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Outcome after repair of tetralogy of Fallot with absent pulmonary valve.

BACKGROUND: Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is associated with pulmonary artery dilatation and airway compression. METHODS: Since January 1, 1984, 28 patients with TOF/ APV have undergone complete repair (median age 11 days, range 1 day to 16 years). RESULTS: Thirteen patients were ventilated for respiratory failure preoperatively and extracorporeal membrane oxygenation was used in 3. Twenty-six patients underwent pulmonary artery plication (11 anterior, 15 anterior/ posterior). The right ventricular outflow tract (RVOT) was reconstructed with a patch (19), valved conduit (5), or monocusp valve (4). Early mortality was 21.4% (6/28), with 1 late death. All early deaths occurred in infants intubated preoperatively. Survival was 77% (95% confidence limit [CL] 56%, 89%) at 1 year and 72% (95% CL 50%, 86%) at 10 years. After surgery, 3 patients underwent reoperation for persistent respiratory symptoms, which resolved after repeat plication and placement of a valved conduit. Freedom from death or reoperation was 68% (95% CL 46%, 83%) at 1 year and 52% (95% CL 29%, 71%) at 10 years. In a multivariable analysis, only preoperative intubation was associated with a worse outcome (p = 0.04). CONCLUSIONS: Long-term outcome for patients with TOF/APV who survive the initial repair is good. Repeat plication and pulmonary valve implantation may improve outcome in patients with persistent airway compression.

Adolescent↗

Pulmonary valve gonococcal endocarditis. A forgotten disease.

Although gonococcal infections of the pulmonary valve were common before the introduction of antibiotics, such infections have rarely been reported since penicillin became available. In an elderly man with gonococcal endocarditis of the pulmonary valve the non-specific signs and symptoms, the late appearance of a pulmonary murmur, and the sterility of early blood cultures made the diagnosis unclear until three weeks after admission. Endocarditis was localised to the pulmonary valve by M-mode and cross-sectional echocardiography. Echocardiography may be useful for diagnosing endocarditis in patients with fever of unknown origin. Gonococcal infection should be suspected in patients with pulmonary vegetations and sterile blood cultures.

Aged↗

Initial and late results after catheter intervention for neonatal critical pulmonary valve stenosis and atresia with intact ventricular septum: a technique in continual evolution.

Critical pulmonary valve stenosis or atresia with intact ventricular septum is a rare congenital cardiac defect that can be technically difficult to alleviate in the catheterization laboratory. Over the past 10 years, several techniques and modifications with variable results have been advocated to facilitate the valvuloplasty procedure. This report describes a single operator's experience using various techniques in 28 neonates with critical pulmonary stenosis or atresia who were considered candidates for transcatheter intervention. The first two patients underwent a gradational balloon valvuloplasty approach that resulted in prolonged fluoroscopy exposure. Thereafter, a "snare assisted" umbilical artery approach was developed which facilitated the valvuloplasty procedure and resulted in significantly fewer balloons used and shorter fluoroscopy times. Early in our experience, stiff guidewire perforation of atretic pulmonary valves was used, whereas in our last two patients, a simplified perforation technique with a new 0.9-mm excimer laser catheter was used. Late echocardiographic and clinical follow-up evaluation in 27 patients demonstrates persistent gradient relief, resolution of tricuspid valve insufficiency, and elimination of right to left shunting at the atrial level. Balloon valvuloplasty is the treatment of choice for critical pulmonary valve stenosis or atresia with intact ventricular septum. When necessary, the use of umbilical artery "snare assistance" facilitates the valvuloplasty technique and shortens procedure time while laser perforation is currently preferable for perforation of the atretic pulmonary valve.

Catheterization↗

Perforation of the atretic pulmonary valve. Long-term follow-up.

