A 45-year-old female patient (164 cm, 52 kg) who visited her previous doctor 3 months ago with complaints of lower abdominal pain and lumps was diagnosed with an ovarian tumor. She was diagnosed with a ventricular septal defect (VSD) at an early age, and with ES at her school age. Medical therapy for ES began 9 years ago with oral tadalafil (phosphodiesterase 5 inhibitor) of 40 mg/day, macitentan (endothelin receptor antagonist) of 10 mg/day, and selexipag (prostacyclin receptor agonist) of 400 μg/day. Home oxygen therapy was introduced for hypoxemia. Thereafter, she led her daily life without problems, but she gradually began to have difficulty in her daily life after becoming aware of lower abdominal pain and lumps and even walking on level ground became difficult. The tumor was suspected to be ovarian cancer, and the patient expressed a strong desire for surgery due to accompanying symptoms. However, because of the presence of severe ES, multiple hospitals found perioperative management challenging, leading to a referral to our hospital.

Her oxygen saturation was 80% with 3 L/min oxygen at rest but dropped to 52% during a 6-min walk test. Transthoracic echocardiography revealed a ventricular septal defect with a bilateral shunt. The right ventricular wall was thickened to 10 mm, and the tricuspid regurgitation pressure gradient was 133 mmHg. There were no findings of decreased left and right ventricular contraction. Right heart catheterization results were as follows: pulmonary artery pressure (PAP) of 123/52 mmHg, arterial pressure of 115/69 mmHg, CO of 3.87 L/min, cardiac index (CI) of 2.44 L/min/m2, pulmonary and systemic flow ratio of 0.53, and pulmonary vascular resistance (PVR) of 28.94 Wood Units.

Anesthesiologists, gynecologists, and cardiologists held a joint preoperative conference to discuss her perioperative management and indicated for open bilateral adnexectomy for the ovarian tumor which could be performed under general anesthesia. First, a cardiologist placed 5-Fr and 7-Fr sheaths in her femoral artery and vein, respectively, before entering the operating room to allow the use of extracorporeal membrane oxygen to prevent intraoperative and postoperative cardiac arrest. Further, a pulmonary artery catheter was placed in her right internal jugular vein. Once in the operating room, the anesthesiologist inserted an arterial catheter and a central venous catheter into the right radial artery and left internal jugular vein, respectively. Noradrenaline of 0.1 μg/kg/min was then initiated before induction of anesthesia.

Perioperative records are shown in Fig. 1. Anesthesia was induced with remimazolam of 2 mg/kg/h, fentanyl of 250 μg, and rocuronium of 50 mg, and maintained with remimazolam of 0.8 mg/kg/h and remifentanil of 0.1 μg/kg/min. The tracheal intubation was performed after 4 ml of 4% lidocaine was sprayed into the trachea. In addition to standard monitoring, invasive arterial blood pressure, PAP, central venous pressure, bispectral index (BIS), cerebral regional oxygen saturation (rSO2), CO/CI/systemic vascular resistance index (SVRI) using the FloTrac system, and transesophageal echocardiography were monitored during surgery. Intraoperative systolic PAP remained between 100 and 140 mmHg, but rarely exceeded the systolic artery pressure and SpO2 always remained > 88% at a fraction of inspiration O2 of 50% (Fig. 1a). SVRI was 2825 Dynes × s/cm5/m2 before induction of anesthesia, but once dropped to 1259 Dynes × s/cm5/m2 after laparotomy and then rose and remained between 1500 and 2300 Dynes × s/cm5/m2 (Fig. 1b). Intraoperative nitric oxide of 10 ppm administration was attempted, but the hemodynamics and SpO2 were unchanged. BIS was 37–62 and rSO2 was 59–77% during general anesthesia. Bilateral transversus abdominis fascia plane blocks were performed after surgical completion, and the patient was transferred to the intensive care unit (ICU) under sedation with tracheal intubation. Anesthesia time was 2 h and 45 min, operation time was 1 h and 53 min, and blood loss was 90 ml. Remimazolam of 0.8 mg/kg/h was continued after ICU admission and was discontinued 30 min thereafter. Dexmedetomidine was initiated at 0.4 μg/kg/h upon ICU admission. Fentanyl of 50 μg/h was administered for postoperative analgesia. Bradycardia, hypotension, and decreased SpO2 were observed with arousal 50 min after admission; therefore, dobutamine of 5 μg/kg/min was initiated, followed by a gradual improvement in hemodynamics and oxygenation. The tracheal tube was removed 1 h and 40 min after admission and fitted with a high-flow nasal cannula. After extubation, SpO2 was maintained at approximately 80%. She was transferred to the general ward on postoperative day (POD) 3 and discharged on POD 11 without any complications. The patient signed written informed consent for this case report.

Intraoperative and postoperative record. a HR heart rate, ABP arterial blood pressure, (S) systolic, (D) diastolic, PAP pulmonary arterial pressure, SpO2 saturation of percutaneous oxygen. b SVRI systemic vascular resistance index, CI cardiac index