Surgical Reconstruction with Autologous Tissue in a Case of Isolated Unilateral Absence of the Right Pulmonary Artery / 日本心臓血管外科学会雑誌

Isolated unilateral absence of the right pulmonary artery without any intracardiac anomaly is a rare congenital cardiovascular disorder. We performed a successful surgical reconstruction with autologous tissue of this anomaly. The patient was a 1-month-old boy who had been transferred to our center at 3 days of age because of tachypnea and heart murmur. Multi-detector CT and radial angiography imaging revealed isolated unilateral absence of the right pulmonary artery and left patent ductus arteriosus. Conservative treatment did not help his progressive heart failure and pulmonary hypertension due to an acute increase of pulmonary blood flow. Therefore surgical correction was determined to avoid the worsening of those symptoms. Under cardiopulmonary bypass, the right pulmonary artery branching off from the brachiocephalic artery was removed and anastomosed to the main pulmonary artery with an autologous pericardium roll. Symptoms improved postoperatively and he was discharged in good condition on the 21st of postoperative day. Cardiac catheterization, 3 months later, showed excellent results.

Midterm Results of ePTFE Trileaflet Dacron Graft Conduit for Reconstruction of Right Ventricular Outflow Tract in Children / 日本心臓血管外科学会雑誌

Reconstruction of the right ventriclar outflow tract (RVOT) in congenital heart disease often requires implantation of a valved conduit. A hand-made expanded polytetrafluoroethylene (ePTFE) trileaflet Dacron graft conduit has been used at our center since 1997, and has been implanted in 31 patients. Midterm results of this conduit were investigated in 30 of the patients who have been followed at our outpatient clinic. There were 16 males and 14 females. The mean age and body weight were 16.4±7.2 (range, 3.4-33.4) years and 41.7±13.3 (range, 13.0-64.0)kg, respectively. Diagnoses were tetralogy of Fallot with pulmonary atresia in 14 patients, RVOT reconstruction associated with Ross procedure in 8, transposition with pulmonary stenosis in 3, pulmonary atresia with intact ventricular septum in 2, tetralogy with absent pulmonary valve syndrome in 1, pulmonary regurgitation developed after tetralogy repair in 1, and hemitruncus in 1. The median size of the graft was 22 (range, 20-26)mm. All patients were in NYHA functional class I at the time of the latest follow-up. The pressure gradient across the conduit was 11.0±5.8mmHg during the same hospitalization and 13.8±6.5mmHg on the latest echocardiogram (Interval, 2.4±1.5 years, <i>p</i>=0.85). The valve function was well maintained in all patients, with the regurgitation graded as non-trivial in 22 patients, mild in 7, and moderate in only 1. Midterm results of hand-made ePTFE trileaflet valved cunduit was satisfactory. A longer follow-up is mandatory to assess its actual durability.

Staged Arterial Switch Operation without Homologous Blood Transfusion / 日本心臓血管外科学会雑誌

Staged arterial switch operation without homologous blood transfusion was successfully performed in 5 patients weighing 4.1-11.0kg (double outlet right ventricle: 2 cases, transposition of great arteries: 3 cases). The postoperative hemodynamics and respiratory status were uneventful in all patients (initial central venous pressure after ICU admission: 9.0-14.5cmH₂O, mean 12.5cmH₂O, duration of intubation: 3.5-18.0h, mean 7.8h). Autologous blood donation immediately after induction of anesthesia and minimization of bypass circuit were effective methods for open heart surgery without homologous blood transfusion, particularly in staged arterial switch operation requiring prolonged cardiopulmonary bypass.