Echocardiographic evaluation, management and outcomes of bilateral arterial ducts and complex congenital heart disease: 16 years' experience

2002 ◽  
Vol 12 (3) ◽  
pp. 272-277 ◽  
Author(s):  
Alejandro Peirone ◽  
Mustafa M. Abdullah ◽  
Frank Dicke ◽  
Robert M. Freedom ◽  
Jeffrey Smallhorn
Keyword(s):  
Blood Supply ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Complex Congenital Heart Disease ◽  
Cross Sectional ◽  
Color Flow ◽  
Poor Outcomes ◽  
Collateral Vessels ◽  
The Right

Bilateral arterial ducts represent an uncommon form of pulmonary or systemic blood supply associated with complex congenital cardiac disease. We sought to determine the role of high-resolution cross-sectional echocardiography and color flow Doppler ultrasonography in assessing this condition, as well as to describe the management and outcome in a group of patients. A retrospective review was conducted of 11 newborns identified over a 16-year period as having bilateral arterial ducts. Pulmonary atresia associated with non-confluent pulmonary arteries was the dominant lesion, with the heterotaxy syndrome also frequently being recognized. Echocardiography best identified the source of blood supply to either the pulmonary or systemic circulations, allowing differentiation from collateral vessels. Stenosis of the right or left pulmonary artery at the initial site of ductal insertion needs careful evaluation on follow-up. Management of patients with this condition remains a challenge, as indicated by the poor outcomes observed in our series.

Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? a 5th aortic arch with a systemic-to-pulmonary arterial connection

2000 ◽  
Vol 10 (4) ◽  
pp. 419-422 ◽  
Author(s):  
Astolfo Serra ◽  
Francisco Chamie ◽  
R.M. Freedom
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right

AbstractMajor abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.

scholarly journals Pulmonary atresia with a ventricular septal defect and left pulmonary artery discontinuity: a case report 

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Hyun-Hwa Cha ◽  
Hae Min Kim ◽  
Won Joon Seong
Keyword(s):  
Pulmonary Artery ◽  
Subclavian Artery ◽  
Septal Defect ◽  
Left Subclavian Artery ◽  
Ductus Arteriosus ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Right Pulmonary Artery

Abstract Background Unilateral pulmonary artery discontinuity is a rare malformation that is associated with other intracardiac abnormalities. Cases accompanied by other cardiac abnormalities are often missed on prenatal echocardiography. The prenatal diagnosis of isolated unilateral pulmonary artery discontinuity can also be delayed. However, undiagnosed this malformation would have an effect on further prognosis. We report our case of a prenatal diagnosis of pulmonary atresia with ventricular septal defect and left pulmonary artery discontinuity. Case presentation A 33-year-old Asian woman visited our institution at 24 weeks of gestation because of suspected fetal congenital heart disease. Fetal echocardiography revealed a small atretic main pulmonary artery giving rise to the right pulmonary artery without bifurcation and the left pulmonary artery arising from the ductus arteriosus originating from the left subclavian artery. The neonate was delivered by cesarean section at 376/7 weeks of gestation. Postnatal echocardiography and multidetector computed tomography showed a right aortic arch, with the small right pulmonary artery originating from the atretic main pulmonary artery and the left pulmonary artery originating from the left subclavian artery. Patency of the ductus arteriosus from the left subclavian artery was maintained with prostaglandin E1. Right ventricular outflow tract reconstruction and pulmonary angioplasty with Gore-Tex graft patch was performed 25th day after birth. Unfortunately, the neonate died because of right heart failure 8 days postoperation. Conclusion There is a possibility that both pulmonary arteries do not arise from the same great artery (main pulmonary artery or common arterial trunk). Therefore, clinicians should check the origin of both pulmonary arteries.

Blood Flow Velocity Profiles in Pulmonary Branch Arteries in Lambs

1995 ◽  
Vol 117 (2) ◽  
pp. 237-241
Author(s):  
H. Katayama ◽  
G. W. Henry ◽  
C. L. Lucas ◽  
B. Ha ◽  
J. I. Ferreiro ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Maximum Velocity ◽  
Left Pulmonary Artery ◽  
Control Group ◽  
Pulsed Doppler Ultrasound ◽  
Right Pulmonary Artery ◽  
Blood Flow Velocities ◽  
The Right

