Tetralogy of fallot with diminutive pulmonary arteries: Preoperative pulmonary valve dilation and transcatheter rehabilitation of pulmonary arteries

AbstractObjectivesTetralogy of Fallot with absent pulmonary valve syndrome is a rare form of tetralogy of Fallot with dilatation of large pulmonary arteries. Prognosis is related to the severity of the cardiac malformation and to bronchial tree compression by dilated pulmonary arteries. This study analyses the prenatal echographic lung appearance in fetuses with tetralogy of Fallot with absent pulmonary valve and discusses its significance.MethodsWe carried out a retrospective review of fetal and postnatal files of nine fetuses diagnosed with tetralogy of Fallot with absent pulmonary valve syndrome in our institution. Correlations of prenatal ultrasound and cardiac imaging findings were obtained with outcome.ResultsAbnormal heterogeneous fetal lung echogenicity was detected in eight cases out of nine, always associated with significant lobar arterial dilatation. This aspect was well correlated with postnatal imaging and outcome in the four neonatal cases. The only fetus with normal lung echogenicity also had lower degree of pulmonary artery dilatation in the series.ConclusionsThis study demonstrates that a heterogeneous ultrasound appearance of the fetal lungs can be detected in utero in the most severe cases. This aspect suggests an already significant compression of the fetal bronchial tree by the dilated arteries that may have prognostic implications.

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

Circulation ◽

10.1161/circ.138.suppl_1.17390 ◽

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.

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

Cardiology in the Young ◽

10.1017/s1047951101000506 ◽

2001 ◽

Vol 11 (4) ◽

pp. 391-398 ◽

Cited By ~ 10

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.

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Modified Repair of Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Asian Cardiovascular and Thoracic Annals ◽

10.1177/021849239600400314 ◽

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.

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Echocardiographic findings in children with Tetralogy of Fallot

Nepalese Heart Journal ◽

10.3126/njh.v12i2.13383 ◽

2015 ◽

Vol 12 (2) ◽

pp. 65-68

Author(s):

Urmila Shakya ◽

Poonam Sharma ◽

Manish Shrestha

Keyword(s):

Pulmonary Artery ◽

Tetralogy Of Fallot ◽

Pulmonary Valve ◽

Ductus Arteriosus ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Anatomical Variations ◽

Left Pulmonary Artery ◽

Double Outlet Right Ventricle ◽

Echocardiographic Findings

Background and Aims: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease in children. The aim of this study was to identify various cardiac defects associated with TOF by transthoracic echocardiography.Methods: All children presenting to Shahid Gangalal National Heart Centre, with the diagnosis of TOF from 2014 to 2015 were included in the study. Echocardiograpic reports of all eighty eight children were reviewed.Results: The mean age of presentation was 3.5 } 3.9 years. Fifty six (64%) children were male and thirty two (36%) were female. Pulmonary artery anomaly was found in nine (10.2%) children which included hypoplastic pulmonary arteries, absent left pulmonary artery, post stenotic and aneurysmal dilatation of pulmonary artery. Pulmonary valve anomalies were seen in 13(14.8%) children which include pulmonary atresia, pin-hole opening of valve and bicuspid and absent pulmonary valve. Double outlet right ventricle, patent ductus arteriosus/major aortico-pulmonary collaterals, atrial septal defect and persistent left superior venacava were present in 12 (13.6%), 21 (23.9%), 22 (22.7%) and nine (10.2%) respectively. Right sided aortic arch was seen in 20 (22.7%).Conclusion: TOF is associated with various anatomical variations which need to be identified to avoid surgical complications. These can be diagnosed by echocardiography which has greatly reduced the need for invasive cardiac catheterization for these patients.Nepalese Heart Journal 2015;12(2):65-68

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One-stage repair in early infancy of tetralogy of Fallot with ‘absent pulmonary valve’ syndrome

Cardiology in the Young ◽

10.1017/s1047951100002018 ◽

1994 ◽

Vol 4 (2) ◽

pp. 103-109 ◽

Cited By ~ 2

Author(s):

Toshikatsu Yagihara ◽

Hidefumi Kishimoto ◽

Fumio Yamamoto ◽

Kyoichi Nishigaki ◽

Osamu Matsuki ◽

...

