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Palliative balloon valvoplasty of the pulmonary valve in tetralogy of Fallot.

Balloon dilation of the pulmonary valve was performed in 54 patients with tetralogy of Fallot with severe cyanosis, high haematocrit and severe valvar pulmonary stenosis. Clinical, echocardiographic, angiographic, and haemodynamic data were analyzed before and after the procedure. After balloon dilation, the systemic oxygen saturation increased from a mean value of 66% to 85%. The mean value of the haematocrit before dilation was 55 + 13, and decreased to 47 after dilation (p < 0.002) in 2 months follow-up. Balloon dilation increased the size of the pulmonary valvar orifice from a mean value of 9 + 5 mm to 11.5 + 2 mm (p < 0.005). The mean Z score of the pulmonary valves, which was -3 + 1.3 before dilation, increased to -1.1 + 1.1 immediately after the procedure (p < 0.05). The size of the right and left pulmonary arteries increased after dilation from 9 mm to 10 mm, and from 8.7 + 2.4 mm to 9.8 + 2.3 mm, respectively (p < 0.05). The comparable mean Z scores increased from -2.8 + 1.9 SD to -1.8 + 1.4 SD, and from -2.4 + 1.9 SD to -1.5 + 1.6 SD for the right and left branches, respectively (p < 0.05). In patients with stenosis at the bifurcation of the pulmonary trunk and hypoplasia of the left artery, successful dilation of the pulmonary valve lead to an increase of flow and improvement in size of the hypoplastic segment. In conclusion, initial balloon dilation of the pulmonary valve in tetralogy of Fallot resulted in increase of the Z score for the pulmonary valve and improved antegrade pulmonary blood flow, inducing growth of the pulmonary arteries and ameliorating the anatomic and physiologic preoperative condition.

Cardiac Catheterization↗

Tetralogy of Fallot with absent pulmonary valve. A continuing challenge.

Absent pulmonary valve syndrome (APVS); the combination of tetralogy of Fallot (TOF) with agenesis of the pulmonary valve, is a relatively rare cardiac malformation. Despite the anatomic similarity with classic TOF, the pathophysiology is strikingly different. Data on 10 patients (3 male, 7 female) with APVS, treated between January 1978 and December 1995, were retrospectively reviewed. During this period a total of 2920 children underwent correction of a variety of congenital cardiac anomalies, of which 246 patients (8%) had a correction for TOF. Two patients with APVS presented within the first four months of life with severe cardiorespiratory distress and required several operative procedures. The remaining eight patients had only mild to moderate respiratory and/or cardiac symptoms and elective intracardiac repair was performed on those between the ages of 10 months and 9.5 years. Associated cardiac anomalies seen in five patients included aberrant coronary artery, absent or interrupted left pulmonary artery, partial AVSD and aberrant azygos continuation. In those electively corrected, the strategies used were ventriculotomy (7), pulmonary homograft (3) and aneurysmorrhaphy (2). There were two deaths, one in each group of patients, as a result of progressive respiratory insufficiency and cardiac tamponade, respectively. The follow-up of the eight survivors ranged from 2 to 11 years (median 6.75). All have a normal effort tolerance; only one child is on digoxin therapy, and one child continues to suffer bronchospastis episodes. Our experience with infants with this lesion is limited but underlines the different approaches required, depending on the age of presentation.

Child↗

Does intraoperative transesophageal echocardiography predict pulmonary valve dysfunction during the Ross procedure?

OBJECTIVE: To determine the value of intraoperative transesophageal echocardiography for the assessment of the pulmonary valve anatomy and the pulmonary autograft performance in patients undergoing the Ross procedure. DESIGN: Open, prospective, observational survey. SETTING: Favaloro Foundation, single institution. PARTICIPANTS: Consecutive patients undergoing elective Ross procedure (n = 87). INTERVENTIONS: Pulmonary valve function and anatomy were assessed by transesophageal echocardiography and the surgeon. Pulmonary autograft function was assessed after implantation. Regurgitation was considered mild (+/4), moderate (++/4), moderate-to-severe (+++/4), and severe (++++/4). Patients were restudied during midterm follow-up. MEASUREMENTS AND MAIN RESULTS: The Ross procedure was done in 74 patients (85%). Overall mortality was 3.4%. Mean follow-up was 24 +/- 13 months. The Ross procedure was not done in 13 patients (15%): 6 patients had a bicuspid pulmonary valve, 6 patients had >3 mm fenestrations, and 1 patient had regurgitation. The surgeon diagnosed anomalies in the pulmonary valve through direct observation. Transesophageal echocardiography was not sensitive enough to diagnose pulmonary valve defects in 12 of 13 patients with anomalies. Pulmonary valve regurgitation was identified by intraoperative transesophageal echocardiography in only 1 patient. Autograft regurgitation was 1.07 +/- 0.35 at postoperative evaluation. At 1, 6, and 12 months, it was 1.25 +/- 0.7 (p = 0.18), 1.27 +/- 0.9 (p = 0.185), and 1.29 +/- 0.8 (p = 0.17). The difference in values was not statistically significant. Four patients (5.4%) showed an increase in regurgitation during the first transthoracic autograft control. CONCLUSION: Intraoperative transesophageal echocardiography allows assessment of autograft performance after implantation. This method is not helpful, however, in detecting pulmonary valve anatomic anomalies.

