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Pulmonary valve replacement for regurgitation after repair of tetralogy of Fallot.

In general, it has been thought that pulmonary valve insufficiency is well tolerated when the valve is excised or when the pulmonary annulus has been widened with an outflow patch during repair of tetralogy of Fallot. However, when pulmonary regurgitation is massive or when it is combined with other causes of right ventricular failure, progressive right ventricular dilation may occur in some patients. Pulmonary valve replacement has not been commonly used in the past. From January, 1980, to August, 1982, 12 patients, 11 months to 17 years old, had pulmonary regurgitation treated by insertion of a valve in the pulmonary position 4 1/2 months to 11 years after initial repair of tetralogy of Fallot. All patients had progressive right ventricular failure not responsive to medical management. There were no major outflow tract obstructions, residual ventricular septal defects, or persistent aortopulmonary shunts. All 12 patients underwent patch reconstruction of the right ventricular outflow tract that allowed placement of a larger valve. There have been no operative or late deaths, and each patient has had improvement in functional status. One patient required tricuspid valve replacement 1 1/2 years after pulmonary valve replacement to achieve sustained relief of symptoms. Only 1 other patient required subsequent operation; this was for pacemaker lead changes. These early results suggest that in patients with right ventricular failure, attention should be directed to pulmonary regurgitation since this is a component of failure that is reversible; pulmonary valve replacement carries a low risk, and it can relieve symptoms and prevent further deterioration of right ventricular function.

Adolescent↗

[Balloon dilatation of the pulmonary valve. Short-, middle- and long-term results].

BACKGROUND: Immediate and long-term results after balloon dilatation of pulmonary valve stenosis in our unit. METHODS AND PATIENTS: All 111 patients (1 day-18 years) who have had balloon dilatation of a pulmonary valvar stenosis between 12/1987 and 8/1997 were divided into 4 groups: Typical valvar pulmonary stenosis (group A; n = 78), stenosis with dysplastic pulmonary valve (group B; n = 10), critical pulmonary stenosis (group C; n = 16) and pulmonary atresia after transcatheter or operative opening of the valve (group D; n = 7). Patients with pulmonary stenosis and complex congenital heart disease were excluded. RESULTS: The average systolic transvalvular gradient was reduced from 68.5 to 27.2 mmHg (60%) immediately after balloon dilatation. After a follow up of 48.8 +/- 37 months 101 patients could be reevaluated. In group A (n = 69 at FU) and C (n = 16 at FU) 81% showed a systolic transvalvular gradient < 30 mmHg after one and 83% (A) respective 94% (C) after two balloon dilatations. In group B (n = 9 at FU) 44% exhibited a systolic gradient < 30 mmHg after one and 56% after two balloon dilatations. In group D (n = 7 at FU) 57% showed a systolic gradient < 30 mmHg with no further improvement by a second dilatation. Over all, 80% of our patients could be treated sufficiently by transcatheter means. The rate of major complications was 7.3% with no lasting residuals at follow up and no deaths. CONCLUSION: Balloon dilatation of the pulmonary valve is secure and effective. Best results are obtained in patients with typical pulmonary valve stenosis and in newborns and infants with critical pulmonary valve stenosis.

Adolescent↗

Critical pulmonary valve stenosis in patients less than 1 year of age: treatment with percutaneous gradational balloon pulmonary valvuloplasty.

A new technique of percutaneous gradational balloon pulmonary valvuloplasty was used successfully in 17 infants less than 1 year of age, including four neonates, with critical congenital pulmonary valve stenosis, who would otherwise have required surgical intervention. This technique uses a sequential series of balloon catheters of gradually increasing size as a means of eventually crossing the very small valve orifice with a balloon catheter of adequate size to achieve successful pulmonary valvuloplasty. The initial dilatations were achieved with balloon catheters 2, 3, or 4 mm in diameter. Immediately after successful balloon valvuloplasty, the mean pulmonary systolic pressure gradient was reduced from 105 +/- 11 to 20 +/- 5 mm Hg. Percutaneous balloon pulmonary valvuloplasty can be performed safely with good results despite critical congenital pulmonary valve obstruction in severely ill infants.

Catheterization↗

Normal mean manifest electrical qRS in dysplastic pulmonary valve associated with hypertrophic non-obstructive left ventricular cardiomyopathy.

Dysplastic pulmonary valve is usually associated with extreme right axis deviation. A mean manifest electrical QRS axis of + 160 degrees or less was present in 11 of 30 cases of DPV studied by us. In 9 of these cases associated left ventricular hypertrophic non-obstructive cardiomyopathy was found. We suggest that a normal mean manifest electrical QRS axis in the presence of dysplastic stenotic pulmonary valve indicates the presence of associated cardiomyopathy.

Adolescent↗

Percutaneous transluminal balloon pulmonary valvuloplasty for the relief of pulmonary valve stenosis with special reference to double-balloon technique.

