Severe pulmonary vascular disease consisting of ventricular septal defect and patent ductus arteriosus. A case diagnosed as the indication for total correction by the hemodynamic study.

A 12-year-old girl with ventricular septal defect, patent ductus arteriosus and pulmonary hypertension was hemodynamically diagnosed as the indication for total correction by the initial cardiac catheterization. However, histologic assessment by lung biopsy revealed the terminal stage of plexogenic pulmonary arteriopathy, i.e. plexiform lesions, dilatation lesions and fibrinoid necrosis of the media which denied the possibility of corrective surgery. We would like to stress that the open lung biopsy should be performed when the hemodynamic study indicates marginal operability, especially in older children and adults in order to investigate the grade of occlusive pulmonary vascular disease.

Histologic findings of ductus arteriosus and pulmonary vascular disease in interrupted aortic arch.

There are still a number of unsolved problems concerning the total correction of interruption of the aortic arch. Here we report a histopathological investigation of the patent ductus arterious and pulmonary vascular disease using autopsy material of a patient with Celoria type B interruption of the aortic arch who died postoperatively. The so-called ductus arteriosus between the pulmonary arterial trunk and the descending aorta was lacking in elastic fibers and smooth muscle cells at the media showing discontinuous, transverse fibrous tissue. The vessel walls were weak and normal development was apparently impossible. Consequently, it was concluded that in the reconstruction of the aortic arch in such cases with PDDT, it is advisable not to use the patent ductus arteriosus. In this case, hypertrophy of the media of small pulmonary arteries was marked, but pulmonary vascular intimal lesions were mild. With regard to the pathogenesis, it is thought that there was sufficient thickening of the media of the pulmonary arterial system in response to the pulmonary hypertension present from birth, and it prevented the progression of the intimal lesions by accommodating to the intraarterial pressure.

Successful surgical correction in two patients with tricuspid atresia using autologous pericardial valve-bearing tube graft.

A modification of the Fontan procedure has been used successfully to correct tricuspid atresia in two children who had severe hypoplasia of pulmonary artery. In the first patient (who had functioning Gleen anastomosis) the hypoplastic left pulmonary artery was widened by an angioplasty, and then a Dacron external conduit containing an autologous pericardial valve (an autologous pericardial valve-bearing tube graft) was positioned to lead from the right atrial appendage to the left pulmonary artery. A large secondum type atrial septal defect was closed by a Dacron patch. In the 2nd patient, a Dacron external conduit without any valve was placed between the right appendage and the hypoplastic right ventricle and then the pulmonary outflow tract was widened by using an autologous pericardial valve patch, which was an extension of the external conduit. The ventricular and atrial septal defects were closed with patches. Both patients made a satisfactory recovery and doing well 4 months after the operation. This type of modification of the Fontan procedure may provide a more effective functional correction for patients with tricuspid atresia who are accompanied with severe pulmonary hypoplasia.

Surgical treatment for hypoplastic left heart syndrome: case reports.

A 58-day-old girl with hypoplastic left heart syndrome underwent bypass grafting from the pulmonary artery to the descending aorta, ligation of the ductus arteriosus, and banding of the main pulmonary artery distal to the graft. Anastomoses of the graft were performed by partial clamping of the arteries under moderate surface hypothermia. The patient tolerated the procedure well and was discharged from the intensive care unit on the eleventh postoperative day, but she died on the twentieth day of metabolic derangement. Our total experience with this anomaly includes three other infants, and a summary of these patients and applied surgical procedures are presented. Surgical and diagnostic considerations for this anomaly based on our experiences are discussed.