Chronic Disease Burden After Congenital Heart Surgery: A 47‐Year Population‐Based Study With 99% Follow‐Up

AbstractBackgroundWith improvements in early survival following congenital heart surgery, it has become increasingly important to understand longer-term outcomes; however, routine collection of these data is challenging and remains very limited. We describe the development and initial results of a collaborative programme incorporating standardised longitudinal follow-up into usual care at the Children’s Hospital of Philadelphia (CHOP) and University of Michigan (UM).MethodsWe included children undergoing benchmark operations of the Society of Thoracic Surgeons. Considerations regarding personnel, patient/parent engagement, funding, regulatory issues, and annual data collection are described, and initial follow-up rates are reported.ResultsThe present analysis included 1737 eligible patients undergoing surgery at CHOP from January 2007 to December 2014 and 887 UM patients from January 2010 to December 2014. Overall, follow-up data, of any type, were obtained from 90.8% of patients at CHOP (median follow-up 4.3 years, 92.2% survival) and 98.3% at UM (median follow-up 2.8 years, 92.7% survival), with similar rates across operations and institutions. Most patients lost to follow-up at CHOP had undergone surgery before 2010. Standardised questionnaires assessing burden of disease/quality of life were completed by 80.2% (CHOP) and 78.4% (UM) via phone follow-up. In subsequent pilot testing of an automated e-mail system, 53.4% of eligible patients completed the follow-up questionnaire through this system.ConclusionsStandardised follow-up data can be obtained on the majority of children undergoing benchmark operations. Ongoing efforts to support automated electronic systems and integration with registry data may reduce resource needs, facilitate expansion across centres, and support multi-centre efforts to understand and improve long-term outcomes in this population.

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Prescription medication use after congenital heart surgery

Cardiology in the Young ◽

10.1017/s1047951121005060 ◽

2022 ◽

pp. 1-8

Author(s):

Alireza Raissadati ◽

Jari Haukka ◽

Tommi Pätilä ◽

Heta Nieminen ◽

Eero Jokinen

Keyword(s):

General Population ◽

Congenital Heart ◽

Heart Surgery ◽

Medication Use ◽

Prescription Medication ◽

Congenital Heart Surgery ◽

Hazard Ratio ◽

Prescription Medications

Abstract Background: Improvements in mortality after congenital heart surgery have necessitated a shift in focus to postoperative morbidity as an outcome measure. We examined late morbidity after congenital heart surgery based on prescription medication use. Methods: Between 1953 and 2009, 10,635 patients underwent congenital heart surgery at <15 years of age in Finland. We obtained 4 age-, sex-, birth-time, and hospital district-matched controls per patient. The Social Insurance Institution of Finland provided data on all prescription medications obtained between 1999 and 2012 by patients and controls. Patients were assigned one diagnosis based on a hierarchical list of cardiac defects and dichotomised into simple and severe groups. Medications were divided into short- and long-term based on indication. Follow-up started at the first operation and ended at death, emigration, or 31 December, 2012. Results: Totally, 8623 patients met inclusion criteria. Follow-up was 99.9%. In total, 8126 (94%) patients required prescription medications. Systemic anti-bacterials were the most common short-term prescriptions among patients (93%) and controls (88%). Patients required betablockers (simple hazard ratio 1.9, 95% confidence interval 1.7–2.1; severe hazard ratio 6.5, 95% confidence interval 5.3–8.1) and diuretics (simple hazard ratio 3.2, 95% CI 2.8–3.7; severe hazard ratio 38.8, 95% CI 27.5–54.7) more often than the general population. Both simple and severe defects required medication for cardiovascular, gastrointestinal, psychiatric, neurologic, metabolic, autoimmune, and infectious diseases more often than the general population. Conclusions: The significant risk for postoperative cardiovascular and non-cardiovascular disease warrants close long-term follow-up after congenital heart surgery for all defects.

