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Airway ciliary dysfunction: Association with adverse postoperative outcomes in nonheterotaxy congenital heart disease patients.

ABSTRACT: OBJECTIVE:Heterotaxy (HTX) congenital heart disease (CHD) patients with ciliary dysfunction (CD) have been shown to have increased postoperative respiratory morbidity. We hypothesized that non-HTX CHD infants with CD also will have increased postoperative morbidity, particularly respiratory complications. METHODS:Sixty-three infants with non-HTX CHD undergoing cardiac surgery were enrolled. Tests commonly used to assess for CD, nasal nitric oxide (nNO) measurements and nasal epithelial ciliary motion (CM) assessment, were obtained. Baseline characteristics and postoperative outcomes were collected and analyzed. RESULTS:Non-HTX CHD infants exhibited a high prevalence of abnormal CM (32%) and low nNO (39%). This finding was not correlated with demographics or surgical complexity. Infants with abnormal CM had increased odds of requiring noninvasive positive pressure ventilation (odds ratio [OR], 6.5; 95% confidence interval [CI], 1.5-29.4; P = .016) and respiratory medication use (OR, 4.4; 95% CI, 1.5-13.3; P = .01). In contrast, infants with low nNO showed evidence of abnormal pre- and postoperative systolic function (40% vs 4%; P = .004, and 34% vs 13%; P = .056, respectively) and had greater odds of acquiring infections (OR, 4.9; 95% CI, 1.4-17; P = .014). CONCLUSIONS:Non-HTX CHD infants with abnormal CM showed increased postoperative morbidity associated with poor respiratory outcomes. In contrast, low nNO correlated with reduced hemodynamic function. These findings suggest screening for abnormal CM may allow perioperative interventions to reduce pulmonary morbidities. Whether low nNO may prognosticate poor hemodynamic function warrants further investigation.

SUBMITTER: Stewart E

PROVIDER: S-EPMC6422347 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature

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Airway ciliary dysfunction: Association with adverse postoperative outcomes in nonheterotaxy congenital heart disease patients.

Stewart Eileen E Adams Phillip S PS Tian Xin X Khalifa Omar O Wearden Peter P Zahid Maliha M Lo Cecilia W CW

The Journal of thoracic and cardiovascular surgery 20170920 2

<h4>Objective</h4>Heterotaxy (HTX) congenital heart disease (CHD) patients with ciliary dysfunction (CD) have been shown to have increased postoperative respiratory morbidity. We hypothesized that non-HTX CHD infants with CD also will have increased postoperative morbidity, particularly respiratory complications.<h4>Methods</h4>Sixty-three infants with non-HTX CHD undergoing cardiac surgery were enrolled. Tests commonly used to assess for CD, nasal nitric oxide (nNO) measurements and nasal epith ...[more]

