Right Heart Catheterization in India and UAE | Complete Patient Guide

• ELIGIBLE PATIENTS:

• Children and adolescents with confirmed or suspected pulmonary arterial hypertension (Group 1 PAH), including idiopathic PAH (IPAH), heritable PAH (BMPR2, ALK1, ENG mutations), and PAH associated with congenital heart disease (PAH-CHD)

• Patients with complex congenital heart disease (CHD) requiring pre-operative haemodynamic assessment — including tetralogy of Fallot (TOF), transposition of the great arteries (TGA), single ventricle physiology (prior to Glenn or Fontan completion), atrioventricular septal defects (AVSD), and anomalous pulmonary venous connections (TAPVC/PAPVC)

• Paediatric patients with unexplained dyspnoea, exercise intolerance, or syncope where non-invasive testing (echocardiography, cardiac MRI) is inconclusive

• Patients being evaluated for advanced heart failure therapies, including cardiac transplantation or mechanical circulatory support (MCS), requiring transplant haemodynamic profiling

• Post-operative surveillance following CHD repair, Fontan palliation, or pulmonary artery reconstruction, to assess residual haemodynamic lesions

• Patients with connective tissue diseases (systemic lupus erythematosus, systemic sclerosis, juvenile idiopathic arthritis) and suspected pulmonary vascular involvement

• Children with sickle cell disease, liver disease (portopulmonary hypertension), or HIV with suspected PAH

• REQUIRED PRE-PROCEDURAL DIAGNOSTICS:

• Transthoracic Echocardiography (TTE) with Doppler — mandatory; estimates PA pressures via tricuspid regurgitation jet velocity, assesses ventricular function, valve morphology, and shunt anatomy

• Cardiac MRI (CMR) — for ventricular volumetry, myocardial tissue characterisation, and pulmonary flow quantification (Qp:Qs) where echo is limited

• CT Pulmonary Angiography (CTPA) — to exclude chronic thromboembolic disease (CTEPH) and delineate pulmonary vascular anatomy

• Six-Minute Walk Test (6MWT) or cardiopulmonary exercise testing (CPET) — functional capacity baseline in cooperative children (≥5 years)

• Pulmonary Function Tests (PFTs) — to exclude parenchymal lung disease contributing to hypoxic pulmonary vasoconstriction

• Laboratory panel — full blood count, coagulation screen (PT/INR, aPTT), renal and hepatic function, BNP/NT-proBNP, thyroid function, and autoimmune/connective tissue disease panel as indicated

• Genetic counselling and testing (BMPR2, ACVRL1, ENG) in familial or early-onset PAH cases

• RELATIVE AND ABSOLUTE CONTRAINDICATIONS:

• Uncorrected coagulopathy or active anticoagulation with supratherapeutic INR (>2.5) that cannot be safely bridged — relative contraindication; requires individualised haematology input

• Active systemic infection or bacteraemia — procedure should be deferred until infection is controlled

• Severe contrast allergy without adequate pre-medication protocol — use of carbon dioxide (CO₂) angiography or intracardiac echocardiography as alternatives should be considered

• Haemodynamic instability requiring vasopressor support — stabilisation required prior to elective catheterisation

• Inability to obtain safe vascular access (e.g., bilateral femoral vein occlusion, superior vena cava syndrome) without alternative access planning (hepatic vein, transhepatic approach)

RIGHT HEART CATHETERISATION APPROACHES AND TECHNIQUES:

