload and after load with use of pulmonary vasodilator therapy. A careful evaluation of specialized situations is required for appropriate treatment response.
Topics: Humans; Hypertension, Pulmonary; Emergencies; Pulmonary Arterial Hypertension; Vasodilator Agents; Critical Care; Heart Failure
PubMed: 38631794
DOI: 10.1016/j.ccl.2024.02.011 -
Seminars in Respiratory and Critical... Dec 2023Pulmonary arterial hypertension is a severe life-threatening condition associated with increased pulmonary vascular resistance and resulting right heart dysfunction....
Pulmonary arterial hypertension is a severe life-threatening condition associated with increased pulmonary vascular resistance and resulting right heart dysfunction. Admission to intensive care unit with acutely decompensated right heart failure is a significant negative prognostic event with a high risk of multisystem organ dysfunction and death. Presentations are heterogenous and may combine signs of both diastolic and systolic dysfunction complicating management. Renal dysfunction is often present, but other organ systems can be involved resulting in findings such as acute hepatic dysfunction or bowel wall congestion and ischemia. The goals of therapy are to rapidly reverse ventriculo-arterial decoupling and reduce right ventricular afterload to prevent progression to refractory or irreversible right heart failure. Triggering events must be investigated for and addressed urgently if identified. Volume status management is critical and both noninvasive and invasive testing can aid in prognostication and guide management, including the use of inotropes and vasopressors. In cases of refractory right heart dysfunction, consideration of urgent lung transplantation and mechanical circulatory support is necessary. These patients should be managed at expert centers in an intensive care setting with a multidisciplinary team of practitioners experienced in the management of right heart dysfunction given the high short- and long-term mortality resulting from acute decompensated right heart failure.
Topics: Humans; Hypertension, Pulmonary; Intensive Care Units; Lung Transplantation; Critical Care; Heart Failure; Ventricular Dysfunction, Right
PubMed: 37369216
DOI: 10.1055/s-0043-1770119 -
Cells Oct 2023Pulmonary hypertension is a debilitating condition that frequently develops in the setting of interstitial lung disease, likely related to chronic alveolar hypoxemia and... (Review)
Review
Pulmonary hypertension is a debilitating condition that frequently develops in the setting of interstitial lung disease, likely related to chronic alveolar hypoxemia and pulmonary vascular remodeling. This disease process is likely to be identified more frequently by providers given recent advancements in definitions and diagnostic modalities, and provides practitioners with emerging opportunities to improve patient outcomes and quality of life. Despite years of data suggesting against the efficacy of pulmonary vasodilator therapy in patients with pulmonary hypertension due to interstitial lung disease, new data have emerged identifying promising advancements in therapeutics. The authors present to you a comprehensive review of pulmonary hypertension in interstitial lung disease, reviewing our current understanding of pathophysiology, updates in diagnostic approaches, and highlights of recent clinical trials which provide an effective approach for medical management.
Topics: Humans; Hypertension, Pulmonary; Quality of Life; Lung Diseases, Interstitial; Lung
PubMed: 37830608
DOI: 10.3390/cells12192394