PMID: 29056267

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Project description:ImportanceAlbuminuria is associated with adverse outcomes in diverse groups of patients, but the importance of albuminuria in the emerging population of increasingly complex adults with congenital heart disease (ACHD) remains unknown.ObjectiveTo assess the prevalence, risk factors, and prognostic implications of albuminuria in ACHD.Design, setting, and participantsThis prospective study assessed a cohort of ambulatory patients aged 18 years and older who were examined at an ACHD referral center and enrolled in the Boston ACHD Biobank between May 17, 2012, to August 5, 2016. Albuminuria was defined as an urine albumin-to-creatinine (ACR) ratio of 30 mg/g or more.Main outcomes and measuresDeath or nonelective cardiovascular hospitalization, defined as overnight admission for heart failure, arrhythmia, thromboembolic events, cerebral hemorrhage, and/or disease-specific events.ResultsWe measured the ACR of 612 adult patients with CHD (mean [SD] age, 38.6 [13.4] years; 308 [50.3%] women). Albuminuria was present in 106 people (17.3%) and was associated with older age (patients with ACR <30 mg/g: mean [SD]: 37.5 [13.2] years; vs patients with ACR ≥30 mg/g: 43.8 [13.1] years; P < .001), presence of diabetes mellitus (ACR <30 mg/g: 13 of 506 [2.6%]; vs ≥30 mg/g: 11 of 106 [10.4%]; P < .001), lower estimated glomerular filtration rate (ACR <30 mg/g: median [interquartile range (IQR)]: 103.3 [90.0-116.4] mL/min/1.73 m2; ACR ≥30 mg/g: 99.1 [78.8-108.7] mL/min/1.73 m2; P = .002), and cyanosis (ACR <30 mg/g: 23 of 506 [5.1%]; vs ACR ≥30 mg/g: 21 of 106 [22.6%]; P < .001). After a mean (SD) follow-up time of 270 (288) days, 17 patients (2.5%) died, while 68 (11.1%) either died or experienced overnight inpatient admission. Albuminuria predicted outcome, with 30 of 106 patients with albuminuria (28.3%) affected vs 38 of 506 patients without albuminuria (7.5%; hazard ratio [HR], 3.0; 95% CI, 1.9-4.9; P < .001). Albuminuria was also associated with increased mortality (11 of 106 [10.4%]; vs 6 of 506 [1.2%] in patients with and without albuminuria, respectively; HR, 6.4; 95% CI, 2.4-17.3; P < .001). Albuminuria was associated with the outcomes only in patients with a biventricular circulation (HR, 4.5; 95% CI, 2.5-8.0) and not those with single-ventricle circulation (HR, 1.0; 95% CI, 0.4-2.8; P = 0.01 compared with biventricular circulation group). Among 133 patients (21.7%) in NYHA functional class 2, albuminuria was strongly associated with death or nonelective hospitalization.Conclusions and relevanceAlbuminuria is common and is associated with increased risk for adverse outcome in patients with ACHD with biventricular circulation. Albuminuria appears especially useful in stratifying risk in patients categorized as NYHA functional class 2.

Project description:ImportanceCongenital heart disease (CHD) carries significant health care costs and out-of-pocket expenses for families. Little is known about how financial hardship because of medical bills affects families' access to essential needs or medical care.ObjectiveTo assess the national prevalence of financial hardship because of medical bills among families of children with CHD in the US and the association of financial hardship with adverse outcomes.Design, setting, and participantsThis cross-sectional survey study used data on children 17 years and younger with self-reported CHD from the National Health Interview Survey of US households between 2011 and 2017. Data were analyzed from March 2019 to April 2020.ExposuresFinancial hardship because of medical bills was classified into 3 categories: no financial hardship, financial hardship but able to pay medical bills, and unable to pay medical bills.Main outcomes and measuresFood insecurity, delayed care because of cost, and cost-related medication nonadherence.ResultsOf 188 families of children with CHD (weighted sample of 151 537 families), 48.9% reported some financial hardship because of medical bills, with 17.0% being unable to pay their medical bills at all. Compared with those who denied financial hardships because of medical bills, families who were unable to pay their medical bills reported significantly higher rates of food insecurity (61.8% [SE, 11.0] vs 13.6% [SE, 4.0]; P < .001) and delays in care because of cost (26.2% [SE, 10.4] vs 4.8% [SE, 2.5]; P = .002). Reported medication adherence did not differ across financial hardship groups. After adjusting for age, race/ethnicity, and maternal education, the differences between the groups persisted. The association of financial hardship with adverse outcomes was stronger among patients with private insurance than those with Medicaid.Conclusions and relevanceIn this study, financial hardship because of medical bills was common among families of children with CHD and was associated with high rates of food insecurity and delays in care because of cost, suggesting possible avenues for intervention.

