Improving Situational Awareness to Decrease Emergency ICU Transfers for Hospitalized Pediatric Cardiology Patients.

Introduction: Failure to recognize and mitigate critical patient deterioration remains a source of serious preventable harm to hospitalized pediatric cardiac patients. Emergency transfers (ETs) occur 10-20 times more often than code events outside the intensive care unit (ICU) and are associated with morbidity and mortality. This quality improvement project aimed to increase days between ETs and code events on an acute care cardiology unit (ACCU) from a baseline median of 17 and 32 days to ≥70 and 90 days within 12 months. Methods: Institutional leaders, cardiology-trained physicians and nurses, and trainees convened, utilizing the Institution for Healthcare Improvement model to achieve the project aims. Interventions implemented focused on improving situational awareness (SA), including a "Must Call List," evening rounds, a visual management board, and daily huddles. Outcome measures included calendar days between ETs and code events in the ACCU. Process measures tracked the utilization of interventions, and cardiac ICU length of stay was a balancing measure. Statistical process control chart methodology was utilized to analyze the impact of interventions. Results: Within the study period, we observed a centerline shift in primary outcome measures with an increase from 17 to 56 days between ETs and 32 to 62 days between code events in the ACCU, with sustained improvement. Intervention utilization ranged from 87% to 100%, and there was no observed special cause variation in our balancing measure. Conclusions: Interventions focused on improving SA in a particularly vulnerable patient population led to sustained improvement with reduced ETs and code events outside the ICU.

Morbidity and mortality in neonates with Down Syndrome based on gestational age.

BACKGROUND: Greater than 50% of neonates with Down Syndrome (DS) have perinatal complications that require admission to the neonatal intensive care unit (NICU) at birth. Previous studies have shown increased morbidity and mortality rates in neonates without DS delivered prior to 39 weeks of completed gestation. OBJECTIVE: To determine if an association exists between gestational age at delivery and adverse outcomes in neonates with DS. STUDY DESIGN: Neonates with DS admitted to a large, tertiary care center NICU from 2010 to 2020 were evaluated. Gestational age (GA) was stratified into 4 groups: <34 (preterm), 34-36 (late-preterm), 37-38 (early-term) and ≥39 (term + post-term) completed weeks. Fisher's exact tests were used to evaluate morbidity and mortality rates between groups. RESULT: Of the 314 neonates with DS, 10% (N = 31) were <34 weeks, 22% (N = 68) 34-36 weeks, 40% (N = 127) 37-38 weeks, and 28% (N = 88) ≥39 completed weeks at birth. Baseline characteristics were similar between groups. GA at birth <34 weeks was associated with a higher in-hospital mortality rate when compared to those born 37-38 (19% vs. 0%, P < 0.001) and ≥39 (19% vs. 3%, P = 0.01). Neonates with DS born <34 weeks had a higher likelihood of oxygen requirement at time of discharge compared to 34-36, 37-38, and ≥39 groups (P = 0.01; P < 0.001; P < 0.001 respectively). Neonates with DS < 34 weeks were more likely to develop necrotizing enterocolitis (P = 0.02) and require nitric oxide (P = 0.014) compared to neonates with DS ≥ 39. We observed no differences in the need for surgical interventions between groups aside from the rate of gastrostomy/jejunostomy tube placement between 34-36 weeks and 37-38 weeks GA. CONCLUSION: Neonates with DS born preterm (<34 weeks) represent a highly vulnerable subgroup. Multidisciplinary strategies are needed to address their higher rates of morbidity and mortality.

Hospitalizations for Respiratory Syncytial Virus and Vaccine Preventable Infections following Pediatric Heart Transplantation.

