Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Pulmonary Atresia”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 19 recordsLinked to original sources
PubMedDate not supplied

[Tricuspid atresia with pulmonary atresia].

The clinical presentation and long-term follow-up of 14 cases of tricuspid atresia associated with pulmonary atresia were reviewed. The electrocardiograms, hemodynamic findings and a definition of anatomic types were outlined in order to facilitate therapeutic decisions. In these types of tricuspid atresia the clinical presentation depends on the patent ductus. Despite the caliber of the ductus arteriosus, the poor tolerance to the malformation is frequent and the clinical presentation is similar to those malformations with decreased pulmonary blood flow (cyanosis since birth and hypoxic spells). In considering the age of these patients, the modified Blalock-Taussig anastomosis is the initial procedure. The Fontan procedure should be considered carefully as an alternative in older children.

Angiocardiography↗
PubMed1999

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↗
PubMed1999

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↗
PubMed1991

The surgical anatomy of tetralogy of Fallot with pulmonary atresia rather than pulmonary stenosis.

We examined the pertinent surgical features of the anatomy of 56 hearts having tetralogy of Fallot with pulmonary atresia instead of stenosis, or malformations with pulmonary atresia closely related to tetralogy. We took particular cognizance of the pulmonary arterial supply in 15 hearts in which this was derived through systemic-to-pulmonary collateral arteries, dissecting, as far as possible, the bronchopulmonary segmental distribution of the collateral arteries compared to the intrapericardial pulmonary arteries in 11 of these hearts. Two of the hearts had absence of intrapericardial pulmonary arteries, so that a solitary arterial trunk left the base of the heart. Evidence of an atretic subpulmonary infundibulum was found in 40 of the hearts, while such an infundibulum was lacking in the remainder. The pulmonary atresia was muscular in 43 hearts, valvar in 11, while the pulmonary trunk was absent in the other two hearts. In the hearts with collateral arteries, on average 2.6 collaterals were found in each case, varying from two to five per case. Only one of these arose from a brachiocephalic artery, the others all arising from the descending aorta. The distribution of collateral arteries in two cases was remarkably reminiscent of the arrangement of bronchial arteries. As far as could be judged, 16.5 bronchopulmonary segments on average were supplied in each heart, 5.1 exclusively by collateral arteries, 11.8 by intrapericardial pulmonary arteries and an average of 0.64 segments per case having a shared supply.

Collateral Circulation↗
PubMed1995

The management of tetralogy of Fallot with pulmonary atresia and diminutive pulmonary arteries.

Since September 1991, 14 consecutive patients with tetralogy of Fallot, pulmonary atresia, and diminutive pulmonary arteries have undergone staged repair. All patients had multiple aortopulmonary collateral arteries and the ductus arteriosus was absent in 11. Mean sizes of the right and left pulmonary arteries were 2.2 +/- 0.7 mm and 1.9 +/- 0.8 mm, respectively (range 0.5 to 3.0 mm). Eight patients (57%) have subsequently received complete repair. Age at initial procedure (shunt, right ventricle-pulmonary artery conduit, or direct aorta-pulmonary artery anastomosis) in this group was 5.3 +/- 6.8 months. The number of operative procedures to achieve complete repair was 2.9 +/- 0.8 per patient (range 2 to 4). Intraoperative postrepair peak right ventricle-left ventricle pressure ratio was 0.57 +/- 0.17. Six of 8 patients (75%) required additional interventional procedures (mean 1.5 +/- 1.2 per patient) for angioplasty of peripheral pulmonary artery stenoses, coil embolization of aortopulmonary collateral arteries, or intra-operative insertion of intravascular pulmonary artery stents. Mean follow-up from complete repair was 8.7 +/- 8.3 months (range 0.5 to 23.8 months) and is complete. There was one in-hospital death at 45 days, and one late cardiac death at 20.3 months. Six patients had initial palliative operations (unifocalization, right ventricle-pulmonary artery conduit, direct aorta-pulmonary artery anastomosis, or transannular outflow patch) but have not undergone complete repair. Age at initial procedure in this group was 27.9 +/- 56.9 months (range 0.27 to 155 months), and mean follow-up from initial procedure was 10.9 +/- 11.2 months (range 0 to 31.4 months). The operative mortality rate was 33% (2 of 6 patients). There was one late noncardiac death at 5.3 months. Three patients are awaiting further intervention or repair. This experience suggests that complete repair is feasible even in patients with extremely diminutive pulmonary arteries (< or = 3.0 mm). Pulmonary artery growth is facilitated by early (3 to 6 month) establishment of central pulmonary artery flow by right ventricle-pulmonary artery conduit (pulmonary arteries > 1.5 mm) or by direct ascending aorta-pulmonary artery anastomosis (pulmonary arteries < 1.5 mm). Subsequent interventional catheterization and operative procedures as required for pulmonary artery stenoses and coil embolization of collateral arteries allow continued recruitment of central pulmonary arteries and may obviate or minimize the need for unifocalization procedures.

Angioplasty↗
PubMed1988

Management of pulmonary atresia or critical pulmonary stenosis and intact ventricular septum with a small or hypoplastic right ventricle.

Twenty-one neonates and infants less than 3 months old undergoing cardiac surgery for an obstructed right ventricular outflow tract, intact ventricular septum and a small or hypoplastic right ventricle were retrospectively analyzed, in order to assess the effects of a change in management protocol. Seven of the 8 patients with critical pulmonary stenosis survived surgery using a transannular outflow patch, whereas only 1 of the 8 patients with pulmonary atresia survived the same operation. Two patients in the latter group died 2 and 3 months after surgery but with complications arising from surgery. Of 5 patients with pulmonary atresia who had a modified Blalock Taussig shunt, 3 patients survived the surgery and were discharged home. These results significantly indicate that there is an unacceptably high mortality for the relief of pulmonary atresia (with intact septum) using a transannular outflow patch, and a Blalock Taussig shunt is the preferred operation. The transannular outflow patch is a safe operation for neonates with critical pulmonary stenosis, irrespective of the size of the right ventricle.

Cardiac Catheterization↗
PubMed2004

[Palliative right ventricle outflow reconstruction in tetralogy of Fallot with pulmonary atresia and hypoplastic pulmonary artery].

The management of small pulmonary artery in tetralogy of Fallot (TOF) with pulmonary atresia (PA) is complicated. Our strategy is palliative right ventricle outflow tract reconstruction (RVOTR). The aim of this study is to determine whether palliative RVOTR is useful as the first stage operation. Since 1994, 7 patients with TOF, pulmonary atresia and hypoplastic pulmonary arteries underwent palliative RVOTR. All patients survived operation without complication except for 1. He had pseudoaneurysm at right ventricular outflow patch requiring aneurysmectomy and re-RVOTR 1.5 months after the initial RVOTR. Nine months after RVOTR in the average, pulmonary artery index (PAI) increased from 139 +/- 87 to 306 +/- 156 (p < 0.05). No patient had pulmonary high flow or pulmonary hypertension. Two of them had pulmonary coractation due to ductal tissue in the pulmonary artery, necessitating additional modified Blalock-Taussig shunts at 8 and 10 months old. One patient with TOF, PA, major aorto-pulmonary collateral artery (MAPCA) had corrective surgery after 2 additional operations and interventional catheterization. Five patients of this series already have undergone corrective surgery, whereas 1 is waiting for it. RVOTR is a useful method for pulmonary artery growth in patients with TOF, PA and diminutive pulmonary artery.

Abnormalities, Multiple↗
PubMed1996

Staged repair of tetralogy of Fallot and pulmonary atresia without central pulmonary arteries.

A 15-year-old boy with tetralogy of Fallot and pulmonary atresia without central pulmonary arteries who was successfully treated using a staged approach is presented. The first stage consisted of the creation of central pulmonary arteries. In the second stage, the continuity between the right ventricle and the pulmonary arteries was established with closure of the ventricular septal defect. Some patients previously considered to be unsuitable candidates for correction may be successfully repaired using this approach.

Adolescent↗
PubMed1997

Unifocalization and systemic-to-pulmonary shunt using internal mammary artery for tetralogy of Fallot and pulmonary atresia with diminutive pulmonary artery and arborization anomaly.

We report successful palliation of tetralogy of Fallot and pulmonary atresia with diminutive pulmonary arteries and arborization anomaly. Unifocalization and systemic-to-pulmonary artery shunt using an internal mammary artery were successfully performed. The internal mammary artery shunt provided a sufficient blood supply for a diminutive and fragile pulmonary artery, and brought a progressive improvement of exercise intolerance and polycythemia probably due to the growth potential.