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Population-based study of cognitive outcomes in congenital heart defects

Archives of Disease in Childhood ◽

10.1136/archdischild-2016-310830 ◽

2017 ◽

Vol 103 (1) ◽

pp. 49-56 ◽

Cited By ~ 7

Author(s):

Johanna Calderon ◽

Marion Willaime ◽

Nathalie Lelong ◽

Damien Bonnet ◽

Lucile Houyel ◽

...

Keyword(s):

Congenital Heart Defects ◽

Congenital Heart ◽

Heart Surgery ◽

Heart Defects ◽

Open Heart Surgery ◽

Population Based ◽

Cognitive Outcomes ◽

Open Heart ◽

Population Based Study ◽

Surgical Group

ObjectiveTo characterise and compare cognitive outcomes in children with operated (open-heart surgery) and non-operated (catheter-based interventions only or no intervention) congenital heart defects (CHD) and to determine associated risk factors.DesignThis prospective population-based study reports outcomes of 3-year-old children with CHD with or without open-heart surgery.Main outcome measuresStandardised cognitive scores (mean scores and proportions below normative values) were assessed with the Kaufman Assessment Battery for Children II. We analysed demographic, perinatal and operative variables as predictors of cognitive outcomes.Results419 children participated (154 with open-heart surgery; 265 without surgery). Global cognitive scores did not differ between the groups. Compared with the non-operated group, children who underwent surgery obtained lower scores in expressive language (p=0.03) and logical reasoning (p=0.05). When compared with test norms, the frequency of global cognitive scores >1 SDs below the expected mean was higher in the surgical group (25% vs 16% in the general population) (p=0.03). A higher-than-expected proportion of children in the non-operated group scored >2 SDs below the expected mean (7% vs 2%) (p=0.05). Being small for gestational age (SGA) significantly increased the risk of cognitive impairment in the surgical group, after adjustments for multiple covariates including maternal education, complexity of the CHD and operative-related variables (adjusted OR=5.9; 95% CI (1.7 to 20.1)).ConclusionsDespite mean scores within the normative range, a high proportion of preschool children with CHD with or without surgery are at early cognitive risk. SGA is a strong predictor of the neurodevelopmental prognosis in CHD.

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Analysis of outcomes for congenital cardiac disease: can we do better?

Cardiology in the Young ◽

10.1017/s1047951107001278 ◽

2007 ◽

Vol 17 (S4) ◽

pp. 145-158 ◽

Cited By ~ 35

Author(s):

Jeffrey P. Jacobs ◽

Gil Wernovsky ◽

Martin J. Elliott

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Thoracic Surgery ◽

Working Group ◽

European Association ◽

Congenital Heart ◽

Heart Surgery ◽

Congenital Heart Surgery ◽

European Paediatric

AbstractThis review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up. During the 1990s, both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature, along with a common core minimal dataset, were adopted by The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons, and published in the Annals of Thoracic Surgery. In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. By 2005, the Nomenclature Working Group crossmapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology, and therefore created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET].This common nomenclature, the International Paediatric and Congenital Cardiac Code, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons. Between 1998 and 2007 inclusive, this nomenclature and database was used by both these two organizations to analyze outcomes of over 100,000 patients undergoing surgical treatment for congenital cardiac disease. Two major multi-institutional efforts that have attempted to measure the complexity of congenital heart surgery are the Risk Adjustment in Congenital Heart Surgery-1 system, and the Aristotle Complexity Score. Current efforts to unify the Risk Adjustment in Congenital Heart Surgery-1 system and the Aristotle Complexity Score are in their early stages, but encouraging. Collaborative efforts involving The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons are under way to develop mechanisms to verify the completeness and accuracy of the data in the databases. Under the leadership of The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease, further collaborative efforts are ongoing between paediatric and congenital cardiac surgeons and other subspecialties, including paediatric cardiac anaesthesiologists, via The Congenital Cardiac Anesthesia Society, paediatric cardiac intensivists, via The Pediatric Cardiac Intensive Care Society, and paediatric cardiologists, via the Joint Council on Congenital Heart Disease and The Association for European Paediatric Cardiology.In finalising our review, we emphasise that analysis of outcomes must move beyond mortality, and encompass longer term follow-up, including cardiac and non cardiac morbidities, and importantly, those morbidities impacting health related quality of life. Methodologies must be implemented in these databases to allow uniform, protocol driven, and meaningful, long term follow-up.

