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[The treatment of pulmonary valve stenosis using balloon dilatation. Long-term results].

Percutaneous pulmonary valvuloplasty has been used successfully in the treatment of pulmonary valve stenosis for several years, but reports on long-term results are still sparse. We have performed pulmonary valve balloon dilatation procedures 23 times in 22 patients since February 1985; the age of the patients ranged from 2 days to 22 years. Three patients did not benefit from the procedure, in two instances because the pulmonary valve was dysplastic and once because of severe associated infundibular stenosis. These patients subsequently underwent surgery. In the other 19 patients the initial transvalvular gradient of 35 to 160 mm Hg (mean 81 mm Hg) was markedly reduced (to values ranging from 10 to 60 mm Hg, mean 31 mm Hg) rendering surgery unnecessary. There were no complications. The patients have been followed up for periods ranging from 2 months to 3 years and all are asymptomatic. Repeat measurements of the transvalvular gradient were carried out either by recatheterization or by echo Doppler 4 months to 3 years after the procedure; we have not observed restenosis and the mean gradient remained unchanged compared to the measurements immediately after valvuloplasty. Pulmonary regurgitation was uncommon and indeed appears to be less prominent than after surgical valvotomy. Thus, percutaneous valvuloplasty now appears to be the preferred treatment for pulmonary valve stenosis and open heart surgery can be avoided in most cases.

Adolescent↗

Preservation of the pulmonary valve complex in tetralogy of fallot: how small is too small?

BACKGROUND: Surgical repair of congenital lesions associated with right ventricular outflow tract obstruction frequently requires the destruction of pulmonary valve (PV) components including the valve annulus. The resultant pulmonary insufficiency may lead to late functional deterioration of right ventricular performance. Acute right ventricular dysfunction has been associated with poor pulmonary runoff, tricuspid valve regurgitation, and pulmonary hypertension. Preservation of PV competence may prevent both early and late right ventricular failure. However, the recent trend towards earlier repair of tetralogy of Fallot (TOF) may preclude preservation of the PV in favor of a transannular patch. We reviewed our experience with surgical repair of TOF to determine if age and/or body size affected the ability to repair the PV. METHODS: We reviewed the clinical records of 50 consecutive children who underwent surgical repair of TOF by one surgeon. The latter 27 patients underwent repair with an intention to preserve their pulmonary valve. In total, 28 patients underwent repair with preservation of their pulmonary valve, and form the basis of this study. Serial echocardiographic assessments were performed early (3 to 6 months) and late (12 months) after surgery. RESULTS: Pulmonary valve preservation was possible in the majority of children (89%) in whom it was intended. Pulmonary valve competence was observed in 68% of children, with only 5 (16%) children demonstrating severe insufficiency at follow-up. Residual right ventricular outflow tract obstruction was present in only 1 child who underwent repair with pulmonary valve preservation at greater than 2 years of age. CONCLUSIONS: Our data suggest that earlier repair of TOF does not preclude preservation of the pulmonary valve and may indeed facilitate repair. The pulmonary valve remains competent at 12 months, with acceptable gradients, and should participate in somatic growth. Pulmonary valve preservation during repair of TOF may prevent free pulmonary insufficiency, progressive right ventricular dilation, and the need for future prosthetic pulmonary valve replacement.

Age Factors↗

[Agenesis of the ductus arteriosus Botalli and premature closure of the foramen ovale, in combination with mitral valve atresia, hypoplastic left ventricle, transposition of the great arteries, and pulmonary valve atresia (author's transl)].

Agenesis of the Ductus arteriosus Botalli is a rare anomaly, which is known to occur in combination with truncus arteriosus, tetralogy of Fallot with pulmonary valve agenesis and also in simple tetralogy. The authors report on a case, where neither a ductus nor any remnant of it could be found in a complex heart malformation consisting of premature closure of the Foramen ovale, mitral atresia, hypoplastic left ventricle, transposition of the great arteries, pulmonary valve atresia and hypoplastic pulmonary artery. Speculations as to the pathogenesis of this anomaly presume slowing and eventual arrest of the ductal blood flow as a consequence of closure of the Foramen ovale in early fetal life. Thus total involution and disappearance of the distal part of the sixth aortic arch also on the left side seem conceivable.

Autopsy↗

Radiofrequency-assisted balloon dilatation in patients with pulmonary valve atresia and an intact ventricular septum.

