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At least 217 records · Page 12Linked to original sources

Evaluation of pulmonary blood supply by multiplanar cine magnetic resonance imaging in patients with pulmonary atresia and severe pulmonary stenosis.

The purpose of this study was to assess the capability of multiplanar cine magnetic resonance imaging (MRI) for evaluating pre- and post-operative pulmonary circulation in patients with pulmonary atresia and severe pulmonary stenosis. Seventy-three multiplanar cine MRIs were performed in 30 patients, aged 1 month to 7 years (mean age, 27 months). The morphology and size of the central pulmonary arteries (PA), source of the major aortopulmonary collateral arteries (MAPCA), patency of Blalock-Taussig (BT) shunt vessels, and the post-operative pulmonary circulation were assessed. The accuracy of cine MRI was compared with that of angiography in all patients. The PA was visualized to the first hilar branch in 21 patients, but not in 8 patients in whom the central PA was absent. On follow-up MRI, PA growth was measured, and the results showed excellent correlation with the results obtained by angiography. In 17 patients who had undergone 23 BT shunt operations, cine MRI correctly demonstrated all patient shunts and 5 of 6 stenotic lesions. Multiplanar cine MRI provided excellent detail of the peripheral PA in all patients, 7 of 8 peripheral pulmonary stenoses, 3 of 4 nonconfluent pulmonary arteries, and 2 of 3 PA obstructions. Although the sources of MAPCA were identified in 7 of 9 patients, the distal connection of the MAPCA was not detected in all patients. Seven patients were reexamined after pulmonary plasty; they exhibited normal pulmonary flow patterns. Multiplanar cine MRI provides high-resolution imaging of PA with dynamic visualization of flow and is an effective noninvasive technique for evaluating pre- and post-operative patients with pulmonary atresia and severe pulmonary stenosis.

Child↗

Balloon pulmonary valvuloplasty in a child with Ebstein's anomaly and valvar pulmonary stenosis.

We performed a balloon pulmonary valvuloplasty in a child with Ebstein's anomaly using the standard technique. The procedure reportedly has not been performed in the face of this defect and, although difficult, was safely and successfully accomplished. In patients with Ebstein's anomaly and right ventricular outflow obstruction compounded by pulmonary valve stenosis, application of this technique may delay the need for surgical intervention.

Catheterization↗

[Percutaneous transluminal valvuloplasty in congenital pulmonary stenosis in adults. Apropos of 34 cases].

Percutaneous pulmonary valvulotomy is the treatment of choice for isolated congenital pulmonary valvular stenosis in childhood. However, experience of this procedure in the adult is much more limited. Between January 1984 and December 1994, 34 patients with severe or moderate pulmonary valvular stenosis underwent percutaneous transluminal valvuloplasty. The age of the patients ranged from 20 to 47 years (mean 22 +/- 4 years). Cardiac catheterisation was performed using the femoral vein in 27 cases and the internal jugular vein in 7 cases. Success was obtained in 28 patients (81% of cases). Pulmonary artery-right ventricular pressure gradient decreased from 113 +/- 35 to 32 +/- 13 mmHg (p < 0.001) after valvuloplasty with one or two balloon catheters. The tolerance of transluminal valvuloplasty was generally good. The poor results were explained by cases of dysplasic valves or of infundibular reactions. There was one death which occurred 24 hours after the procedure. Clinical and echocardiographic follow-up was obtained in 20 patients, 3 to 36 months after valvuloplasty (average: 23 +/- 13 months). No cases of restenosis were observed. Percutaneous transluminal pulmonary valvuloplasty in the adult is feasible and gives good results which are maintained at medium-term; it has become the treatment of choice of valvular pulmonary stenosis and gives good results which are maintained at medium-term, thereby avoiding surgical valvulotomy.

Adult↗

Increased protein synthesis during right ventricular hypertrophy following pulmonary stenosis in the dog.

The synthesis rates of mixed protein in the left and right ventricles were the same in normal dogs, averaging 7.8% per day. After five days, pulmonary stenosis protein synthesis rate in the right ventricle increased rapidly to 13.6% per day and subsequently decreased to near normal by 12 days, during which time the right ventricle protein mass increased by 48% after five days and by 73% after 12 days. Similarly the synthesis rate of the myofibrillar and sarcoplasmic proteins was almost doubled after five days of stenosis. The protein synthesis rate in the left ventricle did not change significantly during the 24 days of pulmonary stenosis.

Animals↗

Balloon dilation of critical valvar pulmonary stenosis in the first month of life.

