Crisscross Hearts in Adults: Echocardiographic Evaluation and Natural History

Deciphering the Mysteries of Crisscross Heart by Transthoracic Echocardiography

“…The clinical outcome of patients relies heavily on addressing the hemodynamic consequence of the associated lesions rather than the crisscross anatomy. All of our patients had abnormal VA connections (TGA or DORV) and VSD which are similar to the patterns described in two recently published series 4,9 . Seven patients received palliative procedures to relieve cyanosis mostly due to the presence of abnormal VA connections.…”

“…All of our patients had abnormal VA connections (TGA or DORV) and VSD which are similar to the patterns described in two recently published series. 4,9 Seven patients received palliative procedures to relieve cyanosis mostly due to the presence of abnormal VA connections. Biventricular repair is only feasible in three as the rest of patients either had RV hypoplasia (n = 3), AV valve straddling (n = 1), or significant pulmonary vascular disease (n = 3).…”

“…The rarity of the disease, distorted cardiac anatomy together with associated cardiac defects 3 all contribute to the difficulty in accurate diagnosis by sonographers and cardiologists. Accurate diagnosis of the condition is important as early palliative surgery has a favorable impact on functional status in adulthood 4 . Conventional catheterization 5–7 is not an ideal soft tissue imaging option for assessment of this complex congenital condition.…”

“…Accurate diagnosis of the condition is important as early palliative surgery has a favorable impact on functional status in adulthood. 4 Conventional catheterization [5][6][7] is not an ideal soft tissue imaging option for assessment of this complex congenital condition. Cardiac magnetic resonance (CMR) imaging 8,9 could be a potential reference standard for the condition but its role in screening is limited because of relative inaccessibility.…”

Echocardiographic characteristics of the criss-cross heart

“…Its frequency is no more than 8 per 1,000,000, and it is found in less than 0.1% of congenitally malformed hearts [3]. To date, nonetheless, at least 150 cases are described in the literature [4], and it has been shown that the essence of the lesion is twisted atrioventricular connections [5]. Patients with the criss-cross arrangement usually have numerous coexisting cardiac defects, such that accurate diagnosis is a significant challenge.…”