Epidemiological, Clinical and imaging features, and outcome of patients with Crisscross heart disease: A Systematic review of 46 published case reports

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Abstract Background and objectives The crisscross heart (CCH) is a rare congenital cardiac abnormality defined by the crossing of the inflow streams of the two ventricles due to the heart's apparent twisting about its long axis. Despite being considered a defect, the CCH is more of a spatial than a functional aberration, so the heart can still work normally. The severity of this anomaly is determined by the difficulty of knowing the obscurity of the etiology, the lack of early diagnosis, and the exacerbation of its symptoms. The choice of optimal surgical interventions for managing complex heart defects leads to a marked reduction in morbidity and mortality incidence. We conducted this study to provide a summary and critical evaluation of the existing evidence knowledge on the nature of this defect and to emphasize the importance of early diagnosis. Methods We searched PubMed, Scopus, Web of Science, and Embase databases in addition to cross-checking against the bibliographies of retrieved articles to identify additional reports until September 15th,2022. Results Our inclusion criteria are met by 46 case reports, which include 101 patients of both sexes ≥1 month of age, published from 1974 to 2021. CCH was diagnosed in 45 (44.6%) females and 56 (55.4%) males with a median age of 66 months [interquartile range (IQR): 12-216 months] who came from 19 countries worldwide. The common clinical features manifested with dyspnea in 17 (16.8%), cyanosis in 39 (38.6%), and tachycardia in 12 (11.9%) patients. The most common associated congenital heart diseases were ventricular septal defect in 85 (84.3%) patients, double outlet right ventricle in 52 (51.5%) patients, and pulmonary stenosis in 62 (61.4%) patients. The Surgical intervention took place in 65.4% of the patients. A two-ventricle repair may not be possible in most patients, and these patients are prepared for a Fontan-type surgery. Conclusion Understanding this complex congenital heart disease allows clinicians to provide their patients with the best care and prompt intervention. Further investigation of the histopathology pictures and genetic sequences is needed to determine a clear pathogenesis.

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