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At least 343 records · Page 19Linked to original sources
PubMed1994

Percutaneous transluminal balloon valvuloplasty for pulmonary valve stenosis: report on six cases.

Percutaneous transluminal baloon valvuloplasty is currently the treatment of choice for most cases of pulmonary valve stenosis. In the first series of cases performed at Kenyatta National Hospital, six patients aged 4 to 24 years with severe pulmonary valve stenosis and no other associated cardiac lesions were selected for the procedure. Immediately following baloon valvuloplasty, the pressure gradients across pulmonary valve measured by both echo-Doppler technique and cardiac catheterisation dropped very significantly (P < 0.001). Catheterisation peak systolic gradients (psg) dropped from 162.5 +/- 23.7 to 56.5 +/- 19.0 while echo-Doppler pressure gradients dropped from 112.0 +/- 11.9 to 42.8 +/- 16.0. No complications occurred during or after the procedure. This initial short-term experience in our set-up confirms the safety and effectiveness of this procedure. Furthermore, this procedure is much cheaper and technically easier to perform than cardiac surgery.

Adolescent↗
PubMed2005

Total percutaneous correction of a tetralogy of Fallot variant with dominant pulmonary valve stenosis.

OBJECTIVE: To study the feasibility, efficacy, and safety of total percutaneous correction of a tetralogy of Fallot variant with dominant pulmonary valve stenosis. DESIGN: Percutaneous correction of a variant of tetralogy of Fallot with dominant pulmonary valve stenosis, on the basis that there are transcatheter methods for the correction of malalignment-type ventricular septal defect (VSD) (transcatheter patch) and valvar pulmonary stenosis (balloon valvoplasty). PATIENTS: Two patients with tetralogy of Fallot, 4 and 7 years old, were admitted for percutaneous correction. Their aortic saturations were 72% and 88%. Both had severe right ventricular outflow obstruction with dominant valvar pulmonary stenosis with total gradients of 120 and 70 mm Hg. Large malalignment subaortic VSDs, 14 and 16 mm in diameter, were present. The first patient had a previous percutaneous correction of a small atrial septal defect and an aortopulmonary collateral. INTERVENTIONS: Balloon valvoplasty was first performed, followed by balloon test occlusion of the VSD and double balloon patch occlusion. Forty eight hours after implantation the supporting balloons were extracted, releasing the patches. RESULTS: Both patients became acyanotic with oxygen saturations of 96%. There was mild residual infundibular stenosis with 40 and 30 mm Hg gradients. Both VSDs were effectively occluded with only trivial residual shunts. One patient developed mild haemolysis, which resolved spontaneously in a few days. Both patients were doing well at six and 12 months' follow up visits. CONCLUSIONS: Total percutaneous correction of the tetralogy of Fallot variants with dominant pulmonary valve stenosis is feasible and successful. Larger clinical trials are required to further assess effectiveness and safety.

Catheterization↗
PubMed1995

A bicuspid pulmonary valve is not a contraindication for the arterial switch operation.

There are no obvious criteria concerning the optimal repair for complete transposition with bicuspid pulmonary valve if neither the organic changes in the valve nor the pressure gradient between the left ventricle and the pulmonary trunk are severe. Instead of intraatrial switching or intraventricular rerouting in such circumstances, we have proceeded to the arterial switch procedure in 6 patients with an adequate diameter of the pulmonary valve (greater than 100% of the calculated normal aortic orifice). Postoperative catheterization (at approximately 8 months after the procedures) showed no pressure gradient between the left ventricle and the neoaorta except for a finding of 34 mm Hg difference in 1 patient who had undergone simultaneous subpulmonary myotomy. Echocardiography (7 years later in the longest follow-up) has shown no more than slight regurgitation across the bicuspid neoaortic valve, with no progressive increase of blood velocity across the valve. From these results in the middle term, we conclude that the arterial switch procedure remains an option of choice for patients with initially bicuspid pulmonary valve, providing there is no severe subpulmonary stenosis.

Aorta↗
PubMed2002

Right ventricular dysfunction and pulmonary valve replacement after correction of tetralogy of Fallot.