OBJECTIVE: To determine the risk factors for acquiring a respiratory syncytial virus (RSV) and vaccine-preventable infections (R/VPI) in pediatric heart transplant recipients and the associated morbidity and hospital resource use. STUDY DESIGN: Patients <18 years who underwent heart transplantation from September 2003 to December 2018 at hospitals using the Pediatric Health Information System database were identified. Their transplant hospitalization and subsequent hospitalizations for R/VPI through December 2018 were analyzed. Risk factors for R/VPI hospitalizations were evaluated using negative regression binomial models adjusted for demographic and clinical confounders. Total hospital costs were adjusted for 2018 US$. RESULTS: Of 3815 transplant recipients, 681 (17.9%) had an R/VPI hospitalization during 23 746 available person-years of follow-up. There were 984 R/VPIs diagnosed during 951 hospitalizations, and 440 (44.7%) occurred the first year after transplantation. The most common causes were RSV (n = 380; 38.6%), influenza (n = 265; 26.9%), and pneumococcus (n = 105; 10.7%). In adjusted analyses, there was an increased risk of R/VPI hospitalization in patients requiring mechanical circulatory support before transplantation, patients receiving induction with ≥2 immunosuppressive agents, and patients <2 years in the first year after transplantation. The median length of stay for an R/VPI hospitalization was 4 days (IQR, 2-8 days) with a median total cost of $11 081 (IQR, $6215-$24 322). CONCLUSIONS: Hospitalization for R/VPIs occurred frequently after heart transplantation and were associated with significant costs. Potential strategies to minimize R/VPI include expanding vaccine use through accelerated immunization schedules, further studies of use of palivizumab beyond 2 years of age, and immunogenicity monitoring after vaccination with re-immunization based on guidelines.

High Acuity Therapy Variation Across Pediatric Acute Care Cardiology Units: Results from the Pediatric Acute Care Cardiology Collaborative Hospital Surveys.

We utilized the multicenter Pediatric Acute Care Cardiology Collaborative (PAC3) 2017 and 2019 surveys to describe practice variation in therapy availability and changes over a 2-year period. A high acuity therapies (ATs) score was derived (1 point per positive response) from 44 survey questions and scores were compared to center surgical volume. Of 31 centers that completed the 2017 survey, 26 also completed the 2019 survey. Scores ranged from 11 to 34 in 2017 and 11 to 35 in 2019. AT scores in 2019 were not statistically different from 2017 scores (29/44, IQR 27-32.5 vs. 29.5/44, IQR 27-31, p = 0.9). In 2019, more centers reported initiation of continuous positive airway pressure (CPAP) and Bi-level positive airway pressure (BiPAP) in Acute Care Cardiology Unit (ACCU) (19/26 vs. 4/26, p < 0.001) and permitting continuous CPAP/BiPAP (22/26 vs. 14/26, p = 0.034) compared to 2017. Scores in both survey years were significantly higher in the highest surgical volume group compared to the lowest, 33 ± 1.5 versus 25 ± 8.5, p = 0.046 and 32 ± 1.7 versus 23 ± 5.5, p = 0.009, respectively. Variation in therapy within the ACCUs participating in PAC3 presents an opportunity for shared learning across the collaborative. Experience with PAC3 was associated with increasing available respiratory therapies from 2017 to 2019. Whether AT scores impact the quality and outcomes of pediatric acute cardiac care will be the subject of further investigation using a comprehensive registry launched in early 2019.

Impact of prenatal screening on congenital heart defects in neonates with Down syndrome in the US.

BACKGROUND: Non-invasive prenatal screening (NIPS) has fundamentally changed the screening process for Down syndrome (DS). Rates of complex congenital heart defects (CHD) have decreased in international studies but whether these shifts exist in the US is unknown. METHODS: Encounters for neonates with DS from 2007 to 2018 were obtained from the Pediatric Health Information System database. CHD were categorized as complex CHD, atrioventricular septal defects (AVSD), ventricular septal defects (VSD), and tetralogy of Fallot (TOF). Comparisons were made between pre-NIPS era (2007-2010) vs. post-NIPS era (2014-2018) and between states with low vs. high access to pregnancy termination as described by the Guttmacher Institute. RESULTS: Among 9122 patients, 6% had complex CHD, 22% had an AVSD, 22% had a VSD, and 4% had TOF. No difference in proportions of CHD was seen between eras. A small difference was observed in the proportion of AVSD between states with low vs. high access to pregnancy termination (23 vs. 17%, p < 0.001). CONCLUSIONS: The proportion of CHD in patients with DS appears to be stable despite widespread adoption of NIPS in the US. Variations were observed between states with low vs. high access to pregnancy termination. Population based studies are needed to fully evaluate the current epidemiology of CHD in DS. IMPACT: Through investigation of the Pediatric Health Information System database, this study assesses contemporary epidemiology of congenital heart disease among patients with Down syndrome. It has been suggested that improved prenatal screening for Down syndrome has altered the cardiac phenotype in international populations. Whether a similar shift also exists in the United States is unknown. In a contemporary United States cohort, a shift in the proportion or type of heart defects over the past decade was not observed. Regional differences in the proportion of heart defects were seen and may be due to differential access to prenatal care.