1. STANDARD DIAGNOSTIC RIGHT HEART CATHETERISATION (Haemodynamic Profiling):

Performed under general anaesthesia (GA) with endotracheal intubation in infants and young children, or conscious sedation with propofol/midazolam/ketamine in cooperative older children. Venous access is established via the right femoral vein using ultrasound-guided Seldinger technique with a 4F–6F introducer sheath appropriate for body weight. A balloon-tipped, flow-directed catheter (paediatric Swan-Ganz or Berman angiographic catheter) is advanced sequentially to measure: right atrial pressure (RAP), right ventricular pressure (RVP — systolic, diastolic, end-diastolic), pulmonary artery pressure (PAP — systolic, diastolic, mean), and pulmonary artery wedge pressure (PAWP) as a surrogate for left atrial pressure. Cardiac output is determined by thermodilution (preferred in patients without significant tricuspid regurgitation or intracardiac shunts) or the direct Fick method (gold standard in shunt quantification, using measured oxygen consumption or assumed Vo₂ by LaFarge equation in children). Oximetry run (oxygen saturation at each chamber level) identifies the anatomical level of any intracardiac shunt and quantifies Qp:Qs.

2. ACUTE VASOREACTIVITY TESTING (AVT):

Following baseline haemodynamic acquisition, AVT is performed with inhaled nitric oxide (iNO) at 20 ppm for 10 minutes (preferred agent per ESC/ERS and AHA/ACC guidelines), with repeat haemodynamic measurements. Alternative agents include intravenous adenosine (50–200 mcg/kg/min) and inhaled iloprost (a prostacyclin analogue). Response is graded using the Sitbon criteria. A positive response identifies the subgroup of PAH patients who may benefit from high-dose calcium channel blocker (CCB) therapy (nifedipine, diltiazem, or amlodipine) rather than advanced PAH-targeted agents (endothelin receptor antagonists such as bosentan/macitentan; PDE-5 inhibitors such as sildenafil/tadalafil; prostacyclin analogues such as epoprostenol, treprostinil, or iloprost; or sGC stimulators such as riociguat).

3. COMBINED DIAGNOSTIC AND INTERVENTIONAL CATHETERISATION:

In many paediatric cases, diagnostic RHC is combined in a single session with a therapeutic intervention to avoid repeat anaesthesia. Interventions may include:

• Transcatheter device closure of secundum ASD (using Amplatzer Septal Occluder, Gore Cardioform, or equivalent) or muscular/perimembranous VSD (Amplatzer Muscular VSD Occluder or ADO II AS device)

• Balloon pulmonary valvuloplasty (BPV) for pulmonary valve stenosis, using Tyshak or Z-Med balloon catheters sized to annulus diameter by Noonan criteria

• Branch pulmonary artery (PA) balloon angioplasty ± cutting balloon angioplasty for discrete PA stenosis, and high-pressure balloon-expandable stent implantation (Palmaz Genesis, Formula, or Atlas Gold stents) for resistant PA stenosis in patients above minimum weight thresholds

• Blade atrial septostomy or radiofrequency-assisted perforation with balloon septostomy for decompression of the left atrium in obstructed TAPVC or as a palliative measure in severe PAH

• Pulmonary arteriovenous malformation (PAVM) embolisation using coils or vascular plugs (Amplatzer Vascular Plug II/IV)

4. ADVANCED IMAGING-INTEGRATED CATHETERISATION:

• 3D Rotational Angiography (3DRA): Single contrast injection with C-arm rotation generates a 3D reconstructed roadmap of cardiac and vascular anatomy, significantly reducing contrast volume and radiation dose — critical in small children

• Intracardiac Echocardiography (ICE): Real-time catheter-based ultrasound imaging eliminates the need for TOE probes in small children, providing intraprocedural anatomical guidance for device closure

• Pressure-Wire Fractional Flow Reserve (FFR): Used in complex PA stenosis assessment to determine haemodynamic significance of intermediate stenoses before committing to stent implantation

• Hybrid Catheterisation-Operating Room (Hybrid OR / Hybrid Suite): Available at the highest-tier centres, enabling simultaneous surgical access (sternotomy or thoracotomy) with catheterisation capabilities for ultra-complex congenital lesions (e.g., perventricular VSD closure, hybrid stage 1 palliation for hypoplastic left heart syndrome)

5. RADIATION DOSE OPTIMISATION:

Leading paediatric catheterisation centres implement ALARA (As Low As Reasonably Achievable) protocols including pulsed fluoroscopy at low frame rates (7.5–15 frames/second), collimation, lead gonadal shielding, and dose-tracking software (e.g., DoseMap, SENTINEL). The CATH-PCI and IMPACT registry benchmarks are used for quality monitoring.

