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One case of atresia of the common pulmonary vein is discussed at the light of the few reports found in the literature. The presence of criteria distinguishing this type of obstacle to pulmonary venous return from the other cases, is of practical diagnostic interest; indeed, its diagnosis leads to surgical intervention, theoretically curative.
BACKGROUND: In patients with complex pulmonary stenosis or atresia, a detailed delineation of all sources of pulmonary blood supply is necessary for planning surgical and transcatheter procedures and usually requires diagnostic cardiac catheterization. The goals of this study were to determine whether gadolinium-enhanced 3D magnetic resonance angiography (MRA) can provide a noninvasive alternative to diagnostic catheterization and to compare MRA and x-ray angiography measurements of pulmonary arteries and aortopulmonary collaterals (APCs). METHODS AND RESULTS: Thirty-two patients with pulmonary stenosis or atresia (median age: 4.7 years, range: 1 day to 46.9 years) underwent both MRA and cardiac catheterization (median time: 1 month). Diagnoses included tetralogy of Fallot (TOF) with pulmonary atresia (n=13), TOF with pulmonary stenosis (n=4), post-Fontan palliation (n=5), and other complex congenital heart disease (n=10). Compared with catheterization and surgical observations, MRA had a 100% sensitivity and specificity for the diagnosis of main (n=10) and branch pulmonary artery (PA) stenosis or hypoplasia (n=38), as well as absent (n=5) or discontinuous (n=4) branch PAs. All 48 major APCs diagnosed by catheterization were correctly diagnosed by MRA. Three additional APCs were diagnosed by MRA but not by catheterization. The mean difference between MRA and catheterization measurements of 33 pulmonary vessel diameters was 0.5+/-1.5 mm, with a mean interobserver difference of 0.4+/-1.5 mm. CONCLUSIONS: Gadolinium-enhanced 3D MRA is a fast and accurate technique for delineation of all sources of pulmonary blood supply in patients with complex pulmonary stenosis and atresia and can be considered a noninvasive alternative to diagnostic catheterization with x-ray angiography.
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Twenty-four patients with previous surgery for repair of tetralogy of Fallot were in need of subsequent operative procedures for either residual pulmonary stenosis or pulmonary valve incompetence. The second operation was performed 1 to 21 years after the initial repair. The patients with pulmonary valve incompetence had evidence of marked right ventricular dysfunction and required pulmonary valve replacement. The patients with residual pulmonary stenosis had modifications of their pulmonary outflow tract to reduce the gradient. Five of the 15 patients with stenosis required pulmonary valve replacement. Clinical improvement has been good, but objective reversal of right ventricular dysfunction has been difficult to document.
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The Maltese population constitutes an ideal location for epidemiological and historical studies dealing with congenital heart malformations. Pulmonary stenosis was studied retrospectively from 1943 to 1994. A sharp, significant decline in age at diagnosis was found, which predates the introduction of echocardiography. All defects are now diagnosed by echocardiography, which has resulted in an increased prevalence at birth of this lesion, particularly of pulmonary stenosis not requiring intervention. The prevalence at birth of pulmonary stenosis from 1990 to 1994 was 1.65/1000 live births (95% CI: 1.21-2.24), with 1.11/1000 mild lesions (95% CI: 0.76-1.62) and 0.54/1000 lesions requiring intervention (95% CI: 0.31-0.92). The prevalence at birth overall was significantly higher than that reported in recent studies with similar methodologies due to the higher pickup of milder variants of pulmonary stenosis by echocardiography. Significantly more pulmonary stenosis was found in females than in males.
A case of infundibular pulmonary stenosis occurring years after a mediastinal radiation for Hodgkin's disease is reported. As far as the literature is known to us, a similar case has not yet been described. It is most likely that the development of the stenosis is due to the radiation therapy, since a murmur was not heard before radiation and a mild stenosis was proved several years after radiation. Now, a high gradient with the typical signs of an infundibular pulmonary stenosis was present. Unfortunately the patient refused the operation. Further possibilities for acquired pulmonary stenosis and cardial complications after mediastinal radiation for Hodgkin's disease will be discussed.
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This study was conducted to investigate the outcome of balloon valvuloplasty for critical pulmonary stenosis in young infants. During a 6.2-year period between January 1992 and February 1998, 34 infants with critical pulmonary stenosis, aged 1 to 58 days (16.8+/-16.6 days), underwent attempted balloon valvuloplasty in this institution. The procedure was accomplished in 28 patients, but failed in six. Surgical pulmonary valvotomy was performed in the six patients with one mortality. Immediately following valvuloplasty, the mean right ventricular systolic pressure decreased from 109.2+/-28.6 to 55.1+/-23.6 mm Hg in the 28 patients (P<0.01). The mean pressure gradient decreased from 85.6+/-29.4 to 26+/-21.4 mm Hg (P<0.01). However, one who had a severely hypoplastic right ventricle requiring prolonged prostaglandin E1 infusion after valvuloplasty underwent a right ventricular outflow tract patch. After a follow-up period ranging from 2 months to 6.4 years (30.5+/-19.1 months), one patient developed recurrent pulmonary stenosis and underwent a repeated balloon valvuloplasty. Of the 27 patients (79%) with a definitive success of balloon valvuloplasty, the mean pressure gradient estimated with Doppler echocardiography at most recent follow-up was 15.2+/-6.8 mm Hg. Therefore, balloon valvuloplasty is the procedure-of-choice for critical pulmonary stenosis. Surgery should be reserved for those with unsuccessful balloon valvuloplasty.