The added value of the advanced practice provider in paediatric acute care cardiology.

OBJECTIVES: Advanced practice providers (APPs) are being employed at increasing rates in order to meet new in-hospital care demands. Utilising the Paediatric Acute Care Cardiology Collaborative (PAC3) hospital survey, we evaluated variations in staffing models regarding first-line providers and assessed associations with programme volume, acuity of care, and post-operative length of stay (LOS). STUDY DESIGN: The PAC3 hospital survey defined staffing models and resource availability across member institutions. A resource acuity score was derived for each participating acute care cardiology unit. Surgical volume was obtained from The Society of Thoracic Surgeons database. Pearson's correlation coefficients were used to evaluate the relationship between staffing models and centre volume as well as unit acuity. A previously developed case-mix adjustment model for total post-operative LOS was utilised in a multinomial regression model to evaluate the association of APP patient coverage with observed-to-expected post-operative LOS. RESULTS: Surveys were completed by 31 (91%) PAC3 centres in 2017. Nearly all centres (94%) employ APPs, with a mean of 1.7 (range 0-5) APPs present on weekday rounds. The number of APPs present has a positive correlation with surgical volume (r = 0.49, p < 0.01) and increased acuity (r = 0.39, p = 0.03). In the multivariate model, as coverage by APPs increased from low to moderate or high, there was greater likelihood of having a shorter-than-expected post-operative LOS (p < 0.001). CONCLUSIONS: The incorporation of paediatric acute care cardiology APPs is associated with reduced post-operative LOS. Future studies are necessary to understand how APPs impact these patient-specific outcomes.

Capture rate of congenital heart defects in the Pediatric Health Information System database.

INTRODUCTION: Congenital heart defects (CHD) are common and are a frequent subject of research using large administrative databases such as the Pediatric Health Information System (PHIS) database. The capture rate of CHD within PHIS, however, has not been described. METHODS: The PHIS database includes inpatient encounters from over 52 tertiary care pediatric hospitals across the United States. We identified all patients less than 1 year of age with a cardiac defect in PHIS from 2010 to 2014 and compared these results with national prevalence estimates based on the National Birth Defects Prevention Network annual report, which served as the gold standard. RESULTS: The capture rate of CHD in PHIS ranged from 30 to 95% depending on the spectrum of severity and heterogeneity of the cardiac defect. The capture rate was higher for critical CHD (CCHD) at 66%, and all lesions with 70% or greater capture rate (interrupted aortic arch, truncus arteriosus, single ventricle, total anomalous pulmonary venous return, double outlet right ventricle, and hypoplastic left heart syndrome) fell within the CCHD category. CONCLUSIONS: Just over half of the predicted CHD patients were identified using the PHIS database. Although there is a high capture rate for CHD that require early hospitalization, there is a low capture rate for defects with a wider spectrum of disease presentation. These attributes of the PHIS database should be used to frame previous and future research using PHIS to study CHD.

Intensive Care Unit and Acute Care Unit Length of Stay After Congenital Heart Surgery.

BACKGROUND: Postoperative length of stay (LOS) is an important quality metric and is known to vary widely across hospitals after congenital heart surgery. Whether this variability is explained by factors associated with the intensive care unit (ICU) or acute care unit (ACU) remains unclear. We evaluated the relationship between ICU and ACU LOS and the impact of ACU characteristics on postoperative LOS. METHODS: Hospitalizations for congenital heart surgery within the Pediatric Cardiac Critical Care Consortium (PC4) registry (August 2014 to February 2018) were included. Models were developed for ICU, ACU, and postoperative LOS by adjusting for differences in case-mix across hospitals. PC4 hospitals participating in the Pediatric Acute Care Cardiology Collaborative (PAC3) were also surveyed on ACU organizational factors and practice patterns. RESULTS: Overall, 19,674 hospitalizations across 27 hospitals were included. There was significant variation in ICU and ACU LOS. Postperative LOS appeared to be most closely related to ICU LOS; 75% (6 of 8) of hospitals with shorter than expected postoperative LOS also had shorter than expected ICU LOS. A clear relationship between postoperative and ACU LOS was not observed. Hospitals with an ACU able to provide higher-acuity care as indexed according to the PAC3 survey were more likely to have shorter postoperative LOS (P < .01). CONCLUSIONS: For hospitals that achieve shorter than expected postoperative LOS after congenital heart surgery, ICU LOS appears to be the primary driver. Higher-acuity resources in the ACU may be an important factor facilitating earlier transfer from the ICU. These data are key to informing quality improvement initiatives geared toward reducing postoperative LOS.

