Comprehensive physiotherapy rehabilitation following bilateral pulmonary thromboendarterectomy in a patient with chronic thromboembolic pulmonary hypertension associated with factor V leiden mutation

Vijayaragavan. J1*, Praveen Kumar2

1Senior Physiotherapist (CTVS), Kauvery Hospital, Vadapalani, Chennai, Tamil Nadu

2Junior Physiotherapist (CTVS), Kauvery Hospital, Vadapalani, Chennai, Tamil Nadu

*Correspondence

Abstract

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a potentially curable form of pulmonary hypertension managed surgically with pulmonary thrombo endarterectomy (PTE). Structured physiotherapy may enhance postoperative recovery.

Case Presentation: A 50-year-old male with CTEPH associated with Factor V Leiden mutation underwent bilateral PTE. A comprehensive perioperative physiotherapy program including breathing exercises, inspiratory muscle training, airway clearance, and progressive mobilization was implemented.

Outcome: The patient demonstrated improved respiratory function, mobility, and independence without major postoperative pulmonary complications.

Conclusion; Early individualized physiotherapy contributed to successful functional recovery following PTE.

Keywords: CTEPH – Chronic Thromboembolic Pulmonary Hypertension; PTE – Pulmonary thrombo endarterectomy; MIP – Maximal Inspiratory Pressure. Clinical measurement that evaluates the strength of respiratory muscles – primarily diaphragm and intercostal muscles; MEP – Maximal Expiratory Pressure. Clinical measurement that measures the maximum strength of expiratory muscles – abdominal muscles and internal intercostal muscles; NYHA – New York Health Association

Introduction

Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive condition caused by persistent organized thromboembolism obstructing the pulmonary arteries, leading to increased pulmonary vascular resistance and right heart failure if untreated. It affects 0.5–5% of patients after acute pulmonary embolism. Risk factors include thrombophilic disorders such as Factor V Leiden mutation, which is the most common inherited thrombophilia in Caucasian populations.

Pulmonary thromboendarterectomy (PTE) is the definitive surgical treatment for eligible patients, offering significant hemodynamic improvement. However, it is a major procedure associated with substantial postoperative morbidity, including pulmonary complications and reperfusion edema. Physiotherapy plays a crucial role in preventing postoperative complications and restoring function after cardiothoracic surgery. Nevertheless, literature specifically on rehabilitation following PTE remains limited. This case report describes the perioperative physiotherapy management of a patient with CTEPH and Factor V Leiden mutation who underwent bilateral PTE.

Case Presentation

A 50-year-old male presented with a six-month history of progressive exertional dyspnea (NYHA class III), fatigue, and chest discomfort. His history included chronic right lower limb deep vein thrombosis (DVT) diagnosed three years prior, heterozygous Factor V Leiden mutation, hyperhomocysteinaemia, obesity (BMI 31 kg/m²), and hypertension. He was previously active but had experienced significant functional decline.

Transthoracic echocardiography revealed severe pulmonary hypertension (estimated systolic pulmonary artery pressure 78 mmHg), right ventricular dilatation and dysfunction (TAPSE 14 mm). Ventilation-perfusion scanning showed bilateral segmental perfusion defects, and CT pulmonary angiography confirmed organized thrombus in both main pulmonary arteries. Right heart catheterization demonstrated a mean pulmonary artery pressure of 46 mmHg and pulmonary vascular resistance of 680 dyn·s·cm⁻⁵. The multidisciplinary CTEPH team deemed him suitable for bilateral PTE.

Physiotherapy Assessment and Intervention

Preoperative Assessment

Baseline assessment revealed a rapid, shallow breathing pattern (respiratory rate 22/min) with accessory muscle use, reduced chest expansion (2 cm bilaterally), SpO₂ 94% on room air, and inspiratory (MIP) and expiratory (MEP) muscle strength of 58 cm H₂O and 72 cmH₂O respectively (65% and 60% of predicted). The 6-minute walk test (6MWT) was 320 m with Borg dyspnoea score 5/10. Mobility was independent but limited in endurance, and the Modified Barthel Index was 85/100. The patient demonstrated a weak cough and expressed significant preoperative anxiety.

