Criss-cross heart: Transthoracic echocardiographic features

Abstract

Objective: To study the echocardiographic features of criss-cross heart (CCH), a congenital cardiac anomaly characterized by crossed ventricular inflow streams, in Indian patients.

Methods: In this retrospective observational study, all pediatric echocardiograms performed in a single tertiary care institution in South India over a three-year period were scrutinized for a diagnosis of CCH. Demographic, clinical and echocardiographic data were collected from patients' medical records and echocardiographic database. Crossed ventricular inflow streams was identified when there was inability to visualize both atrio-ventricular valves in a single imaging plane in cardiac four chamber view.

Results: CCH was diagnosed in five patients from 10,500 pediatric echocardiographic studies. The age at diagnosis ranged from one month to 8 years. Cyanosis was present in all but one of the five cases. Crossed ventricular inflow streams was present by definition in all cases, whereas superior-inferior ventricular relationship was present in only three cases. All cases were associated with ventricular septal defects. Atrio-ventricular discordance was seen in three cases and concordance in two. Ventriculo-arterial discordance was seen in three cases, concordance in one and double outlet right ventricle in one. Three cases had pulmonary stenosis and the other two had pulmonary arterial hypertension. Straddling of AV valve was observed in four cases and hypoplastic aortic arch in one case.

Conclusion: CCH is an extremely rare congenital cardiac anomaly. Superior-inferior ventricular relationship often co-exists with CCH, but is not necessarily present in all cases. CCH requires early diagnosis because of its common association with diverse cardiac anomalies.

Keywords: Criss-cross heart; Echocardiography; Heart defects congenital.

Fig 1

A. Two-dimensional and B. color Doppler transthoracic echocardiographic images obtained in apical four-chamber view showing twisted and discordant atrio-ventricular connections with the right atrium (RA) draining into the contra-laterally located morphological left ventricle (LV).

Fig. 2

A. Two-dimensional and B. color Doppler transthoracic echocardiographic images obtained in apical four-chamber view showing twisted and discordant atrio-ventricular connections with the left atrium (LA) draining into the contra-laterally located morphological right ventricle (RV).

Fig. 3

Two-dimensional transthoracic echocardiographic image obtained in parasternal short-axis view showing horizontal orientation of the inter-ventricular septum and superior-inferior ventricular relationship.

Fig. 4

Two-dimensional transthoracic echocardiographic image obtained in parasternal short axis view showing vertical orientation of the inter-ventricular septum and side-by-side ventricular relationship.