先天性僧帽弁狭窄症

Coronary artery fistula (CAF) is a rare congenital coronary malformation. Sakakibara type B lesions, in which multiple coronary arteries drain concurrently into the coronary sinus (CS), are extremely uncommon, and cases initially presenting with high-burden premature ventricular complexes (PVCs) as the initial manifestation have rarely been reported. This paper reports a 63-year-old female patient presenting with intermittent palpitations for 1 year. A 24-hour ambulatory electrocardiogram (Holter) revealed a PVC burden of up to 40,804 complexes, with a total of 101,582 heartbeats recorded. Transthoracic echocardiography (TTE) demonstrated dilation of the left atrium, left ventricle and right atrium, moderate mitral and tricuspid regurgitation, dilated right coronary artery (RCA) with a right coronary artery-coronary sinus fistula, while lesions of the left circumflex artery (LCX) and second obtuse marginal branch (OM2) were missed. Three-dimensional coronary computed tomography angiography (CCTA) reconstruction showed tortuous and diffusely dilated LCX, OM2 and RCA, with aneurysms at the distal segments of all three vessels. The distal segments of the three vessels anastomosed with each other and drained into the CS. The dilated coronary arteries compressed the great cardiac vein, resulting in focal luminal stenosis. The patient's PVCs originated from the left coronary cusp. Radiofrequency catheter ablation completely relieved palpitations, and follow-up Holter at 3 months recorded only 1 PVC with a different origin from preoperative ectopy. Cardiac surgeons recommended surgical ligation of the fistula, but the patient opted for regular imaging follow-up and long-term clopidogrel antiplatelet therapy due to absence of myocardial ischemia, heart failure and other symptoms. This case indicates that three-dimensional CCTA reconstruction serves as the primary non-invasive modality for definitive diagnosis and therapeutic evaluation of complex CAF. Idiopathic PVCs originating from the aortic sinuses of Valsalva can coexist independently with CAF. Type B CAF with multiple feeding vessels draining into the CS represents a high-risk anatomical subtype, requiring long-term, individualized multidisciplinary management.

OBJECTIVE: To demonstrate the technique of totally endoscopic robotic repair of partial atrioventricular septal defect with complex mitral valve reconstruction in an adult patient. KEY STEPS: Totally endoscopic robotic approach with peripheral cannulation and bicaval control. Bovine pericardial patch closure of the primum atrial septal defect. Left atriotomy for optimal mitral valve exposure. Leaflet shaving, cleft closure, autologous pericardial augmentation, and flexible annuloplasty. POTENTIAL PITFALLS: Inadequate exposure may compromise assessment of the cleft and leaflet pathology. Careful leaflet remodeling, preservation of leaflet mobility, and avoidance of excessive tension during cleft closure are essential to prevent residual regurgitation or mitral stenosis. TAKE-HOME MESSAGES: Totally endoscopic robotic repair enables safe and reproducible treatment of partial atrioventricular septal defect with complex mitral valve reconstruction in selected adults. Enhanced visualization and instrument dexterity facilitate precise intracardiac repair while avoiding median sternotomy.

BACKGROUND: The treatment of primary mitral and secondary tricuspid regurgitation (MR, TR) in the setting of lung agenesis poses major technical challenges, and data on the feasibility of endoscopic tricuspid valve repair in adult congenital anatomy remain limited. CASE SUMMARY: We report the case of a 76-year-old male with severe primary MR due to Barlow's disease, torrential secondary TR, biatrial dilatation, persistent atrial fibrillation, and post-capillary pulmonary hypertension. Anatomical anomalies included dextrocardia, hypoplastic right lung, and scimitar vein syndrome. The patient had a history of surgical correction of an aortic coarctation at the age of 32 with residual stenosis but no proof of residual gradients in invasive assessment. Transcatheter repair was deemed unsuitable due to the massive dextrocardia, extensive leaflet pathology, and annular dilatation. The patient underwent endoscopic mitral and tricuspid repair with dual arterial and venous cannulation to avoid malperfusion of the upper body during extracorporeal circulation. Mitral valve repair included a surgical edge-to-edge repair, along with an implantation of an annuloplasty ring; secondary TR was repaired by ring annuloplasty too. Postoperatively, the patient experienced prolonged weaning and renal support. He was ultimately transferred to rehabilitation for uneventful recovery. Discharge echocardiography revealed good surgical results for the mitral valve and a residual moderate-to severe TR. DISCUSSION: The present case highlights that an endoscopic approach for the treatment of mitral and tricuspid valve pathologies can be used in selected patients with dextrocardia. Tricuspid annuloplasty may reduce but not abolish tricuspid regurgitation in a combination of primary, congenital, and secondary aetiologies.

BACKGROUND Congenital absence of the right coronary artery (RCA) is a rare anomaly often identified incidentally. We report a case of transient left ventricular systolic dysfunction followed by recovery of ejection fraction and new-onset mitral chordal rupture, which became the primary driver of pronounced N-terminal pro-B-type natriuretic peptide (NT-proBNP) elevation. The coexistence of 2 distinct pathologies complicated causal attribution; we discuss proposed mechanisms while recognizing their limitations. CASE REPORT A 58-year-old woman presented with exertional chest pain. At initial admission, she exhibited sinus tachycardia, elevated NT-proBNP (910 pg/mL), and reduced left ventricular ejection fraction (LVEF; 43% [M-mode]) with hypokinesis of the anterior wall and anteroseptum. Symptoms improved with anti-ischemic therapy. Three weeks later, LVEF recovered to 56% (biplane Simpson method), but NT-proBNP increased to 2440 pg/mL. Echocardiography revealed rupture of a small chord of the anterior mitral leaflet with mild-to-moderate regurgitation and elevated filling pressures (E/e'=13.8). Coronary angiography demonstrated congenital absence of the RCA ostium, with an enlarged left circumflex artery supplying the RCA territory and no evidence of atherosclerotic stenosis. Retrospective quantitative mitral regurgitation indices from archived images were examined. Management was conservative; symptoms resolved and NT-proBNP levels decreased during follow-up. CONCLUSIONS In patients with rare coronary anomalies, newly acquired common valvular disease may dominate the clinical presentation. Transient systolic dysfunction and subsequent chordal rupture occurred in our patient; a direct causal relationship remains speculative. Multimodality imaging is essential to accurately attribute hemodynamic changes and guide therapy. Long-term surveillance of coronary anatomy and valve function is recommended.