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Echocardiographic assessment of the size of aortic and pulmonary valve annulus before balloon valvoplasty.

Two dimensional echocardiographic measurements of the size of aortic and pulmonary valve annulus were made in 60 patients before balloon valvoplasty and compared to the angiographic measurements. Aortic valve annulus was measured in 34 patients (26 with valvar aortic stenosis and 8 with discrete subaortic stenosis) in the parasternal long axis or apical 5-chamber views. The pulmonary valve annulus was measured in 26 patients with valvar pulmonary stenosis (PS) in the parasternal short axis view of the right ventricular outflow view. The visualization of the annulus was good in all except 2 patients with valvar PS. Angiographic measurements of the aortic and pulmonary valve annulus were made in aortic root and right ventricular angiograms respectively, taken in both right and left anterior oblique views. There was an excellent correlation between the measurements of the annulus size by the two techniques (r value for pulmonary valve 0.91; for aortic valve 0.96; over all 0.94). Echocardiography can accurately measure valve annulus size and help in choosing balloon dilatation catheter of appropriate size before the valvoplasty procedure.

Adolescent↗

Treatment of critical pulmonary valve stenosis by balloon dilatation in the neonate.

Critical pulmonary valve stenosis represents an emergency, and immediate treatment is mandatory. The purpose of this study was to evaluate the immediate and medium-term results of pulmonary valve dilatation. We report 18 neonates in whom pulmonary valvuloplasty was attempted. The procedure could be accomplished in 14 patients. The angiographically determined diameters of the pulmonary and tricuspid valve at the time of procedure were 5.6 +/- 1.5 mm and 14.0 +/- 5.4 mm. The mean Doppler gradient decreased from 71 +/- 27 mm Hg to 27 +/- 14 mm Hg. Perforation of the right ventricular outflow tract was the major complication in three patients with one fatal event. Infusion of prostaglandin E1 could be discontinued 1 to 5 days after the procedure. On follow-up three children required a second balloon dilatation with good results. Seven patients monitored for more than 9 months with a mean follow-up time of 34.4 +/- 16 months had a residual gradient of 11.6 +/- 6.7 mm Hg. In spite of a hypoplastic pulmonary valve annulus in seven of the patients, results were good and surgery could be avoided.

Catheterization↗

[Pulmonary valve endocarditis, bilateral deep vein thrombosis amd recurrent pulmonary emboli].

A 57-year-old obese man with hypertensive heart disease and long-standing varicose veins developed recurrent bouts of fever, lung infiltrations, cardiac arrhythmias and increasing dyspnoea. On admission multiple lung emboli and bilateral deep vein thromboses were confirmed. The echocardiogram demonstrated floating vegetations on all three pulmonary valve cusps. Biochemical tests indicated an inflammatory constellation (ESR 62/105 mm, C-reactive protein 13.3 mg/dl), partial respiratory insufficiency (pO2 54.6 mm Hg; pCO2 29 mm Hg). Streptococcus bovis was grown from several blood cultures. Conservative treatment over several weeks, complicated by "drug fever", with penicillin G (10 mega IU four times daily) and gentamicin (80 mg twice daily intravenously), later vancomycin (500 mg four times daily intravenously), then roxithromycin (150 mg three times daily by mouth), as well as maintenance anticoagulation with heparin (800-1,200 IU/h intravenously, later 15,000 IU subcutaneously twice daily), followed by phenprocoumon, contained the disease. One year after onset of treatment a good functional results had been achieved.

Endocarditis, Bacterial↗

Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be part of the intracardiac repair?

Thirteen patients with complex of tetralogy of Fallot and absent pulmonary valve were treated. Three infants presented with severe cardiorespiratory distress (Group I) necessitating surgical intervention in two and intensive medical treatment in the third; there were no late survivals. The remaining 10 patients were operated upon electively at an older age (Group II); in six the ventricular septal defect was closed and the right ventricular outflow obstruction relieved (Group IIA), and in the other four a valve was inserted at the pulmonary anulus at the time of intracardiac repair (Group IIB). There was no hospital mortality in Group II. Five of the six patients in Group IIA remained symptomatic postoperatively, and three needed a subsequent pulmonary valve insertion. All patients in Group IIB were symptom free following operation; one patient began having a progressive decrease in exercise performance, decrease in pulmonary artery size, and cardiothoracic ratio were more marked in those patients who had primary insertion of a pulmonary valve. The clinical results and postoperative assessment of cardiac function tend to indicate that pulmonary valve insertion should be performed at the time of the intracardiac repair.

