Surgical treatment and perioperative management of congenital heart disease with severe pulmonary hypertension
AIM: To review the results and methods of surgical treatment and perioperative management of congenital heart disease (CHD) with severe pulmonary hypertension (PH). METHODS: Thirty-six patients (17 males, 19 females, aging from 1-41 years) of congenital heart disease with severe pulmonary hypertension were included in the study, among whom were 9 cases of atrial septal defect and 20 cases of ventricular septal defect. The saturation of artery oxygen ranged from 0.85-0.94 and echocardiograpby showed left to right slow velocity shunt in 23 cases, double direction shunt in 10 cases and no shunt in 3 cases. The pulmonary pressure was 80 to 130 mmHg(1 mmHg=0.133 kPa), the pulmonary pressure/systemic pressure varied from 0.75-1.0 and the pulmonary resistance was 8-27.2 Wood unit. All the patients were treated with corrective surgery, and one way shunt valve (size 0.5-0.6 cm) from right to left shunt on the repaired patch was created especially for the treatment of extremely severe pulmonary hypertension. The therapy of oxygen inhalation, oral intake of captopril and sildenafil, and intravenous injection of sodium nitroprusside and prostaglandin E1 were routinely administrated perioperatively to reduce pulmonary hyper-tension. Nitric oxide and sildenafil were applied especially for the treatment of extremely severe pulmonary hypertension or pulmonary hypertension crisis. RESULTS: Only one early postoperative death occurred due to low output syndrome, and the other 35 patients were recovered and discharged from the hospital. The 0.5 -7 years follow-up showed that the patients were well recovered with NYHA Ⅰ heart function. CONCLUSION: Satisfactory outcome can be achieved in surgical treatment of CHD with severe pulmonary hypertension by meticulous preoperative analysis of surgical indications, selection of appropriate operative procedures and multiple perioperative therapies.