The case of a 9-year-old girl with pulmonary stenosis complicated by acquired bleeding tendency after Jatene's operation is reported. Coagulation study revealed that platelet count and von Willebrand factor were reduced. Catheterization study revealed severe pulmonary stenosis, the pressure gradient between the right ventricle (RV) and pulmonary artery (PA) being 190 mmHg. A link between pulmonary stenosis and bleeding tendency was suggested and the patient was diagnosed as having acquired von Willebrand disease due to activated platelet/von Willebrand factor interactions enhanced by "shear stress" at the site of pulmonary stenosis. Right ventricular outflow reconstruction was done when she was 9 years-old. Due to a protocol based on the results of the challenge test that we instituted to determine the efficacy and effective duration of blood derivatives and hemostatic agents, perioperative massive bleeding was avoided. Bleeding tendency disappeared and coagulation study findings normalized with correction of the abnormal hemodynamic state.
We speculated that a relationship may exist between transient peripheral pulmonary stenosis and the closure of the ductus arteriosus. Fifty preterm infants had pulmonary artery and ductal color Doppler flow velocity assessments performed before and after closure of the ductus arteriosus. No flow turbulence or increase in velocity was observed immediately after birth, although a significant discrepancy in size was observed between the main pulmonary artery and its two branches. After closure of the ductus, 15 infants had signs of transient peripheral pulmonary stenosis of the left pulmonary artery in association with a significant decrease of diameter at the origin of the same artery. In all 50 infants, no significant gradient was observed in the right pulmonary artery. We conclude that, at least in the preterm infant, transient peripheral pulmonary stenosis is not present at birth but is an acquired phenomenon closely related to closure of the ductus arteriosus.
Eight patients with mild to moderate valvular pulmonary stenosis underwent serial physiological studies before surgical intervention. Average pulmonary valve area at the initial study was 0.59 cm.2 and at the second study (average 7.8 years later) was 1.09 cm.2, resulting in an average increase of 0.49 cm.2 (p greater than 0.05). In normal subjects during early years the pulmonary valve area increases linearly with age and with increasing body surface area. Though patients with valvular pulmonary stenosis have smaller valve orifices, they also show an increase in valve size with increasing age and with increasing body growth. The greatest increase in valve area was observed in 4 patients (all under age 6) in whom body surface area increased more than 70 per cent.
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An analysis of 433 observations is presented (164 cases of isolated pulmonary stenosis, 132 cases of its combination with atrial septal defect, and 137--with ventricular septal defect), the patients being 1 year 1 month to 36 years old, and the form of their heart disease being verified during surgery on a "dry" heart. The mentioned diseases were classified into 3 groups according to the tactical importance of the pulmonary stenosis. Haemodynamic studies in patients with pulmonary stenosis and an intact ventricular septum have demonstrated that with age right-ventricular hypertension progresses the faster the greater the pulmonary artery valve is stenosed. A combination of a valvular stenosis with a large ventricular communication is accompanied by a levelling of the pressure in both ventricles, its absolute figures being somewhat higher in patients over 16 years of age.
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Although the results of intracardiac repair for pulmonary stenosis or atresia are generally good, some patients develop symptoms due to to pulmonary regurgitation and right ventricular dilatation. Implantation of a pulmonary homograft may have a beneficial effect on these symptoms. Twenty-seven patients with symptoms due to pulmonary regurgitation and right ventricular dilatation were reoperated. The indication for surgery was dyspnea in 19, arrhythmia in five and both symptoms in three patients. The arrhythmia was supra-ventricular in four, ventricular in two and both supra-ventricular and ventricular in another two patients. A cryopreserved pulmonary homograft was implanted in the right ventricular outflow tract. Mean interval between intracardiac repair and homograft implantation was 15.6 years. Mean age at homograft implantation was 23.0 years. Concomitant procedures were performed in 18 patients. There were two early deaths (7%). Replacement of the homograft was necessary in one patient, 21 months after insertion. This patient had residual pulmonary stenosis immediately after surgery. On mid-term follow up (mean 36 months) all patients with dyspnea as indication for operation improved, except one with right and left ventricular dysfunction. Only two of eight patients with arrhythmia improved. On echocardiography, pulmonary regurgitation decreased in all patients. Right ventricular dilatation decreased in only 14 patients (52%). We conclude that pulmonary regurgitation after transannular patching can be treated with implantation of a cryopreserved pulmonary homograft with acceptable mortality and low morbidity. Clinical improvement results from reduction of the right ventricular volume overload rather than from decline in right ventricular dilatation. Arrhythmia as the only indication for operation is controversial.