Increasing Burden of Lyme Carditis in United States Children's Hospitals.

We sought to characterize the shifting epidemiology and resource utilization of Lyme disease and associated carditis in US children's hospitals. We hypothesized that the Lyme carditis burden has increased and that hospitalizations for Lyme carditis are costlier than those for Lyme disease without carditis. The PHIS database was queried for Lyme disease encounters between January 1, 2007 and December 31, 2013. Additional diagnostic codes consistent with carditis identified Lyme carditis cases. Demographic, clinical, and resource utilization data were analyzed. All costs were adjusted to 2014 US dollars. Lyme disease was identified in 3620 encounters with 189 (5%) associated with carditis. Lyme disease (360 cases in 2007 vs. 672 in 2013, p = 0.01) and Lyme carditis (17 cases in 2007 vs. 40 in 2013, p = 0.03) both significantly increased in frequency. This is primarily accounted for by their increase within the Midwest region. Carditis frequency among cases of Lyme disease was stable (p = 0.15). Encounters for Lyme carditis are dramatically costlier than those for Lyme disease without carditis [median $9104 (3741-19,003) vs. 922 (238-4987), p < 0.001] The increase in Lyme carditis cases in US children's hospitals is associated with an increased Lyme disease incidence, suggesting that there has not been a change in its virulence or cardiac tropism. The increasing number of serious cardiac events and costs associated with Lyme disease emphasize the need for prevention and early detection of disease and control of its spread.

Variation in care practices across pediatric acute care cardiology units: Results of the Pediatric Acute Care Cardiology Collaborative (PAC3 ) hospital survey.

BACKGROUND: The Pediatric Acute Care Cardiology Collaborative (PAC3 ) was established in 2014 to improve the quality, value, and experience of hospital-based cardiac acute care outside of the intensive care unit. An initial PAC3 project was a comprehensive survey to understand unit structure, practices, and resource utilization across the collaborative. This report aims to describe the previously unknown degree of practice variation across member institutions. METHODS: A 126-stem question survey was developed with a total of 412 possible response fields across nine domains including demographics, staffing, available resources and therapies, and standard care practices. Five supplemental questions addressed surgical case volume and number of cardiac acute care unit (CACU) admissions. Responses were recorded and stored in Research Electronic Data Capture (REDCap). RESULTS: Surveys were completed by 31 out of 34 centers (91%) with minimal incomplete fields. A majority (61%) of centers have a single dedicated CACU, which is contiguous or adjacent to the intensive care unit in 48%. A nurse staffing ratio of 3:1 is most common (71%) and most (84%) centers employed a resource nurse. Centralized wireless rhythm monitoring is used in 84% of centers with 54% staffed continuously. There was significant variation in the use of noninvasive respiratory support, vasoactive infusions, and ventricular assist devices across the collaborative. Approximately half of the surveyed centers had lesion-specific postoperative pathways and approximately two-thirds had protocols for single-ventricle patients. CONCLUSIONS: The PAC3 hospital survey is the most comprehensive description of systems and care practices unique to CACUs to date. There exists considerable heterogeneity among unit composition and variation in care practices. These variations may allow for identification of best practices and improved quality of care for patients.

Impact of Changes Over Time in the Stanford University Genotypic Resistance Interpretation Algorithm.

