Crossed pulmonary artery (CPA) is a rare congenital malformation with only about 100 cases reported worldwide. It is often an incidental finding on cardiac imaging with symptoms rarely attributed to this anomaly. Sometimes, it can result in difficulties during catheterization and surgical procedures. Thus, it is necessary to diagnose CPA preoperatively to reduce misadventures. CPA is primarily diagnosed by computerized tomography angiography (CT angio) and missing it during echocardiography is common, even when an accurate diagnosis is possible by echocardiography alone. Echocardiography can also differentiate it from other causes of nonvisualization of pulmonary artery bifurcation. Recent studies suggest that CPA is not as rare as previously described. With increased utilization of CT angio, more cases of CPA are being picked up, which were not noted earlier on echocardiography. Missing CPA on echocardiography is attributed mainly to the lack of awareness regarding this condition among echocardiographers. Raising awareness can avoid missed diagnoses and aid in planning the interventional and surgical procedures.

SummaryWe present a case of a six–year–old boy with complex partial anomalous pulmonary venous connections with accessory pulmonary veins, where multi–detector computed tomography proved crucial for accurate identification prior to planning for surgical correction.

Background: Critical congenital heart disease (CCHD) encompasses congenital structural heart defects that cause significant morbidity and mortality in the first few weeks of life unless treated and/or require surgery or catheter intervention within the 1st year of life. Since these deteriorate acutely due to their cardiac condition, they may be misdiagnosed as septicemia or perinatal asphyxia, especially in resource-poor settings. The American Academy of Pediatrics recommends universal screening with pulse oximetry after 24 h of life by a simple screening protocol. Although specificity is high, CCHD may be missed. We present a case who screened negative became symptomatic on day 10 of life and was finally diagnosed with a CCHD. Clinical Description: A full-term baby with uneventful postnatal course and negative CCHD screening was discharged on day 2 of life. He returned on day 10 with cardiogenic shock. Echocardiography confirmed interrupted aortic arch with large ventricular septal defect (VSD), moderate-sized atrial septal defect (ASD), and a small, restrictive patent ductus arteriosus (PDA). The initial false-negative result was attributed to the presence of large VSD that leads to equalization of preductal and postductal oxygen saturations. Management: The baby was stabilized with prostaglandin infusion and ventilatory support. He underwent staged repair with end-to-end anastomosis of interrupted segment and PDA ligation in the first sitting. The postoperative course was uneventful, and the patient was discharged home at day 25 of life. He is planned for VSD and ASD repair in follow-up. Conclusion: Complex heart diseases may behave unusually due to complicated inter-related hemodynamics arising from the various lesions. Primary health-care personnel should recognize the limitations of CCHD screening protocol and learn to counsel parents accordingly.

14Systemic to pulmonary shunting provides palliation to neonates and small infants with many complex forms of cyanotic heart disease. It is usually the first step palliation in these neonates who are either unsuitable for total correction or due to the higher pulmonary vascular resistance are unable to undergo bidirectional Glenn anastomosis early in life.The modified Blalock-Taussig Shunt (MBTS) is the most commonly used systemic to pulmonary artery shunt whereby a synthetic conduit of Polytetrafluoroethylene (PTFE) is anastomosed between a subclavian artery and a pulmonary artery branch.Background: Systemic to pulmonary shunting is still the main palliation option in many neonates with cyanotic heart disease. Design: A Retrospective Study. Setting: The Mohammed bin Khalifa bin Salman Al-Khalifa Cardiac Center, Bahrain Defence Force Hospital, Bahrain. Method: All infants from January 1995 to December 2008 who had undergone systemic to pulmonary shunting procedures were included in the study.Result: Ninety-three infants underwent 108 shunt type procedures, 39 females and 54 males. One hundred five Modified Blalock-Taussig Shunts (MBTS) and three classic Blalock-Taussig shunts were performed. Tetralogy of Fallot/Double Outlet Right Ventricle (DORV) with right ventricular outflow tract obstruction was the most common diagnosis, 51 (55%).Early postoperative mortality was 1 (1%). Late postoperative mortality was 4 (4%); sepsis was the major cause. Early (<1 month postoperatively) shunt failure/occlusion occurred in 6 (6%) patients. Under 14 days of age at the time of shunting and shunt size of 5mm (18% versus 4.6%) were risk factors for early shunt occlusion/failure. Eight (8%) patients required late shunt revision at two months to 5 years post initial shunting. Fifty-one patients had undergone final corrective surgery.Conclusion: MBTS shunting provides effective and safe palliation in small infants with complex cyanotic heart disease. Early shunting (<14 days of age), and 5mm shunt size are additional risk factors for early shunt failure.

Introduction: Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital cardiovascular condition in which one or some of the pulmonary veins (PV) but not all drain into systemic circulation rather than into the left atrium (LA). Accurate detection of these anomalies is important because of its association with patient morbidity and mortality. Although the PV anatomy can be evaluated by Echocardiography or angiography, noninvasive modalities like multidetector computed tomography (MDCT), magnetic resonance imaging (MRI) now play crucial role in the characterization of pulmonary veins. Aim: The aim of this article is to review various patterns PAPVC diagnosed, the associated cardiac defects, the role of various imaging modalities and their implications in the management and also to review the embryology of the PV. Materials & Methods: A retrospective study was conducted in 21 consecutive patients with PAPVC. The various patterns of PAPVC and associated cardiac defects was studied using echocardiography, cardiac catheterization and MDCT. Results: There were 13 males and 8 females and their age at diagnosis ranged from day 1 to 50 years. Most common type was right sided PAPVC in 15 patients, Left sided PAPVC in 3 patients. Mixed type where PAPVC of both right and left sided veins occur in 3 patients. Accessory pulmonary veins were identified in 4 patients. Most common associated cardiac defect was secundum ASD (OS ASD). Conclusions: PAPVC cannot be classified into stereotypic types as there is wide spectrum of developmental anomalies exist. A single diagnostic modality may not detect every anomaly. Even though echocardiography is the initial imaging technique of choice it is suboptimal and MDCT now provides very rapid and accurate imaging.