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Nomenclature and databases for the surgical treatment of congenital cardiac disease – an updated primer and an analysis of opportunities for improvement

Cardiology in the Young ◽

10.1017/s1047951108003028 ◽

2008 ◽

Vol 18 (S2) ◽

pp. 38-62 ◽

Cited By ~ 52

Author(s):

Jeffrey Phillip Jacobs ◽

Marshall Lewis Jacobs ◽

Constantine Mavroudis ◽

Carl Lewis Backer ◽

Francois G. Lacour-Gayet ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Thoracic Surgery ◽

Working Group ◽

European Association ◽

Congenital Heart ◽

Heart Surgery ◽

Congenital Heart Surgery ◽

European Paediatric

AbstractThis review discusses the historical aspects, current state of the art, and potential future advances in the areas of nomenclature and databases for the analysis of outcomes of treatments for patients with congenitally malformed hearts. We will consider the current state of analysis of outcomes, lay out some principles which might make it possible to achieve life-long monitoring and follow-up using our databases, and describe the next steps those involved in the care of these patients need to take in order to achieve these objectives. In order to perform meaningful multi-institutional analyses, we suggest that any database must incorporate the following six essential elements: use of a common language and nomenclature, use of an established uniform core dataset for collection of information, incorporation of a mechanism of evaluating case complexity, availability of a mechanism to assure and verify the completeness and accuracy of the data collected, collaboration between medical and surgical subspecialties, and standardised protocols for life-long follow-up.During the 1990s, both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons created databases to assess the outcomes of congenital cardiac surgery. Beginning in 1998, these two organizations collaborated to create the International Congenital Heart Surgery Nomenclature and Database Project. By 2000, a common nomenclature, along with a common core minimal dataset, were adopted by The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons, and published in the Annals of Thoracic Surgery. In 2000, The International Nomenclature Committee for Pediatric and Congenital Heart Disease was established. This committee eventually evolved into the International Society for Nomenclature of Paediatric and Congenital Heart Disease. The working component of this international nomenclature society has been The International Working Group for Mapping and Coding of Nomenclatures for Paediatric and Congenital Heart Disease, also known as the Nomenclature Working Group. By 2005, the Nomenclature Working Group crossmapped the nomenclature of the International Congenital Heart Surgery Nomenclature and Database Project of The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons with the European Paediatric Cardiac Code of the Association for European Paediatric Cardiology, and therefore created the International Paediatric and Congenital Cardiac Code, which is available for free download from the internet at [http://www.IPCCC.NET].This common nomenclature, the International Paediatric and Congenital Cardiac Code, and the common minimum database data set created by the International Congenital Heart Surgery Nomenclature and Database Project, are now utilized by both The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons. Between 1998 and 2007 inclusive, this nomenclature and database was used by both of these two organizations to analyze outcomes of over 150,000 operations involving patients undergoing surgical treatment for congenital cardiac disease.Two major multi-institutional efforts that have attempted to measure the complexity of congenital heart surgery are the Risk Adjustment in Congenital Heart Surgery-1 system, and the Aristotle Complexity Score. Current efforts to unify the Risk Adjustment in Congenital Heart Surgery-1 system and the Aristotle Complexity Score are in their early stages, but encouraging. Collaborative efforts involving The European Association for Cardio-Thoracic Surgery and The Society of Thoracic Surgeons are under way to develop mechanisms to verify the completeness and accuracy of the data in the databases. Under the leadership of The MultiSocietal Database Committee for Pediatric and Congenital Heart Disease, further collaborative efforts are ongoing between congenital and paediatric cardiac surgeons and other subspecialties, including paediatric cardiac anaesthesiologists, via The Congenital Cardiac Anesthesia Society, paediatric cardiac intensivists, via The Pediatric Cardiac Intensive Care Society, and paediatric cardiologists, via the Joint Council on Congenital Heart Disease and The Association for European Paediatric Cardiology.In finalising our review, we emphasise that analysis of outcomes must move beyond mortality, and encompass longer term follow-up, including cardiac and non cardiac morbidities, and importantly, those morbidities impacting health related quality of life. Methodologies must be implemented in these databases to allow uniform, protocol driven, and meaningful, long term follow-up.

