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At least 91 records · Page 5Linked to original sources
PubMed1998

Arterial switch in hearts with left ventricular outflow and pulmonary valve abnormalities.

BACKGROUND: Pulmonary valve and left ventricular outflow tract abnormalities (LVOT) may not be absolute contraindications to arterial switch operation (ASO). METHODS: In this study we analyze long-term outcome for 26 such transposition patients (6.3% of our ASO cohort). Median age and weight were 69 days (7 to 3,631 days) and 4.5 kg (2.6 to 34 kg). Pulmonary valve abnormalities included bicuspid valve (n = 4) and dysplastic valve (n = 5). The LVOT abnormalities (n = 17) included accessory atrioventricular valve/endocardial cushion tissue, fibromuscular ring, anomalous muscle bands, and septal malalignment. Patients with dynamic LVOT obstruction were excluded. The median preoperative left ventricular to pulmonary artery peak systolic pressure gradient was 30 mm (0 to 93 mm), or 50 mm (16 to 93 mm) if patients with isolated valve abnormalities are excluded. The ASO was performed according to our standard technique with or without LVOT resection or pulmonary valvotomy as required. RESULTS: There were two perioperative deaths (7.7%; 95% confidence interval, 0.9% to 25%), and no late deaths during 1,934 patient-months of follow-up time. Actuarial freedom from reoperation for neoaortic valve or LVOT problems is 87% (+/- 7) at 130 months, representing two reoperations. One was performed for neoaortic insufficiency plus LVOT obstruction, and the other for isolated LVOT obstruction. One patient currently has significant neoaortic insufficiency, and median gradient at last follow-up is 0 mm Hg (range, 0 to 35 mm Hg). CONCLUSIONS: The ASO can be performed in selected patients with transposition of the great arteries and with LVOT abnormalities with early and late survival and functional status similar to that of matched patients with normal pulmonary valves and LVOT (p > 0.05), but with a greater hazard for reoperation (p < 0.05). Selection for ASO should be based on anatomic criteria rather than left ventricular to pulmonary artery gradient alone, to avoid assigning these patients with transposition of the great arteries to treatment strategies less satisfactory than ASO.

Child↗
PubMedDate not supplied

[Non-surgical treatment of pulmonary valve stenosis].

Percutaneous pulmonary valve valvuloplasty was attempted 17 times in 16 infants and children aged 15 days to 18 years. Valvuloplasty was performed during cardiac catheterization without general anesthesia. Balloon catheter was positioned across the pulmonary valve using a guide wire previously introduced in the right or left pulmonary artery. All patients experienced a decrease in the right ventricular peak systolic pressure from 111.5 +/- 35.9 to 69 +/- 28.9 mmHg (p less than 0.001), a decrease in the ratio of right ventricular pressure to systemic systolic pressure from 1.08 +/- 0.34 to 0.65 +/- 0.28 (p less than 0.001) and a decrease in right ventricular to pulmonary artery gradient from 88.52 +/- 37.2 to 54.82 +/- 29.4 mmHg (p less than 0.001). A child with a history of postoperative seizures had convulsions after the procedure and died 72 hours later. Four other patients are considered as a failure of the technique, 4 had clear hemodynamic improvement but right ventricular pressure remained above 50 mmHg, and 8 had satisfactory relief of their stenosis with a right ventricular pressure below or equal to 50 mmHg and a right ventricular to pulmonary artery gradient below 30 mmHg. These good results were confirmed in 2 patients, one year after the dilatation. Percutaneous balloon valvuloplasty is probably now the best treatment for pulmonary valvular stenosis. Short-term and results at distance from the procedure are good, and morbidity and cost are less than those for a surgical cure.

Adolescent↗
PubMed1989

Successful palliation of the "absent" pulmonary valve syndrome by banding of the pulmonary trunk.

The "absent" pulmonary valve syndrome is associated with aneurysmal dilatation of the pulmonary trunk, stenosis of the ventriculo-arterial junction with or without malalignment of the outlet septum, and ventricular septal defect. When the outlet septum is malaligned, the morphology resembles that of tetralogy of Fallot. We report our experience with 4 infants with this syndrome. All were in severe respiratory distress and cardiac failure when first seen. Cardiac catheterization was performed at 0.5-4.5 months of age in 3 of them. In the other, the clinical and echocardiographic features were considered sufficient to establish the diagnosis. Banding of the pulmonary trunk was carried out at the age of 2.5-5 months. The distal pulmonary arterial pressure in 3 cases dropped to 12-19 mm Hg. These patients could be extubated within one week postoperatively. Their course 1-3 years later is excellent, with rare episodes of mild respiratory problems only and markedly diminished pulmonary insufficiency. One child, weighing 3250 g at surgery, whose pulmonary arterial pressure did not drop below 29 mm Hg, could not be weaned off the respirator. Corrective surgery was undertaken 17 days later, but the patient died of respiratory complications. Based on clinical and Doppler sonographic findings, on control catheterization data and on haemodynamic findings in 3 surviving infants and two further patients with an uneventful course who, as yet, have not undergone surgery, we conclude that the beneficial effect of banding is the combined result of reduced pulmonary arterial pressure and decreased pulmonic regurgitation.

