Criss-cross heart: Transthoracic echocardiographic features.

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.

Prenatal Diagnosis of the Criss-Cross Heart.

We sought to describe the prenatal features and postnatal outcomes of the "criss-cross heart" (CCH) with crossing atrioventricular connections as encountered in 5 cases and to review the literature regarding fetal diagnoses of this lesion. We reviewed the clinical history and fetal echocardiograms of 5 patients with a diagnosis of CCH encountered in our institution from 2010 to 2015. Affected pregnancies were identified through the University of Alberta fetal cardiology database. Fetal and postnatal echocardiograms, prenatal and postnatal clinical records, autopsy, and surgical reports were reviewed. Over the past 5 years, major fetal congenital heart disease was identified in 448 pregnancies in the province of Alberta. Of these, 5 had CCH. All 5 were referred for suspected congenital heart disease, and none had significant extracardiac pathology. Detailed methodical 2-dimensional and color Doppler fetal echocardiography defined the complex diagnoses that were subsequently confirmed postnatally (n = 4) and at fetal autopsy (n = 1). Prenatal diagnosis correctly predicted postnatal presentation and outcomes in 1 with double outlet right ventricle and coarctation, 2 presenting with complete (D) transposition of the great arteries physiology, and 1 presenting as corrected (L) transposition. Three required single ventricle palliation and 1 underwent an arterial switch procedure. In conclusion, detailed evaluation of fetal CCH can result in correct anatomical and pathophysiological diagnoses. It demands a full understanding of the anatomy and pathophysiology to predict accurately the presentation at birth for accurate counseling and planning perinatal management.

Successful ablation of atrioventricular node reentry tachycardia in a patient with crisscross heart and situs inversus levocardia.

Crisscross heart is a complex congenital anomaly that is rarely seen in the absence of important associated structural defects. We describe a 41-year-old woman with recurrent dizziness and tachycardia but no previous cardiovascular symptoms. Narrow-QRS tachycardia was detected, and magnetic resonance imaging showed situs inversus levocardia with a circulatory pattern typical of crisscross heart. Electrophysiologic study revealed atypical atrioventricular (AV) node reentry tachycardia of the "fast-slow" type. Despite the unusual anatomy, we successfully modified the AV node physiology by ablating the "slow AV node inputs" with the guidance of a multielectrode basket catheter in the anatomic right atrium.

[A rationale for the surgical treatment of congenital heart diseases with different variants of disorders of the atrioventricular connections (criss-cross heart)]. / Obosnovanie khirurgicheskogo lecheniia vrozhdennykh porokov serdtsa pri razlichnykh variantakh narushenii atrioventrikuliarnykh soedinenii (criss-cross serdtse).

The authors studied the angiocardiographic picture in different variants of criss-cross atrioventricular connections in 24 patients. This is the greatest number of cases described in the world literature. The tactics of surgical treatment in various types of a criss-cross heart is substantiated on the grounds of analysis of hemodynamics and concomitant congenital heart diseases. Operation on a patient with a criss-cross heart should consist in correction of the concomitant anomalies and, if necessary, it should be supplemented by intracardiac correction of the blood flow (Mustard's operation). Fontana's operation is the only method of surgical treatment of patients with a complicated complex of anomalies.