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Outcomes of home mechanical ventilation with tracheostomy after congenital heart surgery

Cardiology in the Young ◽

10.1017/s1047951121001657 ◽

2021 ◽

pp. 1-5

Author(s):

Bahar Temur ◽

İsmet E Emre ◽

Selim Aydın ◽

Mehmet A Önalan ◽

Serdar Başgöze ◽

...

Keyword(s):

Mechanical Ventilation ◽

Median Duration ◽

Congenital Heart ◽

Heart Surgery ◽

Total Mortality ◽

Congenital Heart Surgery ◽

Home Mechanical Ventilation ◽

Significant Difference

Abstract Objective: After congenital heart surgery, some patients may need long-term mechanical ventilation because of chronic respiratory failure. In this study, we analysed outcomes of the patients who need tracheostomy and home mechanical ventilation. Methods: Amongst 1343 patients who underwent congenital heart surgery between January, 2014 and June, 2018, 45 needed tracheostomy and HMV. The median age of these patients was 6.4 months (12 days–6.5 years). Nineteen patients underwent palliation while 26 patients underwent total repair. Post-operative diaphragm plication was performed in five patients (11%). Median duration of mechanical ventilation before tracheostomy was 32 days (8–154 days). The patients were followed up with their home ventilators in ward and at home. Mean follow-up time was 36.24 ± 11.61 months. Results: The median duration of ICU stay after tracheostomy was 27 days (range 2–93 days). Follow-up time in ward was median 30 days (2–156 days). A total of 12 patients (26.6%) were separated from the ventilator and underwent decannulation during hospital stay. Thirty-two patients (71.1%) were discharged home with home ventilator support. Of them, 15 patients (46.9%) were separated from the respiratory support in median of 6 weeks (1 week–11 months) and decannulations were performed. Total mortality was 31.1%. in which four patients are still HMV dependent. There was no significant difference for decannulation between total repair and palliation patients. Conclusion: HMV via tracheostomy is a useful option for the treatment of children who are dependent on long-term ventilation after congenital heart surgery although there are potential risks.

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Congenital Heart Surgery: Analysis of 102 Cases

Bangladesh Heart Journal ◽

10.3329/bhj.v30i2.28812 ◽

2016 ◽

Vol 30 (2) ◽

pp. 58-60

Author(s):

AKM Manzurul Alam ◽

Istiaq Ahmed ◽

Manzil Ahmed ◽

Al Mamun Hossain

Keyword(s):

Congenital Heart ◽

Heart Surgery ◽

Septal Defect ◽

Heart Diseases ◽

Ductus Arteriosus ◽

Signs And Symptoms ◽

Congenital Heart Surgery ◽

Corrective Surgery ◽

Life Threatening

Congenital heart diseases (CHDs) are a group of problems in the structure of the heart that is present at birth. Signs and symptoms depend on the specific type of problem. Symptoms can vary from none to life-threatening. CHD is prevalent throughout the world including Bangladesh. It is the leading cause of birth defect-related death. Most of the patients with CHDs need corrective surgery and life-long follow up with heart care. In this series, 102 cases of CHD patients were operated, 82 of them were of atrial septal defect (ASD), 12 ventricular septal defect (VSD), 2 Tetralogy of Fallot (TOF) and 6 patent ductus arteriosus (PDA). Operative and post-operative periods were uneventful. There were no major complications including death.Bangladesh Heart Journal 2015; 30(2) : 58-60

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