Adolescent↗

[Isolated florid pulmonary valve endocarditis].

We report on a case of isolated pulmonary valve endocarditis caused by coagulase-negative staphylococci 22 years after total correction of a pentalogy of Fallot. The M-mode and two-dimensional echocardiographic findings are presented, and the patient's clinical progress is described. An overview of the literature shows isolated endocarditis of the pulmonary valve to be an exceptionally rare occurrence. 6 months postoperatively, the patient has not been experiencing a relapse and is now in NYHA stage I.

Adult↗

Initial experience with a bicuspid polytetrafluoroethylene pulmonary valve in 41 children and adults: a new option for right ventricular outflow tract reconstruction.

BACKGROUND: As the population of children with repaired congenital heart disease ages, an increasing number of patients will benefit from pulmonary valve insertion. We report our initial experience in 41 consecutive patients treated with placement of a surgically created polytetrafluoroethylene bicuspid pulmonary valve. METHODS: A bicuspid pulmonary valve with orifice size greater than 24 mm is created with polytetrafluoroethylene and sutured into the right ventricular outflow tract. To obviate the need for reoperation in growing children, this technique is limited to older children and adults. Polytetrafluoroethylene bicuspid pulmonary valves were placed in 41 patients (age: range, 5.0 to 64.7 years, median = 15.7 years; weight: range, 14.2 to 99.0 kilograms, median, 52.0 kg). All patients had pulmonary insufficiency, pulmonary stenosis, or both, after previous intervention for tetralogy of Fallot (27), pulmonary stenosis (11), pulmonary atresia with intact ventricular septum (2), or double outlet right ventricle (1). RESULTS: All patients left the operating theater with transesophageal echocardiography documenting no pulmonic stenosis and zero to trace pulmonic insufficiency. Median hospital length of stay was 5 days (range, 3 to 15 days; mean, 5.8 days). Follow-up including echocardiography ranged from 0.2 to 3.1 year (median follow-up, 1.5 years) and revealed significant improvement in New York Heart Association Classification, pulmonary insufficiency, and right ventricular end diastolic dimension. CONCLUSIONS: Polytetrafluoroethylene bicuspid pulmonary valve reconstruction of the right ventricular outflow tract is a safe, effective, and durable technique for the short term. Appropriate oversizing minimizes outflow tract obstruction while maximizing competence. Long-term follow-up is necessary to determine the true value of this technique.

Adolescent↗

Cartilage in pulmonary valves of Syrian hamsters.

The presence of cartilage in the pulmonary valve has been reported in birds, but not in mammals. We describe here the occurrence of cartilaginous tissue in the pulmonary valves of 40 (11.4%) of 351 Syrian hamsters examined using histological, histochemical and/or immunohistochemical techniques. The cartilaginous deposits were located along the fibrous attachments of the valve leaflets to the wall of the pulmonary artery trunk. Our findings indicate that the proximal attachments of the leaflets to their respective sinuses, and especially that of the ventral leaflet, are the most prone valvular regions to develop cartilaginous foci. Nonetheless, the possible function of these foci remains an open question. Formation of cartilage in the pulmonary valve starts within the first month of life, that is during the period in which the valve reaches histological maturation. The earliest evidence of chondrogenesis is the presence of small groups of cells embedded in a type II collagen-positive extracellular matrix. These groups of cells, which can appear as early as one day after birth, increase moderately in size and differentiate into hyaline cartilaginous tissue. The precursors of the cartilaginous cells are presumed to be neural crest-derived elements. However, the factor or factors involved in the differentiation of these precursors into chondrocytes are still unknown. In this regard, our observations cast doubt on the hypothesis that the formation of cardiac cartilages is primarily due to locally intense mechanical stimulation.