OBJECTIVE: To investigate the efficacy and safety of transcatheter radiofrequency-assisted valve dilatation for infants with pulmonary valve atresia and intact ventricular septum as an alternative to the use of laser heated wires. DESIGN: Prospective clinical study. SETTING: Three paediatric cardiology centres. PATIENTS: Four children (aged 5-101 days, weight 2.8 kg) with pulmonary valve atresia and intact ventricular septum underwent percutaneous radiofrequency-assisted valve dilatation. METHODS: After delineating the atretic valve by angiography, 0.020 inch or 0.018 inch radiofrequency wires were used to perforate the atretic valve. The valve was then dilated with conventional balloon dilatation catheters up to the valve annulus diameter. RESULTS: In all four cases the radiofrequency wire perforated the atretic pulmonary valve and balloon dilatation was successful. In one patient the radiofrequency wire also passed through the anterior wall of the pulmonary artery causing tamponade which required surgical repair shortly afterwards. This patient died from sepsis six days later. One patient died three weeks after the procedure from septicaemia and a paradoxical coronary embolus. Two patients were discharged after 4 and 14 days respectively. CONCLUSIONS: Radiofrequency-assisted valve dilatation is a promising alternative to the recently developed laser wire technique. The major advantages are a reduction in cost and improved safety for the staff performing the procedure.

Balloon Occlusion↗

Absent pulmonary valve syndrome in infancy: surgery reconsidered.

Congenital absence of the pulmonary valve leaflets is generally associated with ventricular septal defect, anular pulmonary stenosis and aneurysmal dilatation of the pulmonary arteries: the absent pulmonary valve syndrome. Symptomatic infants with this syndrome suffer primarily from respiratory insufficiency caused by bronchial compression by the dilatated pulmonary arteries, and have a high mortality rate. Asymptomatic infants are thought to do well, and may have elective surgery late in childhood. Findings in four infants with absent pulmonary valve syndrome are reviewed. One symptomatic infant was successfully treated with closure of a ventricular septal defect and pulmonary arterial plication. One previously asymptomatic infant had a fatal respiratory arrest revealing the complexity of management of infants with absent pulmonary valve syndrome.

Bronchial Diseases↗

Balloon dilatation in infants and children with dysplastic pulmonary valves: short-term and intermediate-term results.

This study was designed to document the results of balloon valvuloplasty in patients with dysplastic pulmonary valves and to determine whether dysplastic pulmonary valves are responsible for recurrence of stenosis after balloon pulmonary valvuloplasty. Balloon valvuloplasty in 13 patients, aged 6 days to 12 years (median 1 year), with dysplastic pulmonary valves reduced the pulmonary valve gradient from 77.2 +/- 44.2 (mean +/- SD) to 26.8 +/- 17.0 mm Hg (p less than 0.001), which remained improved (34.9 +/- 34.6 mm Hg; p less than 0.02) at 6 to 19 months' (mean 10 months) follow-up. Valvuloplasty in 43 patients without dysplastic pulmonary valves reduced the valvar gradient from 94.3 +/- 41.0 to 31.1 +/- 22.4 mm Hg (p less than 0.001) immediately after the procedure, which at 6 to 34 months' follow-up in 23 patients was 29.2 +/- 33.5 mm Hg (p less than 0.001). The right ventricular peak systolic pressures (96.2 +/- 43.3 vs 112.1 +/- 40.1 mm Hg) and pulmonary valvar gradients (77.2 +/- 44.2 vs 94.3 +/- 41.0 mm Hg) before valvuloplasty, residual right ventricular pressures (52.9 +/- 14.5 vs 56.1 +/- 24.2 mm Hg) and pulmonary valvar gradients (26.8 +/- 17.0 vs 31.1 +/- 22.4 mm Hg) immediately after valvuloplasty, and residual right ventricular pressures (59.3 +/- 30.3 vs 53.6 +/- 34.3 mm Hg) and pulmonary valvar gradients (34.9 +/- 34.6 vs 29.2 +/- 33.5 mm Hg) at follow-up catheterization were similar (p greater than 0.1) in both groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Catheterization↗

Functional pulmonary valve regurgitation in the fetus.

