1/10. Retrograde catheterization of the pulmonary veins following surgical correction of transposition of the great vessels.Prior to a Mustard repair for transposition of the great vessels, complete physiologic data of the pulmonary bed can be obtained by catheterization of the pulmonary artery with a "J" formed wire, and direct catheterization of the pulmonary veins via an atrial septal defect. Following "physiologic" repair ready access to the pulmonary veins is denied. Evaluation of the pulmonary venous pressure is of great importance, however, in differentiating pulmonary vascular disease from pulmonary hypertension secondary to patch obstruction of pulmonary venous return. This paper describes a technique of catheterization of the pulmonary veins following a Mustard repair for TGV.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/10. Palliative arterial repair for transposition, ventricular septal defect, and pulmonary vascular disease.Failure to repair transposition of the great arteries and ventricular septal defect in the young infant results in the early development of pulmonary vascular occlusive disease. Complete repair, preferably by an arterial switch procedure and ventricular septal defect closure, may then not be possible. We report a palliative arterial switch procedure in a 5 1/2-year-old patient with transposition, ventricular septal defect, and severe pulmonary vascular obstructive disease in whom progressive hypoxemia and exercise intolerance developed. An arterial repair without ventricular septal defect closure was performed. After the operation, the child's systemic arterial oxygen saturation and exercise tolerance have substantially improved. Although the progression of pulmonary vascular disease may not be altered, arterial repair can provide effective palliation in this subset of patients.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
3/10. Transposition of great arteries with aortopulmonary window: an unusual cause of prepared left ventricle at 11 months.In patients with transposition of great arteries, presence of aortopulmonary window is very uncommon and associated with high morbidity and mortality. This report describes the case of an 11-month-old female patient in which aortopulmonary window was restrictive, and protected the patient from developing pulmonary vascular disease. The patient underwent successful arterial switch and repair of aortopulmonary window.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/10. Palliative Mustard or Rastelli operation in complete transposition of the great arteries. Option decided by lung biopsy.A case history is reported of a patient with complete transposition of the great arteries and a ventricular septal defect in whom pulmonary hypertension developed, with a pulmonary resistence of 10 units M.2. Because of the possibility that the pulmonary vascular obstructive disease might have been of recent onset and that a high hematocrit value of 82 percent may have interfered with the precise determination of pulmonary resistance, an open lung biopsy was performed. The histologic findings suggested that the vascular disease was as yet reversible. Because of these findings and the aforementioned considerations, a Rastelli operation was performed instead of a palliative Mustard procedure. At present, 6 months postoperatively, the patient is asymptomatic.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/10. Mustard operation and creation of ventricular septal defect in two patients with transposition of the great arteries, intact ventricular septum and pulmonary vascular disease.The condition of patients with transposition of the great arteries, intact ventricular septum and severe pulmonary vascular disease is inoperable with present techniques. In a series of 260 surgically treated patients with transposition of the great arteries and intact ventricular spetum, 5 had severely increased pulmonary vascular resistance, and all 5 died; postmortem examination confirmed the presence of severe pulmonary vascular disease. The concept of the "palliative Mustard" procedure was applied in two children with transposition of the great arteries, intact ventricular spetum and pulmonary vascular disease who underwent the Mustard operation with creation of a ventricular septal defect. The postoperative course was uneventful in both patients. Thirteen and 5 months, respectively, after operation, both are physically active and have respective arterial oxygen saturation levels of 94 and 92 percent.- - - - - - - - - - ranking = 7keywords = vascular disease (Clic here for more details about this article) |
