One- to ten-year follow-up results of balloon angioplasty of native coarctation of the aorta in adolescents and adults.

OBJECTIVES: We attempted to evaluate the role of balloon angioplasty in the treatment of discrete coarctation of the aorta in adolescents and adults, with special emphasis on long-term results. BACKGROUND: Controversy persists over the use of balloon dilation for the treatment of native coarctation of the aorta. METHODS: Between July 1986 and January 1997, 43 consecutive adolescent and adult patients with discrete coarctation of the aorta underwent balloon angioplasty. One- to 10-year follow-up data of 37 patients, including results of cardiac catheterization and magnetic resonance imaging (MRI), form the basis of this study. RESULTS: No early or late deaths occurred. Balloon angioplasty produced a reduction in the peak to peak coarctation gradient from a mean +/- SD of 69 +/- 24 mm Hg (95% confidence interval [CI] 61 to 76) to 12 +/- 8 mm Hg (95% CI 10 to 14.8) (p < 0.001). Follow-up catheterization 12 months later (37 patients) revealed a residual gradient of 6.7 +/- 6 mm Hg (95% CI 4.6 to 8.9); 3 (7%) of 43 patients had suboptimal results with development of recoarctation, defined as peak gradient >20 mm Hg, with successful repeat angioplasty. A small aneurysm developed at the site of dilation in 3 (7%) of the 43 patients. MRI follow-up data 1 to 10.8 years (mean 5.2 +/- 2.7) after angioplasty (37 patients) revealed no new aneurysm or appreciable change in the size of the preexisting aneurysm in the three patients. The blood pressure had normalized without medication in 27 (73%) of 37 patients at follow-up examination. CONCLUSIONS: Balloon angioplasty is safe and effective and should be considered a viable alternative to operation for treatment of discrete coarctation of the aorta in adolescents and adults.

Consanguinity and congenital heart disease in Saudi Arabia.

First-cousin marriage may be a significant risk factor for specific types of congenital heart disease in a consanguineous population. Inbreeding studies suggest an autosomal recessive component in the cause of some congenital heart defects. We studied a large sample of patients with structural congenital heart defects (CHD) identified through the Congenital Heart Disease Registry at King Faisal Specialist Hospital in Riyadh, Saudi Arabia. After exclusions of chromosome abnormalities and non-participation, data were collected on 891 consecutive patients who were registered between January and August, 1998. Data on first-cousin consanguinity and type of CHD diagnosis were collected. A z test of proportions was used to determine the association between consanguinity and subtypes of CHD. Data indicate that the proportion of first cousins in the CHD sample is higher than the proportion in the general population, supporting a hypothesis of autosomal recessive gene involvement in congenital heart disease. When subgroups of CHD were analyzed, first-cousin consanguinity was significantly associated with ventricular septal defect (VSD), atrial septal defect (ASD), atrioventricular septal defect (AVSD), pulmonary stenosis (PS), and pulmonary atresia (PA). There was no relationship between consanguinity and tetralogy of Fallot (TOF), tricuspid atresia (TA), aortic stenosis (AS), co-arctation of the aorta (CoA), and patent ductus arteriosus (PDA). Thus, in a population with a high degree of inbreeding, consanguinity may exacerbate underlying genetic risk factors, particularly in the offspring of first cousins. There may be a recessive component in the causation of some cardiac defects.

Thrombotic obstruction of bileaflet valves: surgical management and fiberoptic thrombectomy.

Three patients underwent emergency operation for thrombotic obstruction of a bileaflet mechanical prosthesis (two St. Jude and one Duromedics) in the mitral position. The three valves were successfully thromboectomized with return to normal function. In 2 patients removal of the thrombus at the valve hinges was assisted by the use of a flexible fiberoptic choledochoscope. All 3 patients remain well 2 years after the procedure, maintained on a regimen of warfarin and dipyridamole.

Extracardiac right atrium-to-right ventricle homograft for uncorrectable tricuspid valve disease.

