Structural Abnormalities of the Pulmonary Trunk in Tetralogy of Fallot and Potential Clinical Implications

AbstractVentricular contouring of cardiac magnetic resonance imaging is the gold standard for volumetric analysis for repaired tetralogy of Fallot (rTOF), but can be time-consuming and subject to variability. A convolutional neural network (CNN) ventricular contouring algorithm was developed to generate contours for mostly structural normal hearts. We aimed to improve this algorithm for use in rTOF and propose a more comprehensive method of evaluating algorithm performance. We evaluated the performance of a ventricular contouring CNN, that was trained on mostly structurally normal hearts, on rTOF patients. We then created an updated CNN by adding rTOF training cases and evaluated the new algorithm’s performance generating contours for both the left and right ventricles (LV and RV) on new testing data. Algorithm performance was evaluated with spatial metrics (Dice Similarity Coefficient (DSC), Hausdorff distance, and average Hausdorff distance) and volumetric comparisons (e.g., differences in RV volumes). The original Mostly Structurally Normal (MSN) algorithm was better at contouring the LV than the RV in patients with rTOF. After retraining the algorithm, the new MSN + rTOF algorithm showed improvements for LV epicardial and RV endocardial contours on testing data to which it was naïve (N = 30; e.g., DSC 0.883 vs. 0.905 for LV epicardium at end diastole, p < 0.0001) and improvements in RV end-diastolic volumetrics (median %error 8.1 vs 11.4, p = 0.0022). Even with a small number of cases, CNN-based contouring for rTOF can be improved. This work should be extended to other forms of congenital heart disease with more extreme structural abnormalities. Aspects of this work have already been implemented in clinical practice, representing rapid clinical translation. The combined use of both spatial and volumetric comparisons yielded insights into algorithm errors.

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Aberrant right coronary artery occlusion during the percutaneous pulmonary trunk stenting in a patient with tetralogy of Fallot

Heart and Vessels ◽

10.1007/s00380-007-1023-8 ◽

2008 ◽

Vol 23 (2) ◽

pp. 140-143 ◽

Cited By ~ 11

Author(s):

Xavier Perret ◽

Robert F. Bonvini ◽

Yacine Aggoun ◽

Vitali Verin

Keyword(s):

Coronary Artery ◽

Tetralogy Of Fallot ◽

Right Coronary Artery ◽

Coronary Artery Occlusion ◽

Artery Occlusion ◽

Pulmonary Trunk

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An echocardiographic study of tetralogy of Fallot in the fetus and infant

Cardiology in the Young ◽

10.1017/s1047951103000477 ◽

2003 ◽

Vol 13 (3) ◽

pp. 240-247 ◽

Cited By ~ 35

Author(s):

Litha P. Pepas ◽

Alexandra Savis ◽

Annette Jones ◽

Gurleen K. Sharland ◽

Robert M.R. Tulloh ◽

...

Keyword(s):

Right Ventricular ◽

Tetralogy Of Fallot ◽

Right Ventricular Outflow Tract ◽

Ventricular Outflow Tract ◽

Outflow Tract ◽

Pulmonary Trunk ◽

Doppler Velocity ◽

Fetal Life ◽

Definitive Repair ◽

The Right

Objective: To document the echocardiographic features of tetralogy of Fallot during fetal and postnatal life. Correlation of echocardiographic findings with the requirement for early intervention prior to definitive repair. Design: Retrospective observational study. Setting: A tertiary fetal cardiology unit. Patients: Fetuses with a diagnosis of tetralogy of Fallot identified from a prospective database between 1 January 1999 and 31 October 2002. Main measures of outcome: Growth of aorta and pulmonary trunk during fetal and postnatal life. Doppler assessment of the great arteries both prenatally and postnatally. Clinical outcome to definitive repair. Results: We identified 25 fetuses with tetralogy of Fallot, 23 having a pulmonary valvar diameter below the normal range at some point during gestation. The ratio of the diameter of the aortic to the pulmonary valve was abnormal in all cases. The pulmonary arterial Doppler velocity was within the normal range in six fetuses at presentation, and elevated in the remainder. In two fetuses, the right ventricular outflow tract was patent during fetal life, but had become atretic at birth. Both of these fetuses had reversal of flow in the arterial duct at presentation during fetal life. In 2 fetuses in whom we showed poor growth of the pulmonary trunk in late gestation, it was necessary to intervene early. The Doppler velocity across the pulmonary valve during fetal life did not differentiate between babies who required early intervention and those who were repaired electively. There was a marked increase in pulmonary arterial Doppler velocity following birth, which became more elevated with age. Of 18 liveborn infants, 17 have survived, with 2 having balloon dilation of the right ventricular outflow tract, and 3 insertion of a Blalock-Taussig shunt prior to definitive repair. Conclusions: In tetralogy of Fallot, features of pulmonary valvar hypoplasia and obstruction are evident during fetal life. Progression of obstruction in the right ventricular outflow tract was observed during fetal life as well as postnatally. Reversal of flow in the arterial duct, and failure of growth of the pulmonary trunk, predicted the need for early surgery to maintain pulmonary blood flow. Parents should be counselled about the possibility of emergency intervention being required after birth. Affected fetuses should be delivered at units with experience of managing the cyanosed newborn.

