Amaç: TEKHARF Çalýþmasý’nýn temelde Marmara ve Ýç Anadolu dýþýndaki bölgelerde oturan ve 2006 yazýnda taranan kohortunda tüm-nedenli, koroner kökenli ölüm ve koroner kalp hastalýðýna (KKH) ait insidans ve prevalans verileri incelendi.
Çalýþma planý: Taramada toplam 1585 kiþi (776 erkek, 809 kadýn; ort. yaþ 55.3±11.8) izlendi; bunlar ülke genelinde hayattaki TEKHARF kohortunun %49’unu oluþturuyordu. Ölüm konusunda birinci derece akraba ve/veya saðlýk ocaðý personelinden bilgi alýndý; yaþayanlarda bilgi edinmekten baþka, fizik muayene ve 12 derivasyonlu EKG kaydý yapýldý. Yeni koroner olay, son taramadan beri geliþen, ölüme yol açan veya açmayan miyokard infarktüsü, yeni stabil angina ve/veya miyokard iskemisi þeklinde tanýmlandý.
Bulgular: Örneklemden 946’sý muayene edildi, 599 kiþi hakkýnda bilgi edinildi ve 27 erkek ile 13 kadýnýn öldüðü belirlendi. Yeni takip olarak eklenen 2842 kiþi-yýlý ile, 1990 yýlýndan beri izlemede 45490 kiþi-yýlýna ulaþýldý. Ölümlerden 15’i KKH kökenli sayýldý. Son tarama döneminde yýllýk tüm ölüm oraný bin yetiþkinde 14.1, koroner mortalite binde 5.1 düzeyinde bulundu. Kýrk beþ ile 74 yaþ arasý kesimde toplam yýllýk mortalite son Türkiye taramasýnda binde 10.9’a (p=0.09), KKH’den ölüm prevalansý binde 5.6’ya geriledi. Gerilemeler kadýnlarda belirgindi. Ortalama ölüm yaþýnýn erkeklerde 67.1’ye, kadýnlarda 75.9’a uzadýðý görüldü. Koroner kalp hastalýðý prevalansý 39-49 yaþ grubunda %3, 50-59 yaþ grubunda %11, 60 yaþ ve üzerinde %27 bulundu. Bu prevalansýn ülkede 2.75 milyon erkek ve kadýný kapsadýðý tahminine varýldý.
Sonuç: Kýrk beþ ile 74 yaþ arasý kesimde tüm ölümler ile koroner kökenli ölümlerin özellikle kadýnlarda azalma eðilimi içinde olduðu ve ortalama ölüm yaþýnýn uzadýðý görülmektedir. Buna karþýlýk, yaþ gruplarýna özgü KKH prevalansý hýzla artma eðilimindedir.

Objectives: We analyzed all-cause and coronary mortality, incidence and prevalence of coronary heart disease (CHD) in a cohort of the Turkish Adult Risk Factor Study which was surveyed in the summer of 2006 essentially in geographic regions other than Marmara and Middle Anatolia.
Study design: The survey consisted of 1585 participants (776 men, 809 women; mean age 55.3±11.8 years), accounting for 49% of all living participants of the overall cohort. Information on death was obtained from first-degree relatives and/or health personnel of local health offices. Survivors were evaluated by history, physical examination, and 12-lead electrocardiography. New coronary event was defined as fatal or nonfatal myocardial infarction, new stable angina, and/or myocardial ischemia that had occurred after the former survey.
Results: Of the participants, 946 were examined, 599 subjects were evaluated on the basis of information gathered, and 40 deaths (27 men, 13 women) were documented. Cumulative follow-up of the survey starting from 1990 increased to 45,490 person-years with the addition of 2,842 person-years. Fifteen deaths were attributed to CHD. Annual overall mortality and coronary mortality rates were 14.1 and 5.1 per 1000 adults, respectively. In the 45 to 74 years age bracket, overall mortality declined to 10.9 (p=0.09) and coronary mortality to 5.6 per thousand. A decreasing trend in mortality was pronounced in women. The mean age at death increased to 67.1 years in men, and to 75.9 years in women. The prevalences of CHD were found to be 3%, 11%, and 27% in age groups of 39-49, 50-59, and ≥60 years, respectively, which corresponded to an estimated population of 2.75 million.
Conclusion: In the 45 to 74 years age bracket, overall and coronary deaths show a decreasing trend, particularly in women, which extends the mean age at death. However, the prevalence of CHD specific to age groups continues to rise, as well.

