BACKGROUND: Aortic atresia with ventricular septal defect is a very rare congenital cardiac anomaly, especially in combination with aortic arch interruption. It is always challenging to choose the optimal treatment tactics for such patients. One of the possible types of intervention is the Yasui procedure. There are only 19 reported cases in the literature of aortic atresia with interruption of the aortic arch type B or C, and not a single clinical case of type A.
METHODS: The proband was a 2-day-old boy with diagnosis: aortic atresia with a ventricular septal defect and interruption of the aortic arch type B. The child underwent a Yasui procedure without serious postoperative complications and with good long-term result.
CONCLUSIONS: The Yasui procedure in patients with aortic atresia and interrupted aortic arch can be performed with minimal complications, even in low-weight patients.

UNASSIGNED: Electrosurgery has been long used in endovascular procedures, with only case reports in the aortic field. Our aim is to present a case series with the use of an electrified wire to perform catheter-based electrosurgery by applying external current through an electrocautery pen.
UNASSIGNED: Single-center retrospective case series of all patients undergoing complex aortic surgery from October 2020 to August 2023, in whom the electrified wire technique was used: (1) Perforation of a dissection flap or left subclavian artery (LSA) in situ endograft fenestration-a 0.014\" polytetrafluoroethylene (PTFE) insulated guidewire is detached from the insulation with a scalpel at the end and a cautery pen is here attached with a clamp. A curved tip catheter or sheath is positioned against the aortic flap or the endograft (through a left brachial access in this case) and the wire pushed, crossing the flap by activating the electrocautery pen and (2) slicing a dissection flap (\"powered cheese-wire technique\")-after same preparation as above, the middle section of the 0.014 guidewire is removed from the PTFE and bent into a V-shape. Once in the aorta, the guidewire crosses from the true lumen (TL) to the false lumen (FL) and a through-and-through access is obtained. Sheaths are positioned against the flap from both sides and moved up or down while the electricity is activated, slicing the flap and communicating both lumens. Technical success and technical-related complications were evaluated.
UNASSIGNED: Eleven cases concerning aortic dissections and 1 case of aortic atresia were treated. Four patients presented urgently, whereas the rest were planned procedures. Seven cases underwent perforation of a dissection flap, 2 cases underwent the powered cheese-wire technique, in 2 cases for an LSA in situ fenestration, and in 1 case to cross an aortic atresia at the aortic isthmus. The technique was in all cases successfully applied. No complications related to the technique occurred.
UNASSIGNED: The \"electrified wire\" technique is a feasible and ready-available tool that can be safely used in complex aortic interventions, especially to perforate aortic tissue like dissection flaps or to perform in situ fenestrated repairs by perforation of the endograft fabric.
CONCLUSIONS: The electrified wire technique described herein is a straightforward technique that uses readily available tools to perform electrosurgery. We present its use in complex aortic procedures. However, it could be envisioned for any vascular procedure that requires crossing of the vessel or even prosthetic material. As we have described in this series, when used along with an adequate properative planning, it can be a safe tool of great utility, as has already been demonstarted in the field of the interventional cardiology.

The coexistence of aortic valve atresia and interrupted aortic arch are an extremely rare condition. In this pathology, blood flow to the ascending aorta and coronary arteries should be provided through the ductus arteriosus or collaterals originating from the descending aorta. In rare cases where bilateral ductus arteriosus is present, they can provide circulation. Here, we report two cases in which coronary arteries and ascending aorta were supplied by one ductus arteriosus and distal systemic circulation is supplied by a second ductus arteriosus in one patient and a collateral artery in the other. Initial palliation was successfully performed by bilateral pulmonary artery banding and transcatheter ductal stent implantation in both cases.

A 24-year-old man was referred to our tertiary care center for the management of uncontrolled hypertension secondary to severe coarctation of aorta.

Atresia of the aortic valve is usually associated with hypoplasia of the mitral valve and the left ventricle. In very rare cases, a ventricular septal defect may be associated with aortic atresia, when left ventricle and mitral valve are normal-sized, due to the presence of an outflow for the left ventricle through the ventricular septal defect. We present the multi-modality imaging findings of an adolescent girl who presented with breathlessness and was later found to have aortic valvar atresia with a normal-sized left ventricle.