OBJECTIVES: We evaluated the long-term results of perforation of the pulmonary valve in patients with pulmonary atresia with an intact ventricular septum (PA-IVS). BACKGROUND: Interventional perforation of the pulmonary valve is considered the elective first stage treatment for PA-IVS, particularly in patients with a tripartite right ventricle (RV) and normal coronary circulation. However, the long-term results of this procedure are lacking. METHODS: Between January 1991 and December 2001, 39 newborns with a favorable form of PA-IVS underwent attempted perforation of the pulmonary valve. We evaluated the early and long-term outcomes. RESULTS: Median tricuspid and pulmonary z values were -1.2 and -2.4, respectively. Perforation was successful in 33 patients. Among them, 17 needed neonatal surgery, 13 did not need any surgery, and 3 had elective surgery after the first month of life. There were two procedure-related deaths, seven nonfatal procedural complications, and four postsurgical deaths. Compared with patients needing neonatal surgery, those having no or elective surgery had a higher incidence of a tripartite RV and a higher median tricuspid z value (92% vs. 53%, p = 0.04 and -1.7 vs. -0.5, p = 0.03). At a median follow-up of 5.5 years (range 0.5 to 11.5), survival was 85% and freedom from surgery was 35%. Five patients, four of whom had neonatal surgery, underwent a partial cavo-pulmonary connection. CONCLUSIONS: Our results show that this technique, although burdened by non-negligible mortality and morbidity, is effective in selected patients with a normal-sized RV. Preselection of patients allows interventional or surgical biventricular correction in the majority of cases.

Cardiac Catheterization↗

[Pulmonary valvuloplasty with a balloon catheter in calcified pulmonary valve stenosis].

Since the first pulmonary valvuloplasty report, the procedure has been used frequently, becoming the best option in isolated valvar pulmonary stenosis. Pulmonary valvuloplasty in adults with calcified valvar pulmonary stenosis, however, has been minimal. We have reported the case of a 62-year-old female patient, with calcified valvar pulmonary stenosis, who underwent valvuloplasty with balloon catheter and showed a decrease in the transvalvular systolic gradient, thus proving the success of post procedure.

Calcinosis↗

Percutaneous pulmonary valve implantation.

Percutaneous implantation of valves is a fast evolving field in interventional cardiology. Acquired pulmonary valve disease is uncommon; however, right ventricular outflow tract dysfunction is one of the common indications of reoperations during late follow-up of repaired congenital heart disease. The impact of pulmonary valvular dysfunction on right ventricular function during late follow-up of patients with adult congenital heart disease has led to earlier intervention. The importance of a technique with lower morbidity and mortality, good patient acceptance, and efficacy that is comparable with surgery cannot be underestimated. Percutaneous pulmonary valve implantation uses a valved-stent assembly implanted by means of a double balloon catheter delivery system. The device is a bovine jugular venous valve sutured inside a platinum-iridium. In a series of 58 consecutive patients, we implanted this valve successfully with good clinical and hemodynamic results and no mortality on long-term follow-up. Early device designs led to reintervention by surgical explantation in the early experience, and a second percutaneous pulmonary valve implantation during latter experience. This exciting new technique will lead to review of the current indications and timing of intervention for right ventricular outflow tract dysfunction in late follow-up of congenital heart disease.

Adolescent↗

[Balloon valvuloplasty of congenital pulmonary valve stenosis].

