Persistent primitive hypoglossal artery complicated by atrial septal defect and congenital intrahepatic shunts.

During early embryogenesis, anastomoses are formed between the carotid artery and the basilar or the vertebral artery, and subsequently, these anastomoses regress. In some cases, these anastomoses remain as persistent carotid-basilar or carotid-vertebral anastomoses. Atrial septal defect (ASD), a communication between the atria at the septal level, is a congenital heart anomaly. Intrahepatic venous shunts between the portal and hepatic veins are very rare and only some are considered congenital. We present the first case report of a patient with an ASD, a persistent primitive hypoglossal artery, and congenital portahepatic shunts.

Successful surgical treatment of a gigantic congenital coronary artery fistula immediately after birth.

A foetus was prenatally diagnosed with a gigantic (12 mm) coronary artery fistula (CAF) from the left anterior descending (LAD) coronary artery to right ventricular apex at 38 weeks of gestation. LAD was dilated to 10 mm with partial aneurysmal changes. Because of concern for sudden ischaemic cardiogenic shock soon after birth, the child was electively delivered by caesarean section, with surgical fistula closure subsequently performed 1 h after birth. We also highly suspected the presence of a clinically significant accessory diagonal branch just around the fistula, thus direct fistula closure from outside the heart without cardiopulmonary bypass was abandoned and cardiopulmonary bypass was initiated. The terminal end of LAD was carefully opened, and the fistula was directly closed with four pairs of 6-0 polypropylene mattress sutures under cardioplegic arrest, while the opened terminal end of LAD was also repaired with plegetted 6-0 polypropylene mattress and over-and-over sutures. After 4 days of post-surgical extracorporeal life support for over-systemic pulmonary hypertension, the patient recovered without complications. Although postoperative echocardiography 5 months after the operation showed normal cardiac function without ventricular asynergy, the dilated and aneurysmal LAD remained unchanged.

Deep-hidden anastomoses in monochorionic twin placentae are harmless.

OBJECTIVES: Our objective was to identify the clinical consequences of deep-hidden anastomoses that occur underneath the placental surface. METHODS: Twelve placentae that underwent intrauterine laser ablation of placental anastomoses for twin-twin transfusion syndrome (TTTS) and 14 non-TTTS controls were investigated for deep-hidden anastomoses. Additionally, we investigated the inter-twin haemoglobin differences as an indicator for fetofetal transfusion. Placentae were divided into four groups: TTTS placentae without residual chorionic-plate anastomoses without deep-hidden anastomoses (group 1) and with deep-hidden anastomoses (group 2), and non-TTTS placentae with chorionic-plate anastomoses without deep-hidden anastomoses (group 3) and with deep-hidden anastomoses (group 4). RESULTS: Deep-hidden anastomoses were identified in 58% (7/12) of the TTTS placentae after laser surgery and in 64% (9/14) of the non-TTTS placentae. Groups 1 and 2 had equal inter-twin haemoglobin differences: medians 1.4 and 1.2 gr/dL, respectively (p = 0.48). In group 3, the median inter-twin haemoglobin difference without deep-hidden anastomoses was 2.6 gr/dL (group 3) and with deep-hidden anastomoses (group 4) it was 5.1 gr/dL (p = 0.26). CONCLUSION: Both comparisons imply that deep-hidden anastomoses did not cause any additional increase in Hb difference. In conclusion, haematological and additional hemodynamical analysis show that deep-hidden anastomoses are likely to occur without any clinical consequences.

An embryological perspective on congenital portacaval shunt: a rare anomaly in a patient with hepatocellular carcinoma.

We describe a 42-yr-old woman with hepatocellular carcinoma and a congenital portacaval shunt. A computed tomography (CT) scan of the abdomen showed a prominent left hepatic lobe extending into the lower abdomen. A large encapsulated, necrotic-appearing mass was seen within the right hepatic lobe. The patient underwent hepatic resection, during which the continuation of the confluence of the splenic and superior mesenteric veins was found to empty directly into the inferior vena cava, bypassing the hepatic parenchyma. An extended right hepatic lobectomy was performed with a complete excision of the mass (T3 N0 M0, stage III). The patient had an uneventful postoperative course. To our knowledge, this is the first reported case of this anomaly in a living adult having undergone hepatectomy.

Prenatal diagnosis of tetralogy of Fallot associated with a fistula from the left coronary artery to the left atrium.

In a fetus at 20 weeks gestation, we found a large fistula from the left coronary artery to the left atrium in association with tetralogy of Fallot. Postnatally, the therapeutic issues were complex because of prematurity, low birth weight, decreased flow of blood to the lungs, and volume overload of the left ventricle because of the huge fistula. At three months, the baby underwent repair of tetralogy of Fallot, with surgical ligation of the fistula, but the baby died postoperatively.

Evaluation of the fetal coronary circulation.

Prenatal ultrasound today allows the detailed study of small caliber vascular beds including the fetal coronary arteries and the coronary sinus. The coronary circulation is unique because of its critical role in myocardial metabolism and function and its ability to adapt in many fetal conditions. The ultrasound examination techniques for the evaluation of the fetal coronary circulation are presented. Evaluation of the coronary arteries is primarily achieved by color flow imaging and pulsed wave Doppler. Conditions such as fetal growth restriction, anemia, ductus arteriosus constriction and bradycardia are associated with evidence of enhanced coronary blood flow. These findings suggest that short-term autoregulation and long-term alterations in myocardial flow reserve are present in the human fetus. At present, examination of coronary sinus blood flow has limited clinical utility, while increases of the coronary sinus diameter or attenuation of coronary sinus dynamics may provide useful markers of abnormalities of central venous drainage. Abnormal vascular connections between the coronary arteries and the ventricular cavities may be observed in outflow tract obstructive cardiac lesions. In these conditions prenatal detection of coronary fistulae may have a potential impact on outcome and therefore counseling and perinatal management.