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Congenital absence of the pulmonary valve associated with pulmonary stenosis, large ductus arteriosus and intact ventricular septum. Case report.

A six-day-old neonate was diagnosed with a severe form of the syndrome of absent pulmonary valve associated with pulmonary stenosis, aneurysmal dilatation of the pulmonary artery and rare findings including an intact ventricular septum and large ductus arteriosus. The patient underwent surgical repair by closed technique. Cardiac catheterization data, hemodynamic and clinical findings, and surgical technique are reported. Congenital absence of the pulmonary valve is a rare cardiac anomaly. An especially severe form occurs with ventricular septal defect and pulmonary stenosis. The usual findings are respiratory distress, aneurysmal dilatation of the pulmonary arteries and pulmonary stenosis.

Ductus Arteriosus, Patent↗

[Konno procedure for congenital aortic valve stenosis with pulmonary valve stenosis].

Although aortic and pulmonary valve stenosis are among the most common congenital heart defects, the combination of both aortic and pulmonary valve stenosis in the same patient appears to be very uncommon. Accurate diagnosis of combined valvular stenosis is imperative prior to surgical correction, otherwise surgery of one of the lesions may result in an insufficient hemodynamic improvement. A seven-year-old girl with congenital aortic and pulmonary valve stenosis associated with hypoplastic aortic annulus underwent Konno's operation, pulmonary valvotomy and resection of anomalous muscle of the right ventricular outflow tract simultaneously. The operation was successfully performed and postoperative course was uneventful.

Aortic Valve Stenosis↗

Comparison of biomechanical and structural properties between human aortic and pulmonary valve.

OBJECTIVE: Pulmonary valve autografts have been reported as clinically effective for replacement of diseased aortic valve (Ross procedure). Published data about pulmonary valve mechanical and structural suitability as a long-term substitute for aortic valve are limited. The aim of this study was to compare aortic and pulmonary valve properties. METHODS: Experimental studies of biomechanical properties and structure of aortic and pulmonary valves were carried out on pathologically unchanged human heart valves, collected from 11 cadaveric hearts. Biomechanical properties of 84 specimens (all valve elements: cusps, fibrous ring, commissures, sinotubular junction, sinuses) were investigated using uniaxial tensile tests. Ultrastructure was studied using transmission and scanning electron microscopy. RESULTS: Ultimate stress in circumferential direction for pulmonary valve cusps is higher than for aortic valve (2.78+/-1.05 and 1.74+/-0.29 MPa, respectively). Ultimate stress in radial direction for pulmonary and aortic cusps is practically the same (0.29+/-0.06 and 0.32+/-0.04 MPa, respectively). In ultrastructural study, different layout and density in each construction element are determined. The aortic and pulmonary valves have common ultrastructural properties. CONCLUSIONS: Mechanical differences between aortic and pulmonary valve are minimal. Ultrastructural studies show that the aortic and pulmonary valves have similar structural elements and architecture. This investigation suggests that the pulmonary valve can be considered mechanically and structurally suitable for use as an aortic valve replacement.

Adult↗

Use of the Terumo SP catheter system for crossing the pulmonary valve in infants with critical pulmonary valve stenosis.

In short, this method (the Terumo SP hydrophilic-polymer coated microcatheter system) has proved to be safe, effective, and flexible; it has allowed us to cross critically stenotic valves when standard methods have failed, enabling us to accomplish balloon valvuloplasty with good results in patients who might have otherwise required surgical intervention because of inability to cross the pulmonary valve.

Cardiac Catheterization↗

Pulmonary valve annulus grows after balloon dilatation of neonatal critical pulmonary valve stenosis.

BACKGROUND: Neonates with critical pulmonary valve stenosis often demonstrate small or hypoplastic right ventricular structures. Relief of the obstruction enhances forward flow across the right ventricle and reduces its pressure load. Growth of the right ventricle and especially of the pulmonary valve annulus was evaluated after balloon dilatation. METHODS: Ten consecutive neonates with critical pulmonary valve stenosis who underwent balloon valvuloplasty were studied by serial echocardiography to assess growth of right ventricular structures at follow-up. RESULTS: The mean diameter of the pulmonary valve annulus increased from 6.1 +/- 1.4 mm to 12.6 +/- 3.5 mm (z scores from -2.9 +/- 1.0 SD to - 1.3 +/- 1.2 SD, p < 0.0001) after a mean follow-up period of 2.7 +/- 2.0 years. The mean diameter of the tricuspid valve annulus increased from 12.9 +/- 3.8 mm to 19.0 +/- 3.1 mm; however, the respective z score did not change significantly (from 0.5 +/- 2.4 SD to -0.5 +/- 1.0 SD). Right ventricular cavity size was hypoplastic in four patients initially and normal in all patients at latest follow-up. CONCLUSIONS: Balloon dilatation of critical pulmonary valve stenosis encourages catch-up growth of the pulmonary valve, and surgery may be avoided even in a hypoplastic pulmonary valve annulus.