OBJECTIVE: The purpose of this study was to determine the prevalence and the characteristics of functional pulmonary valve regurgitation in normally grown fetuses. DESIGN: A prospective cross-sectional study. SUBJECTS: A total of 1115 singleton fetuses between 18 and 41 weeks of gestation and who had normal heart anatomy, normal estimated weight for gestational age and normal flow velocity waveforms in the umbilical and middle cerebral arteries and umbilical vein were examined. Cases with agenesis, constriction or other abnormalities of the ductus arteriosus were excluded. METHODS: Examination of the pulmonary valve was performed by color Doppler echocardiography, pulsed wave Doppler and, if necessary, continuous wave Doppler in the short-axis view at the level of the origin of the great arteries and/or in a subcostal view of the right ventricular outflow tract and pulmonary trunk. If pulmonary valve regurgitation was detected by color Doppler flow imaging and confirmed by pulsed wave Doppler echocardiography, the maximum velocity of the regurgitant jets as well as their maximum lengths were measured. RESULTS: The prevalence of functional pulmonary valve regurgitation was 0.54% (n = 6). Pulmonary valve regurgitation was part diastolic in four cases and holodiastolic in two cases, with maximum velocity of < or = 2.05 m/s and maximum length of 3-8 mm. Prenatal re-examination of five of the six fetuses with pulmonary valve regurgitation showed that pulmonary valve regurgitation was a transient phenomenon in four cases. The fetal outcome in the presence of transient pulmonary valve regurgitation was normal; pediatric echocardiographic examination in these six fetuses with transient pulmonary valve regurgitation showed no regurgitations or other cardiac anomalies. CONCLUSIONS: Pulmonary valve regurgitation was functional in all six fetuses.

Blood Flow Velocity↗

Replacement of mitral, aortic, and pulmonary valves for rheumatic heart disease.

Rheumatic disease of the pulmonary valve has been documented at autopsy. Most of the reported cases of calcified pulmonary valves are associated with congenital heart disease. A unique case of rheumatic aortic and mitral valve disease associated with calcific pulmonary stenosis is presented. Prosthetic replacement of all three involved valves was carried out successfully. This is believed to be the first reported triple valve replacement of its kind.

Aortic Valve↗

Independent factors associated with longevity of prosthetic pulmonary valves and valved conduits.

OBJECTIVE: To evaluate the age dependence of variables predictive of pulmonary valve prosthesis replacement, we conducted the following analysis. METHODS: Retrospective analysis of 945 operations in 726 patients undergoing placement of pulmonary valve prostheses was performed. Age was identified as a strong independent predictor of valve failure. The database was stratified into age-based subsets and predictors of valve replacement were identified within each subset. RESULTS: For the entire cohort, freedom from valve replacement at 5 years was 81%. Younger age was strongly associated with decreased time to valve replacement by multivariable analysis (hazard ratio: 0.71/log-year, P <.001). Other independent factors included diagnosis, type of prosthesis, and time-dependent requirement for pulmonary valve stent placement. Important predictors of valve failure varied among age groups and are as follows: for Age Less Than 3 Months: valve type; for Age 3 Months To Less Than 2 Years: smaller normalized valve prosthesis size; for Age 2 Years To Less Than 13 Years: sex, smaller normalized valve prosthesis size, placement of endovascular stents, and valve type; for Age 13 Years To 65 Years: smaller normalized valve prosthesis size, placement of endovascular stents, and increased number of previous valve placements. CONCLUSION: Age is a dominant risk factor predictive of pulmonary valve prosthesis failure. A significant interaction exists between age and the effects of diagnosis, valve type, and size on prosthetic pulmonary valve longevity.

Adolescent↗

Interpretation of cardiac pathophysiology from pressure waveform analysis: the pulmonary valve.

Although an uncommon lesion, when pulmonary stenosis is considered, pulmonary artery and right ventricular pressures should be assessed simultaneously on two-catheter pullback to appreciate the precise location of pulmonary-right ventricular pressure gradients. The case examples demonstrate that the peripheral pulmonic stenosis can mimic pulmonary valve stenosis and that pulmonary artery insufficiency may be difficult to delineate on pressure alone (as is often the case with the hemodynamics of aortic insufficiency). These hemodynamic tracings are complemented by the echocardiographic and angiographic characterization of pulmonic valve lesions. Conduction defects or ventricular hypertrophy can affect the right ventricular pressure tracing and either delay or increase the timing of pressure rise and decline depending on the conduction disturbance and abnormality of myocardial contraction.

