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.

Mycotic pseudoaneurysm of the aorta is a rare and lethal complication of pediatric congenital heart surgery. We report the lethal consequences of recurrent mycotic pseudoaneurysm in an 18-month-old baby, early after subaortic membrane resection. We managed to repair the pseudoaneurysm successfully by replacing the infected ascending aorta using bovine jugular vein graft, but unfortunately, the patient developed new pseudoaneurysm at the site of anastomosis which led to his death. Although prompt diagnosis and surgical management can save the patient life, uncontrolled infection can lead to the recurrence of the problem and lethal results.

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