Aging↗

Autologous monocusp pulmonary valve: preliminary results.

BACKGROUND: There is growing recognition that postoperative pulmonary regurgitation may result in early or late progressive right heart failure. METHOD: A technique for fashioning an autologous monocusp pulmonary valve from the wall of the pulmonary artery was developed. The monocusp valve was fashioned from the anterior wall of the main pulmonary artery, and the remaining defect was filled with autologous pericardium. The procedure was performed in 8 dogs and 5 children. RESULTS: Early follow-up and serial echocardiographic assessment in both dogs and children proved the functionality of this monocusp pulmonary valve. All valves were pliable and demonstrated mild to moderate pulmonary stenosis and insufficiency. CONCLUSIONS: Construction of the autologous monocusp pulmonary valve is a feasible technique, and the valve performs efficiently. The acute performance in the canine model was excellent, and preliminary midterm results in the clinical study are reasonable. It is logical to assume that the monocusp, being an integral part of the arterial wall, will retain its viability and share in the subsequent growth of the pulmonary artery. Should follow-up studies demonstrate its long-term competence, this autologous valve may provide a good solution for various forms of pulmonary regurgitation and be useful in pulmonary autograft replacement of the aortic valve.

Animals↗

[Absent pulmonary valve syndrome in tetralogy of Fallot: report of two cases].

A combination of absence of the pulmonary valve with ventricular septal defect, annular pulmonary stenosis and aneurysm of the pulmonary artery is called "absent pulmonary valve syndrome". Most patients with this condition suffered from severe respiratory symptoms in early infancy. Respiratory failure which is the leading cause of mortality in infants with this syndrome, results from pulmonary emphysema, pulmonary atelectasis or pulmonary infection as a consequence of compression of the bronchus by the pulmonary aneurysm. If these patients can be treated medically to survive beyond one year old, gradual decrease in severity of symptoms is expected. A definite correction in childhood is recommended. Otherwise, palliative operation in infancy has a high mortality rate. This paper reported two cases with severe symptoms in infancy; one patient expired at nine months of age, and the other lived beyond one year to receive surgical correction at the age of three. The etiology, clinical features and treatment of absent pulmonary valve syndrome are also discussed.

Abnormalities, Multiple↗

Replacement of a regurgitant pulmonary valve with a stentless bioprosthesis.

Acquired surgical disease of the pulmonary valve is rare. We report a 72-year-old man who presented with subacute endocarditic pulmonary regurgitation. This lesion was surgically corrected with a stentless bioprosthesis. Previously, homografts and various xenografts have been used for replacement of the pulmonary valve both in the pediatric population and in adult patients with congenital heart disease. Pulmonary regurgitation is a rare lesion, but if it is encountered our case demonstrates that it can be successfully and easily treated with pulmonary valve replacement by using a stentless bioprosthesis.

Aged↗

[A primary epithelioid hemangioendothelioma localized in the pulmonary valve].

A case of epithelioid haemangioendothelioma sited at pulmonary valve, with an excrescence growing invading pulmonary artery troncus and its principal branches, in a 56-year-old woman is reported. The evolutive behavior simulated some clinical situations which made difficult the diagnosis: a mild-moderate pulmonary stenosis, a general syndrome, and, finally, an acute pulmonary thromboembolism that carried to an aggressive management which conducted to the entity diagnosis.

Emergencies↗

Comparative long-term results of surgery versus balloon valvuloplasty for pulmonary valve stenosis in infants and children.

