Appendix

Appendix 1: RHC and acute vasodilator protocol.

Pulmonary Hypertension

Right Heart Catheterization and Acute Vasodilator Protocol

1. Insert a pulmonary artery floating catheter

2. Do hemodynamics readings on room air, which include: RAP, mPAP, PVR, COP/CI (use refrigerated saline), PAWP*, SaO₂, SvO₂, Systemic BP

3. If the patient is hypoxic (SaO₂ < 90% on room air) repeat the same hemodynamics on O₂

4. If PAWP is <15 and diastolic dysfunction is clinically suspected, give 500 cc of normal saline, and repeat PAWP.

5. If the patient has IPAH (or CHD, CTEPH), then proceed to the next step:

*PAWP measurement:

• PAWP should be correctly zeroed and referenced. The referencing (or leveling) is achieved by placing the air-fluid interface of the transducer at the intersection of a frontal plane passing midway between the anterior and posterior surface of the chest and a transverse plane lying at the junction of the 4th intercostal space and the sternal margin.

• PAWP should be measured at end-expiration

• If PAWP cannot be correctly measured, LVEDP should be obtained

Appendix 2: SAPH protocol for PH diagnostic algorithm.

_____ Form 1 _____

Patient AND diagnostic data

Patient Name: ___________________________________ Patient Serial Number: ___________________________

Patient Data

Gender: M ◻ F ◻ Region: _______________Date of Birth: _______________ Place of Birth: _______________ Nationality: Saudi ◻ Non-Saudi ◻

Diagnosis of Pulmonary Hypertension

Diagnosis made: Yes ◻ No ◻

By: Echo ◻ Cath ◻

Other ◻

PH clinical group:

• Group I ◻ Subtype:

• Group I’ ◻

• Group I” ◻

• Group II ◻

• Group III ◻

• Group IV ◻

• Group V ◻

Symptoms

Shortness of Breath: Yes ◻ No ◻

Palpitation: Yes ◻ No ◻

Cough: Yes ◻ No ◻

Chest pain: Yes ◻ No ◻

Pre-syncope: Yes ◻ No ◻

Syncope: Yes ◻ No ◻

Dizziness: Yes ◻ No ◻

Hemoptysis: Yes ◻ No ◻

Duration of Symptoms: Year

Modified NYHA Functional Class

Class I ◻ (no or minimal symptoms)

Class II ◻ (Symptoms with moderate exercise)

Class III ◻ (Symptoms with minimal exercise)

Class IV ◻ (Symptoms with any activities)

Signs

Ht (cm): ________ Wt (Kg): ________ BMI: ________

Pulmonary

Cyanosis: Y ◻ N ◻

Crackles: Y ◻ N ◻

Wheeze: Y ◻ N ◻

GI

Ascites: Y ◻ N ◻

Hepatomegaly: Y ◻ N ◻

Splenomegaly: Y ◻ N ◻

Cardiovascular

Systolic BP: >120 ◻ 120-90 ◻ <90 ◻

JVP: ◻

LL edema: ◻

Loud P2: ◻

Para-sternal Heave: ◻

Pulse: Regular ◻ Irregular ◻

Murmur: Systolic ◻ Diastolic ◻ Both ◻

Appendix 2: SAPH protocol for PH diagnostic algorithm.

_____ Form 1 _____

Patient AND diagnostic data

Patient Name: ___________________________________ Patient Serial Number: ___________________________

Patient Data

Gender: M ◻ F ◻ Region: _______________Date of Birth: _______________ Place of Birth: _______________ Nationality: Saudi ◻ Non-Saudi ◻

Diagnosis of Pulmonary Hypertension

Diagnosis made: Yes ◻ No ◻

By: Echo ◻ Cath ◻

Other ◻

PH clinical group:

• Group I ◻ Subtype:

• Group I’ ◻

• Group I” ◻

• Group II ◻

• Group III ◻

• Group IV ◻

• Group V ◻

Symptoms

Shortness of Breath: Yes ◻ No ◻

Palpitation: Yes ◻ No ◻

Cough: Yes ◻ No ◻

Chest pain: Yes ◻ No ◻

Pre-syncope: Yes ◻ No ◻

Syncope: Yes ◻ No ◻

Dizziness: Yes ◻ No ◻

Hemoptysis: Yes ◻ No ◻

Duration of Symptoms: Year

Modified NYHA Functional Class

Class I ◻ (no or minimal symptoms)

Class II ◻ (Symptoms with moderate exercise)

Class III ◻ (Symptoms with minimal exercise)

Class IV ◻ (Symptoms with any activities)

Signs

Ht (cm): ________ Wt (Kg): ________ BMI: ________

Pulmonary

Cyanosis: Y ◻ N ◻

Crackles: Y ◻ N ◻

Wheeze: Y ◻ N ◻

GI

Ascites: Y ◻ N ◻

Hepatomegaly: Y ◻ N ◻

Splenomegaly: Y ◻ N ◻

Cardiovascular

Systolic BP: >120 ◻ 120-90 ◻ <90 ◻

JVP: ◻

LL edema: ◻

Loud P2: ◻

Para-sternal Heave: ◻

Pulse: Regular ◻ Irregular ◻

Murmur: Systolic ◻ Diastolic ◻ Both ◻

Form 2

Investigational data

General Investigations

Hematology

Hb: HCT: INR:

Renal Profile:

BUN: Creatinine:

Hepatic Profile

Bilirubin: ALT:

AST: Viral screen:

Immune Screen

RF: Positive ◻ Neg ◻ Titer: ______

ANA Positive ◻ Neg ◻ Titer: ______

Schistosoma: Positive ◻ Neg ◻ Titer: ______

HIV 1-2: Positive ◻ Neg ◻

Sickle Cell: Positive ◻ Neg ◻

Thyroid: TSH: Antibody: Positive ◻

Neg ◻

Coagulopathy

Protein C: Normal ◻ Low ◻

Protein S: Normal ◻ Low ◻

Anti - phospholipid: Normal ◻ High ◻

D – Dimer: Normal ◻ High ◻ Titer: ______

Factor V Laiden: Present ◻ Absent ◻

Homocystinuria: Present ◻ Absent ◻

Other Tests

NT-pro BNP: BNP: Troponin: Uric acid:

Specific Investigations

Pulmonary

Arterial Blood Gases on room air

pH: PCO₂: PO₂: HCO₃: O₂ Sat:

6-MWT

Distance (m):

O₂ Sat: Pre: Post: FiO₂:

Borg Scale: Pre: Post:

Pulmonary Function Test

FEV₁: % Predicted FVC: % Predicted

FEV₁/FVC: % Predicted DLco: % Predicted

Cardiopulmonary Exercise Test

VO₂Max: % Predicted

Anaerobic Threshold: % Predicted

Ventilatory Parameters: VE: V_D/V_t: RR:

Cardiac Parameters: BP: PR: O₂

Pulse:

Respiratory Limitation: Y ◻ N ◻

Cardiac Limitation: Y ◻ N ◻

Cardiac

ECG

Sinus Rhythm: ◻ Sinus Tachycardia: ◻

Arrhythmia: ◻ Type: ◻

Right Heart Strain: ◻ (RBBB, Right Axis, RVH, P Pulmonale)

Other Abnormality:

Echo

LVF: EF%:

RV Size and Function: Normal ◻ Abnormal ◻

PAPs: Pericardial Effusion: ◻

Diastolic Dysfunction: ◻

TAPSE: Tei Index: RV Diameter: RA Area:

Other:

Cardiac Catheterization

mPAP: PVR: RAP:

CO: CI: Methods:

Thermodilution: ◻ Fick method: ◻

PAWP: LVEDP:

SaO₂: SVO₂:

Vasoreactive test: Positive ◻ Negative ◻ Agent used:

Imaging

U/S Duplex Leg Study: Positive ◻ Negative ◻

Distal ◻ Proximal ◻

Unilateral ◻ Bilateral ◻

V/Q Scanning

Diagnostic: Yes ◻ No ◻

High probability: Yes ◻ No ◻

Other:

Chest CT Scan

Spiral CT Angiography

Positive ◻ Negative ◻

Central ◻ Peripheral ◻

Other:

HRCT

ILD ◻ Emphysema ◻

Other

Pulmonary Angiography

Positive ◻ Negative ◻

Central ◻ Peripheral ◻

Other:

Appendix 3: SAPH 6-MWT protocol.

Name: ______________________________

MRN: ______________________________

Age: ______________________________

Sex: ______________________________

Height: ______________________________

Weight: ______________________________

Nationality: ______________________________

Smoker: ______________________________

Date: ______________________________

Time: ______________________________

Diagnosis: ______________________________

Attending: ______________________________

Technician: ______________________________

Medication: ______________________________

FIO2: ______________________________

Appendix 4: Criteria for Specialized Pulmonary Hypertension Center.

Pulmonary Hypertension Specialized Center

Center of Excellence

PAH is a rare disease with progressive deterioration and complex management strategy. PAH patients should be managed at highly specialize center with multidisciplinary services and experience staff.

The aim of such centers is to undertake full assessment, initial investigation, and specific management of PAH patients by applying evidence-based diagnostic algorithm and offering PAH-target therapy by expert team in order to obtain best outcome.

PAH center should have a high volume of patients on chronic PAH therapy and accepting newly referred patients.

The following criteria are suggested for pulmonary hypertension specialized center, modified to comply with Saudi health system facilities:

Staff

At least 2 PH consultant physicians specialists (usually from pulmonary and cardiology services)

• Cardiologist with extensive experience in RHC study and hemodynamic studies

• Intensivist with special interest in PH ventilated patients

• At least 1 registered nurse specialized in PH

• Radiologist with adequate experience in PH imaging

• Cardiologist with adequate experience in PH related echocardiography

• Psychologist

• Access to social workers

Volume of activity

• At least 50 patients with PAH or CTEPH in active follow-up

• At least 2 new patients with PAH or CTEPH per month, followed up for 3 years or more

• At least 20 vasoreactivity tests in PAH per year

Experience and quality of care

• Experience with all specific drugs

• Regular clinical review sessions

• Standardized operating procedures for diagnosis and treatment

• Assess indicators of outcome (survival)

Facilities and resources needed

• Specialized respiratory and cardiology department

• Fully equipped ICU

• Advanced echocardiography department

• Cardiac hemodynamics

• Pulmonary function laboratory

• Cardiopulmonary stress testing

• Sleep laboratory

• CT and spiral-CT angiography

• Nuclear medicine

Facilities

• Specialized outpatient department

• Cardiac catheterization with vasoreactivity testing

• Access to all drugs specific to PAH

• 24-h on-call coverage

Information system

Database designed for the assessment of actions taken and results

Research activity

Referral centers should participate in collaborative clinical research in PAH, which includes phase II and phase III clinical trials

Other collaborative services

• Rheumatology specialized cervices

• Heart surgery and thoracic surgery (expertise in

  Pulmonary endarterectomy)

• Lung and heart-lung transplantation

• Congenital heart disease specialty services

• Liver transplantation/liver hemodynamics

• Invasive radiology services/pulmonary angiography

• Infectious disease services (HIV unit, schistosoma)

Contact details

1. Saudi Association for Pulmonary Hypertension (SAPH)

   1. Website: www.saph.med.sa
   2. Email: saph.pht@gmail.com

2. Saudi Thoracic Society (STS)

   1. Email: saudithoracicsociety@yahoo.com
   2. Phone number: + 9661 1 2488966
   3. Fax: + 9661 1 2487431