Description of a multidisciplinary team approach in the management of a nulliparous, previously healthy 46-year-old lady, diagnosed with possible acute on chronic thromboembolic pulmonary hypertension (CTEPH) and pelvic mass, from pre-operative optimization to peri-operative and post-operative management. 

A previously healthy nulliparous 46-year-old lady presented with reduced effort tolerance, shortness of breath, abdominal distension and bilateral lower limb swelling for 3 weeks. Examination revealed pan-systolic murmur at left lower sternal edge with bi-basal lungs crepitation and bilateral lower limb edema.12-leads electrocardiogram showed sinus rhythm with right axis deviation and right bundle branch block. Signs of right heart strain such dilated right heart chambers, right ventricular hypertrophy, plethoric inferior vena cava (IVC), reduced tricuspid annular plane systolic excursion (TAPSE) and D-shaped left ventricle were detected on echocardiography. Estimated pulmonary artery systolic pressure (PASP) was 74.9mmHg.Contrast-enhanced computed tomography of chest, abdomen and pelvis showed large pelvic mass with local mass effect and pulmonary embolism of bilateral segmental arteries. Connective tissue disorder screening was unremarkable.With no feasible route radiological, transvaginal, or trans-rectal for histopathological sampling, patient was counselled for total abdominal hysterectomy with bilateral salpingo-oophorectomy (TAHBSO).A multidisciplinary team approach comprising of Gynae-oncology, General Anesthesiology, Interventional Radiology, Internal Medicine and Cardiology was devised to manage patient through pre-operative optimization to peri-operative and post-operative management. Patient was initiated on subcutaneous enoxaparin for anticoagulation, frusemide for right heart failure, oral Sildenafil 50mg TDS for pulmonary hypertension. An IVC filter was implanted prior to TAHBSO. Nebulized Iloprost 2.5mcg QID was initiated 2 weeks prior to TAHBSO.

There was improvement of TAPSE/PASP ratio from 0.18 to 0.45 prior to TAHBSO. After TAHBSO, patient was monitored in intensive care unit (ICU) for 4 days with low dose Milrinone and Norepinephrine infusion for hemodynamic support. She was transferred to the Gynae-oncology ward for post surgery rehabilitation and discharged well 2 weeks later. Nebulized Iloprost was weaned off prior to discharge. The TAPSE/PASP ratio was 0.66 and estimated PASP was 27 mmHg upon discharge.

We report a successful multidisciplinary team approach with consensus guided pharmacotherapy in minimizing operative risk in a patient with CTEPH undergoing non-cardiac surgery.