Child, Preschool↗
PubMed1996

Tetralogy of Fallot with diminutive pulmonary arteries: preoperative pulmonary valve dilation and transcatheter rehabilitation of pulmonary arteries.

OBJECTIVES: This study sought to determine the results of a novel transcatheter management approach in tetralogy of Fallot with diminutive pulmonary arteries. BACKGROUND: Tetralogy of Fallot with diminutive pulmonary arteries and severe pulmonary stenosis is rare and resembles tetralogy of Fallot with pulmonary atresia: There is a high incidence of aortopulmonary collateral channels, arborization abnormalities, stenoses and need for multiple operations. Because a combined catheter-surgery approach facilitates repair in these patients, such an approach may benefit those with diminutive pulmonary arteries and pulmonary stenosis. METHODS: Clinical, catheterization and surgical data were studied retrospectively for 10 such patients undergoing preoperative pulmonary valve balloon dilation, among other transcatheter interventions, from January 1989 to January 1995. RESULTS: Initially, the Nakata index ranged from 20 to 98 mm2/m2 (mean 67 +/- 28 mm2/m2). The pulmonary valve was first balloon dilated (mean balloon/annulus 1.5 +/- 0.3), and the mean initial valve annulus Z score (-4.0 +/- 1) increased to -33 +/- 1.1 (p < 0.01) Other interventions included branch pulmonary artery balloon dilation (7 patients, 23 vessels) and coil embolization of aortopulmonary collateral channels (8 patients, 31 collateral channels). At preoperative follow-up catheterization, the mean pulmonary annulus Z score was -3.1 +/- 0.7, and the Nakata index increased to 143 +/- 84 mm2/m2 (p < 0.03). All patients underwent complete surgical repair successfully. At a mean follow-up period of 2.6 +/- 2 years, right ventricular pressure was < 70% systemic in all patients and < 50% systemic in seven. CONCLUSIONS: In patients with tetralogy of Fallot, severe pulmonary stenosis and diminutive pulmonary arteries, initial pulmonary valve balloon dilation increases the annulus Z score and anterograde pulmonary blood flow and facilities simultaneous coiling of aortopulmonary collateral channels and access for branch pulmonary artery dilation, all of which results in pulmonary artery growth, simplifying surgical management.

Abnormalities, Multiple↗
PubMed2002

Prenatal role of the ductus arteriosus in absent pulmonary valve syndrome.

Absent pulmonary valve with ventricular septal defect is associated with ductal agenesis and markedly dilated main and branch pulmonary arteries. The less common variant with intact ventricular septum generally exhibits a patent ductus and smaller branch pulmonary arteries, and may be associated with tricuspid atresia. We identified 7 patients with the prenatal diagnosis of absent pulmonary valve, 5 with ventricular septal defect (Group 1) and 2 with an intact ventricular septum (Group 2). Imaging, color Doppler, and pulsed-Doppler recordings were analyzed. The branch and main pulmonary arteries were measured and expressed as a ratio with the descending aorta. Pulmonary regurgitation time (PRT) and diastolic acceleration time (DAT) were derived, and DAT/PRT was calculated to characterize diastolic pulmonary flow. Group 1 patients all had a large ventricular septal defect, normal biventricular size and function, and dilated main and branch pulmonary arteries. Group 2 patients had dilated main but smaller branch pulmonary arteries, moderate right ventricular dilation with severe dysfunction, and limited or absent tricuspid inflow. Group 1 demonstrated shorter acceleration time and earlier peak velocity, resulting in a smaller DAT/RT ratio. We speculate that free communication between the fetal aorta and the ventricles may limit atrial inflow and elevate diastolic pressure, affecting cardiac output, ventricular function, and atrioventricular valve development. With an intact ventricular septum, these physiologic and anatomic repercussions are limited to the right ventricle, but with a ventricular septal defect, both ventricles would experience similar consequences and cardiac performance could be critically impaired.

Ductus Arteriosus, Patent↗
PubMed2002

Valved homograft replacement of aneurysmal pulmonary arteries for severely symptomatic absent pulmonary valve syndrome.

