Mukopolisakkaridozlu hastalarda ekokardiyografi ve holter elektrokardiyografi bulgularının incelenmesi
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Date
2015-08-19
Authors
Aldudak, Bedri
Özbek, Mehmet Nuri
Demirbilek, Hüseyin
Saygı, Semra
Çelik, Muhittin
Kanğın, Murat
Journal Title
Journal ISSN
Volume Title
Publisher
Uludağ Üniversitesi
Abstract
Giriş: Mukopolisakkaridozlar (MPS) glikozaminoglikanların (GAG) yıkımını sağlayan enzimlerin fonksiyonel eksikliği nedeniyle ortaya çıkan kalıtsal lizozomal depo hastalıklarıdır. Kalp kapaklarında kalınlaşma, fonksiyon kaybı, iletim bozuklukları, koroner arter ve diğer damarların tutulumu görülebilir. Elektriksel açıdan iletken olmayan GAG’ların iletim bozukluklarına yol açtığı varsayılmaktadır. Kardiyak nedenli ölümlerin bir kısmından ritim bozuklukları sorumlu tutulmaktadır. Bu çalışmada Holter elektrokardiyografi (EKG) ile hastaların ritim durumunun araştırılması amaçlandı. Gereç ve Yöntem: Bu çalışmada 2010-2011 yılları arasında mukopolisakkaridoz tanısı ile takip edilmekte olan 17 hasta ve 17 kontrol olgu alındı. Olguların hepsine ekokardiyografik inceleme yapıldı. Bütün olgularda yüzey EKG ve 24 saatlik Holter EKG ile ritim durumu incelendi. Bulgular: MPS tanısı alan hastaların sekizi tip VI, dördü tip II, biri tip I, ikişer hasta tip III ve tip IV tanısı almıştı. Yaş ortalaması hasta grubunda 5,82±2,24 yıl idi. Kapak tutulumu %82 oranında saptandı. Mitral kapak (%73) birinci sırada, aort kapağı (%21) ikinci sıradaydı. Hasta grubunda maksimum kalp hızı 161/dk, ortalama kalp hızı 108/dk, minimum kalp hızı 81/dk, kontrol grubunda ise sırasıyla 151/dk, 96/dk, 70/dk olarak bulundu. Hasta grubunun kalp hızları kontrol grubunun kalp hızlarına göre yüksekti (p<0,05). Hasta grubunda sol ventrikül diyastol sonu çapı yüksek bulundu. Olgularda koroner iskemi veya aritmi saptanmadı. Sonuç: MPS’lerde kardiyak tutulum sıktır. Bilindiği üzere en sık tutulan kapak mitral, ikinci sırada aort kapağıdır. Bu çalışma, bildiğimiz kadarıyla Holter EKG inceleme yapılan ilk çalışma olmakla birlikte çocukluk yaş grubunda, özellikle erken dönemde enzim yerine koyma tedavisi alan hastalarda aritminin önemli bir sorun oluşturmadığını göstermektedir. Hasta grubunda ortalama kalp hızının yüksek bulunması kapak yetmezliği nedeniyle oluşan artmış volüm yüküne bağlanmıştır.
Introduction: Mucopolysaccharidosis (MPS) are lysosomal storage disorders characterized with the deficiency of enzymes involving in the destruction of glycosaminoglycans (GAG). Cardiac findings include cardiac valve thickening and dysfunction, conduction abnormalities, coronary artery and other vessels involvement. It is thought that GAGs, which are non-conductive electrically, causes failure in cardiac conduction. Arrhythmias are considered to be responsible from deaths of cardiac origin. In this study cardiac rhythm and structures were evaluated using Holter electrocardiography (ECG) and transthoracic echocardiography. Materials and Methods: Seventeen patients who were followed with the diagnosis of MPS and 17 healthy controls were enrolled in this study in 2010 and 2011. All cases were examined with transthoracic echocardiography. Cardiac rhythms were evaluated using a 24 hour Holter ECG. Results: The mean age was 5.82±2.24 in patient group. Valve involvement was observed in 82%. The mitral valve was the most commonly (73%) affected followed by aortic valve (21%). There was significant heart rate variations between patients and control group, in favour of patient group (p<0.05). The left ventricular end-diastolic diameter in patient group was found higher. Coronary ischemia or arrhythmias were not detected. Conclusions: Cardiac involvement in MPSs is frequent. To our knowledge this is the first study evaluating cardiac rhythms of children with MPS by 24 hour Holter ECG screening. Our findings suggested that arrhythmia is not an important medical problem in pediatric patients, especially in whom enzyme replacement therapy have been commenced at early ages. The high heart rate in patient group was considered as a result of increased cardiac volume due to valvular insufficiency.
Introduction: Mucopolysaccharidosis (MPS) are lysosomal storage disorders characterized with the deficiency of enzymes involving in the destruction of glycosaminoglycans (GAG). Cardiac findings include cardiac valve thickening and dysfunction, conduction abnormalities, coronary artery and other vessels involvement. It is thought that GAGs, which are non-conductive electrically, causes failure in cardiac conduction. Arrhythmias are considered to be responsible from deaths of cardiac origin. In this study cardiac rhythm and structures were evaluated using Holter electrocardiography (ECG) and transthoracic echocardiography. Materials and Methods: Seventeen patients who were followed with the diagnosis of MPS and 17 healthy controls were enrolled in this study in 2010 and 2011. All cases were examined with transthoracic echocardiography. Cardiac rhythms were evaluated using a 24 hour Holter ECG. Results: The mean age was 5.82±2.24 in patient group. Valve involvement was observed in 82%. The mitral valve was the most commonly (73%) affected followed by aortic valve (21%). There was significant heart rate variations between patients and control group, in favour of patient group (p<0.05). The left ventricular end-diastolic diameter in patient group was found higher. Coronary ischemia or arrhythmias were not detected. Conclusions: Cardiac involvement in MPSs is frequent. To our knowledge this is the first study evaluating cardiac rhythms of children with MPS by 24 hour Holter ECG screening. Our findings suggested that arrhythmia is not an important medical problem in pediatric patients, especially in whom enzyme replacement therapy have been commenced at early ages. The high heart rate in patient group was considered as a result of increased cardiac volume due to valvular insufficiency.
Description
Keywords
Mukopolisakkaridozla, Kardiyak tutulum, Ritim bozukluğu, Mucopolysaccharidoses, Cardiac involvement, Arrhythmia
Citation
Albudak, B. vd. (2015). "Mukopolisakkaridozlu hastalarda ekokardiyografi ve holter elektrokardiyografi bulgularının incelenmesi". Güncel Pediatri, 13(3), 165-170.