Nadir Bir Kardiyomiyopati Türü: İzole Sağ Ventrikül Nonkompaksiyonel Kardiyomiyopati
Özet
Sağ ventriküler nonkompaksiyonel kardiyomiyopati, embriyogenez sırasında miyokardiyal kompaksiyonun tamamlanamaması sonucu gelişen, belirgin trabekülasyonlar ve derin intertrabeküler reseslerle karakterize nadir bir kardiyomiyopati fenotipidir. Efor dispnesi, senkop ve kardiyak aritmiler gibi farklı klinik bulgularla prezente olabilmesi ve nadir görülmesi tanıyı güçleştirmektedir. Bu yazıda, 49 yaşında kadın hastada kardiyak manyetik rezonans görüntüleme ile tanı konulan izole sağ ventriküler nonkompaksiyonel kardiyomiyopati olgusu ve tanı-tedavi süreci sunulmaktadır.
Right ventricular noncompaction cardiomyopathy is a rare cardiomyopathic phenotype characterized by prominent trabeculations and deep intertrabecular recesses resulting from incomplete myocardial compaction during embryogenesis. Its clinical presentation may vary, including exertional dyspnea, syncope, and cardiac arrhythmias, and its rarity further complicates the diagnostic process. In this report, we present a 49-year-old female patient diagnosed with isolated right ventricular noncompaction cardiomyopathy by cardiac magnetic resonance imaging, and we discuss the diagnostic and therapeutic approach.
Referanslar
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