Murat ÇİÇEK, Oktay KORUN, Okan YURDAKÖK, Hüsnü Fırat ALTIN, Mehmet Akif ÖNALAN, Mustafa Orhan BULUT, Filiz İZGİ COŞKUN, Ahmet ŞAŞMAZEL, Fatih ÖZDEMİR, Numan Ali AYDEMİR

Total Anormal Pulmoner Venöz Dönüş Tek Merkez Sonuçlarımız

Giriş: Ülkemizde total anormal pulmoner venöz dönüş (TAPVD) onarım sonuçlarına ilişkin sınırlı veri bulunmaktadır. Bu çalışmanın amacı, kliniğimizde ameliyat edilen geniş bir TAPVD hasta serisinin erken postoperatif sonuçlarını değerlendirmektir. Hastalar ve Yöntem: Kliniğimizde Mayıs 2005-Mayıs 2021 tarihleri arasında TAPVD tamiri yapılan hastaların verileri hastane kayıtları kullanılarak geriye dönük olarak incelenmiştir. Bulgular: Yaşları 0 gün ile 39 yaş arasında (medyan 3 ay, IQR: 18 gün-9 ay), 92 erkek (%61) ve 58 kadın (%39) toplam 150 TAPVD hastasına cerrahi girişim uygulanmıştır. Ortanca hastanede kalış süresi 12 (IQR: 7-16) gündü. Genel mortalite %14 (21 hasta) idi. Ek kardiyak anomalili 32 hastadan 17 (%53)’si ve izole TAPVD’li 118 hastadan 4 (%3)’ü kaybedilmiştir. Ek kardiyak anomalilerin varlığı mortalite ile ilişkiliydi (p< 0.00001). Univentriküler hastalarda ölüm oranı %59 (10 hasta) bulunmuştur. Bu oran iki ventrikül hastalarının ölüm oranına göre daha yüksekti (%8; p< 0.00001). Postoperatif pulmoner venöz darlık nedeniyle tekrar ameliyat olan üç hasta vardı. Sonuç: Bu kohortta, izole ve biventriküler TAPVD hastalarında mortalite ve postoperatif pulmoner venöz stenoz oranları düşüktü. Tek ventrikül fizyolojisi ve/veya heterotaksisi olan hastalarda mortalite yüksekti. Bu durum pulmoner ve sistemik dolaşımlar arasındaki denge ile ilgili univentriküler fizyolojinin kendine özgü zorluklarından kaynaklanıyor olabilir. Preoperatif pulmoner venöz obstrüksiyonu olan olgularda artmış mortalite eğilimi, iyileştirilmesi gereken bir noktadır.

Totally Anomalous Pulmonary Venous Connection Outcomes from A Single Center

Introduction: There is limited data on totally anomalous pulmonary venous connection (TAPVC) repair results in our country. The aim of this study is to evaluate the early postoperative results of a large series of TAPVC patients operated in our clinic. Patients and Methods: The data of the patients who underwent TAPVC repair in our clinic between May 2005 and May 2021 were retrospectively reviewed using hospital records. Results: A total of 150 TAPVC patients aged between 0 days and 39 years (median 3 months, IQR: 18 days-9 months), 92 males (61%) and 58 females (39%) underwent surgical intervention. The median length of hospital stay was 12 (IQR: 7-16) days. Overall mortality was 14% (21 patients). Seventeen out of 32 patients with additional cardiac anomalies (53%) and 4 out of 118 patients with isolated TAPVC (3%) died. The presence of additional cardiac anomalies was associated with mortality (p< 0.00001). Mortality rate in univentricular patients was 59% (10 patients). This rate was higher in comparison to the mortality rate of biventricular patients (8%; p< 0.00001). Three patients were reoperated due to postoperative pulmonary venous stenosis. Conclusion: The TAPVC outcomes in isolated and biventricular patients were favorable with a low mortality and postoperative pulmonary venous stenosis in this cohort. Patients with a single ventricle physiology and/or heterotaxia had a significantly increased risk of mortality, which might be due to the intrinsic challenges of the univentricular physiology regarding the balance between the pulmonary and systemic circulations. Tendency for increased mortality in the cases with preoperative pulmonary venous obstruction is a potential target for improvement.

