In patients who require urgent initiation of pulmonary arterial hypertension medications due to disease progression, it is customary to start intravenous prostacyclin therapy, typically during a hospital admission. If there are complicating factors or relative contraindications to intravenous and subcutaneous prostanoids, oral treprostinil provides another pathway to prostanoid therapy, but this usually requires a prolonged titration. We describe the case of a thirty-six-year-old male with severe pulmonary arterial hypertension and contraindication to intravenous and subcutaneous prostanoid therapy due to congenital deafness and the risk of not hearing the intravenous pump alarms. Intravenous treprostinil was initiated, titrated to high dose, and then rapidly transitioned to oral treprostinil. A rapid initiation, titration, and transition from intravenous to oral treprostinil can be safely performed under watchful supervision in order to achieve higher and more efficacious doses of oral treprostinil in a timely manner.

AIM: Pulmonary Hypertension (PH) currently has minimal guidelines for outpatient disease management. Congestive heart failure (CHF) studies however have shown effectiveness of disease management plans in reducing all-cause mortality and all-cause and CHF related hospital readmissions. Heart failure exacerbation is a common reason for readmission in both PH and CHF. Our aim was to review individual studies and comprehensive meta-analyses to identify effective CHF interventions that can be used to develop similar disease management plans for PH. METHODS: A comprehensive literature review from 1993 to 2019, including original articles, systematic reviews and meta-analyses. We reviewed topics of outpatient CHF interventions to decrease CHF mortality and readmission and patient management strategies in CHF. RESULTS: The most studied interventions included case management (CM), multidisciplinary Intervention (MI), structured telephone strategy (STS) and tele-monitoring (TM). CM showed decreased all-cause mortality (ACM) at 12 months, all-cause readmission (ACR) at 12 months and CHF readmission at 6 and 12 months. MI resulted in decreased ACR and CHF readmission. There was some discrepancy on effectiveness of TM programs in individual studies, however meta-analyses suggest TM provided reduced ACM and risk of CHF hospitalization. STS had similar results to TM including decreased risk of CHF hospitalization, without effect on mortality. CONCLUSIONS: Extrapolating from CHF data, it seems strategies to improve the health of PH patients and development of comprehensive care programs (CCP) should include structured telephone strategy and/ or tele-monitoring, case management strategies and multi-disciplinary interventions.

SUMMARY A case of childhood pulmonary haemosiderosis with a fourfold increase in mast cells in the lung is described. This finding is discussed in relation to the pathogenesis of the disease. Comparison is made with controls, and the use of disodium cromoglycate is advocated. Treatment with disodium cromoglycate began and resulted in regression of dyspnoea, maintenance of a steady haemoglobin value, and clinical cessation of pulmonary bleeding for a period of 14 weeks. An episode of massive intrapulmonary haemorrhage then occurred and the patient died. At necropsy the lungs showed the characteristic brown induration with superimposed recent bleeding. Abnormalities shown by light and electron microscopy were similar to those seen in the lung biopsy, with alterations in the mast cells consistent with cromoglycate treatment.3 The other organs, in particular the kidneys, were normal. Lung tissue from this child was compared with that from three adults with PH (one case each of mitral stenosis, immune complex mediated PH, and adult IPH) and three normal adult controls (Fig.

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PURPOSE: Pulmonary Hypertension (PH) is a progressive group of diseases that remains difficult to treat and carries significant morbidity and mortality despite available medical therapy. There are currently minimal guidelines on chronic outpatient management and prevention of hospitalization owing to the low number of patients and orphan status of the disease. In contrast, congestive heart failure (CHF) outpatient management interventions have been investigated in multiple trials. A common reason for admission in PH and CHF is heart failure exacerbation. The disease states share similar dietary and fluid management challenges, disease management and compliance issues. CHF has well established intervention models that have successfully decreased hospital readmission rates and mortality. We aimed to analyze outpatient CHF disease management interventions that have shown reductions in hospital readmissions and mortality as a starting point for developing an outpatient pulmonary hypertension intervention and management plan. METHODS:A comprehensive review of literature was performed, including original trials and meta-analysis and reviews. We reviewed the topics of outpatient CHF interventions to decrease CHF mortality and readmission and patient management strategies in CHF. RESULTS:The most studied interventions included case management (CM-specialist nurse driven, education pre/post discharge, specialist nurse home visits, scheduled telephone calls for symptom management, when to seek help), multidisciplinary Intervention ( MI-coordinated interventions and communications; specialist nurse driven, patient-caregiver education regarding disease, medication and diet, nurse clinic visits, regular telephone calls, individualized follow-up plan, access to physician, nurse, dietician, pharmacist, social worker), remote monitoring programs consisting of structured telephone strategy (STS-monitoring collected data via human-human or human-machine interactive response system) or tele-monitoring (TM-physiologic data transmission of EKG, blood pressure, weight, respiratory rate digitally). Clinic visits did not have a significant effect on CHF readmission or mortality. CM showed decreased all-cause mortality (ACM) at 12 months, all-cause readmission (ACR) at 12 months and CHF readmission at 6 and 12 months. MI resulted in decreased ACR and CHF readmission. There was some discrepancy on effectiveness of TM programs alone in individual studies, however large meta-analysis suggests TM provided a reduction in ACM and risk of CHF hospitalization. STS had similar results to TM including decreased risk of CHF hospitalization, without an effect on mortality. CONCLUSIONS:Extrapolating from CHF data, it seems that strategies to improve the health of PH patients should include structured telephone strategy and/ or tele-monitoring, case management strategies and multi-disciplinary interventions. CLINICAL IMPLICATIONS:There is no consensus on the most effective outpatient management of PH or strategies to reduce hospital readmission. Succe...