BACKGROUND: Patients with absent pulmonary valve syndrome (APVS) with respiratory distress (RD) have previously had a high mortality. In 1990 we adopted a strategy of primary repair including total replacement of the aneurysmal central pulmonary arteries (PAs) for patients with RD. METHODS: Retrospective review was made of 54 consecutive patients with APVS between 1960 and 1998. Median age and weight were 4 months and 4.8 kg. RD was present in 23 patients (10 neonates, 16 required ventilation). Fifteen patients had repair with homograft replacement of the PAs and VSD closure (group 1). Twenty-seven patients had transannular patch with VSD closure with PA-plasty (group 2, n = 21) or without PA plasty (group 3, n = 6). Twelve had miscellaneous procedures (group 4); in 6 the VSD was left open. RESULTS: Operative, 1-, 5-, and 10-year survivals were 83%, 80%, 78%, and 78%, respectively. Risk factors for operative mortality in multivariate analysis were RD (p = 0.04), neonates (p = 0.02), weight less than 3 kg (p = 0.02), open VSD (p = 0.02) and surgery before 1990 (p = 0.04). Since 1990 operative mortality has decreased to 11% (p = 0.04). RD was the only time-related predictor of survival in multivariate analysis (p = 0.004). In patients with RD, survival with homograft was 73% versus 41% with other techniques (p = 0.2). Mean follow-up was 72 +/- 50 months. There were no significant differences in freedom from reintervention rates among the surgical groups (p = 0.08). CONCLUSIONS: Aggressive homograft replacement of the central pulmonary arteries has been associated with improved survival in patients with APVS especially in neonates with severe RD.

Abnormalities, Multiple↗
PubMed2005

Advances in experimental percutaneous pulmonary valve replacement.

BACKGROUND: Percutaneous pulmonary valve implantation is emerging as an alternative and additional option for a successful surgical scheme. To date, these procedures are performed by the balloon-in-balloon technique. The use of self-expanding stents for percutaneous valve replacement is assumed to improve preservation of the valve in its folded condition in the application device and the valve's long-term functioning. Therefore, initial experience with the development of a completely percutaneous transfemoral technique for pulmonary valve implantation using a self-expanding valved stent is described. METHODS: Bovine jugular xenografts were sutured into nitinol stents, and functional in vitro tests of valved stents were carried out. Transfemoral implantation in pulmonary position was acutely evaluated in 6 sheep weighing 22 to 29 kg. Radiologic evaluation was performed by angiography and multislice computed tomography (MSCT) scan. In addition, pathoanatomical studies were performed. RESULTS: Exact implantation in pulmonary valve position was achieved in 5 of 6 sheep, with 1 early stent migration. Another sheep died before stent placement owing to perforation of the right ventricle by the delivery system. Orthotopic pulmonary valved stent position was depicted by MSCT in all other sheep (n = 4). The peak-to-peak transvalvular gradient was 8.2 +/- 3.9 mm Hg (n = 5). Postmortem examination revealed intact stent valves with no adherent clots. No macroscopic damage of the pulmonary artery was noted, whereas minor hematoma of the right atrium and the right ventricular outflow tract were observed in 2 hearts. CONCLUSIONS: This acute study demonstrates that memory nitinol valved stents can be optimally deployed in the pulmonary position through the groin in sheep.

Animals↗
PubMed2005

Misalignment of pulmonary vessels with alveolar capillary dysplasia: association with atrioventricular septal defect and quadricuspid pulmonary valve.

Misalignment of pulmonary vessels (MPV) with alveolar capillary dysplasia (ACD) is a complex congenital vascular abnormality in newborn infants. This condition is often associated with persistent pulmonary hypertension, which leads to severe respiratory distress and finally to the death of the newborn. Here, two cases of MPV with ACD are reported that were associated with atrioventricular septal defects. Both cases showed a combination of a large atrial septal defect and a smaller subvalvular ventricular septal defect. In one newborn, the cardiac malformation was accompanied by a quadricuspid pulmonary valve. These cases emphasize the importance of considering MPV with ACD a possible cause for pulmonary hypertension in the newborn, especially when a concomitant atrioventricular septal defect could represent an alternative explanation for the pulmonary hypertension.

Abnormalities, Multiple↗
PubMed2002

Correction of absent pulmonary valve syndrome using a pericardial valved conduit.

Absent pulmonary valve syndrome in a 4-month-old infant was successfully corrected using a fresh autologous pericardial trileaflet valved conduit. He recovered from operation with only mild pulmonary regurgitation at 4 months postoperatively. This technique is an effective alternative for infants with congenital heart disease who need tissue valved conduits. It may be more suitable than the aortic homograft by reason of the shortage of small homografts and its lower costs.

Echocardiography↗
PubMed1991

[Tetralogy of Fallot with absence of the pulmonary valve and the left branch of the pulmonary artery: echocardiographic diagnosis].

