1. FEATURES AND CONCEPTS SAUDI CONGENITAL HEART DEFECTS REGISTRY Research Advisory Council Number: 991026

2. Congenital Heart Defects Registry (CHDR) is a disease registry. It is an ongoing process of data collection, entry, analyses and reporting, for a defined population diagnosed with Congenital Heart Defect(s). Patients registration is both retrospective and prospective. What is Congenital Heart Defects Registry?

3. The registry started in 1998 on a hospital level, as a research collaboration between The Heart Center and Registries Core Facility at the Biostatistics Epidemiology and Scientific Computing Department, King Faisal Specialist Hospital & Research Center, Riyadh (KFSH&RC). In the year 2003 the registry evolved to multi-institutional level with regional and national hospitals collaboration. About the Registry Heart Center at KFSH&RC, Riyadh. Cardiovascular Department at KFSH&RC, Jeddah. Prince Sultan Cardiac Center, Riyadh. King Abdulaziz Cardiac Center at the National Guard Affairs Hospital, Riyadh. King Fahad Medical City, Riyadh. Maternity Children Hospital, Al-Dammam. Collaborating Hospitals

4. All patients diagnosed to have CHDs are eligible for registration, regardless of their age, gender or nationality. Inclusion Criteria: Coding System: The European Pediatric Cardiology Coding list (EPCC) is the system in use to code the abstracted CHDs and intervention descriptions (heart surgeries and cardiac catheter).

5. Objectives To provide leadership in establishing and maintaining comprehensive CHD registration with other health organizations. To support scientific and clinical research. To provide hospital administrators with sufficient data for proper health planning. To serve as a surveillance tool for monitoring long range trends of congenital heart diseases.

6. Major Activities Patients registration in the cardiovascular clinics and wards. Abstraction of CHD lesions and interventions through reviewing the patients medical chart. Recording patients status and disease status. Death-log update for the deceased patients. Telephone follow-up. Ongoing process to document new intervention episodes (heart surgery, cardiac catheter) while patients admission to the CV wards. Patients (mother, father, sibling, off spring) within the same families with positive history for CHD are clustered and assigned a unique family number.

7. CHDR Life Cycle Data collection and abstraction Data entry Data validation and quality assurance of collected data. Data analysis for reporting and publishing Sharing Information

8. CHDR Collected and Abstracted Data Demographic Data: Patients Arabic name. Patients date of birth. Mother and Father year of birth (age automatically calculated on saving the data). Current residence, home town of the father, hospital/city birth place of the patient (an equivalent codes are displayed on saving entered data). Birth order of the patient. Gender and Nationality. Risk Factors: Gestational age (less than 37 weeks of gestation or less than 8 month and 20 days is considered premature birth). History of Diabetes Mellitus for the mother (gestational, adult onset or juvenile type). Prenatal diagnoses. Assisted conception. History of maternal rubella. Parental consanguinity. Family history of positive CHD. Age at diagnoses. CHDR staff uses set of Case Report Forms (CRFs) that are approved by the Office of Research Affairs (ORA). These forms are designed for the abstraction and collection of a set of information pertaining to CHDs.

9. CHDR Collected and Abstracted Data Diagnoses Data: Date of first presentation to the cardiovascular services areas (patients age at presentation is automatically calculated on saving the data) Height and weight (body mass index is automatically calculated on saving the data). Source of obtaining the diagnosis (operative reports, cardiac catheter reports, Echocardiography, ……..). Segmental Sequential anatomy. Description of the encountered CHDs. Description of the associated co morbid factors (extra cardiac congenital anomalies and health problems) that might be encountered in CHD patients. Intervention Data: Date of intervention. Type of intervention (heart surgery or cardiac catheter intervention). Descriptions of the documented repair(s) in each setting of repair (patients might undergo many repairs in same sitting of an intervention). Follow up Data: Date of the clinic visit, date of next follow up visit. Height and weight Patients status and Disease status according to the physicians notes in the visit sheet. Patients visits to the cardiology clinics is documented every follow up visit to the cardiology clinics. An equivalent EPC code automatically displays upon saving the entered description(s)

10. Some times patients undergo several episodes of intervention according to the management plan set by their treating physicians. Follow up visits are collected as per the patients follow up schedule ordered by their physician.

