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PubMed1983

Clinical and echocardiographic features of pulmonary valve endocarditis.

We studied the clinical and echocardiographic features of eight patients with infective pulmonary valve endocarditis. In two patients, the vegetation was limited to the pulmonary valve; in the six other patients, infective lesions were also present on the mitral or aortic valves. None of the patients were addicted to narcotics. Seven of the eight patients had underlying congenital heart disease. In six patients the organism responsible for the infective endocarditis was Streptococcus viridans. Two-dimensional echocardiography performed using a wide-angle sector scanner was more useful than M-mode echocardiography for evaluating patients with pulmonary valve endocarditis.

Adolescent↗
PubMed1976

Long-term results of surgical treatment for pulmonary valve stenosis.

109 children who survived surgical treatment for isolated pulmonary valve stenosis were followed for up to 17 years. In all the postoperative status was assessed as satisfactory. Cardiac catheterization repeated in 43 gave a resting valve gradient below 40 mmHg. The 22 children whose pulmonary valves had been excised were as healthy as the 87 who had undergone pulmonary valvotomy. Consideration was given to the desirable length of postoperative review. Except for the few children with symptoms before operation, a postoperative increase in exercise tolerance was not a feature.

Adolescent↗
PubMedDate not supplied

The syndrome of absent pulmonary valve and ventricular septal defect--anatomical features and embryological implications.

Four cases of absent pulmonary valve in combination with ventricular septal defect are reported. In this syndrome hypo- and dysplasia of the pulmonary valve is constantly associated with a big ventricular septal defect, formation of a huge pulmonary artery aneurysm and absence of the ductus arteriosus. Presence or absence of a right ventricular outflow tract obstruction is the criteria for classification into two forms. Absence of the pulmonary valve, right ventricular outflow tract obstruction and a malalignment-type ventricular septal defect produced by a conotruncal malseptation process represent the primary complex of malformations. Consecutive intrauterine cardiac failure is most probably prevented by prenatal closure of the ductus arteriosus. Pulmonary artery aneurysm and also dilatation of the right ventricular outflow tract as well as a whole lot of other coexisting deformities can be explained by a cascade of hemodynamical sequelae started by this ductus closure in utero. An embryological scheme explaining the genesis of this syndrome is derived from a morphological analysis of the constituting incoherent-appearing anatomical features.

Angiocardiography↗
PubMed2001

Pulmonary valve insertion late after repair of Fallot's tetralogy.

OBJECTIVES: To analyze the results of pulmonary valve insertion late after initial repair of Fallot's tetralogy. Pulmonary insufficiency (PI) after correction of Fallot's tetralogy is usually well tolerated in the short term, but is associated with symptomatic right ventricular dilatation and an increased risk of ventricular arrhythmias over longer periods of time. METHODS: From 1993 to July 2000, 51 patients were reoperated for PI at a mean age of 25.7+/-11.9 years. The mean age at initial repair was 6.4+/-7.2 years. Patients with a conduit inserted at initial operation, with absent pulmonary valve syndrome or with a more than moderate ventricular septal defect at reoperation were excluded from the study. A cryopreserved pulmonary (96%) or aortic (4%) homograft was implanted in the orthotopic position with the use of cardiopulmonary bypass 19.3+/-9.1 years (2.7-40.3 years) after initial correction. Preoperative symptoms (New York Heart Association, NYHA class), degree of PI (echo-Doppler, MRI), right ventricular dimensions (MRI) and QRS duration were compared to findings at last follow-up. RESULTS: Follow-up is complete and had a mean duration of 1.7+/-1.4 years. Hospital mortality was 2%. No serious morbidity occurred. Severe PI was present preoperatively in all patients. At last follow-up echo-Doppler studies showed PI to be absent or trivial in 96% and mild in 4% of patients. In 13 patients MRI studies were performed both pre- and postoperatively: in this group PI was reduced from a mean of 48 to 4%. After 6 months NYHA capacity class had improved significantly from 2.3+/-0.6 to 1.4+/-0.5. After 1 year end-diastolic and end-systolic right ventricular volumes were reduced significantly. Right ventricular ejection fraction and QRS duration remained unchanged. CONCLUSIONS: PI late after correction of Fallot's tetralogy may lead to serious symptomatic right ventricle dilatation. After pulmonary homograft insertion right ventricular dimensions decrease rapidly and functional improvement is observed in almost all patients.

Adult↗
PubMed1985

[Value of contrast echocardiography in the diagnosis of pulmonary valve insufficiency].

The directional analysis of contrast echographic flow lines is routinely used for tricuspid insufficiency diagnosis. The value of this noninvasive technique for the assessment of pulmonic regurgitation is not yet established. Therefore we evaluated the contrast M-mode echocardiograms at the pulmonary valve in 55 patients. Echographic contrast was obtained by injection of 6-8 ml of indocyanin-saline solution into an antecubital vein. In all 5 patients with documented pulmonic insufficiency (3 patients after repair of tetralogy of Fallot, one with pulmonic endocarditis, one posttraumatic) typical diastolic retrograde directed flow lines crossing the pulmonary valve were recorded. In addition, 6 of 18 patients with primary or secondary pulmonary hypertension exhibited early to middiastolic retrograde flow lines at the pulmonary valve. In contrast, none of the normals (N = 13), and none of 19 patients with various cardiac diseases but unaffected pulmonary valve and normal pulmonary artery pressure, had contrast echographic evidence of pulmonary insufficiency. There were distinctive differences between the contrast flow patterns of the patients with organic pulmonary insufficiency and patients with functional pulmonic regurgitation due to pulmonary hypertension. It is concluded that the directional analysis of contrast echographic flow lines at the pulmonary valve allows a sensitive assessment of pulmonary regurgitation using standard echocardiographic equipment.

Adult↗
PubMed2002

Percutaneous insertion of the pulmonary valve.

OBJECTIVES: We report our experience of percutaneous valve insertion in pulmonary position in humans. BACKGROUND: Over the past 40 years, prosthetic conduits have been developed to surgically establish continuity between the right ventricle and the pulmonary artery. However, stenosis and insufficiency of the conduit due to valvular degeneration or panus ingrowth frequently occur, limiting patients' lifespan. Percutaneous stenting of conduits has recently emerged as a technique for delaying surgical replacement, but it creates a pulmonary regurgitation when crossing the valve. METHODS: Seven children and one adult with stenosis and/or insufficiency of the pulmonary graft underwent percutaneous implantation of a bovine jugular valve in pulmonary position. RESULTS: Percutaneous pulmonary valve (PV) replacement was successful in all patients. No complications occurred in early follow-up. Angiography, hemodynamic studies and echocardiography after the procedure showed no significant regurgitation of the implanted valve. Implantation was effective in relieving the obstruction in five patients. All patients showed improvement in their clinical status at the latest follow-up (mean 10.1 months). CONCLUSIONS: Non-surgical insertion of the PV is possible without any major complications. This new technique may have an important role in the management of conduit obstructions and pulmonary regurgitation.

Adolescent↗
PubMed1998

Clinical performance of the native pulmonary valve in the systemic circulation.

BACKGROUND AND AIM OF THE STUDY: In a number of corrective and palliative procedures the autologous pulmonary valve is used as the systemic semilunar valve. This study reviews the surgical results and function of the native pulmonary valve in the systemic position after various surgical procedures. METHODS: Between January 1994 and December 1997, the autologous pulmonary valve was transferred functionally or anatomically into the systemic circulation in 89 patients. Follow up echocardiograms and cardiac angiograms were reviewed for 51 neonates with transposition of the great arteries after an arterial switch operation (ASO), in 21 patients after first-stage palliation of hypoplastic left heart syndrome (HLHS), in eight children and adults with pulmonary autograft aortic valve replacement (Ross procedure), and in nine patients with a pulmonary artery-to-aortic anastomosis (Damus-Kaye-Stansel (DKS) procedure) in complex heart defects with outflow obstruction. RESULTS: Nine patients (five with HLHS) died; thus, overall mortality rate was 10.2%. There was no evidence of valve-related mortality. Trivial insufficiency following ASO was noted in 11 patients, with no progression of incompetence over time. None of the HLHS patients had pulmonary insufficiency preoperatively, but all showed mild regurgitation on postoperative echocardiography. There was a moderate increase in insufficiency which was attenuated after an early second-stage palliation. Three of nine patients undergoing a DKS anastomosis demonstrated a hemodynamically insignificant insufficiency. Modification of the surgical technique avoided postoperative regurgitation. Four of seven patients having a Ross procedure showed trivial but non-progressive neoaortic regurgitation. CONCLUSIONS: Based on this experience, the autologous pulmonary valve performs adequately at intermediate term follow up. Postoperatively, trivial regurgitation was a frequent finding but was hemodynamically insignificant. Progression or late development of insufficiency as well as stenosis were rare problems.

Adolescent↗
PubMed1995

Pulmonary and left ventricular decompression by artificial pulmonary valve incompetence during percutaneous cardiopulmonary bypass support in cardiac arrest.

BACKGROUND: In cardiac arrest, use of percutaneous cardiopulmonary bypass support (PCPS) may lead to left ventricular loading, with deleterious effects on the myocardium, and is often accompanied by an increase in pulmonary artery pressure. The present study was designed to assess the potential of artificially induced pulmonary valve incompetency to retrogradely decompress the left ventricle during PCPS in ventricular fibrillation. METHODS AND RESULTS: Studies were performed using a standardized experimental animal model in sheep (n = 12; body weight, 77 to 112 kg). When PCPS was used during fibrillation, an increase in left ventricular pressure (from 21.4 +/- 5.0 mm Hg after 1 minute to 28.4 +/- 9.5 mm Hg after 10 minutes of fibrillation) was observed in all animals, with a simultaneous increase in pulmonary artery pressure in 6 animals, from 15.5 +/- 3.8 to 24.3 +/- 5.4 mm Hg (group A). In these animals, artificial pulmonary valve incompetency, which was induced by a special "pulmonary valve spreading catheter," led to effective decompression of both the pulmonary circulation (decrease in pulmonary artery pressure from 24.3 to 11.3 mm Hg) and the left ventricle (decrease in left ventricular pressure from 30.5 to 17.7 mm Hg). We simultaneously measured a decrease in the myocardial release of lactate (increase in arterial coronaryvenous difference in lactate content from -0.01 to 0.14 mmol/L), demonstrating the myocardial protective effect of the procedure. In contrast, in 6 animals without an increase in pulmonary artery pressure during PCPS (group B), artificial pulmonary valve incompetency did not reduce left ventricular loading, which was probably because of competent mitral valves in these animals. CONCLUSIONS: In case of increasing pulmonary artery pressure during PCPS in cardiac arrest, artificial pulmonary valve incompetency might be a useful tool for effective pulmonary and retrograde left ventricular decompression.

Animals↗
PubMed1980

[Specificity and sensitivity of pulmonary valve motion in echocardiographic examination as an index of pulmonary hypertension].

Many investigation have been made to establish the role of echocardiography in the diagnosis of pulmonary hypertension based on some parameters derived from the pulmonic valve leaflet echo motion. By observing 160 echo tracings of pulmonary valve motion, the authors calculated the specificity, sensibility and predictive value of these parameters as predictor of pulmonary hypertension, comparing echo data with corresponding haemodynamic determination of pulmonary artery systolic, diastolic and mean pressure and the degree of pulmonary vascular resistances. By means of the statistical analysis of variance correlation between "a" wave depth and severity of pulmonary hypertension was made. "A" wave depth and RPEP/RVET ratio resulted quite sensitive and highly specific indices of pulmonary hypertension, in particular "a" wave depth proved to correlate very well with the level of pulmonary artery systolic, diastolic and mean pressure and with the degree of pulmonary vascular resistances. Highly specific, but not always detectable, was the presence of a midsystolic notch. In conclusion, pulmonary valve echo motion detected by M-mode technique provides useful index of pulmonary hypertension, chiefly by the measurement of "a" wave depth which can also predict its severity.

Echocardiography↗
PubMed2004

Prenatal diagnosis of absent pulmonary valve syndrome in association with 22q11 deletion.

OBJECTIVE: To describe the prenatal sonographic appearances in cases of absent pulmonary valve syndrome and the importance of investigating the presence of 22q11 deletion. METHODS: We describe 2 cases, which were referred because of a suspicion of a cardiac malformation. In both cases, a large anechoic mass emerging from the right ventricle was visualized and identified as an aneurysmal dilatation of the pulmonary trunk with hypertrophy of the right ventricle. The diagnosis of tetralogy of Fallot with absent pulmonary valve syndrome and a secondary diverticular dilatation of the pulmonary artery was made. A review of the literature revealed another 18 cases of prenatal diagnosis of absent pulmonary valve syndrome with or without knowledge of chromosomal abnormalities. RESULTS: Pathologic examinations confirmed the diagnosis of absent pulmonary valve syndrome in both cases. Final results of fetal karyotyping revealed a 22q11 deletion in the first case. CONCLUSIONS: An abnormal 4-chamber view with an aneurysmal dilatation of the pulmonary trunk should suggest the diagnosis of this rare congenital anomaly. Perinatal death occurs in more than 60% of cases and is usually associated with hydrops fetalis, the presence of other malformations, or both. Even in the absence of extracardiac malformations, investigation for 22q11 deletion in cases of conotruncal cardiac abnormalities is recommended.

Chromosome Deletion↗
PubMed2006

Long-term effect of pulmonary valve replacement on QRS duration in patients with corrected tetralogy of Fallot.

OBJECTIVE: To analyse the long-term course of QRS duration after pulmonary valve replacement in patients with a previous correction for tetralogy of Fallot. SETTING: Tertiary referral centres. METHODS: In a retrospective study, 99 adult patients with tetralogy of Fallot, who had undergone a first pulmonary valve replacement late after initial total correction, were identified from the CONCOR (CONgenital CORvitia) registry. Computer-generated QRS durations were obtained from 12-lead electrocardiogram ECG reports in the medical records. A mixed linear regression model was used to analyse the course of QRS duration over time and to identify risk factors for increase in QRS duration over time. Composite end point was created from sudden cardiac death, ventricular tachycardia or implantable cardioverter-defibrillator discharge. RESULTS: In total, 99 patients (57% men, mean (SD) age at pulmonary valve replacement 29 (11) years) with a median follow-up of 4.9 (0.1-16) years were analysed. In patients with preoperative QRS <120 ms, surgery caused no significant change in QRS duration (increase 1.3 (7.9) ms; p = 0.65), and after surgery, QRS duration remained stable over time (increase 0.0064 (0.059) ms/year; p = 0.98). By contrast, in patients with a preoperative QRS of 150-180 ms or QRS > or =180 ms, surgery resulted in QRS shortening (mean decrease 9.9 (SE 4.3) ms, p = 0.021, and 12.2 (SE 2) ms; p<0.001, respectively). During follow-up, a QRS widening 1.1(1.3) ms/year (p<0.001) in both groups was observed. In patients with a preoperative QRS > or =180 ms, no significant difference was observed in the number of patients reaching the composite end point compared with patients with a preoperative QRS of 150-180 ms (25% vs 7%; p = 0.08). However, the former more often reached QRS > or =180 ms again after surgery compared with the latter (53% vs 13%; p = 0.02, respectively). None of the patients with a preoperative QRS > or =180 ms died during follow-up. CONCLUSION: In our study, we observed a decrease in QRS duration directly after surgery, followed by a steady increase, in patients with a preoperative QRS >150 ms. The beneficial effect of pulmonary valve replacement on QRS duration was transient. The risk of developing ventricular arrhythmias after surgery was substantial when preoperative QRS was > or =180 ms, but mortality remained low.

Death, Sudden, Cardiac↗
PubMed2005

[Anesthetic management for an infant with tetralogy of fallot with absent pulmonary valve].

Tetralogy of Fallot with absent pulmonary valve (TOF/APV) is a rare disease, and characterized by massive dilatation of pulmonary artery. Bronchial compression by the aneurysm-like dilated pulmonary artery sometimes results in severe respiratory compromise. Surgery including closure of VSD and right ventricular outflow construction is often needed in infancy. But, even after the surgical correction, patients often suffer from right ventricular failure due to right ventriculotomy and poor development of pulmonary capillaries. Therefore, preventing pulmonary hypertension is important during the perioperative period. We report an infant with TOF/APV. This 3 month-old boy developed respiratory distress suddenly. After the induction of anesthesia with sevoflurane, fentanyl, and vecuronium, continuous infusion of alprostadil and olprinone was started. Cardiopulmonary bypass (CPB) was instituted smoothly. VSD was closed and right ventricular outflow tract was reconstructed. Dilated pulmonary arteries were resected and plicated. Weaning from CPB was easy and postoperative course was uneventful with mild pulmonary hypertension. In the cases predisposed to right ventricular failure like TOF/APV, olprinone may be useful.

Alprostadil↗
PubMed1998

Echocardiographic detection of pulmonary valve papillary fibroelastoma.

Papillary fibroelastomas of the heart are rare lesions usually discovered at autopsy or incidentally at surgery. Although these lesions are benign and generally asymptomatic, they can cause valvular dysfunction or embolize to vital structures. In this case report, we describe a pulmonary valve papillary fibroelastoma detected by echocardiography in an adult. Most of the 12 cases of pulmonary valve papillary fibroelastoma reported in the literature were discovered incidentally at autopsy or during surgery. To our knowledge, this is the first reported case of pulmonary valve papillary fibroelastoma detected by echocardiography. Rest imaging before exercise echocardiography for evaluation of atypical chest pain in a 42-year-old white female demonstrated a mass on the pulmonary valve. The mass was further characterized by transesophageal echocardiography and excised during open heart surgery. Pulmonary valve papillary fibroelastoma was diagnosed histopathologically. This case illustrates the additional diagnostic value of comprehensive 2D imaging in the rest phase before doing exercise echocardiography.

Adult↗
PubMed1994

[Pulmonary root reconstruction with a cryopreserved pulmonary allograft for mechanical pulmonary valve thrombosis].

A 58-year-old woman suffering from congestive heart failure caused by a mechanical pulmonary valve thrombosis was operated on with a pulmonary allograft. She had experienced pulmonary valvotomy and resection of infundibular stenotic muscle for congenital pulmonary stenosis at the age of 23 years old. She got congestive heart failure caused by pulmonary regurgitation, and underwent pulmonary valve replacement with a St. Jude Medical (SJM) valve when she was 48 years old. She suffered from episodes of a thrombosed SJM valve in 1984 and 1993. Each time, thrombolytic treatment with urokinase or recombinant tissue-type plasminogen activator was effective. She suffered from the third episode of SJM valve thrombosis in January 1994. As thrombolytic treatment was not effective this time, the thrombosed SJM valve was resected and her pulmonary root was reconstructed with a cryopreserved pulmonary allograft. Postoperative course was uneventful, and she now enjoys her life without anticoagulant therapy.

Cryopreservation↗
PubMedDate not supplied

Two-dimensional and Doppler echocardiography in the diagnosis of absent pulmonary valve syndrome (surgery without catheterization).

The majority of severely affected infants with absent pulmonary valve syndrome develop varying degrees of respiratory distress. Cardiac catheterization and angiocardiography are still performed in the diagnosis of these infants prior to surgery. However, considering the high risks of these invasive investigations for severely symptomatic infants, diagnosis using only noninvasive methods becomes important. In this regard, we present three cases of absent pulmonary valve syndrome, diagnosed pre-operatively by both two-dimensional and Doppler echocardiography. The diagnosis was confirmed by cardiac catheterization and angiocardiography in two cases and by surgery in a symptomatic infant. Therefore, we are of the opinion that the two-dimensional and Doppler echocardiographic methods are most reliable in diagnosing absent pulmonary valve syndrome, and that severely symptomatic infants can be referred for surgery without catheterization.

Angiocardiography↗
PubMed1995

Homograft replacement of fungal endocarditic pulmonary valve.

Right-sided infective endocarditis is uncommon but is increasingly seen as a reflection of the prevalence of drug abuse, chronic intravenous catheters and associated congenital malformations. Isolated pulmonary valve endocarditis has rarely been reported and there has been only one previous report of isolated pulmonary valve fungal endocarditis. This resolved with antimicrobials. We describe a case of isolated mycotic pulmonary valve endocarditis resistant to existing anti-fungal chemotherapy, which necessitated pulmonary valve resection and replacement with a homograft.

Adult↗
PubMed1970

Congenital absence of the pulmonary valve.

The clinical, radiological, electrocardiographic, and haemodynamic findings in 7 patients with the syndrome of congenital absence of the pulmonary valve with ventricular septal defect are reported. Findings included a pansystolic murmur and a low-pitched delayed diastolic murmur at the left sternal border with no pulmonary valve closure sound, an rsR' pattern in V1 on the electrocardiogram, and a chest x-ray showing a large heart and gross dilatation of the main, left or right pulmonary arteries but normal or reduced vascularity of the peripheral lung fields. Cardiac catheterization showed a left-to-right shunt at ventricular level in every case, though in one the shunt was bidirectional. Despite right ventricular systolic pressures at least 75 per cent of systemic level in every patient, the pulmonary artery pressure was invariably normal. Pulmonary stenosis was shown to exist at infundibular level, at valve ring level, or at both. Selective right ventricular angiography was diagnostic: the main and right or left pulmonary arteries were aneurysmally dilated and there was an abrupt transition in calibre between these vessels and their immediate branches; enlargement of the right pulmonary artery caused distortion and downward displacement of the left atrium in 4 patients; in 2 there was a flat ;diaphragm' in the pulmonary valve position.The diagnosis was confirmed in 3 patients by necropsy. The pulmonary valve was represented by at most a rudimentary fringe of tissue. Histological examination of the pulmonary arteries in one patient showed a correlation between the dilatation of the artery and the structure of its wall.

Adolescent↗
PubMedDate not supplied

Absent pulmonary valve with tricuspid atresia or severe tricuspid stenosis: report of three cases and review of the literature.

Absence of the pulmonary valve occurs usually in association with tetralogy of Fallot and occasionally with an atrial septal defect or as an isolated lesion. Very rarely it occurs with tricuspid atresia, intact ventricular septum, and dysplasia of the right ventricular free wall and of the ventricular septum. We present the clinical, anatomic, and histologic findings of a new case, and for the first time, the data from two patients with absent pulmonary valve and severe tricuspid stenosis, who exhibited similar histologic findings. We also reviewed the clinical and anatomic data of 24 previously published cases and compared them with the new cases. In all three new cases, the myocardium of the right ventricle was very abnormal. In the two cases with tricuspid stenosis, large segments of myocardium were replaced with sinusoids and fibrous tissue. In the case with tricuspid atresia, the right ventricular free wall contained only fibroelastic tissue. The ventricular septum in all three patients showed asymmetric hypertrophy and in two of the three patients, multiple sinusoids had replaced large segments of myocardial cells. The left ventricular free wall myocardium and the walls of the great arteries were unremarkable. Our data indicate that myocardial depletion involving the right ventricular free wall and the ventricular septum and its replacement by sinusoids and fibroelastic tissue occur not only in cases of absent pulmonary valve with tricuspid atresia but also in cases of absent pulmonary valve with tricuspid stenosis. The degree of myocardial depletion varies and is more severe when the tricuspid valve is atretic.

Fatal Outcome↗