Congenital Heart Defects Registry - the Research Centre Page

Congenital Heart Defects Registry
Cumulative Report (1998 - 2011)

Congenital Heart Defects Registry
Cumulative Report (1998 – 2011)
Prepared by:
Head Office, Congenital Heart Defects Registry
King Faisal Specialist Hospital Research Center, Riyadh
Copies can be obtained from: ndessouky@kfshrc.edu.sa
Telephone: + 966 1 464 7272, EXT: 39209, Pager #: 4308
Fax: + 966 1 4424542
Mailing Address: KFSH&RC, BESC, MBC 03, P.O. Box 3354, Riyadh 11211, Saudi Arabia
E-copy can be downloaded from http://rc.kfshrc.edu.sa/rcf

Acknowledgement
We would like to take this opportunity to express our deep gratitude to administrators of the research center at
King Faisal Specialist Hospital, Riyadh for their great support to the Congenital Heart Defects Registry.
Many thanks go to the Multi‐Institutional Congenital Heart Defects registry committee members for their professional
advice and ongoing support.
Great appreciation goes to Congenital Heart Defects Registry staff for their dedication and patience in maintaining
a true team spirit and loyalty to make this registry a success. Special thanks to Technical Database Core Facility,
Biostatistics Research Unit, and Computer Core Facility. Last but not least we acknowledge secretary group for their
hard work and nurses for their cooperation.
The Congenital Heart Defects Registry Multi-Institutional Committee Members
Dr. Futwan Al Mohanna, PhD, King Faisal Specialist Hospital & Research Center, Riyadh
Dr. Zohair Al Halees, MD, King Faisal Specialist Hospital & Research Center, Riyadh
Ms. Shazia Naz Subhani, M.Sc, Head, Registries Core Facility, King Faisal Specialist Hospital & Research Center, Riyadh
Dr. Mohamed Shoukri, PhD, King Faisal Specialist Hospital & Research Center, Riyadh
Dr. Mansour Al Joufan, MD, King Faisal Specialist Hospital & Research Center, Riyadh
Dr. Ahmad Al Omrani, MD, King Faisal Specialist Hospital & Research Center, Riyadh
Dr. Abdulla Al Sehly, MD, King Faisal Specialist Hospital & Research Center, Riyadh
Dr. Mamdouh Al Ahmadi, MD King Faisal Specialist Hospital & Research Center, Riyadh
Dr. Riyadh Abu‐Sulaiman, MD, King Abdulaziz Cardiac Center, National Guard Affairs Hospital, Riyadh
Dr. Husam Al Tamimi, MD, Maternity Children Hospital Al‐Dammam
Dr. Amjad Al‐Quatli, MD, King Faisal Specialist Hospital & Research Center, Jeddah
Dr. Khadija Al Amodi, MD, Maternity Children Hospital Al‐Dammam
Dr. Nadia Dessouky, MD, Multi‐institutional Registrar, King Faisal Specialist Hospital & Research Center, Riyadh
Dr. Saad Al Yousef, MD, Prince Sultan Cardiac Center, Riyadh
Dr.Tarek S. Momenah, M, Prince Sultan Cardiac Center, Riyadh
Dr.Howaida Al Qethamy, MD, Prince Sultan Cardiac Center, Riyadh
Dr.Abdulrahman Al Redhyan, MD, Prince Sultan Cardiac Center, Riyadh
Dr.Yahya Al Faraidi, MD, Prince Sultan Cardiac Center, Riyadh
Dr.Yahya Al Mashem, MD, King Fahd Medical City, Riyadh

The Congenital Heart Defects Registry Multi-Institutional Registry Staff
Ms. Shazia Naz Subhani, M.Sc
Dr. Nadia Dessouky
Ms. Nawal Abu‐Khalid
Ms. Manal Al‐Marzougy
Ms. Nada Bawayan
Ms. Hala Al‐Assiry
Ms. AlJowhara Al‐Sahan
Dr. Heba Abu‐Zaid
Ms. Latifa Al‐Fayaz
Ms. Hailah Al‐ Angari
Dr. Salem Deraz
Ms. Nada kais
Dr. Amal Al‐Hakami, MD
Ms.Samar Saleh
Ms. Seham Al‐Shammas
Ms. Saba Al‐Ariji

Forward
The incidence and prevalence of Congenital Cardiac Defects in the Saudi population is not well‐documented. The
services for the care of children with CHD have improved tremendously in the last two decades and new centers
have opened in various locations in the country. Outcomes of such care again are not documented. Hence, the
initiation of the Congenital Heart Defects Registry (CHDR), which started at King Faisal Specialist Hospital and Research
Center, Riyadh, Saudi Arabia as a humble effort to answer some of the many questions. With hard work,
enthusiasm and dedication, the registry is now multi‐institutional and with grace of Allah Almighty, this effort will
evolve to a national registry capturing all the children with CHD in the kingdom.
We are proud to present the multi‐institutional report for the years 1998‐2011. This has been the result of continuous
effort of many individuals who work behind the scenes to make this report available, easy to read and follow,
utilizing latest information technology. With more Saudi centers joining, the number of registered patients is increasing
and the data presented is probably representative of what is going in CHD arena in Saudi Arabia. This
should invite all who are interested in Congenital Heart Defects to utilize this information to serve their patients
better. We are also hoping that such registry will help governmental health care providers in their future planning
and also giving them a feedback on their efforts. For example, from this report, it will be noted that there is more
fetal diagnosis and now patients are presenting and being diagnosed earlier in life.
This will lead to early therapy avoiding all the bad sequel of late diagnosis and therapy. Many challenges continue
to face CHDR but we all as one team are determined to keep this registry going and growing.
I would like to thank my colleagues and the staff of Biostatistics, Epidemiology, and Scientific Computing Department
of King Faisal Specialist Hospital Research Center, for their invaluable inputs and efforts in running this very
important project. Our appreciation goes to His Excellency, Dr. Qasim Al Qasabi, Chief Executive Officer (KFSH‐RC
Gen. Org.) and also to Dr. Sultan Al‐Sedairy, Executive Director of the Research Centre (KFSHRC Gen. Org.‐Riyadh),
for their valuable support and advice.
Zohair Yousef Al Halees, MD
Advisor for Cardiovascular Disease to the Chief Executive Director
Chairman Congenital Heart Defect Registry, KFSH&RC

