A case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricular fistula.
Satoshi FujitaHideki TatewakiYusaku NagatomoAkira ShiosePublished in: General thoracic and cardiovascular surgery (2021)
Tricuspid atresia with pulmonary valve absence is a rare malformation characterized by left ventricular outflow obstruction due to asymmetrical ventricular septal hypertrophy and associated with a poor prognosis. Coexisting coronary-right ventricular fistula is rarely described. We encountered a case of tricuspid atresia with pulmonary valve absence coexisting coronary-right ventricle fistula that successfully underwent Fontan completion. Right ventricle dilatation due to coronary-right ventricle fistula was observed in addition to mass like ventricular septal hypertrophy protruding into the left ventricular outflow tract. Right ventricle reduction and prevention of progressive left ventricular outflow obstruction were achieved by closure of the coronary-right ventricle fistula closure and plication of the right ventricle with the bidirectional Glenn procedure.
Keyphrases
- mitral valve
- left ventricular
- aortic stenosis
- pulmonary hypertension
- hypertrophic cardiomyopathy
- coronary artery
- pulmonary artery
- coronary artery disease
- poor prognosis
- left atrial
- aortic valve
- transcatheter aortic valve replacement
- ejection fraction
- cardiac resynchronization therapy
- heart failure
- acute myocardial infarction
- long non coding rna
- pulmonary arterial hypertension
- minimally invasive