Catheterization↗

Pulmonary valve morphology after balloon dilatation of pulmonary valve stenosis.

Percutaneous balloon valvuloplasty (BV) for pulmonic valve stenosis (PS) is increasingly becoming a nonsurgical alternative in patient management. However, the mechanism by which BV dilates the obstructive lesion has not been firmly established. We have had the opportunity to examine the effects of BV in the setting of PS and present two illustrative cases documenting the morphology after BV of the stenotic bicuspid and tricuspid pulmonary valve.

Adolescent↗

Pulmonary valve echo motion in pulmonary regurgitation.

Four cases are presented to illustrate the echo patterns of pulmonary valve motion in patients with pulmonary regurgitation caused by pulmonary hypertension, idiopathic dilatation of the pulmonary artery, or congenital absence of the pulmonary valve or in association with pulmonary stenosis. Absence of the pulmonary 'a' wave, fluttering of the e-f slope, and midsystolic closure or 'notching' of the valve were noted with pulmonary hypertension. In the case with idiopathic dilatation of the pulmonary artery a normal echo pattern of pulmonary valve motion along with distinct dilatation of the pulmonary artery at the valvular level were present. Pronounced dilatation and systolic expansion of the pulmonary artery along with dilatation of the right ventricle were seen with congenital absence of the pulmonary valve. No pulmonary valve could be demonstrated on multiple scans from the right ventricle to pulmonary artery. In Case 4 large 'a' waves (14 mm) were noted, indicating a reversal of the normal end-diastolic gradient across the valve and suggesting that pulmonary regurgitation in this case was associated with right ventricular outflow obstruction. Study of the echo pattern of pulmonary valve motion may therefore provide useful information in establishing the cause of pulmonary regurgitation.

Adolescent↗

Surgical management of patients with pulmonary valve dysplasia.

Pulmonary valve dysplasia is a distinct pathological entity consisting of markedly thickened, deformed, and largely immobile pulmonary valve leaflets. The clinical features and surgical management of 21 consecutive patients operated upon between 1975 and 1985 were reviewed. Mean age at operation was 26.7 months (range: 1 month to 9.5 years). Initial surgical intervention in 3 patients consisted of closed valvotomy or systemic pulmonary artery shunt. One of these patients subsequently had a partial pulmonary valvectomy; the other 2 had total valvectomy and pulmonary annuloplasty. Two patients had partial valvectomy as a primary procedure, and 1 has subsequently required total valvectomy and annuloplasty. Sixteen patients initially had total pulmonary valvectomy, 4 with a simultaneous transannular outflow tract patch. One patient subsequently required a transannular outflow tract patch. Therefore, definitive repair consisted of partial valvectomy in 2 patients, total valvectomy in 19, and a transannular outflow tract patch in 9 patients with a hypoplastic pulmonary annulus. There were no operative deaths, and all patients are asymptomatic at a mean follow-up of 37.9 months. Five patients who underwent total valvectomy were catheterized postoperatively. The mean residual gradient was 25 mm Hg. Pulmonary valvectomy appears to provide both effective relief of stenosis and long-term asymptomatic status in patients with pulmonary valve dysplasia. Total excision of all valve leaflets is recommended. Careful consideration should be given to a simultaneous transannular outflow tract patch if the annulus is hypoplastic.

Child↗

Improved right ventricular function following late pulmonary valve replacement for residual pulmonary insufficiency or stenosis.