PTBPV was performed 33 times in 32 patients in the Riyadh Armed Forces Hospital. Patients ranged in age from 6 months to 12 years (mean 4.5 years); average weight was 14.86 kg. Before dilatation, all patients had grade 4/6 late peaking systolic ejection murmurs, with right-axis deviation and right ventricular hypertrophy on ECG. Moderate to severe pulmonary valve stenosis (right ventricle-pulmonary artery gradient 50 mm Hg systolic) was confirmed both hemodynamically and angiographically. Balloon size was selected to be approximately 2 mm larger than the pulmonary valve anulus was over 25 were inflated to approximately 5 atmospheres of pressure. Two patients required two simultaneous balloons as the pulmonary valve anulus was over 25 mm. Predilatation peak systolic pressure gradients ranged from 50 to 245 mm Hg (mean 99.2 mm Hg); postdilatation gradients ranged from 8 to 93 mm Hg (mean 23.4 mm Hg). There were no deaths or complications. The systolic murmur with early systolic peak decreased in all but three patients. Systolic thrill disappeared in all but three patients. Follow-up at an average of 10 months revealed increasing exercise tolerance in two thirds of patients. Fourteen patients have been recatheterized at least 6 months after dilatation and showed persistent minimal gradient. We conclude that balloon valvuloplasty of pulmonary valve stenosis is an effective, safe procedure. At this writing, a total of 90 successful PTBPVs have been performed, with equally impressive results.

Angioplasty, Balloon↗

Management of tetralogy of Fallot with absent pulmonary valve: early and mid-term results of a uniform approach.

The operative management of absent pulmonary valve syndrome remains controversial regarding palliative or one-stage correction, the need for pulmonary valve implantation and pulmonary arterioplasty. This retrospective report summarises the experience of a single centre with a view to provide some answers to this controversy. Forty-six consecutive patients including five infants, aged 2 months to 43 years, underwent primary surgical correction during the last 8.5 years. All the patients underwent two-dimensional echocardiography and cardiac catheterisation. Nine patients had mild and 10 moderate pulmonary artery hypertension. Repair consisted of patch closure of the ventricular septal defect and reconstruction of the right ventricular outflow tract. A valve was incorporated in the pulmonary position in 19 patients. Pulmonary arterioplasty was performed only in infants. Overall hospital mortality was 4 out of 46 patients (8.6%). Two out of five infants died accounting for 40 percent mortality. Forty-two survivors were followed up from 4 to 101 months; 40 patients are in functional class I and two in class II. Actuarial survival at 8.5 years was 91 percent. It is concluded that reconstruction of the right ventricular outflow tract with a transannular patch is sufficient in majority of patients. A selective approach to pulmonary valve insertion is recommended in patients with pulmonary hypertension or other anomalies. Pulmonary arterioplasty should be performed as the primary treatment in infants.

Adolescent↗

Severe congestive heart failure in mild pulmonic stenosis due to dysplastic pulmonary valve associated with cardiomyopathy.

Congestive heart failure has never been described in patients with dysplastic stenotic pulmonary valve without associated shunt lesions. We describe two patients with mild pulmonic stenosis due to valvular dysplasia associated with cardiomyopathy who developed severe congestive heart failure. Since the small pressure gradients across the pulmonary valve cannot cause this complication, we suggest that it resulted from the associated hypertrophic non-obstructive cardiomyopathy. The presence of cardiomyopathy may alter the clinical presentation and prognosis of patients with dysplastic pulmonary valve. In some cases, like our two cases, the cardiomyopathy rather than the valvular lesion should be considered the main disease.

Cardiomegaly↗

Pulmonary valve vegetations detected with echocardiography.

In a patient with pseudomonas endocarditis a pulmonary regurgitant murmur developed. Sequential echocardiography demonstrated the initial absence of vegetations, the evolution of pulmonary valve vegetations with relapse and finally the apperance of vegetations on all cardiac valves. The findings were confirmed at autopsy. Echocardiography, a useful technique for evaluating mitral, aortic and tricuspid vegetations, can also detect pulmonary valve vegetations.

Adult↗

Valve replacement in carcinoid syndrome. Anaesthetic management for tricuspid and pulmonary valve surgery.

This report describes the anaesthetic management of a patient with carcinoid syndrome for cardiac catheterisation followed by replacement of the tricuspid and pulmonary valves. Apart from the precautionary use of aprotinin and steroids, routine techniques of anaesthesia and monitoring were used without complications attributable to secretions from tumour tissue.

Anesthesia, General↗

Preservation of the pulmonary valve during intracardiac repair of tetralogy of Fallot.

