ouble outlet left ventricle (DOLV) is a rare (incidence of fewer than 1 in 200,000 1 ) congenital cardiac malformation in which the aorta and the pulmonary trunk both arise entirely or predominantly above the morphological left ventricle. Increasing numbers of children with congenital heart disease (CHD) are surviving to reproductive age, and successful pregnancies in patients who have undergone surgery for CHD are being reported with increasing frequency. 2-6 However, there have been no reports to date on pregnant patients who have undergone a radical operation using a prosthetic valve for DOLV.Pregnant women with a prosthetic valve replacement present significant problems in management, primarily because of the high incidence of embolization 7 and therefore they require prophylaxis against thromboembolism.We report here on a successful planned delivery in a patient with DOLV who had undergone a Rastelli operation using a prosthetic valve.
Case ReportA pregnant 35-year-old woman (gravida 4, para 1, aborta 3) who had been diagnosed with DOLV was referred at 4 weeks' gestation. At age 11, she had undergone a Rastelli operation in which the pulmonary valve was replaced with a 21 mm Björk-Shiley prosthesis. After surgery, anticoagulant therapy with warfarin was initiated, and her heart condition improved from New York Heart Association (NYHA) functional class III to class I. Postoperative cardiac catheterization and angiographic study were performed at 2Circulation Journal Vol.68, May 2004 (Table 1) and 7 years (Table 2) after operation and the patient's right ventricular pressure was 56/0 mmHg and 46/0 mmHg, respectively. There was no residual ventricular septal defect shunt.The patient married at age 24 years and became pregnant at 26, but the fetus died at 8 weeks gestation and the patient subsequently underwent an evacuation without complications. Her second pregnancy occurred at the age of 28 years. At 32 weeks' gestation, the prosthetic click disappeared and at this point, the patient's warfarin intake was 3 mg daily. The diagnosis of prosthetic obstruction because of probable valve thrombosis was made, and intravenous heparin therapy was initiated. The prosthetic click remained inaudible, but there was no change in the patient 's Circ J 2004; 68: 501 -503 (Received May 10, 2002; revised manuscript received September 11, 2002; accepted September 18, 2002