[Quantitative studies on the amplitude of the pulmonary valve closure sound in pulmonary stenoses and atrial septum defects].
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Adolescent↗
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Results for “PULMONARY VALVE”
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Value of the pulmonary valve echogram in estimating pulmonary artery pressure.
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Adolescent↗
[Anesthetic management of a neonate with critical pulmonary valve stenosis for balloon pulmonary valvuloplasty].
We administered general anesthesia for balloon pulmonary valvuloplasty (BPV) to a 19 day-old male infant, weighing 2,789g, with critical pulmonary stenosis. The patient had severe cyanosis and mild right heart failure. Atropine (0.01 mg.kg-1) was administered intravenously immediately before induction of anesthesia. Pancuronium (0.4 mg) was used to facilitate endotracheal intubation and for the subsequent control of ventilation. Anesthesia was maintained with oxygen and enflurane (0.25 approximately 0.5%) supplemented with intravenous administration of fentanyl (1.5 micrograms.kg-1). During catheterization for balloon pulmonary valvuloplasty, SpO2 and blood pressure decreased temporarily to 35% and 50 mmHg, respectively. Several side effects of balloon inflation have been reported, such as bradycardia, arrhythmia, and the decrease in systemic blood pressure and arterial oxygen saturation, mainly due to the occlusion of pulmonary blood flow. Therefore, it might be recommended that BPV should be performed under stable state of general anesthesia with continuous monitorings of especially ECG, arterial blood pressure, central temperature, SpO2, ETCO2 and urine output.
Anesthesia, General↗
Congenital absence of pulmonary valve leaflets.
Congenital absence of pulmonary valve leaflets is an uncommon condition usually associated with ventricular septal defect and an obstructive pulmonary valve ring. Twenty-one patients with these malformations are described. Twenty had an associated ventricular septal defect with ventriculoarterial concordance, and one also had transposition of the great arteries, ventricular septal defect, and obstructive pulmonary valve ring. The clinical features, cardiac catheterisation findings, and angiocardiographic results are presented. Twelve patients underwent cardiac surgery. Three patients died, one in the early, and the other two in the late postoperative period. The results, according to the surgical technique employed and postoperative cardiac catheterisation findings, showed that patients in whom the bioprostheses were implanted in the pulmonary position had a better late follow-up.
Adolescent↗
Assessment of pulmonary valve echogram in normal subjects and in patients with pulmonary arterial hypertension.
To assess the validity of ultrasound criteria for pulmonary arterial hypertension, we studied pulmonary valve motion in 28 patients and 20 normal subjects. In the latter group, we categorised normal movement of the posterior leaflet of the pulmonary valve in a fashion not previously described. Of the 28 patients, 19 had pulmonary arterial hypertension (pulmonary artery mean pressure more than 20 mmHg, range 22 to 72). Negative, flat, and positive e to f slopes occurred equally in normal subjects and patients. Maximal a wave excursion was less than 2 mm in 9 of 13 patients with pulmonary hypertension who had sinus rhythm, but was more than 2 mm in all normals and in the 9 patients with normal pulmonary artery pressure (69% sensitivity and 100% specificity). The b to c slope was more than 450 mm/s in 6 of 18 patients with pulmonary arterial hypertension and was less than 450 mm/s in all others (33% sensitivity and 100% specificity). The normalised right ventricular pre-ejection period was more than 0.095 (range 0.10 to 0.16) in 13 of the 19 patients with pulmonary arterial hypertension and less than 0.095 in all others (68% sensitivity and 100% specificity). A midsystolic notch occurred in patients with pulmonary arterial hypertension and in no normal subjects or patients with normal pulmonary arterial pressure. Of the 19 patients with pulmonary arterial hypertension, 18 were identified by one or more ultrasound criteria. Of the 5 patients who met only 1 criterion (increased normalised right ventricular pre-ejection period), 4 had atrial fibrillation. We conclude that measurement of the pulmonary valve e to f slope is useless for identifying pulmonary arterial hypertension. However, changes in normalised right ventricular pre-ejection, maximal a wave excursion, b to c slope, and the presence of a midsystolic notch, while insensitive, are highly specific for pulmonary atrial hypertension.
Adolescent↗
[A case report of atrial septal defect with quadricuspid pulmonary valve].
We reported the successful case of valvuloplasty on the quadriscuspid pulmonary valve which had pulmonary regurgitation. A 38-year-old woman was admitted with the complaint of palpitation. Atrial septal defect and pulmonary regurgitation were revealed preoperatively by the cardiac catheterization and doppler echocardiography. The malcoaptation of the pulmonary valve and pulmonary dilatation were diagnosed in echocardiography. At operation, the pulmonary valve was found quadricuspid and each cusp was same in size. The patient underwent the valvuloplasty on the abnormal cusps and the remarkable improvement of the coaptation was examined postoperatively. The patient had good clinical course for three years after operation.