Project description:ImportanceWith the help of medical advances, more women with adult congenital heart disease (ACHD) are becoming pregnant. Adverse maternal, obstetric, and neonatal events occur more frequently in women with ACHD than in the general obstetric population. Adult congenital heart disease is heterogeneous, yet few studies have assessed whether maternal and neonatal outcomes differ across ACHD subtypes.ObjectiveTo assess the association of ACHD and its subtypes with pregnancy, maternal, and neonatal outcomes.Design, setting, and participantsThis cross-sectional study used data from the Discharge Abstract Database, which contains information on all hospitalizations in Canada (except Quebec) from fiscal years 2001-2002 through 2014-2015. Discharge Abstract Database information was linked with maternal and infant hospital records across Canada. All women who gave birth in hospitals during the study period were included in the study. Data were analyzed from December 18, 2017, to March 22, 2019.ExposuresWomen with ACHD were identified using diagnostic and procedural codes. Subtypes of ACHD were classified using the Anatomic and Clinical Classification of Congenital Heart Defects scheme.Main outcomes and measuresPrimary outcomes were defined a priori and included severe maternal morbidity (measured using the Maternal Morbidity Outcomes Indicator), neonatal morbidity and mortality (measured using the Neonatal Adverse Outcomes Indicator), ischemic placental disease, preterm birth, congenital anomalies, and small-for-gestational-age births. Absolute and relative rates of each outcome were calculated overall and by ACHD subtype. Logistic regression using generalized estimating equations assessed crude and adjusted odds ratios (aORs) for each outcome in women with ACHD compared with women without ACHD after adjustment for comorbidities, mode of delivery, and study year.ResultsThe 2114 women with ACHD included in the analysis (mean [SD] age, 29.4 [5.7] years) had significantly higher odds of maternal morbidity (aOR, 2.7; 95% CI, 2.2-3.4) and neonatal morbidity and mortality (aOR, 1.8; 95% CI, 1.6-2.1) compared with women without ACHD (n = 2 682 451). Substantial variation was observed between women with different subtypes of ACHD. For example, the aORs of preterm birth (<37 weeks) varied from 0.4 (95% CI, 0.4-0.5) for women with anomalies of atrioventricular junctions and valves to 4.7 (95% CI, 2.9-7.5) for women with complex anomalies of atrioventricular connections.Conclusions and relevanceThese results suggest that women with different subtypes of ACHD are not uniformly at risk for adverse maternal and neonatal outcomes. Although some women with ACHD can potentially expect healthy pregnancies, it appears that clinical care should be modified to address the heightened risks of certain ACHD subtypes.

Project description:BackgroundAcute kidney injury (AKI) after surgery for congenital heart disease (CHD) in adults is poorly studied despite being well-recognized as a postoperative complication after cardiac surgery in adults. The primary aim of our study was to determine the frequency of AKI in adults undergoing surgery for CHD. We also aimed to determine risk factors and predictors of AKI in this patient population, and to explore outcomes in terms of duration of mechanical ventilation, intensive care unit (ICU) stay, and hospital stay.MethodsThis retrospective cross-sectional study included all adult patients (18 years) who underwent cardiac surgery with cardiopulmonary bypass for their congenital heart problems from January 2011 to December 2016 in a tertiary-care private hospital.ResultsA total of 166 patients with a mean age of 32.05 ± 12.11 years were included in this study. The postoperative course was complicated by AKI in 29.5% of patients. Thirty-two percent of these patients had moderate-to-severe kidney disease. Two patients (4%) developing AKI required renal replacement therapy in the form of transient hemodialysis. All patients in our study showed complete resolution of AKI, with no mortalities in the postoperative period. On univariable analysis, (Risk adjusted classification for congenital heart surgery-1) RACHS-1 category 2 and 3, aortic valve replacement, preoperative creatinine clearance, ventricular septal defect closure, cardiopulmonary bypass time, aortic cross-clamp time, intra-operative excessive blood loss, intra-operative ionotropic score, and postoperative hypotension were found to be significant predictors for the development of AKI. On age-adjusted multivariable analysis, RACHS-1 category 2 (OR = 3.49; CI = 1.22-9.95) and category 3 (OR = 3.28 = 1.15-9.36), and intra-operative excessive blood loss (OR = 2.9; CI = 1.07-7.85) were significant predictors of AKI development in the postoperative period. Moreover, development of AKI postoperatively was a predictor of a significantly longer cardiac intensive care unit (CICU) stay (OR = 1.21; CI = 1.08-1.37).ConclusionWe found that preoperative creatinine clearance, ACC time, intraoperative excessive blood loss, and RACHS-1Category 2 and 3 are potential risk factors for postoperative AKI development. Moreover, patients who develop AKI are likely to have a significantly longer CICU stay. Our study has tried to fill the lacunae with regard to AKI in adults undergoing surgery for CHD. However, there is a need for more studies with larger cohorts involving more complex surgeries to truly estimate the incidence and potential risk factors for AKI in this group of patients.

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Airway ciliary dysfunction: Association with adverse postoperative outcomes in nonheterotaxy congenital heart disease patients.

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Airway ciliary dysfunction: Association with adverse postoperative outcomes in nonheterotaxy congenital heart disease patients.

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