INTRODUCTION: The Stanford HIV-1 genotypic resistance interpretation algorithm has changed substantially over its lifetime. In many studies, the algorithm version used is not specified. It is easy to assume that results across versions are comparable, but the effects of version changes on resistance calls are unknown. We evaluate these effects for 20 antiretroviral drugs. METHODS: We calculated resistance interpretations for the same 5993 HIV-1 sequences, from participants in AIDS Clinical Trials Group studies, under 14 versions of the Stanford algorithm from 2002 to 2017. Trends over time were assessed using repeated-measures logistic regression. Changes in rule structure and scoring were examined. RESULTS: For most drugs, the proportion of high-level resistance calls on the same sequences was greater using more recent algorithm versions; 16/20 drugs showed significant upward trends. Some drugs, especially tenofovir, had a substantial increase. Only darunavir had a decrease. Algorithm changes impacted calls for subtype C more than B. For intermediate and high-level resistance combined, effects were weaker and more varied. Over time, rules in the Stanford algorithm have become more complex and contain more subrules. The types of rule changes responsible for trends varied widely by drug. DISCUSSION: Reporting the Stanford algorithm version used for resistance analysis is strongly recommended. Caution should be used when comparing results between studies, unless the same version of the algorithm was used. Comparisons using different Stanford versions may be valid for drugs with few changes over time, but for most comparisons, version matters, and for some drugs, the impact is large.

Resource utilization at the time of prostacyclin initiation in children in pulmonary arterial hypertension: a multicenter analysis.

There are limited data investigating the epidemiology and resource utilization associated with parenteral prostacyclin use in children. We sought to examine national trends in treatment practices and resource utilization during prostacyclin initiation for pulmonary arterial hypertension (PAH) at children's hospitals in the United States. Patients with PAH initiated on parenteral epoprostenol and treprostinil (2004-2014) were identified using a nationwide administrative database. Demographics, clinical characteristics, and resource utilization were compared between epoprostenol and treprostinil groups. Costs were indexed in 2014 US dollars. Among 1448 children admitted with a primary or secondary diagnosis of PAH, 280 (19%) were initiated on parenteral prostacyclins (epoprostenol n = 195 and treprostinil n = 85). Epoprostenol predominated early (97% of initiations in 2005); however, treprostinil predominated recently (52-67% of initiations/year). Children initiated on treprostinil had shorter ICU stays (1 [IQR = 0-4] vs. 4 [0-10] days, P < 0.001), shorter total lengths of stay (4 [2-9] vs. 8 [4-18] days, P = 0.001), and lower in-hospital mortality (1 vs. 12%, P = 0.001) with no difference in 30-day (13 vs. 19%, P = 0.19) or one-year readmission rates (56 vs. 61%, P = 0.41). Inpatient costs were lower for treprostinil initiation ($23,779 [11,830-39,535] vs. $32,976 [11,904-94,082], P = 0.03), with a greater difference in the recent era (2009-2013). Though significant variation exists regarding prostacyclin use for PAH across US centers, prostacyclins are common among children with PAH. Treprostinil initiation has been increasing and is associated with less resource utilization and lower cost compared to epoprostenol initiation. Post-discharge outcome data are needed to fully inform decision-making about the relative benefits of parental prostacyclin drug choice.

Health insurance and racial disparities in pulmonary hypertension outcomes.

OBJECTIVES: Pulmonary hypertension portends a poorer prognosis for blacks versus white populations, but the underlying reasons are poorly understood. We investigated associations of disease characteristics, insurance status, and race with clinical outcomes. STUDY DESIGN: Retrospective cohort study of patients presenting for initial pulmonary hypertension evaluation at 2 academic referral centers. METHODS: We recorded insurance status (Medicare, Medicaid, private, self-pay), echocardiographic, and hemodynamics data from 261 patients (79% whites, 17% blacks) with a new diagnosis of pulmonary hypertension. Subjects were followed for 2.3 years for survival. Adjustment for covariates was performed with Cox proportional hazards modeling. RESULTS: Compared with white patients, blacks were younger (50 ± 15 vs 53 ± 12 years; P = .04), with females representing a majority of patients in both groups (80% vs 66%; P = .08) and similar functional class distribution (class 2/3/4: 30%/52%/16% blacks vs 33%/48%/14% whites; P = .69). Blacks diagnosed with incident pulmonary hypertension were more frequently covered by Medicaid (12.5% vs 0.7%) and had less private insurance (50% vs 61%; P = .007) than whites. At presentation, blacks had more right ventricular dysfunction (P = .04), but similar mean pulmonary arterial pressure (46 vs 45 mm Hg, respectively; P = .66). After adjusting for age and functional class, blacks had greater mortality risk (hazard ratio [HR], 2.06; 95% confidence interval [CI], 1.18-3.44), which did not differ by race after additional adjustment for insurance status (HR, 1.74; 95% CI, 0.84-3.32; P =.13). CONCLUSIONS: In a large cohort of patients with incident pulmonary hypertension, black patients had poorer right-side heart function and survival rates than white patients. However, adjustment for insurance status in our cohort removed differences in survival by race.