Adult↗

Absent Pulmonary Valve: Echocardiographic Features.

Congenital absence of the pulmonary valve, a rare anomaly, is characterized by absent or rudimentary pulmonary valve, often with annular stenosis, and aneurysmal dilatation of the pulmonary arteries. This defect is most commonly an accompaniment of tetralogy of Fallot but occasionally occurs alone. Four patients with this abnormality were examined by two-dimensional echocardiography at the Mayo Clinic. Doppler echocardiography provided hemodynamic assessment of the magnitude of outflow obstruction and valve regurgitation. The two-dimensional echocardiographic and Doppler features of absent or rudimentary pulmonary valve provided diagnostic information sufficient to proceed directly to surgical correction.

Journal Article↗

Endovascular stents in pulmonary valve and artery in swine: feasibility study of MR imaging-guided deployment and postinterventional assessment.

PURPOSE: To assess the feasibility of using magnetic resonance (MR) imaging to guide stent deployment in the pulmonary valve and artery and evaluate, after stent deployment, the position and morphology of and blood flow through the stent. MATERIALS AND METHODS: Angiography and 1.5-T MR imaging were performed in a dual-imaging suite. Nitinol stents were placed in the pulmonary valve and main pulmonary artery in five pigs by using MR imaging guidance. For interactive MR imaging monitoring of catheter manipulation and stent delivery, balanced fast field-echo and T1-weighted turbo field-echo sequences were used. Visualization of the delivery system was based on T2* (with air as the contrast material) or T1 (with gadodiamide as the contrast material). After stent deployment, the position and morphology of and flow through the stent were verified with multiphase multisection balanced fast field-echo and velocity-encoded cine MR imaging. Findings at angiography and postmortem examination also helped verify stent placement. The paired Student t test was used for data analysis. RESULTS: The stent was successfully deployed in all animals. The stent was placed distal to the pulmonary valve in four animals and across the pulmonary valve in one animal. The position and morphology of the stent were clearly depicted on balanced fast field-echo images. In the animal with the stent placed across the pulmonary valve, the pulmonary regurgitant fraction was 37%; this was not seen in the animals with stents placed distal to the pulmonary valve. No complication (eg, stent migration, intramural injury, or vascular perforation) was noted during the intervention. Findings at angiography and postmortem examination confirmed the position of the stents. CONCLUSION: MR imaging has the potential to guide stent placement in the pulmonary valve or artery and to evaluate flow volume within the stent lumen after the intervention.

Alloys↗

Off-pump replacement of the pulmonary valve in large right ventricular outflow tracts: a hybrid approach.

BACKGROUND: Percutaneous pulmonary valve replacement has recently been introduced and is under investigation in humans. This technique is, however, limited to patients with a right ventricular outflow tract that does not exceed 22 mm in diameter. We report our experience of off-pump pulmonary valve replacement using a hybrid approach in animals with large right ventricular outflow tracts. METHODS: Eight ewes were included in the protocol and were equally divided into 2 groups. A left thoracotomy was first performed, and the main pulmonary artery was banded by using 2 radiopaque rings with a diameter of 18 mm that allowed for further pulmonary valve replacement. We then intended to implant a valved stent either percutaneously (group 1) or through a transventricular approach (group 2). All animals were killed after valve implantation. The operation allowed the pulmonary diameter to be reduced from 30 to 17.6 mm. RESULTS: The right ventricular pressure did not significantly increase after reduction of the pulmonary artery diameter (25 vs 36 mm Hg). Subsequent pulmonary valve replacement through a percutaneous or a transventricular approach was always possible without any requirement for extracorporeal circulation. All devices were successfully delivered inside the pulmonary artery banding and were functioning perfectly at early evaluation. CONCLUSIONS: Implantation of a pulmonary valve is possible in ewes through a hybrid approach when the right ventricular outflow tract exceeds 22 mm in diameter. This involves both surgeons and interventionists and allows for a staged procedure in which the valvulation is performed percutaneously or, for a combined hybrid approach, in which the valve is implanted off pump transventricularly during the same operation.

Animals↗

Percutaneous balloon pulmonic valvuloplasty following treated endocarditis in a patient with congenital pulmonary valve stenosis.

