BACKGROUND: The purpose of this study was to review echocardiography-based diagnosis of persistent fifth aortic arch (PFAA) in children.
METHODS: From January 2015 to December 2022, we retrospectively analyzed the echocardiographic findings and the relevant clinical data during follow-up of patients with PFAA who were treated in the Third Affiliated Hospital of Zhengzhou University. The diagnosis was confirmed by computed tomography angiography or surgery.
RESULTS: Seven PFAA cases included two Weinberg type A and five Weinberg type B. The anatomical details of PFAA were assessed using a combination of the long-axis view of the left ventricular outflow tract (from the left high parasternal window) and the long-axis view of the aortic arch (from the suprasternal window). In Weinberg type A, the distal fifth and fourth aortic arches were connected to the descending aorta, which was associated with aortic coarctation. In Weinberg type B, the upper arch of the fourth aorta was interrupted, and only the lower arch of the fifth aorta was connected to the descending aorta. Surgical repair of PFAA was indicated in five patients with blood flow disruption, among which four had good postoperative results and one refused surgery. Two patients with unobstructed PFAA blood flow required follow-up rather than surgery.
CONCLUSIONS: It is feasible to diagnose PFAA by echocardiography. Combined application of the high parasternal left ventricular outflow tract view and the suprasternal aortic arch view can improve timely detection of different types of PFAA in children.

PHACES syndrome is an acronym for the syndromic presentation of Posterior fossa malformation, Hemangioma, Arterial anomalies, Coarctation of aorta/cardiac defects, Eye abnormalities and Sternal malformations. Infantile hemangiomas are the most common tumors of infancy. Regional odontodysplasia, commonly referred to as \"ghost teeth\", is a rare localized developmental malformation of enamel and dentin with varying levels of severity that results in unusual clinical and radiographic appearances of affected teeth. This report describes a rare case of a two-year-old Caucasian male diagnosed with PHACES syndrome also presenting with multi-regional odontodysplasia. Ten of twenty teeth were dysplastic. The patient was treated under general anesthesia in a hospital setting. All affected primary teeth were extracted due to sensitivity, abscess and extremely poor long-term prognosis. Moving forward, a long-term interdisciplinary approach will be necessary to address this child\'s dentition as it develops.

Aortic coarctation is a rare cause of secondary hypertension (<1% cases) and can be challenging to detect due to its few clinical manifestations. Early diagnosis and treatment are important because patients with unmanaged aortic coarctation are at increased risk of cardiovascular complications and have a reduced life expectancy. We describe a case of secondary hypertension in a young adult female caused by aortic coarctation, first detected in a general practitioner setting, resulting in the need for a left subclavian-carotid bypass vascular surgery and a descending aortic stent vascular surgery. This case highlights the critical role that proximity medicine in general practice can have in improving the early detection of clinically silent conditions by routinely monitoring blood pressure and other vital parameters, and the increasing importance of medical imaging in assisting early diagnosis and guiding the surgical management of complex cases.

BACKGROUND: Supra aortic obstruction in children is uncommon and is seen in certain unique conditions. While intraluminal obstruction due to heavy calcification is seen in older populations, it is not described in pediatric populations. The coral reef aorta is a rare and distinct calcifying disease causing luminal obstruction of the suprarenal aorta in adults. The definition of this diagnosis relies entirely on the unique aspects and consistency of the lesions, which are rock-hard, irregular, gritty plaques with a white luminal surface resembling a coral reef. However, no such case has been described in children.
METHODS: We present an adolescent boy who presented with a heavily calcified ascending aortic lesion associated with aortopathy and hypertension, 12 years after an aortic coarctation repair. The investigations included echocardiography, magnetic resonance and computer-tomographic imaging. A 3-D model was printed in order to visualize and plan surgical steps in advance for safe placement of clamps and defining the extent of resection. In addition, it provided an idea about tissue quality, thickness, spatial relationship, and orientation in relation to surrounding structures. Successful resection and replacement of the diseased segment of the aorta were achieved on cardiopulmonary bypass support. Post-operative recovery was uneventful, and at 6-month follow-up, the patient is doing well. In this report, various aspects of such lesions have been discussed, including clinical presentations, complications, planning and conduct of a safe cardiopulmonary bypass, and precautions during surgery for a successful outcome.
CONCLUSIONS: Complicated obstructive aortic lesions in children require careful assessment, appropriate advanced imaging, and the use of 3-D printing technology in order to plan and perform safe and effective surgical management. The etiology of severe calcified aorta in children may be related to metabolic factors, previous surgery, use of a homograft, or an inflammatory process. However, it has yet to be proven.