Between 1985 and 1992, 36 consecutive neonates, aged 1-29 days, weight 2.4-5.0 kg, with critical valvar pulmonary stenosis underwent attempted balloon dilation (BD). At catheterization, 30 were on prostaglandin (PGE1) therapy and 20 were intubated. The valve was successfully crossed and dilated in 34/36 (94%), including three with an echocardiographic diagnosis of valvar pulmonary atresia and a right ventricle of adequate size. The valve was first dilated with a 2- to 5-mm balloon and then with serially larger ones (up to 12 mm) to a final balloon/annulus value of 126%. The RV/systemic pressure value fell from 150 +/- 32 to 83 +/- 30%, O2 saturation rose from 91 +/- 6% to 96 +/- 4%, and PGE1 was discontinued at the end of the procedure. There were 11 complications (31%) including one early death from sepsis and necrotizing enterocolitis, endocarditis in another, two myocardial perforations, one femoral-iliac vein tear, and one transient pulse loss. A repeat BD was carried out in five patients, two of whom subsequently had surgery. At follow-up (33 +/- 23 months), the 31 patients managed by BD alone were well and had echocardiographic gradients of < 30 mm Hg in 90% and pulmonary regurgitation, considered mild in most, in 52%. In neonates with critical valvar pulmonary stenosis, we believe BD mortality is less than with surgery and is the treatment of choice.

Catheterization↗

Hemodynamics during and immediately after the surgical creation of a supravalvular pulmonary stenosis in pigs.

The surgical creation of a supravalvular pulmonary stenosis (PS) adequate to prepare the left ventricle (LV) for anatomic correction in cases of simple transposition of the great arteries (TGA) is difficult. To gain more information on the hemodynamic changes immediately following PS of various degrees, pressures (P) in the right atrium (RA), right ventricle (RV), pulmonary artery (PA) and left ventricle (LV) as well as pulmonary flow (F) were monitored 40 minutes (30 to 50 minutes after PS (n = 15) in 10 open chested pigs (mean weight: 20.7 kg). Hemodynamic changes can be separated into an adaptation phase (AP) with an "overshoot" response of most hemodynamic parameters and a stable phase (SP) without further changes, reached after 6.5 minutes (1 to 18 minutes). This pattern was independent of the degree of PS (increase of RV Pmax: 13 to 39 mmHg). Maximal changes (p less than 0.01) during AP included: RV Pmax: +25 +/- 9 mmHg, RV EDP: +6 +/- 3 mmHg, RV dp/dt max: +315 +/- 208 mmHg/sec, LV Pmax: -43 +/- 24 mmHg, PA max. syst. flow: -0.3 +/- 0.15 ml/sec, CO: -0.45 +/- 0.3 l/min. During SP, LV Pmax and PA Pmean were normal. RV EDP and RA Pmean had decreased (p less than 0.01) but remained elevated as compared to the control values (p less than 0.01). The right ventricular maximal pressure had decreased 30% but remained elevated compared to control values (p less than 0.01). A change of RV Pmax during SP correlated (r = 0.93) with the maximal change of RV Pmax during AP.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

Arrhythmias in children with pulmonary stenosis.

Arrhythmias have been recorded in association with congenital pulmonary stenosis. Of six patients who had arrhythmias before operation, anatomical conduction defects were present in two and the arrhythmias persisted after operation. In four, the arrhythmic episodes have not been recorded after operation. No specific causative factors were evident in the pre-operative group. Acute and often fatal arrhythmias have occurred in the early post-operative period. High risk patients were those with cyanosis, high right ventricular pressure (in excess of 175 mm. Hg), and gross right ventricular hypertrophy. There is evidence to suggest that these arrhythmias develop in association with myocardial ischaemia.

Acute Disease↗

Exclusive double outlet right ventricle with atrioventricular concordance and pulmonary stenosis. Results of reconstructive surgery.

Double outlet right ventricle associated with atrioventricular concordance, pulmonary stenosis and situs solitus of the atria is a subset of double outlet right ventricle related through the surgical treatment. From 1974 to 1985, 14 patients, 5 males, 9 females (mean age 8.9 years, range 13 months-22 years) were operated upon. All patients had infundibular stenosis and normal or large pulmonary arteries. The apex of the heart was to the right in 2 patients, the right and left ventricles were superior and inferior in 2 patients and 1 patient had both anomalies. The ventricular septal defect was subaortic in 11 patients (aorto-mitral discontinuity in 5) and non-committed in 3 patients. Three patients had 2 ventricular septal defects. The aorta was anterior in 3 patients and to the right of the pulmonary artery in 11 patients. All patients, through a transventricular and transatrial approach, had a reconstructive surgery. In 3 patients, an aortic homograft valved conduit was used. One patient had the ventricular septal defect enlarged. There was one early death (7.1%) from high residual right ventricle pressure and no late death. One patient had a transient atrioventricular block. One patient was reoperated upon for a residual ventricular septal defect. All survivors had a good clinical result. Re-evaluation in 8 patients confirmed excellent haemodynamics: the right ventricle to pulmonary artery pressure gradient decreased from 80 mm Hg (range 60-95) preoperatively to 24 mm Hg (range 3-32) postoperatively.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Exercise testing in children with pulmonary valvar stenosis.

