P268 Anomalies of pulmonary arteries in Tetralogy of Fallot in developing countries: study of 100 cases in indian population

2020 ◽  
Vol 41 (Supplement_1) ◽  
Author(s):  
A Tiwari ◽  
PARAG Barwad ◽  
U M A Dabi
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Surgical Management ◽  
Pulmonary Valve ◽  
Pulmonary Stenosis ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Great Vessels ◽  
Pulmonary Artery Abnormalities

Abstract Introduction Right ventricular outflow tract (RVOT) obstruction in some or other form is one of the major components of Tetralogy of Fallot. It can occur at any level, isolated or in combination, from Infundibulum (most common), Pulmonary valve, Main pulmonary artery, Right and Left pulmonary arteries and up to their segmental branches. Incidence of pulmonary artery anomalies are reported between 15-20%, though some reported as high as 40%.  Purpose  The objective of this retrospective  observational study is to determine the presence and degree of pulmonary artery  abnormalities and associated cardiac defects in patients with Tetralogy of Fallot and to  define the pulmonary arterial anatomy to guide the further surgical management. Method A total of 100 newly diagnosed or follow up cases of TOF irrespective of age (range 5 days- 32 years) and gender (32 female, 68 male) who were planned for surgical management and referred for evaluation of pulmonary artery anatomy. All patient underwent non-ECG gated CT pulmonary Angiography in Toshiba 64 slice CT scanner.  CT data was analyzed retrospectively to look for cardiac and pulmonary artery anatomy by one independent observer having experience in cardiac radiology. Results 92 patient had infundibular and valvular stenosis and 8 patients has atretic pulmonary valve with additional supravalvar pulmonary stenosis in 17% patient. Out of total 100 patients, 35% had pulmonary artery abnormalities. This included 19 patient (19%) with isolated main pulmonary artery (MPA) abnormality, 6 % had isolated left pulmonary artery (LPA) abnormality, 2 had isolated right pulmonary artery abnormality, 4% had  combined MPA and LPA  abnormality, 2% had combined MPA and RPA abnormalities and 1% patient had all 3 (MPA,LPA,RPA) involvement in form of stenosis or hypoplasia. As TOF is commonly associated with other cardiac structural anomalies, we encountered following associations in our study – aortopulmonary collaterals (37%), Patent ductus arteriosus (29%), right sided aortic arch (19%), bilateral superior vena cava (10%), bovine arch (3%), double aortic arch (2%), d malposition of great vessels (2%), l malposition of great vessels (1%), situs inversus (1%), dextrocardia (1%), annuloaortic ectasia (1%) aortopulmonary window (1%) and cardiac totally anomalous pulmonary venous connection (1%). Conclusions Anomalies of pulmonary arteries are important determinant of surgical outcome of TOF patients. CT angiography is now established modality to delineate pulmonary artery and other anatomical details in TOF patient. Pulmonary artery anomaly can range from isolated branch pulmonary stenosis to diffuse hypoplasia of pulmonary vasculature, based on which management can differ from complete correction to palliative or medical management only. Our study emphasize the importance of pulmonary artery evaluation and its variation among patients of TOF. Abstract P268 Figure.

Modified Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

1996 ◽  
Vol 4 (3) ◽  
pp. 178-180
Author(s):  
Jacques AM van Son ◽  
Volkmar Falk ◽  
Friedrich W Mohr
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Absent Pulmonary Valve ◽  
Modified Technique ◽  
Ventricular Compliance ◽  
Critically Ill Neonates ◽  
Absent Pulmonary Valve Syndrome

A modified technique for the repair of tetralogy of Fallot with absent pulmonary valve syndrome and stenosis at the origin of the lobar or segmental pulmonary artery branches is described. This consists of generous reduction plasty of the redundant anterior and posterior walls of the aneurysmally dilated branches of the pulmonary artery, resection of the main pulmonary artery, pericardial patch augmentation of the stenotic origins of the lobar or segmental pulmonary artery branches, insertion of a valved pulmonary allograft, and closure of the ventricular septal defect. The advantages of this technique, in addition to relief of the left-to-right shunt, consist of complete decompression of the tracheobronchial structures, and restoration of the capacitance of the central pulmonary arteries. This may result in a reduced tendency for postoperative respiratory insufficiency as well as long-term preservation of right ventricular compliance. The technique was successfully performed on 2 critically ill neonates and a 3-month-old infant.