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Amaç: Ekokardiyografik olarak ölçülen transmitral basýnç farký mitral kapak hastalarýnýn kapak fonksiyonlarýný deðerlendirmede kullanýlan önemli bir yöntemdir. Geleneksel yöntemde modifiye Bernoulli eþitliðinden yararlanýlarak elde edilen ortalama transmitral basýnç farký, pulse Doppler sinyal sýnýrlarýnýn elle çizilmesine dayanýr. Son zamanlarda, bu ölçüm için sýrasýyla Devlin M ve ark. ve Yang SS tarafýndan daha kolay iki yöntem tanýmlanmýþtýr. Bu çalýþmada geleneksel yöntemle yeni yöntemlerin karþýlaþtýrýlmasý amaçlandý.
Çalýþma planý: Mitral darlýðý veya mitral kapak replasmaný nedeniyle mitral kapak üzerinde basýnç farký oluþan 78 hasta (55 kadýn, 23 erkek; ort. yaþ 43±14; daðýlým 17-78) prospektif olarak incelendi. Hastalarýn 31’inde atriyal fibrilasyon vardý. Tüm hastalarda geleneksel yöntemle ortalama transmitral basýnç farký bulundu. Yeni yöntemler için, pulse Doppler akým trasesinin sýnýrlarý elle çizilmeden, sadece tepe ve orta hýzlar ölçüldü, tanýmlanan formüller yardýmýyla ortalama transmitral basýnç farký hesaplandý. Transmitral basýnç farký ayrýca 14 hastada kardiyak kateterizasyon sýrasýnda da ölçüldü.
Bulgular: Standart yöntemle, birinci ve ikinci yöntemle ölçülen ortalama transmitral basýnç farklarý sýrasýyla 9.2±5.7 mmHg, 9.4±6.2 mmHg ve 10.6±6.6 mmHg bulundu. Yeni yöntemlerin standart yöntemle iyi bir korelasyon gösterdiði görüldü (her iki yöntem için, r=0.98, p<0.001). Altgrup incelemelerinde, ritim türü, mitral kapak alaný ve eþlik eden lezyon varlýðýnýn bu uyumun derecesini deðiþtirmediði görüldü. Standart yöntem, birinci ve ikinci yöntemlerle ölçülen ortalama transmitral basýnç farklarý, kardiyak kateterizasyon sonuçlarýyla da uyumlu bulundu (sýrasýyla, r=0.97, r=0.96, r=0.96).
Sonuç: Standart Doppler yönetimiyle ve iki yöntemle hesaplanan sonuçlarýn benzer bulunmasý nedeniyle, yeni yöntemlerin günlük klinik uygulamada transmitral basýnç farkýnýn hesaplanmasýný kolaylaþtýrdýðý söylenebilir.

Objectives: Mitral valve pressure gradient measured by echocardiography is a very useful method for the evaluation of mitral valve disease. The standard method is based on the calculation of mean mitral valve pressure gradient with the use of the modified Bernoulli equation, which includes manual drawing of the border of Doppler signal. Recently, two new methods have been described by Devlin M et al. and Yang SS, respectively. This study aimed to compare the results of the standard method with those of the new methods.
Study design: We prospectively studied 78 patients (55 women, 23 men; mean age 43±14 years; range 17 to 78 years) with mitral valve gradient either due to mitral stenosis or mitral valve replacement. Atrial fibrillation was present in 31 patients. The mean mitral valve gradient was measured using the standard method. As for the new methods, measurements of the mean mitral valve gradient were made without manual drawing of the border of Doppler signal and using the formulas which included the peak and trough velocities. Transmitral gradient was also obtained by cardiac catheterization in 14 patients.
Results: The mean transmitral pressure gradients were 9.2±5.7 mmHg, 9.4±6.2 mmHg, and 10.6±6.6 mmHg by the standard method, the first, and the second methods, respectively. The new methods were in good correlation with the standard method (for both methods, r=0.98, p<0.001). Subgroup analyses based on mitral valve area, rhythm, and the presence of associated valve lesions did not change the consistency of correlations. Transmitral gradients obtained by catheterization were also correlated with those obtained by the standard, first, and second methods (r=0.97, r=0.96, r=0.96, respectively).
Conclusion: Considering that the standard Doppler method and two different approaches yield similar results, the new approaches seem to facilitate the measurement of mitral valve gradient in daily clinical practice.