PRE-PROCEDURE PHASE (4–6 weeks before travel):

• Step 1 — Virtual Consultation with GAF Healthcare: Paediatric cardiologist reviews all existing records, imaging (echo reports, CMR, CTPA), and genetic/laboratory results. A pre-travel case summary is generated and a procedural plan is confirmed.

• Step 2 — Pre-Admission Diagnostic Workup (on arrival or within 48–72 hours): Repeat TTE/Doppler by the local team, updated BNP/NT-proBNP, full blood count, coagulation, renal and hepatic panel, and paediatric anaesthesia pre-assessment (airway assessment, weight-based drug planning). Any elective medications affecting coagulation (e.g., aspirin, anticoagulants) are managed per anaesthesia protocol.

• Step 3 — Anaesthesia and Cardiology Team Briefing: A joint paediatric cardiac catheterisation conference reviews anatomy, planned haemodynamic targets, and contingency plans for haemodynamic instability or arrhythmia.

PROCEDURE DAY:

• Step 4 — Fasting and Pre-Medication: Clear fluids until 2 hours pre-procedure (ESAIC guidelines); weight-appropriate anxiolytic pre-medication (oral midazolam 0.3–0.5 mg/kg) administered 30–45 minutes before induction.

• Step 5 — Anaesthesia Induction: General anaesthesia with propofol or sevoflurane induction, followed by LMA or endotracheal intubation depending on age and airway assessment. Continuous invasive arterial pressure monitoring (radial artery line) and pulse oximetry are established.

• Step 6 — Catheterisation Procedure: Ultrasound-guided femoral venous access, heparin anticoagulation (100 units/kg IV bolus, target ACT 200–250 seconds), sequential catheter advancement, haemodynamic data collection, oximetry run, angiography, and — if indicated — acute vasoreactivity testing and/or therapeutic intervention. Total procedure time: 60–180 minutes depending on complexity.

• Step 7 — Closure and Haemostasis: Sheath removal with manual compression (typically 10–15 minutes for 4F–6F sheaths in children); vascular closure devices are used sparingly in paediatric patients given vessel size. Protamine reversal of heparin is administered if early ambulation is required.

POST-PROCEDURE RECOVERY — DAY 0 (Procedure Day):

• Transfer to Paediatric Cardiac ICU (PCICU) or High Dependency Unit (HDU) for 4–6 hours monitoring: continuous ECG, pulse oximetry, non-invasive BP, and observation for access-site haematoma, arterial spasm, or cardiac arrhythmia.

• Restart of oral fluids when fully awake; early mobilisation encouraged in older children.

• Discharge from PCICU to ward if observations stable.

RECOVERY MILESTONES:

• Day 1 (Post-Procedure): Clinical review by paediatric cardiologist; repeat TTE to confirm no new pericardial effusion or procedural complications; access site review. Most diagnostic-only RHC patients are discharged on Day 1.

• Day 2–3: Patients who underwent concurrent interventional procedures (device closure, stenting) remain hospitalised for telemetry, antiplatelet therapy initiation (aspirin 3–5 mg/kg/day for ASD/VSD device closure), and post-interventional echocardiography.

• Day 5–7: Outpatient review with cardiologist; haemodynamic report and final catheterisation data interpreted in the context of the clinical case; PAH medication titration initiated if applicable. Child may begin light activity.

• Day 7–14 (Fit-to-Fly Clearance): Physician issues formal fit-to-fly certificate. Short-haul flights (<4 hours) may be cleared at Day 5–7 for uncomplicated diagnostic cases. Long-haul flights require stability at Day 10–14. Supplemental in-flight oxygen is recommended for children with resting SpO₂ <92% or significant PAH.