Table of Contents
Introduction ..................................................................................................................................................... 7
Disclaimer ........................................................................................................................................................ 2
Report Overview .............................................................................................................................................. 2
PART ONE: OVERVIEW CONGENITAL HEART DEFECT REGISTRY ............................................................................................. 2
What is Congenital Heart Defects Registry ....................................................................................................... 2
Congenital Heart Defects Registry Objectives .................................................................................................... 2
Congenital Heart Defects Registry Inclusion Criteria .......................................................................................... 2
Congenital Heart Defects Registry Coding System ............................................................................................. 2
Collaborating Hospitals ...................................................................................................................................... 2
Congenital Heart Defects Registry (CHDR) Database Features and Applications ............................................... 3
Congenital Heart Defects Registry ...................................................................................................................... 4
Congenital Heart Defects Registry Structure and Organizational Hierarchy ...................................................... 4
Congenital Heart Defects Registry Work Activity ............................................................................................... 5
Main Activities .................................................................................................................................................... 5
Data Processing .................................................................................................................................................. 5
Data Collection ................................................................................................................................................... 5
Part Two: Executive Summary .......................................................................................................................... 7
SECTION 1: MULTI‐INSTITUTIONAL PROGRESS OF THE CONGENITAL HEART DEFECTS REGISTRY ................................................... 7
SECTION 2: OVERVIEW OF DEMOGRAPHIC DATA OF THE CONGENITAL HEART DEFECTS REGISTRY (MULTI‐INSTITUTIONAL)............ 10
SECTION 3: RISK FACTORS DATA FOR CHD PATIENTS ON THE MULTI‐INSTITUTIONAL LEVEL OF COLLABORATION ......................... 12
Part Three: Reporting Multi‐Institutional Congenital Heart Defects Registry Data ........................................... 17
SECTION 1: MULTI‐INSTITUTIONAL CONGENITAL HEART DEFECT LESIONS ............................................................................. 17
SECTION 2: ASSOCIATED EXTRA‐CARDIAC CONGENITAL ANOMALIES AND HEALTH PROBLEMS (MULTI‐INSTITUTIONAL DATA) ......... 19
SECTION 3: REPORTING HEART SURGERY AND CARDIAC CATHETER TREATMENT FOR MULTI‐INSTITUTIONAL CONGENITAL HEART
DEFECTS REGISTRY ..................................................................................................................................................... 20
Congenital Heart Defects Registry Challenges ................................................................................................. 22
Conclusion...................................................................................................................................................... 22
References ..................................................................................................................................................... 23

Tables and Figures
FIGURE 2.1.1: MULTI‐INSTITUTIONAL PROGRESS OF THE CONGENITAL HEART DEFECTS REGISTRY .................................................... 7
FIGURE 2.1.2: SNAP‐SHOT OF THE CONGENITAL HEART DEFECTS REGISTRY DATA BASE ................................................................... 8
FIGURE2. 1.3: DISTRIBUTION OF REGISTERED PATIENTS FOR THE REPORTING HOSPITALS ................................................................. 9
FIGURE 2.2.1: DISTRIBUTIONS OF CHD PATIENTS BY GENDER (MULTI‐INSTITUTIONAL)................................................................ 10
FIGURE 2.2.2: DISTRIBUTION OF CHD PATIENTS BY NATIONALITIES (MULTI‐INSTITUTIONAL) ......................................................... 10
FIGURE 2.2.3: DISTRIBUTION OF CHD PATIENTS POPULATION ACCORDING TO AREA OF RESIDENCE (MULTI‐INSTITUTIONAL)............... 11
TABLE 2.3.1: DISTRIBUTION OF CHD PATIENTS BY AGE AT DIAGNOSIS ....................................................................................... 12
FIGURE 2.3.1: DISTRIBUTION OF CHD PATIENTS BY AGE AT DIAGNOSIS ..................................................................................... 12
FIGURE 2.3.2: LIFE STATUS OF PRENATALLY DIAGNOSED CHD PATIENTS .................................................................................... 13
TABLE 2.3.2: DISTRIBUTION OF CHD PATIENTS BY AGE AT PRESENTATION .................................................................................. 13
TABLE 2.3.3: DISTRIBUTION OF CHD PATIENTS ACCORDING TO GESTATIONAL AGE ...................................................................... 14
TABLE 2.3.4: DISTRIBUTION OF CHD PATIENTS ACCORDING TO HISTORY OF MATERNAL DIABETES MELLITUS .................................... 14
TABLE 2.3.5: DISTRIBUTION OF CHD PATIENTS ACCORDING TO ASSISTED CONCEPTION ................................................................ 14
TABLE 2.3.6: DISTRIBUTION OF CHD PATIENTS ACCORDING TO PARENTAL CONSANGUINITY .......................................................... 14
FIGURE 2.3.3: DISTRIBUTION OF CHD PATIENTS WITH POSITIVE FAMILY HISTORY FOR CHD BASED ON THE PARENTAL CONSANGUINITY
STATUS ................................................................................................................................................................... 16
TABLE3.1.1: DISTRIBUTION OF CONGENITAL HEART LESIONS BY GENDER (MULTI‐INSTITUTIONAL).................................................. 17
FIGURE 3.1.1A: INDIVIDUAL VARIATION OF SELECTED GROUP OF CHD LESIONS BASED ON GENDER (MULTI‐INSTITUTIONAL) ............... 18
TABLE 3.3.2: SELECTED GROUP OF CARDIAC CATHETER INTERVENTIONS (MULTI‐INSTITUTIONAL) ................................................... 22

