Martha A. Carpenter

Incidence and risk of reintervention after coarctation repair

We examined the need for intervention after coarctation repair in a retrospective study of 197 procedures performed between 1967 and 1989. Reintervention was required in 23 patients. No technique of coarctation repair was free from complications. Although there were only two stenoses in the group receiving Dacron patch angioplasty, only seven of these procedures were performed in children under the age of 1 year. The risk of stenosis was inversely correlated to the age at primary repair, with children less than 1 year old being at greater risk than those more than 1 year of age (p less than 0.05). Subclavian flap angioplasty had a lower risk of reoperation than end-to-end anastomosis (p less than 0.02). Formation of true aneurysms was confined to the Dacron patch angioplasty group. The morbidity and mortality for reintervention was low in all groups, with only one procedure-related death and no incidence of paraplegia. Although no technique is free from risk, subclavian flap angioplasty leads to fewer reinterventions in younger patients.

Left ventricular hyperkinesia at rest and during exercise in normotensive patients 2 to 27 years after coarctation repair

The short- and long-term results of effective surgical repair of coarctation of the aorta on left ventricular mass and function in 48 patients were evaluated using echocardiography and stress-gated radionuclide angiography. Thirty-two of the 48 patients who had no additional cardiac problems and had technically adequate radionuclide angiograms form the basis for this report. Among these, three had mild systolic hypertension and none had significant aortic valve dysfunction. Age at the time of study ranged from 6.5 to 59 years (mean 27). Age at the time of surgery ranged from 3 months to 34 years (mean 12 years). Duration from surgery to the time of noninvasive study ranged from 2 to 29 years (mean 15). In the 32 patients, left ventricular mass was 120 +/- 20 g/m2, compared with a control value of 87 +/- 10 g/m2. Mean left ventricular ejection fraction was elevated to 69.2 +/- 1.6% at rest (control 60 +/- 1.3%) and 78.8 +/- 1.3% during exercise (control 70 +/- 1.7%) (p less than 0.01). The systolic ejection rate was significantly increased (p less than 0.01) and end-systolic volume significantly decreased (p less than 0.01) compared with values in control patients. There was no correlation between ejection fraction and either age at the time of surgery or years since surgery. These findings of hyperdynamic left ventricular function and increased left ventricular mass without apparent cause many years after coarctation repair raise important questions as to mechanisms, extension to other forms of afterload stress that have been surgically or medically relieved and long-term outcome.

Aortic aneurysm after patch angioplasty for aortic isthmic coarctation in childhood.

Abstract Repair of aortic coarctation, pioneered by Craaford and Nylin 1 and Gross and Hufnagel 2 in 1944, is an accepted procedure with relatively low morbidity and mortality rates. Repair was initially accomplished by excision of the aortic coarctation with end-to-end anastomosis of healthy aortic tissue. Newer techniques, such as patch angioplasty 3–5 and subclavian flap angioplasty, 6 were introduced to avoid the complications of stricture at anastomotic site and allow for further growth of the aorta. Reports of aneurysm formation in the region of the patch angioplasty 7–9 have raised concern as to long-term safety of this procedure. Development of a large true aneurysm of the aorta in 2 asymptomatic patients (Fig. 1) who underwent primary repair of coarctation by the technique of patch angioplasty (the subject of a previous report 10 ) prompted recall of all patients who underwent repair of coarctation of the aorta at the University of Virginia Hospital since 1971. This study assesses the incidence of aortic aneurysm in the pericoarctation region after surgical repair of coarctation of the aorta in childhood, and attempts to determine if the presence of aneurysmal dilatation was related to the type of operative procedure, age at operation or presence of residual gradient.

Pleuroperitoneal shunts for refractory chylothorax after operation for congenital heart disease

Between 1980 and 1990, 10 of 12 children with a symptomatic chylothorax after operation for congenital heart disease failed to respond to traditional medical therapy (thoracentesis, tube thoracostomy, low-fat diet). All 10 patients underwent placement of a pleuroperitoneal shunt, with complete resolution of the chylothorax in 9 patients (90%). Cardiac catheterization, performed before placement of the pleuroperitoneal shunt in 5 patients, demonstrated elevated right atrial pressure in all patients (range, 10 to 25 mm Hg). The pleuroperitoneal shunt functioned effectively in 4 patients with moderately elevated right atrial pressures (range, 10 to 16 mm Hg; median, 13.5 mm Hg) but not in 1 patient with a right atrial pressure of 25 mm Hg. Pleuroperitoneal shunting as treatment for chylothorax after operation for congenital heart disease is safe and effective, even in the face of moderate elevations in right atrial pressure.

Baffle Obstruction Following the Mustard Operation: Cause and Treatment

Baffle obstruction developed in 11 patients after they had undergone the Mustard procedure. Eight of them required operative revision. The cause of the baffle obstruction seemed to be related to patient age (less than 1 year) and to the use of Dacron for the baffle but not to the shape of the baffle. A technique of revision that involves widely opening the previously placed baffle and enlarging it and the atriotomy with polytetrafluoroethylene was employed for the last 6 patients. All 5 survivors of this operation had good long-term results without recurrence.

Treatment of absent pulmonary valve syndrome with homograft.

We report the successful treatment of absent pulmonary valve syndrome in an infant. The treatment consisted of intracardiac repair of pulmonary regurgitation with a homograft valve, which allowed for early extubation and survival.

Mitral stenosis associated with partial anomalous pulmonary venous return (with intact atrial septum). An unsolved question.

Abstract In a patient with severe mitral stenosis, intact atrial septum and venous drainage of the left lung into the left innominate vein, increased values for pulmonary capillary wedge pressure and flow in the abnormally draining left lung equaled those found in the normally draining right lung. The possible mechanisms for this discrepancy are discussed. Total surgical correction of these lesions was successful.

Aortic aneurysm after patch aortoplasty for coarctation in childhood

Abstract In 2 patients who had previously undergone patch aortoplasty for repair of coarctation of the aorta (CoA), progressive aneurysmal dilatation of the aorta developed at the site of the repair. Both patients underwent operative resection of the aneurysm.

Ductal origin of the left pulmonary artery in severe tetralogy of Fallot: Problems in management

SummaryCardiac catheterization in a neonate demonstrated tetralogy of Fallot and absence of anatomic origin of the left pulmonary artery from the main pulmonary artery. A central aortopulmonary shunt was performed in order to increase pulmonary blood flow. Because of the concern that the left pulmonary artery was actually being supplied by a ductus arteriosus, repeat catheterization was performed and this revealed closure of the ductus arteriosus with obliteration of arterial supply to the left pulmonary artery. A left Blalock-Taussig shunt was subsequently performed and this reestablished blood supply to the left pulmonary artery. This case report describes problems in management of congenital heart disease with absence of anatomic origin of the left pulmonary artery from the main pulmonary artery.

Congenital absence of the pulmonary valve.

Congenital absence of the pumonary valve (CAPV) is a rare anomaly usually associated with ventricular septal defect or Fallots tetralogy. CAPV may be rapidly fatal in infancy because of severe disturbance of pulmonary ventilation resulting from bronchial compression by massively dilated pulmonary arteries. Other cases may be relatively benign. Our group of five cases with CAPV ranged from the severe to one case in which there was documented closure of a patent ductus artierosus and a ventricular septal defect, leaving the CAPV as an apparently isolated, well-tolerated lesion. Plain chest radiographs play a major part in the diagnosis of CAPV since they almost invariably give some indication of the aneurysmal dilatation of pulmonary arteries. On angiography, dilated main pulmonary arteries are shown giving rise to branches of normal or even diminished caliber.