Dilate kardiyomiyopatinin nadir ancak tedavi edilebilir bir nedeni olan alcapa sendromlu altı olgunun değerlendirilmesi
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Date
2020
Authors
Özcan, Nur
Kızılkaya, Metehan
Özlem Mehtap, Bostan
Journal Title
Journal ISSN
Volume Title
Publisher
Bursa Uludağ Üniversitesi
Abstract
Sol koroner arterin pulmoner arterden köken aldığı ALCAPA (anomalousorigin of the left coronary artery from the pulmonary artery) sendromu, nadir görülen ve cerrahi tedavi ile düzelebilen bir doğuştan kalp anomalisidir. Tüm doğuştan kalp hastalıklarının %0,25- 0,50’sini oluşturmaktadır. Olgular yenidoğan döneminde genellikle semptomsuz seyrederler. Kalp yetersizliği semptomları olan olgularda dilate kardiyomiyopati (DKMP) ve mitral kapak yetersizliği (MY) saptanır. DKMP’nin nadir bir nedeni olan ALCAPA’da hayatın ilk yılında erken cerrahi tedavi ile prognoz oldukça iyidir. Cerrahi tedavi olmayan hastalarda mortalite yüksektir. Merkezimizde Ocak 2005-Aralık 2017 tarihleri arasında DKMP tanısı konulan ve etiyolojik değerlendirmede ALCAPA sendromu saptanan altı olgu retrospektif olarak değerlendirildi. Olguların 5’i kız, 1’i erkekti. Ortalama tanı yaşları 4,75 ay idi. Tüm olgularda başvuruda kalp yetersizliği bulgu ve semptomları mevcuttu. Olguların hepsinde elektrokardiyografide (EKG) D1, aVL ile göğüs derivasyonlarında iskemi bulguları mevcuttu. Olguların tümünde ekokardiyografide (EKO) sol ventriküldilatasyonu ile mitral yetersizlik saptandı. Tüm olgularda tanısal kateter anjiyografi ile ALCAPA tanısı doğrulandı. Tüm olgulara reimplantasyon yöntemi ile düzeltme ameliyatı yapıldı. Olguların 4’ü cerrahi tedavi sonrası tamamen düzeldi. Bir olgu operasyondan 5 gün sonra kaybedildi. Bir olgu ise cerrahi tedavi sonrası kardiyak fonksiyonlarında düzelme olmaması nedeniyle medikal tedavi ile izlenmektedir Dilatekardiyomyopati tanısı konulan olguların etiyolojisinde ALCAPA sendromu mutlaka düşünülmeli ve erken tanı ve tedaviyle tamamen iyileşebilen bir hastalık olduğu bilinmelidir.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac anomaly that can be cured with surgical treatment. It accounts for 0.25 to 0.5% of all congenital heart diseases. ALCAPA is generally asymptomatic in the neonatal period. Dilated cardiomyopathy (DCM) and mitral valve insufficiency (MVI) are detected in patients with symptoms of heart failure. ALCAPA, which is a rare cause of DCM, early surgical treatment provides quite good prognosis in the first year of life. However, mortality is high in patientswho do not receive surgical treatment. Six patients diagnosed with DCM at our clinic between January, 2005 and December, 2017 and detected with ALCAPA syndrome in their etiological assessment we reevaluated retrospectively Of the 6 patients, 5 were female and 1 was male. The average age of diagnosis was 4,75 months. All patients had the signs and symptoms of heart failure at admission. All cases had the signs of ischemia in the chest leads, D1 andaVL in the electrocardiograms (ECGs). In al lcases, echocardiography (ECHO) showed mitral insufficiency and the dilation of the left ventricle and the mean ejection fraction was 29,3% (18-39%), and the mean shortening fraction was 12,6% (8-19%) in theECHOs. Diagnostic catheter angiography was performed for all patients and the diagnosis of ALCAPA was confirmed. All patients under went correction surgery with there implantation method. Four of the patients completely recovered after surgery. One patient died 5 days after the surgery. One patient is being followed up with medical treatment since there was no improvement in her cardiac functions after surgery. The ALCAPA syndrome should definitely be considered in th eetiology of cases diagnosed with dilated cardiomyopathy and it should be kept in mind that ALCAPA is completely treatable disease with early diagnosis and treatment
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac anomaly that can be cured with surgical treatment. It accounts for 0.25 to 0.5% of all congenital heart diseases. ALCAPA is generally asymptomatic in the neonatal period. Dilated cardiomyopathy (DCM) and mitral valve insufficiency (MVI) are detected in patients with symptoms of heart failure. ALCAPA, which is a rare cause of DCM, early surgical treatment provides quite good prognosis in the first year of life. However, mortality is high in patientswho do not receive surgical treatment. Six patients diagnosed with DCM at our clinic between January, 2005 and December, 2017 and detected with ALCAPA syndrome in their etiological assessment we reevaluated retrospectively Of the 6 patients, 5 were female and 1 was male. The average age of diagnosis was 4,75 months. All patients had the signs and symptoms of heart failure at admission. All cases had the signs of ischemia in the chest leads, D1 andaVL in the electrocardiograms (ECGs). In al lcases, echocardiography (ECHO) showed mitral insufficiency and the dilation of the left ventricle and the mean ejection fraction was 29,3% (18-39%), and the mean shortening fraction was 12,6% (8-19%) in theECHOs. Diagnostic catheter angiography was performed for all patients and the diagnosis of ALCAPA was confirmed. All patients under went correction surgery with there implantation method. Four of the patients completely recovered after surgery. One patient died 5 days after the surgery. One patient is being followed up with medical treatment since there was no improvement in her cardiac functions after surgery. The ALCAPA syndrome should definitely be considered in th eetiology of cases diagnosed with dilated cardiomyopathy and it should be kept in mind that ALCAPA is completely treatable disease with early diagnosis and treatment
Description
Keywords
Pulmoner arterden çıkan anormal sol koroner arter sendromu, Anomalous origin of the left coronary artery from the pulmonary artery, ALCAPA sendromu, Dilatekardiyomiyopati, Kalp yetersizliği, ALCAPA syndrome, Dilated cardiomyopathy, Cardiac failure
Citation
Özcan, N. vd. (2020). "Dilate kardiyomiyopatinin nadir ancak tedavi edilebilir bir nedeni olan alcapa sendromlu altı olgunun değerlendirilmesi". Bursa Uludağ Üniversitesi Güncel Pediatri, 18(3), 290-299.