Mixed total anomalous pulmonary venous connection: Anatomic variations, surgical approach, techniques, and results

• Published in: The Journal of thoracic and cardiovascular surgery Vol. 135; no. 1; pp. 106 - 116.e5
• Main Authors: Chowdhury, Ujjwal K., MCh, Diplomate NB, Airan, Balram, MCh, Malhotra, Amber, MS, Bisoi, Akshay K., MCh, Saxena, Anita, DM, Kothari, Shyam S., DM, FACC, Kalaivani, Mani, MSc. (Biostatistics), Venugopal, Panangipalli, MCh
• Format: Journal Article
• Language: English
• Published: Philadelphia, PA Mosby, Inc 2008; AATS/WTSA; Elsevier
• Subjects: Adolescent; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Biological and medical sciences; Cardiology. Vascular system; Cardiothoracic Surgery; Cardiovascular Surgical Procedures - methods; Cardiovascular Surgical Procedures - mortality; Child; Child, Preschool; Congenital heart diseases. Malformations of the aorta, pulmonary vessels and vena cava; Female; Heart; Heart Defects, Congenital - mortality; Heart Defects, Congenital - surgery; Humans; Infant; Male; Medical sciences; Pneumology; Pulmonary Veins - abnormalities; Pulmonary Veins - anatomy & histology; Pulmonary Veins - surgery; Risk Factors; 22; computed tomography; SVC; 16; CI; TAPVC; RA; CT; SD; ASD; CPB; right atrium; cardiopulmonary bypass; total anomalous pulmonary venous connection; superior vena cava; confidence interval; pulmonary artery pressure; atrial septal defect; PAP; 21; standard deviation; Anomalous pulmonary venous drainage; Variations; Cardiovascular disease; Anatomy; Congenital disease; Venous disease; Result; Vascular disease; Treatment; Mixed; Heart disease; Surgery; Total; Anesthesia; Surgical approach; Circulatory system; Technique; Cardiology
• ISSN: 0022-5223; 1097-685X
• DOI: 10.1016/j.jtcvs.2007.08.028

Objective The purpose of this study was to identify the morphologic characteristics and other risk factors that may predispose patients with mixed totally anomalous pulmonary venous connection to continuing high mortality after surgery. Methods Fifty-seven consecutive patients aged 15 days to 18 years (median, 6 months) underwent rechanneling of mixed totally anomalous pulmonary venous connection. Twenty-three patients had “2+2” pattern (I category), 29 had “3+1” pattern (II category), and 5 patients had pulmonary venous connections of different combinations (III category). Obstructive patterns involving one or more pulmonary veins were present in 19 (33.3%) patients. Results Operative and late mortality rates were 19.3% and 4.3%, respectively. At a mean follow-up of 63.26 ± 58.47 months, actuarial survival was 86.9% ± 0.07% in category I, 86.2% ± 0.06% in category II, and 20.0% ± 0.18% in category III (log–rank, P = .001), respectively. At their last follow-up, all survivors (n = 43) had a Ross clinical heart failure score of 0 to 2. Conclusions Patients with a “2+2” pattern of mixed totally anomalous pulmonary venous connection constitute the safe anatomic category for rechanneling, followed by the “3+1” variety. Cross-sectional echocardiography and/or computed tomographic angiography are mandatory to provide necessary diagnostic information and define the anatomy. Patients aged 2 months or younger, obstructive totally anomalous pulmonary venous connection, and perioperative pulmonary hypertensive crises were significant risk factors for death by logistic regression analysis. The risk of death was 5.85 times higher (95% confidence interval: 1.46-35.68; P = .02) in patients with category III of mixed TAPVC. The precise technique adopted in an individual patient depends on the pattern of anatomic drainage, and an individualized surgical approach is recommended.