BACKGROUND: Correction of tetralogy of Fallot often leads to pulmonary regurgitation, sometimes warranting pulmonary valve replacement, for which indications and timing to achieve optimal results are not yet clear. This retrospective study describes follow-up and reinterventions in our tetralogy of Fallot population. METHODS: Review of all consecutive patients operated on for tetralogy of Fallot between 1977 and 2000 was conducted. Included are date and type of repair, Doppler echocardiography (two-dimensional echocardiography), electrocardiographs, reoperations, and physical condition. RESULTS: Total repair was performed in 171 patients at a mean age 1.9 +/- 2.5 years, follow-up time counted 9.6 +/- 7.0 years. Right ventriculotomy was used in 92%, and transatrial ventricular septal defect closure was used in 8%; 74% received a transannular outflow patch. Twenty-year survival was 91%. Last follow-up electrocardiographs showed right bundle branch block in 67% and serious arrhythmias in 11%. Two-dimensional echocardiography demonstrated severe pulmonary insufficiency and dilated right ventricle in 31% and 38%, respectively, increasing with postrepair age (p < 0.001). Poor clinical condition (New York Heart Association class II+) and echocardiographic proof of right atrial dilatation (p = 0.012) and arrhythmias (p = 0.03) were significantly associated. Furthermore, the influence of residual hemodynamic lesions, such as a remaining ventricular septal defect or pulmonary stenosis, or right ventricular dilatation was important (p = 0.04). Reintervention was necessary in 32 patients (19%; 10-year freedom, 83%), including angioplasty for residual stenosis and pulmonary valve replacement. At a mean age of 9.2 years after correction, 14 patients received a homograft, and 2 patients received a heterograft. In 7 patients the right ventricle returned to normal dimensions and symptoms disappeared. The incidence of right ventricular dilatation was considerably higher (p = 0.020) in patients with a transannular patch; the transatrial approach showed the opposite (p = 0.03), and patients presented with lower QRS duration (p = 0.007), although no difference could be found between survival after both surgical techniques. Effects of early timing (correction < 6 months) on right ventricular dysfunction could not be established. CONCLUSIONS: Severe right ventricular dilatation and pulmonary regurgitation secondary to outflow tract repair in tetralogy of Fallot are frequently occurring sequelae developing slowly over time. Indications for pulmonary valve replacement remain controversial because echocardiographic findings or arrhythmias are not always accompanied by deterioration of clinical condition. However, right atrial dilatation and additional hemodynamic lesions demand increased vigilance. Transatrial repair is associated with a favorable outcome.

Adolescent↗
PubMed2000

Familial absent pulmonary valve syndrome without deletions of chromosome 22q11.

Deletions of chromosome 22q11 are common in patients with tetralogy of Fallot, and in those with absent pulmonary valve syndrome. In this report, we describe a pair of siblings with absent pulmonary valve syndrome, neither of whom had deletions of chromosome 22q11. The finding of familial absent pulmonary valve syndrome without deletion of 22q11 in our patients suggests an alternative genetic basis for this rare condition.

Child, Preschool↗
PubMed1987

Double-chambered right ventricle with pulmonary valve endocarditis.

A rare case of isolated pulmonary valve endocarditis associated with a double-chambered right ventricle in a nonaddict, was diagnosed on two-dimensional echocardiography and cardiac catheterization and angiography. The patient was successfully operated and the above findings confirmed on the operation table.

Cardiac Catheterization↗
PubMed1979

Reconstruction of the right ventricular outflow tract including pulmonary valve replacement in 6 children under two years of age.

In 6 children under 2 years of age correction of congenital heart defects required reconstruction of the right ventricular outflow tract including replacement of the pulmonary valve. Outflow tract reconstruction consisted in implantation of a size 14 valved conduit in 2 patients with d-TGA and subpulmonary stenosis and 1-TGA and subpulmonary stenosis, and a size 16 valved conduit in 2 other patients with truncus arteriosus. In 2 children with DOLV, VSD and aneurysm of the pulmonary artery trunk, the pulmonary valve was replaced by porcine heterografts, sizes 19 and 21 respectively, after primary patch reconstruction of the right ventricular outflow tract. There was one operative death in a child with d-TGA, intact ventricular septum and severe subpulmonary stenosis. This child died in low cardiac output, probably because too much contractile muscle was lost at the site of anastomosis with the conduit. For reconstruction of the right ventricular outflow tract, pulmonary valve replacement has proven mandatory in cases with pulmonary hypertension in order to prevent postoperative right heart failure. Similarly, in cases with pulmonary stenosis and hypoplastic pulmonary arteries, postoperative pulmonary insufficiency can be deleterious to the right ventricle.

Aneurysm↗
PubMed1983

Tetralogy of Fallot with absent pulmonary valve: definitive diagnosis by two-dimensional echocardiography.

A case is presented of a 27-day-old boy with Tetralogy of Fallot and absent pulmonary valve. The two-dimensional echocardiographic examination demonstrated: subaortic ventricular septal defect, overriding aorta, absence of the pulmonary valve with restrictive anulus, and massive dilatation of the pulmonary artery. These anatomic details were then confirmed by angiography and surgery. The echocardiographic features of Tetralogy of Fallot with absent pulmonary valve are typical, and allow the correct diagnosis to be made noninvasively. The haemodynamic investigation becomes obviously easier, and angiocardiography may be devoted to the study of possible additional defects, that are difficult to detect by echocardiography, like multiple ventricular septal defects, or peripheral pulmonary branch anomalies.