Congenital Heart Defects Registry Cumulative Report (1998-2011)
Introduction
Congenital Heart Defects (CHDs) is a worldwide health problem that has its significant impact on the patients’
survival and quality of life. Even when successfully repaired at its initial stages, patients with CHDs need long term
follow‐up and the possibility of future interventions. Social, psychological, economical are aspects that burden
the patients and their families nonetheless affecting the overall nation productivity and progress. Databases and
registries studying birth defects especially congenital heart defects exist since quite long time, for example:
The International Nomenclature of Pediatric and Congenital Heart Diseases [1] [2]
The Glasgow Register of Congenital Anomalies [3]
The Emilia‐Romagna Registry (Italy) [4]
The German network for Congenital Heart Defects [5]
Such registers function as data warehouse, studying the incidence, prevalence, possible underlying factors, and
common extra cardiac medical problems associated with the CHDs. Other databases aim at the analysis of major
outcomes and process‐of‐care measures that affect congenital heart surgery. Such databases provide practiceperformance
assessment data for research projects. Society of Thoracic Surgeons (STS) and the European Association
for Cardio‐Thoracic Surgery (EACTS) are examples of such registers. During the past decades, health care of
CHDs patients have improved substantially, leading to the increment of grown‐up congenital heart (GUCH) patients.
Worldwide recognition in this regard resulted in the existence of many societies. One of those is the International
Society for Adult Congenital Cardiac Disease [6] which recommended the importance of moving beyond
mortality, and encompass longer‐term follow‐up, including cardiac and non‐cardiac morbidities especially those
influencing quality of life. Adults with CHDs most often require specialized medical or surgical care in a tertiary
center. However this population also needs local follow‐up; therefore, general practitioners and other specialists
have to face the complexity of their disease [7]. King Faisal Specialist Hospital & Research Centre (KFSH&RC Riyadh)
is an outstanding tertiary care center and is one of the leading hospitals in caring for the CHDs patients in
the Middle East. It realizes the importance to have a database registering CHDs patients. Congenital Heart Defects
Registry (CHDR) is prospective since 1998. The registry started on a hospital level as research collaboration
between King Faisal Heart Institute and Biostatistics Epidemiology and Scientific Computing department at
KFSH&RC, Riyadh. Being successful on hospital and regional levels, the registry is now extending its collaborative
efforts on a national level. The goal is to have key hospitals from major cities of the Kingdom on board for this
collaborative program.
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
Disclaimer
Data published in this report is a representation of information from the collaborating hospitals on board of
the Congenital Heart Defects Registry collaborative research. The reported information should not be considered
as a complete and/or necessarily accurate representation of the actual disease distribution nationally.
The data is meant to be available for sharing knowledge. Using the reported data for any kind of publication
is the responsibility of the publisher.
The collaborative efforts from various hospitals were not simultaneous in terms of patient registration from
their respective hospitals over the period of thirteen years (1998 – 2011). This leads to having not an exact
reflection of patient’s flow in the overall numbers reported in the CHD database.
Report Overview
Part One: Overview Congenital Heart Defect Registry
What is Congenital Heart Defects Registry
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.
Congenital Heart Defects Registry Objectives
• To provide leadership in establishing and maintaining comprehensive congenital heart defects registration
in collaboration with other national and international organizations.
• To promote standards to effectively administer congenital heart defects information system in the region.
• To support scientific and clinical research influencing prevention, intervention and overall management
care of congenital heart disease.
Congenital Heart Defects Registry Inclusion Criteria
All patients diagnosed to have CHDs are eligible for registration, regardless of their age, gender or nationality.
Congenital Heart Defects Registry Coding System
The European Pediatric Cardiology coding list (EPCC) is the coding system for coding the abstracted data pertaining
to the congenital Heart Defects, heart surgeries and cardiac catheter treatment.
Collaborating Hospitals
‣ Heart Center at King Faisal Specialist Hospital and Research Center, Riyadh. Since 1998, status is
active.
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
‣ Prince Sultan Cardiac Center, Riyadh. Since 2003, status active
‣ Maternity Children Hospital, Al‐Dammam. Since 2009, status active
‣ Prince Salman Heart Center, King Fahd Medical City , Riyadh. Since 2007, status on hold
‣ Cardio Vascular Department at King Faisal Specialist Hospital Research Center, Jeddah. Since
2010, status active
‣ King Abdul‐Aziz Cardiac Center at the National Guard Health Affairs, Riyadh. Since 2011, status active
Congenital Heart Defects Registry (CHDR) Database Features and Applications
CHDR is an internet real time database. All data is hosted on a centralized secured web‐server. The registry is
web‐based using the latest state‐of‐art technology, designed for the collection, processing, management, and
analysis of data on CHD patients.
Being an internet application all the following activities are real‐time:
Data entry, coding, auditing and data download
Patient transfer application within CHD registries preventing double patient registration hence
avoid patient duplication
Report generation
Chart generation
Real‐time data search
Automated built‐in validation checks in the software preventing invalid data entry
CHDR is a web based soft ware, with high levels of security:
‣ Software cookies set‐up
‣ Unique identification user name and password for each user
‣ Various users’ levels
‣ Tracking system identifying data base access (logging in location, time in and time out)
CHDR ensures 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;
• Patient’s name, medical record number, Telephone #,
• Hospital’s name and registry codes
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
CHDR staff access the CHDR data base of their respective registry and according to the assigned
privileges on their respective institutes.
SQL 2005 database is a back end and internet‐enable design as a front end. The Web Server used for the design
of the Congenital Heart Defects Registry is the Microsoft Internet Information Server (IIS) with a DOT NET framework.
Forms and software functionalities are designed using Visual Studio.NET application. The database including
all the tables, indexes, rules, stored procedures, views and triggers is upgraded and maintained with Microsoft
SQL Server 2005.
Congenital Heart Defects Registry
• CHDR Offices are located within the premises of the collaborating hospitals
• CHDR staff is assigned by the collaborative hospitals
‣ A registrar
‣ Clinical interviewer(s)
‣ Disease coder(s)
• CHDR staff runs CHDR chores through various cardiovascular clinics and wards within the premises of
their respective hospitals.
Congenital Heart Defects Registry Structure and Organizational Hierarchy
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
Congenital Heart Defects Registry Work Activity
The 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 information pertaining to Congenital Heart Defects
patients.
Main Activities
‣ Patients’ registration in the cardiovascular clinics and wards.
‣ Ongoing process to complete and update demographic data in the following visits.
‣ 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.
‣ Ongoing process to document new intervention episodes (heart surgery, cardiac catheter) while
patients’ admission to the cardiovascular wards.
‣ Assigning a unique family number for CHDs patients having positive family history for CHDs.
Family number is assigned if one or more of the following members has CHDs:
Mother, Father, off spring, Sibling(s)
Data Processing
Quarterly audit is carried out on a randomized sample of entered data.
Data validation; assuring data completeness and accuracy.
Data Collection
Demographic Data:
• Patient’s Arabic name.
• Patient’s 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.
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
• Assisted conception.
• History of maternal rubella.
• Parental consanguinity.
• Family history of positive CHD.
• Age at diagnoses.
Demographic and risk factors data are collected from the patients’ medical chart and through a face to face interview
after obtaining the patients’ pedigree agreement to release information.
Diagnoses Data:
Intervention Data:
• Date of first presentation to the cardiovascular services areas (patient’s 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.
• 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).
An equivalent European Pediatric Cardiology code will automatically be displayed upon saving the entered description(s)
Follow up Data:
The following set of data is collected during the patients’ visits to the cardiology clinics.
• Date of the clinic visit, date of next follow up visit.