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1. Kirshbom PM, Myung RJ, Gaynor JW, Ittenbach RF, Paridon SM, DeCampli WM, et al. Preoperative pulmonary venous obstruction affects longterm outcome for survivors of total anomalous pulmonary venous connection repair. Ann Thorac Surg 2002;74:1616-20.
2. Karamlou T, Gurofsky R, Al Sukhni E, Coles JG, Williams WG, Caldarone CA, et al. Factors associated with mortality and reoperation in 377 children with totalanomalous pulmonary venous connection. Circulation 2007;115:1591-8.
3. Cooley D, Ochsner A. Corrections of total anamalous venous dranage. Surgery 1957;42:1014-7.
4. Clabby ML, Canter CL, Strauss AW, et al. Total anomalous pulmonary venous connections. In: Moller JH (ed). Surgery of Conjenital Heart Disease: Pediatric Care Consortium. New York: Armonk, 2005.
5. Öztürk E, Tanıdır İC, Ayyıldız P, Kıplapınar N, Özyılmaz İ, Haydin S, et al. Surgery-related complications and their management in total anomalous pulmonary venous return during intensive care unit stay. Turk Gogus Kalp Damar Cerrahisi Derg 2015;23:229-38.
6. Sarıtaş B, Çelik M, Tatar T, Özkan M, Tokel K, Aşlamacı MS. (2011). The outcome of the vertical vein left intact during the surgery for total anomalous venous connection and its effects on ventricular functions. Anadolu Kardiyoloji Dergisi 2011;11:638-42.
7. Sarıtaş B, Bolat B, Özkan S, Akay T, Vuran C, Özçobanoğlu S, et al. Total anormal pulmoner venöz dönüş anomalisinin cerrahi tedavisinde açık bırakılan vertikal ven ve/veya atriyal septal defektin etkisi. Turk Gogus Kalp Damar Cerrahisi Derg 2008;16:6-10.
8. Kınoğlu B, Sarıoğlu T, Türkoğlu H, Bilal S, Güden M Sarıoğlu A, et al. Total anormal pulmoner venöz dönüşlerde ameliyat sonrası erken ve geç dönem sonuçlar. Türk Kardiyol Dern Arş 1996;24:366-70.
9. Morales DL, Braud BE, Booth JH, Graves DE, Heinle JS, McKenzie ED, et al. Heterotaxy patients with total anomalous pulmonary venous return: improving surgical results. Ann Thorac Surg 2006;82:1621-7.
10. Hancock Friesen CL, Zurakowski D, Thiagarajan RR, Forbess JM, del Nido PJ, Mayer JE, et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg 2005;79:596-606.
11. Khan MS, Bryant 3rd R, Kim SH, Hill KD, Jacobs JP, Jacobs ML, et al. Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg 2015;99:2134-9.
12. Herlong JR, Jaggers JJ, Ungerleider RM. Congenital heart surgery nomenclature and database project: pulmonary venous anomalies. Ann Thorac Surg 2000;69(4 Suppl):S56-69.
13. Seale AN, Uemura H, Webber SA, Partridge J, Roughton M, Ho SY, et al.; British Congenital Cardiac Association. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation 2010;122:2718-26.
14. Jenkins KJ, Sanders SP, Orav EJ, Coleman EA, Mayer JE Jr, Colan SD. Individual pulmonary vein size and survival in infants with totally anomalous pulmonary venous connection. J Am Coll Cardiol 1993;22:201-6.
15. Bando K, Turrentine MW, Ensing GJ, Sun K, Sharp TG, Sekine Y, et al. Surgical management of total anomalous pulmonary venous connection: thirty-year trends. Circulation 1996;94(Suppl):II12-II16.
16. Yong MS, d’Udekem Y, Robertson T, Horton S, Dronavalli M, Brizard C, et al. Outcomes of surgery for simple total anomalous pulmonary venous drainage in neonates. Ann Thorac Surg 2011;91:1921-7.
17. Shi G, Zhu Z, Chen J, Ou Y, Hong H, Nie Z, et al. Total anomalous pulmonary venous connection: the current management strategies in a pediatric cohort of 768 patients. Circulation 2017;135:48-58.
18. Morozov AA, Movsesyan RR, Latypov AK, Martynova OA, Vasichkina ES. Peculiarities of histological structure of pulmonary veinsin patients with total anomalous pulmonary venous drainageas morphological substrates relating to formation of postoperative pulmonary venous obstruction. Bull Exp Biol Med 2020;168:699-703.
19. Caldarone CA, Najm HK, Kadletz M, Smallhorn JF, Freedom RM, Williams WG, et al. Relentless pulmonary vein stenosis after repair of total anomalous pulmonary venous drainage. Ann Thorac Surg 1998;66:1514-20.
20. Sinzobahamvya N, Arenz C, Brecher AM, Blaschczok HC, Urban AE. Early and long-term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants. Eur J Cardiothorac Surg 1996;10:43338.
21. Wu Y, Xin L, Zhou Y, Kuang H, Jin X, Li Y, et al. Is sutureless technique beneficial in the primary repair of total anomalous pulmonary venous connection? A systematic review and meta-analysis. Pediatr Cardiol 2019;40:881-91.
22. Liufu R, Shi G, Zhu F, Guan Y, Lu Z, Chen W, et al. Superior approach for supracardiac total anomalous pulmonary venous connection. Ann Thorac Surg 2018;105:1429-35.

ISSN: 2149-2972
Yayın Aralığı: Yılda 3 Sayı
Başlangıç: 1990
Yayıncı: Sağlık Bilimleri Üniversitesi, Kartal Koşuyolu Yüksek İhtisas Eğitim ve Araştırma Hastanesi