Adult↗
Absent pulmonary valve syndrome with agenesis of the left pulmonary artery.
Absent pulmonary valve syndrome is represented by rudimentary nodules without identifiable leaflets, and it is commonly associated with a ventricular septal defect and tetralogy of Fallot. Unilateral absence of a pulmonary artery is also a rare congenital anomaly. The following is a case report of a one-day-old newborn with cyanosis. The physical examination revealed a to-and-fro murmur and no expansion of the left hemithorax. Echocardiography revealed tetralogy of Fallot, absent pulmonary valve, and enlarged main and right pulmonary arteries. The catheterization and angiography confirmed the diagnosis and absence of the left pulmonary artery.
Cyanosis↗
Pulmonary valve replacement with a porcine aortic heterograft.
Pulmonary valve replacement is an uncommon surgical procedure, yet lifesaving when performed under the appropriate circumstances. The patient reported on here had the classic indications for valve replacement: increased pulmonary vascular resistance and associated pulmonary valve incompetence following repair of a congenital heart defect resulting in right heart failure and secondary tricuspid insufficiency. A review of the literature provides the basis for the decision to replace the pulmonary valve with a glutaraldehyde-preserved porcine heterograft.
Animals↗
Absent pulmonary valve syndrome: surgical treatment and considerations.
BACKGROUND: The operative management of absent pulmonary valve syndrome remains controversial regarding the need for pulmonary valve implantation and remodeling of pulmonary arteries. Moreover, symptomatic infants are considered to have a poor prognosis. This retrospective report summarizes the experience of a single institution. METHODS: Between May 1977 and May 1995, 37 consecutive patients underwent repair of absent pulmonary valve syndrome. Patients were divided into two groups according to age at operation: group A (10 infants less than 1 year old) and group B (27 patients older than 1 year). Mean age at operation was 5 +/- 4 months in group A and 72 +/- 42 months in group B. Initially, repair consisted of ventricular septal defect closure and relief of right ventricular outflow tract obstruction combined with pulmonary valve implantation. More recently, the concept of treatment has evolved with pulmonary arterioplasty without pulmonary valve insertion, except in patients with elevated pulmonary artery pressure. RESULTS: Of the 37 patients, 34 had successful repair. The overall in-hospital mortality rate was 8% (two deaths in group A and one in group B). No hemodynamic data were correlated with operative death. Death was associated with longer extracorporeal circulation time (p = 0.005) and longer aortic cross-clamping time (p = 0.019). In fact, these were clearly related to more complex anatomy (p = 0.001): multiple ventricular septal defects in 1, left pulmonary artery arising from the ductus in another, and left pulmonary artery arising from the aorta in the remainder. Follow-up was available in 22 of the 34 survivors. Mean follow-up time was 30 +/- 47 months in group A and 38 +/- 33 months in group B. All but 1 had no restriction of exercise, and most of them had pulmonary incompetence of Doppler echocardiography. One developed severe exercise intolerance because of pulmonary valve stenosis (xenograft), leading to uneventful reoperation 123 months after initial repair. One infant died suddenly of complete atrioventricular block 3 months after repair. The late mortality rate was 5%. CONCLUSIONS: Surgical treatment of absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. This procedure is feasible and is recommended especially in markedly symptomatic infants.
Case-Control Studies↗
Clinical presentation, natural history, and outcome of patients with the absent pulmonary valve syndrome.
So-called 'absent pulmonary valve syndrome' is a rare cardiac malformation, usually associated with tetralogy of Fallot. Congenital absence of the leaflets of the pulmonary valve is less common when the ventricular septum is intact. Characteristic features of the syndrome include dysplasia or absence of the pulmonary valvar leaflets, permitting severe pulmonary regurgitation, and aneurysmal dilation of the pulmonary arteries. The purpose of our study was to review our experience with patients diagnosed as having the absent pulmonary valve syndrome, and to describe their clinical presentation, natural history, and outcome. We reviewed retrospectively data from 18 patients with absent pulmonary valve syndrome, 10 boys and eight girls, treated between March 1983 and May 2003. We identified two groups of patients, one made up of 11 patients with a ventricular septal defect, in whom the morphology of the subpulmonary outflow tract was phenotypic for tetralogy of Fallot, and another group, with seven patients, having an intact ventricular septum. Family history of congenital heart disease was common only in patients with ventricular septal defect, being found in 73%, all of whom were diagnosed during infancy with variable respiratory distress. Diagnosis was delayed in 43% of the patients with an intact ventricular septum. Cardiac surgery was performed in eight patients with ventricular septal defect (73%), compared to only two patients (28%) with an intact ventricular septum. Overall mortality was 28%, with five patients dying. Although our sample was small, two clinical patterns emerged depending on the presence or absence of a ventricular septal defect. Patients with a ventricular septal defect and phenotypic features of tetralogy of Fallot have a strong family history of congenital cardiac disease, develop respiratory symptoms during infancy and exhibit a variable prognosis, despite cardiac surgery. Patients with an intact ventricular septum are usually asymptomatic, present later in life, and show a relatively benign prognosis.