Percutaneous balloon valvuloplasty can be considered a therapeutic alternative to surgery for pulmonary valve stenosis. In this analysis, our results will be reported with respect to the various balloon sizes as related to the diameter of the valvular ring as well as differing balloon catheters and the presence of other anomalies in addition to pulmonary valve stenosis. Balloon valvuloplasty was carried out in 23 infants and children ranging in age from one month to eleven years and in two adults at the age of 22 and 50 years, respectively, with a systolic pressure gradient across the pulmonary valve of more than 50 mm Hg or, in the presence of pressure gradients between 40 and 50 mm Hg, in association with a right ventricular pressure of at least 60% of that of the systemic pressure. In all patients echocardiography was carried out prior to the procedure to rule out valve dysplasia and Doppler echocardiographic determination of the pressure gradient was performed before and after dilatation. The diameter of the valvular ring was measured echocardiographically as well as from the right ventriculogram and the size of the balloon chosen accordingly. After diagnostic catheterization, the balloon catheter was advanced to the pulmonary valve with a guidewire. Under fluoroscopy, the balloon was completely inflated then the contrast medium immediately withdrawn, a procedure of seven to 23 seconds with the single-balloon catheter and five to nine seconds with the trefoil catheter. At the first dilatation, the larger portion of the balloon was advanced to the main stem of the pulmonary artery and was placed proximally for subsequent dilatations.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

An experimental study on transannular patching of the right ventricular outflow tract with and without a pulmonary valve monocusp mechanism.

The efficacy of a pericardial patch in the right ventricular outflow tract (RVOT) with a monocusp valve mechanism was investigated experimentally. Hemodynamic performance of the right ventricle and angiographic competence of the valve were compared in animals after patching of the RVOT and removal of the pulmonary valve (group I), patching of the RVOT, removal of the pulmonary valve and implantation of a monocusp valve (group II), patching of the RVOT, partial removal of the pulmonary valve and implantation of a monocusp valve (group III) and a control group (IV). Postoperative effective right ventricular cardiac index, stroke volume and stroke work improved significantly from group I to III, but did not reach the control values of group IV. Angiographically the monocusp valve allowed some early diastolic regurgitation due to late closure (group II) if it was not supported by remnants of the animal's own pulmonary valve (group III).

Animals↗

Blood cyst of the pulmonary valve in an adult: report of a case and review of the literature.

We report a case of a blood cyst originating from the pulmonary valve cusp of a 43-year-old Japanese woman with pulmonary stenosis. Cineangiography revealed a pedunculated tumor on the arterial surface of the pulmonary valve. It was successfully removed by a transpulmonary artery approach. The cyst contained old blood and calcified thrombi, and its wall consisted of collagenous fibrous tissue. Immunohistochemically, a monolayer of flat cells lining the cyst was confirmed as endothelium using antibodies to von Willebrand factor and CD34, as well as UEA-I lectin. To the best of our knowledge, blood cysts of the pulmonary valve are rare and only 10 such cases have been reported, including six pediatric cases. This case is the oldest and fifth adult patient with a blood cyst found on the pulmonary valve. A possible histogenesis is discussed.

Adult↗

[Evaluation of pulmonary flow through the aortic and pulmonary valves of the fetus during normal pregnancy from 20 to 41 weeks of gestation with two dimensional doppler (2dd)].

UNLABELLED: Doppler data have been analysed in 49 cases for aortic valve and 35 cases for pulmonary valve. For both valves V max, V mean, Aortic Flow and Pulmonary Flow were measured and compared with gestational age. RESULTS: V max A = 0.85 +/- 0.23 m/s, V max P = 0.72 +/- 0.14 m/s, (p < 0.01); V mean A = 0.28 +/- 0.08 m/s, V mean P = 0.24 +/- 0.06 m/s, (p < 0.02); A Flow = 699 +/- 285 ml/min, P Flow = 740 +/- 310 ml/min, and 275 +/- 100 ml/min/kg for aortic valve and 322 +/- 120 ml/min/kg for pulmonary valve (p > 0.05). There was statistical significant correlation between aortic and pulmonary flow and gestational age and there was no correlation between max velocity and gestational age for these valves.

Aortic Valve↗

Pulmonary valve endocarditis during puerperal sepsis.

A case of pulmonary valve endocarditis caused by Staphylococcus aureus during puerperal sepsis in a female patient is reported. The M-mode and two dimensional echocardiographic finding are described. A review of the literature shows that this entity is rare. A large vegetation in the leaflet of the pulmonary valve was excised and the patient recovered after a full course of antibiotics.

