Evaluation of cardiovascular morphology and airway-related abnormalities in tetralogy of fallot with absent pulmonary valve syndrome on multidetector computed tomography angiography.

AIM: To evaluate the cardiovascular, tracheobronchial and pulmonary abnormalities associated with tetralogy of Fallot with absent pulmonary valve syndrome (TOF-APVS) on multidetector computed tomograpgy (MDCT) angiography.

MATERIALS AND METHODS: We conducted a retrospective review of our electronic database from January, 2014 to December, 2020 to identify pediatric patients diagnosed with TOF-APVS on MDCT angiography. Pulmonary anatomy, associated tracheobronchial and coronary compression, compression of intrapulmonary bronchi, lung parenchymal changes and other associated cardiovascular abnormalities were evaluated.

RESULTS: Forty-seven patients with TOF-APVS were included in the study. Compression of the airways was observed at multiple levels; 4 (8.5%) patients had carinal compression while right main bronchus and left main bronchus compression was seen in 12 (25.5%) and 16 (34.0%) patients, respectively. Forty-three (91.5%) patients showed abnormal pulmonary arterial branching at the segmental level and compression at the level of intrapulmonary bronchi was seen in 24 (51.1%) patients. Some degree of air trapping was seen in 35 (74.5%) patients whereas atelectasis was observed in 15 (31.9%) patients. Mild coronary arterial compression was seen in 3 (6.4%) patients.

CONCLUSION: TOF-APVS is characterized by aneurysmal dilatation of pulmonary arteries leading to a varying degree of airway compression. However, even in the absence of compression of the trachea and main bronchi, there can be distal bronchial compression secondary to abnormal pulmonary arterial branching resulting in lung abnormalities in the majority of these patients. This study highlights the importance of dedicated airway assessment in addition to cardiovascular morphological assessment by MDCT angiography, before planning surgery.