Age Distribution↗
A bicuspid pulmonary valve associated with tetralogy of fallot.
Congenitally bicuspid pulmonary valves are uncommon. When they occur, it is usually in association with other congenital cardiac lesions, most often a tetralogy of Fallot. We present a rare case of a patient with a congenitally bicuspid pulmonary valve who had pulmonary valve and RVOT reconstruction. The patient did well for 17 years, but needed further reconstruction when the pulmonary valve started getting stenosed and RV pressures went up significantly. The pulmonary valve showed fibroses, thickening, and focal calcification.
Adult↗
Echocardiographic evaluation of isolated pulmonary valve disease in adolescents and adults.
Congenital pulmonary valve disease is often not discovered until adolescence or adulthood. Transthoracic two-dimensional echocardiography can provide detailed information regarding right ventricular outflow anatomy, although images are often less satisfactory than those obtained in infants and children. The more recent addition of biplanar transesophageal echocardiography has enhanced our ability to image the right ventricular outflow tract, pulmonary valve, and pulmonary artery noninvasively. Pulsed and continuous-wave Doppler estimates of subvalvular and transvalvular gradients have proved to be accurate. Doppler color flow mapping has proved useful in determining the location and direction of stenotic and regurgitant flow. With no accepted standard for comparison, quantification of regurgitation remains problematic. In many cases, echocardiography has replaced catheterization and angiography in the evaluation and long-term follow-up of congenital pulmonary valve disease before and after intervention.
Adolescent↗
Orthotopic pulmonary valve replacement with a homograft.
Eight pulmonary valve replacements (PVR) have been performed from January 1992 to October 1994. Three patients (mean age 7.7 years, range two to 16 years) had absent pulmonary valve with tetralogy of Fallot and underwent primary PVR at the time of surgical correction. Five other patients, who had correction of tetralogy of Fallot (four cases) and of double outlet right ventricle with ventricular septal defect and pulmonary stenosis (one case), were reoperated for pulmonary regurgitation with progressive right ventricular dysfunction. Mean age at the time of reoperation was 18 years (range seven to 34 years). There was no early death. Early postoperative recovery was satisfactory in all of them. The follow up ranges from six to 35 months (mean 19 months). Seven patients were in functional class I and one in functional class II when they were last evaluated in the out-patient department and five of them were off diuretics and vasodilator. In the presence of right ventricular dysfunction pulmonary regurgitation is poorly tolerated. A competent and non-obstructive pulmonary valve is often life saving in these critically ill patients.
Adolescent↗
Bicuspid aortic and pulmonary valves in the Syrian hamster.
We studied the conditions of the aortic and pulmonary valves of 153 Syrian hamsters belonging to a single family subjected to high endogamous pressure. Semilunar valves of 143 specimens were examined using a stereomicroscope, and in 5 of these cases a histologic study was also performed. The remaining 10 specimens were studied by means of scanning electron microscopy. In 77 specimens both semilunar valves were normal. The aortic valve was bicuspid and the pulmonary valve was normal in 46 animals, while a normal aortic valve and a bicuspid pulmonary valve occurred in 24. In the remaining 6 specimens both semilunar valves were bicuspid. All bicuspid semilunar valves detected herein showed the same morphotype, namely with the leaflets oriented ventrodorsally. The incidence of these anomalous valves did not significantly differ between sexes. Results of a chi 2-test substantiate that conditions of both outflow tract semilunar valves are independent traits. Thus, concurrence of a bicuspid aortic valve and a bicuspid pulmonary valve in an individual is a random event. The present findings support the assumption that bicuspid aortic and pulmonary valves have different morphogenetic origin, although factors producing a bicuspid aortic valve and those leading to a bicuspid pulmonary valve are not mutually exclusive.
Animals↗
Transcatheter guidewire perforation of the pulmonary valve as a palliative procedure in pulmonary atresia with intact interventricular septum.
We report two cases of pulmonary atresia with intact interventricular septum in whom we successfully perforated and performed balloon dilation of the atretic pulmonary valve using a simple guidewire technique. The technical challenges of performing this procedure in small infants are highlighted.
Angiography↗
Cardiopulmonary performance during exercise in patients with repaired tetralogy of Fallot with absent pulmonary valve.