Residual severe pulmonary insufficiency or stenosis may result in significant myocardial dysfunction late after repair of tetralogy of Fallot. Although pulmonary valve replacement has been advocated for selected patients, objective improvement in right ventricular function has been difficult to demonstrate. We undertook pulmonary valve replacement in 11 patients to treat residual insufficiency (n = 8) or stenosis (n = 3) and evaluated them before and after operation by radionuclide ventriculography and M-mode echocardiography. Patients' age at the original repair was 6.6 +/- 0.6 years (range 2 to 8 years) and at subsequent valve replacement was 14.6 +/- 1.5 years (range 5 to 20 years). Indications for pulmonary valve replacement were conduit stenosis indicated by a gradient greater than or equal to 75 mm Hg (n = 3), symptoms (n = 2), progressive cardiomegaly (n = 3), and new onset of tricuspid insufficiency (n = 3). Prior to pulmonary valve replacement, right ventricular ejection fraction was 0.29 +/- 0.12 (range 0.12 to 0.48) and rose to 0.35 +/- 0.10 (range 0.19 to 0.48) at a mean of 10.5 +/- 2.3 months after operation (p less than 0.05). Improvement (defined as an increase in ejection fraction greater than 0.05) was noted in seven patients whereas four demonstrated no change. Left ventricular ejection fraction before operation (0.55 +/- 0.12) was unchanged after pulmonary valve replacement (0.54 +/- 0.06). M-mode echocardiography demonstrated significant reduction in right ventricular dilatation. Right ventricular/left ventricular end-diastolic dimension fell from 1.03 +/- 0.30 to 0.73 +/- 0.13 after operation (p less than 0.01). Cardiothoracic ratio fell from 0.59 +/- 0.02 to 0.55 +/- 0.02 at a mean of 12 months after pulmonary valve replacement (p less than 0.01). Subjective improvement in exercise tolerance was noted in all seven patients who showed an increase in right ventricular ejection fraction. Of the remaining four patients, two had no improvement, one felt symptomatically improved, and one was too young for evaluation. These data demonstrate objective improvement in right ventricular function following pulmonary valve replacement and confirm the usefulness of this procedure in patients with significant right ventricular dysfunction secondary to residual pulmonary insufficiency and stenosis.

Adolescent↗

[Transthoracic and transesophageal echocardiographic study in aortic valve replacement with pulmonary valve autograft].

Eleven patients who underwent pulmonary valve autograft to aortic position with placement of bovine pericardial prosthesis in pulmonary position were studied with echocardiography. Transthoracic echocardiography, was performed on all patients before surgery. Transesophageal echocardiograms were practiced during the surgical procedure. The latter technique aided in immediate postoperative evaluation. Transthoracic and transesophageal echocardiography provide a practical and valuable means of investigating the pre, trans and postoperative conditions of patients undergoing Ross surgery.

Adult↗

The pulmonary valve. Is it mechanically suitable for use as an aortic valve replacement?

Pulmonary autografts have shown a low incidence of early failure and late structural deterioration when placed in the aortic position yet the potential value of the pulmonary valve as a replacement device has not been widely considered. Since the mechanical suitability of pulmonary valves for the high stress aortic position is unclear, we set out to define and compare the tensile mechanical properties of these two valves. We removed all 72 cusps from 12 fresh porcine aortic and pulmonary valves. Eighteen cusps from each of the two groups were fixed flat in 0.25% glutaraldehyde for greater than 24 hr. Circumferential or radial strips were cut from each cusp creating eight test groups, each with nine specimens. Stress-strain curves for each specimen were obtained using an Instron tensile testing machine. Stress-strain curves were obtained at 2, 10, 50, and 200 mm/min strain rates, each specimen was then strained to fracture. We found pulmonary leaflets were thinner than aortic leaflets (0.49 mm versus 0.67 mm) and glutaraldehyde fixation did not affect this relationship. The elastic moduli were comparable within the fresh and glutaraldehyde fixed treatment groups and within the circumferential and radial strip groups. Radial strips were more extensible than circumferential strips. Tissue viscous properties were similar and glutaraldehyde fixation produced minimal changes in stress relaxation rates. Tissue fracture tests emphasized tissue anisotropy with the failure point depending upon strip orientation. Glutaraldehyde had no effect on fracture stress but strain at fracture doubled due to increased collagen fiber crimping during fixation.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Lack of correlation between echocardiographic pulmonary valve morphology and simultaneous pulmonary arterial pressure.

Pulmonary valve echograms recorded simultaneously with right heart pressures were correlated with mean and end-diastolic pulmonary arterial pressures and the peak magnitude of the right atrial a wave in an attempt to predict noninvasively levels and changes in pulmonary arterial pressure. Satisfactory pulmonary valve echograms were obtained in 16 of 23 patients studied. No significant correlation was found between hemodynamic measurements and the echographic pulmonary valve a wave amplitude, diastolic E-F slope or the systolic opening B-C slope. Changes in hemodynamic measurements observed in serial observations were not associated with predictable changes in configuration of the pulmonary valve echogram. The combination of mid systolic pulmonary valve notching and an absent a wave was observed in more advanced degrees of pulmonary hypertension and was specific, but not sensitive, for that condition.

Adult↗