Adult↗

Body surface area as a predictor of aortic and pulmonary valve diameter.

BACKGROUND: Predicting cardiac valve size from noncardiac anatomic measurements would benefit pediatric cardiologists, adult cardiologists, and cardiac surgeons in a number of decision-making situations. Previous studies correlating valve size with body size have been generated with the use of fixed autopsy specimens, angiography, and echocardiography, but primarily in the young. This study examines the relation of body surface area to measurements of the left ventricular-aortic junction (aortic valve anulus diameter) and the right ventricular-pulmonary trunk junction (pulmonary valve anulus diameter) in 6801 hearts across a wide spectrum of ages. METHODS: From June 1985 to October 1998, cardiac valves from 6801 donated hearts were analyzed morphologically. Donor age was newborn to 59 years (mean 31 +/- 17 years; median 32 years). Calculated body surface areas ranged from 0.18 to 3.55 m(2). Aortic (n = 4636) and pulmonary valve diameters (n = 5480) were measured from enucleated valves suitable for allograft transplantation. Mean valve sizes were computed for ranges in body surface area in 0.1-m(2) increments. RESULTS: For adult men (age >/= 17 years), the mean aortic valve diameter was 23.1 +/- 2.0 mm (n = 2214) and the mean pulmonary valve diameter was 26.2 +/- 2.3 mm (n = 2589). For adult women, the mean aortic valve diameter was 21.0 +/- 1.8 mm (n = 1156) and the mean pulmonary valve diameter was 23.9 +/- 2.2 mm (n = 1408). The mean indexed aortic valve area was 2.02 +/- 0.52 cm(2)/m(2) and the pulmonary valve area 2.65 +/- 0.52 cm(2)/m(2). Between 82% and 85% of the variability was explained by the size of the patient. Regression equations were developed both overall and separately for men and women, although the additional contribution of sex above that of body size was less than 1%. CONCLUSIONS: Aortic and pulmonary valve diameters are closely related to body size. Thus, body surface area, when used in conjunction with other clinically accepted evaluations, is a useful tool for estimating normal aortic and pulmonary valve size.

Adolescent↗

Absent pulmonary valve syndrome with associated anomalies of the pulmonary blood supply.

We report four cases of so-called absent pulmonary valve syndrome associated with absence or anomalous origin of the left pulmonary artery. The fate of the patients with this condition appears to be affected mainly by the occurrence of pulmonary complications (three of our patients died of pulmonary causes, two of them after surgery). The proper timing of corrective surgery is still uncertain. Preoperative investigations should strive to obtain a clear-cut identification of the pulmonary arteries, particularly the left one or, in its absence, of the anomalous vascular supply to the lung. Right ventriculography, as employed by us, is insufficient. Pulmonary angiography, aortography and pulmonary vein "wedge" angiography may be needed.

Adult↗

Repair of congenital pulmonary incompetence by bicuspidization of the pulmonary valve.

We report the successful repair of pulmonary incompetence in an adult due to the congenital absence of the posterior leaflet of the pulmonary valve. The repair consisted of bicuspidization of the pulmonary valve, which achieved competence and eliminated the symptoms and echocardiographic manifestations of right ventricular overload while preserving the native valve.

Female↗

Surgical management of tetralogy of Fallot with absent pulmonary valve.

Tetralogy of Fallot and absent pulmonary valve (T-APV) is associated with massively dilated pulmonary arteries that cause tracheobronchial compression in the newborn and heart failure and cyanosis in older patients. Corrective operations have been attended by high mortality rates due to pulmonary insufficiency causing right heart failure (RHF) and pulmonary complications. Pulmonic valve insertion (PVI) with complete repair has resulted in improved survival. During the last 5 years, 152 patients with tetralogy were corrected. Of these, 10 patients (ages 51 days to 34 years) had absent pulmonary valve. One patient (age 51 days) presented with severe RHF and pulmonary insufficiency and 9 patients presented with mild RHF and cyanosis. Chest roentgenograms showed increased cardiothoracic ratio and pulmonary prominence in all. Arteriography revealed massively enlarged pulmonary arteries with a mean ratio of 2:1 for size of pulmonary artery to aorta. Associated pulmonic stenosis and insufficiency was present in all. Seven patients underwent closure of ventricular septal defect (VSD) and PVI. Of these, 3 had PVI (2 tissue and 1 prosthetic) with outflow patch and 4 had right ventricle to pulmonary artery (RV-PA) tissue valved conduits. Two patients had repair without PVI, and 1 had repair with a monocusp pericardial valve patch. Nine patients have done well with no episodes of thromboembolism or infection. Death occurred in a 51-day-old infant who had VSD closure and relief of pulmonic stenosis. Pulmonary valve insertion seems to be indicated in these patients because it lowers peak pulmonary artery pressure and, thus, reduces compression effects on the trachea and bronchi. When PVI was used, RHF did not occur post-operatively.

