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      Pulmonary Arterial Hypertension after Childhood Cancer Therapy and Bone Marrow Transplantation

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          According to the Third World Symposium on Pulmonary Arterial Hypertension (PAH), chemotherapy is considered to be one of the possible risk factors for patients developing PAH. However, to date, no literature has sufficiently addressed the risk, natural history, and effective treatment of this condition. We report our experience on how early diagnosis, detailed monitoring of disease course, and appropriate treatment application have led to a successful outcome of PAH management in childhood after cancer therapy. Our report reaffirmed the fact that PAH is now a recognized complication of chemotherapy and bone marrow transplantation for leukemia. Combined pulmonary vasodilator treatment has a beneficial effect in improving the patient’s condition and functional status as suggested by initial acute pulmonary vasodilator testing.

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          Most cited references 12

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          Clinical classification of pulmonary hypertension.

          In 1998, during the Second World Symposium on Pulmonary Hypertension (PH) held in Evian, France, a clinical classification of PH was proposed. The aim of the Evian classification was to individualize different categories sharing similarities in pathophysiological mechanisms, clinical presentation, and therapeutic options. The Evian classification is now well accepted and widely used in clinical practice, especially in specialized centers. In addition, this classification has been used by the U.S. Food and Drug Administration and the European Agency for Drug Evaluation for the labeling of newly approved medications in PH. In 2003, during the Third World Symposium on Pulmonary Arterial Hypertension held in Venice, Italy, it was decided to maintain the general architecture and philosophy of the Evian classification. However, some modifications have been proposed, mainly to abandon the term "primary pulmonary hypertension" and to replace it with "idiopathic pulmonary hypertension"; to reclassify pulmonary veno-occlusive disease and pulmonary capillary hemangiomatosis; to update risk factors and associated conditions for pulmonary arterial hypertension and to propose guidelines in order to improve the classification of congenital systemic-to-pulmonary shunts.
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            Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension.

            Characteristics of patients with idiopathic pulmonary arterial hypertension (IPAH) who benefit from long-term calcium channel blockers (CCB) are unknown. Acute pulmonary vasodilator testing with epoprostenol or nitric oxide was performed in 557 IPAH patients. Acute responders, defined by a fall in both mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR) >20%, received long-term oral CCB. Patients who benefit from long-term CCB were defined as those being in New York Heart Association (NYHA) functional class I or II after at least 1 year on CCB monotherapy. Among the 70 patients who displayed acute pulmonary vasoreactivity (12.6%; 95% CI, 9.8% to 15.3%) and received CCB therapy, only 38 showed long-term improvement (6.8%; 95% CI, 4.7% to 8.9%). Long-term CCB responders had less severe disease at baseline than patients who failed. During acute vasodilator testing, long-term CCB responders displayed a more pronounced fall in mean PAP (-39+/-11% versus -26+/-7%; P<0.0001), reaching an absolute value of mean PAP lower than that measured in patients who failed (33+/-8 versus 46+/-10 mm Hg; P<0.0001). After 7.0+/-4.1 years, all but 1 long-term CCB responders were alive in NYHA class I or II, with a sustained hemodynamic improvement. In the group of patients who failed on CCB, the 5-year survival rate was 48%. Long-term CCB responders represent <10% of IPAH patients evaluated in a pulmonary vascular referral center. During acute vasodilator testing, these patients showed significantly lower levels of both mean PAP and PVR, which reached near-normal values.
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              A comparison of the acute hemodynamic effects of inhaled nitric oxide and aerosolized iloprost in primary pulmonary hypertension


                Author and article information

                S. Karger AG
                March 2006
                03 April 2006
                : 105
                : 3
                : 188-194
                aDivision of Pediatric Cardiology, and bDivision of Pediatric Hematology-Oncology, Department of Pediatrics, and cDepartment of Pathology, Ramathibodi Hospital, Bangkok, Thailand
                91638 Cardiology 2006;105:188–194
                © 2006 S. Karger AG, Basel

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                Figures: 4, References: 17, Pages: 7
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