Kaan KIRALİ, Ramazan AKDEMİR, Esat AKINCI, Cevat YAKUT, Yücel ÖZEN, Mehmet BALKANAY, Gökhan İPEK, Denyan MANSUROĞLU

Mitral kapak ön yaprakçığına ait kleft ve eşlik eden atriyal septumdaki defektlerin kombine onarımı

Amaç: Doğumsal mitral yetmezliğinin önemli nedenlerinden biri olan mitral kapak ön yaprakçığına ait kleft, sıklıkla atriyal septumdaki defektlerle birliktedir. Materyal ve Metod: 1985 ile Nisan 2001 tarihleri arasında 53 hasta, mitral kapak ön yaprakçığına ait kleft ve eşlik eden atriyal septumdaki defekt nedeniyle ameliyat edildi. Hastaların 30'u kadın (%56.6) ve 23'ü erkek (%43.4) olup hastaların yaş ortalaması 21.6 ± 12.2 yıl (2-55) idi. Atriyal septal defekt 49 hastada (%92.4) primum, 4 olguda (%7.6) sekundum tipindeydi. Sağ atriyoventriküler kapakta kleft 2 hastada mevcuttu. Hastaların ikisi dışında hepsi sinüs ritminde idi. Fonksiyonel kapasite 10 hastada azalmıştı (NYHA klas III). Hastalardaki mitral yetmezliği ortalama 2.3 ± 0.7 derece (1-4 derece olarak) idi. Atriyal septal defekt, olguların hepsinde sentetik veya perikardiyal yama ile onarıldı. Mitral kapaktaki yarık ise primer kapatıldı. Koroner sinüs drenajı üç vakada sol atriyum tarafında bırakıldı. Hastalar ortalama 6.5 ± 3.8 yıl (0.2-15), toplam 348 hasta yılı izlendi. Bulgular: Erken ve geç mortaliteye rastlanmadı. Postoperatif üç hastada (%5.7) tam AV-blok nedeniyle geçici kalp pili desteği gerekti, ancak sadece bir hastada (%1.9) kalıcı pil takılması gerekti. Postoperatif yapılan ekokardiyografik kontrollerde reopere edilen iki hasta dışındaki 51 hastada mitral yetmezliği ikinci derecenin altında idi. Mitral yetmezliği nedeniyle iki hasta (%3.8) reoperasyona alınarak birine mitral kapak replasmanı, diğerine tekrar kleft onarım uygulandı. Reoperasyona alınan hastalardan ikincisinde perikardiyal yama açılması da mevcuttu, primer onarıldı. Reoperasyondan bağımsız yaşam beklentisi 14.5 yıl için %91.7 ± 5.8 olarak bulundu. Sonuç: Doğumsal mitral kapak ön yaprakçığına ait kleft ve eşlik eden atriyal septumdaki defektin tedavisi cerrahidir. Tanısı gecikmiş hastalar konjestif kalp yetmezliği gelişmeden ileri yaşlarda da başarı ile ameliyat edilebilir. Bu kompleks patolojinin en önemli komplikasyonu ileride gerekebilecek reoperasyondur. Bu nedenle uzun dönem yaşam kalitesini etkileyen en önemli faktör mitral kapak tamirinin tam olmasıdır.

Combined surgical repair of anterior mitral leaflet cleft associated with atrial septal defect

