先天性三尖弁狭窄症

Pediatric congenital right heart obstructive lesions encompass a spectrum of diseases that obstruct blood flow from the right atrium to the pulmonary artery. Right ventricular inflow obstructions include tricuspid valve abnormalities, such as Ebstein anomaly, tricuspid valve dysplasia, and tricuspid atresia. Right ventricular outflow obstructions include pulmonary valve stenosis, pulmonary atresia, and tetralogy of Fallot. Cardiac computed tomography angiography (CCTA) is a valuable tool for the diagnosis, treatment planning, and follow-up of these lesions. In this pictorial review, we highlight the diagnostic utility of CCTA in congenital right heart obstructive lesions, emphasizing its role in preoperative planning.

Atrioventricular valvular regurgitation (AVVR) is a principal determinant of morbidity and mortality in adults with congenital heart disease (ACHD), and surgical management carries substantial operative risk owing to prior sternotomies, distorted intracardiac anatomy, impaired ventricular function, and lesion-specific hemodynamic fragility. Transcatheter edge-to-edge repair (TEER)-using MitraClip or TriClip (Abbott) or PASCAL/PASCAL ACE (Edwards Lifesciences)-has emerged as a compelling alternative in high-surgical-risk acquired valvular disease and is rapidly expanding into ACHD. We review the anatomic substrate, device-specific technical considerations, preprocedural imaging requirements, and clinical evidence supporting TEER across the principal ACHD substrates: congenitally corrected transposition of the great arteries (TGA) with systemic tricuspid regurgitation, atrial switch repairs for dextro-TGA, atrioventricular septal defects, and single-ventricle Fontan circulations. We also address procedural challenges unique to ACHD, including venous access anomalies, altered transseptal geometry, absent conventional echocardiographic windows, and iatrogenic stenosis risk in morphologically abnormal valves. Multidisciplinary selection integrating ACHD structural expertise, advanced imaging, and procedural TEER experience is requisite for safe outcomes.

BACKGROUND: Lutembacher syndrome (LS) is a rare condition characterized by the coexistence of mitral stenosis (MS) and an atrial septal defect (ASD). Clinical manifestations vary according to the severity of MS and the size of the ASD. CASE PRESENTATION: We report a 13-year-old female with congenital MS and a secundum ASD who presented with decompensated right heart failure after being lost to follow-up for 10 years. Progressive worsening of MS and enlargement of the ASD resulted in significant left-to-right shunting, severe right heart volume overload, pulmonary hypertension, tricuspid annular dilation, and severe tricuspid regurgitation. Cardiac catheterization demonstrated low cardiac output, pulmonary overcirculation, and postcapillary pulmonary hypertension. The patient underwent mechanical mitral valve replacement, ASD closure, and tricuspid annuloplasty. OUTCOME: Postoperatively, the patient experienced transient left ventricular dysfunction that gradually improved with medical therapy. At 2-year follow-up, she demonstrated normal biventricular systolic function and complete resolution of heart failure symptoms. CONCLUSION: This case highlights how a large ASD may initially mask symptoms of severe MS while simultaneously promoting progressive right-sided heart failure and secondary tricuspid valve disease. Early surveillance and timely intervention are essential to prevent irreversible cardiac remodeling in patients with LS.

Transcatheter aortic valve implantation (TAVI) is increasingly used for the treatment of aortic stenosis. Recently published guidelines favour now TAVI in all patients 70 years or older who have tricuspid valves and suitable anatomy. However, while a number of randomized controlled trials confirm that TAVI and surgical aortic valve replacement (SAVR) provide equivalent outcomes up to 5 years, data beyond 5 years are still scarce and real-world registry data report indeed conflicting results. Higher rates of complications after TAVI such as pacemaker requirement and paravalvular regurgitation, which have been shown to be associated with worse outcome may become more relevant after 5 years and still favour surgery on long-term. In addition, long-term durability data for TAVI are still insufficient. Thus, the question whether SAVR should still be the first choice for patients with a life expectance beyond 5 years is therefore justified. This debate summarizes the pros and cons for this claim.

Cardiac computed tomography (cCT) has emerged as a valuable imaging modality in veterinary cardiology and can be used to visualize and understand diseases that affect the mitral and tricuspid valves of dogs. While echocardiography remains the primary diagnostic tool for atrioventricular valve disease, cCT offers excellent spatial and temporal resolution, at the same time overcoming the limitations posed by acoustic windows. This review article describes the technical aspects of cCT in dogs emphasizing administration of contrast medium, electrocardiographic gating, and scan timing. Specific recommendations and protocols for imaging the atrioventricular valves in dogs are discussed. Clinical applications for cCT of the atrioventricular valves include evaluation of congenital malformations, such as dysplasia and stenosis, and assessment of acquired conditions like degenerative valve disease and neoplasia. Cardiac CT enables precise visualization of valve anatomy, chamber volumes, and may assist in postprocedural evaluations for transcatheter device placement and surgical implant integrity. Advanced postprocessing techniques - such as volume rendering, virtual reality, and three-dimensional printing - enhance anatomical understanding and procedural planning. Cardiac CT is a transformative tool in veterinary cardiology, offering unparalleled anatomical and functional insights. As mechanical therapies for atrioventricular valve disease become more common in veterinary practice, cCT is likely to become a required tool for pre procedural planning as it has been in human medicine.