ADVERSE EVENTS ASSOCIATED WITH ORAL ANTICOAGULATION OF PATIENTS WITH CONGENITAL HEART DISEASE

Background: Adults with congenital heart disease are a growing population. We describe surgical interventions, short- and long-term mortality and morbidity, and risk factors for adverse events in a population-based cohort. Methods: Patients over or equal to 18 years with congenital heart disease who underwent cardiac surgery at Aarhus University Hospital, Denmark, from 1994 to 2012 were included in the study. Diagnoses, surgical procedures, postoperative complications, and survival were identified in hospital databases, medical records, and the Danish Civil Registration System. Results: Four hundred seventy-four surgeries were performed in 445 adults (50% men). The median age was 39 years (range 18-83). Thirty-nine percent had previous surgical or catheter-based interventions. Thirty-day and in-hospital mortality were 1.1%. Postoperative complications occurred in 50% of cases, most were minor such as temporary arrhythmias and pneumonia. Major complications included postoperative bleeding necessitating intervention (6%), stroke (2%), and acute temporary renal failure (1%). Multivariate analysis identified RACHS-1 categories over or equal to 3 compared to category 1 (odds ratio (OR) = 2.3; 95% confidence interval (CI): 1.5-3.7), New York Heart Association functional class III and IV compared to class I (OR = 2.2; 95% CI: 1.3-3.7) and age at surgery (OR = 1.03, 95% CI: 1.01-1.04), as risk factors for adverse events. Survival during a median follow-up of 7.8 years (range 0 days-21.4 years) was 85% (95% CI: 80%-89%). Conclusion: Adults with congenital heart disease constitute a growing population with the need for cardiac surgery. Postoperative complications are frequent but early and late mortality are low.

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Review of 54 patients with complete DiGeorge anomaly enrolled in protocols for thymus transplantation: outcome of 44 consecutive transplants

Blood ◽

10.1182/blood-2006-10-048652 ◽

2007 ◽

Vol 109 (10) ◽

pp. 4539-4547 ◽

Cited By ~ 142

Author(s):

M. Louise Markert ◽

Blythe H. Devlin ◽

Marilyn J. Alexieff ◽

Jie Li ◽

Elizabeth A. McCarthy ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

T Cell ◽

Adverse Events ◽

Congenital Heart ◽

Post Transplantation ◽

Thymus Transplantation ◽

Atypical Phenotype ◽

Digeorge Anomaly

Abstract The purpose of this study was to characterize a large group of infants with complete DiGeorge anomaly and to evaluate the ability of thymus transplantation to reconstitute immune function in these infants. DiGeorge anomaly is characterized by varying defects of the heart, thymus, and parathyroid glands. Complete DiGeorge anomaly refers to the subgroup that is athymic (< 1%). The characteristics of 54 subjects at presentation and results from 44 consecutive thymus transplantations are reported. Remarkably, only 52% had 22q11 hemizygosity and only 57% had congenital heart disease requiring surgery. Thirty-one percent developed an atypical phenotype with rash and lymphadenopathy. To date, 33 of 44 subjects who received a transplant survive (75%) with post-transplantation follow-up as long as 13 years. All deaths occurred within 12 months of transplantation. All 25 subjects who were tested 1 year after transplantation had developed polyclonal T-cell repertoires and proliferative responses to mitogens. Adverse events developing after transplantation included hypothyroidism in 5 subjects and enteritis in 1 subject. In summary, diagnosis of complete DiGeorge anomaly is challenging because of the variability of presentation. Thymus transplantation was well tolerated and resulted in stable immunoreconstitution in these infants.