UNASSIGNED: The Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation.
UNASSIGNED: We retrospectively reviewed all patients undergoing the Yasui operation between 1989 and 2021. Results are reported as median with interquartile range (IQR).
UNASSIGNED: Twenty-five patients underwent a Yasui operation (19 primary), at 11 days (IQR, 7-218 days) of life and weight of 3 kg (IQR, 2.8-4.1 days). Fundamental diagnosis was ventricular septal defect/interrupted aortic arch in 11 patients and ventricular septal defect/aortic atresia in 14. Follow-up was 96% (24 out of 25) at 5 years (IQR, 1.4-14.7) with 92% survival. Freedom from LVOTO reoperation was 91% at late follow-up with 2 patients requiring baffle revision at 6 and 9 years. Latest echocardiogram showed 100% of patients had normal biventricular function and 87% (20 out of 23) less than mild LVOTO at 5 years (IQR, 2.3-14.9). Diagnosis, aortic valve morphology, and material used were not predictors of LVOTO. Freedom from right ventricle-to-pulmonary artery conduit reoperation was 48% at a median of 5 years (IQR, 1.4-14.7). Conduit type was not a predictor of reintervention.
UNASSIGNED: The Yasui operation can be performed with low morbidity and mortality in patients with 2 acceptable-size ventricles and aortic atresia or interrupted aortic arch with severe LVOTO. Despite some burden of reoperation, midterm reoperation for LVOTO is not common and ventricular function is preserved.

As yet, there is no agreed definition for the so-called \"hypoplastic left heart syndrome\". Even its origin remains contentious. Noonan and Nadas, who as far as we can establish first grouped together patients as belonging to a \"syndrome\" in 1958, suggested that Lev had named the entity. Lev, however, when writing in 1952, had described \"hypoplasia of the aortic outflow tract complex\". In his initial description, as with Noonan and Nadas, he included cases with ventricular septal defects. In a subsequent account, he suggested that only those with an intact ventricular septum be included within the syndrome. There is much to commend this later approach. When assessed on the basis of the integrity of the ventricular septum, the hearts to be included can be interpreted as showing an acquired disease of fetal life. Recognition of this fact is important to those seeking to establish the genetic background of left ventricular hypoplasia. Flow is also of importance, with septal integrity then influencing its effect on the structure of the hypoplastic ventricle. In our review, we summarise the evidence supporting the notion that an intact ventricular septum should now be part of the definition of the hypoplastic left heart syndrome.

We present the unique case of atretic aortic valve associated with hypoplastic ascending aorta and double aortic arch of unusual configuration supplying retrograde blood flow to the coronary arteries.

We report a rare case of aortic valve atresia and type C interrupted aortic arch with retrograde filling of the ascending aorta via the right common carotid artery through intracranial collateralisation and a presumed intact circle of Willis, who successfully underwent a complete biventricular repair in the neonatal period.

BACKGROUND: Distal segment atresia (isthmus) is an extremely rare anatomical variant of obstructive aortic arch anomalies.
METHODS: We present the case of a newborn who, at 48 hours of life, presented a clinical picture of heart failure. The initial echocardiogram showed a congenital interrupted aortic arch type A, patent ductus arteriosus, and ventricular septal defect. Prostaglandins were initially indicated. Subsequently, a second echocardiogram showed the absence of ductus arteriosus; the CT angiography study confirmed this finding and revealed blood flow to the descending aorta through small intercostal blood vessels. The possibility of atresia of the distal segment (isthmus) of the aortic arch was considered and confirmed at the time of surgery.
CONCLUSIONS: Aortic atresia should be considered a diagnostic possibility in the presence of type A interrupted aortic arch since the hemodynamic behavior between them is similar. Surgical medical treatment should be individualized since this condition is frequently an emergency in the neonatal period. However, this is not always the case, as other cases have been reported in schoolchildren and adults.
UNASSIGNED: La atresia de segmento distal (istmo) de arco aórtico es una variante anatómica extremadamente rara de las anomalías obstructivas del arco aórtico.
UNASSIGNED: Se presenta el caso de un recién nacido que a las 48 horas de vida presentó un cuadro clínico de insuficiencia cardiaca. El estudio de ecocardiograma inicial mostró una anomalía congénita de interrupción de arco aórtico tipo A, conducto arterioso y comunicación interventricular. De inicio se indicaron prostaglandinas. Posteriormente, el segundo ecocardiograma mostró la ausencia del conducto arterioso; el estudio de angiotomografía confirmó este hallazgo y también reveló flujo sanguíneo hacia aorta descendente a través de pequeños vasos sanguíneos intercostales. Se consideró la posibilidad de atresia del segmento distal (istmo) de arco aórtico y se confirmó al momento del acto quirúrgico.
CONCLUSIONS: La atresia aórtica debe ser considerada como posibilidad diagnóstica en presencia de interrupción de arco aórtico tipo A, ya que el comportamiento hemodinámico entre ellos es similar. El tratamiento médico quirúrgico debe individualizarse, ya que es frecuente que sea una urgencia en el periodo neonatal. Sin embargo, no sucede así siempre, ya que se han reportado casos en escolares y adultos.