• Height and weight.
• Disease status according to the physician’s notes in the visit sheet.
Telephone follow up helps in updating the life status for patients who are discharged from the cardiology follow
up visits or recorded as no show for more than 3 years from the last recorded visit.
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
Part Two: Executive Summary
Section 1: Multi‐Institutional Progress of the Congenital Heart Defects
Registry
The executive summary is a multi‐institutional update for the reported data through the collaborative hospitals.
The reported numbers are since the inception of the CHDR in January 1, 1998 till December 31, 2011. Total numbers
of the following data are displayed cumulatively
‣ Total number of registered patients accounts for 22,141
‣ Total number of Grown‐Up congenital Heart (GUCH) ( patients at age of 16 years and above) accounts
for 3,637 patients with active follow up visits
‣ Total number of valid records accounts 21,421
‣ Total number of Cumulative Follow up visits accounts for 55,894
‣ Total number of Cumulative Heart surgeries episodes accounts for 16,680
‣ Total number of Cumulative Cardiac catheter interventions accounts for 5,582
FIGURE 2.1.1: MULTI‐INSTITUTIONAL PROGRESS OF THE CONGENITAL HEART DEFECTS REGISTRY
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
FIGURE 2.1.2: SNAP‐SHOT OF THE CONGENITAL HEART DEFECTS REGISTRY DATA BASE
Patients’ registration and records’ validation is onetime process. On the other hand; follow‐up visits, heart surgery
and cardiac catheter episodes are an ongoing process of documentation as long as the patient is scheduled
for future follow up visits and while patients’ admission according to the management plan set by the treating
physicians (figure 2.1.2).
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
FIGURE2. 1.3: DISTRIBUTION OF REGISTERED PATIENTS FOR THE REPORTING HOSPITALS
Figure 2.1.3 demonstrates the distribution of the registered patients through the collaborative hospitals with
total of (22,141) since the inception of the registry in the year 1998 till December 31, 2011. There are factors that
affect the pattern of this distribution like;
Frequency of referrals to the collaborative hospitals: In the year 1998 total of 2,299 CHDs patients
were registered at CHDR at King Faisal Specialist Hospital Research Center, Riyadh (KFSH&RC, Riyadh).
Work force and Administrative support: Securing qualified work force and administrative support are
crucial factors to maintain smooth running CHDR within the premises of the collaborative hospital
which in return affects the overall reported data.
Number of reporting hospitals: Current hospitals on board of the CHDR collaborative research work
joined over thirteen years (1998‐2011) showing considerable increment in the total number of registered
patients.
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
Section 2: Overview of Demographic Data of the Congenital Heart Defects
Registry (Multi‐Institutional)
Data shown in this section are coming from 21,421 records accounting for the total number of valid records since
the inception of the CHDR till December 31, 2012.
FIGURE 2.2.1: DISTRIBUTIONS OF CHD PATIENTS BY GENDER (MULTI‐INSTITUTIONAL)
FIGURE 2.2.2: DISTRIBUTION OF CHD PATIENTS BY NATIONALITIES (MULTI‐INSTITUTIONAL)
Figure 2.2.2 shows dominance of Saudi (93%) in comparison to non‐Saudi patients. This is mostly related to referral
and admission regulations observed at the reporting hospitals.
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
FIGURE 2.2.3: DISTRIBUTION OF CHD PATIENTS POPULATION ACCORDING TO AREA OF RESIDENCE (MULTI‐INSTITUTIONAL)
Figure 2.2.3 shows the distribution of CHD patients according to their residence regions as recorded at the time
of patient registration. The shown percentages denote the referral frequencies from all over the Kingdom. Riyadh
Region and Eastern Province are showing as the highest rates for CHD patients’ referrals. Information is
not available for 1.5% of residence area in the Kingdom, which sometimes are due to the uncertainty of the
provided information at the time of patients’ registration.
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
Section 3: Risk Factors Data for CHD Patients on the Multi‐Institutional level
of Collaboration
Data related to the risk factors is obtained from patients’ family at the time of patient registration. However,
some times the required information cannot be documented due to the uncertainty of obtained information.
Also, some patients are registered through the hospital death log where charts are deficient of required information.
Since the completion of data at all levels is a standard requirement for the registry, therefore CHDR staff
ensures to complete all missing risk factors during the patient’s future clinical visits and/or hospital admissions. In
the same context, the telephone follow up project is on going to complete the missing information. Total number
and percentages are shown along with gender variation for each variable in the following tables.
TABLE 2.3.1: DISTRIBUTION OF CHD PATIENTS BY AGE AT DIAGNOSIS
Born BEFORE 2001 Born AFTER 2001
Age at Diagnosis
Male Female Male Female
Total
N % N % N % N % N %
Prenatal 56 13.4 48 11.5 159 37.9 156 37.2 419 2
At Birth to 1 year 3,515 22.3 2,971 18.9 4,838 30.7 4,427 28.1 15,751 73.5
Greater than 1 year 1,873 35.7 2,123 40.4 553 10.5 702 13.4 5,251 24.5
Total 5,444 25.4 5,142 24 5,550 25.9 5,285 24.7 21,421 100
Table 2.3.1 and figure 2.3.1 shows the distribution of the CHDs patients according to their age when diagnosed to
have CHDs. Patients were grouped based on their year of birth.
FIGURE 2.3.1: DISTRIBUTION OF CHD PATIENTS BY AGE AT DIAGNOSIS
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
FIGURE 2.3.2: LIFE STATUS OF PRENATALLY DIAGNOSED CHD PATIENTS
Figure 2.3.2 shows the life status percentage distribution for the 419 patients who were prenatally diagnosed
with congenital heart defects.
TABLE 2.3.2: DISTRIBUTION OF CHD PATIENTS BY AGE AT PRESENTATION
Age at Presentation
Male Female Total
N % N % N %
At Birth 504 51.7 471 48.3 975 4.6
< 1 year 6166 53.6 5333 46.4 11499 53.7
1‐12 years 3773 48.3 4035 51.7 7808 36.5
> 12years 551 48.4 588 51.6 1139 5.3
Total 10994 51.3 10427 48.7 21421 100
CHD patients are distributed according to the age group classification when presented for the first time to cardiovascular
departments at reporting hospitals. More than 50% of the patients are presented during their first year
of life.
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
TABLE 2.3.3: DISTRIBUTION OF CHD PATIENTS ACCORDING TO GESTATIONAL AGE
Gestational Age
Male Female Total
N % N % N %
Premature 932 51.2 887 48.8 1,819 8.5
Full Term 8,856 51.4 8,363 48.6 17,219 80.4
Not Documented 1,206 50.6 1,177 49.4 2,383 11.1
Total 10,994 51.3 10,427 48.7 21,421 100
As per internal policies and procedures (IPPs) followed by the CHD registry, patients are considered to be prematurely
delivered when born at age of 37 weeks of gestation i.e. 8 month and 20 days.
TABLE 2.3.4: DISTRIBUTION OF CHD PATIENTS ACCORDING TO HISTORY OF MATERNAL DIABETES MELLITUS
Diabetes Mellitus
Male Female Total
N % N % N %
Diabetic Mothers 870 52.4 790 47.6 1,660 7.7
Non Diabetic Mothers 8,634 51.4 8,180 48.6 16,814 78.5
Not Documented 1,490 50.6 1,457 49.4 2,947 13.8
Total 10,994 51.3 10,427 48.7 21,421 100
Type of reported maternal diabetes includes gestational diabetes, juvenile diabetes and the adult onset type.
TABLE 2.3.5: DISTRIBUTION OF CHD PATIENTS ACCORDING TO ASSISTED CONCEPTION
Male Female Total
Assisted Conception
N % N % N %
Assisted Conception 277 52.4 252 47.6 529 2.5
Normal Conception 9,243 51.4 8,739 48.6 17,982 83.9
Not Documented 1,474 50.7 1,436 49.3 2,910 13.6
Total 10,994 51.3 10,427 48.7 21,421 100
Conception methods are documented according to the interview data or the attached reports in the patients’
files. Methods used must be medical for example; medications or Invetro‐Firtilization.
TABLE 2.3.6: DISTRIBUTION OF CHD PATIENTS ACCORDING TO PARENTAL CONSANGUINITY
Parental Consanguinity
Male Female Total
N % N % N %
Consanguineous 6,386 51.5 6,007 48.5 12,393 57.9
Non Consanguineous 4,248 50.8 4,109 49.2 8,357 39
Not Documented 360 53.7 311 46.3 671 3.1
Total 10,994 51.3 10,427 48.7 21,421 100
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Congenital Heart Defects Registry Cumulative Report (1998-2011)
TABLE 2.3.7: DISTRIBUTION OF CHD PATIENTS ACCORDING TO FAMILY HISTORY FOR CHD
Family History
Male Female Total
N % N % N %
Yes 982 50.3 972 49.7 1,954 9.1
No 9,483 51.3 9,000 48.7 18,483 86.3
Not Documented 529 53.8 455 46.2 984 4.6
Total 10,994 51.3 10,427 48.7 21,421 100
Positive family history for CHD is confirmed when one or more of the family members e.g. father, mother, sibling(s),
or offspring(s) are affected as well. It is worth mentioning that affected members of the same families
who are registered at the same treating hospital are assigned a family number which facilitates studying different
characteristics of families with positive history for CHD.
TABLE 2.3.8: DISTRIBUTION OF CHD PATIENTS BASED ON FAMILY HISTORY AND PARENTAL CONSANGUINITY STATUS
Total
Parental Consanguinity & Family History
N %
Consanguineous Parents with Positive Family History 1,364 6.4
Consanguineous Parents with Negative Family History 10,850 50.7
Non‐Consanguineous Parents with Positive Family History 573 2.7
Non‐Consanguineous Parents with Negative Family History 7,529 35.2
Data Not Documented 1,105 5
Total 21,421 100
15

