Absent pulmonary valve with tricuspid atresia/stenosis: literature review with new three long-term cases

• Published in: Heart and vessels Vol. 37; no. 1; pp. 142 - 151
• Main Authors: Kawasaki, Yuki, Murakami, Yosuke, Fujino, Mitsuhiro, Sasaki, Takeshi, Nakamura, Kae, Yoshida, Yoko, Suzuki, Tsugutoshi, Nishigaki, Kyoichi, Ehara, Eiji
• Format: Journal Article
• Language: English
• Published: Tokyo Springer Japan 2022; Springer Nature B.V
• Subjects: Aorta; Arrhythmia; Arteries; Biomedical Engineering and Bioengineering; Cardiac arrhythmia; Cardiac Surgery; Cardiology; Congenital diseases; Constriction, Pathologic; Coronary artery; Coronary vessels; Heart; Heart surgery; Humans; Literature reviews; Medical records; Medicine; Medicine & Public Health; Neurological complications; Original Article; Outflow; Patients; Pulmonary arteries; Pulmonary artery; Pulmonary Atresia; Pulmonary Valve - diagnostic imaging; Pulmonary Valve - surgery; Retrospective Studies; Septum; Stenosis; Tetralogy of Fallot; Tricuspid Atresia; Vascular Surgery; Ventricle; Absent pulmonary valve; Intact ventricular septum; Tricuspid valve atresia/stenosis; Fontan
• ISSN: 0910-8327; 1615-2573
• DOI: 10.1007/s00380-021-01887-y

Absent pulmonary valve (APV) syndrome with tricuspid atresia or tricuspid stenosis (TA/TS) is an extremely rare malformation recently reported as a variant of APV with intact ventricular septum (VS). The condition, however, has univentricular physiology and unique structural and clinical features. The purpose of this study was to update the current knowledge about this condition by describing long-term outcomes of three new cases and reviewing the available literatures. A systematic literature search was performed to collect clinical and anatomical data of APV with TA/TS. Institutional medical records were retrospectively reviewed to identify APV with TA/TS patients. In a total of 62 (59 reported and 3 new) cases, patent ductus arteriosus was present in 98% of APV patients with TA/TS. A large ventricular septal defect, dilatation of the pulmonary arteries, which is typically found in APV with tetralogy of Fallot, and respiratory distress at birth were rarely reported. Most of the recent cases were successfully managed by the Glenn or Fontan procedure. Coronary artery anomaly and ventricular arrhythmia were more frequently reported as the cause of death or severe neurological sequelae (9/16 and 3/8, respectively). Additional surgical intervention was required in the mid/long-term period in three cases due to left-ventricular outflow obstruction and in two due to aortic dilatation. The Fontan and Glenn procedures improved the survival in the last two decades. In addition to coronary artery anomaly and ventricular arrhythmia, left-ventricular outflow tract obstruction and aortic dilatation should be carefully monitored.