PINOT NOIR: pulmonic insufficiency improvement with nitric oxide inhalational response.

BACKGROUND: Tetralogy of Fallot (TOF) repair and pulmonary valvotomy for pulmonary stenosis (PS) lead to progressive pulmonary insufficiency (PI), right ventricular enlargement and dysfunction. This study assessed whether pulmonary regurgitant fraction measured by cardiovascular magnetic resonance (CMR) could be reduced with inhaled nitric oxide (iNO). METHODS: Patients with at least moderate PI by echocardiography undergoing clinically indicated CMR were prospectively enrolled. Patients with residual hemodynamic lesions were excluded. Ventricular volume and blood flow sequences were obtained at baseline and during administration of 40 ppm iNO. RESULTS: Sixteen patients (11 with repaired TOF and 5 with repaired PS) completed the protocol with adequate data for analysis. The median age [range] was 35 [19-46] years, BMI was 26 ± 5 kg/m(2) (mean ± SD), 50% were women and 75% were in NYHA class I. Right ventricular end diastolic volume index for the cohort was 157 ± 33 mL/m(2), end systolic volume index was 93 ± 20 mL/m(2) and right ventricular ejection fraction was 40 ± 6%. Baseline pulmonary regurgitant volume was 45 ± 25 mL/beat and regurgitant fraction was 35 ± 16%. During administration of iNO, regurgitant volume was reduced by an average of 6 ± 9% (p=0.01) and regurgitant fraction was reduced by an average of 5 ± 8% (p=0.02). No significant changes were observed in ventricular indices for either the left or right ventricle. CONCLUSION: iNO was successfully administered during CMR acquisition and appears to reduce regurgitant fraction in patients with at least moderate PI suggesting a potential role for selective pulmonary vasodilator therapy in these patients. TRIALS REGISTRATION: ClinicalTrials.gov, NCT00543933.

Modified INOvent for delivery of inhaled nitric oxide during cardiac MRI.

BACKGROUND: The aim of this study was to assess the feasibility of delivering NO through a modified system to allow clearance of the magnetic field and thus compatibility with cardiac magnetic resonance (CMR). Nitric oxide (NO) is an inhalational, selective pulmonary vasodilator with a wide range of applications in a variety of disease states, including diseases that affect the right ventricle. Accurate assessment of dynamic changes in right ventricular function necessitates CMR; however, delivery of NO is only possible using equipment that is not magnetic resonance imaging (MRI) compatible (INOvent delivery system, Ohmeda, Inc., Madison, WI, USA). METHODS: The INOvent delivery system was modified by using 35 ft. of standard oxygen tubing to allow NO delivery through an electrical conduit and into the MRI suite. The concentrations of oxygen (O(2)), nitrogen dioxide (a harmful byproduct, NO(2)) and NO were measured in triplicate using the built-in electrochemical analyzer on the INOvent. After confirmation of safety, the system was used to administer drug to a patient x, and dynamic MRI measurements were performed. RESULTS: When the standard INOvent was set to administer 40 ppm of NO, the mean/standard deviation of gas delivered was as follows: NO: 42/0 ppm; NO(2): 0.3/0.1 ppm; and O(2): 93/0 ppm. In comparison, the gas delivery of the modified INOvent was follows: NO: 41/0 ppm; NO(2): 0.5/0 ppm; and O(2): 93.7/0.6 ppm. During administration to an index patient with severe pulmonic insufficiency (PI), a measurable reduction in PI was observed by CMR. CONCLUSIONS: Nitric oxide can be administered through 35 ft. of standard oxygen tubing without significantly affecting dose delivery. This technique has potential application in patients with right-sided structural heart disease for determination of dynamic physiological changes.

Response to inhaled nitric oxide predicts survival in patients with pulmonary hypertension.