Congenital Heart Defects Registry Cumulative Report (1998-2011)
FIGURE 2.3.3: DISTRIBUTION OF CHD PATIENTS WITH POSITIVE FAMILY HISTORY FOR CHD BASED ON THE PARENTAL CONSANGUINITY STATUS
Table 2.3.8 and Figure 2.3.3 demonstrate that consanguinity may increase the risk of congenital heart defects in
patients with positive family history of Congenital Heart Defects.
TABLE 2.3.9: DISTRIBUTION OF CHD PATIENTS BASED ON MATERNAL AGE
Maternal Age (in years)
Male Female Total
N % N % N %
< 20 457 51.3 433 48.7 890 4.2
20‐29 3,876 51 3,719 49 7,595 35.5
30‐39 3,130 51 3,012 49 6,142 28.7
40 + 779 48.6 825 51.4 1,604 7.5
Not Documented 2,752 53 2,438 47 5,190 24.2
Total 10,994 51.3 10,427 48.7 21,421 100
16

Congenital Heart Defects Registry Cumulative Report (1998-2011)
Part Three: Reporting Multi‐Institutional Congenital Heart Defects
Registry Data
Reported data is coming from analysis of 21,421 records accounting for valid records till December 31, 2012.
Section 1: Multi‐Institutional Congenital Heart Defect Lesions
Total of 52,092 congenital heart defect lesions are encountered in the database of the CHDR on the multiinstitutional
level. Congenital heart lesions are abstracted and coded on individual descriptive basis regardless of
the complexity of the congenital heart disease. European Pediatric Cardiology Coding (EPCC) lists are used for
coding the abstracted diagnoses data. Table 3.1 displays a sample of individual congenital heart defect Lesions.
Congenital heart defects lesions might be either isolated or in combination with other congenital heart defect
lesions. Coarctation of Aorta can be isolated or in combination with other lesions as Ventricular Septal Defect(s).
TABLE3.1.1: DISTRIBUTION OF CONGENITAL HEART LESIONS BY GENDER (MULTI‐INSTITUTIONAL)
Congenital Heart Lesions Male Female Total
N % N % N
Ventricular Septal Defect (all types) 3,795 51 3,643 49 7,438
Patent Ductus Arteriosus 3,542 48.1 3,815 51.9 7,357
Atrial Septal Defect 2,510 48 2,724 52 5,234
Pulmonary Valve Stenosis 1,773 52.8 1,585 47.2 3,358
Tetralogy of Fallot 1,136 57.9 825 42.1 1,961
Coarctation of Aorta 956 60.2 633 39.8 1,589
Transposition of Great Arteries (all types) TGA 991 64 557 36 1,548
Aortic Valve Stenosis with and without BAV 821 68.5 378 31.5 1,199
Pulmonary Valve Atresia PA 543 55.2 441 44.8 984
Corrected Transposition of Great Arteries L‐TGA 218 63.4 126 36.6 344
Congenital Complete Heart Block 165 53.9 141 46.1 306
Supraventricular Tachycardia 120 45.8 142 54.2 262
Hypoplastic Left Heart Syndrome HLHS 126 64.3 70 35.7 196
Wolf Parkinson White Syndrome WPW 53 48.2 57 51.8 110
Hypertrophic Cardiomyopathy (all types) HCM 58 54.2 49 45.8 107
Long QT syndrome 40 63.5 23 36.5 63
Total number of listed CHDs 16,847 52.6 15,209 47.4 32,056
Other CHD Lesions 10,911 54.5 9,125 45.5 20,036
Grand Total CHDs 27,758 53.3 24,334 46.7 52,092
17

Congenital Heart Defects Registry Cumulative Report (1998-2011)
FIGURE 3.1.1A: INDIVIDUAL VARIATION OF SELECTED GROUP OF CHD LESIONS BASED ON GENDER (MULTI‐INSTITUTIONAL)
Figure 3.1.1a shows an individual variation in the frequency of some CHD lesions based on gender in comparison
to congenital heart defects distribution between male and female (Figure 3.1.1b).
FIGURE 3.1.1B: DISTRIBUTIONS OF CHD PATIENTS BY GENDER (MULTI‐INSTITUTIONAL)
18

Congenital Heart Defects Registry Cumulative Report (1998-2011)
Section 2: Associated Extra‐Cardiac Congenital Anomalies and Health
Problems (Multi‐Institutional data)
Associated congenital anomalies and health problems are co‐morbid factors that affect short and long term outcome
of the congenital heart defects. Not only this but more, co‐morbid factors might affect the overall management
plan for congenital heart defects patients. (6,242) accounts for total number of encountered associated
extra‐cardiac congenital anomalies and health problems on the multi‐institutional level. Table 3.2.1 displays data
in descending order according to their frequency. Extra cardiac anomalies and health problems might be isolated
or in association with others.
TABLE 3.2.1: ASSOCIATED EXTRA‐CARDIAC CONGENITAL ANOMALIES AND HEALTH PROBLEMS (CHDR MULTI INSTITUTIONAL DATA)
Associated Congenital Anomalies and Health Problems
Male
Female
N % N %
Total
Down's Syndrome 859 46.2 999 53.8 1,858
Failure to thrive 436 42.7 586 57.3 1,022
Delayed Milestones 292 46.8 332 53.2 624
Cleft Palate with Cleft Lip, Unspecified 73 52.5 66 47.5 139
Unspecified Mental Retardation 43 49.4 44 50.6 87
Non Specific Respiratory Distress Syndrome 49 56.3 38 43.7 87
William's Syndrome 39 50.6 38 49.4 77
Hydrocephalus 26 43.3 34 56.7 60
Tracheosophageal Fistula (Esophageal Atresia and Stenosis) 29 52.7 26 47.3 55
Absent Spleen (Asplenia) 32 61.5 20 38.5 52
G6PD Defeciency Anemia 30 65.2 16 34.8 46
Noonan's Syndrome 30 69.8 13 30.2 43
Marfan's Syndrome 22 53.7 19 46.3 41
Imperforate Anus 17 44.7 21 55.3 38
Digeorge's Syndrome 19 57.6 14 42.4 33
Musculoskeletal Deformity, Scoliosis 7 22.6 24 77.4 31
Congenital Rubella 8 32 17 68 25
Total number 2,079 47.4 2,307 52.6 4,386
Other Associated Congenital Anomalies and Health Problems 1,021 55 835 45 1,856
Grand Total 3,100 49.7 3,142 50.3 6,242
19

Congenital Heart Defects Registry Cumulative Report (1998-2011)
Section 3: Reporting Heart Surgery and Cardiac Catheter treatment for
Multi‐Institutional Congenital Heart Defects Registry
Management plan to repair congenital heart defects widely ranges. Hence CHDs patients might need more than
one episode of heart surgery and/or cardiac catheter intervention. Congenital Heart Defects Registry staff regularly
document new interventions while CHDs patients’ admission to the cardiovascular wards. 22,262 accounts
for the cumulative number of intervention episodes recorded in the congenital heart defects registry database,
16,680 heart surgery episodes and 5,582 cardiac catheter episodes. European Pediatric Cardiology Coding (EPCC)
lists are used for coding the abstracted descriptions.
FIGURE 3.3.1 DISTRIBUTION OF HEART SURGERY AND CARDIAC CATHETER INTERVENTION EPISODES (MULTI‐INSTITUTIONAL)
20

Congenital Heart Defects Registry Cumulative Report (1998-2011)
TABLE 3.3.1: SELECTED GROUP OF HEART SURGERIES (MULTI‐INSTITUTIONAL)
Interventions
Total
Ligation & division of patent arterial duct (PDA) 4,607
Ventricular Septal Defects patch repair 2,349
Tetralogy of Fallot repair 1,535
Modified Blalock interposition shunt 1,485
Ventricular Septal Defects direct suture repair 1,434
Coarctation/hypoplasia of aorta repair 1,016
Fontan type procedure 763
Atrial septal defect (ASD) secundum patch repair 729
Arterial switch procedure 707
Pacemaker system placement: permanent 680
Common arterial trunk repair 189
Norwood type procedure 169
Ross procedure (aortic valve or root replacement with pulmonary autograft) 136
Rastelli procedure 112
Total Heart Surgeries 15,911
Other Heart Surgeries 18,406
Grand Total of CHDR data base Heart Surgeries 34,317
Table 3.3.1 displays the frequency of selected heart surgeries. 34,317 heart surgeries accounts for the total number
of individual heart surgeries recorded in the CHDR database. Listed heart surgeries might be either isolated or
in combination with other heart surgeries as many repairs might be carried out in same heart surgery episode.
For instance; Ventricular Septal Defects patch closure might be isolated or in combination with ligation of the
Patent Arterial Duct in the same sitting. Interventions are listed in a descending order according to their frequency
CHDR database. Documentation of new heart surgery episodes is ongoing while patients’ admission
to the wards for completing their repair plans.
21