Cardiovascular Surgical Procedures↗
PubMed1984

Surgical management of hypoplastic right ventricle with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum.

Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum includes 26 patients with pulmonary atresia and 4 with critical pulmonary stenosis. Group 1 consisted of 8 neonates managed initially by transventricular valvotomy; 6 later required a secondary procedure, with 100% survival. Group 2 had 11 neonates managed by aorta-pulmonary artery shunting without operative death. However, only 3 have survived over the long term and 1 has required an additional shunt procedure. Group 3 had 9 infants who underwent concomitant valvotomy and shunting. There were 4 operative deaths and 1 late death. Finally, Group 4 included 2 infants managed by primary repair at 3 days and 6 days old with prosthetic enlargement of the right ventricle; 1 required the addition of a shunt. Both are alive. Seven of the 15 patients in Groups 1, 2, and 3 who survived neonatal palliative procedures have undergone reparative operations. Two had no growth of the right ventricle and underwent repair after conversion to tricuspid atresia, by a Fontan procedure. Five had prosthetic enlargement of the right ventricle in childhood with 1 late death. Findings of this review were as follows: (1) effective palliation of pulmonary atresia and intact ventricular septum or critical pulmonary stenosis with cavitary hypoplasia of the right ventricle is rare unless transventricular flow can be established; (2) establishment of transventricular flow produces a high incidence of cavitary "growth," which permits later repair; (3) the Fontan operation is available for repair in patients who have no cavitary growth; and (4) when all three portions of the right ventricular cavity can be identified by angiography, a primary repair can be performed in the neonatal period with a good long-term prognosis.

Aorta↗
PubMedDate not supplied

Pulmonary atresia or critical pulmonary stenosis with intact interventricular septum diagnosed in utero: echocardiographic findings and post-natal outcome.

BACKGROUND: Survival of neonates with critical pulmonary obstruction depends on cardiac morphology and function but also on proper perinatal management. METHODS: Postnatal outcome of five neonates with critical pulmonary stenosis or pulmonary atresia detected in utero is reported. RESULTS: Right ventricular morphology and ratio between tricuspid and mitral annulus at Fetal Echocardiography were the most important prognostic parameters. Increasing severity of obstruction and of right ventricular hypoplasia was documented in two fetuses. CONCLUSION: Prenatal diagnosis was useful for planning delivery; we hypothesize that it can be of benefit on postnatal outcome. In selected cases, earlier delivery and relief of obstruction could have beneficial effect on prognosis.

Female↗
PubMed1993

Tetralogy of Fallot with pulmonary atresia. Rehabilitation of diminutive pulmonary arteries.

BACKGROUND: Patients with tetralogy of Fallot, pulmonary atresia, and diminutive pulmonary arteries are a high-risk group for whom there is no consensus on the correct approach to medical management. The purpose of this report is to review a 14-year experience in the treatment of these patients comparing management schemes. METHODS AND RESULTS: Between January 1978 and August 1988, 91 patients with tetralogy of Fallot and pulmonary atresia had an adequate evaluation of their pulmonary artery anatomy before any surgical management. Forty-eight of these patients had diminutive pulmonary arteries (38 to 104 mm2/m2) supplied by aortopulmonary collaterals and were managed in four different fashions. Of the 9 patients repaired primarily, 7 died early and the two survivors had poor hemodynamic outcome. Of the 9 patients conservatively managed with no intervention before 5 years of age, 4 died and only 1 had a satisfactory hemodynamic result after repair. Of 10 shunted patients, 3 died and 3 had satisfactory repairs. Since 1984, we have rehabilitated pulmonary arteries with (1) right ventricle to pulmonary artery surgical graft, (2) balloon dilation of residual pulmonary artery stenoses and embolization of collaterals, and (3) surgical closure of ventricular septal defect and repair of remaining obstructions. Of 20 patients so managed, 7 died after various stages, but 10 of 20 had complete repairs. All repaired patients with subsystemic right ventricular pressures had at least one successful pulmonary artery dilation. CONCLUSIONS: A combined catheter-surgery approach begun at an early age in patients with tetralogy of Fallot and pulmonary atresia with diminutive pulmonary arteries appears to enhance the chances of satisfactory complete repair.

Angioplasty, Balloon↗