A 35-year-old woman, gravida two para zero, at 18+ 4 weeks of gestation presented with shortness of breath and palpitations. In 2018, she underwent double bioprosthetic valve replacement (21 mm Carpentier-Edwards Perimount aortic prosthesis and 25 mm Carpentier-Edwards Perimount prosthesis), tricuspid valvuloplasty (30 mm Carpentier-Edwards MC3), and atrial fibrillation (AF) ablation due to rheumatic cardiac valve disease. Concurrently, she was diagnosed with APS and administered hydroxychloroquine (200 mg/day for) and methylprednisolone (4 mg/day for). Due to APS, the patient experienced difficulty conceiving naturally, with two failed artificial inseminations in 2019 and 2020. Of note, her first test-tube baby ended in an abortion in June 2021 at 8 weeks of gestation due to an unexplained silent miscarriage. Low-molecular-weight heparin (LMWH; 0.6 mg twice daily) was initiated from the sixth week of gestation.

A coagulation test showed a slight increase in fibrinogen and D-dimer levels without other abnormalities. Blood tests for rheumatic immune indicators revealed anti-nuclear antibody (+); anti-cardiolipin antibody, IgA 53.7 APL/ml; anti-β2-glycoprotein I antibody, IgA 56.3 U/ml; and anti-SSA/Ro60 antibody (+++). Preoperative echocardiography showed severe intravalvular regurgitation (color flow jet area, 8.1 cm2; mean gradient, 17.3 mmHg) and severe pulmonary artery hypertension (PAH; 91 mmHg) (Fig. 1). Fetal ultrasonography confirmed a healthy fetus.

Preoperative transthoracic echocardiogram: The top left corner shows the measured reflux area of 8.1cm2

Transcatheter mitral valve-in-valve (TMViV) was performed in a hybrid operating room. Anesthesia was administered according to the principles of general anesthesia during pregnancy. To minimize radiation exposure to the fetus, the patient’s abdomen was wrapped 360° with a lead coat, and fetal electrocardiogram was performed by an obstetrician.

A temporary endocardial pacing lead was inserted into the right internal jugular vein. The left ventricular apex was located under digital subtraction angiography (DSA) and transesophageal echocardiography (TOE) guidance. A 4-cm incision was made in the fifth intercostal space. Heparin was administered to achieve an activated clotting time (ACT) of ≥ 250; the actual ACT reached 450. After exposing the apex, double layers of a 2 − 0 prolene apical purse with a spacer were punctured at the midpoint. Guidewires were placed through the mitral valve into the left atrium, a 6Fr vascular sheath was inserted. Thereafter, the super-hard guidewire was exchanged carefully to avoid wrapping the mitral chordae tendineae. A support track across the original mitral valve bioprosthesis was formed, for which a 20Fr vascular sheath was used to dilate it. During paused breathing and a paced heart rate of 140 beats/min, a 25 mm J-valve (JieCheng Medical Technology Corporation Ltd., Suzhou, China) was delivered (Fig. 2). Breathing was resumed after the valve was released. The fetal heart rate did not decrease significantly. The valve position was determined using DSA and TOE; the mitral valve was not fully expanded. The shape of the valve after dilation with a 25Fr balloon was more appropriate. The J-valve system was released; the purse string was tightened; and protamine was administered for heparin neutralization. A drainage tube was placed to avoid tamponade. Postoperative TOE revealed a mean mitral valve gradient of 2 mmHg without obvious valvular regurgitation (Fig. 3). The systolic blood pressure remained > 70 mmHg throughout the procedure. Ichthyosin (50 mg) was used to neutralize the heparin. The overall operating time was 127 min. Moreover, no contrast agent was administered during the procedure. The endotracheal tube was removed in the operating room.

Fluoroscopic image depicting (A) transesophageal echocardiogram probe, (B) 25 mm J-valve, and (C) mitral bioprosthetic valve failure

Postoperative transesophageal echocardiogram showed no significant mitral regurgitation

The patient was discharged three days after surgery. Fetal and transthoracic ultrasounds were performed at least every two weeks, and rheumatic immune indicators were tested monthly. No significant changes were observed in postoperative blood test results for rheumatic immunity. With the approval of the rheumatologist, hydroxychloroquine (200 mg), methylprednisolone (4 mg), aspirin (100 mg) and LMWH (0.6 ml: 6000iu) were administered daily. At 29 weeks of gestation, aspirin was discontinued, and LMWH dosage was changed into 0.4 ml. At 33+ 3 weeks of gestation, the patient was diagnosed with threatened premature labor. Echocardiography revealed a left ventricular ejection fraction of 40% and a PAH of 50 mmHg. After a multidisciplinary team consultation, a cesarean section was performed due to the high maternal and fetal risks of continued pregnancy. The patient gave birth to a healthy baby at 34+ 1 weeks, and the APGAR scores of the newborn were 10 at both the 1st and 5th min. Echocardiographic and electrocardiographic data before surgery, after surgery, before delivery, after delivery, and at the most recent time points are presented in Table 1.