ABSTRACT
BACKGROUND: The phenotype of patients seen for a suspicion of pulmonary hypertension has changed, with an increasing age and frequency of comorbidities. Selection of elderly patients, in whom a classical work-up is mandatory, is challenging. Comprehensive geriatric assessment (CGA) has modified the management of elderly patients with cancer. Pulmonary hypertension (PH) shares with cancer a functional impact and may evolve rapidly, depending on the group of PH. We assessed the impact of a systematic CGA in patients over 70 years old referred for a suspicion of PH. METHODS: A standardised CGA was performed on every patient older than 70 years old, referred for a PH suspicion, before considering invasive tests for diagnosis and treatment, between July 2014 and May 2019. Our primary aim was to describe the impact of CGA on the decision to stop or pursue the recommended diagnostic work-up for PH. RESULTS: Among the thirty-one patients evaluated [mean age 81,5 (72-91) years], a negative CGA leads to stop the diagnostic work-up in eleven patients. Among the nineteen remaining patients, sixteen had confirmed PH, with half being chronic thromboembolic pulmonary hypertension. CONCLUSIONS: Our study indicates that comprehensive geriatric assessment could be an excellent first screen for elderly patients referred for a PH suspicion. Involving a geriatric physician stopped the investigations in one third of patients. In patients with a favourable CGA, PH was confirmed in most of the cases, with chronic thromboembolic pulmonary hypertension being the first cause of PH.
Subject(s)
Geriatric Assessment/methods , Hypertension, Pulmonary/diagnosis , Age of Onset , Aged , Aged, 80 and over , Diagnosis, Differential , Diagnostic Techniques, Cardiovascular/standards , Female , France/epidemiology , Humans , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/therapy , Implementation Science , Male , Reference Standards , Risk AssessmentABSTRACT
Neurofibromatosis type 1 (NF1) is a genetic disease in which pulmonary complications are rare, but severe, especially pulmonary hypertension (PH). The mechanisms underlying the onset of PH in patients with NF1 are unclear and might be multifactorial. In particular, the frequent presence of pulmonary parenchymal lesions makes etiological diagnosis of PH difficult. We describe here the case of a patient with NF1 admitted to our clinic with dyspnea and right heart failure revealing severe pre-capillary PH. Parenchymal lesions were mild and PH was attributed to pulmonary vascular involvement. Clinical and hemodynamic conditions of the patient improved under pulmonary arterial hypertension-specific combination therapy. This case suggests that treatment of PH due to pulmonary vascular involvement in NF1 may be aligned with recommendations for PAH treatment.
