Hemodynamic predictors of long term survival in end stage cystic fibrosis

Scarsini, Roberto; Prioli, Maria Antonia; Milano, Elena Giulia; Castellani, Carlo; Pesarini, Gabriele; Assael, B.M.; Vassanelli, Corrado; Ribichini, Flavio

doi:10.1016/j.ijcard.2015.09.009

Cited by 10 publications

(9 citation statements)

References 30 publications

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“…The association of PH and poor outcomes in CF remains controversial. Whereas studies in the past did not show association between PH and survival [25][26][27], we found that patients with mPAP �25 mmHg have a higher risk of mortality and poorer outcomes, confirming the findings reported by Hayes et al [28]. Similarly, we found that there was an association between PA enlargement defined and transplant-free survival, and that the presence of PA enlargement portends a worse prognosis.…”

Section: Discussionsupporting

confidence: 90%

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Pulmonary artery enlargement is associated with pulmonary hypertension and decreased survival in severe cystic fibrosis: A cohort study

et al. 2020

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Background Pulmonary artery (PA) enlargement, defined as pulmonary artery to ascending aorta diameter ratio (PA:A)>1 on computed tomography (CT), is a marker of pulmonary vascular disease in chronic lung diseases. PA enlargement is prevalent in cystic fibrosis (CF), but its relationship to hemodynamics and prognostic utility in severe CF are unknown. We hypothesized that the PA:A would have utility in identifying pulmonary hypertension (PH) in severe CF and that PA enlargement would be associated with reduced transplant-free survival. Methods We conducted a retrospective study of adults with CF undergoing lung transplant evaluation at a single center between 2000 and 2015. CT, right heart catheterization (RHC), and clinical data were collected. The PA:A was measured from a single CT slice. We measured associations between PA:A and invasive hemodynamic parameters including PH defined as a mPAP �25mmHg using adjusted linear and logistic regression models. Kaplan-Meier and adjusted Cox regression models were used to measure associations between PA:A>1, RHC-defined PH, and transplant-free survival in severe CF. Results We analyzed 78 adults with CF that had CT scans available for review, including 44 that also had RHC. RHC-defined PH defined as a mPAP �25mmHg was present in 36% of patients with CF undergoing transplant evaluation.

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Section: Discussionsupporting

confidence: 90%

“…However, the prevalence of PH in CF is largely unknown. It is estimated to be between 25-60% [3,5,[25][26][27][28]. PH defined by mPAP �25 mmHg was present in 36% of our cohort that underwent RHC, a finding that is comparable to the prevalence of 38% reported by other investigators [5,26].…”

Section: Discussionsupporting

confidence: 85%

Pulmonary artery enlargement is associated with pulmonary hypertension and decreased survival in severe cystic fibrosis: A cohort study

et al. 2020

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“…PAC is believed to be a measure of pulmonary arterial distensibility expressing the absolute change in volume for a given change in pressure. PAC has been shown to possess important prognostic value in both primary pulmonary hypertension and left‐sided heart failure . The demonstrated impaired pulmonary compliance may be attributable to backward failure, which may be deteriorated by pericardial restraint as a result of elevated total heart volume in CA patients.…”

Section: Discussionmentioning

confidence: 99%

Inotropic myocardial reserve deficiency is the predominant feature of exercise haemodynamics in cardiac amyloidosis

Clemmensen

Mølgaard

Sørensen

et al. 2017

European J of Heart Fail

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“…Pulmonary hypertension is common in individuals with advanced CF-related lung disease [43,[54][55][56][57], but its presence is rarely identified prior to evaluation for LTx. Repeat echocardiogram should be considered for individuals with worsening clinical status.…”

Section: Ormentioning

confidence: 99%

Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines

Ramos

Smith

McKone

et al. 2019

Journal of Cystic Fibrosis

175

145

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Objective: Provide recommendations to the cystic fibrosis (CF) community to facilitate timely referral for lung transplantation for individuals with CF. Methods: The CF Foundation organized a multidisciplinary committee to develop CF Lung Transplant Referral Consensus Guidelines. Three workgroups were formed: timing for transplant referral; modifiable barriers to transplant; and transition to transplant care. A focus group of lung transplant recipients with CF and spouses of CF recipients informed guideline development. Results: The committee formulated 21 recommendation statements based on literature review, committee member practices, focus group insights, and in response to public comment. Critical approaches to optimizing access to lung transplant include early discussion of this treatment option, assessment for modifiable barriers to transplant, and open communication between the CF and lung transplant centers. Conclusions: These guidelines will help CF providers counsel their patients and may reduce the number of individuals with CF who die without consideration for lung transplant.

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Hemodynamic predictors of long term survival in end stage cystic fibrosis

Cited by 10 publications

References 30 publications

Pulmonary artery enlargement is associated with pulmonary hypertension and decreased survival in severe cystic fibrosis: A cohort study

Pulmonary artery enlargement is associated with pulmonary hypertension and decreased survival in severe cystic fibrosis: A cohort study

Inotropic myocardial reserve deficiency is the predominant feature of exercise haemodynamics in cardiac amyloidosis

Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines

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