11. CHDR Structure and Organizational Hierarchy CHDR Multi Institutional Committee CHDR Committee for each collaborating hospital Registrar Clinical Interviewers Clinical Coders Statistician and an Epidemiologist Information Technology support team and Programmer Multi Institutional Registrar

12. The registry is a web based soft ware, with high levels of security: Soft ware cookies set-up. Unique ID and password for each user. Various users levels. Tracking system identifying data base access. Being an internet application all activities are real-time: Data entry, coding, auditing and data download. Patient transfer. Report generation. Chart generation. Real-time data search. Blog (communication form for interested community). CHDR Database Features and Applications CHDR is a centralized real time database. All data is hosted on a secured web-server. Observing and ensuring complete data segregation through: Each collaborating institute has its unique institutional code. Total masking of any identifiable information pertaining to the patients and the collaborating hospitals as; Patients name, medical record number, Telephone #, Hospitals name and registry codes CHDR staff access the CHDR data base of their respective registry and according to the assigned privileges on their respective institutes.

13. CHDR Multi Institutional Data As of April 2012

14. Multi Institutional Data as of April 2012

15. CHDR Database Numbers of Selected group of Congenital Heart Defects The encountered congenital heart defects are documented individually, irrelevant to the complexity of the CHD. Multi Institutional Data as of April 2012

16. Congenital Heart Defects – Gender Distribution and Variation Although gender distribution of CHDs shows slight difference between male and female, yet remarkable variation in the frequency of some individual CHDs is showing. Multi Institutional Data as of April 2012

17. Distribution Of Heart Surgery Episodes Based on Patients Age At Intervention The intervention plan for CHD patients differs and is tailored according to every patients status hence; recording heart surgery episodes is ongoing while patients are admitted to the treating hospitals. Multi Institutional Data as of April 2012

18. CHDR Database Counts of Selected group of Heart Surgeries More than one repair can be done in the same surgical setting Multi Institutional Data as of April 2012

19. CHDR Database Counts of Selected group Co morbid health problems Multi Institutional Data as of April 2012

20. Distribution of CHD Patients based in relation to Parental Consanguinity and Family History of CHD First-Cousin Mating and Congenital Heart Disease in Saudi Arabia Community Genetics; 1999; 2, 2-3; ProQuest Medical Library pg.69 Susan Becker; Zohair Al Halees Multi Institutional Data as of April 2012

21. Distribution Of CHD Patients - Age at Diagnoses Multi Institutional Data as of April 2012

22. Q: I am not a CHDR staff or a CHDR committee member. However I am interested to get an access to the CHDR database for research. A: We welcome all researchers however, there are set of data policies that requires to be followed by all. Following are some guidelines that can help you in getting your required data. 1.First complete the research proposal if not already completed. 2.Submit the proposal with required forms to your institutional IRB for approval. 3.Give a copy of approved project proposal with your request to access CHD data to the IRB of King Faisal Specialist Hospital. 4.Download the CHDR Data Request Form (available at the CHDR web site) 5.Fill in the form by clearly specifying the data of your interest. 6.Contact the CHDR registrar at your respective hospital who will facilitate the process of preparing the requested data. Frequently asked questions and their answers:

23. CHDR Quick and Useful Tools

24. Chart Generation Chart Generation is an easy and quick tool to browse through live statistics with graphical representation of data. Users can generate charts by selecting one of the parameters from the drop down list. Majority of registered patients are Saudi Majority of registered population is Saudi. This is mostly due to the admission eligibility rules.

25. Search Page Search form includes all variables from the database through which researchers may choose set of parameters that suites their criteria for data analysis

26. 1,538 patients had Total Repair of Tetralogy. Data is displayed in serial numbers with total masking of any identifiable data pertaining to the collaborating hospital or a patient. Data also displays the latest status of the patient.

27. Many research projects have been accomplished using CHDR data CHDR is well established on both regional and national levels. There is an open invitation for all to utilize the acquired and published information for a better service to the patients community. It is expected that CHD registry will assist KSA s health care providers in planning and allocation of required resources and will serve as a feedback tool for the invested resources and efforts.

28. Contact Us Shazia Naz Subhani, MSc Senior Technical Specialist Head- Registries Core Facility ssubhani@kfshrc.edu.sa Phone: 966-1-4424868 http://rc.kfshrc.edu.sa/rcf Nadia Dessouky, MD Saudi Congenital Heart Defects Registry ndessouky@kfshrc.edu.sa Phone: 966-1-4647272 ext:39209 http://rc.kfshrc.edu.sa/chd_Program/ Fax: 966-1-4424542 BESC Department King Faisal Specialist Hospital & Research Center Riyadh, Saudi Arabia