Tricuspid Atresia with Absent Pulmonary Valve and Intact Ventricular Septum: A Rare Association

Abstract

The absence of the pulmonary valve in connection with tricuspid atresia and intact ventricular septum is a rare malformation, associated with a poor prognosis. The right ventricle is severely hypertrophied, resembling a cardiac mass protruding into the left ventricular outflow tract. We report such a case that underwent successful palliation with a Blalock-Taussig shunt followed by a superior cavopulmonary anastomosis.

Tricuspid atresia with absent pulmonary valve and intact ventricular septum: successful bidirectional cavopulmonary anastomosis with complete exclusion of the right ventricle

“…[1][2][3] Only a few cases with successful surgical palliation have been reported in the literature. 2,4,[6][7][8] The present case shows that this rare CHD can be safely palliated in a staged approach towards the Fontan operation.…”

“…One of the characteristic morphologic features of this malformation is severely hypertrophied ventricular septum bulging into the left ventricular outflow tract (asymmetrical ventricular septal hypertrophy), which was also evident in our case. [1][2][3][4][5][6][7] This can contribute to the development of left ventricular outflow tract obstruction, which is hazardous for single ventricle physiology. In our case, left ventricular outflow tract obstruction became obvious after the operation, probably due to volume-unloading nature of the bidirectional cavopulmonary anastomosis and dynamic left ventricular wall motion.…”

“…Best et al reported "prophylactic" decompression of the right ventricle at the time of bidirectional cavopulmonary anastomosis by creating a ventricular septal defect to prevent the possibility of a hypertensive right ventricle bulging into the left ventricular outflow tract. 7 Another characteristic morphologic feature of this malformation is the right ventricular dysplasia, in which large segments of the right ventricular myocardium are replaced with sinusoids and fibroelastic tissue. 2,3,[5][6][7][8] This can cause variable degree of myocardial thinning and even aneurysmal change.…”

“…7 Another characteristic morphologic feature of this malformation is the right ventricular dysplasia, in which large segments of the right ventricular myocardium are replaced with sinusoids and fibroelastic tissue. 2,3,[5][6][7][8] This can cause variable degree of myocardial thinning and even aneurysmal change. A case with overt coronary-to-right ventricular fistulas has also been reported.…”

Functional near-tricuspid atresia in a patient with absent pulmonary valve and an intact ventricular septum

“…Absent pulmonary valve and intact ventricular septum is a rare malformation, with only 35 cases reported thus far, to our knowledge. 1,2 The condition can be complicated by tricuspid atresia or stenosis and right ventricle dysplasia. The syndrome is associated with a poor prognosis.…”