scholarly journals Prenatal diagnosis of crossed pulmonary artery with tetralogy of Fallot and right aortic arch: A rare case report and literature review

2021 ◽  
Author(s):  
Jinfeng Cheng ◽  
Yixiu Zhang ◽  
Hua Meng ◽  
Xining Wu ◽  
Yunshu Ouyang ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Tetralogy Of Fallot ◽  
Chromosomal Abnormalities ◽  
Ductus Arteriosus ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Right Aortic Arch ◽  
Diagnosis And Prognosis ◽  
Rare Case Report

Crossed pulmonary arteries (CPA) is an unusual malformation characterized by abnormal origination of the pulmonary arteries from the main pulmonary artery (MPA), which is usually associated with complex cardiac pathologies and chromosomal abnormalities. We report a case of crossed pulmonary artery (CPA) associated with tetralogy of Fallot (TOF), right aortic arch (RAA), and absence of ductus arteriosus. Sonographic findings, complicated malformations, genetic anomalies, differential diagnosis, and prognosis analysis are discussed. Although the isolated CPA is relatively asymptomatic, when it is accompanied by other cardiac anomalies, the prognosis needs to be reevaluated.

Intermediate Outcomes of Staged Tetralogy of Fallot Repair

2019 ◽  
Vol 10 (6) ◽  
pp. 694-701 ◽  
Author(s):  
Poonam Mahajan ◽  
Eric S. Ebenroth ◽  
Kirsten Borsheim ◽  
Sabena Husain ◽  
Na Bo ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Surgical Intervention ◽  
Main Pulmonary Artery ◽  
Postoperative Arrhythmia ◽  
Young Infants ◽  
Intermediate Outcomes ◽  
Valve Annulus ◽  
Early Surgical Intervention

Background: The optimal surgical strategy for tetralogy of Fallot (TOF) repair in neonates and young infants requiring early surgical intervention remains controversial. Our surgical center follows the uniform strategy of a staged approach with initial systemic-to-pulmonary artery shunt the majority of time when early surgical intervention is required. We characterized a contemporary cohort of patients with TOF with pulmonary stenosis (PS) undergoing staged repair in order to determine the rate of pulmonary valve-sparing repair (PVSR), growth of the pulmonary valve annulus and pulmonary arteries, postoperative morbidity and mortality, and need for reintervention. Methods: We retrospectively studied patients with TOF/PS who underwent staged repair from 2000 to 2017. Surgical details, postoperative course, and reinterventions were noted. Echocardiographic measurements and Z-score values of pulmonary valve annulus, main pulmonary artery (MPA), right pulmonary artery (RPA), and left pulmonary artery (LPA) diameters were evaluated. Results: Of the 59 patients with staged TOF/PS, PVSR was performed in 25 (42%). There was a 5% incidence of postoperative arrhythmia. The Z-scores of MPA, RPA, and LPA were significantly higher before complete repair when compared to before palliative shunt. The 5 and 10-year survival rates were both 93%, and the probability of freedom from reoperation at 5 and 10 years was 87% and 82%, respectively. Conclusions: Staged repair of TOF in young symptomatic infants results in 42% receiving PVSR, pulmonary artery growth, low incidence of postoperative arrhythmia, and relatively low rate of reoperations.