Surgically uncorrectable tricuspid valve disease in children is rare. However, when it happens the surgical options are very limited. Tricuspid valve replacement using a mechanical valve or stented bioprosthesis is impractical. Use of homografts in the "anatomic position" has its limitations. We report here the use of an extracardiac homograft connection between the right atrium and right ventricle in a 16-month-old boy in whom severe tricuspid valve stenosis developed after surgical repair of a complex ventricular septal defect associated with dextrocardia and anomalous systemic venous drainage. The patient remains well receiving no cardiac medication 12 months after the procedure.

Reconstruction of supravalvar aortic stenosis with autologous pulmonary artery.

Use of autologous tissue in corrective cardiac operations offers many advantages including the potential for growth. We report a surgical technique using autologous pulmonary artery in the repair of supravalvar aortic stenosis in a 6-year-old child. At 30 months' follow-up, the pulmonary arterial tissue shows no evidence of calcification or dilatation and appears to be growing with the aorta.

Treated bovine and autologous pericardium for aortic valve reconstruction.

BACKGROUND: To determine the differences in clinical behavior of bovine versus autologous pericardium, all consecutive patients undergoing aortic valve reconstruction were reviewed. METHODS: Between October 1988 and December 1995, 91 patients (mean age 30 years) underwent reconstruction with bovine (n = 27) or autologous (n = 64) pericardium. RESULTS: There were 2 hospital deaths, 5 late deaths, and no embolic events. Dysfunction of the aortic reconstruction required reoperation in 6 bovine (infection 1, fibrocalcific 5) and in 5 autologous (infection 3, annulus dilatation 1, commissural tear 1). Actuarial survival and freedom from structural deterioration at 8 years were 82.2%+/-9.6% and 76.2%+/-10.7% for bovine and 91.05%+/-3.96% and 96.8%+/-2.25% for autologous pericardium, respectively. The last Doppler echocardiographic study showed a mean regurgitation (1 to 4+) and gradient in the bovine pericardium of 1.25+/-and 20.7 mm Hg and in the autologous pericardium of 1+ and 7.7 mm Hg. CONCLUSIONS: Aortic valve reconstruction with pericardium can be safely performed with low thromboembolic rate. At 8 years follow up, there is a difference in favor of the autologous pericardium.

Performance of 96 CarboMedics valve replacements in 75 patients less than twenty-one years of age.

Valve replacement still represents a problem in the very young patient. Between July 1988 and November 1993, 96 CarboMedics mechanical valves were implanted in 75 patients with a mean age of 11.76 years (range, 5 months to 20 years). The mean preoperative New York Heart Association functional class was 3.2, and 89.3% of the patients were in sinus rhythm. The cause was rheumatic in 60%, congenital in 24%, and infective in 12%. Mitral valve replacement was undertaken in 43 patients, aortic in 11, and mitroaortic in 21. Among the 18 patients with congenital defects, 13 required simultaneous repair of their complex lesions. The hospital mortality was 12% (9 patients). Cause and age were significant factors responsible for mortality. The mortality was 27.8% for patients with congenital disease and 6.7% for those with rheumatic defects (p = 0.0365); it was 40% for patients younger than 2 years, 14.3% for those between 3 and 10 years old, and 5.9% for those older than 10 years (p = 0.0108). The maximum follow-up was 54 months (mean, 18 months). There were 10 late deaths (15.15%). No embolic events occurred. All patients were on anticoagulation therapy except 2 who were on antiaggregant therapy. One of them underwent successful reoperation for treatment of mitral prosthetic thrombosis. Three reoperations were performed: one for thrombosis, one for perivalvular leak, and one for endocarditis. The total actuarial survival was 68.19% +/- 7.02%. Freedom from embolism was 100%; from thrombosis, 96.72% +/- 3.22%; from severe hemorrhage, 94.94% +/- 3.67%, and from reoperation, 83.07% +/- 9.51%.

Aortic translocation and biventricular outflow tract reconstruction in an infant.

Successful surgical repair of an 8 1/2-month-old infant weighing 8 kg with d-transposition of the great arteries associated with ventricular septal defect and left ventricular outflow tract obstruction was accomplished utilizing a recently described technique. To our knowledge, this is the first attempt to use this technique in an infant.

New surgical technique for type B aortic interruption.