Echocardiography↗
PubMed1983

Surgical approach to critical pulmonary valve stenosis in infants less than six months of age.

During the past 7 1/2 years, 36 infants (less than or equal to 6 months of age) underwent surgical relief of critical pulmonary valve stenosis. The results of closed and open (cardiopulmonary bypass, CPB) procedures were compared. Closed procedures included Brock valvotomy (two patients) and normothermic inflow stasis with transpulmonary valvectomy (10 patients). Twenty-two patients had open procedures with valvectomy alone (five patients) and right ventricular outflow tract (RVOT) reconstruction (17 patients). Two additional patients had staged operations (shunt followed by open procedure at 3 and 5 months postoperatively). Patients who had the closed procedures, as compared with CPB, were significantly younger (10 versus 61 days, p less than 0.01) and more critically ill with congestive heart failure and acidosis. In addition, the mean peak systolic right ventricular-left ventricular pressure ratios (RVP/LVP) were higher (1.6 versus 1.4, p less than 0.02) in the closed group. There were no deaths in the closed group in contrast to six deaths among those having RVOT reconstruction (0/12 versus 6/17 or 35%, p less than 0.04). Critical obstruction of the pulmonary valve, tricuspid valve regurgitation, and hypoplastia of the right ventricle occurred more frequently in the closed group. All operative deaths resulted from right heart failure. There were no late deaths in the entire series. Three patients in the closed group (mean follow-up 2.8 years) required operations for recurrent pulmonary valve stenosis approximately 2 years postoperatively, and none in the open group (mean follow-up 2.0 years). In conclusion, despite current advances in infant CPB and care, the results from our experience suggests that closed procedures, especially inflow stasis and pulmonary valvectomy, are superior to the use of CPB and RVOT reconstruction, even in the presence of pulmonary valve dysplasia.

Blood Gas Analysis↗
PubMed2007

Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve.

OBJECTIVE: Tetralogy of Fallot and absent pulmonary valve (TOF/APV) is associated with significant pulmonary artery dilatation and airway compression. Treatment of infants presenting with respiratory symptoms early in life is associated with high mortality (20-60%). We aim to report our results and identify factors associated with survival and prolonged ventilation. METHODS: We performed a retrospective review of 62 consecutive patients following repair of TOF/APV (1982-2006). Median age at repair was 1.4 years (1 day-35 years). Twenty patients required preoperative intubation. RESULTS: Sixty-one patients underwent complete repair. Thirty-three patients underwent pulmonary artery plication (n=15) or reduction (n=18). The right ventricular outflow tract (RVOT) was reconstructed with valved conduit (n=31), bioprosthetic valve (n=18), monocusp (n=8), or transannular patch (n=4). There were three perioperative and five late deaths. All perioperative deaths were in neonates and before 1995. Five- and ten-year survival was 93+/-4% and 87+/-5%. Mean ventilatory requirements for neonates, infants, and children > or =1 year were 36+/-35, 8+/-8, and 2.6+/-2.4 days (p<0.0001). On multivariable analysis, significant factors associated with prolonged ventilation were neonates (p<0.0001) and preoperative mechanical ventilation (p=0.088). Eight airway reinterventions were needed in seven infants with persistent postoperative airway compromise, pulmonary artery suspension (n=4), innominate artery suspension (n=2), and lobectomy (n=2). Freedom from RVOT reoperation was 89+/-5% and 59+/-9% at 5 and 10 years. There were no significant risk factors for time-related survival or RVOT reoperation on multivariable analysis. CONCLUSIONS: In contrast to children and adults with TOF/APV, neonates and small infants presenting with respiratory symptoms require prolonged ventilation and additional reinterventions for airway compression. Our current surgical approach which includes reduction and suspension of pulmonary arteries, reconstruction of a competent RVOT, and aggressive postoperative ventilatory management to relieve airway obstruction offers satisfactory outcomes.

Abnormalities, Multiple↗
PubMed1997

Single-stage balloon valvuloplasty for critical pulmonary valve stenosis in the neonate.

Balloon valvuloplasty (BV) in neonates with critical pulmonary valve stenosis (CPVS) is limited by technical considerations, mainly the difficulty of traversing the stenotic valve. To simplify the procedure we used a 4F Cobra Type I catheter to cross the pulmonary valve (PV) without the aid of a guidewire, and performed single-stage BV, using low-profile balloons, in 12 neonates with CPVS. Procedure and total fluoroscopy times were 69 +/- 33 min (42-125 min) and 34 +/- 19 min (20-58 min), respectively. Following BV, right ventricular systolic pressure (RVSP) decreased from 102 +/- 17 mm Hg to 56 +/- 15 mm Hg (p < 0.001); and the ratio of RVSP to aortic systolic pressure decreased from 1.39 +/- 0.22 to 0.73 +/- 0.21 (p < 0.001). No significant complications were observed. BV failed in two patients, who subsequently had surgery. At follow-up (a mean of 19 months), Doppler gradient was 19 +/- 12 mm Hg (0.50 mm Hg). BV was repeated in one patient. We conclude that in neonates with CPVS, the use of the 4F Cobra type I catheter facilitates crossing of the PV and allows performance of BV in a single stage; this enhances safety and time-efficiency, and shortens exposure to radiation.

