Left cervical aortic arch with proximal obstruction

Cervical aortic arch is a rare congenital anomaly. We present a case of left-sided cervical aortic arch, found in a patient with mitral regurgitation, mitral stenosis, and a regurgitant bicuspid aortic valve. There was atypical obstruction proximally within the arch. The obstructive segment was resected, and corrected by performing an end-to-side anostomosis. Mitral valvoplasty was performed in the same surgical procedure. To the best of our knowledge, a cervical arch has not previously been described with such atypical obstruction, and in association with multiple lesions involving the left heart

Mitral perforation: a rare cause of congenital mitral regurgitation

In a 4-year-old boy with severe mitral regurgitation, cross sectional echocardiography combined with Doppler interrogation confirmed the presence of isolated perforation of the aortic leaflet of the mitral valve. The perforation was closed with a patch of fresh autologous pericardium. Serial echocardiograms taken postoperatively showed no regurgitation across the mitral valve

Antenatal diagnosis of postductal coarctation of the aorta - A case report

Fetal echocardiography can be used to detect congenital heart disease prenatally with a high degree of accuracy, and complex heart malformations have also been clearly described in the fetus. However, it is difficult to diagnose correctly or to exclude definitely aortic coarctation by fetal echocardiography. A 23-year-old woman was referred for fetal echocardiographic examination at 21 weeks' gestation after discovery of hydrops fetalis (nonimmune) on an obstetric ultrasound examination. Aortic isthmus appeared hypoplastic with a diameter less than or equal to3(rd) percentile for gestational age. There was a narrowing within the descending aorta immediately distal to the origin of the ductus arteriosus. Color flow imaging demonstrated acceleration and turbulent flow and the peak pressure gradient was measured 83 mmHg by continuous wave Doppler in the same area. The pregnancy terminated in spontaneous abortion at 22 weeks' gestation. The fetus was stillborn. The autopsy findings confirmed the prenatal diagnosis. We conclude that together with the quantitative estimation of the aortic arch, color Doppler and continuous wave Doppler are helpful in diagnosis and estimation of the pressure gradient

Prenatal echocardiographic diagnosis of situs inversus totalis and transposition of the great arteries - A case report

A case of situs inversus totalis and transposition of the great arteries (TGA) was diagnosed prenatally at 25 weeks' gestation. Postnatal echocardiographic examination confirmed the antenatal findings. This case underscores the importance of recognizing situs abnormalities during obstetric and fetal echocardiographic examination, as they are often associated with cardiac anomalies. Accurate prenatal diagnosis of structural heart defects is extremely important in family counselling and in planning obstetric and postnatal treatment

Balloon dilatation angioplasty of stenosed systemic - pulmonary artery shunts

Seven children and an adult patient with cyanotic congenital heart defects underwent balloon dilatation angioplasty (BDA) of a stenosed systemic-pulmonary artery shunt to improve arterial oxygen saturation. We attempted to perform BDA using the transvenous route in all patients in whom the aorta connected with the right ventricle, such as in tetralogy of Fallot or double outlet right ventricle, in an effort to avoid femoral artery injury. We could use the transvenous route (antegrade) in three children with tetralogy of Fallot and in one child with tetralogy of Fallot and pulmonary atresia (one of them was 6.6 kg). Fallowing BDA, there was an increase in arterial oxygen saturation from a mean of 65.9 +/- 12.8% to a mean of 78.1 +/- 8.3% (p < 0.05). On follow-up three to 37 months (mean 16.5 +/- 11.2 months) after BDA, the condition of all patients had improved. Pulmonary hypertension developed in one patient during the follow-up period. It is concluded that BDA of stenosed systemic-pulmonary artery shunts is reasonable, effective and safe. Use of the transvenous route, if possible, to perform balloon dilatation angioplasty facilitates the safe advancement of the larger balloons in low-weight children

Balloon atrial septostomy under echocardiographic guidance

A seven-days-old male neonate was transferred to our institution in critically ill condition. Echocardiographic (ECHO) examination revealed the transposition of the great arteries (TGA) with a small ventricular septal defect. In the laboratory examination, arterial oxygen saturation was 29 percent and pH was 7.16, The poor condition of the neonate led us to decide to perform an immediate bedside balloon atrial septostomy (BAS) in the intensive care unit (ICU) with ECHO guidance. The umbilical vein was cannulated with a 5 Fr. Miller BAS catheter, Four balloon passes were performed resulting in large atrial septal defect. After the procedure, arterial oxygen saturation was measured at 40 percent. In TGA, the baby may present with severe hypoxia and may need management in the ICU. Emergency BAS may improve the clinical condition of the patient. Transferring the baby, who is mechanically ventilated (and is in openbed), to the catheterization laboratory takes time and can be harmful for him, and carries risk of extubation and heat loss

Scimitar syndrome with absence of the right pulmonary artery - A case report

We report a two-year-old female child with scimitar syndrome associated with absent pulmonary artery but with normal pulmonary artery pressure although several collaterals originated from the abdominal aorta to the right lung. To our knowledge, this is the fifth case with an absent pulmonary artery. Our case also had microphthalmia, an association not previously described in scimitar syndrome