ABSTRACT
BACKGROUND: The association of total anomalous pulmonary venous connection (TAPVC) with major congenital cardiac anomalies is rare. This coexistence has an impact on the diagnosis and management strategies. The objective of this single centre study was to analyse the outcomes of such association in bi-ventricular hearts. METHODS: This is a single-centre retrospective review of all patients who underwent repair for TAPVC associated with major congenital cardiac anomalies in bi-ventricular hearts from February 2015 to March 2020. Data were obtained from the hospital database. The primary aim was to assess the surgical outcomes. In addition, we described the incidence, pathophysiology, morphology and surgical strategies in this cohort of patients. RESULTS: Out of a total of 274 patients who underwent repair for TAPVC during the study period, 16 (5.8%) patients had one or more major associated cardiac defect anomalies. The major associated lesions were ventricular septal defects (9), tetralogy of Fallot (2), transposition of the great arteries (1), aorto-pulmonary window (1), partial atrioventricular canal defect (1), pulmonary stenosis (1) and severe tricuspid valve regurgitation due to flail chord (1). The mean follow-up period is 32 ± 13 months. There were two deaths in the post-operative period and no deaths in the follow-up period. CONCLUSION: The incidence of major associated cardiovascular defects with TAPVC in bi-ventricular morphology is 5.8%. The presence of a major associated lesion with TAPVC significantly alters the pathophysiology and clinical presentation with an impact on diagnosis and treatment.
Subject(s)
Heart Defects, Congenital , Heart Septal Defects, Ventricular , Heart Septal Defects , Pulmonary Veins , Scimitar Syndrome , Transposition of Great Vessels , Humans , Infant , Transposition of Great Vessels/surgery , Heart Defects, Congenital/diagnostic imaging , Heart Defects, Congenital/epidemiology , Heart Defects, Congenital/surgery , Heart Septal Defects, Ventricular/surgery , Scimitar Syndrome/diagnostic imaging , Scimitar Syndrome/epidemiology , Scimitar Syndrome/surgery , Retrospective Studies , Pulmonary Veins/diagnostic imaging , Pulmonary Veins/surgery , Pulmonary Veins/abnormalitiesSubject(s)
Pulmonary Veins/abnormalities , Vascular Malformations , Computed Tomography Angiography , Humans , Infant , Male , Multidetector Computed Tomography , Phlebography/methods , Pulmonary Veins/diagnostic imaging , Vascular Malformations/complications , Vascular Malformations/diagnostic imagingSubject(s)
Heart Neoplasms/complications , Lipoma/complications , Ventricular Outflow Obstruction/etiology , Cardiac Surgical Procedures , Child , Echocardiography, Doppler, Color , Echocardiography, Transesophageal , Female , Heart Neoplasms/diagnostic imaging , Heart Neoplasms/pathology , Heart Neoplasms/surgery , Humans , Lipoma/diagnostic imaging , Lipoma/pathology , Lipoma/surgery , Treatment Outcome , Ventricular Outflow Obstruction/diagnostic imagingABSTRACT
Double-chambered right ventricle (DCRV) is a developmental cardiac anomaly in which anomalous muscle bundles divide the right ventricular (RV) cavity into two chambers. It is usually associated with other congenital cardiac defects, of which ventricular septal defect is the most common association. Isolated DCRV with an intact interventricular septum is very rarely reported. It manifests itself usually in adolescence and adults as the obstruction progresses gradually. It is important to recognize this anomaly as it can progress to severe RV failure if unaddressed. We report a 13-year-old male who presented to us with an isolated DCRV. He successfully underwent a resection of the RV cavity anomalous muscle bundles. We also stress on the utility of transesophageal echocardiography intraoperatively which accurately delineates the location of the muscle bundles and helps in evaluating the adequacy of resection postsurgery.
