Dataset Information

Pulmonary Vein Stenosis: Outcomes in Children With Congenital Heart Disease and Prematurity.

ABSTRACT: Pulmonary vein stenosis (PVS) is a rare condition that has been linked to prematurity and congenital heart disease (CHD). Despite these associations, treatment options are limited and outcomes are guarded. We investigated differences in PVS outcomes based on the presence of CHD and prematurity, and risk factors for mortality or lung transplantation in PVS. Single-center retrospective cohort study of patients diagnosed with PVS between January 2005 and May 2016 and identified by ICD codes with chart validation. Cox proportional hazard models assessed risk factors for the composite outcome of mortality or lung transplantation. Ninety-three patients with PVS were identified: 65 (70%) had significant CHD, 32 (34%) were premature, and 14 (15%) were premature with CHD. Sixty-five (70%) underwent a PVS intervention and 42 (46%) underwent ?2 interventions. Twenty-five subjects (27%) died or underwent lung transplant 5.8 months (interquartile range [IQR] 1.1, 15.3) after diagnosis. There was no difference in age at diagnosis or mortality based on presence of CHD or prematurity. PVS diagnosis before age 6 months and greater than 1 pulmonary vein affected at diagnosis were associated with higher mortality (hazards ratio [HR] 3.4 (95% confidence interval 1.5, 7.5), P?=?0.003, and HR 2.1 per additional vein affected (95% confidence interval 1.3, 3.4), P?=?0.004, respectively). Survival in children with PVS is poor, independent of underlying CHD or prematurity. Younger age and greater number of veins affected at diagnosis are risk factors for worse outcome. Understanding causal mechanisms and development of treatment strategies are necessary to improve outcomes.

SUBMITTER: DiLorenzo MP

PROVIDER: S-EPMC6440879 | biostudies-literature | 2019 Summer

REPOSITORIES: biostudies-literature

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Pulmonary Vein Stenosis: Outcomes in Children With Congenital Heart Disease and Prematurity.

DiLorenzo Michael P MP Santo Ashley A Rome Jonathan J JJ Zhang Huayan H Faerber Jennifer A JA Mercer-Rosa Laura L Hopper Rachel K RK

Seminars in thoracic and cardiovascular surgery 20180929 2

Pulmonary vein stenosis (PVS) is a rare condition that has been linked to prematurity and congenital heart disease (CHD). Despite these associations, treatment options are limited and outcomes are guarded. We investigated differences in PVS outcomes based on the presence of CHD and prematurity, and risk factors for mortality or lung transplantation in PVS. Single-center retrospective cohort study of patients diagnosed with PVS between January 2005 and May 2016 and identified by ICD codes with ch ...[more]

PMID: 30278272

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Project description:(1) Background: This mixed-method study aims to identify and describe factors associated with acute and long-term parenting stressors and coping strategies in caregivers of children with intraluminal pulmonary vein stenosis (PVS). (2) Methods: Parents of children with severe PVS were recruited from a large quaternary hospital to complete a survey that included demographics, the Pediatric Inventory for Parents (PIP), and the Coping Health Inventory for Parents (CHIP). We determined the Social Vulnerability Index (SVI) based on self-reported home address. A subset of caregivers completed a 60 min semi-structured interview via Zoom exploring the impact their child's diagnosis had on their family; experience managing stress in the hospital and at home; current resources and processes for coping; and potential recommendations for hospitals to build resilience and coping. We used multivariable linear regression to examine the association between SVI and parental stress and coping while adjusting for possible confounders. Thematic analysis identified themes related to stress and coping. Finally, we assessed instances of convergence and difference between the qualitative and quantitative results. (3) Results: Participants included 32 caregivers who were 91% female with a mean age of 39 years. The children of participants were 66% female, with a mean age of five years. The parents reported a high amount of stress with an average PIP score of 120, nearly 46 points higher than similar studies in the congenital heart community. We observed no significant associations between SVI and either parental stress or coping in adjusted models. We identified 13 themes, including medical care, hospital, family, support systems, and home medical routine or support. (4) Conclusions: Our study found high levels of illness-related parental stress among caregivers of children with PVS. Stress evolved over time from what caregivers described as 'survival mode' to a future-oriented outlook. Currently, caregivers rely heavily on support networks that are not available to all caregivers or may experience strain over time. Caregivers indicated that communication and parental role functioning were coping strategies that could be better supported by providers and health systems.

Dataset Information

Pulmonary Vein Stenosis: Outcomes in Children With Congenital Heart Disease and Prematurity.

Publications

Pulmonary Vein Stenosis: Outcomes in Children With Congenital Heart Disease and Prematurity.

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