FEATURES AND CONCEPTS

SAUDI CONGENITAL HEART DEFECTS REGISTRY

Research Advisory Council Number: 991026

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?

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

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).

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.

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.

CHDR Life Cycle

Data collectionand abstraction Data entry

Data validation and quality assurance of collected data.

Data analysis for reporting and publishing

Sharing Information

CHDR Collected and Abstracted Data

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.

• 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.

CHDR Collected and Abstracted Data

Diagnoses 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.

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

• Patient’s status and Disease status according to the physician’s 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)

• 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.

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

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;

Patient’s name, medical record number,

Telephone #,

Hospital’s 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.

CHDR Multi Institutional Data As of April 2012

23,215• Total number of registered patients since the

inception of the registry.

54,494

• Accounts for the cumulative number of follow up visits recorded in the CHDR database.

• Follow up visits are documented while patients attendance to the cardiology clinics.

15,662• Accounts for the total number of heart surgery

episodes recorded in the CHDR database.

5,000• Accounts for the total number of cardiac

catheter intervention episodes recorded in the CHDR database.

Multi Institutional Data as of April 2012

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.

Pulmonary Valve Stenosis

Aortic Valve Stenosis

Tetralogy of Fallot

Coarctation of Aorta

Atrioventricular Septal Defect

D-Transposition of Great Arteries

Congenitally Corrected Transposition of Great Arteries

Patent Ductus Arteriosus

Pulmonary Valve Atresia

Tricuspid Valve Atresia

Hypoplastic Left Heart Syndrome

0 500 1000 1500 2000 2500 3000 3500

3331

500

2060

1544

980

1520

340

1377

956

355

192

Multi Institutional Data as of April 2012

Male; 11928; 51%

Female; 11287; 49%

Tetralogy of Fallot

Pulmonary Valve Stenosis

Patent Ductus Arteriosus

Transposition of Great Arteries

Coarctation of Aorta

Aortic Valve Stenosis

Pulmonary Valve Atresia

Congenitally Corrected Transposition of Great Arteries

Congenital Complete Heart Block

Hypoplastic Left Heart Syndrome

Long QT syndrome

0 10 20 30 40 50 60 70Female Male

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

Distribution Of Heart Surgery Episodes Based on Patients’ Age At Intervention

0-365 During first year of life 2-6 years 7-16 years More than age of 16 years

0

500

1000

1500

2000

2500

3000

3500

4000

4500

50004760

43804216

1908

398

11,497 patients underwent 15,662 episodes of heart surgery

The intervention plan for CHD patients differs and is tailored according to every patient’s status hence; recording heart surgery episodes is ongoing while patients are admitted to the treating hospitals.

Multi Institutional Data as of April 2012

CHDR Database Counts of Selected group of Heart Surgeries

Tetralogy of Fallot Total Repair

Coarctation of Aorta Repair

Arterial Switch Operation

Norwood Procedure

Rastelli Procedure

Fontan Operation

Permanent Pacemaker Insertion

0 200 400 600 800 1000 1200 1400 1600

1538

960

676

165

94

707

563

More than one repair can be done in the same surgical setting

Multi Institutional Data as of April 2012

CHDR Database Counts of Selected group Co morbid health problems

Down's Syndro

me

Willia

ms Syndro

me

Marfan S

yndrome

DiGeorg

syndrome

Absent spleen

Noonan syndrome

Cleft palate / c

left lip

0

200

400

600

800

1000

1200

1400

1600

1800

2000 1820

78 40 31 50 42

138

Multi Institutional Data as of April 2012

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

0%

10%

20%

30%

40%

50%

60% 54%

7%

36%

3%

Multi Institutional Data as of April 2012

Distribution Of CHD Patients - Age at Diagnoses

Multi Institutional Data as of April 2012

Prenatal At Birth to 1 year 1 year and above0

1000

2000

3000

4000

5000

6000

7000

8000

9000

84

5524

3657

259

8582

1200

Born before 2000 Born after 2000

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:

CHDR Quick and Useful Tools

Chart GenerationChart 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.

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

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.

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 patient’s 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.

Contact Us

Shazia Naz Subhani, MScSenior Technical SpecialistHead- Registries Core Facilityssubhani@kfshrc.edu.saPhone: 966-1-4424868http://rc.kfshrc.edu.sa/rcf

Nadia Dessouky, MDSaudi Congenital Heart Defects Registryndessouky@kfshrc.edu.saPhone: 966-1-4647272 ext:39209http://rc.kfshrc.edu.sa/chd_Program/

Fax: 966-1-4424542 BESC DepartmentKing Faisal Specialist Hospital & Research CenterRiyadh, Saudi Arabia