Pulmonary arteriovenous fistula coexisting with secundum atrial septal defect. An unusual cause of stroke in a young woman.

Pulmonary arteriovenous malformations (PAVM) consist of an abnormal connection between the pulmonary artery and vein bypassing the pulmonary capillary bed. Pulmonary arteriovenous fistulae are characterized by a right-to-left shunt of variable magnitude, and they may be accompanied by other congenital anomalies. We describe a case of pulmonary arteriovenous fistula with a secundum type atrial septal defect (ASD) in a 38-year-old woman, who underwent successful transcatheter closure of both the ASD and PAVM.

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 < or = 3rd 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). Following 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.

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.

Balloon atrial septostomy under echocardiographic guidance. Case report.

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. The limitations of transthoracic ECHO guidance of BAS include the possibility of a poor ECHO window in an ill neonate on assisted ventilation and possible interference with maneuverability for both echocardiographer and catheter operator. It also carries the risk of contamination of the sterile field. When the advantages and disadvantages of transthoracic ECHO guidance are considered, transferring the baby to the catheterization laboratory can cause problems and time loss. Thus, ECHO-guided BAS at bedside is an efficient and good alternative. The transumbilical approach may be easier in the first few days of life.

Anomalous origin of the left coronary artery from the pulmonary artery.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCA-PA) is a rare form of congenital heart disease. In this report, three cases with this anomaly are described; two patients presented in infancy with heart failure from myocardial ischemia and infarction, while the third was asymptomatic and ALCA-PA was diagnosed during evaluation of a residual murmur after surgery for associated cardiac defects (ventricular septal defect and patent arterial duct). All three cases underwent aorto-pulmonary tunnel repair (Tukeuchi procedure), and to our knowledge two of them are the first infantile cases reported in Turkey.

Balloon dilatation angioplasty of a stenotic Blalock-Taussig anastomosis. A case report.

A 12-year-old boy with tetralogy of Fallot was evaluated for increasing cyanosis and exercise intolerance. The patient had a right Blalock-Taussig shunt operation 11 years previously and a left modified Blalock-Taussig shunt operation one year previously. After demonstration of hypoplastic pulmonary arteries, the stenosed right Blalock-Taussig shunt and the totally occluded, left modified Blalock-Taussig shunt were successfully dilated with a 7-mm-diameter angioplasty balloon resulting in a 5-mm-diameter patent shunt. We believe that, when possible, balloon dilatation angioplasty of stenosed Blalock-Taussig shunts is a reasonable and safe alternative to surgery.