Transposition of the great vessels and intact ventricular septum: is there an age limit for the arterial switch? Personal experience and review of the literature.

OBJECTIVE: Prognosis of the transposition of the great arteries has completely changed since the introduction of the arterial switch. Time limit to perform this intervention is still controversial. The aim of this study is to demonstrate the early and late outcome of primary arterial switch operation beyond the age of months. METHODS: We included all patients with the diagnosis of transposition of the great arteries with intact ventricular septum beyond the age of 8 weeks who underwent primary arterial switch operation. The procedures were performed by the same surgeon, in two different institutes. Patients who had transposition of the great arteries and associated anomalies (except atrial septal defect and persistent arterial duct) were excluded. Ventricular shape, geometry, and mass were not considered during the decision on procedure type. RESULTS: In the study, 11 patients with the diagnosis of simple d-transposition of the great arteries beyond 8 weeks were undergone primary arterial switch operation with a mean age of 90.63 days (60-137 days), and 7 patients had a Rashkind procedure. All patients had squashed left ventricle shape with preserved function. The sternum was left open in 10 patients. Extracorporeal membrane oxygenation support was necessary in 45.45% of cases. The mean mechanical ventilation time was 7.27 days (1-16 days). No mortality was recorded until now. Post-operatory left ventricular function was preserved in 90.9% of the patients. Only one patient had mild myocardial dysfunction at the time of discharge. CONCLUSIONS: Primary arterial switch procedure can still be the best surgical option in patients with the diagnosis of transposition of the great arteries with intact ventricular septum beyond 8 weeks of age, providing that mechanical circulatory support and an expert cardiac intensive care unit service are available.

Aorto-Right Ventricular Tunnel: A Case Series and Literature Review.

Aorto-right ventricular tunnel (ARVT) is a rare cardiac congenital anomaly where an extracardiac channel connects the ascending aorta above the sinutubular junction to the right ventricle. This defect is caused by an abnormal development of the cushions of the aorto-pulmonary outflow tract. A case series and literature review are described. Two cases of ARVT are described. A literature review was conducted, in which 31 cases were reported. In our 2 cases, both ARVTs connected the ascending aorta above the left aortic sinus to the right ventricle (one to the right ventricular outflow tract and one to the right ventricular apex). Both patients underwent successful surgical correction by patch closure of both tunnel orifices, with uneventful postoperative course. Of the 31 ARVT cases described in our review, only 10 patients (32.3%) had an anatomy similar to the 2 cases described. Coronary artery anomalies can be associated, as reported in our 2 patients and in 16 cases (51.6%) in the review. Surgical correction can be achieved by direct closure or, more often, by patch closure of one or both tunnel orifices, depending mostly on coronary anatomy. Two cases of transcatheter device closure were described in literature, in favorable anatomy cases. Careful attention is required during repair to avoid coronary lesions, due to the high incidence of comorbid coronary anomalies.

Isolated Congenital Coronary Ostial Stenosis in a Young Infant: A Case Report.

Coronary ostial stenosis is a rare congenital cardiac anomaly, frequently associated with hypoplasia of the proximal coronary artery. This condition is potentially life-threatening, as it may present with myocardial ischemia and sudden death. We present a case of left coronary ostial stenosis in a 48-day-old infant symptomatic for sudden cardiac arrest, who successfully underwent surgical angioplasty. Any cardiac arrest in a neonate or young infant should raise suspicion of coronary ostial stenosis/atresia, considering the difficulty in diagnosing this congenital heart defect.

Double-Outlet Right Atrium: Review of a Rare Anomaly With an Exemplary Case.

BACKGROUND: Double-outlet right atrium (DORA) is a rare congenital cardiac defect, where the right atrium (RA) is connected to both the right ventricle (RV) and the left ventricle (LV). Double-outlet right atrium is classified into two types, each containing two subtypes: malaligned atrial septum with common or single atrioventricular (AV) valve and malaligned ventricular septum (VS) with adequate or inadequate RV. The VS type is characterized by straddling right AV valve (RAVV) with intact VS, resulting in two RAVV orifices. METHODS: We report a case of "acquired DORA" in a 17-year-old male patient previously treated with subaortic VSD closure. At admission, we diagnosed DORA with VS malalignment and adequate RV. The patient had 2 orifices within the RAVV, connecting the RA to both the RV (via RAVV orifice 1) and the LV (via RAVV orifice 2). The latter was insufficient, with severe LV to RA shunt. A review of the literature indexed in PubMed and Scopus databases was undertaken. RESULTS: The patient underwent biventricular repair through closure of the RAVV orifice 2 with a pericardial patch. Pacemaker implantation for complete AV block was necessary. Postoperative course and follow-up were regular. The literature review showed 39 cases of DORA, of which 8 had a malaligned VS and an adequate RV, as in our case. CONCLUSION: Acquired DORA is an interesting post-surgical variant of a rare congenital heart defect. Biventricular repair is a feasible and viable option, which involves closing the third AV orifice. Careful attention is required in order to avoid injury to the conduction system.