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.

Double chamber right ventricle in a patient with supracristal ventricular septal defect and prolapsing right coronary cusp: role of intraoperative transesophageal echocardiography.

A supracristal ventricular septal defect (VSD), an outlet VSD situated in subaortic and subpulmonic regions, has a strong association with prolapse of the aortic valve cusp. The authors report the case of a patient operated for a supracristal VSD with prolapse of the right coronary cusp. The VSD was found to be in the subaortic position on preoperative transthoracic echocardiography, which failed to detect the presence of a double-chamber right ventricle.

Unusual coarctation-the PHACE syndrome: report of three cases.

Objectives: To report the clinical features, nature of vasculopathy observed and the management instituted in three cases of PHACE syndrome.

Results: All three patients were noted to have diffuse vasculopathy and aortic arch hypoplasia in addition to aortic coarctation adding substantial surgical risk or making surgery palliative.

Conclusion: PHACE syndrome should be considered in the differential diagnosis of aortic coarctation associated with aortic arch hypoplasia.

Severe hypotension and overflowing of venous reservoir at the initiation of cardiopulmonary bypass in a patient undergoing repair of ruptured sinus of Valsalva aneurysm: management issues.

Aneurysm of sinus of Valsalva is a rare cardiac lesion that may be acquired or congenital. The presentations of RSOV range from incidental detection to frank heart failure. Right sinus of Valsalva aneurysm usually ruptures into the right ventricle.

Pulmonary hypertension alters natural history of anomalous left coronary artery.

A girl who was diagnosed at 8 months old with anomalous left coronary artery from the pulmonary artery and was subsequently lost to follow-up, presented at the age of 11 years with congestive heart failure and severe mitral regurgitation with supra-systemic pulmonary artery pressure. With progressive mitral regurgitation, coronary steal is reduced by the rising pulmonary artery pressure, and patients present late with severe valvular cardiomyopathy.