From January 1981 to December 1990, intracardiac repair of tetralogy of Fallot in 148 pediatric patients, with one surgical death, was directed toward preservation of the native pulmonary valve. Using the accepted preoperative angiographic criterion for the pulmonary valve annulus area (PVA) of 1.8 cm2/m2, 85 patients were candidates for transannular right ventricular outflow patch (TAP). However, in 54 patients with a mean PVA of 1.5 cm2/m2 (range 1.06-1.76), the valve was preserved without using TAP because the morphological changes (cusp thickening and annular distensibility) seemed acceptable for preservation in view of its probable hemodynamic efficiency and growth potential. A morphological classification of pulmonary valve changes has evolved. Retrospectively, 24 (77%) of the 31 patients with TAP had moderate to severe cusp thickening and ring rigidity; this incidence was significantly higher (p < 0.001) than that in preserved patients (18 of 54 or 33%). The incidence of morphological changes increased with operative age; that is, 2 of 13 (15%) patients younger than 1 year versus 23 of 40 (58%) patients older than 4 years (p < 0.01). All 54 patients with preserved pulmonary valves were catheterized one month postoperatively. The intraoperative right to left ventricular systolic pressure ratio (RVP/LVP) decreased significantly (p < 0.005) in one month, from a mean of 0.79 (range 0.44-1.36) to 0.57 (range 0.36-0.97). The PVA increased from a mean of 1.5 to 1.9 cm2/m2 (range 1.20-2.65), and the rate of its increase was significantly larger (p < 0.005) as operative age decreased. Pulmonary valve regurgitation of greater than mild degree occurred in 8 of 54 (15%) patients with the valve preserved.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗

[A report of two cases of absent pulmonary valve syndrome].

Two infants, aged 8 and 18 months, with absent pulmonary valve syndrome unaccompanied by severe pulmonary symptoms or roentgenographic evidence of emphysema underwent a corrective operation consisting of infundibular muscle resection, patch closure for ventricular septal defect (perimembranous outlet type), and right ventricular outflow tract reconstruction with a monocusp patch. The patients tolerated pulmonary regurgitation relatively well, although aneurysmal dilatation of the pulmonary arteries was present preoperatively. Since the postoperative course was uneventful, we think that, in milder cases of this syndrome, a pulmonary valve prosthesis, the insertion of which is advocated by some authors, is not essential, and that right ventricular outflow tract reconstruction with a monocusp patch may suffice in selected cases.

Female↗

Morphologic changes induced by dilation of the pulmonary valve anulus with overlarge balloons in normal newborn lambs.

The optimal method for balloon dilation valvuloplasty of congenital pulmonary valve stenosis has not been established. The cardiac damage produced by using a balloon the same size as the pulmonary anulus has not been described. The use of balloons that are larger than the anulus or of a dynamic dilation technique may enhance gradient reduction, but their added risks are not known. The pulmonary valve anuli of 14 normal newborn lambs were dilated with angioplasty balloons 20% smaller to 90% larger than the anulus; in 3 dilations, a dynamic technique was used, consisting of withdrawal of the fully inflated balloon from the anulus into the body of the right ventricle. Twelve lambs were killed immediately after the procedure and 2 were killed later, and detailed gross anatomic and microscopic observations of the heart were made. These observations support the following conclusions: (1) Dilation of the pulmonary valve anulus with overlarge balloons is "clinically" well tolerated in normal newborn lambs. (2) The major damage to the heart from any balloon is not the pulmonary anulus, but to the right ventricular outflow tract and free wall, with mural hemorrhages of varying size. (3) Trauma is minor if the balloon is 30% larger than the anulus, but is considerable when the balloon is 50% larger; (4) Trauma is worst subjacent to the proximal end of the balloon, so that longer balloons may cause greater damage; and (5) Resolution of acute right ventricular hemorrhage occurs with small, patchy areas of fibrosis.

Angioplasty, Balloon↗

Isolated congenital absence of a single pulmonary valve cusp.

Isolated absence of a single pulmonary valve is extremely unusual. We present a 16-year-old male with the absence of one cusp resulting in significant pulmonary regurgitation, right ventricular enlargement, and dilatation of the main pulmonary artery and its branches. Surgery consisted of creating a valve cusp from the posterior wall of the pulmonary artery at the base of the pulmonary trunk. Follow-up at 3.5 years revealed good results with only mild pulmonary stenosis and regurgitation.

Adolescent↗

Prenatal diagnosis of familial absent pulmonary valve syndrome: case report and review of the literature.

We report on a case of absent pulmonary valve syndrome in a woman with a history of one healthy child and one child with tetralogy of Fallot with absent pulmonary valve. The diagnosis was missed at the first ultrasound examination performed at 13 + 5 weeks of gestation and correctly diagnosed at 21 + 5 weeks. Re-evaluation of the ultrasound examination recorded at 13 + 5 weeks exhibited severe insufficiency of the pulmonary valve at this time. However, neither dilatation of the right and left pulmonary arteries nor asymmetry of the ventricles were present at that time. The pregnancy was terminated at 22 + 1 weeks of gestation when autopsy confirmed the diagnosis of absent pulmonary valve syndrome. Karyotyping of the fetus after termination of pregnancy revealed normal chromosomes. Echocardiography of the parents and the healthy sibling revealed normal results.

Adult↗

Management of absent pulmonary valve syndrome with patent ductus arteriosus.

The etiology of absent pulmonary valve syndrome is unknown. Several theories have been advanced to explain the associated findings of a rudimentary pulmonary valve, ventricular septal defect, aneurysmal pulmonary arteries, and absence of the ductus arteriosus. The patient presented here would contradict these currently held theories. A review of the literature follows the case report.

Abnormalities, Multiple↗