Clinical practice: pulmonary hypertension in children (original) (raw)
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Pulmonary arterial hypertension: closing the gap in congenital heart disease
Current Opinion in Pulmonary Medicine, 2020
Purpose of review Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a common association adversely affecting quality of life and survival in these patients. We provide herewith recent advances in the understanding and management of PAH-CHD. Recent findings Significant progress has been made in disease-targeting therapy with pulmonary vasodilators for the treatment of Eisenmenger syndrome, the most severe form of PAH-CHD. Important gaps, however, still exist in the assessment and management of patients with PAH-CHD with systemic to pulmonary shunts. The choice of therapy, either interventional, medical, or both is an ongoing dilemma that requires more longterm data. PAH after defect closure represents the most concerning subgroup of patients with the worst prognosis, requiring close follow-up and proactive disease-targeting therapy treatment. Small defects are not considered responsible for patients who have severe PAH and therefore, present different subgroup of patients similar to idiopathic PAH. Summary Even with advances in diagnosis and treatment PAH-CHD remains a challenging field requiring lifelong follow-up and meticulous treatment in centres specialized in both CHD and PAH.
Pulmonary hypertension related to congenital heart disease: A comprehensive review
Global Cardiology Science and Practice, 2015
Pulmonary arterial hypertension (PAH) is a serious complication of congenital heart disease, causing an increase in morbidity and mortality. The progressive and irreversible pulmonary vascular disease is more often the consequence of a significant, uncorrected, left-to-right shunt. The rise in pulmonary vascular resistance may lead to the reversal of the shunt and cyanosis, condition known as Eisenmenger syndrome. The management of this population is challenging and requires specific expertise both for diagnosis and follow-up. The progress in the understanding of the underlying pathophysiology of this condition has promoted recent pharmacological trials. New therapeutic options are now available that could improve the long-term prognosis and the quality of life of these patients, but several controversial points still remain and need to be addressed.
Pulmonary Hypertension Management Challenges in Pediatric Age Group
2017
The major causes of pulmonary hypertension (PH) in children are congenital heart defect (CHD) and PH associated with prematurity, respiratory distress syndrome (RDS) and bronchopulmonary dysplasia (BPD). Idiopathic pulmonary arterial hypertension (PAH) and PAH associated with genetic mutations are also known to manifest in the pediatric age group. This review will mainly discuss the problems encountered in the management of PAH associated with CHD, especially left to right shunts, and in PH associated with premature birth and BPD.
Cardiology in the Young, 2008
Pulmonary hypertension associated with congenital systemic-to-pulmonary shunts has been classified in the Evian-Venice classification as pulmonary arterial hypertension (PAH), which includes a heterogeneous group of conditions. Emerging treatment options for patients with PAH are mostly investigated in those with idiopathic PAH but may also improve quality of life and survival in patients with congenital heart disease and PAH. However, despite the evident similarities in pulmonary vascular disease, important differences have to be recognized between patients with PAH associated with systemic-to-pulmonary shunts and those with other conditions. Patients with pulmonary hypertension associated with congenital heart disease form a rather heterogeneous patient population in which generalization is hazardous. Specific considerations with respect to cardiac diagnosis, prognosis, evolution of pulmonary vascular disease and circulatory physiology have to be made in the individual patient, before embarking on new medical treatment strategies. This review highlights the features that require specific attention in these patients. Further, the currently available data on effectiveness of new PAH-drugs in Eisenmenger patients will be discussed shortly.
Evaluation of pediatric patients with severe pulmonary arterial hypertension
Eastern Journal Of Medicine
Pulmonary arterial hypertension (PAH) is an important cause of morbidity and mortality. Congenital heart disease associated PAH (APAH-CHD) and idiopathic PAH are classified in Group 1 PAH. There are limited studies about pediatric patients with PAH. The aim of our study is to evaluate the clinical, diagnostic and treatment characteristics of pediatric PAH. 53 consecutive patients with PAH in a 7 years' study period were retrospectively analyzed. Clinical, echocardiographic and cardiac catheterization findings and targeted treatment modalities were noted. Thirty (56.6%) patients were male and mean age at diagnosis was 5.2±4.30 years. All patients were classified as group 1 consisting of APAH-CHD and idiopathic PAH. Patients with Eisenmenger syndrome w ere the largest group. Ventricular septal defect was the most CHD associated with PAH overall. Atrial septal defect, patent ductus arteriosus, atrioventricular septal defect, aortopulmonary window, double inlet left ventricle, double outlet right ventricle, d-transposition of great arteries and truncus arteriosus were other congenital heart malformat ions were detected. Targeted therapy were given to 34 patients (%64.1%), of them, 22 were under monotherapy, while 12 were under combined therapy. Bosentan was the most chosen drug in all. NYHA FC, exercise capacity with 6MWT improved well by targeted therapy. Life quality and survey are improved with the targeted therapies in pediatric patients with PAH. Single drug or combination therapies including bosentan, tadalafil and inhaled iloprost are effective, safe and well tolerated with rare and minor side effects in pediatric patients with group 1 PAH.
Pulmonary Hypertension in Children
Pulmonary Vascular Disease, 2006
Pulmonary hypertension in children previously carried a poor prognosis. Recent advances in the understanding of normal and hypertensive pulmonary circulations have led to the introduction of pharmacological therapies and, in turn, this has led to an improved prognosis for children with this condition. The diagnosis and evaluation of Pulmonary hypertension in children is discussed and a regimen for treatment is outlined with a rational for the use of pharmacological agents. The selection of appropriate therapies is complex, requiring familiarity with the underlying disease process, complicated delivery systems and dosing regimens, and medication complications.
Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. Methods and results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, >8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients' median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH)