Introduction: Generalized arterial calcification of infancy (GACI) is a rare cause of infantile heart failure and systemic hypertension with a poor prognosis, characterized by extensive calcification and proliferation of the intimal layer of large and medium sized arteries.Case report: We present the first case report of successful surgical treatment of severe aortic arch obstruction by calcified plaques mimicking severe coarctation of the aorta and the outcome (of bisphosphonate therapy) in a newborn with GACI. Furthermore, we report the identification of a variant in ATP Binding Cassette Subfamily C, Member 6 (ABCC6) gene, possibly associated with severe early-onset manifestations of GACI.Conclusion: This case report highlights the importance of considering GACI in an infant with heart failure, systemic hypertension, and evidence of increased echogenicity of the arterial vessels. We noted the favorable outcome in improving the aortic calcification in our patient after surgical treatment and bisphosphonates therapy. Early diagnosis and treatment improve the long-term prognosis. A better understanding of this rare genetic disease could lead to new therapeutic strategies.

Background: The term ‘left atrial appendage aneurysm’ (LAAA) has been recognized since 1962, when it was first described. It is an exceedingly rare pathology that can affect both adults and children. Often asymptomatic, it may be discovered incidentally. The anomaly consists of an exaggerated dilation of the primitive portion of the left atrium, resulting from pectinate muscle dysplasia or as a consequence of an obstructive lesion between the left atrium and the mitral valve. Surgical intervention represents a reliable strategy for preventing catastrophic complications such as stroke, thromboembolism, and rupture. This is a very rare condition, which is why we aimed to present a case report along with a review of the literature. Case presentation: We report the case of a 6-year-old asymptomatic girl in whom a giant left atrial appendage aneurysm was incidentally detected during a routine transthoracic echocardiogram, associated with a small atrial septal defect and a prothrombotic genetic profile. The aneurysm was successfully excised, and the atrial septal defect was closed. The postoperative course was uneventful, and the patient was discharged home on the 8th postoperative day. Conclusions: Left atrial appendage aneurysm is rare in children and often asymptomatic, yet it may be life-threatening due to stroke or thromboembolism. Fetal echocardiography may be considered in selected high-risk pregnancies, and routine postnatal assessment is advised, with surgical intervention recommended particularly for patients with risk factors for thrombus formation in the left atrium or its appendage.

Right ventricular (RV) failure is a common complication in multiple congenital heart disease (CHD), significantly increasing morbidity and mortality. Despite its impact, no therapies specifically target the failing RV. The growing population of CHD patients underscores the need to understand the pathophysiology of RV failure through preclinical research. This study aimed to develop an ovine model of RV failure induced by pressure and volume overload. A total of 14 juvenile sheep randomly underwent pulmonary artery banding (n = 6), pulmonary leaflet perforation (n = 4), and pulmonary annulotomy with transannular patching (TAP) (n = 4). Detailed anesthetic and surgical protocols were described, and intraoperative and early postoperative complications were evaluated. Acute RV pressure overload resulted in a 120% increase in RV pressure (p = 0.0312). The stroke volume index and cardiac index significantly declined (p = 0.0312), and there was a significant decrease in ScvO₂ (p = 0.0312). Both TAP and leaflet perforation achieved moderate-to-severe pulmonary regurgitation. Both procedures led to an incremental trend in RV pressures and resulted in a 24% increase in the stroke volume index. All techniques demonstrated safety and feasibility, with low mortality. This comprehensive model could be reproducible in other large animal models, offering a robust platform for preclinical research into CHD-RV failure models.

Introduction: Coarctation of the aorta represents a narrowing of the thoracic aorta. Hypertensive patients with blood pressure differences ≥20 millimetres of mercury have an indication for surgical or interventional treatment. Implantation of a covered stent became the preferred therapy for the management of this pathology in adolescents/adults. Case report: We report the case of a 14-year-old male sportsman, who presented in the emergency room with headache, dizziness, and tinnitus. The clinical exam revealed blood pressure differences between the upper and lower limbs of up to 50 mmHg. Based on the clinical and paraclinical data, we established the diagnosis of coarctation of the aorta and severe secondary arterial hypertension. The case was discussed by a multidisciplinary team and accepted for covered stent implantation. The 24 h blood pressure Holter monitoring after the procedure indicated the persistence of stage I arterial hypertension. Conclusions: Coarctation of the aorta is a congenital cardiovascular anomaly with high morbidity and mortality rates. Arterial hypertension, heart failure, and aortic dissection are complications of this pathology, some of them being sometimes direct consequences of secondary hypertension. Periodic cardiology follow up after the procedure is mandatory to assess the hemodynamic response, to identify potential complications, and to stratify the cardiovascular risk.

Cardiotoxicity remains one of the leading causes of morbidity and mortality in childhood cancer survivors. This study aimed to evaluate cardiotoxic side effects of chemotherapeutic regimens in a pediatric population, as revealed by assessment of baseline end-of-treatment and in follow-up after 1 year. A prospective study was conducted on 39 children, enrolled over a period of 5 years (2017-2022), diagnosed with leukemia and receiving chemotherapy regimens. A multimodal echocardiographic evaluation was done (two-dimensional echocardiography and M-mode, tissue doppler imaging (TDI) and 2D-speckle tracking (2DST) before the initiation of the cytostatic treatment, at the end of the treatment and 1 year after the treatment. Ultrasound-derived parameter values were compared between evaluations and correlated with cumulative doses of chemotherapeutic agents used. There were no significant differences in mean values for any of the imagistic parameters analyzed. However, a significant correlation was found between E’value percentage variation and cumulative dose of both anthracyclines and antimetabolics administered (p=0.03). Moreover, a significant linear regression correlation was found between alkylating agent dose and global longitudinal strain (GLS)(p<0.01). Non-invasive cardiovascular imaging parameter values did not differ significantly from baseline in our study, but some of them seem to be dependent upon cumulative dose of cytostatic agents used. Further studies on larger populations and involving a longer follow-up period might provide more insight into cardiotoxicity of chemotherapy regimens.

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