Adolescent↗

Pulmonary valve echo motion in clinical practice.

Examination of the pattern of pulmonary valve echo motion provides useful diagnostic information in a variety of clinical situations. This report describes the normal patterns and variations in pulmonary valve echo motion. It further discusses the applications and limitations of M-mode and cross-sectional echocardiography in detecting obstruction to right ventricular outflow at both the valvular and infundibular level; the wide ranging effects of increases in pulmonary artery pressure on the pulmonary valve echogram; and alterations in right ventricular compliance and volume which may combine to produce diastolic opening of the pulmonary valve. It is emphasized that the thin pliable pulmonary leaflets move in response to local alterations in pressure and flow. The patterns of pulmonary valve echo motion, therefore, although not specific for any particular clinical disorder, may provide valuable, indirect information concerning local pressure and flow characteristics and, as such, may prove extremely helpful when applied to a specific clinical situation.

Echocardiography↗

The quantification of pulmonary valve haemodynamics using MRI.

The optimum slice location within the pulmonary root to quantify pulmonary valve haemodynamics was examined using magnetic resonance (MR) phase velocity mapping. MRI was carried out on 15 patients with congenital aortic valve disease. Although the patients had aortic valve disease, all measurements were made on the pulmonary valve. Systolic (Q(SYS)) and diastolic (Q(DIAS)) blood flow volume and cardiac index (CI) were determined at four pulmonary artery locations. The change in diastolic flow volume relative to slice 1, closest to the pulmonary valve, was also calculated. For a change in axial position of 1.5 cm, i.e. from 0.5 to 2 cm from the annulus, there was a change in diastolic flow volume of 4.4 ml. There was a significant increase in the mean diastolic flow from 3.4 to 7.7 ml (p = 0.01 between slice positions 0.5 and 2 cm. However, there was no significant change in CI, 3.4-3.7 l/min/m2 (p = 0.14) over the same distance. We believe that two factors are responsible for these results. The first is that of compliance, whose effects can be minimized by placing the MR slice close to the valve, however, this will not account for the second factor, being that of valve motion, and hence diastolic pulmonary valve flow or regurgitant volume will be underestimated. The degree of underestimation may only be important at mild and moderate levels of regurgitation or if changes in regurgitation are to be temporally measured.

Adolescent↗

Enhanced accumulation of the isoprostane, 8-epi-PGF2 alpha, in human aortic and pulmonary valves of patients with coronary heart disease.

The aim of the present study was to investigate whether the isoprostane 8-epi-PGF2 alpha differently accumulates in semilunar valves of patients suffering from coronary heart disease (CHD, n = 19) as compared to valves from healthy heart donors (controls, n = 6). Sections from isolated aortic and pulmonary valves were analyzed by semiquantitative immunohistochemistry. The 8-epi-PGF2 alpha-content was determined by using a specific radioimmunoassay. The accumulation of 8-epi-PGF2 alpha in both valves was higher in CHD-patients in comparison to controls (Aortic valves: 36.49 +/- 11.26% vs. 15.78 +/- 3.04%; pulmonary valves: 46.79 +/- 9.80% vs. 14.99 +/- 3.57%). The results from the radioimmunoassay revealed comparable findings in both groups (CHD vs. controls: 395.95 +/- 86.09 vs. 139.50 +/- 47.46 pg/mg protein in the aortic valves and 430.47 +/- 76.30 vs. 147.33 +/- 53.84 pg/mg protein in pulmonary valves). Pulmonary valves seem to be more susceptible to oxidative stress than aortic valves as evidenced by a higher accumulation of 8-epi-PGF2 alpha in CHD patients. Considering the data presented in this study, we suggest that 8-epi-PGF2 alpha is a valuable indicator of oxidative injury in human semilunar valves.

Aged↗

Tetralogy of Fallot with absent pulmonary valve syndrome.

Tetralogy of Fallot with absent pulmonary valve syndrome is a rare variant of tetralogy of Fallot marked by severe pulmonary insufficiency in utero and significant, sometimes massive, dilation of the pulmonary arteries. This syndrome can be associated with airway abnormalities that can cause respiratory compromise. Patients who present with respiratory symptoms early in life continue to present a major clinical challenge associated with relatively high mortality rates, typically in the 20% to 30% range. A variety of surgical techniques have been developed to address absent pulmonary valve syndrome that may improve long-term outcomes.

