Tricuspid atresia associated with aortopulmonary window: diagnostic and therapeutic dilemmas

Abstract

The association of tricuspid atresia with aortopulmonary window is exceptionally rare. We report a patient with tricuspid atresia, normally related great arteries, non-restrictive ventricular septal defect, and no pulmonary stenosis (type IC) with an aortopulmonary window who underwent successful initial surgical palliation. The unique anatomical feature of this case, other than the presence of the aortopulmonary window, was the absence of pulmonary stenosis at the level of either the ventricular septal defect or the pulmonary valve. All other reported cases have described some degree of restriction of anterograde pulmonary flow due to pulmonary stenosis or atresia.

Uncommon Association of Aortopulmonary Window in a Patient with Complex Univentricular Heart and CHARGE Syndrome.

“…There were two earlier case reports with APW in the setting of complex univentricular heart, one with tricuspid atresia and the other had double inlet ventricle with large ventricular septal defect. 3,4 Univentricular hearts are uncommon and there is controversy about the definition, classification, and nomenclature. Van Praagh et al 5 has described them as hearts with one ventricular chamber that receives both atrioventricular valves or a common AV valve, excluding mitral and tricuspid atresia.…”

“…CHARGE syndrome initially was defined as a non-random association of anomalies (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness). 3,4 The cardiac anomalies are present in up to 75% of these patients. Septal defects and tetralogy of Fallot are most common cardiac defects in CHARGE syndrome.…”

“…Only a handful of case reports of APW associated with complex single ventricle lesions have been described in the literature. 3,4 To our knowledge, there is no literature on association of CHARGE syndrome (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness) with complex univentricular heart and APW. We report a case of unique combination of large APW in a complex univentricular heart of undetermined morphology with malposed great arteries in a neonate with absent thymus and CHARGE syndrome.…”

Tricuspid atresia: Where are we now?

“…69 In this study, the authors conclude that the Kawashima operation is a safe and effective method of treating bilateral SVCs and enables preparation for future total cavopulmonary shunting. 69 Milovanovic et al 70 report the rare occurrence of TA with an aortopulmonary window or connection between the aorta and pulmonary trunk. They subsequently suggest that the initial stage of palliation should involve full separation of the aorta and pulmonary artery using patch angioplasty.…”

“…Milovanovic et al 70 report the rare occurrence of TA with an aortopulmonary window or connection between the aorta and pulmonary trunk. They subsequently suggest that the initial stage of palliation should involve full separation of the aorta and pulmonary artery using patch angioplasty 70 . Following this, a systemic‐to‐pulmonary shunt (such as the MBTS) is placed, with the aim of establishing a full Fontan circulation in the future.…”