Preoperative Intervention (Days -2 to -1)

Preoperative physiotherapy included patient education on surgery and postoperative expectations, diaphragmatic breathing, thoracic expansion exercises, incentive spirometry (target 1,000 mL, 4× daily), and inspiratory muscle training (IMT) at 40% of MIP (23 cmH₂O, 2× daily). Limb exercises and supervised ambulation with pulse oximetry monitoring were also performed twice daily. These interventions aimed to optimize respiratory function and reduce anxiety.

Postoperative Intervention

Phase 1 – Intensive Care Unit (Days 0–2): Following uncomplicated PTE (circulatory arrest time 38 minutes), the patient was admitted intubated. On Day 1, a spontaneous breathing trial commenced, alongside positioning for secretion drainage, active-assisted limb exercises, and bed mobility training. Diaphragmatic breathing and incentive spirometry were initiated. Airway clearance included assisted cough and gentle manual hyperinflation as tolerated. On Day 2, the patient was extubated successfully. Sitting out of bed, standing at the bedside, deep breathing exercises, IMT at 30% MIP, and huffing techniques were introduced.

Phase 2 – Ward (Days 3–7): Mobilization progressed from marching on the spot to bed-to-chair transfers and assisted ambulation (15–20 m on Day 3, increasing to 100 m by Day 7). Breathing exercises continued with incentive spirometry hourly. IMT advanced to 40% MIP. The active cycle of breathing technique (ACBT) was introduced for airway clearance. Upper limb exercises with 1 kg weights and lower limb strengthening with Thera Band were added. Stair climbing was initiated (3 steps on Day 4, progressing to one flight by Day 7). Sit-to-stand practice and energy conservation strategies were also taught.

Phase 3 – Home-based Rehabilitation (Week 2 onwards): The patient was discharged on Day 10 with a home program including breathing exercises, IMT (40–50% MIP, 2× daily), a progressive walking program (target 20–30 min daily), Thera Band strengthening exercises, and stair climbing practice. Outpatient physiotherapy follow-up was scheduled at 2 and 6 weeks.

Outcome Measures

Significant improvements were observed across all domains:

Outcome MeasurePreoperativeAt Discharge (Day 10)6-Week Follow-up3-Month Follow-up
MIP(cmH2O)58728495
MEP(cmH2O)728596108
6MWT (m)320Not Performed450520
Modified Barthel Index85/10090/10095/100100/100
Borg Dyspnoea Score5/103/102/101/10
SpO2(rest/ room air)94%96%97%98%
NYHA ClassIIIIIIII

No major postoperative pulmonary complications (atelectasis, pneumonia, or reintubation) occurred. Hospital length of stay was 10 days. Pain was well-controlled (VAS 2–3/10 at discharge). The patient returned to work part-time at 8 weeks and fully at 12 weeks.

Discussion

This case demonstrates that comprehensive perioperative physiotherapy can contribute to excellent functional recovery following bilateral PTE in a patient with CTEPH and Factor V Leiden mutation. The structured program encompassing preoperative optimization, early mobilization, breathing exercises, and ongoing rehabilitation was associated with significant improvements in respiratory muscle strength, exercise capacity, and independence, without major pulmonary complications. Patients with CTEPH often present with deconditioning, respiratory muscle weakness, and impaired exercise tolerance that may persist despite surgery. Preoperative IMT has been shown to reduce postoperative complications in cardiothoracic patients. In this case, preoperative education and IMT likely facilitated early engagement and improved baseline respiratory status.

Postoperatively, balancing early mobilization with bleeding risk especially given therapeutic anticoagulation required careful monitoring. The patient’s early extubation, sitting out of bed, and progressive ambulation were achieved without hemodynamic compromise or bleeding events. Breathing exercises and ACBT were effective in maintaining airway clearance and preventing atelectasis, which is particularly important after PTE due to surgical manipulation and cardiopulmonary bypass effects on mucociliary clearance. The role of physiotherapy in CTEPH remains under-researched. However, available evidence supports rehabilitation in pulmonary hypertension to improve exercise capacity and quality of life. Our case adds to this limited literature by detailing a safe and effective perioperative rehabilitation protocol for PTE patients. Success also reflects the value of a multidisciplinary approach involving surgeons, intensivists, nurses, and physiotherapists.

Conclusion

Comprehensive perioperative physiotherapy, including breathing exercises, IMT, airway clearance, and progressive mobilization, likely contributed to successful functional recovery in this patient following bilateral PTE. Physiotherapy should be considered an essential component of multidisciplinary CTEPH care. Further research is guaranteed to develop standardized rehabilitation guidelines for this population.

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