OBJECTIVE: To examine the ability of vasodilator response to predict survival in a diverse cohort of patients with pulmonary hypertension (PH). PATIENTS & METHODS: A total of 214 consecutive treatment-naive patients referred for invasive PH evaluation were enrolled between November 1998 and December 2008. Vasoreactivity was assessed during inhalation of 40 parts per million nitric oxide (iNO) and vasodilator responders were defined as those participants who achieved a mean pulmonary artery pressure (PAP) of ≤ 40 mm Hg and a drop in mean PAP ≥ the median for the cohort (13%). Kaplan-Meier analysis and Cox proportional hazards modeling were used to identify predictors of survival. RESULTS: There were 51 deaths (25.9%) over a mean follow-up period of 2.3 years. Kaplan-Meier analysis demonstrated that vasodilator responders had significantly improved survival (P < .01). Vasodilator responders had improved survival regardless of whether or not they had idiopathic or nonidiopathic PH (P = .02, P < .01) or whether or not they had Dana Point class 1 or non-Dana Point class 1 PH (P < .01, P = .01). In multivariate modeling, advanced age, elevated right atrial pressure, elevated serum creatinine, and worsened functional class significantly predicted shorter survival (P = .01, P = .01, P = .01, P < .01), whereas vasodilator response predicted improved survival (P = .01). CONCLUSIONS: Vasodilator responsiveness to iNO is an important method of risk stratifying PH patients, with results that apply regardless of clinical etiology.

Association of anemia and long-term survival in patients with pulmonary hypertension.

BACKGROUND: Anemia is a marker of worsened clinical outcome in patients with heart failure from left ventricular dysfunction. Pulmonary hypertension often results in right ventricular dysfunction. Accordingly we sought to examine the association of hemoglobin levels and long-term all-cause mortality in a cohort of patients with pulmonary hypertension. METHODS: Baseline demographic information, clinical characteristics and fasting blood work were obtained in a cohort of 145 patients with pulmonary hypertension referred for pulmonary vasodilator testing. Data was retrospectively analyzed with Cox-proportional hazards analysis. RESULTS: Baseline characteristics of the cohort included age (mean±SD) 55.8±14.6 years, 75% women, 50% with idiopathic pulmonary hypertension, mean pulmonary artery pressure 46.1±14.2 mm Hg and arterial O(2) saturation 91±6 %. The most commonly utilized pulmonary hypertension specific therapeutic agents in descending order of frequency were epoprostenol (27%), sildenafil (21%), bosentan (17%), and treprostinil (6%). Over a median follow-up of 2.1 years, there were 39 deaths (26.9%). Patients who died had significantly lower hemoglobin levels than those survived (12.2±2.3 vs. 13.7±2.0, p<0.001). After adjustment for known predictors of death and pulmonary hypertension etiology, anemic patients were 3.3 times more likely to die than non-anemic patients (95% CI [1.43-7.51], p=0.005). CONCLUSIONS: Hemoglobin levels closely parallel survival in pulmonary hypertension. Modification of anemia in this disorder could alter the clinical course and calls for further research in this area.

When 'blue babies' grow up: What you need to know about tetralogy of Fallot.

Most babies born with tetralogy of Fallot undergo corrective surgery and survive to adulthood. However, as they get older they are prone to a number of long-term problems, and they often do not receive expert-level follow-up care. This review of the adult complications of tetralogy of Fallot should help primary care practitioners identify these patients, make appropriate and timely referrals, and educate patients and their families.

Impact of fibrinogen levels on angiographic progression and 12-year survival in the armed forces regression study.

We assessed the role of fibrinogen levels on angiographic progression and long-term survival among 111 patients with coronary disease enrolled in the Armed Forces Regression Study (AFREGS). Baseline fibrinogen levels and quantitative coronary angiography were performed initially and at 30 months. Progression or nonregression of coronary disease was more prevalent in patients with high fibrinogen than patients with normal fibrinogen (66.1% vs 45.5%; P = .022). Twelve-year cardiovascular (CV) mortality was substantially higher if fibrinogen was elevated (17.9% vs 3.6%, P = .016). Among patients with elevated fibrinogen and angiographic progression or nonregression, there were 10 deaths and all were CV. Elevated levels of fibrinogen predict the angiographic progression of existing coronary disease and likelihood of CV death. Among patients with elevated levels of fibrinogen, angiographic progression identifies a significantly increased likelihood of a fatal CV event.