Coronary-bronchial artery fistula with partial absence of a pulmonary artery: association with partial anomalous pulmonary venous drainage.

A patient is presented with partial absence of the right pulmonary artery, left coronary artery to bronchial artery fistula and systemic arterial desaturation with a patent foramen ovale. The various aspects of the syndrome are discussed and the rationale for nonoperative closure of the foramen is described.

Oral hydralazine in patients with pulmonary vascular disease secondary to congenital heart disease.

Two patients with pulmonary vascular obstructive disease secondary to congenital heart disease were evaluated hemodynamically before and during oral hydralazine therapy. Both patients were assessed postoperatively and had no significant residual shunts. Pulmonary vascular resistance failed to decrease, and an increase in pulmonary arterial pressure occurred because of increased cardiac output secondary to systemic arteriolar dilatation. These responses were sufficiently consistent to warrant a warning against the use of oral hydralazine except under controlled conditions in patients with secondary pulmonary vascular obstructive disease.

Ascending aorta-right pulmonary artery anastomosis. Long-term results in 137 patients with cyanotic congenital heart disease.

Long-term follow-up of 137 patients who had an ascending aorta-right pulmonary artery anastomosis at the Children's Hospital of Philadelphia between 1966 and 1975 is presented. One hundred four patients survived the first hospitalization. Of the 81 patients currently alive, 30 have had a succesful corrective. operation. Nine patients died at the time of correction. Fifty patients have adequate pallation. Of the 56 deaths, only nine were shunt related. Late complications in the 104 first admission survivors included congestive heart failure (23%), pulmonary artery hypertension (7%), pulmonary vascular obstructive disease (1%), and shunt failure (9%). Special catheterization techniques to evaluate the shunt and its effect on the pulmonary arteries prior to surgical correction are described. The ascending aorta-right pulmonary artery anastomosis can provide effective palliation without interfering with subsequent corrective operations.

Coarctation resection in children with Turner's syndrome: a note of caution.

Eight children were recognized to have Turner's syndrome, among 353 patients over 1 year of age who had undergone surgical treatment for coarctation of the aorta. Of these eight children, three developed a significant perioperative hemorrhage from aortic rupture, resulting in one death and one instance of paraparesis related to a period of prolonged hypotension. In two of the other five patients with Turner's syndrome, a decision was made to perform an angioplasty rather than a resection of the coarctation because of apparent friability of the aortic wall. In contrast, only one of the 345 patients without Turner's syndrome died as a result of surgical treatment, and none developed spontaneous perioperative aortic rupture or neurologic deficit. This experience suggests that the operative risk for coarctation of the aorta in this subgroup of patients is considerably greater than that in patients without Turner's syndrome (p < 0.001). Special precautions should include use of rubber-jaw vascular clamps, choice of technique to avoid tension at the anastomotic suture line, and careful control of systemic blood pressure intraoperatively and postoperatively. Indications for surgical treatment of coarctation as well as the type of operative procedure must be individualized cautiously in patients with Turner's syndrome.

Replacement of ventricular myocardium with diaphragmatic skeletal muscle: short-term studies.

A pedicled diaphragmatic skeletal muscle graft was used to replace a portion of resected right ventricle in 35 dogs. The graft contracted when electrically stimulated directly or via the phrenic nerve before and after insertion. The electrical pacing threshold was lower for phrenic nerve stimulation (0.9 +/- 0.20 mamp) than for direct graft stimulation (2.3 +/- 1.19 mamp). The heart could be captured and paced by stimulating the muscle graft with higher current (16.2 +/- 4.49 mamp). The delay from pacing stimulus to muscle graft contraction when the graft was paced directly was 10 msec. The epicardial activation time delay when the heart was paced through the muscle graft was 27.0 +/- 9.08 msec. When the muscle graft pedicle was transected, the graft lost its ability to contract. The heart, however, could still be captured electrically through the graft for up to 4 hours. Strain gauge studies of the nonstimulated muscle graft showed tension development during pre-ejection ventricular contraction identical to that of the right ventricle. In the ejection phase, muscle graft tension slowly declined. The stimulated muscle graft developed active tension and echographically demonstrated muscle thickening during contraction. This study demonstrates that a vascularized, neurally innervated diaphragmatic muscle graft can be placed into the right ventricle. The graft retains its ability to contract in response to direct or phrenic nerve stimulation. It can be made to contract during any phase of the cardiac cycle. Thus diaphragmatic muscle grafts may provide a method to augment ventricular cavity size with synchronously contracting muscle.