Background: Anterior mitral leaflet cleft, which is one of the most important causes for congenital mitral regurgitation, is usually associated with interatrial septal defects. Methods: Fifty-three patients with anterior mitral leaflet cleft associated with interatrial septal defects were operated between 1985 and April 2001. There were 30 (56.6%) women and 23 (43.4%) men with a mean age 21.6 ± 12.2 years (range 2 to 55 years). Atrial septal defect was primum type in 49 (92.4%) patients and secundum type in 4 (7.6%) patients. There were two cleft on the right atrioventricular valve. Fifty-one patients had sinus rhythm. Functional capacity decreased in 10 patients (NYHA class III). The mean mitral regurgitation was 2.3 ± 0.7 degree (range 1 to 4). Pericardial or prosthetic patch was used to close atrial septal defects. All clefts were closed with interrupted suture technique. Coronary sinus was left on the left side in three patients. Total follow-up was 348 patient-years, with a mean of 6.5 ± 3.8 years (range, 0.2 to 15 years). Results: There was no early or late mortality. Transient atrioventricular block developed in three (5.7%) patients in the early postoperative period and only one (1.9%) patient required replacement of a permanent pacemaker. Fifty-one patients had mild or no mitral regurgitation after operation. Two (3.8%) patients underwent reoperation, and one patient received mitral valve replacement, and re-closure of residual cleft with residual atrial septal defect through pericardial patch was performed in the other. Actuarial freedom form reoperation was 91.7% ± 5.8% at 14.5 years. Conclusions: Treatment of congenital anterior mitral leaflet cleft associated with interatrial septal defect is surgical. Patients, who are diagnosed late, can be operated successfully before congestive heart failure develops. The most important late complication is necessity of a reoperation. The most significant factor effecting long-term outcome is the successfull repair of anterior mitral leaflet cleft.

PDF

___

1. Carpentier A. Congenital malformations of the mitral valve. In: Stark J, de Leval M, eds. Surgery for Congenital Heart Defects. London: Grune and Stratton, 1994:599-614.
2. Mitruka SN, Lamberti JJ. Congenital heart surgery nomenclature and database project: Mitral valve disease. Ann Thorac Surg 2000;69:S132-46.
3. Jacobs JP, Quintessenza JA, Burke RP, Mavroudis C. Congenital heart surgery nomenclature and database project: Atrial septal defect. Ann Thorac Surg 2000;69:S18-24.
4. Jacobs JP, Burke RP, Quintessenza JA, Mavroudis C. Congenital heart surgery nomenclature and database project: Atrioventricular canal defect. Ann Thorac Surg 2000;69:S36-43.
5. Kent SM, Markwood TT, Vernalis MN, Tighe JF, Jr. Cleft posterior mitral valve leaflet associated with counterclockwise papillary muscle malrotation. J Am Soc Echocardiogr 2001;14:303-4.
6. Mohanty SR, Choudhary SK, Ramamurthy S, Kumar AS. Isolated congenital anterior mitral leaflet cleft: Arare cause of mitral insufficiency. J Heart Valve Dis 1999;8:67-70.
7. Sigfusson G, Ettedgui JA, Silverman NH, Anderson RH. Is a cleft in the anterior leaflet of an otherwise normal mitral valve an atrioventricular canal malformation? J Am Coll Cardiol 1995;26:508-15.
8. Najm HK, Williams WG, Chuaratanaphong S, Watzka SB, Coles JG, Freedom RM. Primum atrial septal defect in children: Early results, risk factors, and freedom form reoperation. Ann Thorac Surg1998;66:829-35.
9. Burke RP, Horvath K, Landzberg M, Hyde P, Collins JJ Jr, Cohn LH. Long-term follow-up after surgical repair of ostium primum atrial septal defect in adults. J Am Coll Cardiol 1996;27:696-9.
10. Agny M, Çobanoğlu A. Repair of partial atrioventricular septal defect in children less than five years of age: Late results. Ann Thorac Surg 1999;67:1412-4.
11. Kuralay E, zal E, Demirkılıç U, Cingiz F, Tatar H. Left atrioventricular valve repair technique in partial atrioventricular septal defects. Ann Thorac Surg 1999;68:1746-50.
12. Baufreton C, Journois D, Leca F, Khoury W, Tamisier D, Vouhe P. Ten-year experience with surgical treatment of partial atrioventricular septal defect: Risk factors in early postoperative period. J Thorac Cardiocasc Surg 1996;112:14-20.
13. Ceithaml EL, Midgley FM, Perry LW. Long-term results after surgical repair of incomplete endocardial cushion defects. Ann Thorac Surg 1989;48:413-6.
14. Abbruzzese PA, Napoleone A, Bini M, Annecchino FP, Merlo M, Prezan L. Late left atrioventricular valve insufficiency after repair of partial atrioventricular septal defects: Anatomical and surgical determinants. Ann Thorac Surg 1990;49:111-4.