Isolated hypoplastic right ventricle - a challenge in medical practice.

Isolated right ventricle hypoplasia (IRVH) is a disease characterized by an underdeveloped right ventricle. It is a congenital heart disease than can associate heterogeneous structural defects and nonspecific clinical features, which can often present a challenging therapeutic management. In this article, there are presented diagnostic methods and treatment options for right ventricle hypoplasia (RVH) according to clinical features, patients age and associated structural heart defects. RVH has a different prognosis in accordance with the severity of the heart defects and the patient's age at which the diagnosis is established. Thus, isolated forms of RVH generally present mild structural and functional defects that can be associated with the onset of symptoms in adolescence or even in adulthood. In these cases, atrial septal defect closure with or without superior cavo-pulmonary anastomosis can be the only procedures needed to correct the hemodynamic abnormalities and relief the symptomatology. Patients with severe form of RVH associated with complex cardiac malformations and onset of the symptoms in the neonatal period require prompt intervention and necessitate palliative procedures. In the long term, these patients could need multiple reinterventions. The family physician should be aware of the cardiac origin of isolated symptoms or clinical signs, such as exertional dyspnea or clubbing fingers, and send the patient for pediatric cardiological evaluation.

Unroofed coronary sinus: update on diagnosis and treatment.

Unroofed coronary sinus (UCS) is among the rarest congenital heart malformations in pediatric practice. Often, they are associated with persistent left superior vena cava (PLSVC) draining into the left atrium, and in this situation, there is a double intracardiac shunt, right-to-left and left-to-right. They are asymptomatic in the majority of the cases. Each ordinary imagistic method (transthoracic echocardiography, contrast transesophageal echocardiography, angiography) can be helpful in finding the diagnosis, but enhanced computed tomography scan can give an irrefutable diagnosis. The surgical intervention may consist in the recreation of a roof for the coronary sinus, and anastomosis of the PLSVC to the right atrial appendage.

Complex atrial septal defect closure in children.

Atrial septal defect (ASD) is one of the most common congenital heart diseases (CHDs) diagnosed in children. Symptoms in ASD may be absent, but palpitations and dyspnea in children sometimes reveal a complex structural and CHD. Diagnosis is established usually by transthoracic echocardiography, but in more complex cases such as coronary sinus defect, enhanced computed tomography or cardiac magnetic resonance may be used. Indication for closure depends on the dimension and on the hemodynamical impact. There are two methods for closure: surgical and interventional. Surgery may be applied to all types of ASD, while transcatheter closure may be used only for ostium secundum ASD. The most important issue in the interventional closure is the anatomical aspect related to the position of the defect. Both methods may have complications. Complex cases in children usually are not recommended for interventional closure, surgery being the recommended method. Oversizing of the device to be implanted according to the sizing balloon and not to the initial defect diameter may give a solution for large ASDs. Interventional closure of large defects in small children with an aneurysmal, multi-fenestrated, malaligned septum, or with other CHD associated may be challenging. Complications are present for both types of closure, but they are relatively different.

Scimitar syndrome associated with aberrant right subclavian artery, diaphragmatic hernia, and urinary anomalies - case report and review of the literature.

Scimitar syndrome is a form of a partially or totally right pulmonary venous return to the inferior vena cava, which may associate variably right lung hypoplasia, right pulmonary artery hypoplasia, pulmonary sequestration together with the presence of aortopulmonary collaterals from the descending aorta towards the right lung. In many cases, there are also other cardiac anomalies associated. We present a unique association of a partially anomalous pulmonary venous return to the inferior vena cava with other vascular and thoracic anomalies: inferior sinus venosus and secundum atrial septal defect, retroesophageal right subclavian artery, obstructed accessory right bronchus, diaphragmatic hernia with ectopic liver, "S"-type thoracic scoliosis and malformations of the urinary tract (duplication of the right ureter and of the left basinet). The patient had a reimplantation of the "scimitar" vein to the left atrium and closure of the inferior sinus venosus and secundum atrial septal defect.

Atretic aortic coarctation - transradial approach. Case series and review of the literature.

Stenting of aortic coarctation became the method of choice. In severe aortic coarctation, when retrograde crossing of the coarctation is impossible, a secondary approach with antegrade arterial crossing proved to be the solution in many cases. Here we report two cases of severe aortic coarctation in males aged 12 and 14, respectively, in which we used a secondary transradial approach (right and left) followed by guidewire snaring. For the first case, a right radial-right femoral arterio-arterial circuit was created, which offered a stable position during stent implantation. In both cases, we predilated the lesion and then implanted a 45 mm Cheatham-platinum (CP) covered stent. The final result was very good in both patients; only the first one required post-dilatation of both ends of the stent. Asymptomatic post-procedural absence of left radial pulse was noticed in the second case. In conclusion, even in the case of children, secondary transradial approach is suitable for antegrade crossing of the coarctation in extremely severe cases and possibly in stabilization of the circuit for stent implantation.