We studied the detailed profiles of blood flow in the right and left pulmonary arteries using 20 MHz pulsed Doppler ultrasound equipment in a lamb model. Fourteen lambs aged four to six weeks were selected. In six lambs, monocrotaline pyrrole was injected parenterally to create pulmonary hypertension (PH group). Eight other lambs served as unaltered controls (control group). The blood flow velocities were sampled in 1mm increments along the anterior—posterior axis of the branch arteries. The maximum velocity of the forward flow in the left pulmonary artery was higher than that in the right pulmonary artery in the control group (71.7 ± 15.9cm/s vs 60.2 ± 13.5; p < 0.05). The fastest backward flow was located at the posterior position of the vessel in the right pulmonary artery in the control group. No significant bias in location was shown in the left pulmonary artery. Using indices of P90, acceleration time, P90*AcT, the velocity waveforms in the PH group were compared with those in the control group. In the left pulmonary artery, every index in the control group showed a significantly greater value that in the PH group. On the other hand, no significant differences were found between either group in the right pulmonary artery.

Coronary artery to pulmonary artery collateral as the principal source of pulmonary blood supply in pulmonary atresia and ventricular septal defect with absent left pulmonary artery

2020 ◽  
Vol 30 (11) ◽  
pp. 1728-1729
Author(s):  
Ibrahima S. Barry ◽  
Stefano Di Bernardo ◽  
Milan Prša
Keyword(s):  
Coronary Artery ◽  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Blood Supply ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Principal Source ◽  
Absent Left Pulmonary Artery

AbstractPulmonary atresia and ventricular septal defect is associated with variable sources of pulmonary blood supply. We present a case of a coronary artery to pulmonary artery collateral as the principal source of pulmonary blood supply.

Mechanical properties and dimensions of the major pulmonary arteries

1960 ◽  
Vol 15 (1) ◽  
pp. 92-96 ◽  
Author(s):  
Dali J. Patel ◽  
Donald P. Schilder ◽  
Alexander J. Mallos
Keyword(s):  
Pulmonary Artery ◽  
Pulse Pressure ◽  
Technical Assistance ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Cross Sectional ◽  
Measurement Control ◽  
The Mean ◽  
The Right ◽  
The Relationship

The relationship between instantaneous distending pressure and diameter of the pulmonary artery was studied in 18 living thoracotomized dogs. An electrical caliper with adequate recording characteristics was developed for instantaneous diameter measurement. Control observations were made over a range of pressures induced by rapid, right heart dextran-infusion and were compared with those obtained during norepinephrine administration. Results indicate: a) the pulse contours of the pulmonary artery pressure and diameter are essentially identical, indicating negligible inertance and viscous resistance of the vessel wall, b) the mean change in average radius during a cardiac cycle was ±7.8% ± 2.86 S.D. ± .32 S.E.M. (0.48%/cm H2O pulse pressure) under control conditions, c) the ratio of change in radius to pulse pressure, R/P, showed a significant decrease during norepinephrine administration when compared to control values within the same pressure range (P < .01) and d) the cross-sectional area of the main pulmonary artery exceeded that of the right and left combined. Note: (With the Technical Assistance of Alfred G. T. Casper) Submitted on August 25, 1959

Pulmonary atresia with intact ventricular septum in the fetus

1992 ◽  
Vol 2 (4) ◽  
pp. 367-376 ◽  
Author(s):  
Lindsey D. Allan ◽  
Andrew Cook
Keyword(s):  
Right Ventricle ◽  
Poor Prognosis ◽  
Pulmonary Atresia ◽  
Ventricular Septum ◽  
Fetal Life ◽  
Intact Ventricular Septum ◽  
Cross Sectional ◽  
The One ◽  
The Right ◽  
Echocardiographic Findings

AbstractThe objective of this study was to review the echocardiographic findings and autopsy correlates of pulmonary atresia with an intact ventricular septum when diagnosed during fetal life. All cases with this lesion (nearly 60) seen in the Perinatal Cardiology unit of Guy's Hospital since 1983 are part of this study. More than half of the cases were detected within the last two years, and all bar three were referred because of suspicion of a cardiac malformation detected on routine obstetric scanning. The cases were divided into two distinct groups. On the one hand were those with dilatation of the chambers of the right heart. These had a uniformly poor prognosis. Severe regurgitation of the tricuspid valve was a characteristic feature. On the other hand were the cases with cavitary hypoplasia due to mural overgrowth. Direct measurements of pressure in one such case revealed suprasystemic values in the right ventricle. Although contemplated, fetal intervention proved impossible. Autopsy revealed cases with either valvar or infundibular atresia. In most cases diagnosed prior to 24 weeks of gestation, mothers opted for termination of pregnancy. All cases with dilatation of the right ventricle died. Of pregnancies which continued, only 30% of children are still alive, all but one being less than two years of age at present. The use of cross-sectional and Doppler echocardiography permits the accurate diagnosis of pulmonary atresia with an intact ventricular septum during fetal life. Autopsied cases showed strong correlation with the echocardiographic findings. The poor prognosis in most cases warrants consideration in the future of intervention during fetal life.