Adult↗

Tetralogy of Fallot with absent pulmonary valve: echocardiographic morphometric features of the right-sided structures and their relationship to presentation and outcome.

Respiratory symptoms in tetralogy of Fallot with absent pulmonary valve are believed to be due to bronchial compression secondary to dilated pulmonary arteries; however, not all patients are born compromised. Echocardiographic morphometry of the right-sided structures was investigated to determine the possible relationship between anatomy and clinical presentation. Twenty-five patients were identified, and 15 had preoperative echocardiograms. Patients were divided into two groups: those with respiratory distress (group I, n = 9) and those without (group II, n = 6). No difference was noted in branch pulmonary artery diameters between groups; however, the pulmonary valve/ aortic valve ratio, reflecting the dimension of the narrowest pathway from the right ventricle, was larger in group I (0.74 +/- 0.15 versus 0.60 +/- 0.07, p < 0.05). Pulmonary valve diameter correlated with main and right pulmonary artery diameters. We conclude that patients with tetralogy of Fallot with absent pulmonary valve and respiratory compromise have a greater pulmonary valve/aortic valve ratio but do not have greater dilatation of proximal branch pulmonary arteries. This suggests that the pathophysiology is not due solely to compression of the bronchi but is also related to the blood flow dynamics in the pulmonary vessels.

Adolescent↗

Disruption of sutured pulmonary valve in Fontan operation.

Two patients who had undergone a Fontan operation presented late with considerable disruption of a sutured pulmonary valve. Both patients had increasing ascites, decreased exercise tolerance, atrial arrhythmias, high right atrial pressure, and a large ratio of pulmonary blood flow to systemic blood flow. At operation, the main pulmonary artery was closed either by suturing the anterior and posterior walls together immediately distal to the pulmonary valve or by reinforcing the resutured pulmonary valve with a polytetrafluoroethylene patch. Both patients had an uneventful postoperative course, with disappearance of the symptoms and return of sinus rhythm. Although it is tempting to simply suture the usually thickened pulmonary valve in the Fontan operation, approximation of the pulmonary artery walls or patch reinforcement is necessary to minimize disruption.

Adult↗

Procollagen synthesis by fresh and cryopreserved rat pulmonary valve grafts.

OBJECTIVE: Allograft valves are frequently used in the repair of congenital cardiac anomalies. The failure rate may differ depending on the type of allograft used. Previous studies have shown that rat aortic valve grafts exhibit synthesis of procollagen, suggesting a capacity for repair and regeneration after implantation. No studies of pulmonary valve grafts in the heterotopic rat implant model have thus far been reported. This study was designed to investigate whether pulmonary valve grafts maintain in vivo viability, as demonstrated by procollagen synthesis, and whether cryopreservation, histocompatibility, or both affect this property. METHODS: Cryopreserved and fresh rat pulmonary valves were implanted into the abdominal aorta of syngeneic and allogeneic recipients. The grafts and native valves were excised 3 to 21 days after implantation. Valves were sectioned and immunohistochemically stained for procollagen. Computerized morphometry was used to calculate changes in intima, media, and adventitia as a percentage of cross-sectional area of the graft. Procollagen content was graded by semiquantitative methods. RESULTS: Pulmonary valve grafts had significantly greater collagen density in the intima and adventitia compared with native aortic and pulmonary tissues, but collagen density in the media was similar in all groups. The grafts demonstrated appreciably greater procollagen than the corresponding native valves. These findings were consistent in all grafts (ie, both fresh and cryopreserved, both syngeneic and allogeneic), irrespective of duration of implantation. CONCLUSIONS: Procollagen synthesis occurs in pulmonary valve grafts early after implantation, indicating viability of these tissues. This model of pulmonary valve implantation may have wide applicability to questions of allograft biology.

Animals↗

The relationship of pulmonary valve motion to the motion of surrounding cardiac structures: a two-dimensional and dual M-mode echocardiographic study.