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Outcomes and Prognostic Factors for Adult Patients With Congenital Heart Disease Undergoing Primary or Reoperative Systemic Atrioventricular Valve Surgery

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135117692974 ◽

2017 ◽

Vol 8 (3) ◽

pp. 346-353

Author(s):

Elizabeth H. Stephens ◽

Jiho Han ◽

Jonathan Ginns ◽

Marlon Rosenbaum ◽

Paul Chai ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Prognostic Factors ◽

Adverse Events ◽

Congenital Heart ◽

Atrioventricular Septal Defect ◽

The Other ◽

Mitral Valve Stenosis ◽

Atrioventricular Valve ◽

Exact Test

Background: Adults with congenital heart disease (ACHD) undergoing systemic atrioventricular valve (SAVV) surgery are a complex, understudied population. We assessed midterm outcomes and prognostic factors in ACHD undergoing SAVV surgery. Methods: We performed retrospective evaluation of ACHD undergoing SAVV surgery from January 2005 to February 2016: 14 (33%) patients with congenital mitral valve stenosis/regurgitation, 15 (35%) with atrioventricular septal defect (AVSD), and 14 (33%) with congenitally corrected transposition of the great arteries (ccTGA) with systemic tricuspid valve regurgitation. Adverse events were defined as mortality, reoperation on SAVV, and late more-than-moderate (> moderate) SAVV regurgitation. Statistical analysis was performed using Fisher’s exact test and one-way analysis of variance as well as univariate and multivariate risk factor analysis. Results: Fifteen (35%) patients had preoperative systemic ventricular dysfunction, including 13 patients with ccTGA (93%, P < .001). Twenty-three (54%) patients underwent valve repair, 20 (47%) patients underwent replacement, and 20 (47%) patients underwent an associated procedure. Replacement was higher in patients with ccTGA (86%) than the other groups ( P < 0.01). Thirty-seven patients (91% of survivors) were free of significant SAVV regurgitation at last follow-up, with patients with AVSD having greater regurgitation grades compared to the other groups ( P < 0.01). In-hospital mortality, late mortality, late > moderate SAVV regurgitation, and SAVV reoperation rates were 5% (n = 2), 2% (n = 1), 9% (n = 3), and 7% (n = 3), respectively. On multivariate analysis, predischarge SAVV regurgitation grade was the only significant predictor of adverse events (odds ratio = 8.2, 95% confidence interval: 1.1-63.8, P = .045). Conclusion: Overall outcomes in this challenging population are good. The single factor associated with adverse events was predischarge SAVV regurgitation grade.

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Characteristics and safety of interventions and procedures performed during catheterisation of patients with congenital heart disease: early report from the national cardiovascular data registry

Cardiology in the Young ◽

10.1017/s1047951115002218 ◽

2015 ◽

Vol 26 (6) ◽

pp. 1202-1212 ◽

Cited By ~ 8

Author(s):

Ralf Holzer ◽

Robert Beekman ◽

Lee Benson ◽

Lisa Bergersen ◽

Natalie Jayaram ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Data Collection ◽

Adverse Events ◽

Congenital Heart ◽

Early Report ◽

Time Frame ◽

Adult Congenital ◽

Data Elements ◽

Major Adverse Events

AbstractObjectivesThe objective of this study was to report procedural characteristics and adverse events on the data collected in the IMproving Paediatric and Adult Congenital Treatment registry.BackgroundThe IMproving Paediatric and Adult Congenital Treatment– registry is a catheterisation registry focussed on paediatric and adult patients with congenital heart disease who are undergoing diagnostic catheterisations and catheter-based interventions. This study reports procedural characteristics and adverse events of patients who have undergone selected catheterisation procedures from January, 2011 to June, 2013.MethodsDemographic, clinical, procedural, and institutional data elements were collected at participating centres and entered via either a web-based platform or software provided by the American College of Cardiology-certified vendors, and were collected in a secure, centralised database. For the purpose of this study, procedures that were not classified as one of the ‘core’ IMproving Paediatric and Adult Congenital Treatment procedures originally chosen for additional data collection were identified and selected for further data analysis.ResultsDuring the time frame of data collection, a total of 8021 cases were classified as other procedures and/or multiple procedures. The most commonly performed case types – isolated or in combination with other procedures – were right ventricular biopsy in 3433 (42.8%), conduit/MPA interventions in 979 (12.3%), and systemic pulmonary artery collateral occlusion in 601 (7.5%). For the whole cohort, adverse events of any severity occurred in 957 (12.0%) cases, whereas major adverse events occurred in 113 (1.4%) cases; six patients (0.1%) died in the catheterisation laboratory.ConclusionsThe IMproving Paediatric and Adult Congenital Treatment registry has provided important data on the frequency and spectrum of cardiac catheterisation procedures performed in the present era. For many procedures, more data and work are needed to identify more subtle differences between case categories, especially as it relates to the incidence of major adverse events, and to further develop a risk-adjustment methodology to allow equitable comparisons among institutions.