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Hypercyanotic spells caused by antegrade catheterization of the pulmonary arteries in tetralogy of Fallot: fact or fiction?

Cardiology in the Young ◽

10.1017/s1047951100000251 ◽

1991 ◽

Vol 1 (2) ◽

pp. 136-140 ◽

Cited By ~ 2

Author(s):

Ian A. Murdoch ◽

Shakeel A. Qureshi ◽

Rue Dos Anjos ◽

Jonathan M. Parsons ◽

Edward J. Baker ◽

...

Keyword(s):

General Anesthesia ◽

Tetralogy Of Fallot ◽

Cardiac Catheterization ◽

Balloon Dilatation ◽

Pulmonary Arteries ◽

Pulmonary Trunk ◽

Diagnostic Cardiac Catheterization ◽

Pulmonary Arterial ◽

The Mean ◽

Antegrade Catheterization

SummaryBetween January 1985 and March 1990, 66 children with the tetralogy of Fallot underwent 85 cardiac catheterization procedures. The mean age at first procedure was 2.5 years (range 0.1–;14.4 years) and the mean weight was 10.4 kg ( range 2.4–36.0 kg). Diagnostic cardiac catheterization was performed in 60 procedures and balloon dilatation in 25. Hypercyanotic spells had occurred prior to 24 (28%) of the procedures (all the patients being on propranolol) and a systemic-to-pulmonary arterial shunt had been constructed before 28 (33%) procedures. Of the procedures, 54 (64%) were performed under local and 31(36%) under general anesthesia. The pulmonary trunk was entered antegradely in 52 procedures, retrogradely through a shunt in 6 and not entered in 27. Balloon dilatation was performed under general anesthesia on 25 occasions. No procedure was abandoned because of a cyanotic spell. Nine (11%) spells occurred during 86 procedures, one of the procedures being postponed because ofa spell occurring after premedication, the procedure and not, therefore, continuing to catheterization. Five spells occurred before the catheter was positioned in the heart, 2 spells occurred during catheterization. Of the spells, eight occurred during procedures in children who had not had previous shunts. Antegrade entry into the pulmonary trunk in the group with shunts was associated with no spells compared with 6/38 (18%) in the group not having undergone surgery (p<0.l). In the group not undergoing surgery, when the pulmonary trunk was not entered, 1 (5%) spell occurred during 19 procedures compared with 6/38 (18%) when the pulmonary trunk was entered (p<0.4). The only clinically important factor which significantly influenced the incidence of spells was the use of general anesthesia, which was associated with 6/31 (19%) spells compared with local anesthesia which was associated with 2/54 (4%) spells (p<0.026).

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Technique for constructing the pulmonary trunk for tetralogy of fallot with pulmonary atresia

The Annals of Thoracic Surgery ◽

10.1016/0003-4975(95)00110-7 ◽

1995 ◽

Vol 59 (5) ◽

pp. 1245-1248 ◽

Cited By ~ 10

Author(s):

Tetsuya Kitagawa ◽

Itsuo Katoh ◽

Fumio Chikugo ◽

Takaki Hori ◽

Yoshiaki Fukumura ◽

...