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Amaç: Bu çalýþmada, sað ventrikül çýkýþ yolu kaynaklý (SVÇY) sýk ventrikül erken vurularýnýn (VEV) kalbin diyastolik fonksiyonlarý üzerine etkisi ve VEV’lerin serum N-terminal pro B-tip natriüretik peptid (NT-proBNP) düzeyleriyle iliþkisi araþtýrýldý.
Çalýþma planý: Çalýþmaya 24 saatlik Holter monitörizasyonunda SVÇY kaynaklý sýk VEV saptanan, sol ventrikül sistolik fonksiyonlarý normal 33 semptomatik hasta (10 erkek, 23 kadýn; ort. yaþ 40±8) alýndý. Tüm olgularda ikiboyutlu transtorasik ekokardiyografi ve Doppler analizi yapýldý, mitral akým paternleri ve serum NT-proBNP düzeyleri deðerlendirildi. Sonuçlar 30 saðlýklý bireyden (9 erkek, 21 kadýn; ort. yaþ 37±9 yýl) oluþan kontrol grubuyla karþýlaþtýrýldý.
Bulgular: Hasta grubunda izovolumetrik gevþeme zamaný (IVGZ) (p<0.0001) ve E-dalga deselerasyon zamaný (EDZ) (p<0.0001) daha uzun, erken diyastolik dalganýn atriyal dalgaya oraný (E/A) daha düþük (p=0.001) ve NT-proBNP düzeyleri daha yüksek (p=0.016) bulundu. Mitral doluþ paterni kontrol grubunun tamamýnda normal iken, 13 hastada bozulmuþ relaksasyon ile uyumlu bulundu. Diyastolik disfonksiyon bulunan hastalarýn (n=13) NT-proBNP düzeyleri diyastolik disfonksiyon bulunmayanlara göre (n=20) daha yüksek (p=0.03), ortalama VEV sayýlarý daha fazlaydý (p=0.001). Ventrikül erken vuru sayýsý ile mitral E/A oraný arasýnda negatif, EDZ, IVGZ ve NT-proBNP düzeyi arasýnda pozitif korelasyon saptandý.
Sonuç: Yapýsal kalp hastalýðý bulunmayan kiþilerde SVÇY kaynaklý sýk VEV’ler diyastolik fonksiyonlarda bozulmaya neden olmaktadýr. N-terminal proBNP düzeyinin VEV sayýsý ile paralellik göstermesi, semptomatik hastalarda diyastolik disfonksiyonun bir göstergesi olarak kullanýlabileceðini düþündürmektedir.

Objectives: We investigated the effect of frequent ventricular premature beats (VPB) originating from the right ventricular outflow tract (RVOT) on diastolic functions and the relationship between VPBs and serum N-terminal pro-brain natriuretic peptide (NT-proBNP) levels.
Study design: The study included 33 symptomatic patients (10 males, 23 females; mean age 40±8 years) with normal left ventricular functions, who had frequent VPBs originating from the RVOT on 24-hour Holter monitoring. All the patients underwent 2D transthoracic echocardiography and Doppler analyses and mitral inflow patterns and serum NT-proBNP levels were evaluated. The results were compared with those of 30 healthy individuals (9 males, 21 females; 37±9 years).
Results: Compared to the controls, the patients had a longer isovolumetric relaxation time (IVRT) (p<0.0001) and E-wave deceleration time (EDT) (p<0.0001), a smaller ratio of early diastolic wave to atrial wave (E/A) (p=0.001), and higher NT-proBNP levels (p=0.016). While the mitral inflow pattern was normal in all the controls, it was associated with impaired relaxation in 13 patients. Patients with diastolic dysfunction (n=13) had higher NT-proBNP levels (p=0.03) and greater VPB counts (p=0.001) than those without diastolic dysfunction (n=20). The number of VPBs was inversely correlated with the mitral E/A ratio, and positively correlated with EDT, IVRT, and NT-proBNP levels.
Conclusion: Frequent VPBs from the RVOT cause deterioration in diastolic functions in patients without structural heart disease. Considering a parallel rise in NT-proBNP levels with the VPB count, NT-proBNP measurement can be used as a predictor of diastolic dysfunction in symptomatic patients.

