日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

We describe the case of an infant with Marfan syndrome (MFS) complicated by ventricular septal defect (VSD), who developed rapidly progressive heart failure and reduced cardiac function after surgery. The patient was a 2-month-old girl who was diagnosed with heart failure caused by excessive pulmonary blood flow due to VSD and referred to our hospital. MFS was suspected based on family history and physical findings. Since excessive pulmonary blood flow was accompanied by a decrease in cardiac function, a staged repair was performed. Although the cardiac function decreased immediately after pulmonary artery banding and intracardiac repair, it gradually recovered. At the age of 3 years, lens luxation was found. Since the patient’s father had MFS, she met the revised Ghent criteria. MFS may induce cardiac dysfunction. If a patient with congenital heart disease requires therapeutic intervention, attention should be paid to the progression of preoperative heart failure and postoperative management, and follow-up and treatment strategies should be carefully considered.