Pulmonary valvar stenosis with intact ventricular septum is a common anomaly. This lesion poses a fixed obstruction to the right ventricular outflow. The right ventricle ejects the entire cardiac output across the stenotic valve. Right ventricular systolic pressure and oxygen demand are increased at rest and more so with exercise. Exercise tolerance in children and adults with mild valvar pulmonary stenosis is nearly normal, but is diminished in those with moderate and severe stenosis, indicating impaired ability to sustain adequate cardiac output. Following relief of stenosis, cardiac performance improves in children, but remains abnormal in adults. This appears to be related to postoperative resolution of right ventricular hypertrophy in children, whereas myocardial fibrosis may explain the lack of improvement in adults.

Adult↗

Late re-interventions following arterial switch operations in transposition of the great arteries. Incidence and surgical treatment of postoperative pulmonary stenosis.

Seventy-six patients were studied after arterial switch operation (ASO) between May 1977 and February 1992. Pulmonary artery reconstruction was initially performed by: conduit interposition in 5 patients, direct main pulmonary artery anastomosis and button patches in 60 patients, and pantaloon-like patch repair in 11 patients. Pulmonary stenosis developed in 17 patients (22%), requiring a total of 26 late re-interventions. Re-intervention was required in four out of five patients operated with pulmonary artery conduits, 11 out of 60 with a button patch repair and 2 out of 11 following pantaloon-type repair. In this series pulmonary artery stenosis (PS) involving the pulmonary valve occurred in 9/17 patients. Involvement of the pulmonary valve was related to the technique of pulmonary artery reconstruction. In these patients surgery is necessary. Balloon angioplasty can be a valuable tool when the stenosis is more distal. The incidence of PS was not influenced by the type of reconstruction or the use of Lecompte's maneuver.

Anastomosis, Surgical↗

Percutaneous balloon valvotomy of congenital pulmonary stenosis using oversized balloons.

Percutaneous balloon valvotomy was attempted in 27 patients (aged 6 days to 19 years, median 2 years, 11 months) with unoperated typical valvular pulmonary stenosis using a balloon 7 to 60% (mean 30%) larger than the valve anulus. One patient had undergone a previous balloon valvotomy elsewhere. To achieve an oversized dilation diameter in three larger patients, two balloons were inflated side by side. Their "effective dilation diameter" was determined by the diameter of the circle with the same area as that of the oval enveloping the two balloons. A significant reduction of the transvalvular gradient occurred in all patients (mean +/- SD = 74.3 +/- 14.7%, range 33 to 100%). The average gradient of 65.0 +/- 19.0 mm Hg (mean +/- SD) fell to 15.9 +/- 7.6 mm Hg (0 to 30 mm Hg). Twenty-five of 27 patients had a residual transvalvular gradient of less than 25 mm Hg. The calculated valve orifice area increased by an average of 183 +/- 80%. No significant complications occurred. It is concluded that percutaneous balloon valvotomy with a balloon 20 to 40% larger than the valve anulus is the treatment of choice for typical congenital valvular pulmonary stenosis.

Adolescent↗

[Critical pulmonary stenosis in infants (author's transl)].

Eighteen infants carrying pulmonary stenosis with a complete interventricular wall whose most frequent clinical data are: precocius cyanosis, congestive cardiac insufficiency and in 25% of them hypoxic crises are presented. Complementary explorations of this entity are not definitive, but clinical diagnosis described with evolutional cardiomegaly at the expense of the right cavities and pulmonary ischemia forces authors to think that there is a severe obstruction of the infumdibulum with complete interventricular septum. The different parameters which have been proposed to evaluate severity of the stenosis are discussed and compared, finding among them significative differences. Authors consider the concept of the right diminutive ventricle, finding in these cases the electrocardiographic pattern rS in V1 and observing a great mortality both spontaneous and postsurgical. The high mortality of this heart disease, both in its' natural evolution as well as after surgery is to be pointed out as in most published series.

Child, Preschool↗

Shortening patterns of the infundibulum in valvular pulmonary stenosis before and after balloon valvuloplasty.