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Amaç: Bu çalýþmada metabolik sendromun (MS) primer perkütan koroner giriþim (PKG) sonrasý erken dönemde majör kardiyak olay (MKO) geliþimi üzerine etkisi araþtýrýldý.
Çalýþma planý: Çalýþmaya, akut ST-segment yükselmeli miyokard infarktüsü geçiren ve ≤12 saat içinde hastaneye baþvurup primer PKG uygulanan 152 hasta (132 erkek, 20 kadýn; ort. yaþ 56.5±11) alýndý. Diyabetli hastalar çalýþma dýþý býrakýldý. Hastalar NCEP ATP III metabolik sendrom ölçütlerine göre MS grubu (n=69, %45.4) ve kontrol grubu (n=83, %54.6) olarak ikiye ayrýldý. Ýki grup klinik, anjiyografik, 12 derivasyonlu EKG bulgularý ve iþlem sonrasý ilk bir aylýk takipte MKO geliþimi (ölüm, tekrarlayan infarkt, tekrarlayan damar revaskülarizasyonu) açýsýndan karþýlaþtýrýldý.
Bulgular: Ýki grup infarkttan sorumlu arter ve hasta damar sayýsý açýsýndan benzer bulundu. Ýnfarkttan sorumlu arterde baþlangýç TIMI akým derecesi MS grubunda kontrol grubuna göre daha düþüktü (p=0.009). Ýþlem sonu TIMI akým derecesi iki grupta farklýlýk göstermedi (p=0.5). Ancak, iþlem sonrasý 90. dakikada saptanan ST-segment yüksekliðinde %50 veya daha fazla gerileme sýklýðý MS grubunda daha düþük bulundu (p=0.002). Bir aylýk takip sonunda kontrol grubunda hiçbir olguda MKO geliþmezken, MS grubunda dokuz hastada (%13) MKO görüldü (p=0.001).
Sonuç: Metabolik sendromlu hastalarda primer PKG sonrasý erken dönemde MKO geliþme riski daha fazladýr. Bu nedenle, primer PKG uygulamalarýnda MS varlýðý göz önüne alýnmalýdýr.

Objectives: This study sought to determine the effect of metabolic syndrome (MS) on the development of major adverse cardiac events (MACE) in the early period of primary coronary intervention (PCI).
Study design: The study included 152 patients (132 males, 20 females; mean age 56.5±11 years) who underwent primary PCI within ≤12 hours of acute myocardial infarction with ST-segment elevation. Patients with diabetes mellitus were excluded. The patients were divided into two groups according to whether they met the NCEP ATP III criteria for MS; hence, 69 patients (45.4%) with MS, and 83 patients (54.6%) without MS (controls). The two groups were compared with respect to clinical and angiographic features, ECG findings, and the development of MACE (death, reinfarction, and repeat vessel revascularization) at one month.
Results: The two groups were similar with respect to culprit arteries and the number of diseased vessels. Initial TIMI flow grade was lower in the MS group compared to controls (p=0.009), but the final TIMI flow grades were similar in both groups (p=0.5). However, patients with MS had a lower rate of ST-segment resolution ≥50% at 90 minutes (p=0.002). At one month follow-up, none of the patients in the control group had MACE, whereas nine patients (13%) in the MS group developed MACE (p=0.001).
Conclusion: Patients with MS have a higher risk for developing MACE after primary PCI. Therefore, the presence of MS should be taken into consideration in patients undergoing PCI.

