Neonatal Flail Tricuspid Valve: Diagnosis and Management

Loftus, Patrick D.; Arrington, Cammon B.; Kaza, Aditya K.

doi:10.1016/j.athoracsur.2013.11.003

Cited by 6 publications

(15 citation statements)

References 5 publications

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“…leaflet secondary to chordae rupture is a rare, life-threatening event. [1][2][3] This case is of interest because of the possible involvement of maternal antiphospholipid syndrome, not previously reported in the literature.…”

Section: T Ricuspid Regurgitation Due To Flail Anteriormentioning

confidence: 80%

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Flail anterior tricuspid valve leaflet in a neonate: association with maternal antiphospholipid syndrome

2017

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This brief report describes a case of flail anterior tricuspid valve leaflet in a neonate associated with maternal antiphospholipid syndrome. Fetal echocardiography at 27 weeks of gestation showed competent atrioventricular valves with biventricular echogenic chordae. Fetal distress was noted at delivery, and echocardiography showed a flail anterior leaflet of the tricuspid valve with severe regurgitation. Possible causation and implications of maternal antiphospholipid syndrome are discussed.

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Section: T Ricuspid Regurgitation Due To Flail Anteriormentioning

confidence: 80%

“…Flail tricuspid valve leaflet secondary to papillary muscle rupture is a rare condition with perinatal mortality up to 80%. [1][2][3] Early recognition and treatment is imperative for good outcomes. 3 Haemodynamically, the presentation can vary from virtually asymptomatic to extreme cyanosis and cardiac arrest shortly after birth.…”

Section: Discussionmentioning

confidence: 99%

Flail anterior tricuspid valve leaflet in a neonate: association with maternal antiphospholipid syndrome

2017

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“…Alternatively, the ductus arteriosus can be maintained by timely prostaglandin E1 infusion to stabilize the baby. Potential causes of chordae or papillary muscle rupture of tricuspid valve in the neonate include myocardial ischemia due to birth asphyxia, premature ductal closure, congenital endocarditis, maternal autoimmune disease, thromboembolism, traumatic rupture during birth process, or other obscure causes (4,5). In general, the anterior papillary muscle of the tricuspid valve is susceptible to the ischemia because of its high oxygen demand, diastolic coronary perfusion during high right ventricular pressure, and its distal extreme of the coronary circulation.…”

Section: Discussionmentioning

confidence: 99%

“…The patient may be suffered from severe cyanosis and profound distress when associated with ductal closure because of the unique hemodynamic characteristics mimic to that of the pulmonary atresia with intact ventricular septum (3). When the ductus closed abruptly, extracorporeal membranous oxygenation support is necessary with subsequent emergent surgical intervention for life-saving (3)(4)(5). Alternatively, the ductus arteriosus can be maintained by timely prostaglandin E1 infusion to stabilize the baby.…”

Section: Discussionmentioning

confidence: 99%

Successful repair of neonatal tricuspid regurgitation due to chordae rupture

2018

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IntroductionFunctional tricuspid regurgitation (TR) is transient and benign in stressed infants. In contrast, severe TR caused by a flail leaflet is a rare cause of neonatal cyanosis. The outcome may be fatal if not recognized early (1,2). We report on a full-term female neonate presented with cyanosis caused by severe TR due to anterior leaflet chordal rupture. After initial stabilization by prostaglandin E1 infusion for keeping ductal patency, successful early repair was achieved with polytetrafluoroethylene artificial chordae implantation. Early diagnosis and timely surgery can be lifesaving. Case presentationA 3,044-gm full term female was born by Cesarean section because of fetal distress and meconium stain. The first and fifth minutes Apgar score was 5 and 9. On physical examination, a grade III/VI pansystolic murmur was noted diffusely. She was cyanotic and the oxygen saturation was 85%. A two-dimensional echocardiography showed severe TR due to a flail and thickened anterior leaflet (Figure 1A,B) with the calculated tricuspid Z score of 1.28, and large patent ductus arteriosus. The antegrade pulmonary flow was compromised and a right-to-left shunt through the patent foramen ovale was noted. The hemodynamic characteristics were quite similar to that of the pulmonary atresia with intact ventricular septum.At 6-day-old, cyanosis got worse due to impending ductal closure. She was intubated and prostaglandin E1 was infused (0.2 µg/kg/min), then the condition was stabilized and surgery was carried. Under mild hypothermia with cold crystalloid cardioplegic cardiac arrest, the patent ductus was ligated and the right atrium was opened. A flail tricuspid anterior leaflet due to extensive marginal chordae rupture was confirmed. The papillary muscle showed no ischemic change. The length of the septal leaflet chordae was measured as the reference and two loops of 6-0 expanded polytetrafluoroethylene artificial chordae were implanted over the unsupported margin of the anterior leaflet. A tricuspid annuloplasty was not necessary because the annulus was not dilated. The patent foramen ovale was not closed for pop-off purpose. Nitric oxide was not needed and under minimal inotrope support, the patient was recovered uneventfully. She was extubated 7 days after the operation Abstract: Neonatal rupture of the chordae of tricuspid valve with severe regurgitation is rare and disastrous. We report on a full-term female neonate presented with cyanosis caused by severe tricuspid regurgitation (TR) due to anterior leaflet chordal rupture. After initial stabilization by prostaglandin E1 infusion, successful early repair was achieved with artificial chordae implantation. The unique pathophysiology and the therapeutic strategy of this situation will be described.

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“…Isolated TR requiring repair in infancy is a rare entity, with only scattered case reports in the literature. 1,2 The use of neochordae in valve repair of infants or young patients is effective in achieving immediate postoperative valve competency. However, long-term competency of such repair is unknown due to the rapid increase in heart size through maturation and somatic growth.…”

Section: Commentmentioning

confidence: 99%

Tricuspid Valve Repair in Infancy Using Neochordae: Three-Dimensional Echocardiographic Imaging

Martin

Khoo

Smallhorn

et al. 2017

World J Pediatr Congenit Heart Surg

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Tricuspid regurgitation (TR) in infancy poses a surgical challenge. Both two- and three-dimensional echocardiography (3DE) can provide detailed information about the mechanism(s) of valve failure and insights into valve adaptation during follow-up. We report two patients who underwent tricuspid valve repair using Gore-Tex neochordae, repairs which were facilitated by and assessed with 3DE. Both infants had less than mild residual TR and no valve tethering at hospital discharge. Furthermore, follow-up 3DEs have helped to confirm valve competence, lack of tethering, and growth of the valve and valve apparatus.

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Neonatal Flail Tricuspid Valve: Diagnosis and Management

Cited by 6 publications

References 5 publications

Flail anterior tricuspid valve leaflet in a neonate: association with maternal antiphospholipid syndrome

Flail anterior tricuspid valve leaflet in a neonate: association with maternal antiphospholipid syndrome

Successful repair of neonatal tricuspid regurgitation due to chordae rupture

Tricuspid Valve Repair in Infancy Using Neochordae: Three-Dimensional Echocardiographic Imaging

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