A 36-year-old woman with congenital pulmonary valve stenosis developed the rare complication of endocarditis of the valve. After successful sterilization of the valve, the patient underwent percutaneous balloon pulmonic valvuloplasty at a later date. The procedure successfully reduced the peak pulmonary valve gradient from 94 to 45 mm Hg. Percutaneous balloon valvuloplasty is the procedure of choice for treatment of congenital pulmonary valvular stenosis, even in the unusual patient who has healed endocarditis of the pulmonary valve.

Adult↗

[Pulmonary valve replacement for endocarditis. Apropos of 2 cases].

The authors report two cases of pulmonary valve endocarditis which required emergency surgical treatment. A 74 year old patient with trivalvular endocarditis (pulmonary, aortic, mitral), due to Sptreptococcus D bovis, developed cardiogenic shock with acute pulmonary oedema and underwent double aortic and pulmonary valve replacement with Carpentier-Edwards prostheses and simple resection of a mitral valve vegetation. Another 36 year old drug addict developed isolated pulmonary valve endocarditis due to Staphylococcus aureus infection complicated by pulmonary regurgitation with right ventricular failure and by septic pulmonary embolism with persistent sepsis: he underwent pulmonary valve replacement with a Bravo 300 bioprosthesis. The postoperative course was uncomplicated in both cases, with interruption of the infection and normalisation of the haemodynamic status. The insidious and severe nature of pulmonary valve endocarditis is demonstrated by these two cases, confirming previous reports which have underlined the poor prognosis of this condition. Surgery has been shown to be effective and well tolerated and should be integrated early in the therapeutic strategy, the results being all the better when an aggressive attitude is taken.

Adult↗

A simple method for ECG and VCG assessment of the severity of pulmonary valve stenosis.

In patients with isolated pulmonary valve stenosis, the right ventricular (RV) hypertrophy is related to the hemodynamic severity of their lesions. We estimated the peak RV pressure noninvasively in 112 patients with isolated pulmonary valve stenosis by a Frank lead vectorcardiogram (VCG), an orthogonal electrocardiogram (ECG), and a standard scalar ECG. In 78 randomly assigned patients, 10 ECG measurements and 13 VCG measurements were correlated with the peak RV pressure at cardiac catheterization; the best multiple linear regression equation used the R wave in standard lead 1 (ECG), maximal rightward voltage in the terminal part of the frontal vector loop (VCG), and the Q wave in the orthogonal Z lead, giving R = 0.66 (SEM 25). The equation was validated in the other 34 patients; correlation of estimated with actual peak RV pressure was r = 0.64. A nomogram for clinical use was devised from this regression equation.

Adolescent↗

Physiologic correlates of echocardiographic pulmonary valve motion in diastole.

It has been shown that the echographic motion of the posterior aortic wall in diastole is closely related to the underlying left atrial events, possibly due to the anatomical proximity of the two structures. We observed that the pulmonary artery shares a similar close anatomical relationship with the left atrium. The present study in 55 consecutive patients with adequate echocardiographic recordings of the aortic root and the pulmonary valve demonstrates that the diastolic waveforms of the pulmonary valve and the posterior aortic wall are nearly identical in early diastole. The pulmonary valve e-f slope correlated with the posterior aortic wall O-R slope (r = 0.95) and the S2-f interval (second heart sound to f on the pulmonary valve) correlated closely with the S2-R interval (second heart sound to R on the posterior aortic root) (r = 0.94)). No significant correlation was found between the pulmonary valve e-f slope and the pulmonary artery pressures. The presence and amplitude of the maximum "a dip," on the other hand, correlated with the presence of pulmonary hypertension, with some notable exceptions. In addition, the "a dip" on the pulmonary valve and the depth of the A wave on the posterior aortic wall were significantly correlated (r = 0.85). It appears that the "a dip" on the pulmonary valve is influenced by dual mechanisms: pressure differences between the pulmonary artery and the right ventricle in late diastole and the left atrial events. Thus, the early diastolic waveform of the pulmonary valve, like the posterior aortic wall waveform, may primarily reflect underlying left atrial events and is not a measure of pulmonary artery pressure. The late diastolic waveform "a dip" has a dual mechanism, related in part to the underlying left atrial events, and also reflecting the instantaneous pressure differences across the pulmonary valve following atrial systole.

Adolescent↗