Noncompaction and dilated cardiomyopathy in carvajal syndrome

Carvajal syndrome involves multisystem manifestations, with prominent dermatologic and cardiovascular features. Unlike Naxos disease, which primarily affects the right ventricle, Carvajal syndrome predominantly involves the left ventricle, as observed in this case. Notably, left ventricular noncompaction was a striking feature in our patient, further exacerbating cardiac dysfunction and complicating the clinical course. Although noncompaction cardiomyopathy is less frequently reported in Carvajal syndrome, its pronounced presence in this case underscores the phenotypic variability and severity of myocardial involvement. Intensive care management with a multidisciplinary approach was essential in stabilising this patient. Genetic testing confirmed the diagnosis and highlighted the importance of molecular diagnostics in differentiating cardiocutaneous syndromes.