Restoring continuity of the aortic arch in aortic interruption continues to be a problem in terms of both surgical technique and long-term results. We report here a surgical technique using the left subclavian artery along with an aberrant right subclavian artery to form a conduit in a patient with type B interruption, to reestablish aortic continuity.

Pulmonary autograft for aortic valve replacement in rheumatic disease: a caveat.

Pulmonary autograft replacement of the aortic valve offers an attractive option in the younger patient with growth potential and long-term survival. In our institution between January 1990 and August 1994, 78 patients have undergone this procedure. The mean age was 18.6 +/- 7.36 years (range, 1 to 41 years). The etiology was rheumatic in 63 patients (80.7%). Aortic regurgitation was the predominant lesion in 60 patients (76.9%). Significant mitral regurgitation requiring operation was present in 22 patients (28.2%). All patients underwent pulmonary autograft replacement of the diseased aortic valve and the mitral valve was repaired in 22 patients. There were no hospital mortality, endocarditis, or thromboembolism in the series up to date. There have been two late non-cardiac deaths. Five patients (6.4%) required reoperation, one for mitral repair failure and four for autograft failure. Acute rheumatic valvulitis was demonstrated in one of the reoperated patients. Echocardiography of 68 patients followed up more than 2 months show progression of aortic regurgitation more than 2/4+ in 12 patients (15.4%). Four of these patients have been reoperated without mortality. In conclusion, although the Ross procedure remains a safe and attractive alternative in aortic valve operation, the progression of aortic regurgitation, especially in the younger patient with rheumatic etiology, remains a concern.

Unstented semilunar homograft replacement of tricuspid valve in Ebstein's malformation.

Tricuspid valve pathology in Ebstein's malformation requires replacement when it is not possible to repair or reconstruct this valve. In smaller children, in whom the right-sided atrioventricular valve is severely dysplastic and right ventricular volume is prohibitive, prosthetic replacement is not always possible. We report here on 3 patients who underwent stentless semilunar homograft replacement (top-hat procedure) of tricuspid valve for Ebstein's anomaly with good short-term outcome. This provides an attractive alternative in the management of a certain difficult subset of patients, avoids long term anticoagulation and probably is more durable.

Aortic translocation for D-TGA associated with LVOTO and VSD.

Aortic translocation is a useful surgical option in certain difficult subsets of transposition of great arteries with ventricular septal defect and left ventricular outflow tract obstruction. We report here the use of this technique with pulmonary homograft reconstruction of right ventricular pulmonary artery continuity in a child with transposition of the great arteries, left ventricular outflow tract obstruction, and restrictive ventricular septal defect.

Aneurysms complicating pulmonary autograft procedure for aortic valve replacement.

Aneurysm formation in the left ventricular outflow tract related to the proximal end of the pulmonary autograft after the Ross procedure was present in 2 patients. Both occurred late after operation and were associated with prolapse of a leaflet of the autograft and significant regurgitation. Both were repaired with no immediate complications. There was no evidence of infection at time of operation. The probable mechanisms underlying this complication and the possibilities of avoiding it are discussed.

Triple-valve operation in the young rheumatic patient.

Multiple-valve operation for the young rheumatic patient remains a problem. There is a paucity of information on the results of repair versus replacement in this age group. Between July 1988 and December 1991, 242 patients less than 20 years of age underwent a valve operation for rheumatic heart disease at our institution. Twenty-four (9.9%) of them had simultaneous mitral, aortic, and tricuspid valve procedures. The mean age was 14.71 years. All 24 patients were in functional class III or IV preoperatively. Valvar regurgitation was the predominant lesion. Four patients (16.7%) had active rheumatic myocarditis at the time of operation. Valve repair was attempted in the absence of infective endocarditis. Triple-valve repair was possible in 12 patients (50%). The hospital mortality rate was 16.7%. Reoperation was performed after repair in 9 patients (45%) without any deaths. The reason for reoperation was failure of the mitral valve repair in all patients, and the cause was technical in 3 patients, progression or recurrence of rheumatic myocarditis in 5, and endocarditis in 1 patient. The three late deaths (15%) were in patients who had mitral valve replacement. Valve repair was associated with a higher reoperation rate, and replacement of left-sided valves was associated with a higher early and late mortality. In conclusion, although valve repair would be ideal in the young rheumatic patient, multiple-valve repair is associated with a high reoperation rate.

