Tamura, Yuichi Kumamaru, Hiraku Abe, Kohtaro Satoh, Toru Miyata, Hiroaki Ogawa, Aiko Tanabe, Nobuhiro Hatano, Masaru Yao, Atsushi Tsujino, Ichizo
...
Published in
BMC Pulmonary Medicine
BackgroundSince there was no previous report, we analyzed the relationship between French Risk Stratification parameters in pulmonary arterial hypertension (PAH) and mean pulmonary arterial pressures (mPAP) using Japan PH Registry (JAPHR) national-wide cohort.MethodsWe enrolled 108 patients with PAH from JAPHR from previous reported cohort and anal...
Zhang, Feng Chen, Aidong Pan, Yan Wang, Xingxing Xu, Yu Desai, Ankit A. Tang, Haiyang Han, Ying
Published in
Cardiovascular Drugs and Therapy
Pulmonary arterial hypertension (PAH) is a progressive disease with a complex aetiology and high mortality. Functional and structural changes in the small pulmonary arteries lead to elevated pulmonary arterial pressure, resulting in right heart failure. The pathobiology of PAH is not fully understood, and novel treatment targets in PAH are desperat...
Kang, Juyeon Lim, Dae Hyun Kim, Gheun-Ho
Published in
Electrolytes & Blood Pressure : E & BP
Because it is associated with mortality, hyponatremia is an important feature of pulmonary arterial hypertension. Its mechanisms remain unclear, although right heart failure resulting from pulmonary arterial hypertension may lead to systemic congestion and arterial underfilling. However, most patients with pulmonary arterial hypertension are clinic...
Gin-Sing, Wendy
Published in
Journal of Congenital Cardiology
Over the past 15 years there have been significant improvements in the treatment of pulmonary arterial hypertension due to congenital heart disease. Patients now live for several decades, but morbidity and mortality remain high. This article describes the holistic management of this patient group with an emphasis on both the physical and psychosoci...
Condliffe, Robin
Published in
Journal of Congenital Cardiology
BackgroundPregnancy in patients with pulmonary arterial hypertension associated with congenital heart disease is associated with a high risk of maternal mortality.Case presentationAn illustrative case study based on experience gained from management of patients through pregnancy is discussed. A 22-year-old female with a history of a closed patent d...
Brida, Margarita Favoccia, Carla Constantine, Andrew Dimopoulos, Konstantinos
Published in
Journal of Congenital Cardiology
BackgroundAn increasing number of patients with previously repaired congenital heart disease (CHD) present with pulmonary arterial hypertension (PAH). This can occur immediately after repair (residual PAH) or years later.Case presentationWe present the case of a young woman who underwent repair of a ventricular septal defect in later childhood. Thr...
Constantine, Andrew Clift, Paul
Published in
Journal of Congenital Cardiology
BackgroundCurrent guidelines for the peri-operative assessment and management are not sufficient to allow effective risk assessment and management of the patient with pulmonary arterial hypertension associated with congenital heart disease. Well-established risk stratification tools are not validated in this group. Additional, disease-specific risk...
Condliffe, Robin
Published in
Journal of Congenital Cardiology
While the development of pulmonary arterial hypertension is not uncommon in adult congenital heart disease patients, other forms of pulmonary hypertension (PH) may also be present. A good understanding of PH classification is therefore vital for clinicians managing adult patients with congenital heart disease. This paper reviews both the general cl...
Constantine, Andrew Tulloh, Robert M. R. Turquet, Rebecca Dimopoulos, Konstantinos Moledina, Shahin
Published in
Journal of Congenital Cardiology
BackgroundA structured transition provides a framework of care that bridges the gap between paediatric and adult medicine. It is essential for achieving continuity of care and providing support and education around the challenging period of adolescence for young people with pulmonary arterial hypertension associated with congenital heart disease (P...
Nashat, Heba Constantine, Andrew Dimopoulos, Konstantinos
Published in
Journal of Congenital Cardiology
BackgroundThis is a case report of a patient diagnosed with Eisenmenger syndrome in adult life.Case presentationThe patient had a large secundum atrial septal defect diagnosed incidentally in her twenties, with established pulmonary vascular disease and thus the defect was not closed. Over several years the patient showed signs of progressive disea...