The absence of the pulmonary valve and the left pulmonary artery were recognized by cross-sectional and Doppler echocardiography in one patient with tetralogy of Fallot. The right oblique subxiphoid view was the best approach for visualization of these anatomical abnormalities. The diagnosis was confirmed at catheterization and at operation. It is suggested that premature closure of the ductus arteriosus may be responsible for the failure in the development of both the pulmonary valve and the left pulmonary artery.

Child, Preschool↗
PubMed2003

Visualization of the pulmonary valve using cine MR imaging.

PURPOSE: To evaluate the capability of bright-blood cine MR to directly visualize the leaflets of the valve replacement in pulmonary position following Ross operation. MATERIAL AND METHODS: Long and short axis views of the pulmonary valve were obtained in 10 normal subjects and 14 patients after Ross operation. Valve morphology and function were analyzed and signal-to-noise (SNR) and contrast-to-noise ratios (CNR) were calculated. Flow measurements were performed in the pulmonary trunk to assess pulmonary regurgitation. RESULTS: In all subjects, tricuspid morphology of the pulmonary valve was visualized. SNR of the leaflets in normal subjects (9.8 +/- 3.0) and in patients after Ross operation (7.5 +/- 2.2) differed significantly from blood (12.6 +/- 3.2 and 11.3 +/- 2.5, respectively, p < 0.05). Valvular regurgitation was seen in 5 patients as an insufficient closure of the valve which was confirmed by flow measurements. CONCLUSION: Cine MR enables in-plane visualization of the pulmonary valve and allows for functional and morphological evaluation in patients after pulmonary valve surgery.

Adult↗
PubMed2000

Pulmonary valve endocarditis: mid-term follow up of pulmonary valvectomies.

Four males aged 20-37 years (three drug addicts and one with a congenital mixed pulmonary valve lesion) were diagnosed in 1989, 1991 and 1993 with pulmonary valve endocarditis without tricuspid infection. Three patients were positive for hepatitis B, C or both, and one patient was HIV-positive. The predominant organism in blood cultures was Staphylococcus aureus. Antibiotic treatment of pulmonary valve endocarditis had failed; thus partial or total valvectomies were performed. Postoperatively, all patients were cured of infection and initial recovery was good. At mid-term follow up (5-10 years) there were no recurrences, and tolerance of the resultant pulmonary insufficiency was good. Slight to severe tricuspid valve insufficiency developed, together with right ventricular dilatation, in all cases. Hepatomegaly was apparent in two cases and peripheral edema in one. Despite treatment, the latter patient remained in moderate right ventricular failure, and may require homograft valve replacement. The other three patients remained in good clinical condition. Eradication of the infection was achieved in all patients. It is concluded that pulmonary valve resection is the treatment of choice for pulmonary valve endocarditis when antibiotic treatment has failed. Complete resection of all affected tissue should be performed in these cases. Analysis of preoperative data did not permit differentiation of those patients likely to develop right heart failure.

Adult↗
PubMed1990

Percutaneous balloon valvuloplasty of pulmonary valve stenosis, dysplasia, and residual stenosis after surgical valvotomy for pulmonary atresia with intact ventricular septum: long-term results.

Eight-six children (aged 20 days to 14 years, 20 under age 1 year) underwent 94 percutaneous balloon valvuloplasty for pulmonary valve stenosis. The patients were divided into three groups: typical pulmonary valve stenosis (71), pulmonary valve dysplasia (9), and residual stenosis after surgical valvotomy for pulmonary atresia with intact ventricular septum (PAIS) (6). Each of the three groups was divided into two subgroups. In the early cases, balloon catheters with diameter 10-20% exceeding pulmonary valve annulus were used and the drop of the gradient was 39.5%. In the later cases, balloon diameters 30-40% greater than the valve anulus or double balloons were used and a drop of 66.7% in the RV-PA pressure gradient was achieved. The dilation in patients with dysplastic valve and residual stenosis after surgical valvotomy for PAIS was less effective. Doppler echocardiography was the technique used to evaluate residual gradient. Six months to 4 years follow-up demonstrated a persistent decrease of the valve gradient.

Adolescent↗
PubMed1983

Absent pulmonary valve syndrome. Surgical correction with pulmonary arterioplasty.

A 13 month old child with classic features of absent pulmonary valve with tetralogy of Fallot underwent successful surgical repair by closure of the ventricular septal defect, relief of the right ventricular outflow tract obstruction, and partial resection and plastic repair of the aneurysmally dilated pulmonary arteries. Cardiac catheterisation data and clinical follow up for more than 18 months after the operation indicated excellent results. It is suggested that plastic repair of the aneurysmally dilated pulmonary arteries along with closure of the ventricular septal defect and relief of the right ventricular outflow tract obstruction should be performed early, perhaps between 1 and 2 years of age.

Cardiac Catheterization↗