A Rare Case of ARCAPA in an Adult Patient With Severe Mitral Regurgitation

Introduction 

Mitral regurgitation is a common valvular heart disease that can lead to significant morbidity and mortality if left untreated. This case report describes the surgical management of severe mitral regurgitation in a patient with polycystic kidney disease and an anomalous right coronary artery. 

Case Presentation 

A 53-year-old male patient with a medical background of hypertension and polycystic kidney disease associated with chronic kidney failure presented to the authors’ hospital with symptomatic severe mitral regurgitation. The patient’s symptoms included shortness of breath, hemoptysis, and palpitation. Physical examination revealed an irregular pulse and a blowing systolic murmur at the apex radiating to the left axilla. Electrocardiogram  showed atrial fibrillation, and echocardiography confirmed the presence of severe mitral valve regurgitation due to a flail P2 segment of the posterior mitral valve leaflet. Additionally, an anomalous right coronary artery was detected during a coronary angiogram. 

Surgical Intervention 

The patient underwent surgical intervention, which involved mitral valve repair and correction of the anomalous right coronary artery. The proximal part of the right coronary artery was mobilized and divided, and multiple accessory arterial and venous channels were ligated. The remaining hole in the pulmonary artery was repaired with a synthetic pericardial patch. Mitral valve repair was performed, including the placement of synthetic neochordae and plication of the small cleft between the P2 and P3 segments. A semirigid annuloplasty ring was implanted, and the competency of the mitral valve was confirmed. The right coronary artery button was reimplanted to the proximal aorta, ensuring it was not twisted or kinked. 

Postoperative Course and Follow-up 

The patient’s recovery was unremarkable, and he did not require postoperative dialysis. He was discharged on the ninth postoperative day on anticoagulation for atrial fibrillation. At the one-month follow-up, the patient was doing well with no complaints. Echocardiographic assessment revealed successful mitral valve repair with trivial regurgitation. 

Conclusion 

This case highlights the successful surgical management of severe mitral regurgitation in a patient with polycystic kidney disease and an anomalous right coronary artery. The combination of mitral valve repair and correction of the anomalous coronary artery resulted in a favorable outcome for the patient. Long-term follow-up will be necessary to monitor the durability of the mitral valve repair and the patient’s overall cardiac function. 

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