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Amaç: Metabolik sendrom (MS), NCEP-ATP III ve Macchia ve ark. tarafýndan geliþtirilen MS skorlama yöntemine göre iki farklý biçimde tanýmlandý ve bu iki yöntemin koroner arter hastalýðý (KAH) ciddiyeti ve yaygýnlýðýný belirlemedeki etkinliði incelendi.
Çalýþma planý: Çalýþmaya KAH nedeniyle koroner anjiyografi yapýlan ardýþýk 158 hasta (103 erkek, 55 kadýn) alýndý. Metabolik sendrom, hem NCEP-ATP III'e, hem de MS skoruna göre deðerlendirildi. MS skorlamasý, yaþ, cinsiyet, beden kütle indeksi, hipertansiyon, HDL-kolesterol, trigliserid ve glukoz düzeylerine göre belirtilmiþ olan puanlarýn her bir hasta için ayrý ayrý toplanmasýyla hesaplandý. Koroner arter hastalýðý ciddiyeti ve yaygýnlýðý Gensini puanlamasýna göre deðerlendirildi. Hastalar önce Gensini skoruna göre ciddi KAH (≥20, n=69) ve hafif KAH (<20, n=89); sonra ROC analizinde kesim noktasý olarak bulunan MS skoruna göre, yüksek (≥27.5, n=103) ve düþük (<27.5, n=55) MS skoru varlýðýna göre gruplara ayrýldý.
Bulgular: Ciddi KAH olan grupta MS skoru hafif KAH grubuna göre anlamlý derecede daha yüksekti (p<0.001); buna karþýn, NCEP-ATP III'ün MS parametrelerinden glukoz ve trigliserid düzeyleri dýþýnda ve ayný yöntemle MS tanýsý konan hasta sayýsý açýsýndan iki grup arasýnda anlamlý farklýlýk yoktu. Yüksek MS skoru (≥27.5) olan hastalarda, düþük MS skoru olanlara göre Gensini skoru anlamlý derecede yüksekti (p=0.001). MS skoru ile KAH yaygýnlýðý ve ciddiyeti arasýnda anlamlý, orta derecede korelasyon vardý (r=0.347; p<0.001). Çokdeðiþkenli lojistik regresyon analizinde sadece yüksek MS skorunun KAH yaygýnlýðý ve ciddiyetini baðýmsýz olarak 3.4 kat artýrdýðý gözlendi (p=0.012; %95 güven aralýðý: 1.3-8.9).
Sonuç: MS skoru, MS'ye ait metabolik riskin, NCEP-ATP III ölçütlerinden daha iyi öngördürücüsü olabilir. MS skoru yüksek olan hastalarda daha yaygýn ve ciddi KAH olmasý beklenebilir.

Objectives: Definition of metabolic syndrome (MS) was made according to the NCEP-ATP III criteria and the new MS scoring system developed by Macchia et al., and the role of each method was assessed in predicting the severity and extent of coronary artery disease (CAD).
Study design: The study included 158 patients (103 males, 55 females) who underwent coronary angiography for CAD. Metabolic syndrome was identified according to both the NCEP-ATP III criteria and the MS score. MS scores were calculated by summing all the points rated for sex, age, body mass index, hypertension, and levels of HDL-cholesterol, triglyceride, and fasting glucose. The severity and extent of CAD were evaluated by the Gensini score. Patients were classified as having severe (≥20, n=69) or mild (<20, n=89) CAD according to the Gensini scores, and by high (≥27.5, n=103) and low (<27.5, n=55) MS scores, depending on the optimal cut-off point by ROC analysis.
Results: The mean MS score was significantly higher in patients having severe CAD than those having mild disease (p<0.001). However, these two groups did not differ significantly with respect to the number of patients diagnosed as having MS according to the NCEP-ATP III criteria and parameters thereof other than glucose and triglyceride levels. The Gensini score was significantly higher in patients having a MS score of ≥27.5 (p=0.001). There was a moderate correlation between the MS score and the severity and extent of CAD (r=0.347; p<0.001). Multivariate logistic regression analysis revealed that the MS score was the only independent factor that significantly increased the severity and extent of CAD by 3.4 times (p=0.012; %95 confidence interval: 1.3-8.9).
Conclusion: The MS score may be a better predictor of metabolic risk in MS patients than the NCEP-ATP III criteria. Patients with a high MS score are more likely to develop severe CAD.