CORRECTED TRANSPOSITION OF THE GREAT VESSELS OF THE HEART

PEDIATRICS ◽  
1957 ◽  
Vol 20 (4) ◽  
pp. 626-646
Author(s):  
Ray C. Anderson ◽  
C. Walton Lillehei ◽  
Richard G. Lester
Keyword(s):  
Ventricular Septal Defect ◽  
Pulmonary Artery ◽  
Septal Defect ◽  
Ductus Arteriosus ◽  
Pulmonary Stenosis ◽  
Main Pulmonary Artery ◽  
Laboratory Findings ◽  
Left Border ◽  
Great Vessels ◽  
Corrected Transposition

Corrected transposition of the great vessels is described, together with the probable embryologic basis for the defect. This defect has assumed great importance because of the necessity of recognizing its presence before surgical exploration for correction of the frequently associated ventricular septal defects and pulmonary stenosis. The presence of corrected transposition interferes with the surgical approach because of the anomalous coronary pattern and the inverted location of the defects. The physical and laboratory findings in 17 patients are tabulated and discussed. These included six with a ventricular septal defect, three with pulmonary stenosis, one with a ventricular and an atrial septal defect, one with a ventricular septal defect and left-sided atrioventricular valve stenosis, one with a ventricular septal defect and pulmonary stenosis, two with a reversing patent ductus arteriosus, and three with a ventricular septal defect and a small leftsided ventricle. The electrocardiogram usually shows A-V block, most often first degree, or A-V dissociation, inverted QRS patterns in the precordial leads (qR in V1 and RS in V6), peaked P waves in lead 2, widened QRS complexes, and upright T waves in the precordial leads, beginning either in RV4 or V1. Roentgenograms may demonstrate an unusual appearance of the upper left border of the heart. The main pulmonary artery may deeply indent the barium-filled esophagus, and the left pulmonary artery may be noted to be medially placed. The diagnosis can be definitely established by angiocardiography in the anteriorposterior view. The main pulmonary artery lies medially, and the aorta arises from the upper left border of the heart. Also diagnostic is the anomalous and difficult course taken by the cardiac catheter in entering the medially-placed pulmonary artery. This defect should be suspected in all patients where the pulmonary artery cannot be entered at cardiac catheterization. If pulmonary stenosis is present, the second sound below the left clavicle will not be as soft as usually noted with this defect. Certain considerations deemed of value to the surgical management of the associated intracardiac defects occurring in this series of patients with corrected transposition are presented.

Abstract 17390: Pre-Operative Right Ventricular Outflow Tract and Pulmonary Artery Geometry Predicts Pulmonary Valve Replacement Outcomes in Patients With Tetralogy of Fallot

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Veronica Toro Arana ◽  
Frandics Chan ◽  
Nicole Shiavone ◽  
Doff McElhinney ◽  
Sushma Reddy ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Valve Replacement ◽  
Prosthetic Valve ◽  
Pulmonary Valve ◽  
Pulmonary Arteries ◽  
Right Ventricular Outflow Tract ◽  
Ventricular Outflow Tract ◽  
Pulmonary Valve Replacement ◽  
Valve Dysfunction

Introduction: Patients with Tetralogy of Fallot who had pulmonary valve replacement (PVR) are at risk for prosthetic valve failure that requires repeated valve replacement. Hypothesis: We hypothesize that the pre-operative geometry of the right ventricular outflow tract (RVOT) and the central pulmonary arteries is a predictor of future prosthetic valve dysfunction. Methods: In a retrospective study, using pre-operative cardiac MRI, we measured morphologic parameters including bifurcation angles, length, major and minor diameters, area, and circumference in various locations along the RVOT, pulmonary trunk (PT) and branch pulmonary arteries (BPAs) in 48 patients with Tetralogy of Fallot before they underwent PVR. Physiologic data was collected from their imaging reports (age, weight, height, body surface area (BSA), ventricular volumes and ejection fractions, valvular regurgitant fractions). All measurements were normalized by the patients’ BSA. Post-operative pulmonary valve function was assessed using Echocardiograms performed at an average of 5.5 years after the surgery. Valve dysfunction was defined as pulmonary regurgitation and/or pulmonary stenosis of at least moderate intensity. All geometric and physiologic parameters were compared between the group of patients who developed pulmonary valve dysfunction and those who did not, using a two-tailed Student t-test. Results: Patients who developed valve dysfunction had (1) greater RVOT circumference (p=0.038), (2) a more acute bifurcation angle between the PT and the Left Pulmonary Artery (p=0.016), and (3) smaller cross-sectional area at the distal BPAs (p=0.031, p=0.026). Conclusions: A dilated RVOT leading to flow vortices may increase the shear stress experienced by the valve, a sharp bifurcation angle disrupts flow patterns, adding dynamic load to the valve, and stenosis in the distal BPAs lead to increased resistance and an increased volume load to the valve - all promoting valve degeneration. Our findings are consistent with physiologic expectations and will be further explored using computational fluid dynamic simulations to elucidate how the parameters identified impact the hemodynamics around the pulmonary valve. A deeper understanding of the hemodynamic implications may ultimately reduce the incidence of valve degeneration by helping surgeons identify patients who are at high risk for valve dysfunction and guiding them to reconstruct the RVOT in specific configurations.