Keyword(s):

Respiratory Failure ◽

Tetralogy Of Fallot ◽

Pulmonary Valve ◽

Pulmonary Arteries ◽

Right Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Early Infancy ◽

One Stage ◽

Age At Surgery ◽

The Right

AbstractFive infants under three months of age with tetralogy of Fallot and absence of the leaflets of the pulmonary valve were successfully corrected using a one-stage repair. All patients suffered from respiratory failure preoperatively and surgery was performed somewhat urgently. The age at surgery ranged from 26 to 72 days, with an average of 47±17 days. The surgical procedures involved four steps. First, we plicated the aneurysmally dilated central pulmonary arteries. Second, we closed the ventricular septal defect. We then inserted a handmade heterograft pericardial valve at the site of the pulmonary valve, and completed the reconstruction of the right ventricular outflow tract with a patch. All patients survived. Two of them were successfully weaned from ventilation at early (two and 11 days, respectively) periods after surgery, but the remaining three patients required artificial ventilation for one to two months. We conclude that one-stage repair is possible for this anomaly, but that plication of the pulmonary trunk is an essential technique todeliver patients in early infancy from respiratory failure.

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

European Heart Journal ◽

10.1093/ehjci/ehz872.091 ◽

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.

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The essential echocardiographic features of tetralogy of Fallot

Cardiology in the Young ◽

10.1017/s1047951113001704 ◽

2013 ◽

Vol 23 (6) ◽

pp. 871-882 ◽

Cited By ~ 2

Author(s):

Norman H. Silverman

Keyword(s):

Tetralogy Of Fallot ◽

Ventricular Hypertrophy ◽

Septal Defect ◽

Pulmonary Valve ◽

Pulmonary Arteries ◽

First Trimester ◽

Major Aortopulmonary Collateral Arteries ◽

Collateral Arteries ◽

Aortopulmonary Collateral ◽

Subpulmonary Stenosis

AbstractThis presentation will demonstrate the essential features of tetralogy of Fallot in the infant and child before surgery, as well as some noteworthy features in the foetus. The four features, namely, subpulmonary stenosis, ventricular septal defect, aortic override, and right ventricular hypertrophy, can all be easily demonstrated by echocardiography. In addition, morphology of the pulmonary valve and the main and branch pulmonary arteries can be seen. The position of the coronary arteries and the major variants of proximal coronary anatomy can be defined. The arch anatomy and the presence of associated major aortopulmonary collateral arteries can be defined. All these features can be demonstrated in the foetus as well, after the first trimester, and the presence of major aortopulmonary collateral arteries can be seen more clearly because the lungs, being fluid filled, aid in ultrasound and do not provide the barrier that the air-filled lung presents after birth.

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Changes in 99m Technegas ventilation lung scan in a newborn with absent pulmonary valve syndrome

Cardiology in the Young ◽

10.1017/s1047951101001093 ◽

2001 ◽

Vol 11 (6) ◽

pp. 673-675

Author(s):

Kazuhiro Takahashi ◽

Takashi Kuwahara ◽

Masayoshi Nagatsu

Keyword(s):

Surgical Treatment ◽

Tetralogy Of Fallot ◽

Respiratory Symptoms ◽

Pulmonary Valve ◽

Pulmonary Arteries ◽

Absent Pulmonary Valve ◽

Lung Scan ◽

Two Stages ◽

Lung Scanning ◽

Absent Pulmonary Valve Syndrome

A newborn infant with tetralogy of Fallot and absent pulmonary valve was successfully corrected in two stages. Absent pulmonary valve syndrome presenting in early infancy manifests severe respiratory symptoms that still make challenging both management and surgical treatment. This is ascribed to tracheobronchial compression by the extremely dilated pulmonary arteries, and to the resultant pulmonary obstructive lesions. We report herein the first findings of 99m Technegas ventilation lung scanning in an infant with the syndrome to assess the pulmonary obstructive lesions.

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