Keyword(s):

Tetralogy Of Fallot ◽

Pulmonary Atresia ◽

Pulmonary Trunk

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Anomalous Origin of the Coronary Arteries from the Pulmonary Trunk in Two Separate Patients with a Review of the Clinical Implications and Current Treatment Recommendations

The American Journal of the Medical Sciences ◽

10.1097/00000441-200408000-00007 ◽

2004 ◽

Vol 328 (2) ◽

pp. 112-115 ◽

Cited By ~ 6

Author(s):

William J. Nicholson ◽

Brian Schuler ◽

Stamatios Lerakis ◽

Tarek Helmy

Keyword(s):

Coronary Arteries ◽

Current Treatment ◽

Treatment Recommendations ◽

Pulmonary Trunk ◽

Anomalous Origin ◽

Clinical Implications

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Power loss and right ventricular efficiency in patients after tetralogy of Fallot repair with pulmonary insufficiency: Clinical implications

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/j.jtcvs.2011.10.066 ◽

2012 ◽

Vol 143 (6) ◽

pp. 1279-1285 ◽

Cited By ~ 19

Author(s):

Mark A. Fogel ◽

Kartik S. Sundareswaran ◽

Diane de Zelicourt ◽

Lakshmi P. Dasi ◽

Tom Pawlowski ◽

...

Keyword(s):

Right Ventricular ◽

Tetralogy Of Fallot ◽

Power Loss ◽

Pulmonary Insufficiency ◽

Clinical Implications ◽

Ventricular Efficiency ◽

Tetralogy Of Fallot Repair

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The aortic arch in tetralogy of Fallot: types of branching and clinical implications

Cardiology in the Young ◽

10.1017/s1047951120001705 ◽

2020 ◽

Vol 30 (8) ◽

pp. 1144-1150 ◽

Cited By ~ 1

Author(s):

Sudesh Prabhu ◽

Srikanth Kasturi ◽

Siddhant Mehra ◽

Rishi Tiwari ◽

Abhijit Joshi ◽

...

Keyword(s):

Aortic Arch ◽

Tetralogy Of Fallot ◽

Anatomical Variations ◽

Interrupted Aortic Arch ◽

Comprehensive Analysis ◽

Right Aortic Arch ◽

Clinical Implications ◽

Inclusion Criteria ◽

Live Births ◽

Morphological Patterns

AbstractIntroduction:Tetralogy of Fallot is the most common form of cyanotic CHD, with an incidence of 421 cases per million live births, constituting around 10% of CHD. Variations in aortic arch anatomy associated with tetralogy of Fallot, other than the incidence of right aortic arch (13–34%), are not frequently reported. A comprehensive analysis of a large number of tetralogy of Fallot cases is required to arrive at a compendious description of aortic arch anatomy.Materials and methods:All patients with a diagnosis of tetralogy of Fallot who had CT or MRI either pre or post procedures between 1 January 2010 and 31 December 2019 at our hospital were included in the study. Using radiology consultants’ reports and analysis of individual images, we identified the various morphological patterns of aortic arches prevalent in these patients.Result:Out of 2684 patients who met the inclusion criteria, 1983 patients had left aortic arch (73.9%), 688 patients had right aortic arch (25.7%), four patients had cervical aortic arch (0.15%), eight patients had double aortic arch (0.3%), one patient had an aorto-pulmonary window (0.04%), and none of the patients had interrupted aortic arch. Sub-classification and clinical implications of the arch variations are described.Conclusion:Up to 10% of tetralogy of Fallot patients may have significant anatomical variations of aortic arch that would necessitate changes or additional steps in their surgical or interventional procedures.

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Solitary arterial trunk

Cardiology in the Young ◽

10.1017/s104795110001091x ◽

1994 ◽

Vol 4 (1) ◽

pp. 71-74

Author(s):

Daniel J. Penny ◽

Rakesh Dua ◽

James L. Wilkinson

Keyword(s):

Ventricular Septal Defect ◽

Tetralogy Of Fallot ◽

Septal Defect ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Pulmonary Trunk ◽

Descending Aorta ◽

Perimembranous Ventricular Septal Defect ◽

Arterial Trunk ◽

Pulmonary Arterial

AbstractA heart is described in which there was a solitary arterial trunk that exited from the heart astride a large perimembranous ventricular septal defect, with the pulmonary arterial supply originating entirely from collaterals from the descending aorta. There was no evidence of either hilar pulmonary arteries or an atretic pulmonary trunk. The morphology of the ventricular outlet component, with absence of the outlet septum supports the description of this heart as solitary arterial trunk, rather than tetralogy of Fallot with pulmonary atresia.

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