Unexpected Internalization of a Pulmonary Artery Band in a Porcine Model of Tetralogy of Fallot

2016 ◽  
Vol 8 (1) ◽  
pp. 48-54 ◽  
Author(s):  
Zakaria Jalal ◽  
François Roubertie ◽  
Emmanuelle Fournier ◽  
Virginie Dubes ◽  
David Benoist ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Histological Analysis ◽  
Porcine Model ◽  
Pulmonary Stenosis ◽  
Main Pulmonary Artery ◽  
Pulmonary Regurgitation ◽  
Inflammatory Cells ◽  
Pulmonary Insufficiency ◽  
Unusual Complication

Background: We report our experience of an unexpected complication of internalization of a pulmonary artery (PA) band in the vascular lumen, which occurred in a chronic porcine model of repaired tetralogy of Fallot (TOF). Methods: Twelve piglets were divided into 3 groups: (1) TOF model animals (PA band plus pulmonary valvotomy, n = 4), (2) pulmonary insufficiency (PI) animals (pulmonary valvotomy, n = 4), and (3) control animals (n = 4). A nonabsorbable, coated braided polyester tape was used to perform the main pulmonary artery banding. Echocardiography was performed 4 months postoperatively. After each animal was euthanized, PA histological analysis was performed in animals with band internalization. Results: Significant postsurgical pulmonary regurgitation and right ventricular enlargement were present in the TOF and PI, compared with control animals, whereas no significant pulmonary stenosis was observed in TOF animals when compared with PI group. Postmortem examination of all TOF animals revealed the constricting band to be intact but partially internalized into the PA lumen, allowing blood flow around the stenosis. Histological sections of the banded PA in the area of internalization showed a significant disorganization of the medial layer, with significant scarring and fibrotic reaction surrounding the outside of the band and the presence of inflammatory cells suggesting a significant inflammatory response during band internalization. Conclusions: Band internalization may occur after PA banding using a nonabsorbable, coated braided polyester tape in a chronic porcine model of repaired TOF. This unusual complication was likely due to the type of material used for banding.

Primary repair of tetralogy of Fallot in infancy – the effect on growth of the pulmonary arteries and the risk for late reinterventions

2001 ◽  
Vol 11 (4) ◽  
pp. 391-398 ◽  
Author(s):  
Renate Kaulitz ◽  
Christian Jux ◽  
Harald Bertram ◽  
Thomas Paul ◽  
Gerhard Ziemer ◽  
...  
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Pulmonary Valve ◽  
Primary Repair ◽  
Pulmonary Arteries ◽  
Arterial System ◽  
Asymptomatic Patients ◽  
The Right ◽  
Normal Controls