We analyzed shortening patterns of the infundibulum in 11 patients with valvular pulmonary stenosis (PS) before and immediately after balloon valvuloplasty and at follow-up. The control group consisted of 32 patients with Kawasaki disease. The valvuloplasty was performed at the age of 5.8 +/- 2.9 (mean +/- SD) years and a satisfactory relief of obstruction was achieved in each. The follow-up study was done at 1.1 +/- 0.2 years after the valvuloplasty. With the use of lateral projection of right ventriculograms, transverse diameters of the midinfundibulum were measured over one cardiac cycle from initiation of the pulmonary valve opening. Indices demonstrating shortening patterns of the infundibulum were as follows: time to the beginning of shortening (TBS), time to the shortest diameter (TSD), and shortening fraction (SF). TBS were prolonged before the valvuloplasty, whereas it was comparable with that in the control group, immediately after the procedure and at follow-up. TSD was increased before and immediately after the valvuloplasty, but it was normalized at follow-up. SF was increased at every observation. Thus, shortening patterns of the infundibulum in PS were characterized by increasing TBS, TSD, and SF. At short-term follow-up following the valvuloplasty, TBS and TSD were normalized, which could be a result of a successful relief of pulmonary obstruction. In contrast, SF remained elevated, which indicates that the musculature of the infundibulum remains hypertrophic up to 1 year after the valvuloplasty.

Case-Control Studies↗

[A rare complication of catheter balloon valvuloplasty of pulmonary stenosis--a case report].

We describe a rare complication which occurred during catheter balloon valvuloplasty to the stenosis of the pulmonary artery. A 4-year-old girl with cyanosis who was diagnosed as having tetralogy of Fallot, patent foramen ovale and Down's syndrome underwent the cardiac catheralisation and angiography. Angiography showed the stenosis from outlet of right ventricle to bifurcation of the pulmonary artery. We attempted catheter balloon valvuloplasty to the stenosis of the pulmonary artery subsequently. We used the PTA catheter (NuMED INC, canada), 10 mm in diameter. The balloon was inflated twice (6 atm, 5 sec, 8 atm, 7 sec) at the bifurcation of the pulmonary artery safely. However, during third trial to inflate to 8 atm, it proved impossible to increase pressure to the desired level of 8 atm. Blood backed up in the catheter and it became impossible to deflate the balloon. We failed to remove the catheter through the left femoral vein, so we led the catheter to the right internal jugular vein with a guidewire. The catheter was successfully extracted in this way. The proximal connection between the balloon and the outer tube was broken and the balloon was compressed. To our knowledge, such as complication as this has not reported previously.

Catheterization↗

Echocardiographic findings of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum in utero.

BACKGROUND: Neonates with right ventricular outflow obstruction and intact ventricular septum show serious hemodynamic problems, such as severe hypoxemia, congestive heart failure due to massive tricuspid regurgitation, respiratory distress related to huge pulmonary hypoplasia or ventricular dysfunction due to right ventricle-coronary communication. Recent advances in fetal diagnosis include many cases of in utero diagnosis of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum. METHOD: Among the fetuses examined from April 1994 to March 1998, five fetuses were found with pulmonary atresia (PA) or critical pulmonary stenosis (CPS). Fetal echocardiograms were reviewed to elucidate the accuracy of fetal information and the efficacy of fetal diagnosis in the perinatal management of patient with CPS or PA and intact ventricular septum. RESULTS: The five cases were divided into two groups: two with a very small right ventricle (group 1) and three with a tripartite right ventricle (group 2). Fetal cardiomegaly and right atrial dilatation were prominent in group 2, whereas cardiac sizes were normal in group 1. Serial fetal examination in one group 2 fetus revealed developing right ventricular hypertrophy in utero. All group 2 cases showed massive tricuspid regurgitation (TR). Estimated right ventricular pressures from TR always exceeded the systemic blood pressures of gestational age-matched neonates. Reversed flow through the ductus arteriosus was recorded in both groups and ductus-dependent pulmonary circulation after birth was anticipated. Patency of both tricuspid and pulmonary valves was difficult to recognize in utero, as was right ventricle-coronary artery communication. Four of the five cases were maternally transported and survived palliative and/or definitive intervention. One fetus with chromosomal abnormality was observed without intervention during infancy and received palliative surgery when she was two years old. CONCLUSION: Fetal hemodynamic information was useful for making decisions not only after birth, but also in utero, and may eventually result in improving the prognosis of babies with PA/CPS. Serial observation of the fetuses with PA/CPS may also suggest the possible pathogenesis of PA/CPS in utero.

Echocardiography↗