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PubMedDate not supplied

Antenatal diagnosis of tetralogy of Fallot with absent pulmonary valve accompanied by hydrops fetalis and polyhydramnios.

The case presented is an antenatal diagnosis of tetralogy of Fallot with absent pulmonary valve which was accompanied by hydrops fetalis and polyhydramnios with an absent stomach echo. Absent pulmonary valve was diagnosed by a cystic, pulsatile, aneurysmal dilatation of pulmonary arteries and a relatively small pulmonary valve annulus without a definite valve echo, in which a typical to-and-fro flow pattern was detected by the Doppler flow analysis. We will discuss some plausible roles of the grossly enlarged pulmonary arteries on hydrops fetalis and polyhydramnios.

Adult↗
PubMed1989

Surgical pathology of the pulmonary valve: a study of 116 cases spanning 15 years.

The gross surgical pathologic features of the pulmonary valve were reviewed in 116 patients (63 male and 53 female) who had undergone a cardiac operation with pulmonary valve excision at our institution during the period 1973 through 1987. Although the mean age was 12 years, subjects ranged in age from 3 months to 73 years, and 25 patients, including 19 with congenital heart disease, were older than 20 years of age. Among 105 patients who had pure pulmonary stenosis, 61 (58%) had tetralogy of Fallot, 18 had isolated pulmonary stenosis, 23 had other congenital cardiac anomalies, and 3 had carcinoid heart disease. Five patients had pure pulmonary regurgitation (four with tetralogy and one with infective endocarditis), and four had combined pulmonary stenosis and regurgitation (two with congenital cardiac anomalies and two with carcinoid heart disease). In two patients, the valve was neither stenotic nor regurgitant. Thus, congenital heart disease accounted for 110 of the 116 cases (95%), and tetralogy of Fallot was the most commonly observed form (65 cases). Bicuspid pulmonary valve was the most common anomaly and was present in 58% of patients with tetralogy but in only 17% of those with isolated pulmonary stenosis.

Adolescent↗
PubMed1984

Percutaneous transluminal balloon valvuloplasty for pulmonary valve stenosis.

Transluminal balloon valvuloplasty was used to treat congenital pulmonary valve stenosis in 20 patients. Follow-up cardiac catheterization was performed in 11 patients at intervals of from 2 to 12 months after the procedure. Peak systolic pressure gradient across the pulmonic valve decreased from 68 +/- 27 to 23 +/- 5 mm Hg (p less than .001) after valvuloplasty. There were no complications. Follow-up catheterization demonstrated persistent relief of right ventricular hypertension in the patients with typical pulmonary valve stenosis.

Adolescent↗
PubMed2006

Deep sternal wire infection resulting in severe pulmonary valve endocarditis.

Right-sided infective endocarditis is uncommon, comprising less than 5% of all cases of endocarditis. This is primarily seen in patients with drug abuse, long-term intravenous catheters, and congenital malformations, or a combination of these. Isolated pulmonary valve endocarditis is difficult to recognize due to its rarity, minimal cardiac manifestations, and predominance of pulmonary infections secondary to embolization of the vegetations. We describe an unusual case of chronic sternal wound infection and migration of an infected braided sternal wire causing right ventricular outflow tract and pulmonary valve endocarditis, which necessitated a complicated reoperation including pulmonary valve replacement with a homograft.

Aged↗
PubMed1989

Dilatation with progressively larger balloons for severe stenosis of the pulmonary valve presenting in the late neonatal period and early infancy.

Balloon dilatation in infants with severe pulmonary valve stenosis may not be a straightforward procedure once the arterial duct has closed. Balloon dilatation was attempted in three neonates and infants. In an 11 week old infant hypotension and bradycardia developed shortly after a 5 French end hole catheter was passed through the severely stenosed pulmonary valve. An emergency Waterston shunt was subsequently performed, but he died three days later. After this experience the technique was modified so that progressively larger balloons were used for dilatation in two infants, aged one and three weeks, with severe pulmonary valve stenosis in whom the arterial duct had closed. It was successful in both.

Cardiac Catheterization↗
PubMed1991

Success determining factors in percutaneous transluminal balloon valvuloplasty of pulmonary valve stenosis.

Twenty-two percutaneous transluminal balloon valvuloplasty procedures were performed on 21 patients with congenital pulmonary valve stenosis. The peak systolic pressure gradient was immediately reduced from 79.1 +/- 7.4 to 22.2 +/- 1.8 mmHg, (P less than 0.0001) and follow-up cardiac catheterization at 5.3 +/- 0.4 months in 19 patients revealed no further significant change in gradient (23.5 +/- 3.2 mmHg). The best results were obtained when balloons larger than the pulmonary annulus were used, i.e. an immediate residual transvalvular gradient of 22.0 +/- 2.2 mmHg with a balloon/annulus ratio greater than 1, as opposed to 44.2 +/- 5.4 with a balloon/annulus ratio = 1 (P less than 0.001). The angiographically determined cusp thickness of the stenotic pulmonary valves was significantly greater than that of the control group of 24 patients without pulmonary valve stenosis (1.21 +/- 0.09 vs 0.59 +/- 0.02 mmHg, P less than 0.00001). The relationship between this parameter and the residual transvalvular gradient at follow-up was found to be significant (r = 0.77, P less than 0.001). It is concluded that balloon size is a determinant factor in achieving good results with percutaneous balloon valvuloplasty although cusp thickness, a factor to which scant regard has hitherto been paid, also plays a significant role in the residual transvalvular gradient measured at follow-up.

