Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle

Abstract

Background: Total anomalous pulmonary venous connection (TAPVC) is a major risk factor in infants with single ventricle (SV). Exact definition of TAPVC anatomy is crucial for surgical planning.Aim: To evaluate the role of cardiac computed tomography (CT) in this setting.Methods: Retrospective review of 13 infants who underwent TAPVC repair associated with SV from May 2016 to October 2021. Anatomy, incidence, and mechanisms of pulmonary venous obstruction (PVO) were described. Cardiac CT diagnostic yield was compared to echocardiography (echo).Results: Of 13 infants, median age and weight were 24 days (range 2-303 days) and 3.2 (range 2.6-9.1) kg, 8 (62%) were male, 4 (31%) premature, and 11 (85%) had heterotaxy syndrome. All infants had pre-and postoperative echo; 13 had preoperative and 8 (62%) had postoperative cardiac CT. Type of TAPVC: six (46%) supracardiac, two (15%) intracardiac, one (8%) infracardiac, and four (31%) mixed, with pulmonary veins draining in >1 confluence in nine (69%). PVO was present in 6/13 (46%) preoperatively and 5/13 (31%) postoperatively. Mechanisms of PVO: 9/11 (82%) stenosis, 1/9 (9%) membrane formation, and 1/9 (9%) external compression. The sensitivity to diagnose PVO was 45.5% for echo and 100% for cardiac CT, the specificity was 100% for both. No discrepancy was found between cardiac CT and intraoperative findings, but echo had a complete preoperative diagnosis in 1/13 (8%) (p < .00001, Fisher exact test).Conclusions: Cardiac CT is essential to evaluate pre-and postoperative TAPVC in SV for surgical decision making and long term follow up.

The pendulum of Fontan fenestration

“…There has been a steady increase in the use of Fontan fenestration based on much broader indications. Largely related to better post-operative management, the option of Fontan completion has been extended to different patient populations, including patients undergoing completion at younger age, and with complex congenital heart defects, such as hypoplastic left heart syndrome ( 21 ), heterotaxy syndrome ( 22 ), and single ventricle physiology with associated total anomalous pulmonary venous connection ( 23 ). Because of these changes in the risk stratification of the patients accepted for surgery ( 24 ), the cohort of patients presented for Fontan completion are frequently at high risk for a complicated post-operative course.…”

Invited commentary for: Essential role of cardiac computed tomography for surgical decision making in children with total anomalous pulmonary venous connection and single ventricle

“…Surgical planning using AAI strategies and computational modeling approaches takes time (e.g., hours to days) and effort (e.g., research personnel), and thus this should be factored into the overall clinical management strategy and only used when most appropriate for the patient. 2,3 Studies, like Vasquez Choy et al, 1 set the stage for the evaluation of other modes of imaging analysis or AAI with echocardiography.…”

“…While imaging techniques (e.g., 3D/4D magnetic resonance imaging [MRI], computed tomography [CT], single‐photon emission CT [SPECT], and positron emission tomography [PET] scans, echocardiography, ultrasound, angiography) have advanced in transformative ways, we are still deciphering which method to employ for each patient‐specific anatomy or singular diagnosis. This challenge is further complicated by dual‐diagnoses of multiple malformations and congenital cardiac defects, like those discussed in Vasquez Choy et al 1 …”

“…In this study, Vasquez Choy et al 1 performed a retrospective chart review examining the limitations of echocardiography in conjunction with additional CT imaging in patients with a dual‐diagnosis of single ventricle (SV) physiology and total anomalous pulmonary venous connection. They studied patients having this dual‐diagnosis between 2016 until 2021 ( n = 13).…”

“…Studies, like Vasquez Choy et al, 1 set the stage for the evaluation of other modes of imaging analysis or AAI with echocardiography. For instance, challenges of MRI in comparison to diagnostic catheterization for stage II palliation and complementary examinations would be of substantial interest to the scientific and medical community.…”