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Otuz üç yaþýnda kadýn hasta doðumdan 20 gün sonra bilinç kaybý ve tam kalp bloku ile baþvurdu. Baþvuru sýrasýnda kan basýncý 70/50 mmHg, kalp hýzý 30/dk ölçüldü. Hastaya vakit kaybetmeden sað femoral ven yoluyla geçici transvenöz pacemaker takýldý. Hastanýn kan basýncý 100/70 mmHg’ye yükseldi, bilinci geri geldi. Ekokardiyografik incelemede sol ventrikül sistolik fonksiyonlarýnýn bozuk olduðu izlendi. Bir gün sonra, tam kalp bloku kendiliðinden düzelerek ritim sað dal bloklu sinus ritmine döndü. On gün sonra sað dal bloku kaybolurken sol ventrikül ejeksiyon fraksiyonunda da iyileþme görüldü. Bilgilerimize göre, peripartum kardiyomiyopatisi ile tam kalp blokunun bir arada görüldüðü bir olgu bildirilmemiþtir.

A 33-year-old woman presented with unconsciousness and complete heart block 20 days postpartum. On admission, her blood pressure was 70/50 mmHg and heart rate was 30/min. Immediately, a temporary transvenous pacemaker was inserted via the right femoral vein and her blood pressure increased to 100/70 mmHg with restoration of consciousness. Echocardiographic examination showed reduced left ventricular systolic function. The following day, complete heart block resolved spontaneously to sinus rhythm with right bundle branch block. Ten days later, right bundle branch block resolved together with improvement in left ventricular ejection fraction. To our knowledge, a case of peripartum cardiomyopathy presenting with complete heart block has not been reported.

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Kuadriküspid aort kapaðý oldukça nadir bir doðuþtan anomalidir. Önceleri otopsilerde, aort kapak replasmaný sýrasýnda ve aort kökü anjiyografisinde tesadüfen rastlanmakta iken, günümüzde transözofajiyal ekokardiyografideki geliþmeler sayesinde daha erken dönemlerde saptanabilmektedir. Kuadriküspid aort kapaðý, fonksiyonel olarak normal olabileceði gibi çoðunlukla aort yetmezliðine neden olmaktadýr. Otuz üç yaþýnda, akut romatizmal ateþ öyküsü olan erkek hastada dekompanse sol kalp yetersizliði saptandý. Transtorasik ekokardiyografide sol ventrikül dilatasyonu, mitral kapakta kalýnlaþma ve fibrozis, üçüncü derece aort yetersizliði ve kuadriküspid aort kapaðý saptandý. Koroner anjiyografide global hipokinezi, aort ve mitral kapak yetersizliði izlendi. Hastaya aort kapak replasmaný ve mitral kapak tamiri önerildi.

Quadricuspid aortic valve is a rare congenital malformation which used to be discovered incidentally at necropsy, or during aortic valve replacement surgery or aortic angiography until the recent advances in transesophegeal echocardiography. It mostly results in aortic insufficiency, though it may be functionally normal. Congestive heart failure was detected in a 33-year-old male patient with a history of acute rheumatic fever. Transthoracic echocardiography showed dilatation of the left ventricle, thickening and fibrosis of the mitral valve, third degree mitral regurgitation, and a quadricuspid aortic valve. Coronary angiography showed global hypokinesia, aortic and mitral insufficiency. Aortic valve replacement and mitral valve repair were recommended to the patient.

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Kýrk dört yaþýnda erkek hasta egzersiz sýrasýnda nefes darlýðý yakýnmasýyla baþvurdu. Transtorasik ikiboyutlu ekokardiyografide interventriküler septumun bazal bölgesinde 4.5x8 cm boyutunda kist hidatikle uyumlu kitle izlendi. Renkli Doppler ekokardiyografide bu bölgede türbülans akým ve hafif aort ve hafif mitral yetersizliði görüldü. Ýndirekt hemaglutinasyon inhibisyon testi pozitifti. Hasta cerrahi tedavi önerisini kabul etmedi. Bir ay sonra, hastanýn baþka bir merkezde kist hidatikle ilgili ameliyat sýrasýnda öldüðü öðrenildi.

