Tricuspid atresia: Where are we now?

Abstract

Tricuspid atresia (TA) is a complex congenital heart disease that presents with cyanosis in the neonatal period. It is invariably fatal if left untreated and requires multiple stages of palliation. Early recognition and timely surgical intervention are therefore pivotal in the management of these infants. This literature review considers the pathophysiology, presentation, investigations, and classification of TA. Moreover, it discusses the evidence upon which the latest medical and surgical treatments are based, as well as numerous recent case reports. Further work is needed to elucidate the etiology of TA, clarify the role of pharmacotherapy, and optimize the surgical management that these patients receive.

Prevalence of critical congenital heart defects and selected co‐occurring congenital anomalies, 2014–2018: A U.S. population‐based study

“…The high prevalence of co-occurring septal defects among identified CCHD cases is likely the result of two key processes: first, a common developmental pathway and, second, a strong survival advantage conferred by the septal defect (Mostefa-Kara, Houyel, & Bonnet, 2018). For example, while tricuspid atresia may co-occur at random with septal defects among embryos, those surviving to diagnosis are more likely to have a septal defect or other defect (e.g., d-TGA), which provides a bypass for deoxygenated blood around the absent tricuspid valve (Mostefa-Kara et al, 2018;Sumal, Kyriacou, & Mostafa, 2020). While the fetal circulatory pathways (i.e., foramen ovale and ductus arteriosus) help bypass obstructions to pulmonary circulation, in infants without an additional bypass from septal or other cardiac defects, the closure of these pathways after birth can lead to rapid cardiovascular collapse (Remien & Majmundar, 2021;Sumal et al, 2020).…”

“…For example, while tricuspid atresia may co-occur at random with septal defects among embryos, those surviving to diagnosis are more likely to have a septal defect or other defect (e.g., d-TGA), which provides a bypass for deoxygenated blood around the absent tricuspid valve (Mostefa-Kara et al, 2018;Sumal, Kyriacou, & Mostafa, 2020). While the fetal circulatory pathways (i.e., foramen ovale and ductus arteriosus) help bypass obstructions to pulmonary circulation, in infants without an additional bypass from septal or other cardiac defects, the closure of these pathways after birth can lead to rapid cardiovascular collapse (Remien & Majmundar, 2021;Sumal et al, 2020). However, infants with such bypasses may seem normal after birth; it is these infants that pulse oximetry screening aims to capture so that interventions can begin before complications arise (Oster et al, 2016;Sumal et al, 2020).…”

“…While the fetal circulatory pathways (i.e., foramen ovale and ductus arteriosus) help bypass obstructions to pulmonary circulation, in infants without an additional bypass from septal or other cardiac defects, the closure of these pathways after birth can lead to rapid cardiovascular collapse (Remien & Majmundar, 2021;Sumal et al, 2020). However, infants with such bypasses may seem normal after birth; it is these infants that pulse oximetry screening aims to capture so that interventions can begin before complications arise (Oster et al, 2016;Sumal et al, 2020).…”

The management of newborns with critical congenital heart diseases prior to transport to a cardiac center

“…Rarely, the atrial communication is restrictive and the newborn will need balloon atrial septostomy. The connection of the great arteries and the presence of pulmonary stenosis are the determinants of the newborn hemodynamics [15]. If there is pulmonary atresia or critical pulmonary stenosis, the PDA must be kept open to provide for pulmonary blood flow.…”

Interventional Management of Acute Thrombosis of the Modified Blalock-taussig Shunt in a Case of Tricuspid Atresia

“…Shunt creation between the pulmonary and systemic circulations has been one of the biggest steps in the management of cyanotic congenital heart diseases (CCHD) [1]. The modified Blalock-Taussig shunt (mBTS) is a tubular conduit, made of polytetrafluoroethylene, interposed between the right subclavian artery/brachiocephalic trunk and the right pulmonary artery [2]. Initially used for the treatment of pulmonary stenosis/atresia associated with tetralogy of Fallot, implantation of a mBTS rapidly became one of the first palliative steps in the treatment of more complex CCHD, including univentricular physiology, such as tricuspid atresia [2].…”