Congenital Heart Defects Registry Cumulative Report (1998-2011)
TABLE 3.3.2: SELECTED GROUP OF CARDIAC CATHETER INTERVENTIONS (MULTI‐INSTITUTIONAL)
Cardiac Catheter Intervention
Total
Patent Ductus Arteriosus occlusion 1,069
Pulmonary Valve Balloon dilatation 919
Atrial septal defect (ASD) secundum device closure 537
Aortic Coarctation/hypoplasia Balloon dilatation 364
Balloon atrial septostomy 286
Aortic Valve Balloon dilatation 226
Major systemic‐pulmonary collateral artery (MAPCA) occlusion 132
Ventricular Septal Defect device closure 117
Radiofrequency Ablation for arrhythmia 86
Radiofrequency Pulmonary Valvotomy 44
Stent placement at site of thoracic aortic coarctation 43
pacemaker system placement 31
Pulmonary valvar insertion 28
Total Cardiac Catheter 3,882
Other Cardiac Catheter 1,324
Grand Total Cardiac Catheter 5,206
Table 3.3.2 demonstrates the frequency of selected group of interventional cardiac catheter. 5,206 is the total
number of cardiac catheter interventions that are recorded in the CHDR on the multi‐institutional level. The reported
numbers are listed in descending order according to their frequency. Documentation of new cardiac catheter
interventions are ongoing while patients’ admission to the wards.
Congenital Heart Defects Registry Challenges
The Congenital Heart Defects Registry faced and is still facing many challenges. Funds deficiency results in a state
of instability of securing smooth running of the CHD registry in the collaborating hospitals. It is the most prominent
challenge. Funds deficiency leads to the inability to hire and retain a qualified staff to look after the registry.
Hence; securing funds is a mandate in order to hire well qualified staff to run the registry along with securing the
equipments (computers, Laptops) and spaces needed.
Conclusion
Congenital Heart Defects (CHDs) is a worldwide health problem that has its significant impact on the patients’
survival and quality of life. Social, psychological, economical are aspects that burden the patients and their families
nonetheless affecting the overall nation productivity and progress. The Congenital Heart Defects Registry is
successful surveillance tool. Expanding the collaborative efforts along with the support of the administration and
the administrators in different hospitals will take this important collaboration to a true national level while finding
the incidence of this health problem in the Kingdom of Saudi Arabia. We are confident that this collaboration
will be of great help to the administrators while improving the level of the medical care provided to this sector of
population.
22

References
Congenital Heart Defects Registry Cumulative Report (1998-2011)
1. Tchervenkov CI, Jacobs JP, Bernier PL, Stellin G, Kurosawa H, Mavroudis C,Jonas RA, Cicek SM, Al‐Halees Z,
Elliott MJ, Jatene MB, Kinsley RH, Kreutzer C,Leon‐Wyss J, Liu J, Maruszewski B, Nunn GR, Ramirez‐
Marroquin S, Sandoval N, SanoS, Sarris GE, Sharma R, Shoeb A, Spray TL, Ungerleider RM, Yangni‐Angate
H,Ziemer G. The improvement of care for paediatric and congenital cardiac diseaseacross the World: a
challenge for the World Society for Pediatric and Congenital. Heart Surgery. Cardiol Young. 2008 Dec;18
Suppl 2:63‐9.
2. Tchervenkov CI, Stellin G, Kurosawa H, Jacobs JP, Mavroudis C, Bernier PL, Maruszewski B, Kreutzer C,
Cicek S, Kinsley RH, Nunn GR, Jonas RA. The World Society for Pediatric and Congenital Heart Surgery: its
mission and history Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009:3‐7.
3. www.heartstats.org
4. Calzolari E, Garani G, Cocchi G, Magnani C, Rivieri F, Neville A, Astolfi G,Baroncini A, Garavelli L, Gualandi F,
Scorrano M, Bosi G; IMER Working Group.Congenital heart defects: 15 years of experience of the Emilia‐
Romagna Registry (Italy). Eur J Epidemiol. 2003; 18(8):773‐80.
5. www.kompetenznetz‐ahf.de
6. Jacobs JP, Wernovsky G, and Elliott MJ.Analysis of outcomes for congenital cardiac disease: can we do better
Cardiol Young. 2007 Sep; 17 Suppl 2: 145‐58.
7. Bédard E, Shore DF, Gatzoulis MA. Adult congenital heart disease: a 2008 overview. Br Med Bull. 2008;
85:151‐80. Review
8. Bernier PL,Otal N,Tchervenkov CI,Jacobs JP,Stellin G,Kurosawa H,Mavroudis C, Cicek S, Al‐Halees Z, Elliott
M, Jatene M,Jonas RA, Kinsley R,Kreutzer C, Leon‐Wyss J,Liu J, Maruszewski B,Nunn G, Ramirez‐Marroquin
S,Sandoval N, Sano S, Sarris G, Sharma R, Spray T, Ungerleider R, Yangni‐Angate H,Ziemer G. An invitation
to the medical students of the world to join the global coalition to improve care for children and adults
with congenital heart disease across the world. Mcgill J Med. 2008 Jul; 11(2):185‐90.
23

Appendix I
Case Report Forms

CONFIDENTIAL INFORMATION. TO BE USED ONLY FOR THE CHD REGISTRATION.
CONGENITAL HEART DEFECTS REGISTRY
COLLABORATIVE RESEARCH
Demographic Form 1 a
® All rights reserved. Registries Core Facility 2011
Registry Number:
−
−
National Identification
Patient
Registration Date: Passport Mother
− − Iqama Father
DD MM YY YY
Current Residence
Home Town of Father
Telephone Number:
− − −
Mobile Number:
Birth Date (Hijrah): − − Gender: Male Female Unknown
DD MM YY YY
Birth Date (Gregorian): − − Nationality:
DD MM YY YY Saudi Arabs Others
Birth Place: (Hospital/City)
Birth Order:
Mother’s Year of Birth:
Father’s Year of Birth:
YY YY YY YY
Family Member Interviewed:
YY YY YY YY
Premature birth(

CONFIDENTIAL INFORMATION. TO BE USED ONLY FOR THE CHD REGISTRATION.
CONGENITAL HEART DEFECTS REGISTRY
COLLABORATIVE RESEARCH
Diagnosis and Coding Form (1 b)
® All rights reserved. Registries Core Facility 2011
Registry Number
−
−
Date of first Diagnoses
Date of presentation at registering hospital (CVS services)
− − − −
DD MM YY YY DD MM YY YY
At presentation
At birth
Height (cm) . Height (cm) .
Weight (Kg) . Weight (gms)
Sequential Segmental Anatomy (Check if Normal ):
Valid Basis of diagnosis( check all that apply)
(a) Cardiac Situs (b) Position of the Heart Typed Operative Report Electrophysiological Studies 2 D Echo.
Solitus Levocardia Operative Notes Trans-esophageal Echo Others
Cardiac Cath Report
Holter Monitoring
Cardiac Congenital Anomalies :
EPC Codes
Primary Diagnosis: . .
Secondary Diagnoses: . .
. .
. .
. .
. .
. .
. .
. .
. .
. .
Associated non Cardiac Diseases / Syndromes:
ICD-9CM Codes
. .
. .
. .
. .
. .
. .
Diagnosis Confirmation:
Remarks:
Filled by (Initials & date)
Charts Volumes
− − 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
DD MM YY YY
Entered by (Initials & date)
Updated by (Initials & date)
− − − −
DD MM YY YY DD MM YY YY