Transatrial repair of tetralogy of Fallot.

In 12 months since March, 1975, 25 of 27 patients with tetralogy of Fallot have had corrective operations without ventriculotomy. Infundibular obstructions were excised and ventricular septal defects were closed through a right artiotomy with retraction of the anterior leaflet of the tricuspid valve. Pulmonary valve stenosis was relieved through a pulmonary arteriotomy. In five patients the pulmonary annulus was patched 0.5 to 1.5 cm. into the right ventricle. Immediately after repair peak right ventricular-pulmonary arterial systolic pressure difference averaged 17 mm. Hg and ranged between zero and 40 mm. Hg. Cardiac indices averaged 2.85 L. per square meter per minute 4 hours after operation. All but two patients developed right bundle branch block. One patient with severe pulmonary hypertension died. Fourteen patients have been recatheterized. Right ventricular-pulmonary peak systolic pressure differences ranged between zero and 45 mm. Hg (mean, 22). Cineangiograms show contraction of the free right ventricular wall during systole. Transatrial repair of tetralogy of Fallot is feasible technically in many patients, avoids muscle necrosis and coronary arterial injury, and improves cardiac output in the immediate postoperative period.

Congenital left aortic sinus-left ventricle fistula and review of aortocardiac fistulas.

A fistula from the left aortic sinus to the left ventricle is reported that was successfully repaired using cardiopulmonary bypass. In addition, 175 cases of fistula to the heart from the aortic sinuses published in the English literature from 1839 through 1972 were studied, with particular emphasis on the 126 patients who underwent operative repair. The major cause of the fistulas (76%) was found to be rupture of a congenital aortic sinus aneurysm, usually during the third or fourth decade of life. The remainder of these fistulas to the heart consisted of simply a tract in an otherwise normal sinus. Ventricular septal defect was the most common associated defect and, when present, was nearly always related to a fistula arising from the right aortic sinus. Repair was successful in 86% of the 126 operated patients. The principles of operative treatment of these fistulas are reviewed.

Interventional cardiac catheterization in congenital heart disease.

Cardiac catheterization has proved its value as a major tool in the diagnosis of congenital cardiac defects. The advent of non-invasive imaging of various sorts has altered the role of diagnostic catheterization. Within the past two decades cardiac catheter instruments to provide therapy have been applied to many lesions. Improvements in design and methods will expand the use of therapeutic catheterization. It is inevitable that better results will be obtained for those defects currently being treated that way, and that the method will be applied to other conditions.

Fistula from left main coronary artery to right atrium: treatment by combined transaortic and transatrial approach.

Fistulas from the left main coronary artery to the right atrium are unusual lesions that have been treated by fistula ligation. The present report of a case treated by a combined transaortic and transatrial approach stresses the ease and advantages of this technique.

Historical aspects of interventional cardiology: past, present and future.

The purpose of this presentation is to report the progress of two interventional catheter techniques that have occupied my attention for the last 10 years; namely, transcatheter patent ductus arteriosus occlusion and patch atrial septal defect closure. A brief survey of the aspects of interventional cardiology, including its past, present and future, will also be included.

Palliative procedures for transposition of the great arteries.

Palliation in uncomplicated transposition of the great arteries is a matter of achieving increased intracardiac mixing. This can be done by surgical atrioseptectomy or by balloon atrioseptostomy. The latter technique has been proved to be a safe and effective procedure for long-term palliation. In the presence of complicating lesions adjustments of the pulmonary blood flow must be made. When the complicating lesion is a ventricular septal defect with increased pulmonary blood flow, pulmonary artery banding is often required. In the presence of severe pulmonic stenosis with a ventricular septal defect, aorticopulmonary shunting may be required. Judicious application of these palliative procedures has completely altered the outlook of the infant born with transposed great arteries, with or without complicating lesions.

Interventional cardiac catheterization in congenital heart disease.

Cardiac catheterization has proved its value as a major tool in the diagnosis of congenital cardiac defects. The advent of noninvasive imaging of various sorts has altered the role of diagnostic catheterization. Within the past two decades, cardiac catheter instruments to provide therapy have been applied to many lesions. Improvements in design and methods will expand the use of therapeutic catheterization. It is inevitable that better results will be obtained for those defects currently being treated that way, and undoubtedly the method will be applied to other conditions. These developments should continue to cement the relationship between pediatric cardiologists and cardiovascular surgeons. As balloon atrioseptostomy led to more "corrective" operations for transposition patients, so these new techniques will lead to less frequent use of that regrettable term, inoperable.