To assess the relationship of late diastolic pulmonary valve motion to motion of adjacent cardiac structures, we performed two-dimensional and dual M-mode echocardiography on 15 pulmonary normotensive (group A) and nine pulmonary hypertensive subjects (group B). Simultaneous pulmonary valve and posterior aortic wall a-waves were less prominent in group B than in group A (p less than 0.001), and their amplitudes were linearly related within each group (r = 0.83). Analysis of two-dimensional studies confirmed a relationship between pulmonary valve and posterior aortic wall late diastolic motion. No subject had independent presystolic motion of the pulmonary valve within the pulmonary artery. Subjects with shallow a-waves had impaired left atrial emptying compared with those with normal a-wave amplitudes (p less than 0.01). We conclude that the pulmonary valve a-wave does not represent independent valvular displacement, but rather, reflects motion of the entire cardiac base. Variations in a-wave morphology may result, at least in part, from the effects of altered ventricular geometry and compliance on left atrial emptying.

Aorta↗

Fetal cardiac morphology of tetralogy of Fallot with absent pulmonary valve in the rat.

Fetal in situ cardiovascular and bronchial morphologies were studied in rats with tetralogy of Fallot and absent pulmonary valve to clarify the prenatal pathology of this complex. There were 42 fetal rats with this complex among 300 fetuses treated with N,N'-bis-(dichloroacetyl)-1,8-octamethylenediamine (bis-diamine) (200 mg) on the 10th day of pregnancy. After undergoing rapid whole-body freezing on the 21st day, they were studied by means of serial cross-sectional photographs of the frozen thorax. Fetuses with normal heart treated with bis-diamine served as control. Tetralogy of Fallot and absent pulmonary valve in the fetus was associated with absence of the ductus arteriosus, enlargement and hypertrophy of both ventricles, and enlargement of the pulmonary arteries that compressed the bronchial trees. These abnormalities were inversely correlated with the degree of pulmonary stenosis. Milder stenosis (pulmonary valve ring greater than 50% of control, n = 28) was associated with larger pulmonary arteries (250 +/- 12% of control) (mean +/- SEM) and larger ventricles (volume, 200 +/- 15%; mass, 130 +/- 4%). Severe stenosis (pulmonary valve ring less than 50% of control, n = 14) was associated with mildly enlarged pulmonary arteries and mild bronchial compression. We concluded that enlargement of the pulmonary arteries and bronchial compression develop in fetal life and are associated with mild pulmonary stenosis in tetralogy of Fallot and absent pulmonary valve.

Animals↗

Pulmonary valve incompetence. II. Application of electromagnetic flow velocity catheters in children.

During cardiac catheterization in 49 children regurgitant flow through competent and incompetent pulmonary valves was evaluated with electromagnetic flow velocity catheters using the ratio of the separately integrated backward and forward flow velocity signals (vb/vf). When the pulmonary valve was normal, vb/vf was less than or equal to 0.10. After operation for pulmonary valve stenosis or tetralogy of Fallot larger values were often found. In patients with an outflow patch across the pulmonary valve ring vb/vf values of up to 0.54 were found.

Adolescent↗

Pulmonary valve papillary fibroelastoma. A case report.

We present a case of pulmonary valve fibroelastoma diagnosed by echocardiogram which was confirmed by surgical resection in a patient in whom elective coronary artery bypass surgery was performed. The patient had no clinical or constitutional symptoms suggesting the presence of an intracardiac tumor. Routine preoperative transthoracic echocardiography revealed a pulmonary valve tumor. Histopathological analysis resulted in a diagnosis of papillary fibroelastoma. Papillary fibroelastomas are rare and benign cardiac tumors. They usually arise from the cardiac valves. In the literature we found only a few cases, so it seems there is a need for further description of additional cases of pulmonary valve fibroelastoma.

Coronary Artery Bypass↗