BACKGROUND: We compared the long-term results of surgical valvotomy (S) versus balloon valvuloplasty (BV) for pulmonary valve stenosis in infants and children. METHODS: Results after surgical pulmonary valvotomy (with concomitant ASD/VSD closure) (n = 62, age 2.9 +/- 3.5 years) and balloon valvuloplasty (n = 108, age 3.6 +/- 3.9 years) were analyzed. Transvalvular mean pressure gradient decrease, freedom from reintervention for restenosis, pulmonary valve insufficiency, and tricuspid valve insufficiency were considered. RESULTS: Mean pressure gradient decreased significantly more in the surgical group (from 64.8 +/- 30.8 mm Hg to 12.8 +/- 9.8 mm Hg at a mean follow-up of 9.8 years) than after BV (decreasing from 66.2 +/- 21.4 mm Hg to 21.5 +/- 15.9 mm Hg after a mean of 5.4 years; p < 0.001). Moderate pulmonary valve insufficiency occurred in 44% after surgery, and in 11% after BV (p < 0.001). Tricuspid valve insufficiency occurred in 2% after surgery, and in 5% after BV. Restenosis occurred in 3 surgical patients (5.6%), 2 patients required reoperation, and 1 patient required a balloon valvotomy. Restenosis developed in 13 BV patients (14.1%): 6 patients were redilated and 7 patients required surgery. Surgical valvotomy led to significantly less reinterventions than balloon valvuloplasty (p < 0.04). CONCLUSIONS: Surgical relief of pulmonary valve stenosis produces lower long-term gradients and results in longer freedom from reintervention. Balloon valvuloplasty may remain, despite these results, the preferred therapy for isolated pulmonary valve stenosis, because it is less invasive, less expensive, and requires a shorter hospital stay. Surgery should remain the exclusive form of therapy in the presence of concomitant intracardiac defects, which need to be addressed.

Catheterization↗

Intermediate-term results of balloon valvuloplasty for isolated and complicated pulmonary valve stenosis.

UNLABELLED: The purpose of this study is to report our experience regarding the acute and intermediate-term results of balloon pulmonary valvuloplasty (BPV) in various types of congenital pulmonary valve stenosis. METHODS AND RESULTS: Twenty-four consecutive patients with a median age of 6.6 years (ranging from 1 month to 24 years old) underwent BPV between January 1988 and September 1991. These patients were divided into 2 groups; Group 1 consisting of 13 patients with isolated pulmonary valve stenosis, and Group 2 consisting of 11 patients with complicated pulmonary valve stenosis (supravalvular, subvalvular, valved conduit and post-right ventricular outflow reconstruction). Mean peak systolic pressure gradients from the right ventricle to the pulmonary artery were as follows: In group 1, 48 +/- 21 (mean +/- SD) mmHg before BPV, 18 +/- 8 mmHg immediately after BPV and 13 +/- 5 mmHg at the longest follow-up based on catheterization or Doppler echocardiographic studies. The gradients in group 2 were 65 +/- 28 mmHg before BPV, 46 +/- 25 mmHg immediately after BPV and 47 +/- 21 mmHg at the longest follow-up. CONCLUSIONS: BPV provides both acute and intermediate-term gradient relief in patients with isolated pulmonary valve stenosis. In complicated pulmonary valve stenosis, on the other hand, the effect of BPV was unsatisfactory and appears to depend on the mechanism of associated obstruction. Therefore accurate evaluation of the anatomy of associated obstruction in the pulmonary valve region is needed to determine that BPV is indicated.

Adolescent↗

Congenital absence of the pulmonary valve: report of two cases with review of the literature.

Congenital absence of the pulmonary valve is an uncommon combination of anomalies. Recently, we encountered two such cases, one associated with tetralogy of Fallot (case 1), the other with ventricular septal defect (case 2). Clinically, our cases had minimum symptoms. The congenital cardiac anomalies were diagnosed with physical examination, chest x-ray film, echocardiography, and confirmed with cineangiography. These procedures disclosed a rudimentary pulmonary valve, pulmonary regurgitation and aneurysmal dilatation of the main and right pulmonary artery. In case 2, the right lower pulmonary artery occlusion and left hydronephrosis were also demonstrated by magnetic resonance imaging. The two cases underwent surgical repair to correct the tetralogy of Fallot and the ventricular septal defect respectively. Our experience in diagnosing these cardiac anomalies is reported.

Child↗

The dysplastic pulmonary valve: echocardiographic features and results of balloon dilatation.

