Case report: the 'vanished' left pulmonary artery.

Background: We report a case of isolated ductal origin of pulmonary artery (DOPA) diagnosed in an asymptomatic newborn. The primary aim of this case is to highlight the need to investigate for DOPA in patients diagnosed with an 'absent branch pulmonary artery'.

Case Summary: Our patient was an asymptomatic newborn infant, with normal intracardiac anatomy.

Endocardial Fibroelastosis as an Independent Predictor of Atrioventricular Valve Rupture in Maternal Autoimmune Antibody Exposed Fetus: A Systematic Review with Clinicopathologic Analysis.

Background: Neonatal lupus (NL) is a clinical syndrome that develops in the fetus as a result of maternal autoimmune antibodies. Congenital complete heart block (CHB) is the most common manifestation, while extranodal cardiac manifestations of NL, such as endocardial fibroelastosis (EFE) and myocarditis, are rare but more serious. Less is known about this atrioventricular valve rupture due to valvulitis as a consequence of maternal autoantibodies.

Saline contrast echocardiography complements cardiac interventions in neonates with transposition of great arteries and abnormal ductus venosus anatomy.

We present a rare case of premature low birthweight neonate with right diaphragmatic hernia and transposition of great vessels requiring balloon atrial septostomy. Congenital diaphragmatic hernia poses a unique challenge to umbilical venous catheterisation. Based on the radiographic position of umbilical vein catheter, umbilical venous cannulation was attempted; however, the catheter could not be navigated to the right atrium.

Visceral heterotaxy in the developing world.

Background: The challenge of visceral heterotaxy (VH) in the developing world has not been analysed in detail.

Method: Retrospective chart review of 69 consecutive patients over ten years assessed the clinical profile and surgical outcome of VH. Median age: 3 years; median weight: 15kg.

Computed tomographic angiography in tetralogy of Fallot.

Echocardiography is often inadequate for imaging tetralogy of Fallot, prompting cineangiography. This study prospectively evaluated multidetector computed tomographic angiography for preoperative evaluation of tetralogy of Fallot in 112 consecutive patients. Forty-eight had nonconfluent or hypoplastic pulmonary arteries (mean z-score, -2; range, -11.

Absent pulmonary valve syndrome coexisting with coarctation of aorta in an adult.

The combination of absent pulmonary valve syndrome with coarctation of aorta is extremely uncommon and pathogenetically intriguing. This unusual association, which defies popular theories of pathogenesis of coarctation, may need to be looked at in the light of the current concepts regarding the morphogenesis of ventricular outflow tracts and aortic arch and the role of neural crest cells in both. A 19-year-old man with this combination underwent single-stage surgical correction though median sternotomy.

Scimitar syndrome with right hemianomalous pulmonary venous drainage into superior vena cava/right atrium junction.

Scimitar syndrome is characterized by anomalous pulmonary venous drainage of a part or the whole of the right lung into the inferior vena cava (IVC). It is extremely uncommon in scimitar syndrome for the pulmonary veins to drain anomalously to a site other than the IVC. The following case report describes an otherwise classical scimitar syndrome with anomalous drainage of the right lung cephalad into the right atrium near its junction with the superior vena cava.