In patients with tetralogy of Fallot and absent pulmonary valve, the dilated pulmonary arteries sometimes result in bronchial compression and pulmonary symptoms due to airway obstruction, recurrent pulmonary infection, and development of bronchiectasis. After complete intracardiac repair, residual pulmonary disease may be expected to result in impaired cardiopulmonary performance during exercise. To assess this hypothesis, nine patients with tetralogy of Fallot and absent pulmonary valve underwent exercise testing and were compared to 38 patients with tetralogy of Fallot repaired using a transannular patch. All patients were exercised to maximum volition using a 1 minute incremental treadmill protocol with monitoring of pulmonary functions and expired gases. Maximal heart rate, maximal oxygen consumption, oxygen consumption at anaerobic threshold, and maximal respiratory exchange ratio were similar for the two groups. There was no significant difference for ventilation and gas exchange parameters at rest or at maximal exercise, and values for both groups were below the predicted normal for healthy subjects. Breathing reserve, however, did tend to be somewhat lower in the group with tetralogy of Fallot with absent pulmonary valve. In summary, despite significant preoperative symptoms, cardiopulmonary performance during exercise in patients with tetralogy of Fallot and absent pulmonary valve is similar to patients with tetralogy of Fallot repaired with a transannular patch.
Adolescent↗
[Percutaneous dilatation of pulmonary valve stenosis].
We present 11 cases of pulmonary valve stenosis who underwent percutaneous dilatation angioplasty. Patients were between 2 and 15 years old (mean age = 6.5 yrs) and all of them except 3 had moderate or severe degree of valve stenosis. Pulmonary anulus diameter ranged between 11 and 23 mm. at ECHO and between 10 and 21 mm. at angio. The catheter was chosen to be 20-40% larger than pulmonary anulus. The mean pressure gradient between RV and PA was 65 mmHg. before dilatation and 21 mmHg. after the procedure. The procedures were without complications, except one in which we observed an intermittent idioventricular rhythm for few days after the procedure. Percutaneous dilatation angioplasty has to be considered the treatment of choice for pulmonary valve stenosis.
Adolescent↗
Anatomy and formation of congenital bicuspid and quadricuspid pulmonary valves in Syrian hamsters.
BACKGROUND: Congenital bicuspid and quadricuspid pulmonary valves have received little attention because of their limited clinical relevance. However, knowledge of the mechanisms by which these anomalous valves develop is essential to obtain a more accurate survey of the etiological factors implicated in the malformations of the cardiac outflow tract in mammals. The present study was designed to assess the anatomical features of bicuspid and quadricuspid pulmonary valves in Syrian hamsters as well as to elucidate the mechanisms involved in the formation of these defective valves. METHODS: The sample examined consisted of 206 adults and 28 embryos belonging to a laboratory-inbred family of Syrian hamsters with a high incidence of congenital anomalies of the pulmonary and aortic valves. The study was carried out using histological techniques for light microscopy, semithin sections, and scanning electron microscopy. RESULTS: The pulmonary valve was tricuspid in 140 of the 206 adult hamsters, and in 124 of these tricuspid valves the dorsal commissure was more or less extensively fused. Another 45 hamsters possessed a bicuspid pulmonary valve with the sinuses oriented ventrodorsally. In 43 of these bicuspid valves, a raphe was located in the dorsal pulmonary sinus. The pulmonary valve was quadricuspid in a further nine specimens. The remaining 12 hamsters had a tricuspid pulmonary valve with a raphe-like ridge located in the right pulmonary sinus. In seven of these valves, the dorsal commissure showed a more or less extensive fusion. The embryos examined, aged between 11 days, 3 hours and 12 days, 6 hours postcoitum, were at the beginning of the valvulogenesis. In five of the 28 embryos, the pulmonary valve consisted of three mesenchymal valve cushions, right, left, and dorsal. In a further 17 embryos, the right and left valve cushions were more or less fused toward the lumen of the pulmonary artery. In the remaining six embryos, the left and dorsal valve cushions were normal, whereas the right cushion was divided into two lobes. CONCLUSIONS: The present findings suggest that in the Syrian hamster: (1) bicuspid pulmonary valves result from the extensive fusion of the right and left pulmonary valve cushions at the beginning of the valvulogenesis, (2) the partial fusion of the right and left pulmonary valve cushions leads to the formation of tricuspid pulmonary valves with a more or less extensive fusion of the dorsal commissure, (3) quadricuspid pulmonary valves result from the partition of one of the three valve cushions at a very early stage of the valvulogenesis, and (4) the partial division of the right pulmonary valve cushion may lead to the development of tricupsid pulmonary valves with a raphe-like ridge located in the right pulmonary sinus. In addition, the present findings, together with previous observations in Syrian hamsters, indicate that in this species the mechanisms by which bicuspid and quadricuspid pulmonary valves develop are similar to those by which bicuspid and quadricuspid aortic valves form, respectively. However, the primary factor or factors that induce the malformations of the pulmonary valve operate independently from those inducing the malformations of the aortic valve.
Animals↗