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

A very low-birth-weight neonate was referred to our hospital because of total anomalous pulmonary venous connection (TAPVC) and extracardiac multiple anomalies, including high imperforate anus. The TAPVC was classified as type Ib, and a diagnosis of pulmonary venous obstruction was made. Furthermore, left unilateral renal agenesis and right multicystic dysplastic kidney were found. We discussed the treatment strategy with physicians from other departments. TAPVC was urgently corrected using a sutureless surgical technique on day 1 of life. During the management of the imperforate anus, the patient was deeply sedated to avoid aerophagia. Peritoneal dialysis was used to improve the edema postoperatively. Colostomy was performed on postoperative day 12. The patient was extubated on postoperative day 19. Later, the patient was diagnosed with cat eye syndrome by chromosome analysis. Cardiac catheterization at 8 months of age revealed pulmonary hypertension. Moreover, home oxygenation therapy and medications such as bosentan hydrate and tadalafil were administered. Consequently, the imperforate anus was radically corrected at 1 year of age. At 5 years of age, the patient is doing well with good renal function. This paper reports the operation and management of a very low-birth-weight neonate with critical congenital heart disease and extracardiac multiple anomalies.