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Incidence and Management of Life-Threatening Adverse Events During Cardiac Catheterization for Congenital Heart Disease

Pediatric Cardiology ◽

10.1007/s00246-013-0752-y ◽

2013 ◽

Vol 35 (1) ◽

pp. 140-148 ◽

Cited By ~ 24

Author(s):

C. Huie Lin ◽

Sanjeet Hegde ◽

Audrey C. Marshall ◽

Diego Porras ◽

Kimberlee Gauvreau ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Adverse Events ◽

Cardiac Catheterization ◽

Congenital Heart ◽

Life Threatening

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77Infective endocarditis in patients with congenital heart disease: Results of a nationwide study including 1494 endocarditis cases

European Heart Journal ◽

10.1093/eurheartj/ehz747.0013 ◽

2019 ◽

Vol 40 (Supplement_1) ◽

Author(s):

G P Diller ◽

E Freisinger ◽

L Bronstein ◽

J Koeppe ◽

J Gerss ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Relative Risk ◽

Infective Endocarditis ◽

Adverse Events ◽

Congenital Heart ◽

Surgical Intervention ◽

High Morbidity ◽

Heart Defect ◽

Major Adverse Events

Abstract Background Infective endocarditis (IE) represents a major complication in patients with congenital heart disease (CHD) and is associated with high morbidity and mortality. The aim of this study was to analyse the frequency and outcome of IE in contemporary CHD patients based on all IE hospital admissions in Germany between 2011 and 2016. Methods Based on the German diagnosis related groups data of patients treated between 2011 and 2016, we identified all CHD patients with a diagnosis of IE. The data contains information on patient demographics, primary and secondary diagnoses, interventional or surgical procedures, duration of stay and outcome including mortality. The primary endpoint of the study was endocarditis-associated mortality as well as major adverse events (defined as death or myocardial infarction, stroke, pulmonary embolism, sepsis, resuscitation or intubation). Results Overall, 181,924 CHD patients were included in the analysis (55% male; 73% children, mean age 2.3 years; 27% adults, mean age 58.4 years; underlying heart defect of simple complexity 55%, moderate complexity 23% and complex heart defect 22%, respectively). During the study period 1494 cases were treated for IE corresponding to 0.82% of all inpatient treatment cases in CHD patients. Mortality rate was 6.6% (95% CI: 5.0–7.6%) with a major adverse events rate of 44.6% (95% CI: 41.3–48.2%). In total, 682 IE patients (45.7% CI: 42.3%-49–2%) required a surgical intervention. The relative risk of dying due to endocarditis in CHD patients was significantly lower compared to older IE patients without CHD (relative risk 0.39; 95% CI: 0.32–0.47). Conclusions Infective endocarditis accounts for a minority of CHD related hospitalizations but remains a deadly disease with a high proportion of patients requiring surgical intervention. In addition, major adverse events are common in this setting, with almost half of the IE population presenting with a major adverse event. Due to different demographic and comorbidity spectrum encountered in CHD patients, these younger patients tend to have significantly better survival prospects compared to non-CHD IE patients in the current era.

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