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Background Persistent left superior vena cava is a well-recognized thoracic venous abnormality, most of which do not present with any symptoms. Its opening into the left atrium is uncommonly encountered and, if present, is noted to be associated with various other complex intracardiac malformations, which further complicates its management. Herein, we highlight a case of an adult with persistent left superior vena cava with a spectrum of other cardiac anomalies rarely encountered with it, which was effectively managed by tailored surgical intervention. Case presentation A 26-year-old female on meticulous evaluation was found to have left isomerism with an interrupted inferior vena cava and hemiazygos continuing as a persistent left superior vena cava. It was also associated with common atrium, partial atrioventricular canal defect, and complete heart block. This complex array of anomalies was managed surgically. Common atrium was closed with atrial neo-septation using autologous pericardial patch tailored in a way, such that the persistent left superior vena cava drained into right atrium with left atrial appendage being on right side of neo-septum. Cleft in the left atrioventricular valve was also repaired. Despite preoperative complete heart block, the patient had an uneventful postoperative recovery and was in atrial fibrillation with controlled ventricular rate, thus precluding the need for permanent pacemaker implantation. Conclusions A rare spectrum of adult congenital cyanotic heart disease was successfully managed with thorough preoperative evaluation and judicious intraoperative management. Surgical management should be tailored based on specific anatomy for reduction of morbidity and optimum results.
Background Persistent left superior vena cava is a well-recognized thoracic venous abnormality, most of which do not present with any symptoms. Its opening into the left atrium is uncommonly encountered and, if present, is noted to be associated with various other complex intracardiac malformations, which further complicates its management. Herein, we highlight a case of an adult with persistent left superior vena cava with a spectrum of other cardiac anomalies rarely encountered with it, which was effectively managed by tailored surgical intervention. Case presentation A 26-year-old female on meticulous evaluation was found to have left isomerism with an interrupted inferior vena cava and hemiazygos continuing as a persistent left superior vena cava. It was also associated with common atrium, partial atrioventricular canal defect, and complete heart block. This complex array of anomalies was managed surgically. Common atrium was closed with atrial neo-septation using autologous pericardial patch tailored in a way, such that the persistent left superior vena cava drained into right atrium with left atrial appendage being on right side of neo-septum. Cleft in the left atrioventricular valve was also repaired. Despite preoperative complete heart block, the patient had an uneventful postoperative recovery and was in atrial fibrillation with controlled ventricular rate, thus precluding the need for permanent pacemaker implantation. Conclusions A rare spectrum of adult congenital cyanotic heart disease was successfully managed with thorough preoperative evaluation and judicious intraoperative management. Surgical management should be tailored based on specific anatomy for reduction of morbidity and optimum results.
Introduction: Systemic venous anomalies are quite rare and can be associated with congenital heart disease requiring surgery.Materials and Methods: All consecutive patients (pts) undergoing surgery for congenital heart defects were retrospectively analyzed for presence of systemic venous anomalies: (a) Persistent left superior vena cava (PLSVC)(b) Inferior vena cava (IVC) interruption(c) Retro-aortic innominate veinResults: From 9/2010 to 5/2012 155 pts, median age 7 months, mean age 1.3 years (3 days–50 years), median weight 4 kg, mean weight 7.2 kg (0.6–110 kg) underwent congenital heart surgery. Twenty-nine systemic venous anomalies were identified in 28/155 patients (=18.1%). PLSVC was present in 21 pts (=13.5%), median age 4 months, mean age 2.7 years (3 days–22 years), median weight 6 kg, mean weight 10.1 kg (2.4–43.0 kg). IVC interruption was identified in 5 pts (=3.2%), median age 2 months, mean age 5.4 years (30 days–26 years), median weight 3.7 kg, median weight 17 kg (2.3–68.0 kg). Retro-aortic innominate vein was diagnosed in 3 pts (=1.9%), median age 5 years, mean age 3.7 years (10 months–5 years), median weight 12 kg, mean weight 10.1 kg (4.5–14 kg). Complete pre-operative diagnosis was obtained in 14/28 (=50%) pts with echocardiography and in other 8/28 (=28.6%) only after computed tomography (CT) scan, for a total of 22/28 (=78.6%) correct pre-operative diagnosis. In 6/28 (=21.4%) patients the diagnosis was intra-operative. Total incidence of systemic venous anomalies was 18.1% (vs. 4% in the literature, P = 0.0009), with presence of PLSVC = 13.5% (vs. 0.3–4.0%, respectively P = 0.0004 and P = 0.0012), IVC interruption = 3.2% (vs. 0.1–1.3%, N.S.), and retro-aortic innominate vein = 1.9% (vs. 0.2–1%, N.S.).Conclusions: Our study showed an incidence of systemic venous anomalies in Middle Eastern pts with congenital heart defects higher than previously reported. In 78.6% of pts the diagnosis was correctly made before surgery (echocardiography or CT scan), with 21.4% of complete diagnosis made at surgery. A careful pre-operative screening should be performed in all pts with congenital heart defects from this region to better identify all systemic venous anomalies for a more accurate surgical planning.
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