Adolescent↗
PubMed1997

Tetralogy of Fallot with absent pulmonary valve: evaluation with magnetic resonance imaging.

Two infants with tetralogy of Fallot with absent pulmonary valve were studied with electrocardiographically gated magnetic resonance imaging using a 1.5 T imaging system. In each case, imaging was performed successfully. The magnetic resonance imaging clearly demonstrated a rudimentary pulmonary valve, an aneurysmal pulmonary artery, and compression of the mainstem bronchi. Cine MRI revealed phasic compression of the bronchi by an aneurysmally dilated pulmonary artery. Magnetic resonance imaging produced high quality images and provided all of the anatomic information required for preoperative evaluation.

Bronchi↗
PubMed1975

Effect of coexistent coarctation of pulmonary trunk in natural history of complete absence of pulmonary valve with ventricular septal defect.

A case of complete absence of the pulmonary valve in association with a ventricular septal defect and a pulmonary artery coarctation is described. Despite these defects the patient had minimal symptoms, probably because the pulmonary artery coarctation acted as a natural banding and limited the pulmonary regurgitant flow and reduced the left-to-right shunt across the ventricular septal defect. The patient was operated on at the age of 40 and the ventricular septal defect was closed but the pulmonary artery coarctation was left alone. In view of the long-term survival in this case, it is suggested that pulmonary artery banding would reduce the symptoms and increase the life expectancy of patients with congenital absence of the pulmonary valve and a ventricular septal defect.

Adult↗
PubMed2005

Early insertion of a pulmonary valve for chronic regurgitation helps restoration of ventricular dimensions.

BACKGROUND: This study assesses the results of early insertion of a pulmonary valve for chronic pulmonary regurgitation based on right ventricular volume measurements. METHODS: Valved conduits were prospectively inserted in 39 patients (aged 14 to 39 years) when the right ventricular (RV) end-diastolic volume index on magnetic resonance imaging (MRI) exceeded 150 mL/m2. Changes in morphology and function of the RV were prospectively analyzed by an MRI at 6 months postoperatively (available in 21 patients). RESULTS: There were no early or late deaths. All conduits showed good function at a median 15 month follow-up. Postoperative RV end-systolic and end-diastolic volumes showed a significant positive correlation (p = 0.005 and p < 0.0001), while postoperative left ventricular (LV) ejection fraction showed a significant negative correlation (p = 0.03) with preoperative RV end-diastolic volume index. Seven patients who achieved normal RV end-diastolic volume index (< or = 100 mL/m2) (group 1) when compared with 14 remaining patients (group 2) showed that they differed significantly with respect to their preoperative RV end-diastolic volume index (170.3 +/- 21.1 vs 203.6 +/- 35.6; p = 0.02) and postoperative LV ejection fraction (59.9 +/- 4.2 vs 54 +/- 7%, p = 0.03). CONCLUSIONS: Our results show that the improvement in ventricular dimensions and functions directly correlates with the timing of pulmonary valve insertion. Early insertion leads to normalization and late insertion leads only to improvement. These observations, along with a low morbidity for these reoperations, justify earlier reintervention in cases of chronic pulmonary regurgitation. A RV end-diastolic volume index of 150 mL/m2 seems to be a practical cutoff value to prescribe pulmonary valve insertion.

Adolescent↗
PubMed1999

[Incidence and treatment of reactive infundibular obstruction after balloon dilatation of critical pulmonary valve stenoses].

Seventeen consecutive newborn and premature babies with critical pulmonary stenosis underwent a technically successful balloon valvuloplasty at our institution from March 1991 to February 1998. The only major complication was a thrombosis of the femoral vein in one patient, causing no clinical problems. Four patients (24%) showed a reactive infundibular obstruction after balloon valvuloplasty. The outflow tract obstruction became evident immediately after successful dilatation of the pulmonary valve with persistently high pressures in the right ventricle. Pathognomonic was a typical notch in the ascending part of the right ventricular pressure curve. We were unable to predict this reaction based on echocardiography or angiography. To relieve the muscular subvalvar obstruction, we treated the first two patients with i.v. Propranolol (0.05 mg/kg over 2 min). The last 2 patients received Esmolol (0.5 mg/kg over 2 min followed by a continuous infusion with 100 micrograms/kg/min), a very short acting beta-blocker. In the medium-term follow-up, all 17 patients had a very good result with only mild pulmonary valve regurgitation. All 4 patients with a reactive infundibular obstruction required no repeat intervention. In the medium-term follow-up there were no differences between these 4 patients and the whole group.