Shone complex (SC) is a rare congenital heart disease characterized by four obstructive anomalies, including parachute mitral valve (PMV), left atrial supra-valvular ring, subaortic stenosis, and coarctation of the aorta. Typically, SC manifests early in life. However, we encountered a 52-year-old female with a history of hypertension diagnosed at 26 years and left-sided weakness poststroke. She presented with worsening dyspnea and palpitations, prompting a thorough investigation. Echocardiography revealed a heavily calcified bicuspid aortic valve with severe aortic stenosis and parachute mitral valve with severe mitral stenosis and preserved ejection fraction, raising suspicions regarding the presence of SC. Cardiac catheterization, aortic-angiography, and noncontrast chest computed tomography (CT) revealed abrupt occlusion of the postductal aorta, giving a picture of aortic coarctation with well-established collateral vessels including prominent right and left internal mammary arteries. So, she was diagnosed with an incomplete SC at the age of 52. Shone complex is a rare congenital heart disease that typically presents in early childhood, but late presentations due to misdiagnosis or incomplete work up are possible. This case emphasizes the rarity of late presentations of SC and highlights the importance of early diagnosis and intervention to improve outcomes. An incomplete SC should be considered in adult patients presenting with left-sided obstructive lesions.

LACHT (Lung Agenesis, Congenital Heart, and Thumb anomalies) syndrome is an extremely rare congenital anomaly and presents significant challenges in adults due to its poor survival rates. Herein, we report a case of late diagnosis and successful transcatheter treatment of aortic coarctation in a 58-year-old male patient with LACHT syndrome, medically resistant arterial hypertension, and left lung agenesis. Baseline CT angiography showed isthmic aortic coarctation and left lung agenesis, with compensatory right pulmonary artery and vein thickenings. The patient underwent balloon dilation and subsequent implantation of a covered NuMED 45 mm 8-ZIG CP stent with satisfactory outcomes. The pressure gradient decreased from 43 to 23 mmHg. The arterial pressures normalized during the follow-up with fewer medications. Genetic testing identified a heterozygous mutation (c.6583C > T) in the FBN2, supporting the diagnosis of variant Marfan syndrome.

We present the case of a 63-year-old woman who was hospitalized five times in 4 months for episodes of heart failure, associated with a sustained hypertension despite a fivefold therapy. The pathophysiological mechanism of the hypertension was a secondary hyperaldosteronism linked to a renal hypoperfusion due to the narrowing of the thoracic aorta by a huge calcified plaque, mimicking an aortic coarctation.

A 47-year-old man with a history of hypertension was found to have a prominent aortic knob on routine chest X-ray and was referred to our hospital. Enhanced computed tomography angiography showed severe flexion at the proximal descending aorta with chronic type B dissection localized to the flexion region. Graft replacement of the distal aortic arch was performed. Surgical management of chronic pseudocoarctation dissection is sparsely reported in the literature because of its rare occurrence. We present an operative case of a patient with chronic dissection of distal aortic arch pseudocoarctation.

We present the case of a 38-year-old male with a diagnosis of Stanford A aortic dissection and associated coarctation of the thoracic aorta. Acute dissection associated with coarctation of the aorta is a rare problem and difficult to manage surgically. Establishing a cardiopulmonary bypass (CPB) with adequate flows is the main objective of the procedure; optimal cannulation ensures the protection of cerebral and visceral organs. We successfully performed aortic valve re-implantation surgery (T. David Surgery), replacement of the ascending aorta and aortic arch, as well as debranching of the supra-aortic trunks. The cannulation technique was axillary and femoral to guarantee flows through the coarctation area.
Presentamos el caso de un varón de 38 años con diagnóstico de disección de aorta Stanford A y coartación de aorta torácica asociada. La disección aguda asociada a la coartación de la aorta es un problema raro y difícil de manejar quirúrgicamente. Establecer un bypass cardiopulmonar con flujos adecuados es el principal objetivo del procedimiento; una canulación óptima asegura la protección de órganos cerebrales y viscerales. Realizamos con éxito una cirugía de reimplante de válvula aórtica (cirugía de T. David), reemplazo de la aorta ascendente y del arco aórtico, además de debranching de los troncos supraaórticos. La técnica de canulación fue axilar y femoral para garantizar flujos a través de la zona de coartación.

Aortic coarctation is a congenital malformation that is relatively prevalent, occurring in approximately 5 out of every 1000 births. The narrowing typically happens at the aortic isthmus between the left subclavian artery and the arterial ligament. It is frequently associated with a bicuspid aortic valve. Generally, coarctation of the aorta is identified and treated during childhood or early adulthood. If left untreated, this condition can lead to a reduced life expectancy in individuals who have not received treatment. We present a case of a 52-year-old man who complained of chest pain, sputum, and hemoptysis persisting for approximately 2 years. Contrast-enhanced computed tomography (CT) scans revealed the presence of an anterior mediastinal mass, which was later confirmed to be a thymic carcinoma (on histological study). Additionally, an incidental finding of a thoracic aortic coarctation with a well-developed collateral circulation was observed. The discovery of aortic coarctation in adult patients as an incidental finding is rare and particularly uncommon in association with mediastinal or thoracic tumor pathology. Adult and elderly patients with uncorrected coarctation generally have a low survival rate, and the management strategies for such cases are controversial, especially when it is associated to other pathologies. Due to the complexity of therapeutic management and the limited literature available on postsurgical outcomes in these cases, making therapeutic decisions requires a multidisciplinary approach and personalized consideration for each individual case. This approach is necessary to evaluate the risk-benefit ratio and determine the most suitable therapeutic solution.