Author + information
- Received June 26, 2019
- Revision received August 5, 2019
- Accepted August 10, 2019
- Published online December 18, 2019.
- Ahmed Elmaghraby, MSa,
- Abdel Naser Ghareep, MDb,c,
- Maryam Alkuwari, MDb,
- Ziyad M. Hijazi, MD, MPHd and
- Alessandro Salustri, MD, PhDa,∗ ()
- aDepartment of Noninvasive Cardiology, Heart Hospital, Doha, Qatar
- bDepartment of Radiology, Heart Hospital, Doha, Qatar
- cAl Azhar Faculty of Medicine, Cairo, Egypt
- dDepartment of Pediatrics, Sidra Medicine, Doha, Qatar
- ↵∗Address for correspondence:
Dr. Alessandro Salustri, Department of Noninvasive Cardiology, Heart Hospital, Hamad Medical Corporation, PO Box 3050 Doha, Qatar.
A 3-dimensional printed model derived from a computed tomography dataset was obtained in a patient with tetralogy of Fallot and major aortopulmonary collaterals. The virtual and solid 3-dimensional heart models provided intuitive representation of such complex and highly individual pathologies and allowed comprehensive spatial conceptualization of the cardiac structures. (Level of Difficulty: Advanced.)
A 36-year-old male patient came to our noninvasive laboratory for a pre-employment visit. He had a history of congenital heart disease with a surgical procedure in early infancy in his home country; however, no details were available. At physical examination, he was cyanotic with clubbing; a medial sternotomy scar was present, and the auscultation revealed a harsh 3/6 pansystolic murmur at the left sternal edge. His oxygen saturation in room air was 83%. An electrocardiogram showed sinus rhythm and marked right ventricular hypertrophy. A chest x-ray revealed cardiomegaly with bilateral prominent vascular markings. His hemoglobin was 16.8 g/dl, and his hematocrit was 48.2%.
A transthoracic echocardiogram showed a right-sided aortic arch and a large ventricular septal defect (VSD) with an over-riding aorta. The main pulmonary artery was hypoplastic, and there was right ventricular hypertrophy. Contrast cardiac computed tomography was performed (Video 1, Supplemental Figure 1), which provided data for subsequent 3-dimensional (3D) printing in end diastole (Materialise Inc., Leuven, Belgium) (Video 2). The virtual model and 3D solid printouts confirmed and visualized the over-riding aorta and the hypoplastic right ventricular outflow tract (Figures 1A and 1B). The large VSD was seen in real anatomy, and its size and relationships with the adjacent structures were easily appreciated (Figures 1C and 1D). A central shunt was identified connecting the ascending aorta to the hypoplastic main pulmonary artery, and the course of the left and right pulmonary arteries was clearly appreciated using interactive views (Figures 1E and 1F). Major aortopulmonary collateral arteries (MAPCAs) were visible, and 3D reconstruction was able to identify the origin from the descending aorta and to render their spatial relationship with the pulmonary arteries (Figures 1G and 1H). The patient refused other investigations, including angiographic study, and eventually returned to his home country.
The cardinal features of tetralogy of Fallot (pulmonary infundibular stenosis, an interventricular communication, biventricular connection of the aorta, and right ventricular hypertrophy) can be associated with a wide spectrum of associated anomalies, including pulmonary atresia, atrioventricular septal defect, abnormal branching of the coronary arteries, a right aortic arch, and persistence of left superior vena cava (1). Pulmonary atresia, VSD, and MAPCAs are rare variants of tetralogy of Fallot in which surgical management is tailored to the anatomy of each individual patient and depends on the presence and caliber of true pulmonary arteries and the anatomy of the MAPCAs. Therefore, pre-operative imaging should be focused on variants that have implications for operative repair.
Three-dimensional printing has the potential to convey the anatomic arrangement and to provide a comprehensive spatial conceptualization of the cardiac structures of such complex and highly individual pathologies (2). In the present case, solid printouts of the personalized 3D heart model were able to provide accurate anatomic representation of the pathologic structures, including pulmonary atresia, MAPCAs, and the right aortic arch. Although randomized data and recommendations on the use of 3D printing in congenital heart disease are still lacking, significant advantages in pre-operative and pre-procedural planning may be anticipated (3).
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Informed consent was obtained for this case.
- Abbreviations and Acronyms
- major aortopulmonary collateral artery
- ventricular septal defect
- Received June 26, 2019.
- Revision received August 5, 2019.
- Accepted August 10, 2019.
- 2019 The Authors
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