Angiography↗
PubMed1996

[Pulmonary valve replacement in children].

From February 1988 to October 1994, 15 pulmonary valve replacements (PVR) have been performed at St-Justine Hospital in children with a mean age of 145.7 months. Ten children previously had a correction of tetralogy of Fallot; two had absent pulmonary valve syndrome; one had been operated on for pulmonary atresia with intact ventricular septum, one other had a correction for a ventricular septal defect with pulmonary artery banding; the last patient developed degeneration of a pulmonary bioprosthesis. The time between the primary repair and the PVR ranged from 61 to 221 months. Fourteen bioprosthesis and one aortic homograft were implanted. All patients had antiplatelet treatment. There was one operative death due to a fatal anaphylactic reaction and one late death occurred unrelated to valvular surgery. At follow-up from 1 to 187 months (mean, 40.7 months) all patients were in New York Heart Association Class 1. No hemorrhagic nor thromboembolic complication have been observed and no reoperation for bioprosthesis failure was necessary. Nevertheless in subsequent echocardiographic studies, two patients with the smallest bioprosthesis (21 mm) have developed pulmonary gradients of 80 and 85 mmHg, 65 and 80 months following PVR. While our results with PVR in children have been satisfactory, this operation should be performed only in symptomatic patients with severe pulmonary regurgitation because of progressive deterioration of the available bioprosthesis.

Adolescent↗
PubMed1980

M-mode and cross-sectional echocardiographic features of flail pulmonary valve. A case report.

In a patient with patent ductus arteriosus, a pulmonary regurgitant murmur developed after the treatment of endocarditis. M-mode echocardiograms showed abnormal echo mass, characterized by fine diastolic fluttering, in the right ventricular outflow tract during diastole which were continuous with anterior pulmonary leaflet and thick pedunculated lesions attached to the anterior pulmonary leaflet were seen to be moving freely by cross-sectional echocardiography. At autopsy the destruction of the pulmonary valve was confirmed. This study suggests that diagnosis of flail pulmonary valve is feasible by M-mode and cross-sectional echocardiography.

Adult↗
PubMed1997

Balloon pulmonary valvuloplasty for pulmonary valve stenosis with atrial septal defect.

We performed successful balloon pulmonary valvuloplasty (BPV) in 6 patients with pulmonary valve stenosis and atrial septal defect (ASD) accompanied by atrial left-to-right shunt without an increase of shunting immediately after BPV. It suggests that such patients should be treated by BPV initially and the need for ASD repair can be assessed during long-term follow-up.

Catheterization↗
PubMed1980

Reoperation for massively calcified pulmonary valve heterografts. Surgical considerations.

Calcifications of pulmonary valve heterografts inserted in children are more frequently recognized with increasing follow-up periods and may lead to reoperation. This complication is common with our non-industrially prepared procine valves and may also be observed with Hancock porcine heterografts. Reoperation is rendered difficult by incrustation of the massively calcified heterograft into the posterior aspect of the sternum and because of the need of adequate exposure of the pulmonary bifurcation. Four patients were successfully reoperated on by the authors. The valves were not replaced. An improved surgical technique used in the last three cases is described.

Adolescent↗
PubMed1976

Primary intimal sarcoma of pulmonary valve and trunk with osteogenic sarcomatous elements. Report of a case considered to be pulmonary embolus.

A 49-year-old woman had a six-week history of increasing shortness of breath and fatigue. X-ray films and pulmonary scans showed multiple areas of emboli, especially in the right lung. Treatment with heparin was unsuccessful, and although a repeat scan showed only slight changes, the patient's condition deteriorated. Acute pulmonary edema and ventricular tachycardia preceded her death. Autopsy revealed a primary intimal sarcoma with osteogenic elements arising in the posterior leaflet of the pulmonary valve and obstructing the main pulmonary artery and its right branch.

Female↗
PubMed1996

[Tetralogy of Fallot with absent pulmonary valve--prenatal diagnosis and management in the 2nd trimester].

Tetralogy of Fallot with absent pulmonary valve is a rare congenital cardiac malformation detected in 3% to 6% of tetralogy of Fallot patients. The prognosis depends on respiratory complications. In the present case the diagnosis was made at 18 week's gestation with two-dimensional and Doppler echocardiography. A ventricular septal defect and overriding aorta, absent valve echo, pulmonary regurgitation, and cystic pulsatile dilatation of the left pulmonary artery were the main diagnostic criteria. Severe respiratory complications were assumed in combination with a hyperechogenic left lung and termination of pregnancy was performed at 20 weeks' of gestation.

Abortion, Eugenic↗