Child, Preschool↗

[Large patent ductus arteriosus and interventricular communication associated with congenital absence of the pulmonary valve].

A case of pulmonary valve agenesis with ventricular septal defect and large patent ductus arteriosus is reported in a neonate. Heart failure occurred at 10 days of life; clinical examination showed continuous murmur. Echocardiography and angiocardiography visualized a major dilatation of the pulmonary artery. At cardiac catheterization there was an important left to right shunt through a large ductus, and pulmonary hypertension. There was also marked pulmonic regurgitation. Because of respiratory distress, ligation of ductus arteriosus was performed at 5 weeks of life. Five years later the patient is still in good condition. Patent ductus arterious is rarely associated with pulmonary valvular agenesis and ventricular septal defect. Our case is not explained by the fetal circulation theory described in this heart malformation.

Abnormalities, Multiple↗

Expanding the indications for pulmonary valve replacement after repair of tetralogy of fallot.

BACKGROUND: Insertion of a competent pulmonary valve has been advocated to reduce right ventricular volume overload associated with pulmonary regurgitation (PR) after repair of tetralogy of Fallot. However the indications, proper timing, and long-term benefits of restoring pulmonary valve function remain controversial. METHODS: Thirty-six patients (aged 15.2 +/- 9.2 years) underwent pulmonary valve implantation (31 homografts, 5 heterografts) 12.2 +/- 6.9 years after tetralogy repair. Additional surgical procedures included pulmonary artery augmentation (n = 14), closure of septal defects (n = 10), and cryoablation and endocardial resection of ventricular tachycardia (n = 2). RESULTS: All patients have had clinical improvement in their exercise capacity. Preoperative and postoperative bicycle ergometry tests in 6 patients demonstrated significant improvement in the percent of predicted peak workload (68.5% +/- 19.8% to 80.7% +/- 17.4%, p < 0.015). One midterm death occurred in a 38-year-old patient with a history of ventricular tachycardia who died suddenly 2 years after pulmonary valve insertion. Postoperative echocardiographic measurements were available in 34 patients at a mean follow-up of 5 years. There was a 30% reduction in right ventricular end-diastolic diameter indexed to body surface area after surgery (30.1 +/- 10.2 to 18.6 +/- 6.0 mm/m(2), p < 0.0001). Two patients required conduit replacements at 1 and 9 years postoperatively. CONCLUSIONS: Timely insertion of a competent pulmonary valve in children, adolescents, and young adults with significant PR after tetralogy of Fallot repair results in subjective and objective improvement in exercise capacity and is associated with reduction in right ventricle size.

Adolescent↗

Cross-sectional echocardiographic visualization of the stenotic pulmonary valve.

Real-time, cross-sectional echocardiograms of the pulmonary valve were recorded in 22 patients with valvular pulmonary stenosis (VPS) (14 mild, eight moderate or severe) and 25 normal subjects. Normally during systole the pulmonary leaflet echoes moved rapidly apart and in the fully opened position lay parallel and in close apposition to the margins of the pulmonary artery. In 20 of 22 patients with VPS in whom the pulmonary valve was recorded the systolic configuration of the leaflets, opening pattern of the leaflet echoes, and presence of presystolic doming served to differentiate the stenotic valve from normal. In contrast M-mode recordings of the pulmonary valve were possible in only 12 of these 22 cases (seven mild and five moderate or severe) and suggested VPS in only the five cases with moderate or severe stenosis. Cross-sectional echocardiography offers a direct, noninvasive method for visualizing the stenotic pulmonary valve and should be improvement over the indirect M-mode data.

Adolescent↗

Percutaneous pulmonary valve placement in a 10-month-old patient using a hand crafted stent-mounted porcine valve.

Percutaneous replacement of the pulmonary valve in a right ventricle to pulmonary artery (RV-PA) conduit using bovine jugular valves has recently been described as an alternative to surgical options in selected patients weighing >or=20 kg. We report the first case of transcatheter pulmonary valve implantation in an infant and the first use of "off the shelf" components. A 12-mm (Hancock) porcine valve was sutured into a predilated Genesis 2510b stent. The valved stent was manually crimped and mounted on a 12-mm balloon. Snare-assisted deployment in the middle of the RV-PA conduit was accomplished without the use of a long sheath. After the initial deployment, a 14-mm balloon was used to further approximate the stent to the walls of the conduit. Short-term (11 month) follow-up has demonstrated continued valve integrity, markedly decreased right ventricular size and improved function and symptomatic improvement. At 10 months of age, this case represents the youngest patient to undergo percutaneous valve placement and documents the technical feasibility of the procedure.

Angioplasty, Balloon, Coronary↗