Cesarean section in a patient with non-compaction cardiomyopathy managed with ECMO
The 39-year old, 68 kg and 165 cm patient, mother of a 20 year old son, was referred to our institution during the 33rd week of pregnancy. One year before, she had experienced multiple transitory ischemic attacks, she was in NYHA (New York Heart Association) class IV and IVNC was diagnosed based on echocardiography. Besides warfarin, which during pregnancy was switched to low molecular weight heparin, pharmacological therapy with an angiotensin converting enzyme inhibitor had been established. Although severe ventricular arrhythmias had been diagnosed, the patient refused implantation of an internal defibrillator. The transthoracic echocardiography (TTE) at admission showed the typical picture of IVNC: a massively dilated left ventricle (end-diastolic diameter of 80 mm - normal value <50mm), a mitral valve regurgitation grade I, a severely impaired left ventricular ejection fraction (LVEF) of 25% (normal value>55%) and a semi-systemic systolic pulmonary artery pressure (PAP) of approximately 60 mmHg. Due to the inherent risk of cardiac decompensation in the late pregnancy, the patient was admitted to our heart centre one week later and elective caesarean section scheduled for the 35th week of gestation. Due to the risk of sudden life-threatening decompensation during surgery, it was planned to perform the operation under general anaesthesia and “stand by” of veno-arterial extracorporeal membrane oxygenation (ECMO) for extracorporeal life support. On the day before surgery, TTE revealed further decrease of the LVEF to 20%, increase of the mitral valve regurgitation to grade II and increase of the systolic PAP to 2/3rds of the systemic value of approximately 80 mmHg.\nThe patient received 150 mg of ranitidine 12 and 2 hours before the operation. After having arrived in the anaesthesia induction room in nearly sitting position, 30 ml of sodium citrate (0.3 molar) was administered orally. Standard monitoring with electrocardiogram and pulsoxymetry was established. In addition, paddle leads for external defibrillation were placed. Thereafter, an arterial line was placed into the left radial artery; a 4 lumen central venous catheter and a 9 gauge introducer were placed into the right jugular internal vein in local anaesthesia. A pulmonary artery (PA) catheter with atrial and ventricular pacing option (Baxter International, Illinois, USA) was inserted into the introducer and placed into the PA.\nAt this time the heart rate was 110/min with multiple polytopic ventricular extra-systolic (VES) beats. In the nearly sitting position, the arterial blood pressure was 110/70/90 mmHg and the PAP 75/30/50 mmHg with a pulmonary capillary wedge pressure of 30 mmHg. Pharmacologic therapy with a continuous infusion of dobutamine 3 µg/kg/min and milrinone 0.25/kg/min was started. Additionally, two 6 French introducer catheters were placed under local anaesthesia into the right femoral artery and right femoral vein. These catheters were inserted to facilitate quick percutaneous access to the vessels for emergency installation of ECMO for extracorporeal life support in case of acute cardiac decompensation.\nAfter these preparations, the patient was transferred into the operating room and preparations for surgery were performed.\nAfter pre-oxygenation, anaesthesia was induced with 5 mg of rocoronium, 15 mg of etomidate, 35 mg of S ketamine and 100 mg of succinylcholine and a rapid sequence intubation was performed. Three minutes later, a healthy 2800 g child was delivered by classic longitudinal caesarean section (APGAR values of 9-10-10). Thereafter, anaesthesia was continued with a bolus of 30 mcg of sufentanil and 35 mg of rocoronium and a continuous infusion of remifentanil (2 µg/kg/min) and propofol (0.1 mg/kg/min) without using Target Controlled Infusion (TCI). Additionally, 1 g of paracetamol and 1 g of novamin-sulfate and 4 mg of ondansetron were infused. At the end of the procedure residual muscle relaxation was antagonized with 300 mg of sugammadex.\nDuring the entire period of anesthesia, hemodynamics remained completely stable, the PAP decreased to 1/3rd systemic values of 50/30/20 mmHg while, however, polytopic VES were still observed. The completely awake and orientated patient was extubated on the surgical room. The patient was transferred to the intensive care unit (ICU) and she remained here for 5 days. Therapy with milrinone and dobutamine was continued during ICU stay. The patient was discharged uneventfully from hospital 14 days after surgery.