Rapid onset of intrapulmonary arteriovenous shunting after surgical repair of tetralogy of Fallot with pulmonary atresia

2001 ◽  
Vol 11 (2) ◽  
pp. 236-239 ◽  
Author(s):  
Victor D. Ofoe ◽  
Usha Pratap ◽  
Zdenek Slavik
Keyword(s):  
Tetralogy Of Fallot ◽  
Pulmonary Atresia ◽  
Rapid Onset ◽  
Pulmonary Angiography ◽  
Complex Congenital Heart Disease ◽  
Arteriovenous Shunting ◽  
Intrapulmonary Shunting ◽  
Chest X Ray ◽  
The Right ◽  
Lung Lobes

We describe a 2-year-old girl with tetralogy of Fallot and pulmonary atresia, palliated as a neonate with a right modified Blalock Taussig shunt, who developed severe cyanosis following total correction in the absence of corresponding evidence of parenchymal lung disease on the chest X-ray. Selective pulmonary angiography showed new intrapulmonary shunting involving only the right middle and lower lobes only. The cyanosis resolved rapidly subsequent to inhalation of nitric oxide. To our knowledge, this is the first documented case of rapid onset of localised intrapulmonary right-to-left shunting, involving only two lung lobes, following biventricular repair for complex congenital heart disease.

scholarly journals Partial Anomalous Left Pulmonary Artery Sling in an Adult

2020 ◽  
Vol 10 ◽  
pp. 5
Author(s):  
Pierre D. Maldjian ◽  
Kevin R. Adams
Keyword(s):  
Pulmonary Artery ◽  
Incidental Finding ◽  
Pulmonary Arteries ◽  
Left Pulmonary Artery ◽  
Pulmonary Trunk ◽  
Pulmonary Artery Sling ◽  
Airway Compression ◽  
Right Pulmonary Artery ◽  
Left Pulmonary Artery Sling ◽  
The Right

We report a case of a partial anomalous left pulmonary artery sling in an adult patient as an incidental finding on computed tomography. There is a normal bifurcation of the pulmonary trunk into right and left pulmonary arteries with anomalous origin of the left upper lobe pulmonary artery from the right pulmonary artery. The anomalous vessel passes between the trachea and esophagus forming a partial left pulmonary artery sling without airway compression.

scholarly journals Staged Correction of Pulmonary Atresia, Ventricular Septal Defect and Collateral Arteries

2021 ◽  
Author(s):  
Pieter van de Woestijne ◽  
M. Mokhles ◽  
Ingrid van Beynum ◽  
Peter de Jong ◽  
Jeroen Wilschut ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Pulmonary Vasculature ◽  
Collateral Arteries ◽  
Staged Approach ◽  
The Right ◽  
Rv Function

Objectives Pulmonary atresia (PA) with ventricular septal defect (VSD) and systemic-pulmonary collateral arteries (SPCA’s) has a variable anatomy with regard to the pulmonary vasculature, asking for an individualized surgical treatment. A protocol was applied consisting of staged unifocalization and correction. Methods Since 1989 39 consecutive patients were included (median age at first operation 13 months). In selected cases a central aorto-pulmonary shunt was performed as first procedure. Unifocalization procedures were performed through a lateral thoracotomy. Correction consisted of shunt takedown, VSD closure and interposition of an allograft between the right ventricle and the reconstructed pulmonary artery. Postoperatively and at follow up echocardiographic data were obtained. Results In 39 patients 66 unifocalization procedures were performed. Early mortality was 5%. Seven patients were considered not suitable for correction, four of them died. One patient is awaiting further correction. Correction was done successfully in 28 patients. Operative mortality was 3% and late mortality 11%. Median follow-up after correction was 19 years. Eleven patients needed homograft replacement. Freedom from conduit replacement was 88%, 73% and 60% at 5, 10 and 15 years respectively. Right ventricular function was reasonable or good in 75 % of the patients. Conclusions After complete unifocalization 30/37 patients (81%) were considered correctable. The main reasons for palliative treatment without correction were pulmonary hypertension and/or inadequate outgrowth of pulmonary arteries. Staged approach of PA, VSD and SPCA’s results in adequate correction and good functional capacity. RV function after correction remains reasonable or good in the majority of patients.

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