We sought to analyse the long-term follow-up after primary repair of tetralogy of Fallot in infancy in the first year of life, paying particular attention to growth of the pulmonary arteries and the need for reintervention. We performed a combined retro- and prospective echocardiographic study, including measurements of the pulmonary valve and right and left pulmonary arteries, indexed to the square root of body surface area, in 62 patients prior to primary repair, 18 to 24 months after this event, and at the most recent follow-up, with a mean of 80.4 ± 24 months. Of these, 38 patients, with an age at operation of 5.0 ± 3.4 months, had presented preoperatively with hypoxic spells or increasing cyanosis. The remaining 24 patients had been asymptomatic, with adequate flow of blood to the lungs. Their age at elective operation was 7.4 ± 3.0 months. A transannular patch was needed in 37 patients (63%). There were 3 early postoperative deaths (4.8%).Cross-sectional echocardiography revealed a significantly smaller diameter for the pulmonary valve in patients who had been symptomatic preoperatively compared to the asymptomatic patients (1.09 versus 1.3 cm/BSA0.5; p = 0.019). The diameters of the right and left pulmonary arteries did not differ significantly between the groups. Examination of echocardiographic data obtained 18 to 24 months postoperatively in 43 patients revealed a significant increase in the diameter of the pulmonary arteries; 0.83 ± 0.17 cm/BSA0.5 versus 1.1 ± 0.26 cm/BSA0.5 for the diameter of the right pulmonary artery, 0.85 ± 0.2 cm/BSA0.5 versus 1.0 ± 0.25 cm/BSA0.5 for the left pulmonary artery. On comparison between individuals, 18.6% and 25.6% of the patients, respectively, did not show any change in the diameters of their right and left pulmonary arteries, whereas the increase in diameter reached or exceeded the measurements in normal controls in 55.8% and 46.5% of the patients, respectively. On recent follow-up, with a range from 56 to 147 months, no further increase in the indexed diameters of the pulmonaries could be documented in 21 of 33 patients. Of these, 9.1% and 18.2%, respectively, presented with small right and left pulmonary arteries compared with measurements obtained in normal controls. Moderate pulmonary incompetence was found on colour flow mapping in one-third. Of 56 longterm survivors, 8 (14%) had required reinterventions, which were surgical in 6 and achieved by transcatheter techniques in the other 2 patients. Thus, primary correction of tetralogy of Fallot in infancy, with restoration of normal pressures and flows, resulted in sustained increase in the diameters of the right and left pulmonary arteries. It allowed for early normal development of the proximal pulmonary arterial system in most patients regardless of their age and symptomatic status at operation. Patients with persistent subnormal diameter of the pulmonary arteries did not present with significantly elevated right ventricular pressure. Early one-stage repair of tetralogy of Fallot in infancy was associated with a low rate of reinterventions.

Main pulmonary artery cross-section ratio is low in fetuses with tetralogy of Fallot and ductus arteriosus-dependent pulmonary circulation

2017 ◽  
Vol 27 (6) ◽  
pp. 1162-1166 ◽  
Author(s):  
Hironori Ebishima ◽  
Kenichi Kurosaki ◽  
Jun Yoshimatsu ◽  
Isao Shiraishi
Keyword(s):  
Pulmonary Artery ◽  
Tetralogy Of Fallot ◽  
Cross Section ◽  
Pulmonary Circulation ◽  
Ductus Arteriosus ◽  
Main Pulmonary Artery ◽  
Pulmonary Arteries ◽  
Cross Section Ratio ◽  
Section Ratio ◽  
Z Scores

AbstractObjectivesThis study aimed to determine fetal echocardiographic features of tetralogy of Fallot in association with postnatal outcomes.MethodsThe Z-scores of the main and bilateral pulmonary arteries and the aorta were measured, and the following variables were calculated in 13 fetuses with tetralogy of Fallot: pulmonary artery-to-aorta ratio and main pulmonary artery cross-section ratio – the main pulmonary artery diameter squared divided by the sum of the diameter squared of the left and right pulmonary arteries. Fetuses were classified as having ductus arteriosus-dependent or ductus arteriosus-independent pulmonary circulation.ResultsWe included two infants with pulmonary atresia and six infants with ductus-dependent pulmonary circulation, who underwent systemic-to-pulmonary shunt surgeries at ⩽1 month of age. The Z-scores of the main pulmonary artery and the pulmonary artery-to-aorta ratio in fetuses with ductus-dependent pulmonary circulation were lesser than those in fetuses with ductus independence, but not significantly. The main pulmonary artery cross-section ratio in fetuses with ductus dependence was significantly lesser (0.65±0.44 versus 1.56±0.48, p<0.005). Besides, the flow of the ductus arteriosus was directed from the aorta to the pulmonary artery in the ductus arteriosus-dependent group during the fetal period.ConclusionsThe main pulmonary artery cross-section ratio was the most significant variable for predicting postnatal outcomes in fetuses with tetralogy of Fallot.

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