Cardiac Catheterization↗
PubMed2006

Correction of tetralogy of fallot with absent pulmonary valve syndrome in a young infant using a bicuspid equine pericardial tube.

Absent pulmonary valve syndrome (APVS) is an uncommon variant of tetralogy of Fallot (TOF), which manifests morphologically as vestigial pulmonary valve cusps at the right ventricle-pulmonary trunk junction. The aneurysmally dilated pulmonary arteries may compress the tracheobronchial tree and cause severe respiratory distress in the neonatal or infant stage. Early surgical correction in these patients is necessary despite the high operative mortality rate. A 1-day-old male neonate suffered from progressive shortness of breath after birth. Echocardiography confirmed the diagnosis of TOF with APVS. The marked dilatation of pulmonary arteries resulted in airway compression in addition to heart failure. Total surgical correction was performed at 40 days of age, using a homemade bicuspid equine pericardial tube for right ventricular outflow reconstruction. The short-term follow-up echocardiogram demonstrated good motility of the pericardial leaflet. However, patients receiving this type of valved conduit require meticulous long-term follow-up.

Cardiac Surgical Procedures↗
PubMed1993

[A case of two-chambered right ventricle associated with severe pulmonary valve regurgitation].

A 40-year-old female was admitted to the hospital with the chief complaints of dyspnea on effort. Echocardiography and cardiac catheterization revealed an abnormal muscle bundle in the right ventricle which divided right ventricle into two chambers with the pressure gradient of 86 mmHg between them. And severe regurgitation associated with hypoplastic pulmonary valve and ventricular septal defect were noted. We removed the abnormal muscle bundle through the right atrial and ventricular approach, and pulmonary valve replacement and VSD closure were carried out. We report a case of two-chambered right ventricle with severe pulmonary valve regurgitation because of its rarity.

Adult↗
PubMed1988

The Ross operation: the autologous pulmonary valve in the aortic position.

Aortic valve replacement (AVR) with a pulmonary valve autograft (PVA) was first reported by Donald N. Ross (DNR) in 1967. The expectation of this procedure was to avoid degenerative changes seen in other biological tissue valves such as calcification, attenuation, and rupture of the leaflets. Recent reports by the original investigator's group have confirmed the lack of degenerative changes in PVA. To corroborate their conclusions, the fate of 12 patients undergoing AVR with PVA by one of us (LGL) has been ascertained. From March 1969 to June 1971, 12 patients underwent AVR with PVA. The right ventricular outflow tract (RVOT) was reconstructed with an aortic homograft valved conduit. The mean age was 42.7 years (range 21 to 52 years). The mean follow-up for 11 hospital survivors is 12.4 years. Three PVA have been replaced; one following infective endocarditis at 13 years, and two at 15 and 73 months due to technical malalignment. There was no evidence of PVA degeneration during histological examination of these explanted PVAs. Six patients are alive and retain the original PVA at 12 years (55%). This analysis corroborates the conclusions of the DNR report and strongly suggests an immunological mechanism in the process of calcification of other biological tissue valves. The Ross operation is advocated for AVR in young patients as valve durability is of paramount importance especially in this group.

Adult↗
PubMed2001

Transposition with absent pulmonary valve syndrome: early repair of a rare case.

The congenital absence of the pulmonary valve cusps can occur either isolated or in association with other heart lesions. We report a very rare case of a 40-day-old infant with transposition of the great arteries, ventricular septal defect, pulmonary annular stenosis, absent pulmonary valve and aneurysmal dilation of the central pulmonary arteries, who received surgical treatment at our institution.

Blood Vessel Prosthesis Implantation↗
PubMed2006

Current experience with percutaneous pulmonary valve implantation.

Transcatheter valve replacement has recently been introduced into clinical practice and has the potential to transform the management of valvular heart disease. To date, the largest human experience exists with percutaneous pulmonary valve implantation in patients with repaired congenital heart disease who require re-intervention to the right ventricular outflow tract. The application of this approach, however, is presently restricted to certain right ventricular outflow tract morphologies, because the device needs to be anchored safely to prevent device dislodgement. Early results of percutaneous pulmonary valve implantation show lower morbidity than surgery and significant early symptomatic improvement. In the future, the challenge will be to extend percutaneous pulmonary valve implantation to all patients with a clinical indication to delay or avoid repeat open-heart surgery.

Coronary Angiography↗