The feasibility of using balloon dilatation to relieve stenosis caused by dysplasia of the pulmonary valve was assessed in seven patients (five female, mean age two years) with angiographically confirmed dysplasia who were identified among 38 patients with pulmonary valve stenosis selected for balloon dilatation over a two year period. The clinical features in three patients were consistent with Noonan's syndrome. In all patients the gradient across the valve was assessed by cross sectional echocardiography and Doppler echocardiography before cardiac catheterisation. Balloon dilatation was performed by conventional techniques. In one patient, who had balloon dilatation in the operating room before surgical valvectomy, the diameter of the valve orifice increased from 3 mm to 10 mm. Inspection showed a tear along the anterior commissure. The mean (SD) pressure gradients between the right ventricle and pulmonary artery before and immediately after dilatation in five patients were not significantly different (58 (28) and 47 (12) mm Hg) respectively. There was no overall significant change in the degree of stenosis when four of these patients were examined by Doppler echocardiography six months after operation (44 (17) mm Hg), although one patient (case 5) did show a significant reduction in gradient. This patient had angiographic and echocardiographic features of dysplasia and commissural fusion. Several echographic features were common to all patients and distinguished them from cases of typical pulmonary valve stenosis. These were: pronounced thickening of leaflets; leaflet immobility in diastole and systole; no dilatation of the sinuses of Valsalva in diastole, and supra-annular narrowing. These poor results of balloon dilatation suggest that commissural fusion is not an important mechanism for causing stenosis in the dysplastic pulmonary valve. When dysplasia of the pulmonary valve is identified clinically and echocardiographically, balloon dilatation is unlikely to improve haemodynamic function; it should be attempted if commissural fusion is present.

Angioplasty, Balloon↗

"Balloon valvulotomy" of congenital pulmonary valve stenosis with tricuspid valve insufficiency.

The rare congenital anomaly of pulmonary valve stenosis and massive tricuspid valve insufficiency with intact ventricular septum is a lethal condition without reported survival after attempted treatment. In a neonate suffering from this syndrome, the pulmonary valve stenosis was relieved by rupturing the fused valve with a balloon catheter introduced transvenously. The desperate condition of the patient quickly improved after this procedure, with subsequent disappearance of the tricuspid valve incompetence. Balloon rupturing of fused valves at angiography may represent a therapeutic alternative in cases in which surgical valvulotomy is associated with a high mortality.

Cardiac Catheterization↗

High frequency oscillations of the pulmonary valve leaflet in double-chamber right ventricle.

The abnormal pulmonary valve echocardiographic tracing in a patient with a double-chambered right ventricle is presented. Preoperatively, the posterior pulmonary valve leaflet demonstrated a partial closure in systole, followed by coarse fluttering of the leaflet. The postoperative echocardiographic tracing of the pulmonary valve was normal. It is suggested that an obstructive process proximal to either semilunar valve which converts laminar flow to turbulent flow is capable of producing a similar pattern in the leaflets during ventricular ejection.

Child, Preschool↗

Relationships between the dimensions of the human aortic and pulmonary valve leaflets: implications on Ross' operation.

The technique of replacing the aortic valve with the patient's pulmonary valve and the implantation of a homograft in the pulmonary position was first introduced by D. Ross in 1967. Despite the many advantages and successes of this procedure, it frequently results in some degree of valve insufficiency. In order to optimize the results of Ross' operation, we carried out a comparative study (n = 22) of the relative dimensions of the human aortic and pulmonary valve leaflets using a digitizer. The digitizer supplies the spatial coordinates of a given point (in a pre-chosen reference frame), by the positioning of its sensor on that point. By digitizing relevant points marked on the arterial wall we were able to calculate the distance between the commissures and the perimeter of each leaflet. Analysing the values thus obtained, we found that in 17 of the 22 cases studied (77.3%) there was one orientation (out of the three anatomically possible orientations) of the pulmonary valve, relative to the configuration of the recipient aortic root, that was clearly better than the other two. This study led to the formulation of a "rule" that, in most of the cases studied (86.4%), led to the best fitting orientation of the autograft using the knowledge of the intercommissural distances.

Adult↗