CONFIDENTIAL INFORMATION. TO BE USED ONLY FOR THE CHD REGISTRATION.
CONGENITAL HEART DEFECTS REGISTRY
COLLABORATIVE RESEARCH
Interventions Form 2
® All rights reserved. Registries Core Facility 2011
Registry Number
−
−
Date of intervention
−
−
DD MM YY YY
Procedure:
Successful Failed Residual lesion
Cardiac Catheter Procedure
EPC Code
. .
. .
. .
. .
. .
Documentation for surgical procedure(s)
Dictated Operative Report
Operative Notes
Surgical Procedures:
EPC Code
Primary . .
Secondary
. .
. .
. .
. .
. .
. .
. .
. .
. .
Surgical Confirmation:
Remarks:
Name of the coder:
Filled by (Initials & date)
Charts Volumes
− − 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15
DD MM YY YY
Entered by (Initials & date)
Updated by (Initials & date)
− − − −
DD MM YY YY DD MM YY YY

CONFIDENTIAL INFORMATION. TO BE USED ONLY FOR THE CHD REGISTRATION.
CONGENITAL HEART DEFECTS REGISTRY
COLLABORATIVE RESEARCH
Follow up Form 3
® All rights reserved. Registries Core Facility 2011
Registry Number:
−
−
Follow-up Date
Next Follow-up Date
− − − −
DD MM YY YY DD MM YY YY
Check if Patient was seen in Adult Cardio logy Hight (cm): ·
Weight (Kg): ·
Patient Status:
Alive
Discharged to be followed elsewhere
Discharged from Cardiology Clinic. Not on Follow-up
Deceased. If YES, where:
Disease Status:
Asymptomatic
With symptoms
Well without symptoms & needs follow-up
With symptoms & needs follow-up and/or future intervention
Registration Hospital
Other Hospital
If symptomatic then state the symptoms:
Home
Unknown
Telephone Followup: Date − −
Alive
Lost to Follow up
Deceased
If deceased then give details:
Form filled by:
Entered by:
Date − − Date: −
DD MM YY YY DD MM − YY YY

Appendix II
Data Request Form

King Faisal Specialist Hospital and Research Centre, Riyadh
Data Request Form
Congenital Heart Defects Registry
Name: ID No. Position:
Department: Institution: MBC No. Ext.
Date Requested:
Date by which data is required:
1. Data Requested (specify patient population, time period, etc.) Hospital Level Multi-institutional level
If Hospital level, specify name below:
A. Required Patient Information (specify variables. Use separate sheets if required)
1. 3. 5.
2. 4. 6.
B. Time period From: To:
2. Purpose of the request
Presentation at conference/meeting
Publication
Other, please specify
Spin-off Research Study
Patient Care
Other than Research (specify the reason and provide approval from the Chairman of the Department in your institution)
3. Is the research study for which the data is requested, approved from Research Advisory Council (RAC)
Yes
No
If Yes, provide the RAC Number and attach a copy of the approval memo
If No, explain the reasons
4. If presentation or publication of data is anticipated, identify collaborators and co-authors to be credited:
1. 2.
3. 4.
(Printed Name)
(Signature)
Request Received By:
Confidentiality Statement signed
Registrar, CHD Registry Dated (DD/MM/YYYY) Yes No
For CHD Registry Committee
Request Granted
No
Yes If Yes, date request granted: Signed by
Dated (DD/MM/YYYY) Chairman Registry Committee :
For CHD Registry Use Only
Request Control Number: ---- Request Completed by:
Date received:
Completion date:

King Faisal Specialist Hospital Research Center
Database Access Form
Congenital Heart Defects Registry
To: Head Office
Congenital Heart Defects Registry
Please provide the following member access to the registry database as per rights below
Staff Name:
Hospital / Institute:
Job Description: Clinical Interviewer Clinical Coder/Registrar Researcher
Rights:
Timing:
Read Only (No Download)
Read Only (With Download)
Registrar
National Registrar
Others as below specified by time:
Hospital Working Days (SAT - WED)
24 hours a day (Only Working days)
24 hours a day 7 days a week
Limited by time specified below
Read and Write (No Deletion - No Data Download)
Read and Write (With Deletion - No Data Download)
Read and Write (No Deletion - With Data Download)
Read and Write (With Deletion - With Data Download)
Browse only user
From:
To:
Date:
Time:
Date:
Time:
To be completed by the registrar
Undertaking
I understand that
* information in any format, maintained in the Registry files is strictly confidential.
* if I am found to be involved or trying to be involved in any act leading to breach of patients' Privacy and Confidentiality, the Administration
reserves all the rights to take legal action against me.
* no piece of information I encounter during the routine working with the database will at any time be used anywhere, verbally or in writing,
nor it will be referenced, in such a way that it will disclose the identity of the patient.
* administration at Saudi Congenital Heart Defects Registry Program reserve all the rights to stop me accessing any information at any
time without prior intimation or specifying any reason to it.
Requestor's Signature
(Signature of Registrar)
Date
Date

Appendix III
Confidentiality Policies

King Faisal Specialist Hospital and Research Centre, Riyadh
CONFIDENTIALITY STATEMENT
Congenital Heart Defects Registry
Name: ID No. Position:
Department:
Institution:
I declare that I understand and abide by the rules on confidentiality, security and release of information for users of the
Saudi National Congenital Heart Defects Registry as outlined below.
(Print Name)
(Signature)
(Date)
Rules of Confidentiality, Security and Release of Information for users of
Saudi National Congenital Heart Defects Registry Data
1. Data held by Registries Core Facility at Biostatistics, Epidemiology and Scientific Computing Department on patients in Saudi National Congenital Heart Defects Registry
is intended for the purposes of Scientific Research and Statistical Analyses, Healthcare and Hospital Administration support only. The data cannot be used
for any other purpose.
2. Data received from Saudi National Congenital Heart Defects Registry should not be divulged to any person whose name is not specified as a co-user of the data nor
should it be used for any other purposes than that declared in Registry Data Request Form.
3. Proper safeguards should be applied in keeping and destroying the data upon completion of the work/project in order to prevent any breach of confidentiality.
The Chairman of the Registry Committee should be notified immediately of any misuse or loss of data.
4. No patient is to be contacted by a research worker as a result of information supplied by the registry without prior review and consent of the
Registry Committee
5. Any statistics or results of research based on data received from the registry should not be made available in a form which directly identifies individual data
subjects and/or is not covered by the purpose of request specified in the Data Request Form.