The role of surgical ligation of patent ductus arteriosus in the era of the Rashkind device.

BACKGROUND: The role of surgery in managing patent ductus arteriosus (PDA) was studied in the era of the Rashkind double-umbrella device. METHODS: All 354 patients with PDA referred to our center in a 5-year period were included in this report. Of the 354 patients, 236 underwent cardiac catheterization with the intent of transcatheter PDA closure, and 118 had surgical intervention. RESULTS: In 46 (19.5%) of the 236 patients having cardiac catheterization, the procedure either was abandoned or failed. Color Doppler echocardiography demonstrated total occlusion of the ductus after 24 hours in 97 patients (41%) in the cardiac catheterization group. An additional 20 patients had no residual leaks at follow-up. Twenty other patients underwent reocclusion because of a residual shunt. Thus, of the 236 patients, 137 (58%) had successful complete closure of the PDA. Surgical PDA ligation was performed in 118 patients as the initial procedure and in 26 of the 46 patients in whom transcatheter closure was abandoned. If the remaining 20 patients in whom transcatheter closure failed are added to the 144 patients who underwent PDA ligation, the percentage having surgical intervention versus transcatheter occlusion is higher than 46%. CONCLUSIONS: Our data suggest that surgery plays a major role in the management of patients with PDA despite the advent of new interventional catheterization techniques.

Indications and limitations of aortic valve reconstruction.

To elucidate the value of conservative operation for aortic regurgitation, all consecutive patients operated on between July 1988 and July 1990 were reviewed. Of 251 patients with aortic regurgitation, 107 (42.6%) had nonprosthetic operation. The mean age was 23 years, and 90 patients (84.1%) were rheumatic. Two techniques were used: repair (annular and leaflet plasties, 69 cases) and cusp extension with glutaraldehyde-treated pericardium (25 bovine, 13 autologous). There were two hospital deaths (1.8%), both in the repair group, and no late deaths or embolic events. Only 5 patients (4.7%) were anticoagulated. In the repair group there were 12 reoperations, four (5.9%) due to aortic and eight to mitral dysfunction. In the cusp extension group there were two reoperations due to mitral dysfunction. Echocardiographic follow-up showed better results with cusp extension. In conclusion, conservative operation for aortic regurgitation is possible in a high percentage of young rheumatic patients and does not require anticoagulation. Cusp extension is more reliable than repair in terms of early results, although its long-term durability is not yet known.

Total correction of transposition of great arteries with atrioventricular septal defect.

A successful anatomical correction of transposition of the great arteries with an atrioventricular septal defect is reported. This combination of anomalies is rare, and the anatomical correction is unusual in that the operation involves all four heart valves.

Intracardiac rhabdomyosarcoma: transesophageal echocardiographic findings and diagnosis.

Transesophageal echocardiography (TEE) was performed on a 41-year-old woman who presented with a cerebrovascular accident. TEE confirmed the presence of a morphologically bizarre biatrial tumor with precarious, vigorous motion throughout the cardiac cycle. Surgical intervention was decided on, and the patient underwent cardiac surgery for tumor excision 16 hours after TEE. Intraoperative frozen section diagnosis was spindle cell sarcoma, and subsequent immunohistochemical analysis showed the tumor to be a rhabdomyosarcoma. The data are presented here, and the role of TEE to establish a preoperative diagnosis of intracardiac tumor is discussed.

Reopening of the arterial duct after balloon dilatation of native coarctation.

We report an unusual complication of balloon dilatation. In an 8-year-old boy, the arterial duct reopened after balloon dilatation of native coarctation.

Left internal thoracic artery bypass graft in arterial switch with coronary transfer for transposition of the great arteries.

The left internal thoracic artery was anastomosed to the circumflex coronary artery to treat myocardial ischemia during an arterial switch procedure for transposition of the great arteries in a 6-month-old infant. Follow-up angiography revealed complete patency of the anastomosis.