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Spinal muscular atrophy (SMA), an autosomal recessive disease caused by a decrease in levels of the survival motor neuron (SMN) protein, is the most common genetic cause of infant mortality. Although neuromuscular pathology is the most severe feature ofSMA, other organs and tissues, including the heart, are also known to be affected in both patients and animal models. Here, we provide new insights into changes occurring in the heart, predominantly at pre‐ and early symptomatic ages, in the Taiwanese mouse model of severeSMA. Thinning of the interventricular septum and dilation of the ventricles occurred at pre‐ and early symptomatic ages. However, the left ventricular wall was significantly thinner inSMAmice from birth, occurring prior to any overt neuromuscular symptoms. Alterations in collagenIVprotein from birth indicated changes to the basement membrane and contributed to the abnormal arrangement of cardiomyocytes inSMAhearts. This raises the possibility that developmental defects, occurring prenatally, may contribute to cardiac pathology inSMA. In addition, cardiomyocytes inSMAhearts exhibited oxidative stress at pre‐symptomatic ages and increased apoptosis during early symptomatic stages of disease. Heart microvasculature was similarly decreased at an early symptomatic age, likely contributing to the oxidative stress and apoptosis phenotypes observed. Finally, an increased incidence of blood retention inSMAhearts post‐fixation suggests the likelihood of functional defects, resulting in blood pooling. These pathologies mirror dilated cardiomyopathy, with clear consequences for heart function that would likely contribute to potential heart failure. Our findings add significant additional experimental evidence in support of the requirement to develop systemic therapies forSMAcapable of treating non‐neuromuscular pathologies.