A 44-year-old man presented with a complaint of dyspnea on exertion. Transthoracic two-dimensional echocardiography showed a cystic lesion in the basal region of the interventricular septum, 4.5x8 cm in size, suggesting a hydatid cyst. Color Doppler echocardiography demonstrated turbulent flow and mild aortic and mitral insufficiency. An indirect hemagglutination test yielded a positive result. The patient refused surgical treatment. A month later, it was found that he had died during surgery for the hydatid cyst at another center.

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Kardiyak iyon kanallarýnýn iþlevlerindeki bozukluklar elektrokardiyografik anormalliklerle ve aritmilerle kendini gösterir. Kardiyak iyon kanallarýndaki bu bozukluklar kardiyak kanalopatiler olarak tanýmlanmaktadýr. Yapýsal olarak normal kalplerde ortaya çýkan bu kalýtsal aritmojenik bozukluklara, özelleþmiþ iyon kanallarýný kodlayan genlerdeki mutasyonlarýn neden olduðu gösterilmiþtir. Bu bozukluklar arasýnda uzun QT sendromlarý, kýsa QT sendromlarý (SQTS), Brugada sendromu, ilerleyici kardiyak ileti defekti, idiyopatik hasta sinus sendromu, katekolaminerjik polimorfik ventrikül taþikardisi ve ailesel atriyal fibrilasyon bulunmaktadýr. Atriyal fibrilasyon ve ani kardiyak ölüme eðilimin yaný sýra anormal kýsa QT intervali (<300 msn) ile karakterize SQTS’ler bu grubun yeni üyeleridir. Bu sendromun görüldüðü hastalarda, kardiyak potasyum kanallarýný kodlayan genlerde üç farklý mutasyon bildirilmiþtir. Günümüzde, defibrilatör (ICD) takýlmasý ilk seçenek tedavidir. QT intervalini belirgin olarak uzatan tek ilacýn kinidin olduðunun gösterilmesinden dolayý, ICD takýlmasýný reddeden veya ICD'nin sýk þok uyguladýðý hastalara kinidin tedavisi önerilebilir. Bu makalede kardiyak kanalopatiler özetlenmiþ ve SQTS'nin tanýsý, patofizyolojisi ve tedavisi gözden geçirilmiþtir.

Disorders in the function of cardiac ion channels present as electrocardiographic abnormalities and arrhythmias and are described as cardiac channelopathies. Mutations in genes encoding specific ion channels have been shown to underlie these heritable arrhythmogenic disorders occurring in structurally normal hearts. These disorders include long QT syndromes, short QT syndromes (SQTS), Brugada syndrome, progressive cardiac conduction defect, idiopathic sick sinus syndrome, catecholaminergic polymorphic ventricular tachycardia, and familial atrial fibrillation. Short QT syndromes characterized by an abnormally short QT interval (<300 ms) and a propensity to atrial fibrillation and sudden cardiac death are new members of this group. Three distinct mutations in genes encoding cardiac potassium channels have been identified in this syndrome. Implantable cardioverter defibrillator (ICD) is presently the first choice of treatment. Because quinidine has been shown as the only drug that markedly prolongs the QT interval, this therapy can be considered in patients who refuse an ICD or in those who get frequent shocks from the ICD. This article aims to summarize cardiac channelopathies, with special reference to the diagnosis, pathophysiology, and treatment of SQTS

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Abstract | Full Text PDF

Abstract | Full Text PDF

Görüntülü Olgu Sunumu, özet yok

Case Image, no abstract.

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Daha öncesinden miyokard infarktüsü hikayesi olan 73 yaþýnda erkek hasta, giderek artan nefes darlýðý þikayeti ile hastanemize baþvurdu.

73-year-old man with a history of previous myocardial infarction was admitted to the hospital with progressive dyspnea.

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Abstract | Full Text PDF

Abstract | Full Text PDF