RCF Policies & Procedures
Fourth Revision
Chapter: Three Section: Four
Policy No. 03-04-01
Policy:
Registry Data – Release of Data and/or Information and Result Reporting Policy
Issued: January 2002 Revised: April 2008
General:
Data collected by disease registries is directly related to the health care of the patients. This
data collected on a hospital/national level is for research purposes available for
researchers/doctors without disclosing patient’s identification. Anyone interested in the
registry data for research purposes has to submit their research project proposal for approval
to the Office of Research Affairs (ORA) of King Faisal Specialist Hospital and Research
Center. Once ORA approve the research project, the data can be made available by the
registrar to the requestor after necessary documentation.
Statement:
1. Responsibility of Reporting the descriptive statistics based on the yearly collection of
data in the form of an Annual Report rests upon the registrar of the relevant registry.
2. Any request for release of information / data for research or other purposes should be
processed by the Registrar who is responsible for documenting the request and informing
the Registry Committee regarding the release of data from the registry. The data export
facility from the web-database software will allow the registrar to furnish the data
request. It should be made certain that the whole procedure is in conformity to the RCF
Confidentiality Policy. Registrar is also responsible for maintaining the log of all such
releases of information.
Policy Objective:
• To safeguard against unauthorized release of registry information.
• To provide a smooth mechanism for the provision of registry data/information to
authorized individuals.
Application / Scope:
All registries under RCF.
Monitoring:
Annual
References
RCF IPP # 03-05-01 Confidentiality Policy

RCF Policies & Procedures
Fourth Revision
Chapter: Three Section: Five
Policy No. 03-05-01
Policy:
Confidentiality Policy
Issued: January 2002 Revised: April 2008
General:
Since a disease registry requires the review of significant amounts of data there is normally a
very thorough review of each patient's medical record. All information obtained on patients
shall be considered extremely confidential. The actual medical record is the property of the
hospital and is kept to document the course of a patient's care and provide communication
between all health care professionals for both current and future care of the patient. The actual
information contained within the medical record is the patient's property and cannot be released
to anyone without proper authorization from the patient, a subpoena or court order. It is
important to stress the strictest confidentiality, as new employees are hired as well as periodic
reminders for other employees. RCF members have an obligation to safeguard the
confidentiality of personal information maintained in the disease registries. This is governed by
ethical and professional codes of conduct. Because of the rapid development of electronic
processing of information making sensitive data widely available it is required by the users of
sensitive data to ensure they also use common sense when handling data. Different professional
and ethical considerations apply depending on the purpose for which the information is used.
Policy Definition:
Confidentiality
Whilst RCF accepts that great benefits can be made from the information it has
collected through disease registries and that medical professionals and hospital
management should have ready access to the information they need, it is also
important that personal information is kept confidential and that privacy is
respected. Disciplinary action may result from a breach of confidentiality, where a
breach of contract can be proved.
Principles of Confidentiality
a. The purpose for which data collected by the registry are to be used should be
clearly defined.
b. All disease registries in the RCF must maintain the same standards of
confidentiality as customarily apply to the doctor-patient relationship; this
obligation extends indefinitely, even after the death of the patient.
c. Identifiable data may be provided to a clinician for use in the treatment of a
particular disease / patient observing that only the data necessary for the stated
purpose are released. Access to patient identifiable information should be on a
strict need to know basis. Only those individuals who need access to patient
identifiable information should have access to it, and they should only have access
to the information items that they need to see. Use the minimum necessary patient
identifiable information.
d. The scope of confidentiality extends not only to identifiable data about data
subjects and data suppliers, but also to others directly or indirectly identifiable data
stored in or provided to the registry.
e. Data on deceased persons should subject to the same procedures for confidentiality
as data on living persons.

RCF Policies & Procedures
Fourth Revision
f. Don't use patient identifiable information unless it is absolutely necessary. Patient
identifiable items should only be used if there is no alternative.
g. Everyone should be aware of their responsibilities. Action should be taken to
ensure that those handling patient identifiable information, both clinical and nonclinical
staff, are aware of their responsibilities and obligations to respect patient
confidentiality.
h. Guidelines for confidentiality apply to all data regardless of storage or transmission
media.
Policy Statement:
1. Registrar of each registry is responsible for assuring the confidentiality and security
of registry data.
2. The RCF staff should sign, as part of their contract of employment, a declaration
that they will not release confidential information to unauthorized persons. The
declaration should remain in force after cessation of employment. They are also
given a copy of the statement. It is essential that the requirements and
responsibilities for people working with all the registries, record and databases
maintained by Registries Core Facility (RCF) are clearly defined and understood.
This policy outlines the steps that registry database users must adopt. 'Users' are
authorized personnel to access any database. The policy also includes those staff
members who are charged with the responsibility of creation, maintenance and
development of registry databases and relevant software in Biostatistics,
Epidemiology and Scientific Computing Department.
3. Suitable control of access to the registry, both physical and electronic, and a list of
persons, authorized to enter the registry should be maintained by the Registrar.
4. The Registrar should maintain a list of staff members indicating the nature and
extent of their access to registry data.
5. Notices reminding staff of the need to maintain confidentiality should be promptly
displayed.
6. Registries at RCF should provide proof of identity to staff engaged in active patient
registration.
7. Identifiable data should not be transmitted by any means (post, telephone or
electronic) without explicit authority from the Head, RCF or staff member to whom
such authority has been delegated. Transmission by telephone should in general be
avoided.
8. Registries should consider the use of courier services for confidential data, as well
as separating names from other data for transmission.
9. Precautions should be taken for both physical and electronic security of
confidential data sent on magnetic, optical or electronic media. This could be done
by separating identifying information or via encryption of the identification.
10. Use of computer for confidential data should be controlled for electronic and if
possible physical measures to enhance the security of the data, including use of
separate room, passwords, different levels of access to data, automatic logging of
all attempts to enter the system, and automatic closure of sessions after a period of
inactivity.
11. Demonstration of the computer system / database management software should be
performed with separate and fictitious or anonymous data sets.
12. Special precautions should be taken for the physical security of electronic backup
media.

RCF Policies & Procedures
Fourth Revision
13. Expert advice on security against unauthorized remote electronic access should be
sought if necessary.
14. Measures should be taken to ensure the physical security of confidential records
held on paper or any other media and to protect such data from corruption.
15. A policy should be developed for the safe disposal of confidential waste.
16. Security procedures should be reviewed at suitable intervals, and consideration
should be given to obtaining specialist advice.
17. Any unauthorized release of patient information will be punishable as stated in
“Oath of Confidentiality”.
Release of Data
a. Release of registry data for research and for healthcare planning is central to the
utility of a registry. The registry should develop procedures for data release that
ensures the maintenance of confidentiality.
b. The registrar is made responsible to present the request for identifiable data to the
Registry Committee and make recommendations to the committee that the
particular request meets the requirement of the law and the registry guidelines on
confidentiality.
c. In the absence of written consent from data subjects a registry should not release
identifiable data on data subjects for the purpose other than research and statistics.
National legislation with respect to confidential data should be observed.
d. Physicians should be given access to data needed for the management of their
patients if identified as such and if in accordance with national / institutional
regulations.
e. Enquiries from the press should be directed to the Chairman of the relevant
Registry Committee or to a staff member nominated for this purpose.
f. Requests for identifiable data to be used for research should include a detailed
justification with a commitment to adhere to the registry’s guidelines on
confidentiality.
g. Registries should provide a document describing their procedures and criteria for
the release of data especially identifiable data to researchers who request access to
the data.
h. If allowed by the institutional and/or national regulations, cross-border transfer of
identifiable individual data should only be carried out if required for the conduct of
a research project and if the level of protection is satisfactory.
Policy Objective:
• The need for a code of conduct in the maintenance of confidentiality in disease registries
and the definition of what should be considered confidential.
• The principles of confidentiality including measures to maintain and survey security
procedures.
• Guidelines for the preservation of confidentiality and for the use and release of registry data
in accordance with these principles.
Application / Scope:
All registries under RCF