6/10. heart-lung transplantation: successful therapy for patients with pulmonary vascular disease.We report our initial experience with three patients who received heart-lung transplants. The primary immunosuppressive agent used was cyclosporin A, although conventional drugs were also administered. In the first patient, a 45-year-old woman with primary pulmonary hypertension, acute rejection of the transplant was diagnosed 10 and 25 days after surgery but was treated successfully; this patient still had normal exercise tolerance 10 months late. The second patient, a 30-year-old man, underwent transplantation for Eisenmenger's syndrome due to atrial and ventricular septal defects. His graft was not rejected, and his condition was markedly improved eight months after surgery. The third patient, a 29-year-old woman with transposition of the great vessels and associated defects, died four days postoperatively of renal, hepatic, and pulmonary complications. We attribute our success to experience with heart-lung transplantation in primates, to the use of cyclosporin A, and to the anatomic and physiologic advantages of combined heart-lung replacement. We hope that such transplants may ultimately provide an improved outlook for selected terminally ill patients with pulmonary vascular disease and certain other intractable cardiopulmonary disorders.- - - - - - - - - - ranking = 5keywords = vascular disease (Clic here for more details about this article) |
7/10. Management of transposition after the baffes procedure: a case report and review of our experience.A case report details the operative technique used in the repair of complete transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis in a patient who had had Baffes procedure previously. Experience in managing 10 patients with complete TGA who had undergone a Baffes procedure is discussed. Four patients with a large VSD had pulmonary vascular disease. The condition of 1 of them was improved by a palliative Mustard operation. Four children with isolated TGA underwent a successful modified Mustard repair. The 2 remaining patients had a VSD and pulmonary stenosis; in 1 the condition was palliated by a Glenn shunt. The other is the subject of the case report. The mean interval between the Baffes procedure and the second operation was 11 years. There was 1 late death after secondary repair. Follow-up in the remaining 6 patients at a mean of 10.6 years indicates a favorable outcome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/10. Pulmonary vascular disease in neonates with transposition of the great arteries and intact ventricular septum.BACKGROUND--Progressive pulmonary vascular disease in surgically unrepaired transposition of the great arteries with or without ventricular septal defect had been frequently described in the past. Occurrence of progressive pulmonary vascular disease has been reported even after atrial switch procedure done at three months of age. With the advent of neonatal surgical repair, this problem is virtually non-existent. There is a small subgroup of infants with transposition of the great arteries who show pulmonary vascular disease in the neonatal period that can adversely affect the surgical outcome. The clinico-pathological correlation in this group of patients was studied. OBSERVATIONS--Three patients, with transposition of the great arteries and intact ventricular septum, who showed histological evidence of pulmonary vascular disease in the neonatal period or early infancy are described. Two of these patients, continued to have poor systemic oxygenation despite adequate atrial communication. One patient had a close ductus arteriosus within the first two hours of birth while on prostaglandin E1 infusion. CONCLUSIONS--In the absence of left ventricular outflow tract obstruction, a poor response to atrial septostomy suggests pulmonary hypertension and pulmonary vascular disease. Antenatal constriction of the ductus arteriosus may contribute to such changes in pulmonary vasculature.- - - - - - - - - - ranking = 9keywords = vascular disease (Clic here for more details about this article) |
9/10. Unusual longevity without surgical intervention in complete transposition of the great arteries.A case of unusual longevity to the age of 58 years is reported for a female patient with complete transposition of the great arteries. The association with a wide atrial septal defect with intact interventricular septum may have contributed to the long survival without surgery. Factors determining intercirculatory mixing and systemic oxygen saturation may be the high pulmonary flow, the location of the anatomic communication, sufficient hemoglobin concentration to allow an adequate level of systemic resistance and recirculated systemic flow, and the belated development of pulmonary vascular disease.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
10/10. Development of advanced pulmonary vascular disease in D-transposition of the great arteries after the neonatal arterial switch operation.We report the case of a neonate with D-transposition of the great arteries who, after undergoing an uneventful arterial switch operation at the age of 4 days, was found at the age of 42 months to have developed advanced pulmonary vascular disease. Because the arterial switch operation was performed when our patient was only 4 days old, this case challenges the hypothesis that postnatal hemodynamics alone dictate the development of advanced pulmonary vascular disease in infants and children with transposition of the great arteries.- - - - - - - - - - ranking = 6keywords = vascular disease (Clic here for more details about this article) |
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